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Sample records for muscular ventricular septal

  1. Intraoperative closure of infant multiple muscular ventricular septal defects with Amplatzer occluder

    International Nuclear Information System (INIS)

    Liu Jinfen; Gao Wei; Zhu Zhongqun; Chen Huiwen; Zhang Yuqi

    2005-01-01

    Objective: To report the preliminary experience of intraoperative hybrid therapy for closure of multiple muscular ventricular septal defects (VSD) in a small infant. Methods: After median sternotomy, a AGA Amplatzer occluder was introduced through right ventricular surface to close 2 muscular ventricular septal defects under transesophageal echocardiographic guidance. Results: The infant survived after the treatment without residual shunting, and rehabilitated rapidly. Conclusions: Intraoperative hybrid therapy with combined surgical technique and interventional procedure for closure of multiple muscular VSD in small infant is a safe and effective method. (authors)

  2. Percutaneous treatment of atrial septal defects, muscular ventricular septal defects and patent ductus arteriosus in infants under one year of age.

    Science.gov (United States)

    Prada, Fredy; Mortera, Carlos; Bartrons, Joaquim; Rissech, Miguel; Jiménez, Lorenzo; Carretero, Juan; Llevadias, Judit; Araica, Mireya

    2009-09-01

    Amplatzer devices are used for the percutaneous closure of ostium secundum atrial septal defects, muscular ventricular septal defects and patent ductus arteriosus. However, very little experience has been gained in using these devices in infants under 1 year of age. Between January 2001 and January 2008, 22 symptomatic infants aged under 1 year underwent percutaneous treatment: three had an ostium secundum atrial septal defect, 15 had patent ductus arteriosus, and four had a muscular ventricular septal defect. All the procedures were completed successfully. No immediate or medium-term complications were observed. Closure of these types of defect using an Amplatzer device in infants under 1 year of age, who would otherwise require surgery, is a safe and effective procedure.

  3. Epicardial deployment of right ventricular disk during perventricular device closure in a child with apical muscular ventricular septal defect

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    Nageswara Rao Koneti

    2013-01-01

    Full Text Available We report a successful perventricular closure of an apical muscular ventricular septal defect (mVSD by a modified technique. An eight-month-old infant, weighing 6.5 kilograms, presented with refractory heart failure. The transthoracic echocardiogram showed multiple apical mVSDs with the largest one measuring 10 mm. perventricular device closure using a 12 mm Amplatzer mVSD occluder was planned. The left ventricular disk was positioned approximating the interventricular septum; however, the right ventricular (RV disk was deployed on the free wall of the RV due to an absent apical muscular septum and a small cavity at the apex. The RV disk of the device was covered using an autologous pericardium. His heart failure improved during follow-up.

  4. Eisenmenger ventricular septal defect in a Humboldt penguin (Spheniscus humboldti).

    Science.gov (United States)

    Laughlin, D S; Ialeggio, D M; Trupkiewicz, J G; Sleeper, M M

    2016-09-01

    The Eisenmenger ventricular septal defect is an uncommon type of ventricular septal defect characterised in humans by a traditionally perimembranous ventricular septal defect, anterior deviation (cranioventral deviation in small animal patients) of the muscular outlet septum causing malalignment relative to the remainder of the muscular septum, and overriding of the aortic valve. This anomaly is reported infrequently in human patients and was identified in a 45-day-old Humboldt Penguin, Spheniscus humboldti, with signs of poor growth and a cardiac murmur. This case report describes the findings in this penguin and summarises the anatomy and classification of this cardiac anomaly. To the authors' knowledge this is the first report of an Eisenmenger ventricular septal defect in a veterinary patient. Copyright © 2016 Elsevier B.V. All rights reserved.

  5. Angiographic differentiation of type of ventricular septal defects

    International Nuclear Information System (INIS)

    Cheon, Mal Soon; Park, Hee Young; Kim, Yang Sook

    1989-01-01

    Defects of the ventricular septum are the commonest type of congenital cardiac malformations. A classification with axial angiography of the subtypes of ventricular septal defects is proposed on the study of 126 patients with defects of the ventricular septum. The results were as follows: 1. The incidence of the ventricular septal defects was 39.6% of congenital heart malformation. 2. The sex distribution of cases were 70 males and 56 females, the age ranged from 13 months to 26 years. 3. Angiographic features seen by axial angiography were as follows: a. Perimembranous defects as seen on long axial view of left ventriculogram were in continuity wity aortic valve. The relation of the defect to the tricuspid valve allows distinction of the extension of the preimembranous defect toward inlet, trabecular, or infundibular zones. This relation was determined angiographically, using the course of the contrast medium from the left ventricle through the ventricular septal defect, opacifying the right ventricle. In inlet excavation, the shunted blood opacified the recess between septal leaflet of tricuspid valve and interventricular septum in early phase, in infundibular excavation, opacified the recess between anterior leaflet of tricuspid valve and anterior free wall of right ventricle and in trabecular excavation, the shunted blood traversed anterior portion of tricuspid valve ring, opacified trabecular portion of right ventricle. b. Muscular defects were separated from the semilunar and atrioventricular valves. c, Subarterial defects were related to both semilunar valves, and they were best demonstrated on the elongated right anterior oblique view of the left ventriculogram. d. Total infundibular defects were profiled in right anterior oblique 30 and long axial view, subaortic in location in both views

  6. [Percutaneous closure of ductus arteriosus and muscular ventricular defect with amplatzer occluder in a patient with severe pulmonary hypertension].

    Science.gov (United States)

    García-Montes, José Antonio; Zabal Cerdeira, Carlos; Calderón-Colmenero, Juan; Espínola, Nilda; Fernández de la Reguera, Guillermo; Buendía Hernández, Alfonso

    2005-01-01

    Surgical treatment of multiple muscular ventricular septal defects with associated lesions and severe pulmonary hypertension has a high morbility and mortality. Closure of these defects by the Amplatzer muscular VSD occluder is an alternative to surgery, avoiding the need of cardiopulmonary bypass. We present the case of a 38 year-old woman with signs of heart failure in NYHA functional class IV, with two muscular ventricular septal defects, patent ductus arteriosus and severe pulmonary hypertension, that were treated with three Amplatzer muscular VSD occluders, with significant reduction of pulmonary pressure and functional class improvement.

  7. Recent advances in managing septal defects: ventricular septal defects and atrioventricular septal defects [version 1; referees: 3 approved

    Directory of Open Access Journals (Sweden)

    P Syamasundar Rao

    2018-04-01

    Full Text Available This review discusses the management of ventricular septal defects (VSDs and atrioventricular septal defects (AVSDs. There are several types of VSDs: perimembranous, supracristal, atrioventricular septal, and muscular. The indications for closure are moderate to large VSDs with enlarged left atrium and left ventricle or elevated pulmonary artery pressure (or both and a pulmonary-to-systemic flow ratio greater than 2:1. Surgical closure is recommended for large perimembranous VSDs, supracristal VSDs, and VSDs with aortic valve prolapse. Large muscular VSDs may be closed by percutaneous techniques. A large number of devices have been used in the past for VSD occlusion, but currently Amplatzer Muscular VSD Occluder is the only device approved by the US Food and Drug Administration for clinical use. A hybrid approach may be used for large muscular VSDs in small babies. Timely intervention to prevent pulmonary vascular obstructive disease (PVOD is germane in the management of these babies. There are several types of AVSDs: partial, transitional, intermediate, and complete. Complete AVSDs are also classified as balanced and unbalanced. All intermediate and complete balanced AVSDs require surgical correction, and early repair is needed to prevent the onset of PVOD. Surgical correction with closure of atrial septal defect and VSD, along with repair and reconstruction of atrioventricular valves, is recommended. Palliative pulmonary artery banding may be considered in babies weighing less than 5 kg and those with significant co-morbidities. The management of unbalanced AVSDs is more complex, and staged single-ventricle palliation is the common management strategy. However, recent data suggest that achieving two-ventricle repair may be a better option in patients with suitable anatomy, particularly in patients in whom outcomes of single-ventricle palliation are less than optimal. The majority of treatment modes in the management of VSDs and AVSDs are safe

  8. Changes in Serum Natriuretic Peptide Levels after Percutaneous Closure of Small to Moderate Ventricular Septal Defects

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    Yuksel Kaya

    2012-01-01

    Full Text Available Background. B-type natriuretic peptide has been shown to be a very sensitive and specific marker of heart failure. In this study, we aimed to investigate the effect of percutaneous closure of ventricular septal defects with Amplatzer septal occluders on brain natriuretic peptide levels. Methods. Between 2008 and 2011, 23 patients underwent successfully percutaneous ventricular septal defect closure in 4 cardiology centers. Brain natriuretic peptide levels were measured in nine patients (4 male, mean ages were 25.3±14.3 who underwent percutaneous closure with Amplatzer occluders for membranous or muscular ventricular septal defects were enrolled in the study. Brain natriuretic peptide levels were measured one day before and one month after the closure. Patients were evaluated clinically and by echocardiography one month after the procedure. Results. Percutaneous closures of ventricular septal defects were successfully performed in all patients. There was not any significant adverse event in patients group during followup. Decrease in brain natriuretic peptide levels after closure were statistically significant (97.3±78.6 versus 26.8±15.6, =0.013. Conclusion. Brain Natriuretic Peptide levels are elevated in patients with ventricular septal defects as compared to controls. Percutaneous closure of Ventricular Septal Defect with Amplatzer occluders decreases the BNP levels.

  9. Ventricular Septal Defect: Peculiarities of Early Neonatal and Postnatal Diagnosis, Clinical Manifestations, Treatment and Prognosis at the Contemporary Stage

    Directory of Open Access Journals (Sweden)

    K.A. Kalashnikova

    2016-05-01

    Full Text Available The article presents the literature data on the incidence, the main clinical manifestations, modern methods for early neonatal and postnatal diagnosis and treatment of ventricular septal defect in children, as well as the prognosis of this disease. According to the International Classification of Diseases, 10th revision, ventricular septal defect is classified as Q21.0 Ventricular septal defect. Incidence. In the overall structure of congenital malformations of the cardiovascular system, ventricular septal defect has about 20 %. Diagnosis. Moderate ventricular septal defect is manifested by shortness of breath, rapid fatigability during feeding, delay in physical development. Significant arterial-venous shunt in the first month of life is accompanied by a transient mild cyanosis when the baby is fed and cries. Infants develop high pulmonary hypertension, circulatory failure, malnutrition. Small noise intensity is typical for newborns in the first weeks or even months of life, which is due to physiologically increased intravascular pulmonary resistance. Systolic murmur is extended to the entire systole with maximum amplitude at the left edge of the sternum at the level of III–IV intercostal spaces. Sclerotic phase of pulmonary hypertension with ventricular septal defect is defined as Eisenmenger reaction. The clinical picture of this disorder depends on the degree of hemodynamic instability caused by the defect parameters, the pressure level in the pulmonary artery, vascular pulmonary resistance, the magnitude and direction of the shunt through the defect. Diagnosis is confirmed by characteristic changes in the electrocardiogram, echocardiography and chest radiograph. Treatment. Small muscular ventricular septal defects often close spontaneously during the first 2 years of life. Drug correction is needed in the development of congestive heart failure. The optimum age for surgery — 5–9 years.

  10. Ventricular Septal Defect (VSD)

    Science.gov (United States)

    ... Call your doctor if your baby or child: Tires easily when eating or playing Is not gaining ... heart procedures. Risk factors Ventricular septal defects may run in families and sometimes may occur with other ...

  11. Ventricular Septal Dissection Complicating Inferior Wall Myocardial Infarction

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    Lindsey Kalvin

    2017-01-01

    Full Text Available Postmyocardial infarction ventricular septal defect is an increasingly rare mechanical complication of acute myocardial infarction. We present a case of acute myocardial infarction from right coronary artery occlusion that developed hypotension and systolic murmur 12 hours after successful percutaneous coronary intervention. Although preoperative imaging suggested a large ventricular septal defect and a pseudoaneurysm, intraoperative findings concluded a serpiginous dissection of the ventricular septum. The imaging technicalities are discussed.

  12. Surgical ablation of ventricular tachycardia secondary to congenital ventricular septal aneurysm.

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    Graffigna, A; Minzioni, G; Ressia, L; Vigano, M

    1994-04-01

    Three patients underwent surgical ablation for ventricular tachycardia resulting from an aneurysm of the membranous portion of the ventricular septum. Two patients had a definite history of cardiac murmur during infancy, and one of them was found at the time of operation to have a left-to-right shunt through the apex of the aneurysm. The earliest ventricular activation sites were located around the neck of the aneurysm and were ablated in 1 patient by encircling the endocardial ventriculotomy and by cryoablation in the remaining 2. After focus resection had been completed, aneurysm resection and ventricular septal reconstruction were performed. All patients were alive and free of ventricular tachycardia and did not need medication as of 61, 66, and 88 months postoperatively. Spontaneous closure of a ventricular septal defect may lead to the formation of an aneurysm in the ventricular septum that may sustain ventricular tachycardias. Such arrhythmias can be effectively treated using electrically guided surgical techniques.

  13. Visualization of a Small Ventricular Septal Defect at First-pass Contrast-enhanced Cardiac Magnetic Resonance Imaging

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    Francesco Secchi

    2013-01-01

    Full Text Available Ventricular septal defect (VSD is a congenital heart disease that accounts for up to 40% of all congenital cardiac malformations. VSD is a connection between right and left ventricle, through the ventricular septum. Echocardiography and magnetic resonance imaging (MRI help identify this entity. This case presents a 12-year-old male diagnosed with a small muscular apical VSD of 3 mm in diameter, at echocardiography. Cardiac MRI using first-pass perfusion sequence, combining the right plane of acquisition with a short bolus of contrast material, clearly confirmed the presence of VSD.

  14. Ventricular septal defect closure in a patient with achondroplasia.

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    Nakanishi, Keisuke; Kawasaki, Shiori; Amano, Atsushi

    2017-01-01

    Achondroplasia with co-morbid CHD is rare, as are reports of surgical treatment for such patients. We present the case of a 13-year-old girl with achondroplasia and ventricular septal defect. Her ventricular septal defect was surgically repaired focussing on the cardiopulmonary bypass flow, healing of the sternum, and her frail neck cartilage. The surgery and recovery were without complications.

  15. Transcatheter closure of ventricular septal defect with Occlutech Duct Occluder.

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    Atik-Ugan, Sezen; Saltik, Irfan Levent

    2018-04-01

    Patent ductus arteriosus occluders are used for transcatheter closure of ventricular septal defects, as well as for closure of patent ductus arteriosus. The Occlutech Duct Occluder is a newly introduced device for transcatheter closure of patent ductus arteriosus. Here, we present a case in which the Occlutech Duct Occluder was successfully used on a patient for the closure of a perimembraneous ventricular septal defect.

  16. Radionuclide analysis of right and left ventricular response to exercise in patients with atrial and ventricular septal defects

    International Nuclear Information System (INIS)

    Peter, C.A.; Bowyer, K.; Jones, R.H.

    1983-01-01

    In patients with ventricular or atrial septal defect, the ventricle which is chronically volume overloaded might not appropriately respond to increased demand for an augmentation in output and thereby might limit total cardiac function. In this study we simultaneously measured right and left ventricular response to exercise in 10 normal individuals, 10 patients with ventricular septal defect (VSD), and 10 patients with atrial septal defect (ASD). The normal subjects increased both right and left ventricular ejection fraction, end-diastolic volume, and stroke volume to achieve a higher cardiac output during exercise. Patients with VSD failed to increase right ventricular ejection fraction, but increased right ventricular end-diastolic volume and stroke volume. Left ventricular end-diastolic volume did not increase in these patients but ejection fraction, stroke volume, and forward left ventricular output achieved during exercise were comparable to the response observed in healthy subjects. In the patients with ASD, no rest-to-exercise change occurred in either right ventricular ejection fraction, end-diastolic volume, or stroke volume. In addition, left ventricular end-diastolic volume failed to increase, and despite an increase in ejection fraction, left ventricular stroke volume remained unchanged from rest to exercise. Therefore, cardiac output was augmented only by the heart rate increase in these patients. Right ventricular function appeared to be the major determinant of total cardiac output during exercise in patients with cardiac septal defects and left-to-right shunt

  17. Percutaneous closure of a post-traumatic ventricular septal defect with a patent ductus arteriosus occluder

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    Er-Ping Xi

    2012-11-01

    Full Text Available OBJECTIVE: Ventricular septal defects resulting from post-traumatic cardiac injury are very rare. Percutaneous closure has emerged as a method for treating this disorder. We wish to report our experience in three patients who underwent percutaneous closure of a post-traumatic ventricular septal defect with a patent ductus arteriosus occluder. METHODS: We treated three patients with post-traumatic ventricular septal defects caused by stab wounds with knives. After the heart wound was repaired, patient examinations revealed ventricular septal defects with pulmonary/systemic flow ratios (Qp/Qs of over 1.7. The post-traumatic ventricular septal defects were closed percutaneously with a patent ductus arteriosus occluder (Lifetech Scientific (Shenzhen Co., LTD, Guangdong, China utilizing standard techniques. RESULTS: Post-operative transthoracic echocardiography revealed no residual left-to-right shunt and indicated normal ventricular function. In addition, 320-slice computerized tomography showed that the occluder was well placed and exhibited normal morphology. CONCLUSION: Our experiences indicate that closure of a post-traumatic ventricular septal defect using a patent ductus arteriosus occluder is feasible, safe, and effective.

  18. Percutaneous closure of a post-traumatic ventricular septal defect with a patent ductus arteriosus occluder.

    Science.gov (United States)

    Xi, Er-Ping; Zhu, Jian; Zhu, Shui-Bo; Yin, Gui-Lin; Liu, Yong; Dong, Yong-Qiang; Zhang, Yu; Xia, Feng

    2012-11-01

    Ventricular septal defects resulting from post-traumatic cardiac injury are very rare. Percutaneous closure has emerged as a method for treating this disorder. We wish to report our experience in three patients who underwent percutaneous closure of a post-traumatic ventricular septal defect with a patent ductus arteriosus occluder. We treated three patients with post-traumatic ventricular septal defects caused by stab wounds with knives. After the heart wound was repaired, patient examinations revealed ventricular septal defects with pulmonary/systemic flow ratios (Qp/Qs) of over 1.7. The post-traumatic ventricular septal defects were closed percutaneously with a patent ductus arteriosus occluder (Lifetech Scientific (Shenzhen) Co., LTD, Guangdong, China) utilizing standard techniques. Post-operative transthoracic echocardiography revealed no residual left-to-right shunt and indicated normal ventricular function. In addition, 320-slice computerized tomography showed that the occluder was well placed and exhibited normal morphology. Our experiences indicate that closure of a post-traumatic ventricular septal defect using a patent ductus arteriosus occluder is feasible, safe, and effective.

  19. The angulation of the septal structures impacts ventricular imbalance in atrioventricular septal defects with a common atrioventricular junction.

    Science.gov (United States)

    Ahmad, Zaheer; Lim, Zek; Roman, Kevin; Haw, Marcus; Anderson, Robert H; Vettukattil, Joseph

    2016-02-01

    Multiplanar re-formatting of full-volume three-dimensional echocardiography data sets offers new insights into the morphology of atrioventricular septal defects. We hypothesised that distortion of the alignment between the atrial and ventricular septums results in imbalanced venous return to the ventricles, with consequent proportional ventricular hypoplasia. A single observer evaluated 31 patients, with a mean age of 52.09 months, standard deviation of 55, and with a range from 2 to 264 months, with atrioventricular septal defects, of whom 17 were boys. Ventricular imbalance, observed in nine patients, was determined by two-dimensional assessment, and confirmed at surgical inspection in selected cases when a univentricular strategy was undertaken. Offline analysis using multiplanar re-formatting was performed. A line was drawn though the length of the ventricular septum and a second line along the plane of the atrial septum, taking the angle between these two lines as the atrioventricular septal angle. We compared the angle between 22 patients with adequately sized ventricles, and those with ventricular imbalance undergoing univentricular repair. In the 22 patients undergoing biventricular repair, the septal angle was 0 in 14 patients; the other eight patients having angles ranging from 1 to 36, with a mean angle of 7.4°, and standard deviation of 11.1°.The mean angle in the nine patients with ventricle imbalance was 28.6°, with a standard deviation of 3.04°, and with a range from 26 to 35°. Of those undergoing univentricular repair, two patients died, with angles of 26 and 30°, respectively. The atrioventricular septal angle derived via multiplanar formatting gives important information regarding the degree of ventricular hypoplasia and imbalance. When this angle is above 25°, patients are likely to have ventricular imbalance requiring univentricular repair.

  20. Delayed ventricular septal rupture complicating acute inferior wall myocardial infarction

    OpenAIRE

    Cho, Jae Hyung; Sattiraju, Srinivasan; Mehta, Sanjay; Missov, Emil

    2013-01-01

    Background Ventricular septal rupture is a potentially fatal complication of acute myocardial infarction. Its incidence has declined with modern reperfusion therapy. In the era of percutaneous coronary interventions, it occurs a median of 18?24?hours after myocardial infarction and is most commonly associated with anterior myocardial infarction. We present a case of delayed ventricular septal rupture complicating acute inferior wall myocardial infarction. Case presentation A 53-year-old Cauca...

  1. Percutaneous closure of a post-traumatic ventricular septal defect with a patent ductus arteriosus occluder

    OpenAIRE

    Xi, Er-Ping; Zhu, Jian; Zhu, Shui-Bo; Yin, Gui-Lin; Liu, Yong; Dong, Yong-Qiang; Zhang, Yu; Xia, Feng

    2012-01-01

    OBJECTIVE: Ventricular septal defects resulting from post-traumatic cardiac injury are very rare. Percutaneous closure has emerged as a method for treating this disorder. We wish to report our experience in three patients who underwent percutaneous closure of a post-traumatic ventricular septal defect with a patent ductus arteriosus occluder. METHODS: We treated three patients with post-traumatic ventricular septal defects caused by stab wounds with knives. After the heart wound was repaired,...

  2. Doubly committed subarterial ventricular septal defect with prolapsed right coronary cusp with moderate aortic regurgitation

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    Redoy Ranjan

    2017-11-01

    Full Text Available A 4 year old girl was presented with the respiratory tract infection, breathlessness after taking meal, failure to thrive, abnormal movement of the chest on left side overlying the area of heart and systolic murmur. She developed these symptoms gradually for the last 3.5 years. Echocardiography revealed doubly committed subarterial ventricular septal defect with moderate aortic regurgitation. The size of the ventricular septal defect was 7 x 9 mm at the left ventricular outflow tract. The right coronary cusp of the aortic valve was prolapsed. Left atrium and left ventricle were dilated. The pulmonary artery systolic pressure was 35 mm Hg. The ventricular septal defect was closed with the standard surgical procedure using cardiopulmonary bypass followed by aortotomy and right atriotomy. Immediate post-operative period of this case was uneventful and the patient was discharged on 9th post-operative day. Follow-up echocardiography showed no residual ventricular septal defect or aortic regurgitation and the ventricular function was good.

  3. Intermittent′ restrictive ventricular septal defect in Tetralogy of Fallot

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    Sudhir S Shetkar

    2015-01-01

    Full Text Available Ventricular septal defect (VSD in Tetralogy of Fallot (TOF is usually large and non-restrictive with equalization of right and left ventricular pressures. Restrictive VSD in TOF is rare. We present an unusual case of TOF with restriction to VSD caused by accessory tricuspid valve tissue that varied with respiration.

  4. Multiple ventricular septal defects: a new strategy.

    Science.gov (United States)

    Corno, Antonio F; Kandakure, Pramod R; Dhannapuneni, Ramana Rao V; Gladman, Gordon; Venugopal, Prem; Alphonso, Nelson

    2013-01-01

    A multicenter prospective study was conducted to evaluate a new strategy for multiple Ventricular Septal Defects (VSDs). From 2004 to 2012 17 consecutive children (3 premature, 14 infants), mean age 3.2 months (9 days-9 months), mean body weight 4.2 kg (3.1-6.1 kg), with multiple VSDs underwent Pulmonary Artery Banding (PAB) with an adjustable FloWatch-PAB(®). Associated cardiac anomalies included patent ductus arteriosus (1), aortic coarctation (2), hypoplastic aortic arch (2), and left isomerism (3). Five patients (5/17 = 29.4%) required pre-operative mechanical ventilation, with a mean duration of 64 days (7-240 days) There were no early or late deaths during a mean follow-up of 48 months (7-98 months), with either FloWatch removal or last observation as end-points. FloWatch-PAB(®) adjustments were required in all patients: a mean of 4.8 times/patient (2-9) to tighten the PAB, and a mean of 1.1 times/patient (0-3) to release the PAB with the patient's growth. After a mean interval of 29 months (8-69 months) 10/17 (59%) patients underwent re-operation: 7/10 PAB removal, with closure of a remaining unrestrictive VSD in 6 (peri-membranous in 3 patients, mid-muscular in 2, and inlet in 1) and Damus-Kaye-Stansel, bi-directional Glenn, and atrial septectomy in 1; 3/9 patients required only PAB removal. All muscular multiple VSDs had closed in all 10 patients. PA reconstruction was required in 1/10 patient. In 5/7 of the remaining patients with the PAB still in situ, all muscular VSDs had already closed. The only 2 patients with persistent muscular multiple VSDs are the 2 patients with the shortest follow-up. This reproducible new strategy with an adjustable PAB simplifies the management of infants with multiple VSDs and provides the following advantages: (a) good results (0% mortality), delayed surgery with a high incidence (15/17 = 88%) of spontaneous closure of multiple muscular VSDs, and facilitated closure of residual unrestrictive

  5. Radiological evaluation of ventricular septal defect with aortic insufficiency - An analysis of cineangiography in 15 cases -

    International Nuclear Information System (INIS)

    Park, Jae Hyung; Yeon, Kyung Mo; Han, Man Chung

    1981-01-01

    Fifteen cases of ventricular septal defect with aortic insufficiency were diagnosed radiographically and confirmed after operation at Seoul National University Hospital in recent two half years since 1979. Cineangiographies of ascending aorta and left ventricle were done in those cases and revealed some characteristic findings. The results of the analysis are as follow: 1. Among the 15 cases, 14 cases were male and 1 case was female. Age distribution was from 7 years to 23 years. 2. Those 15 cases were corresponded to 8% among total 193 cases of ventricular septal defect, to 11% among total 135 cases of aortic insufficiency and especially to 48% among 48 cases of aortic insufficiency below age of 20 years. 3. After operation, 11 cases were confirmed as subpulmonary type ventricular septal defect and 4 cases as subcristal type. The sizes of the ventricular septal defects were ranged between 0.6 and 2.5 cm in diameter. 4. Regurgitation of contrast media was noticed in cine aortography of all cases, and the grades of regurgitation were II-III/IV in 13 cases. 5. Various types of herniated aortic cusp through ventricular septal defect were seen. In the cases of subpulmonary ventricular septal defect characteristic saccular aneurysm was found in 7 cases. Asymmetry or mild bulging of aortic sinus was found in the cases of subcristal ventricular septal defect. 6. Infundibular stenosis was found in 3 cases with right ventriculography and those were caused by the herniated saccular aneurysm of aortic cusp. 7. It is essential for the diagnosis of ventricular septal defect with aortic insufficiency to undertake biplane cineangiography of ascending aorta and left ventricle in long axial view and right ventriculography should be done in suspicion of infundibular pulmonary stenosis

  6. Nonsustained Repetitive Upper Septal Idiopathic Fascicular Left Ventricular Tachycardia: Rare Type of VT

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    Gokhan Aksan

    2016-05-01

    Full Text Available Upper septal fascicular ventricular tachycardia is a very rare form of idiopathic fascicular ventricular tachycardia. Upper septal fascicular tachycardia uses the posterior fascicle as the anterograde limb and the septal fascicle as the retrograde limb. When evaluating the electrocardiography for this form of tachycardia, the presence of narrow QRS morphology and normal axis may be misinterpreted as supraventricular tachycardia. Here, we report a very rare subtype of fascicular tachycardia that originates more proximally in the His-Purkinje system at the base of the heart.

  7. Comparison of right ventricular septal pacing and right ventricular apical pacing in patients receiving cardiac resynchronization therapy defibrillators: the SEPTAL CRT Study

    Science.gov (United States)

    Leclercq, Christophe; Sadoul, Nicolas; Mont, Lluis; Defaye, Pascal; Osca, Joaquim; Mouton, Elisabeth; Isnard, Richard; Habib, Gilbert; Zamorano, Jose; Derumeaux, Genevieve; Fernandez-Lozano, Ignacio; Dupuis, Jean-Marc; Rouleau, Frédéric; Tassin, Aude; Bordachar, Pierre; Clémenty, Jacques; Lafitte, Stephane; Ploux, Sylvan; Reant, Patricia; Ritter, Philippe; Defaye, Pascal; Jacon, Peggy; Mondesert, Blandine; Saunier, Carole; Vautrin, Estelle; Kacet, Salem; Guedon-Moreau, Laurence; Klug, Didier; Kouakam, Claude; Marechaux, Sylvestre; Marquie, Christelle; Polge, Anne Sophie; Richardson, Marjorie; Chevallier, Philippe; De Breyne, Brigitte; Lotek, Marcin M.; Nonin, Emilie; Pineau, Julien; Deharo, Jean-Claude; Bastard, Emilie; Franceschi, Frédéric; Habib, Gilbert; Jego, Christophe; Peyrouse, Eric; Prevot, Sebastien; Saint-Joseph, Hôpital; Bremondy, Michel; Faure, Jacques; Ferracci, Ange; Lefevre, Jean; Pisapia, Andre; Davy, Jean-Marc; Cransac, Frederic; Cung, Tien Tri; Georger, Frederic; Pasquie, Jean-Luc; Raczka, Franck; Sportouch-Dukhan, Catherine; Sadoul, Nicolas; Blangy, Hugues; Bruntz, Jean-François; Freysz, Luc; Groben, Laurent; Huttin, Olivier; Bammert, Antoine; Burban, Marc; Cebron, Jean-Pierre; Gras, Daniel; Frank, Robert; Duthoit, Guillaume; Hidden-Lucet, Françoise; Himbert, Caroline; Isnard, Richard; Lacotte, Jérôme; Pousset, Françoise; Zerah, Thierry; Leclercq, Christophe; Bellouin, Annaïk; Crocq, Christophe; Deplace, Christian; Donal, Erwan; Hamon, Cécile; Mabo, Philippe; Romain, Olivier; Solnon, Aude; Frederic, Anselme; Bauer, Fabrice; Bernard, Mathieu; Godin, Benedicte; Kurtz, Baptiste; Savoure, Arnaud; Copie, Xavier; Lascault, Gilles; Paziaud, Olivier; Piot, Olivier; Touche, Thierry; Delay, Toulouse Marc; Chilon, Talia; Detis, Nicolas; Duparc, Alexandre; Hebrard, Aurélien; Massabuau, Pierre; Maury, Philippe; Mondoly, Pierre; Rumeau, Philippe; Pasteur, Clinique; Boveda, Serge; Adrover, Laurence; Combes, Nicolas; Deplagne, Antoine; Marco-Baertich, Isabelle; Fondard, Olivier; Martínez, Juan Gabriel; Ibañez Criado, José Luis; Ortuño, Diego; Mont, Lluis; Berruezo, Antonio; Eduard, Belu; Martín, Ana; Merschon, Franco M.; Sitges, Marta; Tolosana, José María; Vidal, Bárbara; Hebron, H. Valle; i Mitjans, Angel Moya; Rodriguez, Oscar Alcalde; Rodriguez Palomares, José Fernando; Rivas, Nuria; Teixidó, Gisela; de Hierro, H. Puerta; Lozano, Ignacio Fernández; Ruiz Bautista, Maria Lorena; Castro, Victor; Cavero, Miguel Angel; Gutierrez, Carlos; Ros, Natalia; de la Victoria, H. Virgen; Alzueta Rodriguez, Francisco Javier; Cabrera, Fernando; Cordero, Alberto Barrera; Peña, José Luis; de Valme Sevilla, H.; Gonzáles, Juan Lealdel Ojo; Garcia Medina, Mª Dolores; Jiménez, Ricardo Pavón; Villagomez, David; de la Salud Toledo, H. Virgen; Castellanos Martinez, Eduardo; Alcalá, Juan; Maicas, Carolina; Arias Palomares, Miguel Angel; Puchol, Alberto; Valencia, H. La Fé; OscaAsensi, Joaquim; Carmona, Anastasio Quesada; De Carranza, Mª José Sancho-Tello; De Ros, José Olagüe; Pareja, Enrique Castro; Pérez, Oscar Cano; Saez, Ana Osa; Hortega, H. Rio; Guilarte, Benito Herreros; Muñoz San Jose, Juan Francisco; Pérez Sanz, Teresa Myriam; Logeart, Damien; Gil, Maria Lopez; Leclercq, Christophe; Lozano, Ignacio Fernandez; de Hierro, H. Puerta; Derumeaux, Genevieve

    2016-01-01

    Abstract Aims Cardiac resynchronization therapy (CRT) is a recommended treatment of heart failure (HF) patients with depressed left ventricular ejection fraction and wide QRS. The optimal right ventricular (RV) lead position being a matter of debate, we sought to examine whether RV septal (RVS) pacing was not inferior to RV apical (RVA) pacing on left ventricular reverse remodelling in patients receiving a CRT-defibrillator. Methods and results Patients (n = 263, age = 63.4 ± 9.5 years) were randomly assigned in a 1:1 ratio to RVS (n = 131) vs. RVA (n = 132) pacing. Left ventricular end-systolic volume (LVESV) reduction between baseline and 6 months was not different between the two groups (−25.3 ± 39.4 mL in RVS group vs. −29.3 ± 44.5 mL in RVA group, P = 0.79). Right ventricular septal pacing was not non-inferior (primary endpoint) to RVA pacing with regard to LVESV reduction (average difference = −4.06 mL; P = 0.006 with a −20 mL non-inferiority margin). The percentage of ‘echo-responders’ defined by LVESV reduction >15% between baseline and 6 months was similar in both groups (50%) with no difference in the time to first HF hospitalization or death (P = 0.532). Procedural or device-related serious adverse events occurred in 68 patients (RVS = 37) with no difference between the two groups (P = 0.401). Conclusion This study demonstrates that septal RV pacing in CRT is non-inferior to apical RV pacing for LV reverse remodelling at 6 months with no difference in the clinical outcome. No recommendation for optimal RV lead position can hence be drawn from this study. ClinicalTrials. gov number NCT 00833352. PMID:26374852

  8. MULTIPLE VENTRICULAR SEPTAL DEFECTS: A NEW STRATEGY

    Directory of Open Access Journals (Sweden)

    Antonio Francesco Corno

    2013-07-01

    Full Text Available INTRODUCTIONA multicenter prospective study was conducted to evaluate a new strategy for multiple Ventricular Septal Defects (VSDs.MATERIALS AND METHODSFrom 2004 to 2012 17 consecutive children (3 premature, 14 infants, mean age 3.2months (9 days to 9 months, mean body weight 4.2kg (3.1 to 6.1 kg, with multiple VSDs underwent Pulmonary Artery Banding (PAB with an adjustable FloWatch-PAB. Associated cardiac anomalies included patent ductus arteriosus (9, aortic coarctation (2, hypoplastic aortic arch (2 and left isomerism (1. Five patients (5/17 =29.4% required pre-operative mechanical ventilation, with a mean duration of 64 days (7 to 240 daysRESULTSThere were no early or late deaths during a mean follow-up of 48 months (7 to 98 months, with either FloWatch removal or last observation as end-points.FloWatch-PAB adjustments were required in all patients: a mean of 4.8 times/patient (2 to 9 to tighten the PAB, and a mean of 1.1 times/patient (0 to 3 to release the PAB with the patient’s growth. After a mean interval of 29 months (8 to 69 months 10/17 (59% patients underwent reoperation: 7/10 PAB removal, with closure of a remaining peri-membranous VSD in 6 and Damus-Kaye-Stansel, bi-directional Glenn, and atrial septectomy in 1; 3/9 patients required only PAB removal. All muscular multiple VSDs had closed in all 10 patients. PA reconstruction was required in 1/10 patient. In 5/7 of the remaining patients with the PAB still in situ, all muscular VSDs had already closed. The only 2 patients with persistent muscular multiple VSDs are the 2 patients with the shortest follow-up.CONCLUSIONS This reproducible new strategy with an adjustable PAB simplifies the management of infants with multiple VSDs and provides the following advantages: a good results (0% mortality, delayed surgery with a high incidence (15/17=88% of spontaneous closure of multiple muscular VSDs, and facilitated closure of residual peri-membranous VSD at an older age and h

  9. Aspects of surgery for congenital ventricular septal defect

    NARCIS (Netherlands)

    G. Bol-Raap (Goris)

    2007-01-01

    textabstractIn chapter 1, an outline of the thesis is given. This thesis focuses on aspects of surgical closure of a congenital ventricular septal defect. In Chapter 2, the accuracy and the potential of 3-D echocardiography in the preoperative assessment of a congenital VSD were evaluated. 3-D

  10. Patent ductus arteriosus closure using an Amplatzer™ ventricular septal defect closure device

    Science.gov (United States)

    Fernando, Rajeev; Koranne, Ketan; Loyalka, Pranav; Kar, Biswajit; Gregoric, Igor

    2013-01-01

    The ductus arteriosus originates from the persistence of the distal portion of the left sixth aortic arch. It connects the descending aorta (immediately distal to the left subclavian artery) to the roof of the main pulmonary artery, near the origin of the left pulmonary artery. Persistence of the duct beyond 48 h after birth is abnormal and results in patent ductus arteriosus (PDA). PDA is rare in adults because it is usually discovered and treated in childhood. Mechanical closure remains the definitive therapy because the patency of ductus arteriosus may lead to multiple complications, depending on the size and flow through the ductus. PDA closure is indicated in patients with symptoms and evidence of left heart enlargement, and in patients with elevated pulmonary pressures when reversal is possible. Transcatheter closure is the preferred technique in adults because it avoids sternotomy, reduces the length of hospital stay and is associated with fewer complications compared with surgery. First demonstrated in 1967, both the technique and the occluder devices used have since evolved. However, designing an ideal PDA occluder has been a challenge due to the variability in size, shape and orientation of PDAs. The present article describes a case involving a 35-year-old woman who presented to the Center for Advanced Heart Failure (Houston, USA) with congestive heart failure due to a large PDA, which was successfully occluded using an Amplatzer (St Jude Medical, USA) muscular ventricular septal defect closure device. The wider waist and dual-retention discs of these ventricular septal defect closure devices may be important factors to consider in the future development of devices for the occlusion of large PDAs. PMID:24294051

  11. Rest and exercise ventricular function in adults with congenital ventricular septal defects

    International Nuclear Information System (INIS)

    Jablonsky, G.; Hilton, J.D.; Liu, P.P.; Morch, J.E.; Druck, M.N.; Bar-Shlomo, B.Z.; McLaughlin, P.R.

    1983-01-01

    Rest and exercise right and left ventricular function were compared using equilibrium gated radionuclide angiography in 19 normal sedentary control subjects and 34 patients with hemodynamically documented congenital ventricular septal defect (VSD). Gated radionuclide angiography was performed at rest and during each level of graded supine bicycle exercise to fatigue. Heart rate, blood pressure, maximal work load achieved, and right and left ventricular ejection fractions were assessed. The control subjects demonstrated an increase in both the left and right ventricular ejection fractions with exercise. All study groups failed to demonstrate an increase in ejection fraction in either ventricle with exercise. Furthermore, resting left ventricular ejection fraction in Groups 2 and 3 was lower than that in the control subjects and resting right ventricular ejection fraction was lower in Group 3 versus control subjects. Thus left and right ventricular function on exercise were abnormal in patients with residual VSD as compared with control subjects; rest and exercise left ventricular ejection fractions remained abnormal despite surgical closure of VSD in the remote past; resting left and right ventricular function was abnormal in patients with Eisenmenger's complex; lifelong volume overload may be detrimental to myocardial function

  12. Classification of Atrial Septal Defect and Ventricular Septal Defect with Documented Hemodynamic Parameters via Cardiac Catheterization by Genetic Algorithms and Multi-Layered Artificial Neural Network

    Directory of Open Access Journals (Sweden)

    Mustafa Yıldız

    2012-08-01

    Full Text Available Introduction: We aimed to develop a classification method to discriminate ventricular septal defect and atrial septal defect by using severalhemodynamic parameters.Patients and Methods: Forty three patients (30 atrial septal defect, 13 ventricular septal defect; 26 female, 17 male with documentedhemodynamic parameters via cardiac catheterization are included to study. Such parameters as blood pressure values of different areas,gender, age and Qp/Qs ratios are used for classification. Parameters, we used in classification are determined by divergence analysismethod. Those parameters are; i pulmonary artery diastolic pressure, ii Qp/Qs ratio, iii right atrium pressure, iv age, v pulmonary arterysystolic pressure, vi left ventricular sistolic pressure, vii aorta mean pressure, viii left ventricular diastolic pressure, ix aorta diastolicpressure, x aorta systolic pressure. Those parameters detected from our study population, are uploaded to multi-layered artificial neuralnetwork and the network was trained by genetic algorithm.Results: Trained cluster consists of 14 factors (7 atrial septal defect and 7 ventricular septal defect. Overall success ratio is 79.2%, andwith a proper instruction of artificial neural network this ratio increases up to 89%.Conclusion: Parameters, belonging to artificial neural network, which are needed to be detected by the investigator in classical methods,can easily be detected with the help of genetic algorithms. During the instruction of artificial neural network by genetic algorithms, boththe topology of network and factors of network can be determined. During the test stage, elements, not included in instruction cluster, areassumed as in test cluster, and as a result of this study, we observed that multi-layered artificial neural network can be instructed properly,and neural network is a successful method for aimed classification.

  13. Right ventricular pressure response to exercise in adults with isolated ventricular septal defect closed in early childhood.

    Science.gov (United States)

    Moller, Thomas; Lindberg, Harald; Lund, May Brit; Holmstrom, Henrik; Dohlen, Gaute; Thaulow, Erik

    2018-06-01

    We previously demonstrated an abnormally high right ventricular systolic pressure response to exercise in 50% of adolescents operated on for isolated ventricular septal defect. The present study investigated the prevalence of abnormal right ventricular systolic pressure response in 20 adult (age 30-45 years) patients who underwent surgery for early ventricular septal defect closure and its association with impaired ventricular function, pulmonary function, or exercise capacity. The patients underwent cardiopulmonary tests, including exercise stress echocardiography. Five of 19 patients (26%) presented an abnormal right ventricular systolic pressure response to exercise ⩾ 52 mmHg. Right ventricular systolic function was mixed, with normal tricuspid annular plane systolic excursion and fractional area change, but abnormal tricuspid annular systolic motion velocity (median 6.7 cm/second) and isovolumetric acceleration (median 0.8 m/second2). Left ventricular systolic and diastolic function was normal at rest as measured by the peak systolic velocity of the lateral wall and isovolumic acceleration, early diastolic velocity, and ratio of early diastolic flow to tissue velocity, except for ejection fraction (median 53%). The myocardial performance index was abnormal for both the left and right ventricle. Peak oxygen uptake was normal (mean z score -0.4, 95% CI -2.8-0.3). There was no association between an abnormal right ventricular systolic pressure response during exercise and right or left ventricular function, pulmonary function, or exercise capacity. Abnormal right ventricular pressure response is not more frequent in adult patients compared with adolescents. This does not support the theory of progressive pulmonary vascular disease following closure of left-to-right shunts.

  14. Patent ductus arteriosus closure using an Amplatzer(™) ventricular septal defect closure device.

    Science.gov (United States)

    Fernando, Rajeev; Koranne, Ketan; Loyalka, Pranav; Kar, Biswajit; Gregoric, Igor

    2013-01-01

    The ductus arteriosus originates from the persistence of the distal portion of the left sixth aortic arch. It connects the descending aorta (immediately distal to the left subclavian artery) to the roof of the main pulmonary artery, near the origin of the left pulmonary artery. Persistence of the duct beyond 48 h after birth is abnormal and results in patent ductus arteriosus (PDA). PDA is rare in adults because it is usually discovered and treated in childhood. Mechanical closure remains the definitive therapy because the patency of ductus arteriosus may lead to multiple complications, depending on the size and flow through the ductus. PDA closure is indicated in patients with symptoms and evidence of left heart enlargement, and in patients with elevated pulmonary pressures when reversal is possible. Transcatheter closure is the preferred technique in adults because it avoids sternotomy, reduces the length of hospital stay and is associated with fewer complications compared with surgery. First demonstrated in 1967, both the technique and the occluder devices used have since evolved. However, designing an ideal PDA occluder has been a challenge due to the variability in size, shape and orientation of PDAs. The present article describes a case involving a 35-year-old woman who presented to the Center for Advanced Heart Failure (Houston, USA) with congestive heart failure due to a large PDA, which was successfully occluded using an Amplatzer (St Jude Medical, USA) muscular ventricular septal defect closure device. The wider waist and dual-retention discs of these ventricular septal defect closure devices may be important factors to consider in the future development of devices for the occlusion of large PDAs.

  15. Right and Left Ventricular Volumes in Atrial Septal Defect Studied by Radiocardiography

    Energy Technology Data Exchange (ETDEWEB)

    Ivancevic, D. [Radioisotope Department, Internal Clinic, Rebro, Zagreb, Yugoslavia (Croatia); Vernejoul, P. de; Kellershohn, C. [CEA, Service Hospitalier Frederic Joliot, Departement de Biologie, Orsay (France)

    1971-02-15

    Radiocardiography with radioiodinated ({sup 131}I) human serum albumin and barium ({sup 137m}Ba) solution injected into the right subclavian vein has been performed in a group of 43 patients with atrial septal defect and left-to-right shunt. Data on the output and ejection index of each ventricle are essential for the estimation of the diastolic and residual volumes of the right and left ventricle. The systemic flow was therefore calculated according to Veall's formula and the pulmonary flow and the shunt How were determined using the method of de Vernejoul and co-workers. The formulas for the calculation of ventricular volumes were modified. The results show that many cases of atrial septal defect have an enlarged right ventricle whereas the left ventricle remains normal or is diminished. These changes correlate well with the amount of the shunt flow. In both ventricles the ventricular volumes show a good correlation with the stroke volumes. For the regulation of the pulmonary blood volume the right ventricle seems to be more important than the left ventricle. The operative closure of atrial septal defect (in 14 patients) has normalized the size of ventricular volumes. (author)

  16. Tetralogy of Fallot with restrictive ventricular septal defect by accessory tricuspid leaflet tissue

    OpenAIRE

    Mahipat Raj Soni; Deepak A. Bohara; Ajay U. Mahajan; Pratap J. Nathani

    2012-01-01

    In tetralogy of Fallot septal defect is usually large because of malalignment of outlet septum, restrictive defect has been reported rarely. We present a case of tetralogy of Fallot with accessory tricuspid leaflet tissue restricting ventricular septal defect. The report includes echocardiographic and catheter images of this rare presentation of tetralogy of Fallot.

  17. Aniridia--Wilms′ tumour association--a case with 11p 13-14.1 deletion and ventricular septal defect.

    OpenAIRE

    Rao S; Athale U; Kadam P; Gladstone B; Nair C; Pai S; Kurkure P; Advani S

    1992-01-01

    A two year old female child with bilateral wilms tumor (WT) along with multiple congenital anomalies like bilateral aniridia with congenital cataracts and nystagmus, microcephaly, mental retardation and ventricular septal defect has been described. The karyotype analysis revealed 46 xx, del 11p 13-14.1. Association of ventricular septal defect with the classical features of ′Aniridia-Wilms′ tumor association′ is an unusual feature in this case.

  18. Anaesthetic management of a child with "cor-triatriatum" and multiple ventricular septal defects - A rare congenital anomaly

    Directory of Open Access Journals (Sweden)

    Sriram Sabade

    2010-01-01

    Full Text Available Cor-triatriatum is a rare congenital cardiac anomaly. It accounts for 0.1% of congenital heart diseases. Its association with multiple ventricular septal defects (VSD is even rarer. A five-month-old baby was admitted with respiratory distress and failure to thrive. Clinical examination revealed diastolic murmur over mitral area. Chest X-ray showed cardiomegaly. Haematological and biochemical investigations were within normal limits. Electrocardiogram showed left atrial enlargement. 2D echo showed double-chambered left atrium (cor-triatriatum, atrial septal defect (ASD and muscular VSD with moderate pulmonary arterial hypertension. The child was treated with 100% oxygen, diuretics and digoxin and was stabilized medically. We used balanced anaesthetic technique using oxygen, air, isoflurane, fentanyl, midazolam and vecuronium. Patient was operated under cardiopulmonary bypass (CPB with moderate hypothermia. Through right atriotomy abnormal membrane in the left atrium was excised to make one chamber. VSD were closed with Dacron patches and ASD was closed with autologous pericardial patch. Patient tolerated the whole procedure well and was ventilated electively for 12h in the intensive care unit. He was discharged on the 10 th postoperative day.

  19. Acquired ventricular septal defect: A rare sequel of blunt chest ...

    African Journals Online (AJOL)

    Ventricular septal defect (VSD) is the most common congenital cardiac lesion encountered worldwide. Only very rarely is it acquired, and causation through blunt injury in a child is extremely rare. A previously healthy 7‑year‑old boy suffered blunt chest trauma while at play. He presented 11 days later with features of acute ...

  20. Double-chambered right ventricle, ventricular septal defect, patent ductus arteriosus in a dog

    Energy Technology Data Exchange (ETDEWEB)

    Morozumi, M. [Togasaki Animal Hospital, Misato, Saitama (Japan); Kurosu, Y.; Kogure, K.; Chimura, S.; Shibata, S.; Kanemoto, I.

    1989-12-15

    A 4-month-old female mongrel puppy was presented with an anophthalmos. On physical examination, systolic murmur was heard at the 4th left intercostal space near the sternum. However the dog appeared healthy without cyanosis and had no history of exercise intolerance. The phonocardiogram revealed a pansystolic murmur and a continuous murmur on the mitral area. A systolic ejection murmur was also recorded on the pulmonic area. The electrocardiogram indicated bi-ventricular hypertrophy. Left ventricular enlargement was seen on chest radiographs. Ventricular septal defect (VSD) and patent ductus arteriosus (PDA) were diagnosed from these findings. PDA closure was performed at 2 years of age. After 2 months from the operation, the dog died during an attempted repair of the VSD. At necropsy, it was found that the double-chambered right ventricle (DCRV) was formed by an anomalous septal band. The VSD was localized on the proximal conus and was 8 mm in diameter. (author)

  1. Double-chambered right ventricle, ventricular septal defect, patent ductus arteriosus in a dog

    International Nuclear Information System (INIS)

    Morozumi, M.; Kurosu, Y.; Kogure, K.; Chimura, S.; Shibata, S.; Kanemoto, I.

    1989-01-01

    A 4-month-old female mongrel puppy was presented with an anophthalmos. On physical examination, systolic murmur was heard at the 4th left intercostal space near the sternum. However the dog appeared healthy without cyanosis and had no history of exercise intolerance. The phonocardiogram revealed a pansystolic murmur and a continuous murmur on the mitral area. A systolic ejection murmur was also recorded on the pulmonic area. The electrocardiogram indicated bi-ventricular hypertrophy. Left ventricular enlargement was seen on chest radiographs. Ventricular septal defect (VSD) and patent ductus arteriosus (PDA) were diagnosed from these findings. PDA closure was performed at 2 years of age. After 2 months from the operation, the dog died during an attempted repair of the VSD. At necropsy, it was found that the double-chambered right ventricle (DCRV) was formed by an anomalous septal band. The VSD was localized on the proximal conus and was 8 mm in diameter. (author)

  2. Fourier ventricular amplitude ratio to evaluate atrial septal defect

    International Nuclear Information System (INIS)

    Makler, P.T. Jr.; McCarthy, D.M.; Adler, L.; Alavi, A.

    1985-01-01

    First harmonic Fourier analysis of gated blood pool scans results in the formation of two functional images, a phase and amplitude image. The authors have previously shown that the total amplitude values of the two ventricles can be used to quantitate valvular insufficiency. The ventricular amplitude ratio (VAR, left/right) in normals is 1.14 0.11 and patients with valvular insufficiency is elevated (0.3 0.77). In patients with atrial septal defect (ASD), the right ventricle has a larger stroke volume than the left ventricle, and the VAR should be less than unity. To evaluate whether the amplitude image would permit quantification of shunt flow in ASD, the authors compared the VAR to the OP/QS ratio determined by cardiac catheterization (cath) in 3 groups of patients; group I (n=9) had ASD without valvular insufficiency (one patient had right-to-left shunting due to tricuspid stenosis; group II (n=4) had ventricular septal defect; and group III (n=2) had ASD plus valvular insufficiency. QP/QS shunt flow is also determined in group I using standard first-pass radionuclide angiography (rna). The data suggest that the VAR technique accurately determines the magnitude of shunt flow in ASD patients without concomitant valvular insufficiency

  3. Time course of an X-ray picture following operation for the ventricular septal defect

    Energy Technology Data Exchange (ETDEWEB)

    Khenynya, R.L.; Latsis, A.T.; Rubene, M.Ya.

    1983-01-01

    An x-ray picture was studied over time in 80 pediatric patients by hemodynamic groups in accordance with the classification adopted in the A.I. Bakulev Institute of Cardiovascular Surgery, USSR AMS. Of them in 15 patients in addition to the ventricular septal defect, stenosis of the pulmonary artery was revealed, in 11 concomitant insufficiency of the tricuspid valve. The best results after radical correction of the defect were achieved in patients with insignificant or moderate disorder of the pulmonary hemodynamics; positive results in patients with a high pulmonary hypertension were observed over time later. X-ray studies on the regression of disorders of the pulmonary hemodynamics emphasize the necessity of early diagnosis of the ventricular septal defect followed by surgical correction.

  4. A large ventricular septal defect complicating resuscitation after blunt trauma

    Directory of Open Access Journals (Sweden)

    Henry D I De′Ath

    2012-01-01

    Full Text Available A young adult pedestrian was admitted to hospital after being hit by a car. On arrival to the Accident and Emergency Department, the patient was tachycardic, hypotensive, hypoxic, and acidotic with a Glasgow Coma Scale of 3. Despite initial interventions, the patient remained persistently hypotensive. An echocardiogram demonstrated a traumatic ventricular septal defect (VSD with right ventricular strain and increased pulmonary artery pressure. Following a period of stabilization, open cardiothoracic surgery was performed and revealed an aneurysmal septum with a single large defect. This was repaired with a bovine patch, resulting in normalization of right ventricular function. This case provides a vivid depiction of a large VSD in a patient following blunt chest trauma with hemodynamic compromise. In all thoracic trauma patients, and particularly those poorly responsive to resuscitation, VSDs should be considered. Relevant investigations and management strategies are discussed.

  5. Supracristal ventricular septal defect with severe right coronary cusp prolapse

    International Nuclear Information System (INIS)

    Hussain, A.H.; Hanif, B.; Khan, G.; Hasan, K.

    2011-01-01

    The case of a 20 years old male, diagnosed as supracristal ventricular septal defect (VSD) for last 6 years is being presented. He came in emergency department with decompensated congestive cardiac failure. After initial stabilization, he underwent trans thoracic echocardiogram which showed large supracristal VSD, severely prolapsing right coronary cusp, severe aortic regurgitation and severe pulmonary hypertension. Right heart catheterization was performed which documented reversible pulmonary vascular resistance after high flow oxygen inhalation. He underwent VSD repair, right coronary cusp was excised and aortic valve was replaced by mechanical prosthesis. Post operative recovery was uneventful. He was discharged home in one week.The case of a 20 years old male, diagnosed as supracristal ventricular septal defect (VSD) for last 6 years is being presented. He came in emergency department with decompensated congestive cardiac failure. After initial stabilization, he underwent trans thoracic echocardiogram which showed large supracristal VSD, severely prolapsing right coronary cusp, severe aortic regurgitation and severe pulmonary hypertension. Right heart catheterization was performed which documented reversible pulmonary vascular resistance after high flow oxygen inhalation. He underwent VSD repair, right coronary cusp was excised and aortic valve was replaced by mechanical prosthesis. Post operative recovery was uneventful. He was discharged home in one week. (author)

  6. Acquired ventricular septal defect due to infective endocarditis

    Directory of Open Access Journals (Sweden)

    Randi E Durden

    2018-01-01

    Full Text Available Acquired intracardiac left-to-right shunts are rare occurrences. Chest trauma and myocardial infection are well-known causes of acquired ventricular septal defect (VSD. There have been several case reports describing left ventricle to right atrium shunt after infective endocarditis (IE. We present here a patient found to have an acquired VSD secondary to IE of the aortic and tricuspid valves in the setting of a known bicuspid aortic valve. This is the first case reported of acquired VSD in a pediatric patient in the setting of IE along with literature review of acquired left-to-right shunts.

  7. The time course of an X-ray picture following operation for the ventricular septal defect

    International Nuclear Information System (INIS)

    Khenynya, R.L.; Latsis, A.T.; Rubene, M.Ya.

    1983-01-01

    An x-ray picture was studied over time in 80 pediatric patients by hemodynamic groUps in accordance with the classification adopted in the A.I.Bakulev Institute of Cardiovascular Surgery, USSR AMS. Of them in 15 patients in addition to the ventricUlar septal defect, stenosis of the pulmonary artery was revealed, in 11 concomitant insUfficiency of the tricuspid valve. The best results after radical correction of the defect were achieved in patients with insignificant or moderate disorder of the pulmonary hemdynamics; positive results in patients with a high pulmonary hypertension were observed over time later. X-ray studies on the regression of disorders of the pulmonary hemodynamics emphasize the necessity of early diagnosis of the ventricular septal defect followed by surgical correction

  8. Interventional therapy for acute myocardial infarction associated with postinfarction ventricular septal rupture: report of 6 cases

    International Nuclear Information System (INIS)

    Zhou Ling; Xie Dujiang; Dong Jing; Wu Chengquan; Tian Nailiang; Li Xiaobo; Wang Rong; Chen Shaoliang

    2014-01-01

    Objective: To evaluate the curative effect of interventional therapy in treating acute myocardial infarction (AMI) associated with postinfarction ventricular septal rupture (VSR). Methods: Transcatheter closure was performed in 6 patients with VSR which was caused by AMI. Percutaneous coronary intervention (PCI) was also carried out according to patient's clinical condition. The results were analyzed. Results: The interval between the occurrence of VSR and the performance of transcatheter closure ranged from 3 to 30 days. Of the six patients, transcatheter closure failed in two, who died from cardiogenic shock during hospitalization. The remaining 4 patients experienced a successful transcatheter closure procedure, and their cardiac function was improved during the follow-up period lasting for 0.5-4 years. Conclusion: Interventional transcatheter treatment is safe and feasible for acute myocardial infarction complicated by postinfarction ventricular septal rupture. (authors)

  9. The unnatural history of the ventricular septal defect: Outcome up to 40 years after surgical closure

    NARCIS (Netherlands)

    M.E. Menting (Myrthe); J.A.A.E. Cuypers (Judith); P. Opic (Petra); E.M.W.J. Utens (Elisabeth); M. Witsenburg (Maarten); A.E. van den Bosch (Annemien); R.T. van Domburg (Ron); F.J. Meijboom (Folkert); H. Boersma (Eric); A.J.J.C. Bogers (Ad); J.W. Roos-Hesselink (Jolien)

    2015-01-01

    textabstractBackground Few prospective data are available regarding long-term outcomes after surgical closure of a ventricular septal defect (VSD). Objectives The objective of this study was to investigate clinical outcomes >30 years after surgical VSD closure. Methods Patients who underwent

  10. Classical fluoroscopy criteria poorly predict right ventricular lead septal positioning by comparison with echocardiography.

    Science.gov (United States)

    Squara, Fabien; Scarlatti, Didier; Riccini, Philippe; Garret, Gauthier; Moceri, Pamela; Ferrari, Emile

    2018-03-13

    Fluoroscopic criteria have been described for the documentation of septal right ventricular (RV) lead positioning, but their accuracy remains questioned. Consecutive patients undergoing pacemaker or defibrillator implantation were prospectively included. RV lead was positioned using postero-anterior and left anterior oblique 40° incidences, and right anterior oblique 30° to rule out coronary sinus positioning when suspected. RV lead positioning using fluoroscopy was compared to true RV lead positioning as assessed by transthoracic echocardiography (TTE). Precise anatomical localizations were determined with both modalities; then, RV lead positioning was ultimately dichotomized into two simple clinically relevant categories: RV septal or RV free wall. Accuracy of fluoroscopy for RV lead positioning was then assessed by comparison with TTE. We included 100 patients. On TTE, 66/100 had a septal RV lead and 34/100 had a free wall RV lead. Fluoroscopy had moderate agreement with TTE for precise anatomical localization of RV lead (k = 0.53), and poor agreement for septal/free wall localization (k = 0.36). For predicting septal RV lead positioning, classical fluoroscopy criteria had a high sensitivity (95.5%; 63/66 patients having a septal RV lead on TTE were correctly identified by fluoroscopy) but a very low specificity (35.3%; only 12/34 patients having a free wall RV lead on TTE were correctly identified by fluoroscopy). Classical fluoroscopy criteria have a poor accuracy for identifying RV free wall leads, which are most of the time misclassified as septal. This raises important concerns about the efficacy and safety of RV lead positioning using classical fluoroscopy criteria.

  11. Echocardiographic identification of ventricular septal rupture caused by acute stent thrombosis.

    Science.gov (United States)

    Garg, Scot; Bourantas, Christos V; Thackray, Simon; Alamgir, Mohamed F

    2010-05-01

    Coronary stenting is an increasingly common procedure. Complications are rare. However, when they do occur, they often require urgent invasive treatment. Investigations that are critical for establishing a diagnosis as well as such guide treatment as a detailed assessment of myocardial morphology and function using transthoracic echocardiography may be overlooked in the haste to treat the patient. We present a case report of subacute drug-eluting stent thrombosis in which a meticulous echocardiographic examination allowed the identification of a ventricular septal rupture, which ultimately modified treatment.

  12. The unnatural history of the ventricular septal defect : outcome up to 40 years after surgical closure

    NARCIS (Netherlands)

    Menting, Myrthe E; Cuypers, Judith A A E; Opić, Petra; Utens, Elisabeth M W J; Witsenburg, Maarten; van den Bosch, Annemien E; van Domburg, Ron T; Meijboom, Folkert J; Boersma, Eric; Bogers, Ad J J C; Roos-Hesselink, Jolien W

    2015-01-01

    BACKGROUND: Few prospective data are available regarding long-term outcomes after surgical closure of a ventricular septal defect (VSD). OBJECTIVES: The objective of this study was to investigate clinical outcomes>30 years after surgical VSD closure. METHODS: Patients who underwent surgical VSD

  13. Transcatheter closure of membranous ventricular septal defects with home-made nitinol occluder

    Energy Technology Data Exchange (ETDEWEB)

    Yongwen, Qin; Xianxian, Zhao; Xing, Zheng; Jijun, Ding; Jiang, Cao [Second Military Medical Univ., Shanghai (China). Changhai Hospital, Dept. of Cardiology

    2004-04-01

    Objective: To evaluate the feasibility and efficacy of transcatheter perimembranous ventricular septal defects (VSD) occlusion with home-made nitinol occluder. Methods: Transcatheter closure was attempted in 196 patients with perimembranous VSD. The diameter of VSD measured by echocardiography was 3 to 15 mm, mean (4.94 {+-} 2.23) mm. The angiographic diameter of the VSD was 3 to 6 mm, mean (3.92 {+-} 1.44) mm. A 7-10 F delivery sheath was advanced across the perimembranous VSD over a wire from femoral vein to deploy the occluder with the guidance of echocardiography and fluoroscopy. The device diameter selected was from 4 to 20 mm, mean (6.68 {+-} 2.76) mm. Left ventriculography and transthoracic echocardiography were repeated to assess the closure of the defects 15 min after the procedure. Continuous electrocardiogram monitoring lasted for 5 days. The echocardiography and electrocardiogram examination were scheduled for 1, 6 and 12 months of follow-up. Results: The occluders were successfully deployed in 191 patients. There were five procedural failures, two with device-related aortic insufficiency, and three of inability to pass through VSD. After deployment of the devices, there were no residual shunt in 180 of 191 patients, 11 patients with a trivial residual shunt that disappeared in 8 patients after one month of follow up. 3 patients developed mild tricuspid insufficiency. 12 developed transient complete right bundle branch block, and 5 transient complete left bundle branch block, and 2 transient complete atrioventricular block. There were repetitive nonparoxysmal ventricular tachycardia in 4 patients 1 week after the procedure. One patient had a detached device embolized into the left pulmonary artery but with a successful catheter retrieval by snare and transcatheter closure. The devices were similarly applied to patients with VSD associated with patent ductus arteriosus, and 4 patients with VSD complicated by atrial septal defects. The fluoroscopy time

  14. The unnatural history of the ventricular septal defect: outcome up to 40 years after surgical closure

    NARCIS (Netherlands)

    Menting, Myrthe E.; Cuypers, Judith A. A. E.; Opić, Petra; Utens, Elisabeth M. W. J.; Witsenburg, Maarten; van den Bosch, Annemien E.; van Domburg, Ron T.; Meijboom, Folkert J.; Boersma, Eric; Bogers, Ad J. J. C.; Roos-Hesselink, Jolien W.

    2015-01-01

    Few prospective data are available regarding long-term outcomes after surgical closure of a ventricular septal defect (VSD). The objective of this study was to investigate clinical outcomes>30 years after surgical VSD closure. Patients who underwent surgical VSD closure during childhood between 1968

  15. Ventricular septal necrosis after blunt chest trauma

    Directory of Open Access Journals (Sweden)

    Alireza Ahmadi

    2012-07-01

    Full Text Available Ventricular Septal Defect (VSD after blunt chest trauma is a very rare traumatic affection.We report here a case of blunt chest injury-related VSD and pseudoaneurysm.A 30-year old male truck driver was referred from a trauma center to our hospital seven days after a blunt chest trauma and rib fracture. The patient had severe pulmonary edemaand echocardiography showed large VSD. Several mechanisms are involved in the pathogenesis of this affection including an acute compression of the heart muscle between the sternum and the spine, leading to excessive changes in the intrathoracic and most likely theintracardiac pressure after blunt chest injury. Traumatical patients with the same symptoms may be at risk of sudden death. Therefore, a high grade of suspicion is mandatory even without solid evidence of myocardial damage on the initial evaluation. In continue somehidden angles of this case was discussed. Given the prognostic implications of traumatic VSD with associated pseudoaneurysm, its detection has critical value for preventing its clinicalsequelae.

  16. Muscular anatomy of the human ventricular folds.

    Science.gov (United States)

    Moon, Jerald; Alipour, Fariborz

    2013-09-01

    Our purpose in this study was to better understand the muscular anatomy of the ventricular folds in order to help improve biomechanical modeling of phonation and to better understand the role of these muscles during phonatory and nonphonatory tasks. Four human larynges were decalcified, sectioned coronally from posterior to anterior by a CryoJane tape transfer system, and stained with Masson's trichrome. The total and relative areas of muscles observed in each section were calculated and used for characterizing the muscle distribution within the ventricular folds. The ventricular folds contained anteriorly coursing thyroarytenoid and ventricularis muscle fibers that were in the lower half of the ventricular fold posteriorly, and some ventricularis muscle was evident in the upper and lateral portions of the fold more anteriorly. Very little muscle tissue was observed in the medial half of the fold, and the anterior half of the ventricular fold was largely devoid of any muscle tissue. All 4 larynges contained muscle bundles that coursed superiorly and medially through the upper half of the fold, toward the lateral margin of the epiglottis. Although variability of expression was evident, a well-defined thyroarytenoid muscle was readily apparent lateral to the arytenoid cartilage in all specimens.

  17. Case report: paradoxical ventricular septal motion in the setting of primary right ventricular myocardial failure.

    Science.gov (United States)

    Maslow, Andrew; Schwartz, Carl; Mahmood, Feroze; Singh, Arun; Heerdt, Paul M

    2009-07-01

    In this report, a case of right ventricular (RV) failure, hemodynamic instability, and systemic organ failure is described to highlight how paradoxical ventricular systolic septal motion (PVSM), or a rightward systolic displacement of the interventricular septum, may contribute to RV ejection. Multiple inotropic medications and vasopressors were administered to treat right heart failure and systemic hypotension in a patient following combined aortic and mitral valve replacement. In the early postoperative period, echocardiographic evaluation revealed adequate left ventricular systolic function, akinesis of the RV myocardial tissues, and PVSM. In the presence of PVSM, RV fractional area of contraction was > or =35% despite akinesis of the primary RV myocardial walls. The PVSM appeared to contribute toward RV ejection. As a result, the need for multiple inotropes was re-evaluated, in considering that end-organ dysfunction was the result of systemic hypotension and prolonged vasopressor administration. After discontinuation of phosphodiesterase inhibitors, native vascular tone returned and the need for vasopressors declined. This was followed by recovery of systemic organ function. Echocardiographic re-evaluation two years later, revealed persistent akinesis of the RV myocardial tissues and PVSM, the latter appearing to contribute toward RV ejection. This case highlights the importance of left to RV interactions, and how PVSM may mediate these hemodynamic interactions.

  18. A neonate with mitral stenosis due to accessory mitral valve, ventricular septal defect, and patent ductus arteriosus: changes in echocardiographical findings during the neonatal period.

    Science.gov (United States)

    Ito, Tadahiko; Okubo, Tadashi

    2002-12-01

    A female neonate with mitral stenosis due to accessory mitral valve with ventricular septal defect and patent ductus arteriosus is described. She was referred to our hospital because of neonatal asphyxia. Asphyxia was improved by ventilator support, but rapid deterioration of respiration with pulmonary congestion and hemorrhage appeared 8 days after birth. Echocardiography revealed an accessory mitral valve attached to the anterior mitral leaflet with a perimembranous ventricular septal defect and patent ductus arteriosus. Although there were no echocardiographical findings indicating mitral stenosis on admission, the mitral stenosis blood flow patterns were detected by color and pulsed Doppler examination performed on the eighth day after admission. Transaortic resection of accessory mitral valve tissue was performed with patch closure of the ventricular septal defect and ligation of the ductus arteriosus 35 days after birth. After operation, pulmonary congestion and hemorrhage were improved. Postoperative echocardiography showed complete resection of the accessory mitral valve and no mitral insufficiency. We concluded that the combination of the accessory mitral valve and left-to-right shunt due to ventricular septal defect or patent ducturs arteriosus might have led to a critical hemodynamic condition due to relative mitral stenosis in the neonatal period with the decrease in pulmonary vascular resistance.

  19. Closure of large patent ductus arteriosus using the Amplatzer Septal Occluder.

    Science.gov (United States)

    García-Montes, José A; Camacho-Castro, Anahí; Sandoval-Jones, Juan P; Buendía-Hernández, Alfonso; Calderón-Colmenero, Juan; Patiño-Bahena, Emilia; Zabal, Carlos

    2015-03-01

    Percutaneous closure of patent ductus arteriosus has become the treatment of choice in many centres. In patients with large ducts and pulmonary hypertension, transcatheter closure has been achieved with success using the Amplatzer Duct Occluder or even the Amplatzer Muscular Ventricular Septal Defect Occluder. We present a series of 17 patients with large and hypertensive ductus arteriosus who were treated with an Amplatzer Septal Occluder. The group had 11 female patients (64.7%) and a mean age of 18.6±12.1 years. The haemodynamic and anatomical data are as follows: pulmonary artery systolic pressure 71.3±31.8 mmHg, pulmonary to systemic flow ratio 3.14±1.36, ductal diameter at the pulmonary end 12.5±3.8 mm, and at the aortic end 20.2±7.7 mm; 14 cases (82.3%) had type A ducts. In 11 patients, we began the procedure using a different device - six with duct occluder and five with ventricular septal occluder - and it was changed because of device embolisation in six (35.3%). All septal occluders were delivered successfully. Residual shunt was moderate in six patients (35.3%), mild in eight (47%), trivial in two (11.8%), and no shunt in one (5.9%). Pulmonary systolic pressure decreased to 48.9±10.8 mmHg after occlusion (p=0.0015). Follow-up in 15 patients (88.2%) for 28.4±14.4 months showed complete closure in all cases but one, and continuous decrease of the pulmonary systolic pressure to 31.4±10.5 mmHg. No complications at follow-up have been reported. The Amplatzer Septal Occluder is a good alternative to percutaneously treat large and hypertensive ductus arteriosus.

  20. Off-Pump Repair of a Post Myocardial Infarction Ventricular Septal Defect

    Directory of Open Access Journals (Sweden)

    Feridoun Sabzi

    2014-01-01

    Full Text Available Refractory cardiogenic shock meant that traditional patch repairs requiring cardiopulmonary bypass would be poorly tolerated and external sandwich closure of post myocardial ventricular septal defect (VSD appears to be simple and effective after initial myocardial infarction (MI. The three cases presented with a VSD after of acute MI with or without thrombolysed with streptokinase during patient admission. The general condition of the three patients was poor with pulmonary edema, low cardiac output and renal failure. The heart was approached through a median sternotomy. Off-pump coronary artery bypass grafting of the coronary artery lesion was done first using octopus and beating heart surgery method and latero - lateral septal plication was performed using sandwich technique. Low cardiac output managed with intra-aortic balloon pump in these patients accompanied with inotropic drugs. Post-operative transesophageal echocardiography revealed that VSD was closed completely in one patient and in two patients small residual VSD remained. More experience is required to ascertain whether this technique will become an accepted alternative to patch repairs.

  1. Membranous Septal Aneurysm: An Unusual Case for Sub-Pulmonary Obstruction in cTGA

    Directory of Open Access Journals (Sweden)

    Onur Isik

    2013-10-01

    Full Text Available Aneurysm of the membranous portion of the interventricular septum is an uncommon congenital cardiac malformation that is rarely diagnosed during life. Perimembranous ventricular septal defects are the most common type of the ventricular septal defects and originates from morphologically membranous interventricular septum. Surgical exposure and accurate closure of a ventricular septal defect with a membranous septal aneurysm beneath the tricuspid septal leaflet carries a risk of tricuspid valve insufficiency and conduction disturbances. The current study presents a case with membranous septal aneursym with congenitally corrected transposition of the great arteries and sub-pulmonary obstruction, which was surgically corrected.

  2. Transcatheter closure of membranous ventricular septal defects with home-made nitinol occluder

    International Nuclear Information System (INIS)

    Qin Yongwen; Zhao Xianxian; Zheng Xing; Ding Jijun; Cao Jiang

    2004-01-01

    Objective: To evaluate the feasibility and efficacy of transcatheter perimembranous ventricular septal defects (VSD) occlusion with home-made nitinol occluder. Methods: Transcatheter closure was attempted in 196 patients with perimembranous VSD. The diameter of VSD measured by echocardiography was 3 to 15 mm, mean (4.94 ± 2.23) mm. The angiographic diameter of the VSD was 3 to 6 mm, mean (3.92 ± 1.44) mm. A 7-10 F delivery sheath was advanced across the perimembranous VSD over a wire from femoral vein to deploy the occluder with the guidance of echocardiography and fluoroscopy. The device diameter selected was from 4 to 20 mm, mean (6.68 ± 2.76) mm. Left ventriculography and transthoracic echocardiography were repeated to assess the closure of the defects 15 min after the procedure. Continuous electrocardiogram monitoring lasted for 5 days. The echocardiography and electrocardiogram examination were scheduled for 1, 6 and 12 months of follow-up. Results: The occluders were successfully deployed in 191 patients. There were five procedural failures, two with device-related aortic insufficiency, and three of inability to pass through VSD. After deployment of the devices, there were no residual shunt in 180 of 191 patients, 11 patients with a trivial residual shunt that disappeared in 8 patients after one month of follow up. 3 patients developed mild tricuspid insufficiency. 12 developed transient complete right bundle branch block, and 5 transient complete left bundle branch block, and 2 transient complete atrioventricular block. There were repetitive nonparoxysmal ventricular tachycardia in 4 patients 1 week after the procedure. One patient had a detached device embolized into the left pulmonary artery but with a successful catheter retrieval by snare and transcatheter closure. The devices were similarly applied to patients with VSD associated with patent ductus arteriosus, and 4 patients with VSD complicated by atrial septal defects. The fluoroscopy time for the

  3. Nuclear magnetic resonance zeugmatographic imaging of the heart: application to the study of ventricular septal defect

    International Nuclear Information System (INIS)

    Heneghan, M.A.; Biancaniello, T.M.; Heidel, E.; Peterson, S.B.; Marsh, M.J.; Lauterbur, P.C.

    1982-01-01

    The present work was undertaken to determine the applicability of nuclear magnetic resonance (NMR) imaging to the study of congenital heart disease. Three-dimensional proton density images of preserved lamb hearts with and without an artificially created ventricular septal defect were reconstructed and displayed in multiple planes. Sections obtained in the sagittal plane through the ventricular septum clearly showed the size, shape, and location of the defect. Results of these experiments suggest that NMR zeugmatography will become a valuable addition to existing imaging techniques for the study of congenital heart disease

  4. Potts shunt in a child with end-stage pulmonary hypertension after late repair of ventricular septal defect

    DEFF Research Database (Denmark)

    Petersen, Cecilie; Helvind, Morten; Jensen, Tim

    2013-01-01

    We report on a 10-year-old boy with medically refractory pulmonary arterial hypertension (PAH) and end-stage right heart failure after closure of a ventricular septal defect. The boy was a candidate for lung transplantation (LTX), but an alternative option was to create an Eisenmenger physiology ...

  5. Multimodality cardiac imaging of a ventricular septal rupture post myocardial infarction: a case report

    Directory of Open Access Journals (Sweden)

    Dhaliwal Surinder

    2012-10-01

    Full Text Available Abstract Background Ventricular septal rupture (VSR, a mechanical complication following an acute myocardial infarction (MI, is thought to result from coagulation necrosis due to lack of collateral reperfusion. Although the gold standard test to confirm left-to-right shunting between ventricular cavities remains invasive ventriculography, two-dimensional transthoracic echocardiography (TTE with color flow Doppler and cardiac MRI (CMR are reliable tests for the non-invasive diagnosis of VSR. Case presentation A 62-year-old Caucasian female presented with a late case of a VSR post inferior MI diagnosed by multimodality cardiac imaging including TTE, CMR and ventriculography. Conclusion We review the presentation, diagnosis and management of VSR post MI.

  6. Virtual reality 3D echocardiography in the assessment of tricuspid valve function after surgical closure of ventricular septal defect

    NARCIS (Netherlands)

    G. Bol-Raap (Goris); A.H.J. Koning (Anton); T.V. Scohy (Thierry); A.D.J. ten Harkel (Arend); F.J. Meijboom (Folkert); A.P. Kappetein (Arie Pieter); P.J. van der Spek (Peter); A.J.J.C. Bogers (Ad)

    2007-01-01

    textabstractBackground. This study was done to investigate the potential additional role of virtual reality, using three-dimensional (3D) echocardiographic holograms, in the postoperative assessment of tricuspid valve function after surgical closure of ventricular septal defect (VSD). Methods. 12

  7. Right ventricular load and function during exercise in patients with open and closed atrial septal defect type secundum

    NARCIS (Netherlands)

    Pieter De Meester; Roselien Buys; Werner Budts; Marion Delcroix; Prof. Dr. Luc L.E.M.J. Vanhees; Jens-Uwe Voigt; Alexander Van de Bruaene

    2013-01-01

    Abstract Purpose: This study aimed at evaluating (1) right ventricular (RV) mean power during exercise, (2) the contribution of flow and pressure to RV mean power, and (3) the impact of pulmonary artery pressure on RV function during exercise. Methods: Fifty patients with atrial septal defect (ASD)

  8. Disincronía ventricular izquierda en la estimulación septal prolongada

    OpenAIRE

    Ferrando-Castagnetto, Federico; Ricca-Mallada, Roberto; Vidal, Alejandro; Martínez, Fabián; Ferrando, Rodolfo

    2016-01-01

    La estimulación por marcapasos se asocia con eventos adversos graves. En la siguiente serie de casos se evaluó la disincronía mecánica intraventricular izquierda (DMVI) en la estimulación endocárdica prolongada del ventrículo derecho a nivel septal. Se estudiaron 6 personas con marcapasos implantados no antes de un año utilizando SPECT gatillado con 99mTc-MIBI y análisis de fase en reposo. Se registraron variables clínicas, duración del QRS, tasa y modo de estimulación ventricular, presencia ...

  9. Clinical significance of right ventricular ejection fraction in cases with atrial septal defect

    Energy Technology Data Exchange (ETDEWEB)

    Hamada, Seiki; Nishimura, Tsunehiko; Hayashida, Kouhei; Uehara, Toshiisa

    1989-04-01

    Right ventricular ejection fraction (RVEF) assessed by cardiac radionuclide angiography has been applied to evaluate ventricular function such as ischemic, valvular and congenital heart disease. Using this modality, previous reports also suggest that there is good correlation between RVEF and mean pulmonary arterial pressure (mPA) from catheterization findings in mitral valvular disease and chronic obstructive lung disease. In this study, cardiac RI angiography were performed on 33 adult patients with atrial septal defect (ASD). RVEF is not so good correlation (r=-0.42) with mPA, but in cases within pulmonary to systemic ratio (Qp/Qs) less than 2.0 limits, there is good correlation between RVEF and mPA (n=9, r=-0.71). As a conclusion, in ASD, both afterload assessed by mPA and preload assessed by Qp/Qs decrease RVEF. (author).

  10. Isolated pulmonary regurgitation causes decreased right ventricular longitudinal function and compensatory increased septal pumping in a porcine model

    DEFF Research Database (Denmark)

    Kopic, S; Stephensen, S S; Heiberg, E

    2017-01-01

    AIM: Longitudinal ventricular contraction is a parameter of cardiac performance with predictive power. Right ventricular (RV) longitudinal function is impaired in patients with free pulmonary regurgitation (PR) following corrective surgery for Tetralogy of Fallot (TOF). It remains unclear whether...... received a stent in the pulmonary valve orifice, inducing PR. After 2-3 months, animals were subjected to cardiac magnetic resonance imaging. A subset of animals (n = 6) then underwent percutaneous pulmonary valve replacement (PPVR) with follow-up 1 month later. Longitudinal, lateral and septal...

  11. Situs inversus totalis associated with subaortic stenosis, restrictive ventricular septal defect, and tricuspid dysplasia in an adult dog.

    Science.gov (United States)

    Piantedosi, Diego; Cortese, Laura; Meomartino, Leonardo; Di Loria, Antonio; Ciaramella, Paolo

    2011-11-01

    A rare association between situs inversus totalis (SIT), restrictive ventricular septal defect, severe subaortic stenosis, and tricuspid dysplasia was observed in an adult mixed-breed dog. Primary ciliary dyskinesia and Kartagener's syndrome were excluded. After 15 mo the dog died suddenly. The association between SIT and congenital heart diseases is discussed.

  12. Imaging of the ventricular septal wall and the portal vein by digital subtraction angiography

    Energy Technology Data Exchange (ETDEWEB)

    Fujii, Kyoichi; Harashima, Haruhiko; Yamada, Hiromi; Hiraishi, Takahisa; Mikami, Fumio [National Medical Center of Hospital, Tokyo (Japan)

    1983-06-01

    Digital subtraction angiography (DSA) was performed in 2 cases of hypertrophic cardiomyopathy and 2 cases of portal vein disorder. In hypertrophic cardiomyopathy, DSA with the infusion of isopaque 370 at the speed of 5 to 7 ml/sec produced the image of the ventricular septal wall, showing a small ejection fraction. Of portal vein disorders, DSA revealed the portal vein and was helpful in diagnosing tumoral thrombosis of the portal vein in a case of liver carcinoma and portal hypertension.

  13. Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect

    Directory of Open Access Journals (Sweden)

    L. Rozendaal

    2011-01-01

    Full Text Available We describe an infant presenting with contractures of the fingers, a large ventricular septal defect (VSD, and severe pulmonary artery dilatation. He had clinical and echocardiographic features of both neonatal or infantile Marfan syndrome (MFS and congenital contractural arachnodactyly. After surgical VSD closure, the aortic root developed progressive dilatation while the size of pulmonary artery returned to normal limits. Eventually the diagnosis of MFS was confirmed by DNA analysis.

  14. Situs inversus totalis associated with subaortic stenosis, restrictive ventricular septal defect, and tricuspid dysplasia in an adult dog

    OpenAIRE

    Piantedosi, Diego; Cortese, Laura; Meomartino, Leonardo; Di Loria, Antonio; Ciaramella, Paolo

    2011-01-01

    A rare association between situs inversus totalis (SIT), restrictive ventricular septal defect, severe subaortic stenosis, and tricuspid dysplasia was observed in an adult mixed-breed dog. Primary ciliary dyskinesia and Kartagener’s syndrome were excluded. After 15 mo the dog died suddenly. The association between SIT and congenital heart diseases is discussed.

  15. Early closure of postinfarction ventricular septal defects.

    Science.gov (United States)

    Martinelli, Luigi; Dottori, Vincenzo; Caputo, Enrico; Graffigna, Angelo; Pederzolli, Carlo

    2003-05-01

    According to the guidelines of the American College of Cardiology/American Heart Association early closure of postinfarction septal defects is now a class I indication although it still carries a relevant morbidity and mortality. The operative risk is related both to the critical hemodynamic conditions of the patient and to the technical difficulties posed by the friable tissue of the infarcted area. The most recent techniques involving the use of pericardial patches reinforced by acrylic glue have significantly reduced the hospital mortality. The aim of this study was to discuss the reliability of an aggressive, tissue-sparing surgical approach to this complication. We present a consecutive series of 12 patients operated upon between January 1998 and October 2001 within 12 hours of the onset of clinical evidence of postinfarction septal rupture. Repair was achieved with minimal septal debridement and the use of a large pericardial patch reinforced by a biological glue. Three cases of dehiscence required early reoperation with no hospital mortality. This procedure is technically feasible and allows early aggressive treatment of postinfarction septal rupture with satisfactory results.

  16. MORTAL PULPOTOMY ON CHILDREN WITH VENTRICULAR SEPTAL DEFECT

    Directory of Open Access Journals (Sweden)

    Devi N. R. Devy

    2006-04-01

    Full Text Available Congenital heart disease is a condition of heart anomaly found since birth. The most common is ventricular septal defect whereby an aperture is found in the partition of heart chamber. It is estimated that 40,000 newborn in Indonesia have this defective condition. Dental treatment for patients with such condition must be undertaken in a very cautious way. Tooth with multiple caries can potentially lead to endocarditis bacteria. As such, dentist must be watchful on dental treatment that may worsen the patient’s condition. To prevent endocarditis bacteria, a prophylaxis antibiotic is required as recommended by the American Heart Association (AHA. In this particular case, a dental treatment was undertaken to a child patient with congenital heart disease. Previously, the patient has undergone dental treatment in the form of multiple extractions under general anesthesia before conducting cardiac surgery. In this case the treatment includes mortal pulpotomy, GIC restoration, and fissure sealant – all conducted in one visit under general anesthesia. A year after the treatment, there are no complaints from the patient and no irregularity on x-ray results.

  17. Is right ventricular mid-septal pacing superior to apical pacing in patients with high degree atrio-ventricular block and moderately depressed left ventricular function?

    Science.gov (United States)

    Chen, Kang; Mao, Ye; Liu, Shao-hua; Wu, Qiong; Luo, Qing-zhi; Pan, Wen-qi; Jin, Qi; Zhang, Ning; Ling, Tian-you; Chen, Ying; Gu, Gang; Shen, Wei-feng; Wu, Li-qun

    2014-06-01

    We are aimed to investigate whether right ventricular mid-septal pacing (RVMSP) is superior to conventional right ventricular apical pacing (RVAP) in improving clinical functional capacity and left ventricular ejection fraction (LVEF) for patients with high-degree atrio-ventricular block and moderately depressed left ventricle (LV) function. Ninety-two patients with high-degree atrio-ventricular block and moderately reduced LVEF (ranging from 35% to 50%) were randomly allocated to RVMSP (n=45) and RVAP (n=47). New York Heart Association (NYHA) functional class, echocardiographic LVEF, and distance during a 6-min walk test (6MWT) were determined at 18 months after pacemaker implantation. Serum levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) were measured using an enzyme-linked immunosorbent assay (ELISA) kit. Compared with baseline, NYHA functional class remained unchanged at 18 months, distance during 6MWT (485 m vs. 517 m) and LVEF (36.7% vs. 41.8%) were increased, but BNP levels were reduced (2352 pg/ml vs. 710 pg/ml) in the RVMSP group compared with those in the RVAP group, especially in patients with LVEF 35%-40% (for all comparisons, Pfunction capacity and LV function measurements were not significantly changed in patients with RVAP, despite the pacing measurements being similar in both groups, such as R-wave amplitude and capture threshold. RVMSP provides a better clinical utility, compared with RVAP, in patients with high-degree atrioventricular block and moderately depressed LV function whose LVEF levels ranged from 35% to 40%.

  18. THE RESTRICTED SURGICAL RELEVANCE OF MORPHOLOGIC CRITERIA TO CLASSIFY SYSTEMIC-PULMONARY COLLATERAL ARTERIES IN PULMONARY ATRESIA WITH VENTRICULAR SEPTAL-DEFECT

    NARCIS (Netherlands)

    DERUITER, MC; GITTENBERGERDEGROOT, AC; BOGERS, AJJC; ELZENGA, NJ

    1994-01-01

    Now that systemic-pulmonary collateral arteries are used for unifocalization in patients with pulmonary atresia and ventricular septal defect, the question arises whether morphologic criteria of these collateral arteries could help to provide better results. In an attempt to classify the morphologic

  19. Evaluation of Paradoxical Septal Motion Following Cardiac Surgery with Gated Cardiac Blood Pool Scan

    International Nuclear Information System (INIS)

    Shin, Seong Hae; Chung, June Key; Lee, Myung Chul; Cho, Bo Youn; Koh, Chang Soon; Suh, Kyung Phil

    1985-01-01

    The development of paradoxical interventricular septal motion is a common consequence of cardiopulmonary bypass operation. The reason for this postoperative abnormal septal motion is not clear. 41 patients were studied preoperatively and postoperatively with radionuclide blood pool scan to evaluate the frequency of development of paradoxical septal motion with right ventricular volume overload before surgery and the frequency of development of paradoxical septal motion after cardiac surgery with cardiopulmonary bypass, and to evaluate the change of EF related to the development of paradoxical septal motion after cardiac surgery. The results were as follows; 1) 7 of 41 patients with right ventricular volume overload (that is 17%) showed paradoxical septal motion before surgery. But 13 of 34 patients (that is 42%) had paradoxical septal motion after cardiac surgery with cardiopulmonary bypass. So open heart surgery with cardiopulmonary bypass related the development of paradoxical septal motion after surgery. 2) EF significantly decreased in patients who developed paradoxical septal motion after surgery, whereas the EF did not change in the patients who retained normal interventricular septal motion after surgery. So paradoxical septal motion usually reflected some diminution of left ventricular function, immediately after cardiac surgery.

  20. Evaluation of Paradoxical Septal Motion Following Cardiac Surgery with Gated Cardiac Blood Pool Scan

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Seong Hae; Chung, June Key; Lee, Myung Chul; Cho, Bo Youn; Koh, Chang Soon; Suh, Kyung Phil [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1985-03-15

    The development of paradoxical interventricular septal motion is a common consequence of cardiopulmonary bypass operation. The reason for this postoperative abnormal septal motion is not clear. 41 patients were studied preoperatively and postoperatively with radionuclide blood pool scan to evaluate the frequency of development of paradoxical septal motion with right ventricular volume overload before surgery and the frequency of development of paradoxical septal motion after cardiac surgery with cardiopulmonary bypass, and to evaluate the change of EF related to the development of paradoxical septal motion after cardiac surgery. The results were as follows; 1) 7 of 41 patients with right ventricular volume overload (that is 17%) showed paradoxical septal motion before surgery. But 13 of 34 patients (that is 42%) had paradoxical septal motion after cardiac surgery with cardiopulmonary bypass. So open heart surgery with cardiopulmonary bypass related the development of paradoxical septal motion after surgery. 2) EF significantly decreased in patients who developed paradoxical septal motion after surgery, whereas the EF did not change in the patients who retained normal interventricular septal motion after surgery. So paradoxical septal motion usually reflected some diminution of left ventricular function, immediately after cardiac surgery.

  1. Transcatheter closure of ventricular septal defects with nitinol wire occluders of type patent ductus arteriosus.

    Science.gov (United States)

    Wierzyk, Arkadiusz; Szkutnik, Małgorzata; Fiszer, Roland; Banaszak, Paweł; Pawlak, Szymon; Białkowski, Jacek

    2014-01-01

    Ventricular septal defects closure (VSD) depending on the anatomy and clinical setting can be performed surgically or by a hybrid and transcatheter approach. Two cases of children with VSD will be presented. Patients' defects were closed with various types of occluders made of nitinol wire mesh occluder, patent ductus arteriosus (PDA) type. The first case was a 2.5-year-old boy after cardiosurgical correction of tetralogy of Fallot (TOF). After the procedure, a significant haemodynamic residual VSD was observed, which was not successfully closed during the subsequent reoperation. Despite pharmacological treatment, symptoms of heart failure were observed in this patient. In echocardiographic images the residual VSD was presented as a tunnel-like dissection of the ventricular septum (length 6 mm and diameter 3.4 mm). The defect was closed via arterial access with an Amplatzer Duct Occluder II (ADO II). The procedure was successfully performed without any medical complications. In this child, a significant shunt reduction and a noticeable improvement in the patient's clinical status and diminished symptoms of heart failure were noticed. The second patient was a 4-year-old girl suffering from a multi-perforated perimembranous VSD accompanied by a ventricular septal defect with aneurysm. The defect was closed by a venous approach with a PDA Cardio-O-Fix occluder (very similar to ADO I). No short-term or long-term complications were visible during or after the procedure. Only a mild residual shunt through the VSD was observed 6 months afterwards. Transcatheter VSD closure with a proper morphology, with occluders of type Amplatzer Duct Occluder ADO I or ADO II, constitutes a safe and effective therapeutic alternative.

  2. Surgical anatomy of the atrioventricular conduction bundle in anomalous muscle bundle of the right ventricle with subarterial ventricular septal defect

    NARCIS (Netherlands)

    Kurosawa, H.; Becker, A. E.

    1985-01-01

    A stillborn baby girl was found to have an anomalous muscle bundle of the right ventricle, associated with a doubly committed subarterial ventricular septal defect. The latter was separated from the area of the atrioventricular conduction bundle by muscle. Serial histologic sectioning of the

  3. Surgical treatment of atrial and ventricular septal defects after unsuccessful interventional therapy: a retrospective analysis

    International Nuclear Information System (INIS)

    Han Hongguang; Zhang Nanbin; Wang Zengwei; Wang Huishan; Zhu Hongyu; Li Xinmin

    2010-01-01

    Objective: To investigate the causes of failure in treating atrial septal defect (ASD) and ventricular septal defect (VSD) with interventional procedures and to evaluate the clinical efficacy of surgical treatment in order to increase the successful rate. Methods: A total of 13 patients, who underwent surgical therapy because of unsuccessful interventional treatment for ASD or VSD during the period of January 2001-December 2007, were selected,and the clinical data were retrospectively analyzed. The surgical indications included the occluder abscission (n=7), III degree atrioventricular conduction block (n=3), valvular regurgitation (n=2), residual shunt (n=1) and interventional failure (n=1). The cardiac surgeries, including removal of the displaced occluder and / or the repair of atrioventricular septal defects, were performed with the help of cardiopulmonary bypass in all 13 cases. After surgical treatment, all patients were transferred into ICU for further supervision and treatment. Results: The average diameter of ASD on surgical exploration was 31 mm, which was greater than the preoperative average diameter (26 mm), with a significant difference (P 0.05). The III degree atrioventricular conduction block in 3 cases restored sinus rhythm after operation. All the procedures were successfully completed in all patients. No death occurred during the hospitalization period. Conclusion: Proper and timely cardiac surgery is an effective and safe measure for the treatment of the complications due to unsuccessful interventional therapy as well as the atrioventricular septal defect itself. (authors)

  4. Anesthetic management of Amplatzer atrial septal defect closure device embolization to right ventricular outflow tract

    Directory of Open Access Journals (Sweden)

    S Das

    2016-01-01

    Full Text Available Percutaneous device closure of atrial septal defect (ASD is an alternative treatment to surgery with advantages of avoidance of surgery, short procedure time, early discharge from hospital, and lower rates of complications. However, percutaneous device closure is associated with infrequent life-threatening complications such as device embolization. We report a case device embolization of the ASD occlude device into right ventricular outflow tract resulting progressive hypoxia. The role of anesthesiologist as a team leader in managing such emergency is discussed.

  5. Does septal thickness influence outcome of myectomy for hypertrophic obstructive cardiomyopathy?

    Science.gov (United States)

    Nguyen, Anita; Schaff, Hartzell V; Nishimura, Rick A; Dearani, Joseph A; Geske, Jeffrey B; Lahr, Brian D; Ommen, Steve R

    2018-03-01

    Patients with hypertrophic obstructive cardiomyopathy and basal septal thickness 21 mm, n = 505), were performed with the Kruskal-Wallis and the Pearson χ2 tests and semiparametric analysis of covariance. Median group ages were 57, 57 and 54 years (P = 0.007); men comprised 50.4%, 56.7% and 62.0%, respectively (P = 0.003). Intrinsic MV disease was present in 5.9%, 5.2% and 4.6%, respectively (P = 0.80). All patients underwent transaortic septal myectomy. Additional mitral procedures were performed in 7.6%, 7.8% and 8.1%, respectively (P = 0.90). Reasons for MV surgery included intrinsic MV disease (66.7%), residual mitral regurgitation (30.8%) and residual gradient (2.6%). All groups had postoperative gradient relief (median reduction: 51, 54 and 50 mmHg; P = 0.11). Ventricular septal defect occurred in 4 patients (0.3%), and risk did not differ by group (P = 0.24). Adequate relief of left ventricular outflow tract obstruction can be achieved via transaortic septal myectomy without concomitant MV procedures when septal thickness is < 18 mm, and the risk of ventricular septal defect is minimal. Concomitant MV repair/replacement should be reserved for patients with intrinsic MV disease or inadequate relief of mitral regurgitation/left ventricular outflow tract obstruction following adequate extended septal myectomy. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  6. The off-label use of the Amplatzer muscular VSD occluder for large patent ductus arteriosus: a case report and review.

    Science.gov (United States)

    Cubeddu, Roberto J; Babin, Ivan; Inglessis, Ignacio

    2014-07-01

    A percutaneous approach to the closure of patent ductus arteriosus (PDA) is the preferred procedure in the majority of cases. However, there is little experience with percutaneous closure of unusually large PDA. We report the case of a 28-year-old female with moderate left ventricular dilation and pulmonary hypertension resulting from a large 16 mm PDA. Percutaneous closure was successfully performed using an off-label Amplatzer muscular ventricular septal defect occluder after intravascular ultrasound assessment. Technical challenges, including accurate assessment of defect size and device selection are exemplified along with a comprehensive overview of the available literature.

  7. Septal Leaflet versus Chordal Detachment in Closure of Hard-To-Expose Ventricular Septal Defects.

    Science.gov (United States)

    Pourmoghadam, Kamal K; Boron, Agnieszka; Ruzmetov, Mark; Narasimhulu, Sukumar Suguna; Kube, Alicia; O'Brien, Michael C; DeCampli, William M

    2018-04-04

    Different techniques have been used for exposure of ventricular septal defect (VSD) margins when there is crowding of the VSD anatomy by tricuspid valve (TV) subvalvar apparatus. The aim of this study was to compare surgical outcomes, for the two techniques of TV leaflet detachment and the rarely described TV chordal detachment for hard-to-expose VSDs. Patients undergoing transatrial VSD repair were identified from our institutional database. Follow-up echocardiography and patient data were obtained from medical records. Between 1/2005-8/2016, 130 isolated conoventricular VSDs were repaired. Among these, 26patients had leaflet detachment, while 15 underwent chordal detachment, and 89 had regular VSD repair (reference group). There was no significant difference between the groups in age, weight, postoperative length-of-stay, genetic/syndromic abnormalities, time-to-extubation, and left and right ventricular systolic function. The cardiopulmonary bypass and cross-clamp time were significantly higher in leaflet detachment group, when compared with reference group (118+28vs102+32, p=0.02; and 73+20vs61+23, p=0.01, respectively). Echocardiographic follow-up were available for 87patients at a mean of 2.6years (1month-11years). Tricuspid regurgitation was rated as none or trivial in 66(76%), mild in 20(23%) and moderate in one reference group patient. There was no difference in presence of residual VSD, or degree of tricuspid regurgitation amongst the three groups. There was no reoperation for tricuspid regurgitation. Tricuspid valve leaflet and chordal detachment techniques provide equally viable and safe alternative to closure of hard-to-expose VSDs while maintaining appropriate TV function. Their use in our series did not lead to increased TV dysfunction at early-to-midterm echocardiographic assessment. Copyright © 2018. Published by Elsevier Inc.

  8. Normal development of the muscular region of the interventricular septum--I. The significance of the ventricular trabeculations.

    Science.gov (United States)

    Contreras-Ramos, A; Sánchez-Gómez, C; García-Romero, H L; Cimarosti, L O

    2008-10-01

    The structures that participate in normal ventricular septation, and to what extent they do so, are questions not yet clarified. Even less is known about how much each of the embryonic structures contributes to the topography of the mature interventricular septum (IVS). The aim of the present paper is to investigate the significance of ventricular trabeculations in the normal development of the muscular region (the middle and apical thirds) of the IVS and to determine the direction in which it grows during cardiac septation. Anatomical studies and in vivo labelling were carried out in chicken embryo hearts at stage 18HH, tracing the labels up to stage 36HH. We analysed the results by measuring the distance between the labelled structures at the beginning and end of the experiments. We demonstrate that the muscular region of the septum originates by the fusion of the ventricular trabeculations with evidence that during cardiac development, the IVS as well as the ventricular cavities grow in opposite direction to the atria.

  9. Myocardial fibrosis burden predicts left ventricular ejection fraction and is associated with age and steroid treatment duration in duchenne muscular dystrophy.

    Science.gov (United States)

    Tandon, Animesh; Villa, Chet R; Hor, Kan N; Jefferies, John L; Gao, Zhiqian; Towbin, Jeffrey A; Wong, Brenda L; Mazur, Wojciech; Fleck, Robert J; Sticka, Joshua J; Benson, D Woodrow; Taylor, Michael D

    2015-03-26

    Patients with Duchenne muscular dystrophy exhibit progressive cardiac and skeletal muscle dysfunction. Based on prior data, cardiac dysfunction in Duchenne muscular dystrophy patients may be influenced by myocardial fibrosis and steroid therapy. We examined the longitudinal relationship of myocardial fibrosis and ventricular dysfunction using cardiac magnetic resonance in a large Duchenne muscular dystrophy cohort. We reviewed 465 serial cardiac magnetic resonance studies (98 Duchenne muscular dystrophy patients with ≥4 cardiac magnetic resonance studies) for left ventricular ejection fraction (LVEF) and presence of late gadolinium enhancement (LGE), a marker for myocardial fibrosis. LVEF was modeled by examining LGE status, myocardial fibrosis burden (as assessed by the number of LGE-positive left ventricular segments), patient age, and steroid treatment duration. An age-only model demonstrated that LVEF declined 0.58 ± 0.10% per year. In patients with both LGE-negative and LGE-positive studies (n=51), LVEF did not decline significantly over time if LGE was absent but declined 2.2 ± 0.31% per year when LGE was present. Univariate modeling showed significant associations between LVEF and steroid treatment duration, presence of LGE, and number of LGE-positive left ventricular segments; multivariate modeling showed that LVEF declined by 0.93 ± 0.09% for each LGE-positive left ventricular segment, whereas age and steroid treatment duration were not significant. The number of LGE-positive left ventricular segments increased with age, and longer steroid treatment duration was associated with lower age-related increases. Progressive myocardial fibrosis, as detected by LGE, was strongly correlated with the LVEF decline in Duchenne muscular dystrophy patients. Longer steroid treatment duration was associated with a lower age-related increase in myocardial fibrosis burden. © 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley

  10. Atrial and ventricular septal defect with pulmonary and tricuspid valvular anomalies in a dog

    International Nuclear Information System (INIS)

    Ishikawa, Y.; Wakao, Y.; Watanabe, T.; Minami, T.; Muto, M.; Suzuki, T.; Takahashi, M.; Une, Y.; Nomura, Y.; Ichioka, N.

    1989-01-01

    A 15-month-old male boxer dog weighing 22 kg was referred to Azabu University Animal Hospital for evaluation of the syncopal attack. There was no cardiac murmur, but electrocardiograms revealed an atrial fibrillation. Thoracic radiograph revealed enlargement of the right and left atrial regions. The medical treatment with digitalis and captopril was made for conversion from the atrial fibrillation to the sinus rhythm. By cardiac catheterization, atrial and ventricular septal defect with pulmonary stenosis was demonstrated. The patient died at 20 months from the first medical examination. At autopsy, there were severe enlargement of both atria, atrial defect, and pulmonary and tricuspid valvular anomalies. (author)

  11. Atrial and ventricular septal defect with pulmonary and tricuspid valvular anomalies in a dog

    Energy Technology Data Exchange (ETDEWEB)

    Ishikawa, Y. [Azabu Univ., Sagamihara, Kanagawa (Japan); Wakao, Y.; Watanabe, T.; Minami, T.; Muto, M.; Suzuki, T.; Takahashi, M.; Une, Y.; Nomura, Y.; Ichioka, N.

    1989-12-15

    A 15-month-old male boxer dog weighing 22 kg was referred to Azabu University Animal Hospital for evaluation of the syncopal attack. There was no cardiac murmur, but electrocardiograms revealed an atrial fibrillation. Thoracic radiograph revealed enlargement of the right and left atrial regions. The medical treatment with digitalis and captopril was made for conversion from the atrial fibrillation to the sinus rhythm. By cardiac catheterization, atrial and ventricular septal defect with pulmonary stenosis was demonstrated. The patient died at 20 months from the first medical examination. At autopsy, there were severe enlargement of both atria, atrial defect, and pulmonary and tricuspid valvular anomalies. (author)

  12. Ventricular septal rupture, right ventricular free wall rupture, hemopericardium, cardiac tamponade, cardiogenic shock, and death in a patient with acute ST elevation myocardial infarction during transthoracic echocardiography

    Directory of Open Access Journals (Sweden)

    Osama A El Kady

    2017-01-01

    Full Text Available The incidence of mechanical complications related to myocardial infarction has decreased due to various factors over the last few decades. Patients admitted for acute ST segment elevation myocardial infarction (STEMI may respond well to thrombolytic therapy before being taken up for coronary angiography and percutaneous coronary intervention depending on the facilities available at the specific center. Unfortunately, some patients develop complications of myocardial infarction during hospital stay or postdischarge. We present a patient admitted with acute STEMI responding well to thrombolytic therapy. During transthoracic echocardiography of the patient in Intensive Care Unit, the patient developed ventricular septal rupture, right ventricular free wall rupture, hemopericardium, cardiac tamponade, and cardiogenic shock and expired.

  13. An Infectious Pseudoaneurysm Caused by Ventricular Septal Defect Occluder in Patent Ductus Arteriosus Closure in a Two-Year-Old Child.

    Science.gov (United States)

    Li, Dingyang; Qiu, Qiu; Jin, Jing; Zhang, Changdong; Wang, Lijun; Zhang, Gangcheng

    2017-12-12

    We present a case of an infectious pseudoaneurysm after patent ductus arteriosus (PDA) closure with a ventricular septal defect (VSD) occluder in a two-year-old child. The aneurysm grew rapidly but was successfully removed in time and the patient survived. To our knowledge, this is the first report of an infectious pseudoaneurysm caused by VSD occluder in PDA closure.

  14. Hybrid management of a large atrial septal defect and a patent ductus arteriosus in an infant with chronic lung disease

    Directory of Open Access Journals (Sweden)

    Pedra Simone

    2010-01-01

    Full Text Available We report a case wherein a dysmorphic four-month-old infant (weighing 4.5 kgs with an 8 mm atrial septal defect (ASD, a 1.5 mm patent ductus arteriosus (PDA, a 2 mm mid-muscular ventricular septal defect (VSD associated with chronic lung disease, and severe pulmonary hypertension, was successfully managed using a hybrid approach, without the use of cardiopulmonary bypass (CPB. Through a median sternotomy, the PDA was ligated and the ASD was closed with a 9 mm Amplatzer septal occluder implanted through peratrial access. The VSD was left untouched. Serial echocardiograms showed complete closure of the ASD and PDA, with progressive normalization of the pulmonary artery (PA pressures within three months. The child rapidly gained weight and was weaned from sildenafil and oxygen administration. After 12 months, the VSD closed spontaneously and the child remained well, with normal PA pressures. A hybrid approach without the use of CPB should be considered in the management of infants with congenital heart disease, associated with chronic lung disease and pulmonary hypertension.

  15. Hybrid management of a large atrial septal defect and a patent ductus arteriosus in an infant with chronic lung disease

    International Nuclear Information System (INIS)

    Pedra, Simone F; Jatene, Marcelo; Pedra, Carlos AC

    2010-01-01

    We report a case wherein a dysmorphic four-month-old infant (weighing 4.5 kgs) with an 8 mm atrial septal defect (ASD), a 1.5 mm patent ductus arteriosus (PDA), a 2 mm mid-muscular ventricular septal defect (VSD) associated with chronic lung disease, and severe pulmonary hypertension, was successfully managed using a hybrid approach, without the use of cardiopulmonary bypass (CPB). Through a median sternotomy, the PDA was ligated and the ASD was closed with a 9 mm Amplatzer septal occluder implanted through peratrial access. The VSD was left untouched. Serial echocardiograms showed complete closure of the ASD and PDA, with progressive normalization of the pulmonary artery (PA) pressures within three months. The child rapidly gained weight and was weaned from sildenafil and oxygen administration. After 12 months, the VSD closed spontaneously and the child remained well, with normal PA pressures. A hybrid approach without the use of CPB should be considered in the management of infants with congenital heart disease, associated with chronic lung disease and pulmonary hypertension

  16. Hybrid management of a large atrial septal defect and a patent ductus arteriosus in an infant with chronic lung disease

    Energy Technology Data Exchange (ETDEWEB)

    Pedra, Simone F; Jatene, Marcelo; Pedra, Carlos AC, E-mail: carlosacpedra@hotmail.com [Hospital do Coração da Associação Sanatório Sírio, São Paulo (Brazil)

    2010-01-01

    We report a case wherein a dysmorphic four-month-old infant (weighing 4.5 kgs) with an 8 mm atrial septal defect (ASD), a 1.5 mm patent ductus arteriosus (PDA), a 2 mm mid-muscular ventricular septal defect (VSD) associated with chronic lung disease, and severe pulmonary hypertension, was successfully managed using a hybrid approach, without the use of cardiopulmonary bypass (CPB). Through a median sternotomy, the PDA was ligated and the ASD was closed with a 9 mm Amplatzer septal occluder implanted through peratrial access. The VSD was left untouched. Serial echocardiograms showed complete closure of the ASD and PDA, with progressive normalization of the pulmonary artery (PA) pressures within three months. The child rapidly gained weight and was weaned from sildenafil and oxygen administration. After 12 months, the VSD closed spontaneously and the child remained well, with normal PA pressures. A hybrid approach without the use of CPB should be considered in the management of infants with congenital heart disease, associated with chronic lung disease and pulmonary hypertension.

  17. Pre-operative evaluation with MR in tetralogy of Fallot and pulmonary atresia with ventricular septal defect

    International Nuclear Information System (INIS)

    Holmqvist, C.; Hochbergs, P.; Bjoerkhem, G.; Brockstedt, S.; Laurin, S.

    2000-01-01

    To assess whether MR imaging could replace angiography in pre-operative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule. Fourteen patients with tetralogy of Fallot (n=10) or pulmonary atresia with VSD (n=4), mean age 7.5±4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction. There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supra valvular stenosis, but the agreement was somewhat lower for the sub valvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the sub valvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts. Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD

  18. Pre-operative evaluation with MR in tetralogy of Fallot and pulmonary atresia with ventricular septal defect

    Energy Technology Data Exchange (ETDEWEB)

    Holmqvist, C.; Hochbergs, P. [Univ. Hospital, Lund (Sweden). Dept of Diagnostic Radiology; Bjoerkhem, G. [Univ. Hospital, Lund (Sweden). Dept of Paediatrics; Brockstedt, S.; Laurin, S. [Univ. Hospital, Lund (Sweden). Dept of Diagnostic Radiology

    2000-01-01

    To assess whether MR imaging could replace angiography in pre-operative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule. Fourteen patients with tetralogy of Fallot (n=10) or pulmonary atresia with VSD (n=4), mean age 7.5{+-}4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction. There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supra valvular stenosis, but the agreement was somewhat lower for the sub valvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the sub valvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts. Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD.

  19. Infective Endocarditis Complicated by Septic Pulmonary Emboli in a Case of a Ventricular Septal Defect

    Directory of Open Access Journals (Sweden)

    Roodpeyma

    2015-11-01

    Full Text Available Introduction Infective endocarditis (IE causes serious complications in patients. Congenital heart disease (CHD is an important underlying condition in children. Septic pulmonary embolism is an uncommon syndrome, and pulmonary valve IE is rare. The current study presented a case of right-sided IE with pulmonary valve involvement and its complications as pulmonary septic emboli in a child with CHD. Case Presentation A 6-year-old girl with a ventricular septal defect (VSD was presented. Echocardiography revealed large vegetation in the right ventricular outflow tract near the pulmonary valve. The patient showed clinical symptoms of lung involvement, and radiologic investigation was compatible with a diagnosis of septic pulmonary emboli. She had good response to antibacterial therapy and underwent a successful surgical closure of the heart defect. Conclusions Children with CHD are at risk of severe complications with the involvement of other organs. long-term febrile illness should be taken seriously in these children. They need hospitalization and careful evaluation.

  20. Ruptura do septo interventricular após trauma torácico fechado Ventricular septal rupture following blunt chest trauma

    Directory of Open Access Journals (Sweden)

    Ronaldo Peixoto de Mello

    2006-12-01

    Full Text Available Relatamos a seguir um caso de ruptura do septo interventricular após trauma torácico automobilístico com evolução relativamente benigna a despeito da extensão da lesão anatômica.We report a case of ventricular septal rupture following chest trauma resulting from a car accident. The outcome was relatively favorable despite the extension of the anatomic lesion.

  1. The effect of residual ventricular septal defects on early clinical outcome: initial experience

    International Nuclear Information System (INIS)

    Zaman, H.; Cheema, M.A.; Jalal, A.

    2000-01-01

    Residual ventricular septal defect (VSD)after repair of isolated VSD or Fallot's tetralogy is one of the main causes of morbidity and re-operations. In this paper we have presented the results of out initial experience regarding the management of this problem. The data consists of 71 patients (22 isolated VSD and 29 fallout's tetralogy) operate during January 1991 to July, 1993. The incidence of residual VSD as shown by color doppler study at the time of discharge from hospital and at three month's follow-up was 3 out of 60 patients(73%) and 12 out of to (20%) respectively. Six out of these 12 patients were NYHA class III-IV who underwent cardiac catheterization which revealed patch dehiscence in two patients and residual right ventricular outflow tract obstruction in four patients. The two patients, with patch dehiscence were reported successfully with very good outcome. Simple color doppler mapping tends to over-diagnose residual VSDs since it can pick up small haemodynamically insignificant leaks around stitches and needle holes which seal off in due course. The findings of color doppler should, therefore, be correlated with actual clinical outcome in order to proceed with further investigation and re-operation. (author)

  2. Surgical repair of tricuspid valve leaflet tear following percutaneous closure of perimembranous ventricular septal defect using Amplatzer duct occluder I: Report of two cases

    Directory of Open Access Journals (Sweden)

    Saatchi Mahesh Kuwelker

    2017-01-01

    Full Text Available Tricuspid valve (TV injury following transcatheter closure of perimembranous ventricular septal defect (PMVSD with Amplatzer ductal occluder I (ADO I, requiring surgical repair, is rare. We report two cases of TV tear involving the anterior and septal leaflets following PMVSD closure using ADO I. In both the patients, the subvalvular apparatus remained unaffected. The patients presented with severe tricuspid regurgitation (TR 6 weeks and 3 months following the device closure. They underwent surgical repair with patch augmentation of the TV leaflets. Postoperatively, both are asymptomatic with a mild residual TR.

  3. Oxidized CaMKII (Ca2+/Calmodulin-Dependent Protein Kinase II) Is Essential for Ventricular Arrhythmia in a Mouse Model of Duchenne Muscular Dystrophy.

    Science.gov (United States)

    Wang, Qiongling; Quick, Ann P; Cao, Shuyi; Reynolds, Julia; Chiang, David Y; Beavers, David; Li, Na; Wang, Guoliang; Rodney, George G; Anderson, Mark E; Wehrens, Xander H T

    2018-04-01

    Duchenne muscular dystrophy patients are prone to ventricular arrhythmias, which may be caused by abnormal calcium (Ca 2+ ) homeostasis and elevated reactive oxygen species. CaMKII (Ca 2+ /calmodulin-dependent protein kinase II) is vital for normal Ca 2+ homeostasis, but excessive CaMKII activity contributes to abnormal Ca 2+ homeostasis and arrhythmias in cardiomyocytes. Reactive oxygen species induce CaMKII to become autonomously active. We hypothesized that genetic inhibition of CaMKII oxidation (ox-CaMKII) in a mouse model of Duchenne muscular dystrophy can alleviate abnormal Ca 2+ homeostasis, thus, preventing ventricular arrhythmia. The objective of this study was to test if selective loss of ox-CaMKII affects ventricular arrhythmias in the mdx mouse model of Duchenne muscular dystrophy. 5-(6)-Chloromethyl-2,7-dichlorodihydrofluorescein diacetate staining revealed increased reactive oxygen species production in ventricular myocytes isolated from mdx mice, which coincides with elevated ventricular ox-CaMKII demonstrated by Western blotting. Genetic inhibition of ox-CaMKII by knockin replacement of the regulatory domain methionines with valines (MM-VV [CaMKII M281/282V]) prevented ventricular tachycardia in mdx mice. Confocal calcium imaging of ventricular myocytes isolated from mdx :MM-VV mice revealed normalization of intracellular Ca 2+ release events compared with cardiomyocytes from mdx mice. Abnormal action potentials assessed by optical mapping in mdx mice were also alleviated by genetic inhibition of ox-CaMKII. Knockout of the NADPH oxidase regulatory subunit p47 phox normalized elevated ox-CaMKII, repaired intracellular Ca 2+ homeostasis, and rescued inducible ventricular arrhythmias in mdx mice. Inhibition of reactive oxygen species or ox-CaMKII protects against proarrhythmic intracellular Ca 2+ handling and prevents ventricular arrhythmia in a mouse model of Duchenne muscular dystrophy. © 2018 American Heart Association, Inc.

  4. Transcatheter coil occluder for closure of ventricular septal defect (a report of 4 cases)

    International Nuclear Information System (INIS)

    Gao Wei; Zhou Aiqing; Yu Zhiqing; Li Fen; Zhong Yumin; Zhang Yuqi; Huang Meirong; Sun Kun

    2005-01-01

    Objective: To explore the indication, methodology and complication of transcatheter coil closure of ventricular septal defect (VSD) in children. Methods: Transcatheter closure of perimembranous VSD with coils was performed in 4 cases from 2003 to 2005. The Duct-Occlude (pfm) and detachable coil (Cook) were chosen for embolization depending on the results of the left ventricular angiogram. The coil size was generally about 1-4 mm larger than the diameter of VSD. Follow up was carried out with echocardiography, ultrasound and clinical examination. Results: The defect diameters of the four cases were 2.0 mm, 2.7 mm, 2.5 mm and 1.5 mm respectively. The Duct-Occlude were successfully implanted in 3 cases of perimembranous VSD with the same type coil (OD[mm]7-3-6, windings 5-3-4) for each. One detachable coil (Cook) (5 x 5) was implanted in the remaining case. All cases had trivial residual shunt immediately after implantation which disappeared 24 hours later. Follow-up for 2 months to one year showed no coil displacement and secondary bacterial arteritis. No tricuspid and aortic regurgitation, no emboli, no endocarditis, and no arrhythmia were found. Conclusions: Coil closure of some small VSD with membranate part pseudo-ventricular aneurysm has good efficacy with the advantages of simple operation, less metal content and mini-invasion also applicable for infants. (authors)

  5. Guillain - Barre syndrome in a patient with acute myocardial infarction with ventricular septal defect repair treated with plasma exchange

    Directory of Open Access Journals (Sweden)

    Maitrey D Gajjar

    2015-01-01

    Full Text Available Guillain - Barre syndrome (GBS is an acute, frequently severe progressive illness of peripheral nervous system that is autoimmune in nature. GBS after myocardial infarction (MI with ventricular septal defect (VSD is uncommon with high mortality rate if not treated promptly. [1] We report a successful outcome of GBS post MI with VSD in a 60-year-old male patient who was on a ventilator treated successfully with therapeutic plasma exchange.

  6. Prenatal Isolated Ventricular Septal Defect May Not Be Associated with Trisomy 21

    Directory of Open Access Journals (Sweden)

    Ori Shen

    2014-04-01

    Full Text Available The aim of this study was to examine if isolated fetal ventricular septal defect (VSD is associated with trisomy 21. One hundred twenty six cases with prenatal VSD diagnosed by a pediatric cardiologist were reviewed. Cases with known risk factors for congenital heart disease, the presence of other major anomalies, soft signs for trisomy 21 or a positive screen test for trisomy 21 were excluded. Ninety two cases formed the study group. None of the cases in the study group had trisomy 21. The upper limit of prevalence for trisomy 21 in isolated VSD is 3%. When prenatal VSD is not associated with other major anomalies, soft markers for trisomy 21 or a positive nuchal translucency or biochemical screen, a decision whether to perform genetic amniocentesis should be individualized. The currently unknown association between isolated VSD and microdeletions and microduplications should be considered when discussing this option.

  7. Left ventricular function in right ventricular overload

    International Nuclear Information System (INIS)

    Iwanaga, Shiro; Handa, Shunnosuke; Abe, Sumihisa; Onishi, Shohei; Nakamura, Yoshiro; Kunieda, Etsuo; Ogawa, Koichi; Kubo, Atsushi

    1989-01-01

    This study clarified regional and global functions of the distorted left ventricle due to right ventricular overload by gated radionuclide ventriculography (RNV). Cardiac catheterization and RNV were performed in 13 cases of atrial septal defect (ASD), 13 of pure mitral stenosis (MS), 10 of primary pulmonary hypertension (PPH), and 10 of normal subjects (NL). Right ventricular systolic pressure (RVSP) was 32.9±13.9, 45.0±12.2, 88.3±17.1, and 21.2±4.5 mmHg, respectively. The end-systolic LAO view of the left ventricle was halved into septal and free-wall sides. The end-diastolic halves were determined in the same plane. Ejection fractions of the global left ventricle (LVEF), global right ventricle (RVEF), the septal half of the left ventricle (SEPEF), and the free-wall half of the left ventricle (FWEF) were obtained. LVEF was 56.8±9.8% in NL, 52.8±10.5% in ASD, and 49.5±12.9% in PPH. In MS, LVEF (47.0±13.0%) was smaller than those in the other groups. RVEF was 37.0±5.2% in NL, 43.7±15.5% in ASD, and 32.8±11.5% in MS. In PPH, RVEF (25.0±10.6%) was smaller than those in the other groups. SEPEF was smaller in ASD (42.5±13.2%), MS (40.4±13.1%), PPH (40.5±12.5%) than in NL (53.5±8.5%). Systolic function of the septal half of the left ventricle was disturbed by right ventricular overload. RVEF (r=-0.35, p<0.05) and SEPEF (r=-0.51, p<0.01) had negative correlations with RVSP. As RVSP rose, systolic function of the septal half of the left ventricle was more severely disturbed. FWEF was the same among the four groups; NL (57.0±12.6%), ASD (48.6±15.2%), MS (50.5±12.0%), and PPH (51.1±12.3%). There was a good correlation between SEPEF and LVEF in NL (r=0.81), although in PPH this correlation was poor (r=0.64). These data showed that the distorted left ventricular due to right ventricular overload maintains its global function with preserved function of the free-wall side. (J.P.N.)

  8. Exploring energy loss by vector flow mapping in children with ventricular septal defect: Pathophysiologic significance.

    Science.gov (United States)

    Honda, Takashi; Itatani, Keiichi; Takanashi, Manabu; Kitagawa, Atsushi; Ando, Hisashi; Kimura, Sumito; Oka, Norihiko; Miyaji, Kagami; Ishii, Masahiro

    2017-10-01

    Vector flow mapping is a novel echocardiographic flow visualization method, and it has enabled us to quantitatively evaluate the energy loss in the left ventricle (intraventricular energy loss). Although intraventricular energy loss is assumed to be a part of left ventricular workload itself, it is unclear what this parameter actually represents. The aim of the present study was to elucidate the characteristics of intraventricular energy loss. We enrolled 26 consecutive children with ventricular septal defect (VSD). On echocardiography vector flow mapping, intraventricular energy loss was measured in the apical 3-chamber view. We measured peak energy loss and averaged energy loss in the diastolic and systolic phases, and subsequently compared these parameters with catheterization parameters and serum brain natrium peptide (BNP) level. Diastolic, peak, and systolic energy loss were strongly and positively correlated with right ventricular systolic pressure (r=0.76, 0.68, and 0.56, p<0.0001, = 0.0001, and 0.0029, respectively) and right ventricular end diastolic pressure (r=0.55, 0.49, and 0.49, p=0.0038, 0.0120, and 0.0111, respectively). In addition, diastolic, peak, and systolic energy loss were significantly correlated with BNP (r=0.75, 0.69 and 0.49, p<0.0001, < 0.0001, and=0.0116, respectively). In children with VSD, elevated right ventricular pressure is one of the factors that increase energy loss in the left ventricle. The results of the present study encourage further studies in other study populations to elucidate the characteristics of intraventricular energy loss for its possible clinical application. Copyright © 2017 Elsevier B.V. All rights reserved.

  9. The Cardiac MR Images and Causes of Paradoxical Septal Motion

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dong Hun [Soonchunhyang University Bucheon Hospital, Bucheon (Korea, Republic of); Choi, Sang Il; Chun, Eun Ju [Seoul National University Bundang Hospital, Seongnam (Korea, Republic of); Choi, Sung Hun [Ulsan University Hospital, Ulsan (Korea, Republic of); Park, Jae Hyung [Seoul National University Hospital, Seoul (Korea, Republic of)

    2010-10-15

    Real-time cine MRI studies using the steady-state free precession (SSFP) technique are very useful for evaluating cardiac and septal motion. During diastole, the septum acts as a compliant membrane between the two ventricles, and its position and geometry respond to even small alterations in the trans-septal pressure gradients. Abnormal septal motion can be caused by an overload of the right ventricle, delayed ventricular filling and abnormal conduction. In this study, we illustrate, based on our experiences, the causes of abnormal septal motion such as corrective surgery for tetralogy of Fallot, an atrial septal defect, pulmonary thromboembolism, mitral stenosis, constrictive pericarditis and left bundle branch block. In addition, we discuss the significance of paradoxical septal motion in the context of cardiac MR imaging.

  10. The Cardiac MR Images and Causes of Paradoxical Septal Motion

    International Nuclear Information System (INIS)

    Kim, Dong Hun; Choi, Sang Il; Chun, Eun Ju; Choi, Sung Hun; Park, Jae Hyung

    2010-01-01

    Real-time cine MRI studies using the steady-state free precession (SSFP) technique are very useful for evaluating cardiac and septal motion. During diastole, the septum acts as a compliant membrane between the two ventricles, and its position and geometry respond to even small alterations in the trans-septal pressure gradients. Abnormal septal motion can be caused by an overload of the right ventricle, delayed ventricular filling and abnormal conduction. In this study, we illustrate, based on our experiences, the causes of abnormal septal motion such as corrective surgery for tetralogy of Fallot, an atrial septal defect, pulmonary thromboembolism, mitral stenosis, constrictive pericarditis and left bundle branch block. In addition, we discuss the significance of paradoxical septal motion in the context of cardiac MR imaging

  11. Intra-cardiac echocardiography in alcohol septal ablation

    DEFF Research Database (Denmark)

    Cooper, Robert M; Shahzad, Adeel; Newton, James

    2015-01-01

    Alcohol septal ablation (ASA) in hypertrophic obstructive cardiomyopathy reduces left ventricular outflow tract gradients. A third of patients do not respond; inaccurate localisation of the iatrogenic infarct can be responsible. Transthoracic echocardiography (TTE) using myocardial contrast can...

  12. Transcatheter closure of post-operative residual ventricular septal defect using a patent ductus arteriosus closure device in an adult: a case report.

    Science.gov (United States)

    Djer, Mulyadi M; Idris, Nikmah S; Alwi, Idrus; Wijaya, Ika P

    2014-07-01

    Transcatheter closure of perimembranous and muscular ventricular septal defect (VSD) has been performed widely and it has more advantages compare to surgery. However, transcatheter closure of residual VSD post operation of complex congenital heart disease is still challenging because of the complexity of anatomy and concern about device stability, so the operator should meticulously choose the most appropriate technique and device. We would like to report a case of transcatheter closure of residual VSD post Rastelli operation in a patient with double outlet right ventricle (DORV), sub-aortic VSD, severe infundibulum pulmonary stenosis (PS) and single coronary artery. The patient had undergone operations for four times, but he still had intractable heart failure that did not response to medications. On the first attempt. we closed the VSD using a VSD occluder, unfortunately the device embolized into the descending aorta, but fortunately we was able to snare it out. Then we decided to close the VSD using a patent ductus arteriosus (PDA occluder). On transesophageal echocardiography (TEE) and angiography evaluation, the device position was stable. Post transcatheter VSD closure, the patient clinical condition improved significantly and he could finally be discharged after a long post-surgery hospitalization. Based on this experience we concluded that the transcatheter closure of residual VSD in complex CHD using PDA occluder could be an effective alternative treatment.

  13. Percutaneous closure of postoperative ventricular septal residual left-to-right shunt with the China made device

    International Nuclear Information System (INIS)

    Qin Yongwen; Zhao Xianxian; Wu Hong; Ding Jijun; Cao Jiang; Zheng Xin

    2007-01-01

    Objective: To evaluate the feasibility and efficacy of percutaneous transcatheter closure of residual ventricular septal defect (VSD)after surgical closure using China made-nitinol VSD device. Methods: Transcatheter closure was attempted in 11 patients (5 males, 6 females)with a residual VSD following surgical closure. The mean residual VSD narrowest diameter was(5.82 ± 2.09) mm (range from 3 to 9 mm)by echocardiography. A 6 F-9 F delivery sheath was advanced across the residual VSD over a guidewire from femoral vein to deploy the occluder under guidance of left ventriculography and transthoracic echocardiography. Results: The left ventriculography showed membranous part aneurism-like residual VSD in 8 patients and funnel type in 3 cases. There were multiple outlet in 5 cases and one outlets in 6 cases, with mean residual VSD narrowest diameter of (6.09 ± 1.58) mm (range from 3 to 9 mm)measured by left ventriculography. The diameter of occluder was (9.18 ± 2.79) mm (range from 8 to 12 mm). Complete closure of the defect was obtained in 10 cases, and another small residual shunt still remained in one case who had four outlets been treated by 2 occluders. No aortic valvular regurgitation occured in all patients except 1 patient presented complete atrioventricular block within 3 days after the procedure and recovered 2 weeks later with intravenous steroids therapy; and no other complications occured. The fluoroscopy time was(16.91 ± 4.23) min (range from 8 to 30 min). During follow-up from 1 m - 4 y, only 1 case showed residual shunt, and the other had no episodes of endocarditis, thromboembolism, hemolysis, infectious endocarditis, displacement of the occluder and aortic valvular regurgitation. Conclusion: Transcatheter closure of postoperative ventricular septal residual left-to-right shunt with China made-nitinol occluder is safe and effective. (authors)

  14. Effects of exercise training on pulmonary vessel muscularization and right ventricular function in an animal model of COPD.

    Science.gov (United States)

    Hassel, Erlend; Berre, Anne Marie; Skjulsvik, Anne Jarstein; Steinshamn, Sigurd

    2014-09-28

    Right ventricular dysfunction in COPD is common, even in the absence of pulmonary hypertension. The aim of the present study was to examine the effects of high intensity interval training (HIIT) on right ventricular (RV) function, as well as pulmonary blood vessel remodeling in a mouse model of COPD. 42 female A/JOlaHsd mice were randomized to exposure to either cigarette smoke or air for 6 hours/day, 5 days/week for 14 weeks. Mice from both groups were further randomized to sedentariness or HIIT for 4 weeks. Cardiac function was evaluated by echocardiography and muscularization of pulmonary vessel walls by immunohistochemistry. Smoke exposure induced RV systolic dysfunction demonstrated by reduced tricuspid annular plane systolic excursion. HIIT in smoke-exposed mice reversed RV dysfunction. There were no significant effects on the left ventricle of neither smoke exposure nor HIIT. Muscularization of the pulmonary vessels was reduced after exercise intervention, but no significant effects on muscularization were observed from smoke exposure. RV function was reduced in mice exposed to cigarette smoke. No Increase in pulmonary vessel muscularization was observed in these mice, implying that other mechanisms caused the RV dysfunction. HIIT attenuated the RV dysfunction in the smoke exposed mice. Reduced muscularization of the pulmonary vessels due to HIIT suggests that exercise training not only affects the heart muscle, but also has important effects on the pulmonary vasculature.

  15. Right ventricular function in patients with ischemic heart disease

    International Nuclear Information System (INIS)

    Araki, Haruo; Hisano, Ryuichi; Nagata, Yoshiyuki; Caglar, N.; Nakamura, Motoomi

    1985-01-01

    Thirty-five patients with ischemic heart disease (IHD) and 10 normal subjects were studied. Right and left ventricular ejecction fractions (EF) were determined using equilibrium radionuclide ventriculography with technetium-99m. Furthermore, abnormal motion of the right ventricular septal wall was obtained by cardiac cathetelization, and its relation to the right ventricular EF was examined. In IHD patients with anterior myocardial infarction, left ventricular EF decreased, but right ventricular EF was normal. This suggested that left ventricular dysfunction does not always have an effect on right ventricular function. Right ventricular EF was normal even when akinesis or dyskinesis was present in the ventricular septul, suggesting that abnormal motion of the ventricular septal wall has no significantly stimulant effect on right ventricular function. A decreased right ventricular EF was likely to occur only when the right ventricular free wall became ischemic or necrotic simultaneously with occurrence of posterior myocardial infarction. (Namekawa, K.)

  16. Successful two-stage correction of ventricular septal defect and patent ductus arteriosus in a patient with fixed pulmonary hypertension.

    Science.gov (United States)

    Pac, Aysenur; Polat, Tugcin Bora; Vural, Kerem; Pac, Mustafa

    2010-01-01

    We report a case of a 6-year-old boy with fixed severe pulmonary artery hypertension secondary to a ventricular septal defect (VSD) together with a patent ductus arteriosus (PDA). As a preliminary step, PDA embolization was performed following therapy with inhaled prostacyclin over a period of 6 months. Further, the patient underwent successful surgical VSD closure. We postulate that a staged procedure with long-term prostaglandin therapy might be capable of reducing pulmonary artery resistance and permitting total correction in a patient once considered to have inoperable pulmonary arteriopathy.

  17. Evaluation of atrial, ventricular and atrioventricular septal defects by cine magnetic resonance imaging

    International Nuclear Information System (INIS)

    Akagi, Teiji; Kato, Hirohisa; Kiyomatsu, Yumi; Saiki, Kuninobu; Suzuki, Kazushige; Eto, Takaharu

    1992-01-01

    Cine magnetic resonance imaging (MRI) was performed on 20 patients (mean age: 5.3±4.4 years) with atrial, ventricular, or atrioventricular septal defects for evaluation of cardiac structure and blood flow. Prior to cine MRI, electrocardiographycally gated MRI using multislice scquisition was performed on all patients to localize optimal slice location. Cine-MRI was obtained with a 30 deg flip angle, 15 msec echo time, and 30 msec pulse repetition time, on a 256 x 256 or 128 x 128 acquisition matrix. Abnormalities of cardiac structure were well defined in all patients by gated cardiac imaging. In 18 of the 20 patients, cine-MRI was able to detect shunt flow, visualized as a low intensity signal in comparison with the surrounding blood flow. Cine-MRI can provide not only accurate anatomy of cardiac structures but functional assessment of the cardiac chamber, wall topology and flow relations. Cine-MRI will become an important noninvasive technique for assessment of anatomy and physiology in congenital heart disease. (author)

  18. Application of muscular ventricular septal defect occluders in the therapy of uderweight infants and young children with patent dutus arterriosus%应用肌部室间隔缺损封闭器封堵低体质量婴幼儿动脉导管未闭

    Institute of Scientific and Technical Information of China (English)

    戴辰程; 郭保静; 金梅

    2011-01-01

    Objective: To evaluate the safety and advantage of application of muscular ventricular septal defect in the therapy of uderweight infants and young children with patent dutus arterriosus. Methods: Nine uderweight infants and young children (1 male and 8 fomale) with patent dutus arterriosus, aged from 6 to 21 months and weighted from 5. 5 to 10kg,underwent transcathter intervention with muscular VSD occluders from October2010 to June 2011. All patients accept transthoracic echocardiography,cardiac catheterization and angiocardiography. Results: 8 patients were associated with pulmonary hypertension. There are 2,5,and 1 patients were diganosed as mild,middle and severe pulmonary hypertension respectively. QP/QS ranged from 1.94 to 2. 87 and total pulmonary resistance index ranged from 1. 82 to 4. 7 Wood. M2. Angiocardiography indicated there were 4 large PDAs, 1 PDA with large infundibulum,and 4 PDAs with short and small infundibulum,one of which was right aortic arch and left PDA. Nine PDAs were successfully closed with muscular ventricular septal defect occluders. Pulmonary tension decreased to normal or nearly normal. No residual shunt, occluders falling off,vascular injury,or iatrogenic stenosis of pulmonary artery and descending aorta were found. 3 of 4 patients with mitral regurgitation relieved. Conclusion: Muscular VSD occluder is available and effective to close large PDA,PDA with short and small infundibulum or PDA which can not be successfully closed with Amplatzer PDA occluders in uderweight infants and young children.%目的:探讨肌部室间隔缺损(VSD)封闭器在低体质量婴幼儿动脉导管未闭(PDA)介入治疗中临床应用的安全性及优点.方法:2010年10月至2011年6月,应用肌部VSD封闭器对9例低体质量婴幼儿PDA进行介入治疗,男性1例,女性8例,年龄6-21个月,体质量5.5 ~10 kg.本组病例均行经胸超声心动图、心导管检查及造影检查.结果:合并肺动脉高压者8

  19. Coexisting ventricular septal defect affects the features of ruptured sinus of Valsalva aneurysms

    Directory of Open Access Journals (Sweden)

    Yan Jin

    2017-03-01

    Full Text Available Objectives: To determine the correlation exists between ventricular septal defect (VSD and ruptured sinus of Valsalva aneurysm (RSVA. Methods: Between September 2003 and April 2014, 80 RSVA patients underwent surgical repair. These patients were retrospectively divided into two groups: the VSD group (38 cases and the non-VSD group (42 cases. Results: Rupture points of SVA originated more frequently in the right coronary sinus (RCS of patients in the VSD group than those in the non-VSD group (p=0.002. In the VSD group, more than 92.1% tended to rupture into the right ventricular outflow tract. The rupture points are diverse in the non-VSD group. A significant difference was found in rupture points of RSVA between the two groups (p<0.001. Patients in the VSD group presented with aortic valve disease more often than those in the non-VSD group (p<0.001. A total of 67 patients were repaired with a patch at the opening of RSVA; of those, all patients in VSD group and 29 patients in non-VSD group were repaired with a patch. Nine patients in non-VSD group received transcatheter closure of RSVA. Conclusion: The presence or absence of VSD affects the rupture points of SVA, aortic valve disease involved, and therapeutic schedule. Ruptured sinus of Valsalva aneurysm type should be clinically modified on the basis of presence or absence of VSD.

  20. Transcatheter occlusion of perimembranous ventricular septal defects with Amplatzer duct occluder

    International Nuclear Information System (INIS)

    Ren Sengen; Kang Kang; Wu Danning; Shi Hong; Wu Jingzhang; Yang Mei; Han Feizhou; Zhou Fei; Zhu Zhijun

    2003-01-01

    Objective: To study the feasibility of congenital perimembranous ventricular septal defect (VSD) occlusion by Amplatzer duct occluder (ADO) and to establish guidelines for its safe and effective application. Methods: From May 2001 to December 2002, percutaneous transcatheter occlusion of congenital perimembranous VSD was performed in 41 patients, in which 18 patients had an associated aneurysm of the membranous septum. There were 20 male and 21 female subjects, with age ranging from 6 to 38 years (median 14.9) and weights of 18 to 62 kg (median 41.5). The diameter of VSD ranged from 4 to 12.8 mm (median 6.0), and the distance of VSD to the aortic valve were 4.5 to 12 mm (median 6.6). The ratio of pulmonary to systemic blood flow (Qp/Qs) was 1.4 to 2.6 (median 1.7) and the pulmonary systolic pressure was 19 to 34 mmHg (median 25). Results: All the patients underwent successfully complete occlusion of VSD with ADO, and the size of ADO ranged from 6/4 to 16/14 mm. After the procedure, left ventricular angiography and transthoracic echocardiography showing the ADOs were precisely placed inside the VSDs with no residual shunt and also no abnormalities revealed by auscultation. Chest X-ray showed the relief of congestion of the lungs. Electrocardiogram demonstrated complete left bundle branch block in one case and incomplete right bundle branch block in other 12 cases, all disappeared within 1 to 2 weeks. Over a period of 2 to 21 months follow-up, all the devices maintained in good position and the patients' condition turned much better. No patient developed aortic or tricuspid regurgitation and other complications. Conclusions: ADO is a safe, effective and convenient occlusion device which could be practically applied for the congenital perimembranous VSDs

  1. Idiopathic dilated cardiomyopathy: computerized anatomic study of relashionship between septal and free left ventricle wall.

    Science.gov (United States)

    Juliani, Paulo Sérgio; Costa, Eder França da; Correia, Aristides Tadeu; Monteiro, Rosangela; Jatene, Fabio Biscegli

    2014-01-01

    A feature of dilated cardiomyopathy is the deformation of ventricular cavity, which contributes to systolic dysfunction. Few studies have evaluated this deformation bearing in mind ventricular regions and segments of the ventricle, which could reveal important details of the remodeling process, supporting a better understanding of its role in functional impairment and the development of new therapeutic strategies. To evaluate if, in basal, equatorial and apical regions, increased internal transverse perimeter of left ventricle in idiopathic dilated cardiomyopathy occurs proportionally between the septal and non-septal segment. We performed an anatomical study with 28 adult hearts from human cadavers. One group consisted of 18 hearts with idiopathic dilated cardiomyopathy and another group with 10 normal hearts. After lamination and left ventricle digital image capture, in three different regions (base, equator and apex), the transversal internal perimeter of left ventricle was divided into two segments: septal and not septal. These segments were measured by proper software. It was established an index of proportionality between these segments, called septal and non-septal segment index. Then we determined whether this index was the same in both groups. Among patients with normal hearts and idiopathic dilated cardiomyopathy, the index of proportionality between the two segments (septal and non-septal) showed no significant difference in the three regions analyzed. The comparison results of the indices NSS/SS among normal and enlarged hearts were respectively: in base 1.99 versus 1.86 (P=0.46), in equator 2.22 versus 2.18 (P=0.79) and in apex 2.96 versus 3.56 (P=0.11). In the idiopathic dilated cardiomyopathy, the transversal dilatation of left ventricular internal perimeter occurs proportionally between the segments corresponding to the septum and free wall at the basal, equatorial and apical regions of this chamber.

  2. Surface ECG and Fluoroscopy are Not Predictive of Right Ventricular Septal Lead Position Compared to Cardiac CT.

    Science.gov (United States)

    Rowe, Matthew K; Moore, Peter; Pratap, Jit; Coucher, John; Gould, Paul A; Kaye, Gerald C

    2017-05-01

    Controversy exists regarding the optimal lead position for chronic right ventricular (RV) pacing. Placing a lead at the RV septum relies upon fluoroscopy assisted by a surface 12-lead electrocardiogram (ECG). We compared the postimplant lead position determined by ECG-gated multidetector contrast-enhanced computed tomography (MDCT) with the position derived from the surface 12-lead ECG. Eighteen patients with permanent RV leads were prospectively enrolled. Leads were placed in the RV septum (RVS) in 10 and the RV apex (RVA) in eight using fluoroscopy with anteroposterior and left anterior oblique 30° views. All patients underwent MDCT imaging and paced ECG analysis. ECG criteria were: QRS duration; QRS axis; positive or negative net QRS amplitude in leads I, aVL, V1, and V6; presence of notching in the inferior leads; and transition point in precordial leads at or after V4. Of the 10 leads implanted in the RVS, computed tomography (CT) imaging revealed seven to be at the anterior RV wall, two at the anteroseptal junction, and one in the true septum. For the eight RVA leads, four were anterior, two septal, and two anteroseptal. All leads implanted in the RVS met at least one ECG criteria (median 3, range 1-6). However, no criteria were specific for septal position as judged by MDCT. Mean QRS duration was 160 ± 24 ms in the RVS group compared with 168 ± 14 ms for RVA pacing (P = 0.38). We conclude that the surface ECG is not sufficiently accurate to determine RV septal lead tip position compared to cardiac CT. © 2017 Wiley Periodicals, Inc.

  3. Comparison of left ventricular function assessment between echocardiography and MRI in Duchenne muscular dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Buddhe, Sujatha; Lewin, Mark; Olson, Aaron; Soriano, Brian D. [University of Washington School of Medicine and Seattle Children' s Hospital, Division of Cardiology, Department of Pediatrics, Seattle, WA (United States); Ferguson, Mark [University of Washington School of Medicine and Seattle Children' s Hospital, Department of Radiology, Seattle, WA (United States)

    2016-09-15

    Cardiomyopathy in Duchenne muscular dystrophy (DMD) is associated with death in approximately 40% of patients. Echocardiography is routinely used to assess left ventricular (LV) function; however, it has limitations in these patients. We compared echocardiographic measures of cardiac function assessment to cardiac MRI. We included children and young adults with DMD who had MRI performed between January 2010 and July 2015. We measured echocardiographic and MRI parameters of function assessment, including strain. Presence of late gadolinium enhancement (LGE) was assessed by MRI. Subjects were divided into two groups based on MRI left ventricular ejection fraction (LVEF): group I, LVEF ≥55% and group II, LVEF <55%. We included 41 studies in 33 subjects, with 25 in group I and 16 in group II. Mean age of subjects was 13.6 ± 2.8 years and mean duration between echocardiogram and MRI was 7.6 ± 4.1 months. Only 8 of 16 (50%) patients in group II had diminished function on echocardiogram. Echocardiographic images were suboptimal in 16 subjects (39%). Overall, echocardiographic parameters had weak correlation with MRI-derived ejection fraction percentage. MRI-derived myocardial strain assessment has better correlation with MRI ejection fraction as compared to echocardiography-derived strain parameters. Echocardiography-based ventricular functional assessment has weak correlation with MRI parameters in children and young adults with Duchenne muscular dystrophy. While this correlation improves in the subset of subjects with adequate echocardiographic image quality, it remains modest and potentially suboptimal for clinical management. Accordingly, we conclude that MRI should be performed routinely and early in children with DMD, not only for LGE imaging but also for functional assessment. (orig.)

  4. Comparison of left ventricular function assessment between echocardiography and MRI in Duchenne muscular dystrophy

    International Nuclear Information System (INIS)

    Buddhe, Sujatha; Lewin, Mark; Olson, Aaron; Soriano, Brian D.; Ferguson, Mark

    2016-01-01

    Cardiomyopathy in Duchenne muscular dystrophy (DMD) is associated with death in approximately 40% of patients. Echocardiography is routinely used to assess left ventricular (LV) function; however, it has limitations in these patients. We compared echocardiographic measures of cardiac function assessment to cardiac MRI. We included children and young adults with DMD who had MRI performed between January 2010 and July 2015. We measured echocardiographic and MRI parameters of function assessment, including strain. Presence of late gadolinium enhancement (LGE) was assessed by MRI. Subjects were divided into two groups based on MRI left ventricular ejection fraction (LVEF): group I, LVEF ≥55% and group II, LVEF <55%. We included 41 studies in 33 subjects, with 25 in group I and 16 in group II. Mean age of subjects was 13.6 ± 2.8 years and mean duration between echocardiogram and MRI was 7.6 ± 4.1 months. Only 8 of 16 (50%) patients in group II had diminished function on echocardiogram. Echocardiographic images were suboptimal in 16 subjects (39%). Overall, echocardiographic parameters had weak correlation with MRI-derived ejection fraction percentage. MRI-derived myocardial strain assessment has better correlation with MRI ejection fraction as compared to echocardiography-derived strain parameters. Echocardiography-based ventricular functional assessment has weak correlation with MRI parameters in children and young adults with Duchenne muscular dystrophy. While this correlation improves in the subset of subjects with adequate echocardiographic image quality, it remains modest and potentially suboptimal for clinical management. Accordingly, we conclude that MRI should be performed routinely and early in children with DMD, not only for LGE imaging but also for functional assessment. (orig.)

  5. Large right ventricular sinusoids in an infant with aorta-left ventricular tunnel and proximal right coronary artery atresia.

    Science.gov (United States)

    Chen, Peter C; Spinner, Joseph A; Heinle, Jeffrey S

    2018-07-01

    We report a 1-month-old infant diagnosed with an aorta-left ventricular tunnel, ventricular septal defect, and right coronary atresia with right ventricular sinusoids. The patient's anatomy and physiology did not indicate right-ventricular-dependent coronary circulation, and therefore right ventricular decompression could be performed without compromising coronary perfusion during surgical correction. A detailed understanding of the coronary anatomy is critical in managing this defect when coronary anomalies are present.

  6. Clinical studies on myocardial perfusion imaging in patients with right ventricular overload

    International Nuclear Information System (INIS)

    Abo, Kenji; Yamagata, Takashi; Nakajima, Masao; Fujita, Kimiaki; Morita, Nobuo

    1979-01-01

    Patients with heart disease which had been clinically diagnosed underwent 201 Tl myocardial perfusion imaging. The thickness of right ventricular wall measured from original images was directly proportional to systolic pressure of the right ventricle measured by cardiac catheterization, and 201 Tl activity in the right ventricle was more directly proportional to systolic pressure of the right ventricle. Imaging patterns of various diseases were also described. Images of patients with hypertrophic cardiomyopathy revealed that right ventricular wall was thin and right ventricular cavity was small, but the thickness of septal wall and left ventricular wall were maximal. Images of patients with mitral insufficiency revealed that the thickness of right ventricular wall, septal wall, and left ventricular wall was medium, and the right ventricular cavity was smaller than the left ventricular cavity. Images of patients with congestive cardiomyopathy and congestive cardiac failure showed that enlargement of both ventricular cavities was disproportionate to the thickness of each wall. Images of patients with arterial septal defect revealed that the thickness of each wall was comparatively normal, the right ventricular cavity was maximal, and the left ventricular cavity was minimal. Images of patients with primary pulmonary hypertention, pulmonary stenosis and tetralogy of Fallot in whom pressure overload was recognized revealed severe thickenings of right ventricular wall, moderate enlargement of the right ventricle, small left ventricle, and thin left ventricular wall. (Tsunoda, M.)

  7. Postinfarction Ventricular Septal Rupture – A Rare Complication Remains Challenge for Cardiac Surgical Team

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    Vilém Rohn

    2013-01-01

    Full Text Available The incidence of post infarction ventricular septal rupture (PIVSR is decreasing in the last years due to aggressive treatment of myocardial infarction with early percutaneous coronary interventions. As a consequence patients with PIVSR are referred to surgery more often with significant heart failure. The aim of this retrospective study was to assess the influence of these on the operative results and to identify the risk factors of operative mortality. A retrospective analysis of prospectively collected data of patients with the PIVSR admitted to our center from November 2004 to February 2012 was performed. Variables were analyzed using two-dimensional correspondence analysis. There were 25 patients (12 males and 13 females with mean age 70.2 years (47–82 operated on; 17 (68% presented with anterior and 8 (32% with posterior PIVSR. Eighteen patients (72% had acute heart failure, 13 (52% presented with cardiogenic shock. Before surgery, intraaortic balloon pump (IABP had 20 (80% patients; in 4 (16% a ventricular assist device was used, either Extracorporeal Membrane Oxygenation (ECMO or centrifugal pumps as biventricular assist. Operative mortality was 40% (10 pts.. Four patients (12% had small non-significant recurrent shunt on postoperative echocardiography. Although majority of patients with PIVSR have significant heart failure prior to surgery the operative mortality remains comparable to older studies. Predictors of perioperative death were concomitant surgical reconstruction of the left ventricle, renal impairment before operation, male gender, history of coronary artery disease, PIVSR location posterior, and shock at surgery.

  8. Unbalanced atrioventricular septal defect: definition and decision making.

    Science.gov (United States)

    Overman, David M; Baffa, Jeanne M; Cohen, Meryl S; Mertens, Luc; Gremmels, David B; Jegatheeswaram, Anusha; McCrindle, Brian W; Blackstone, Eugene H; Morell, Victor O; Caldarone, Christopher; Williams, William G; Pizarro, Christian

    2010-04-01

    Unbalanced atrioventricular septal defect is an uncommon lesion with widely varying anatomic manifestations. When unbalance is severe, diagnosis and treatment is straightforward, directed toward single-ventricle palliation. Milder forms, however, pose a challenge to current diagnostic and therapeutic approaches. The transition from anatomies that are capable of sustaining biventricular physiology to those that cannot is obscure, resulting in uneven application of surgical strategy and excess mortality. Imprecise assessments of ventricular competence have dominated clinical decision making in this regard. Malalignment of the atrioventricular junction and its attendant derangement of inflow physiology is a critical factor in determining the feasibility of biventricular repair in the setting of unbalanced atrioventricular septal defect. The atrioventricular valve index accurately identifies unbalanced atrioventricular septal defect and also brings into focus a zone of transition from anatomies that can support a biventricular end state and those that cannot.

  9. Pulmonary valve endocarditis associated to a septal interventricular defect and infundibular and pulmonary valve Stenosis

    International Nuclear Information System (INIS)

    Echeverri, Juan G; Diaz, Alejandro; Jaramillo, Nicolas; Gonzalez, Sergio

    2004-01-01

    Ventricular septal defects generate 10% of all adult congenital cardiopathies. 4% to 8% of patients to whom the defect has not been corrected are in risk of developing endocarditis. Pulmonary valve endocarditis is a rare event (1.5% to 2% of all endocarditis cases) and its mean etiology is intravenous drug abuse. The most frequently isolated microorganism in these cases is staphylococcus aurous. We report a case of pulmonary valve endocarditis associated with ventricular septal defect and valvular and infundibular pulmonary stenosis caused by streptococcus sp. in a patient without past medical history of drug abuse, alcoholism or previous invasive procedures

  10. Left Ventricular Myocardial Function in Children With Pulmonary Hypertension: Relation to Right Ventricular Performance and Hemodynamics.

    Science.gov (United States)

    Burkett, Dale A; Slorach, Cameron; Patel, Sonali S; Redington, Andrew N; Ivy, D Dunbar; Mertens, Luc; Younoszai, Adel K; Friedberg, Mark K

    2015-08-01

    Through ventricular interdependence, pulmonary hypertension (PH) induces left ventricular (LV) dysfunction. We hypothesized that LV strain/strain rate, surrogate measures of myocardial contractility, are reduced in pediatric PH and relate to invasive hemodynamics, right ventricular strain, and functional measures of PH. At 2 institutions, echocardiography was prospectively performed in 54 pediatric PH patients during cardiac catheterization, and in 54 matched controls. Patients with PH had reduced LV global longitudinal strain (LS; -18.8 [-17.3 to -20.4]% versus -20.2 [-19.0 to -20.9]%; P=0.0046) predominantly because of reduced basal (-12.9 [-10.8 to -16.3]% versus -17.9 [-14.5 to -20.7]%; Pright ventricular free-wall LS (r=0.64; PBrain natriuretic peptide levels correlated moderately with septal LS (r=0.48; P=0.0038). PH functional class correlated moderately with LV free-wall LS (r=-0.48; P=0.0051). The septum, shared between ventricles and affected by septal shift, was the most affected LV region in PH. Pediatric PH patients demonstrate reduced LV strain/strain rate, predominantly within the septum, with relationships to invasive hemodynamics, right ventricular strain, and functional PH measures. © 2015 American Heart Association, Inc.

  11. Crista Supraventricularis Purkinje Network and Its Relation to Intraseptal Purkinje Network.

    Science.gov (United States)

    De Almeida, Marcos C; Araujo, Mayssa; Duque, Mathias; Vilhena, Virginia

    2017-10-01

    Using transparent specimens with a dual color injection, microscopy, and computer tomography, this report shows that the right and left ventricular subendocardial Purkinje networks are connected by an extensive septal network in the bovine heart. The septal network is present along the entire septum except at a free zone below ventricular valves. Being the only communication of the basal right septum with the right free wall, the supraventricular crest is an enigmatic but not, by any means, hidden muscular structure. It is one of the last structures to be activated in human heart. It is shown here that the supraventricular crest Purkinje network connects the anterosuperior right ventricular basal free wall Purkinje network to anterior right ventricular basal septal Purkinje network. It is suggested that the stimulus initiated at middle left ventricular endocardium will activate the supraventricular crest. The intraseptal connection found between the basal left ventricular subendocardial septal Purkinje network and the right ventricular basal septal Purkinje network is, probably, the pathway for the stimulus. An anatomic basis is provided to explain why the inflow tract contracts earlier than the outflow tract in the right ventricle systole. Anat Rec, 2017. © 2017 Wiley Periodicals, Inc. Anat Rec, 300:1793-1801, 2017. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  12. Congenital ventricular septal defects and prenatal exposure to cyclooxygenase inhibitors

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    F. Burdan

    2006-07-01

    Full Text Available Ventricular septal defects (VSDs are common congenital abnormalities which have been reported to be associated with maternal fever and various environmental factors. The aim of the present study was to evaluate the effect of prenatal exposure to cyclooxygenase (COX inhibitors on heart defects. A retrospective statistical analysis was performed using data collected in our laboratory during various teratological studies carried out on albino CRL:(WIWUBR Wistar strain rats from 1997 to 2004. The observations were compared with concurrent and historic control data, as well as findings from other developmental toxicological studies with selective and nonselective COX-2 inhibitors. Despite the lack of significant differences in the frequency of VSDs between drug-exposed and control groups, statistical analysis by the two-sided Mantel-Haenszel test and historical control data showed a higher incidence of heart defects in offspring exposed to nonselective COX inhibitors (30.06/10,000. Unlike other specific inhibitors, aspirin (46.26/10,000 and ibuprofen (106.95/10,000 significantly increased the incidence of the VSD when compared with various control groups (5.38-19.72/10,000. No significant differences in length or weight were detected between fetuses exposed to COX inhibitors and born with VSD and non-malformed offsprings. However, a statistically significant increase of fetal body length and decrease of body mass index were found in fetuses exposed to COX inhibitors when compared with untreated control. We conclude that prenatal exposure to COX inhibitors, especially aspirin and ibuprofen, increased the incidence of VSDs in rat offspring but was not related to fetal growth retardation.

  13. Pulmonary edema following transcatheter closure of atrial septal defect

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    Keerthi Chigurupati

    2015-01-01

    Full Text Available We describe an incident of development of acute pulmonary edema after the device closure of a secundum atrial septal defect in a 52-year-old lady, which was treated with inotropes, diuretics and artificial ventilation. Possibility of acute left ventricular dysfunction should be considered after the defect closure in the middle-aged patients as the left ventricular compliance may be reduced due to increased elastic stiffness and diastolic dysfunction. Baseline left atrial pressure may be > 10 mmHg in these patients. Associated risk factors for the left ventricular dysfunction are a large Qp:Qs ratio, systemic hypertension, severe pulmonary hypertension and paroxysmal atrial fibrillation.

  14. Ventricular Dyssynchrony and Function Improve following Catheter Ablation of Nonseptal Accessory Pathways in Children

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    Sylvia Abadir

    2013-01-01

    Full Text Available Introduction. Paradoxical or hypokinetic interventricular septal motion has been described in patients with septal or paraseptal accessory pathways. Data regarding nonseptal pathways is limited. Methods and Results. We quantified left ventricular dyssynchrony and function in 16 consecutive children, 14.2±3.7 years, weighing 53 ± 17 kg, prior to and following catheter ablation of bidirectional septal (N=6 and nonseptal (N=10 accessory pathways. Following ablation, the left ventricular ejection fraction increased by 4.9±2.1% (P=0.038 from a baseline value of 57.0%±7.8%. By tissue Doppler imaging, the interval between QRS onset and peak systolic velocity (Ts decreased from a median of 33.0 ms to 18.0 ms (P=0.013. The left ventricular ejection fraction increased to a greater extent following catheter ablation of nonseptal (5.9%±2.6%, P=0.023 versus septal (2.5%±4.1%, P=0.461 pathways. The four patients with an ejection fraction 50% after ablation. Similarly, the improvement in dyssynchrony was more marked in patients with nonseptal versus septal pathways (difference between septal and lateral wall motion delay before and after ablation 20.6±7.1 ms (P=0.015 versus 1.4±11.4 ms (P=0.655. Conclusion. Left ventricular systolic function and dyssynchrony improve after ablation of antegrade-conducting accessory pathways in children, with more pronounced changes noted for nonseptal pathways.

  15. Relationship of left ventricular, elastic and muscular arteries remodeling in patients with uncontrolled arterial hypertension

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    S. Ya. Dotsenko

    2013-04-01

    Full Text Available Introduction. Uncontrolled hypertension is observed in 65-92% of hypertensive patients. It plays an important role in the development of adverse cardiovascular events and survival, which depend on subclinical target organ damage. There are reports on the relationship between ineffective hypertension control and left ventricular (LV hypertrophy or large arteries stiffness. However, the nature of the remodeling in uncontrolled hypertension remains poorly understood. Objective: to study the character and relationship of left ventricular and arterial remodeling depending on effectiveness of hypertension control. Design and method. We performed a study of 363 hypertensive patients (160 men and 203 women aged 50,8 ± 1,2 years without comorbidities, which were divided into 3 groups according to the effectiveness of blood pressure (BP control: 160 patients with controlled hypertension, 142 patients with uncontrolled hypertension and 61 patients with resistant hypertension. Uncontrolled BP based on measured systolic BP≥140 mmHg and diastolic BP≥90 mmHg. Remodeling indexes of left ventricular, elastic (common carotid and muscular (brachial artery were evaluated by the ultrasonic method. The severity and character of diastolic dysfunction, hypertrophy, types of remodeling and stiffness were assessed. Statistical processing of the results was performed using Student's t criterion and Pearson correlation analysis. Results and discussion. According to the results of the study, uncontrolled hypertension affected the development of subclinical cardiovascular lesions negatively. Thus, LV hypertrophy was detected more frequently in the third group (91,8% in resistant hypertension versus 46,8% in controlled hypertension, p<0,05. Differences in LV geometry with increasing of concentric remodeling types were also observed more frequently in the third group, where concentric remodeling and concentric hypertrophy types were founded in 14,8% and 59

  16. Repair of tetralogy of Fallot associated with atrioventricular septal defect.

    Science.gov (United States)

    Tláskal, T; Hucín, B; Kostelka, M; Chaloupecký, V; Marek, J; Tax, P; Janouàek, J; Kuèera, V; Hruda, J; Reich, O; Skovránek, J

    1998-01-01

    Tetralogy of Fallot, when associated with atrioventricular septal defect permitting shunting at ventricular level, represents a complex cyanotic congenital malformation. Experience with surgical repair is limited, and results vary considerably. Between 1984 and 1996, we repaired 14 consecutive patients with this combination seen in our center. Their ages ranged from 8 months to 21 years (median 7.4 years). Six (42.9%) had Down's syndrome. In eight patients the correct diagnosis was made using echocardiography alone. In the remaining six patients, who had previously-constructed arterial shunts and/or suspected pulmonary arterial stenosis, catheterization and angiocardiography were also performed. The repair consisted of double patch closure of the septal defect, reconstruction of two atrioventricular orifices, and relief of pulmonary stenosis at all levels. In five patients with a hypoplastic pulmonary trunk, a monocusp transannular patch (four patients) or an allograft (one patient) was used for restoration of continuity from the right ventricle to the pulmonary arteries. Patch enlargement of one or both pulmonary arteries was necessary in five patients. One patient (7.1%) died early, and another late. The twelve surviving (85.8%) patients have been followed for 1.2-12.5 years after surgery (median 4.9 years, mean 5.9+/-3.9 years). During the follow-up, reoperation was necessary for repair of residual ventricular septal defect and pulmonary regurgitation in two patients, and closure of an atrial septal defect and alteration to left atrioventricular valvar regurgitation in one patient. Seven patients are in class I of the New York Heart Association, four in class II, and one in class III. Tetralogy of Fallot associated with atrioventricular septal defect can be corrected with low mortality and good long-term results. Residual lesions, however, have a tendency to progress, especially when seen in combination. After surgery, all patients need long-term close follow-up.

  17. Traumatic ventricular septal defect in a 4-year-old boy after blunt chest injury

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    Yun Mi Kim

    2011-02-01

    Full Text Available Traumatic ventricular septal defect (VSD resulting from blunt chest injury is a very rare event. The mechanisms of traumatic VSD have been of little concern to dateuntil now, but two dominant theories have been described. In one, the rupture occurs due to acute compression of the heart; in the other, it is due to myocardial infarction of the septum. The clinical symptoms and timing of presentation are variable, so appropriate diagnosis can be difficult or delayed. Closure of traumatic VSD has been based on a combination of heart failure symptoms, hemodynamics, and defect size. Here, we present a case of a 4-year-old boy who presented with a traumatic VSD following a car accident. He showed normal cardiac structure at the time of injury, but after 8 days, his repeated echocardiography revealed a VSD. He was successfully treated by surgical closure of the VSD, and has been doing well up to the present. This report suggests that the clinician should pay great close attention to the patients injured by blunt chest trauma, keeping in mind the possibility of cardiac injury.

  18. Estenose subaórtica associada a comunicação interventricular perimembranosa: acompanhamento clínico de 36 pacientes Subaortic stenosis associated with perimembranous ventricular septal defect: clinical follow-up of 36 patients

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    Maria da Gloria Cruvinel Horta

    2005-02-01

    Full Text Available OBJETIVO: Estudar o comportamento clínico da estenose subaórtica associada a comunicação interventricular perimembranosa. MÉTODOS: Foram acompanhadas, de janeiro 1979 a junho 2000, quanto às características anatômicas, caráter evolutivo e eventos clínicos, 36 crianças com comunicação interventricular perimembranosa e estenose subaórtica fixa. RESULTADOS: A idade de diagnóstico da estenose subaórtica fixa variou de seis meses a 170 meses, sendo abaixo de 1 ano apenas em duas crianças. Quanto ao sexo a distribuição foi de 2:1 com grande predomínio do masculino. A comunicação interventricular era de tamanho pequeno em 61,00% dos casos, médio em 30,56% e grande em 8,40%, apresentando diminuição do tamanho da comunicação durante o acompanhamento em 30,56% (11 casos. Em todos os pacientes a estenose subaórtica era fixa, em membrana. Durante o tempo de acompanhamento, 23 pacientes apresentaram progressão da estenose. Foi realizado tratamento cirúrgico em 21 casos, sendo um paciente reoperado por reestenose. Endocardite bacteriana ocorreu em dois casos, um deles faleceu. CONCLUSÃO: A estenose subaórtica ocorre na história natural da comunicação interventricular geralmente após o 1º ano de vida, apresentando caráter progressivo e necessitando de cirurgia na maioria dos casos.OBJECTIVE: To study the clinical pattern of subaortic stenosis associated with perimembranous ventricular septal defect. METHODS: From January 1979 to June 2000, 36 children with perimembranous ventricular septal defect and fixed subaortic stenosis were followed-up regarding anatomic characteristics, evolvement, and clinical events. RESULTS: Age at diagnosis of subaortic stenosis ranged from 6 months to 170 months, and it was less than 1 year in only 2 children. Regarding sex, the distribution was 2:1 with a greater predominance of males. Ventricular septal defect was small in 61.0% of cases, medium in 30.56%, and large in 8.40%; the size of the

  19. Right ventricular pacemaker lead position is associated with differences in long-term outcomes and complications.

    Science.gov (United States)

    Witt, Chance M; Lenz, Charles J; Shih, Henry H; Ebrille, Elisa; Rosenbaum, Andrew N; van Zyl, Martin; Aung, Htin; Manocha, Kevin K; Deshmukh, Abhishek J; Hodge, David O; Mulpuru, Siva K; Cha, Yong-Mei; Espinosa, Raul E; Asirvatham, Samuel J; Mcleod, Christopher J

    2017-08-01

    Cardiac pacing from the right ventricular apex is associated with detrimental long-term effects and nonapical pacing locations may be associated with improved outcomes. There is little data regarding complications with nonapical lead positions. The aim of this study was to assess long-term outcomes and lead-related complications associated with differing ventricular lead tip position. All adult patients who underwent dual-chamber pacemaker implantation from 2004 to 2014 were included if they had postprocedure chest radiographs amenable to lead position determination. Long-term outcomes and lead-related complication rates were recorded. These were compared at 5 years between: (1) apical and septal leads, (2) apical and nonseptal nonapical (NSNA), and (3) apical and septal with >40% ventricular pacing. We retrospectively evaluated 3,450 patients, which included 238 with a septal position and 733 with NSNA lead positions. Septal lead position was associated with a lower mortality compared to apical leads (24% vs. 31%, P = 0.02). In patients with greater than 40% pacing, septal leads were associated with significantly higher rates of incident atrial fibrillation compared to apical leads (49% vs. 34%, P = 0.04). NSNA positions were associated with a significantly higher rate of lead dislodgement (4% vs. 2%, P = 0.005) and need for revision (8% vs. 5%, P = 0.005). Septal pacemaker lead position is associated with a lower mortality compared to apically placed leads, but a higher incidence of atrial fibrillation with higher percentage ventricular pacing. NSNA lead locations are associated with more complications and should be avoided. © 2017 Wiley Periodicals, Inc.

  20. Transcatheter device closure of perimembranous ventricular septal defect in children treated with prophylactic oral steroids: acute and mid-term results of a single-centre, prospective, observational study.

    Science.gov (United States)

    Thakkar, Bhavesh; Patel, Nehal; Bohora, Shomu; Bhalodiya, Dharmin; Singh, Tarandeep; Madan, Tarun; Shah, Saurin; Poptani, Vishal; Shukla, Anand

    2016-04-01

    Background and Objective Although transcatheter closure of perimembranous ventricular septal defect is emerging as an accepted, viable alternative, conduction disturbances still remain a major concern. Although steroid treatment has shown encouraging results with complete recovery, efficacy of prophylactic use of steroids is still speculative. We aim to study the mid-term outcome of perimembranous ventricular septal defect closure in children who received prophylactic oral steroids. Materials and methods A prospective study was designed and antegrade device closure was attempted in eligible children who met the following inclusion criteria: age 3-18 years and weight >10 kg, defect diameter ⩽12 mm, and symptomatic, haemodynamic changes or history of infective endocarditis. Prophylactic steroid protocol consisted of 2 weeks oral prednisolone (1 mg/kg/day) initiated immediately after the procedure, and in the event of bradyarrhythmia it was escalated to 2 mg/kg. Patients were regularly followed-up at 1, 6, and 12 months and then annually. Patients with post-procedure heart block underwent Holter monitoring after a minimum of 1 year interval. Between May, 2007 and August, 2012, successful device closure was accomplished in 290/297 patients. Mean age and weight were 9±3.12 years and 21±8.27 kg, respectively. The defect measured 5±1.38 mm on echocardiography. Mean fluoroscopy time was 12.98±8.64 minutes. Eight patients with major complications included one each with device embolisation, haemolysis, severe aortic regurgitation, and five with bradyarrhythmias, including complete atrioventricular block in three, Mobitz II in one, and bifascicular block in one. Patients with complete atrioventricular block responded to high-dose steroid and temporary pacemaker. Minor complications included post-procedure heart block (n=22) and blood loss (n=2). At 18.23±13.15 months follow-up, 8/27 (five major, 22 minor) with arrhythmia had persistent post-procedure heart block of no

  1. Pulmonary artery-to-left atrial fistula discovered after the closure of atrial septal defect: A rare clinical scenario

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    Akshay Chauhan

    2018-01-01

    Full Text Available A case of the right pulmonary artery-to- left atrial fistula with atrial septal defect (ASD is presented. The fistula was detected after the patient developed desaturation following surgical closure of the ASD. It was managed with a transcatheter (trans-RPA route closure of the fistula using a 12-mm Amplatzer ventricular septal defect closure device.

  2. Systolic ventricular filling.

    Science.gov (United States)

    Torrent-Guasp, Francisco; Kocica, Mladen J; Corno, Antonio; Komeda, Masashi; Cox, James; Flotats, A; Ballester-Rodes, Manel; Carreras-Costa, Francesc

    2004-03-01

    The evidence of the ventricular myocardial band (VMB) has revealed unavoidable coherence and mutual coupling of form and function in the ventricular myocardium, making it possible to understand the principles governing electrical, mechanical and energetical events within the human heart. From the earliest Erasistratus' observations, principal mechanisms responsible for the ventricular filling have still remained obscured. Contemporary experimental and clinical investigations unequivocally support the attitude that only powerful suction force, developed by the normal ventricles, would be able to produce an efficient filling of the ventricular cavities. The true origin and the precise time frame for generating such force are still controversial. Elastic recoil and muscular contraction were the most commonly mentioned, but yet, still not clearly explained mechanisms involved in the ventricular suction. Classical concepts about timing of successive mechanical events during the cardiac cycle, also do not offer understandable insight into the mechanism of the ventricular filling. The net result is the current state of insufficient knowledge of systolic and particularly diastolic function of normal and diseased heart. Here we summarize experimental evidence and theoretical backgrounds, which could be useful in understanding the phenomenon of the ventricular filling. Anatomy of the VMB, and recent proofs for its segmental electrical and mechanical activation, undoubtedly indicates that ventricular filling is the consequence of an active muscular contraction. Contraction of the ascendent segment of the VMB, with simultaneous shortening and rectifying of its fibers, produces the paradoxical increase of the ventricular volume and lengthening of its long axis. Specific spatial arrangement of the ascendent segment fibers, their interaction with adjacent descendent segment fibers, elastic elements and intra-cavitary blood volume (hemoskeleton), explain the physical principles

  3. Managing Ventricular Septal Defect with Associated Aortic Regurgitation: Two Decades of Experience.

    Science.gov (United States)

    Sanoussi, Ahmed; Demanet, Helene; Dessy, Hughes; Massin, Martial; Biarent, Dominique; Deville, Andree; Wauthy, Pierre

    2015-09-01

    Ventricular septal defect (VSD) with aortic regurgitation (AR) is a well-known association. However, there is still no agreement about its management, particularly regarding the technical details of its operative treatment. The study aim was to describe all components of the syndrome and to evaluate the various techniques used with regards to its anatomical and functional features. A total of 31 patients (mean age 7.4 years; range: 1.0-14.3 years) who underwent repair of VSD and AR between 1990 and 2013 was reviewed. The VSD was perimembranous in 22 patients, and subarterial in nine. Trusler's valvuloplasty technique was used in 15 patients, Yacoub's technique in seven, and Carpentier's technique (triangular resection) in four. Two patients underwent aortic valve replacement (AVR), and three patients with no significant aortic valve lesions underwent a simple patch repair of the VSD. The aortic valvuloplasty results were generally good, with an initial aortic valvuloplasty avoiding AVR. During the immediate postoperative period, valvuloplasty failure occurred in three patients, regardless of the technique used, and all three patients were reoperated on. The mean duration of follow up was 8.5 years (range: 3.2-20.6 years). The initial result was maintained in all patients, except for four who underwent late AVR. The study findings contributed to an analysis of VSD and AR, and helped to clarify the best surgical strategy. The results obtained suggest that adequacy of the initial repair is the most important determinant of subsequent evolution.

  4. Comunicação interatrial do tipo seio coronário, comunicação interventricular e ausência de veia cava superior esquerda Coronary sinus atrial septal defect and ventricular septal defect with no left superior vena cava

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    Fábio Alves Almeida

    1998-10-01

    Full Text Available Relatamos um caso raro de um paciente de 21 meses, portador de comunicação interatrial do tipo seio coronário, associada a comunicação interventricular perimembranosa, e ausência de veia cava superior esquerda. O diagnóstico foi realizado através da ecocardiografia e confirmado pela angiografia. O paciente foi operado sem intercorrências, ambos os defeitos foram fechados com patch de pericárdio bovino e o fluxo das veias coronárias ficou direcionado para o átrio esquerdo. Um ecocardiograma mostrou ausência de shunt residual através dos defeitos.We report a rare case of a 21 month old child with a coronary sinus atrial septal defect associated with perimembranous ventricular septal defect and no left superior vena cava. The diagnosis was made by transthoracic echocardiogram and confirmed by angiography. The patient was operated on uneventfully, both defects were closed with bovine pericardial patches and the flow from the coronary veins was directed towards the left atrium. An echocardiogram revealed complete closure of both defects.

  5. Surgical treatment of ventricular septal defect combined with tricuspid valve insufficiency

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    L. Maniuc

    2016-11-01

    Full Text Available The aim – to evaluate different methods of surgical treatment of ventricular septal defect (VSD, combined with failure of the tricuspid valve (TC, and to develop optimal algorithm for the treatment of patients with this pathology. Materials and methods. Between 2010 and 2014, 35 patients, average age 80.9±20.5 months, underwent tricuspidal annuloplasty within correction of VSD in Center of Cardiac Surgery of Republic of Moldova. Tricuspidal regurgitation of the II grade was diagnosed valve in 20 (57.0 % cases, III grade – in 8 (23.0 % cases, IV grade – in 7 (20.0 % of cases. Within correction of VSD plastics of tricuspidal valve was performed: in 4 cases (11.0 % of patients plastics by De Vega, in 14 cases (40.0 % of patients – comissuroplastics, in 6 cases (17.0 % – comissuroplastics and suture of cleft, in 1 case (4.0 % plastics by De Vega with comissuroplastics, in 10 cases (29.0 % – comissuroplastics and suture of cleft. Results. After operation the clinic status improved significantly: breathlessness reduced from 91.7 % to 8.3 % cases, tachycardia reduced from 91.7 % to 33.3 % cases and other cardiac failure symptoms – from 10.8 % to 4.2 % cases. The number of patients with NYHA class I heart failure after surgery was 54.2 % compared to its absence before operation, class 2 diminished from 60.0 % to 41.7 % cases, class 3 – from 36.0 % to 4.2 % cases. Conclusions. Anteroseptal comissuroplastics was used in majority of cases. This method is simple, reliable and inexpensive, requires not more than 5–10 min and significantly reduces tricuspidal valve insufficiency.

  6. Surgical removal of atrial septal defect occlusion device and mitral valve replacement in a 39-year-old female patient with infective endocarditis

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    A S Zotov

    2018-02-01

    Full Text Available Atrial septal defects represent the second most frequent congenital heart disease after ventricular septal defects. Transcatheter closure of an atrial septal defect is usually performed following strict indications on patients with significant left-to-right shunt. Infective endocarditis after transcatheter implantation of atrial septal defect occluder is an extremely rare complication. We report a case of infective endocarditis of the mitral valve (with severe mitral valve insufficiency in a 39-year-old female patient 13 years after transcatheter closure of an atrial septal defect. Complex prophylactic antibiotic coverage was performed prior to surgical intervention. Surgical removal of atrial septal defect occluder, mitral valve replacement, atrial septal defect closure and left atrial appendage resection were performed. Postoperative course was uneventful.

  7. Combined perventricular septal defect closure and patent ductus arteriosus ligation via the lower ministernotomy approach.

    Science.gov (United States)

    Voitov, Alexey; Omelchenko, Alexander; Gorbatykh, Yuriy; Bogachev-Prokophiev, Alexander; Karaskov, Alexander

    2018-02-01

    Over the past decade, minimally invasive approaches have been advocated for surgical correction of congenital defects to reduce costs related to hospitalization and for improved cosmesis. Minimal skin incisions and partial sternotomy reduce surgical trauma, however these techniques might not be successful in treating a number of congenital pathological conditions, particularly for combined congenital defects. We focused on cases with a combined presentation of ventricular septal defect and patent ductus arteriosus. We studied 12 infants who successfully underwent surgical treatment for a combined single-stage ventricular septal defect and patent ductus arteriosus closure through a lower ministernotomy without using cardiopulmonary bypass and X-rays. No intraoperative and early postoperative complications or mortality were noted. Postoperative echocardiography did not reveal residual shunts. The proposed technique is safe and reproducible in infants. © Crown copyright 2017.

  8. Perforation of the right aortic valve cusp: complication of ventricular septal defect closure with a modified Rashkind umbrella.

    Science.gov (United States)

    Vogel, M; Rigby, M L; Shore, D

    1996-01-01

    An 18-month-old boy with a perimembranous ventricular septal defect (VSD) had undergone transcatheter closure of the defect with a modified 17 mm Rashkind umbrella device at age 4 months (weight 3.8 kg). The clinical signs of a VSD persisted, and he developed aortic incompetence, first detected 5 months after the procedure, which progressed from mild to moderate. A three-dimensional echocardiographic study demonstrated that one of the four arms holding the umbrella was protruding into the aortic valve and had perforated the right aortic valve cusp. This diagnosis was confirmed at subsequent surgery. Surgical repair of the perforated right aortic valve leaflet was necessary. The umbrella was adherent to the tricuspid valve and could not be removed. Instead it was left in situ, but three of the stainless steel arms were cut off. When umbrella closure of a perimembranous VSD is undertaken, the close proximity of part of the distal umbrella to the aortic valve can lead to aortic regurgitation.

  9. Evaluation of right ventricular function using gated equilibrium blood pool radionuclide ventriculography in patients with congenital volume and pressure overload late after surgical repair

    International Nuclear Information System (INIS)

    Hirata, Nobuaki; Sakakibara, Tetsuo; Watanabe, Shinichiro; Nomura, Fumikazu; Akamatsu, Hiroki; Matsumura, Yasushi; Yamamoto, Kazuhiro; Sasaki, Jiro; Kodama, Kazuhisa

    1991-01-01

    The effects of congenital right ventricular pressure and volume overload were studied in 3 patients with pulmonary stenosis, 7 with atrial septal defect and 6 with atrial septal defect plus pulmonary stenosis late after successful surgical correction. Gated equilibrium blood pool radionuclide ventriculography was used to measure right ventricular function at rest and during exercise and to compare it with eight normal subjects. Right ventricular ejection fractions at rest and during exercise were measured to be 61±9% and 66±13%, respectively, in the group with pulmonary stenosis, 49±7% and 54±8% in the group with atrial septal defect, and 65±13% and 69±13% in the group with atrial septal defect plus pulmonary stenosis. The values in the groups with pulmonary stenosis and atrial septal defect plus pulmonary stenosis were significantly higher than the control subjects (45±5% and 51±5%, p<0.01). The peak filling rate at rest and during exercise was also significantly higher in the groups with pulmonary stenosis and atrial septal defect plus pulmonary stenosis than in controls (at rest, 2.72±0.72, 2.53±0.94 vs. 1.64±0.24 p<0.05; during exercise, 4.38±1.23, 4.13±1.18 vs. 2.25±0.62, p<0.01). When patients with right ventricular systolic pressure equal to or greater than left ventricular systolic pressure and those with right ventricular systolic pressure less than left ventricular systolic pressure were compared, the right ventricular ejection fraction and peak filling rate were greater with the higher pressure at rest (71±10% and 3.12±0.81% vs. 55±3% and 2.30±0.27, p<0.05) and during exercise (75±11% and 4.86±1.01 vs. 59±3% and 2.61±0.35, p<0.05). Postoperative right ventricular hyperfunction may be due to preoperative pressure, but not volume, overload. (author)

  10. [The complex origin of ventricular tachycardia after the total correction of tetralogy of Fallot].

    Science.gov (United States)

    Ressia, L; Graffigna, A; Salerno-Uriarte, J A; Viganò, M

    1993-09-01

    Two patients underwent surgical treatment of ventricular tachycardia after repair of tetralogy of Fallot. Both patients had right bundle branch block, moderate pulmonary valve incompetence and right ventricular dilatation, and were refractory to electrophysiologically guided drug therapy. Both patients underwent intraoperative epicardial mapping, which located the arrhythmogenic focus on the right ventricular outflow tract, on the border of the previous ventriculotomy. In one patient removal of the previous scar and endocardial cryoablation was successful in ablating the arrhythmia. In the other, the same procedure was only temporarily effective. VT recurred and was subsequently identified at the superior border of the closed ventricular septal defect. It was ablated by means of transcatheter radiofrequency. While VT from foci located on the right ventricular free wall can be easily detected and ablated, septal origin of VT requires extensive preoperative and intraoperative electrophysiological evaluation and may necessitate combined surgical and transcatheter procedures.

  11. Ventricular Septal Defect in an Octogenarian: A Case Report of VSD Surgical Repair Concomitant with Coronary Artery Bypass and Valvular Surgery

    Directory of Open Access Journals (Sweden)

    Eiki Tayama

    2012-01-01

    Full Text Available Finding an untreated or asymptomatic large ventricular septal defect (VSD in an elderly patient is uncommon. The present case was an 81-year-old man who suffered from acute myocardial infarction due to three-vessel coronary disease, mitral and tricuspid valve insufficiency, and high-flow perimembranous VSD (Qp/Qs 2.3. Although the patient was elderly and the VSD had been asymptomatic for a long time, we considered that high-flow VSD and valve diseases should be repaired simultaneously with coronary disease. Then, he underwent elective surgery, namely, VSD patch repair concomitant with coronary artery bypass grafting, and mitral and tricuspid annuloplasty. His postoperative course was uneventful. We conclude that, even for an octogenarian, surgical repair of VSD is recommendable, if surgical indications are appropriate.

  12. Ebstein’s Anomaly, Left Ventricular Noncompaction, and Sudden Cardiac Death

    Directory of Open Access Journals (Sweden)

    Michael McGee

    2015-01-01

    Full Text Available Ebstein’s anomaly is a congenital disorder characterized by apical displacement of the septal leaflet of the tricuspid valve. Ebstein’s anomaly may be seen in association with other cardiac conditions, including patent foramen ovale, atrial septal defect, and left ventricular noncompaction (LVNC. LVNC is characterized by increased trabeculation within the left ventricular apex. Echocardiography is often used to diagnose LVNC; however, magnetic resonance (MR imaging offers superior characterization of the myocardium. We report a case of sudden cardiac death in a patient with Ebstein’s anomaly with unrecognized LVNC noted on post mortem examination with screening documenting the presence of LVNC in one of the patient’s twin sons.

  13. Ventricular septal defect in children and adolescents in Angola: experience of a tertiary center.

    Science.gov (United States)

    Manuel, Valdano; Morais, Humberto; Manuel, Ana; David, Bruna; Gamboa, Sebastiana

    2014-10-01

    This is the first study in Angola with the aim of characterizing ventricular septal defect (VSD) among children and adolescents. A cross-sectional study based on echocardiographic records of the largest pediatric cardiology center in Angola included all children and adolescents (0 to 18 years old) with VSD between April 2010 and March 2011. The diagnosis was made by transthoracic and Doppler echocardiography with a Medison SA 8000 system. The sample was divided into two groups: Group 1, isolated VSD; and Group 2, VSD associated with other congenital heart defects (CHDs). Age, gender, type of VSD, associated CHDs and genetic syndromes were assessed. A total of 490 CHDs were diagnosed, of which 283 were VSDs. In Group 1 (140, 49%) the mean age was 29±36 months. The most frequent age (mode) at diagnosis was 24 months. There was no predominance of gender (ratio 1:1). The majority (127, 91%) had perimembranous VSD. In Group 2 (143, 51%) 113 patients (79%) had one, 27 patients (19%) had two and three patients (2%) had three other CHDs. Trisomy 21 was the most common genetic syndrome (23, 96%). The study shows that VSD is the most common CHD in childhood, the diagnosis is made late and almost half of VSDs are associated with other CHDs. Copyright © 2014 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  14. Interventricular Septal Hematoma and Coronary-Ventricular Fistula: A Complication of Retrograde Chronic Total Occlusion Intervention

    OpenAIRE

    Abdul-rahman R. Abdel-karim; Minh Vo; Michael L. Main; J. Aaron Grantham

    2016-01-01

    Interventricular septal hematoma is a rare complication of retrograde chronic total occlusion (CTO) percutaneous coronary interventions (PCI) with a typically benign course. Here we report two cases of interventricular septal hematoma and coronary-cameral fistula development after right coronary artery (RCA) CTO-PCI using a retrograde approach. Both were complicated by development of ST-segment elevation and chest pain. One case was managed actively and the other conservatively, both with a f...

  15. Unruptured Aneurysm of Sinus of Valsalva Coexisting with the Large Ventricular Septal Defect and Severe Aortic Regurgitation in a Young Man

    Directory of Open Access Journals (Sweden)

    Pouya Nezafati

    2015-01-01

    Full Text Available Introduction. Unruptured sinus of valsalva aneurysm (SVA is a rare congenital anomaly, particularly, when it coexists with a ventricular septal defect (VSD and aortic regurgitation due to the prolapse of the elongated aortic cusp into the VSD. In this report, we present the case of a 19-year-old young man with VSD challenging in spite of dyspnea and lower limb edema. Presentation of Case. Its diagnosis was made on the basis of transthoracic echocardiography results. Surgical management consisted of replacing the SVA with mechanical valve prosthesis. A Gore-Tex patch repaired the VSD. Discussion. In the follow-up periods, clinical and echocardiographic tests showed that the patient was in excellent status. Conclusion. SVA requires a surgical procedure due to its high risk of mortality in unoperated patients and a good safety of surgery.

  16. [Closure of wide patent ductus arteriosus using a fenestrated muscular VSD occluder device in a pediatric patient with Down syndrome and pulmonary hypertension].

    Science.gov (United States)

    Güvenç, Osman; Saygı, Murat; Demir, İbrahim Halil; Ödemiş, Ender

    2017-06-01

    Patients with wide patent ductus arteriosus and significant pulmonary hypertension not treated in time constitute a significant problem for cardiologists. For these patients, tests that could aid in decision-making for further planning include reversibility and balloon occlusion tests performed in the catheterization laboratory. Devices developed for the closure of ductus as well as different devices with off-label use may be employed in patients scheduled for transcatheter occlusion. When result of reversibility test is borderline positive, the use of fenestrated device may be applicable for selected patients. Presently described is case of a 10-year-old patient with Down syndrome who had a wide ductus and systemic pulmonary hypertension. Transcatheter closure procedure was performed with off-label use of a fenestrated muscular ventricular septal defect occluder device.

  17. Interstitial deletion of 1p22.2p31.1 and medium-chain acyl-CoA dehydrogenase deficiency in a patient with global developmental delay

    DEFF Research Database (Denmark)

    Maegawa, Gustavo H B; Poplawski, Nicola K; Andresen, Brage Storstein

    2008-01-01

    We report on a 6-year-old girl who presented at 6 months of age with seizures, delayed psychomotor development and mild facial dysmorphism. A small muscular ventricular septal defect was documented on echocardiogram and brain MRI showed a frontal brain anomaly. Urine organic acid analysis revealed...

  18. Two-dimensional echocardiographic features of right ventricular infarction

    International Nuclear Information System (INIS)

    D'Arcy, B.; Nanda, N.C.

    1982-01-01

    Real-time, two-dimensional echocardiographic studies were performed in 10 patients with acute myocardial infarction who had clinical features suggestive of right ventricular involvement. All patients showed right ventricular wall motion abnormalities. In the four-chamber view, seven patients showed akinesis of the entire right ventricular diaphragmatic wall and three showed akinesis of segments of the diaphragmatic wall. Segmental dyskinetic areas involving the right ventricular free wall were identified in four patients. One patient showed a large right ventricular apical aneurysm. Other echocardiographic features included enlargement of the right ventricle in eight cases, paradoxical ventricular septal motion in seven cases, tricuspid incompetence in eight cases, dilation of the stomach in four cases and localized pericardial effusion in two cases. Right ventricular infarction was confirmed by radionuclide methods in seven patients, at surgery in one patient and at autopsy in two patients

  19. Effect of amiodarone-induced hyperthyroidism on left ventricular outflow obstruction after septal myectomy for hypertrophic cardiomyopathy.

    Science.gov (United States)

    Pokorney, Sean D; Stone, Neil J; Passman, Rod; Oyer, David; Rigolin, Vera H; Bonow, Robert O

    2010-12-01

    Patients with obstructive hypertrophic cardiomyopathy who undergo septal myectomy are at risk for developing postoperative atrial fibrillation. Amiodarone is effective in treating this arrhythmia but is associated with multiple adverse effects, often with delayed onset. A novel case is described of a patient who developed type 2 amiodarone-induced hyperthyroidism that presented as recurrence of outflow obstruction after septal myectomy. The patient's symptoms and echocardiographic findings of outflow obstruction resolved substantially with the treatment of the amiodarone-induced hyperthyroidism. Amiodarone-induced hyperthyroidism of delayed onset can be a subtle diagnosis, requiring a high index of suspicion. In conclusion, recognition of this diagnosis in patients with recurrence of outflow obstruction by symptoms and cardiac imaging after septal myectomy may avoid unnecessary repeat surgical intervention. Copyright © 2010 Elsevier Inc. All rights reserved.

  20. Cardiac involvement in children with neuro-muscular disorders

    Directory of Open Access Journals (Sweden)

    E. N. Arkhipova

    2015-01-01

    Full Text Available Many inherited neuromuscular disorders include cardiac involvement as a typical clinical feature. Among the most common of them is the group of muscular dystrophies. Dilated cardiomyopathy, ventricular arrhythmias, atrial fibrillations, atrioventricular and intraventricular conduction abnormalities, and sudden cardiac death are well known pathological findings in Duchenne muscular dystrophies, myotonic dystrophy type I and 2, Emery-Dreifuss muscular dystrophies and different types of limb-girdle muscular dystrophies and other disorders. Detection of cardiac pathology in patients with different muscular dystrophies is possible with ECG, echocardiography and cardiovascular magnetic resonance imaging, which are recommended for screening and early cardioprotective treatment.

  1. Natural history of ventricular septal defects in Nigerian children ...

    African Journals Online (AJOL)

    There were 2 (3.3%) deaths from bronchopneumonia and bacterial endocarditis. Conclusion. Spontaneous closure readily occurs in small-sized defects and muscular VSDs. However, most patients with moderate to large VSDs are confined to long-term medical management, highlighting the need for indigenous surgical ...

  2. The effect of surgical treatment for secundum atrial septal defect in patients more than 30 years old.

    Science.gov (United States)

    Erkut, Bilgehan; Becit, Necip; Unlu, Yahya; Ceviz, Munacettin; Kocogullari, Cevdet Ugur; Ates, Azman; Karapolat, Bekir Sami; Kaygin, Mehmet Ali; Kocak, Hikmet

    2007-01-01

    We prospectively examined whether surgical treatment of secundum atrial septal defects in patients 30 years old improves their early- and mid-term clinical outcomes. Our clinical experience is reviewed to assess the importance of surgical management in elderly patients with atrial septal defect. We analyzed 41 patients older than 30 years of age who underwent surgical correction of a secundum atrial septal defect. To evaluate the effects of surgical treatment, we compared functional capacity, diuretic administration, rhythm status, and echocardiographic parameters of all patients before and after the operation. The median follow-up period was 4.2 years (range, 6 months-7 years). There were no operative deaths. Functional class in most of the patients improved after operation. Two patients reverted to normal sinus rhythm after the operation. There was only one new atrial fibrilation among patients in the postoperative term. Right atrial and right ventricular dimensions and pulmonary artery pressures were significantly decreased, and ejection fractions were significantly increased after the operation. The need for diuretic treatment was decreased after surgical repair. No residual intracardiac shunts were identified during follow-up. There were no cerebrovascular thromboembolic accidents in the early postoperative period. Surgical closure of atrial septal defects in patients over 30 years old can improve their clinical status and prevent right ventricular dilatation and insufficiency. The operation must be performed as soon as possible, even if the symptoms or the hemodynamic impact seem to be minimal.

  3. Radionuclide study for cardiac lesion in Duchenne muscular dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Oguni, Hirokazu; Osawa, Makiko; Shishikura, Keiko

    1985-12-01

    Tl-201 myocardial scintigraphy and radionuclide ventriculography with Tc-99m were performed in 10 patients with Duchenne muscular dystropohy (DMD) and 2 siblings with Becker muscular dystrophy (BMD). Perfusion defect especially in the left ventricular posterolateral wall (LVPLW) and cardiac apex was seen on Tl-201 imaging in 6 of the DMD patients and one of the BMD patients. For these patients, Tc-99m imaging also showed left ventricular local wall motion abnormality in 5 patients and a decreased left ventricular ejection fraction in 4 patients. These findings coincided well with fibrosis of the LVPLW found on autopsy. There were individual differences regarding the occurrence of cardiac complications. One of the BMD patients, as well as DMD patients, had also cardiac complications which have long been considered less common. (Namekawa, K.).

  4. Radionuclide study for cardiac lesion in Duchenne muscular dystrophy

    International Nuclear Information System (INIS)

    Oguni, Hirokazu; Osawa, Makiko; Shishikura, Keiko

    1985-01-01

    Tl-201 myocardial scintigraphy and radionuclide ventriculography with Tc-99m were performed in 10 patients with Duchenne muscular dystropohy (DMD) and 2 siblings with Becker muscular dystrophy (BMD). Perfusion defect especially in the left ventricular posterolateral wall (LVPLW) and cardiac apex was seen on Tl-201 imaging in 6 of the DMD patients and one of the BMD patients. For these patients, Tc-99m imaging also showed left ventricular local wall motion abnormality in 5 patients and a decreased left ventricular ejection fraction in 4 patients. These findings coincided well with fibrosis of the LVPLW found on autopsy. There were individual differences regarding the occurrence of cardiac complications. One of the BMD patients, as well as DMD patients, had also cardiac complications which have long been considered less common. (Namekawa, K.)

  5. Reverse right ventricular remodeling after pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension: utility of magnetic resonance imaging to demonstrate restoration of the right ventricle

    NARCIS (Netherlands)

    Reesink, Herre J.; Marcus, J. Tim; Tulevski, Igor I.; Jamieson, Stuart; Kloek, Jaap J.; Vonk Noordegraaf, Anton; Bresser, Paul

    2007-01-01

    OBJECTIVES: Pulmonary arterial hypertension causes right ventricular remodeling; that is, right ventricular dilatation, hypertrophy, and leftward ventricular septal bowing. We studied the effect of pulmonary endarterectomy on the restoration of right ventricular remodeling in patients with chronic

  6. Dyssynchronous Ventricular Activation in Asymptomatic Wolff-Parkinson-White Syndrome: A Risk Factor for Development of Dilated Cardiomyopathy

    Science.gov (United States)

    Udink ten Cate, Floris EA; Wiesner, Nathalie; Trieschmann, Uwe; Khalil, Markus; Sreeram, Narayanswami

    2010-01-01

    A subset of children and adults with Wolff-Parkinson-White (WPW) syndrome develop dilated cardiomyopathy (DCM). Although DCM may occur in symptomatic WPW patients with sustained tachyarrhythmias, emerging evidence suggests that significant left ventricular dysfunction may arise in WPW in the absence of incessant tachyarrhythmias. An invariable electrophysiological feature in this non-tachyarrhythmia type of DCM is the presence of a right-sided septal or paraseptal accessory pathway. It is thought that premature ventricular activation over these accessory pathways induces septal wall motion abnormalities and ventricular dyssynchrony. LV dyssynchrony induces cellular and structural ventricular remodelling, which may have detrimental effects on cardiac performance. This review summarizes recent evidence for development of DCM in asymptomatic patients with WPW, discusses its pathogenesis, clinical presentation, management and treatment. The prognosis of accessory pathway-induced DCM is excellent. LV dysfunction reverses following catheter ablation of the accessory pathway, suggesting an association between DCM and ventricular preexcitation. Accessory pathway-induced DCM should be suspected in all patients presenting with heart failure and overt ventricular preexcitation, in whom no cause for their DCM can be found. PMID:20552060

  7. Regional left ventricular myocardial contraction abnormalities and asynchrony in patients with hypertrophic cardiomyopathy evaluated by magnetic resonance spatial modulation of magnetization myocardial tagging

    International Nuclear Information System (INIS)

    Mishiro, Yuichiro; Oki, Takashi; Iuchi, Arata

    1999-01-01

    Global left ventricular (LV) pump function is generally preserved in patients with hypertrophic cardiomyopathy (HCM). However, it is unknown whether regional myocardial contractility is impaired, especially in nonhypertrophied regions. The purpose of this study was to evaluate regional LV myocardial contraction in patients with HCM using magnetic resonance (MR) spatial modulation of magnetization (SPAMM) myocardial tagging. The study group comprised 20 patients with asymmetric septal hypertrophy (HCM group) and 16 age-matched normal patients (control group), and data were collected using transthoracic M-mode and 2-dimensional echocardiography, and MR SPAMM myocardial tagging. The systolic strain ratio, maximum systolic strain velocity, and time from end-diastole to maximum systolic strain (ΔT) in the anterior, ventricular septal, inferior and lateral regions for 2 LV short-axis sections at the levels of the chordae tendineae and papillary muscles were measured at 50-ms intervals by MR myocardial tagging. The end-diastolic anterior and ventricular septal wall thicknesses and LV mass index were significantly different between the HCM and control groups. The systolic strain ratio for all 4 walls, particularly the anterior and ventricular septal regions, was significantly lower in the HCM group. In the HCM group, the maximum systolic strain velocity was significantly lower and ΔT was significantly shorter for all 4 walls, particularly the anterior and ventricular septal regions. The standard deviation for the ΔT, calculated from the ΔT for the 8 regions of the 2 LV short-axis sections, was significantly greater in the HCM group. In conclusion, regional LV myocardial contraction is impaired in both hypertrophied and nonhypertrophied regions, and systolic LV wall asynchrony occurs in patients with HCM. (author)

  8. Reduced Sodium Current in the Lateral Ventricular Wall Induces Inferolateral J-Waves.

    Science.gov (United States)

    Meijborg, Veronique M F; Potse, Mark; Conrath, Chantal E; Belterman, Charly N W; De Bakker, Jacques M T; Coronel, Ruben

    2016-01-01

    J-waves in inferolateral leads are associated with a higher risk for idiopathic ventricular fibrillation. We aimed to test potential mechanisms (depolarization or repolarization dependent) responsible for inferolateral J-waves. We hypothesized that inferolateral J-waves can be caused by regional delayed activation of myocardium that is activated late during normal conditions. Computer simulations were performed to evaluate how J-point elevation is influenced by reducing sodium current conductivity (GNa), increasing transient outward current conductivity (Gto), or cellular uncoupling in three predefined ventricular regions (lateral, anterior, or septal). Two pig hearts were Langendorff-perfused with selective perfusion with a sodium channel blocker of lateral or anterior/septal regions. Volume-conducted pseudo-electrocardiograms (ECG) were recorded to detect the presence of J-waves. Epicardial unipolar electrograms were simultaneously recorded to obtain activation times (AT). Simulation data showed that conduction slowing, caused by reduced sodium current, in lateral, but not in other regions induced inferolateral J-waves. An increase in transient outward potassium current or cellular uncoupling in the lateral zone elicited slight J-point elevations which did not meet J-wave criteria. Additional conduction slowing in the entire heart attenuated J-waves and J-point elevations on the ECG, because of masking by the QRS. Experimental data confirmed that conduction slowing attributed to sodium channel blockade in the left lateral but not in the anterior/septal ventricular region induced inferolateral J-waves. J-waves coincided with the delayed activation. Reduced sodium current in the left lateral ventricular myocardium can cause inferolateral J-waves on the ECG.

  9. Asymmetric septal hypertrophy of sporadic form with abnormal thallium perfusion and myocardial enzymes

    International Nuclear Information System (INIS)

    Nagata, Seiki; Minamikawa, Tetsuhiro; Park, Yung-Dae; Nishimura, Tsunehiko; Yutani, Chikao; Ohmori, Fumio; Sakakibara, Hiroshi; Nimura, Yasuharu

    1986-01-01

    Asymmetric septal hypertrophy with abnormal thallium scintigram and elevated cardiac enzymes were observed in five patients and were studied with special reference to the clinical significance of their clinicopathological features. They were not familial cardiomyopathy patients. Two of the five patients (Cases 1 and 2) exhibited the clinical features characteristic of hypertrophic cardiomyopathy without abnormal thallium perfusion and serum cardiac enzyme levels. A right endomyocardial biopsy for Case 1 disclosed myocardial fibrosis in addition to hypertrophy and disarray of myocardial fibers. The left ventricular cavities of two other patients (Cases 4 and 5) tended to be dilated with signs of impaired systolic function and asymmetric septal hypertrophy. A regional area of reduced thickness was observed in the medial portion of the left ventricular posterior wall of Case 4. The remaining case (Case 3) exhibited left ventricular dilatation and reduced left ventricular systolic function, disproportionate hypertrophy, and had clinical signs of congestive heart failure. Necropsy disclosed massive fibrosis and diffuse disarray of myocardial fibers. Some patients with familial hypertrophic cardiomyopathy progress to exhibit clinical features of dilated cardiomyopathy in the termimal stages, and have massive fibrosis of the myocardium histologically. Thallium scintigraphic abnormalities and elevated serum levels of cardiac enzymes, especially the LDH 1 isoenzyme, in patients with hypertrophic cardiomyopathy may be a meaningful indicator of such progression in its early stages. The five patients in the present study exhibited a variety of clinical and histological features which may comprise a spectrum of clinical conditions during the progression from hypertrophic cardiomyopathy to a condition like dilated cardiomyopathy, similar to that in familial patients. This progression and the factors promoting it should be studied further in the near future. (author)

  10. Transthoracic device closure of ventricular septal defects without cardiopulmonary bypass: experience in infants weighting less than 8 kg.

    Science.gov (United States)

    Xing, Quansheng; Wu, Qin; Pan, Silin; Ren, Yueyi; Wan, Hao

    2011-09-01

    Both surgical and percutaneous device closure of ventricular septal defect (VSD) have drawbacks and limitations in infants. We report our experiences and midterm results of transthoracic device closure of VSDs (TDCVs) without cardiopulmonary bypass (CPB) in infants. Between September 2007 and September 2009, 32 patients, with a mean age of 7.2 ± 4.7 months, body weight of 6.8 ± 2.8 kg, underwent this procedure. The procedure was performed in the operating room. A small subxiphoid incision was made. A purse-string suture was placed on the right ventricular free wall. The free wall was punctured using a trocar, then a guide wire was inserted and advanced to cross the VSD into the left ventricle under transesophageal echocardiographic guidance. A modified delivery sheath was then introduced over the guide wire. The device was delivered and deployed in position along the sheath to close the defect. A total of 30 cases (94%) were successfully closed, and the remaining two cases (6%) were converted to open heart repair. No patients received transfusion. There was no perioperative mortality, or any major complication. The mean size of the devices was 7.6 ± 3.4mm. The total operative time was less than 60 min, and the mean time for device implantation was 18.3 ± 9.4 min. All patients were extubated within 2h, and were discharged within 5 days after operation. The follow-up period ranged from 6 to 31 months (18.3 ± 9.6 months). There was no late major complication detected. Minimally invasive TDCV without CPB is a safe and effective alternative to the conventional operation in low-body-weight infants. Copyright © 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

  11. Left ventricular filling pressure by septal and lateral E/e' equally predict cardiovascular events in the general population

    DEFF Research Database (Denmark)

    Wang, Joanna Nan; Biering-Sørensen, Tor; Jørgensen, Peter Godsk

    2017-01-01

    /e'lateral were equally strong predictors of cardiac events; in age- and sex-adjusted models they did not differ in AUC (septal: 0.8385, lateral: 0.8389; p = 0.94) or in continuous NRI (p = 0.84). Models using E/e'average did not improve AUC or NRI, and the intra-individual difference between sites had...... no predictive value (p = 0.79). E/e'septal was generally higher than E/e'lateral, thus age- and sex-specific normal values were reported for both sites for a population free of cardiac events during 10 years of follow-up. CONCLUSIONS: Septal and lateral E/e' are equally useful in predicting cardiac events...

  12. The effect of acute mechanical left ventricular unloading on ovine tricuspid annular size and geometry.

    Science.gov (United States)

    Malinowski, Marcin; Wilton, Penny; Khaghani, Asghar; Brown, Michael; Langholz, David; Hooker, Victoria; Eberhart, Lenora; Hooker, Robert L; Timek, Tomasz A

    2016-09-01

    Left ventricular assist device (LVAD) implantation may alter right ventricular shape and function and lead to tricuspid regurgitation. This in turn has been reported to be a determinant of right ventricular (RV) failure after LVAD implantation, but the effect of mechanical left ventricular (LV) unloading on the tricuspid annulus is unknown. The aim of the study was to provide insight into the effect of LVAD support on tricuspid annular geometry and dynamics that may help to optimize LV unloading with the least deleterious effect on the right-sided geometry. In seven open-chest anaesthetized sheep, nine sonomicrometry crystals were implanted on the right ventricle. Additional nine crystals were implanted around the tricuspid annulus, with one crystal at each commissure defining three separate annular regions: anterior, posterior and septal. Left ventricular unloading was achieved by connecting a cannula in the left atrium and the aorta to a continuous-flow pump. The pump was used for 15 min at a full flow of 3.8 ± 0.3 l/min. Epicardial echocardiography was used to assess the degree of tricuspid insufficiency. Haemodynamic, echocardiographic and sonomicrometry data were collected before and during full unloading. Tricuspid annular area, and the regional and total perimeter were calculated from crystal coordinates, while 3D annular geometry was expressed as the orthogonal distance of each annular crystal to the least squares plane of all annular crystals. There was no significant tricuspid regurgitation observed either before or during LV unloading. Right ventricular free wall to septum diameter increased significantly at end-diastole during unloading from 23.6 ± 5.8 to 26.3 ± 6.5 mm (P = 0.009), but the right ventricular volume, tricuspid annular area and total perimeter did not change from baseline. However, the septal part of the annulus significantly decreased its maximal length (38.6 ± 8.1 to 37.9 ± 8.2 mm, P = 0.03). Annular contraction was not altered. The

  13. Natural history of ventricular septal defects in Nigerian children

    African Journals Online (AJOL)

    Most (35 – 57.4%) of the patients were female, their mean age at presentation was 11.2±5.2 months, and the most common type of VSD ..... 26. 13 (50.0). Nutritional status. Malnutrition. 27. 8 (29.6). 0.104. Normal nutrition. 28. 19 (67.9). VSD type. Muscular. 17. 14 (82.4). 0.003. Perimembranous. 39. 14 (35.9). Subaortic. 5. 0.

  14. Deletion of ETS-1, a gene in the Jacobsen syndrome critical region, causes ventricular septal defects and abnormal ventricular morphology in mice

    Science.gov (United States)

    Ye, Maoqing; Coldren, Chris; Liang, Xingqun; Mattina, Teresa; Goldmuntz, Elizabeth; Benson, D. Woodrow; Ivy, Dunbar; Perryman, M.B.; Garrett-Sinha, Lee Ann; Grossfeld, Paul

    2010-01-01

    Congenital heart defects comprise the most common form of major birth defects, affecting 0.7% of all newborn infants. Jacobsen syndrome (11q-) is a rare chromosomal disorder caused by deletions in distal 11q. We have previously determined that a wide spectrum of the most common congenital heart defects occur in 11q-, including an unprecedented high frequency of hypoplastic left heart syndrome (HLHS). We identified an ∼7 Mb ‘cardiac critical region’ in distal 11q that contains a putative causative gene(s) for congenital heart disease. In this study, we utilized chromosomal microarray mapping to characterize three patients with 11q- and congenital heart defects that carry interstitial deletions overlapping the 7 Mb cardiac critical region. We propose that this 1.2 Mb region of overlap harbors a gene(s) that causes at least a subset of the congenital heart defects that occur in 11q-. We demonstrate that one gene in this region, ETS-1 (a member of the ETS family of transcription factors), is expressed in the endocardium and neural crest during early mouse heart development. Gene-targeted deletion of ETS-1 in mice in a C57/B6 background causes, with high penetrance, large membranous ventricular septal defects and a bifid cardiac apex, and less frequently a non-apex-forming left ventricle (one of the hallmarks of HLHS). Our results implicate an important role for the ETS-1 transcription factor in mammalian heart development and should provide important insights into some of the most common forms of congenital heart disease. PMID:19942620

  15. The Fate of the Tricuspid Valve Following the Transatrial Closure of the Ventricular Septal Defect.

    Science.gov (United States)

    Giordano, Raffaele; Cantinotti, Massimiliano; Di Tommaso, Luigi; Comentale, Giuseppe; Tozzi, Andrea; Pilato, Emanuele; Iannelli, Gabriele; Palma, Gaetano

    2018-05-17

    The transatrial repair of the ventricular septal defect (VSD) requires an adequate exposure of its rim. We retrospectively evaluated the impact of adopting the tricuspid valve incision (TVI) technique, either with detachment or radial incision, on the postoperative outcome of children undergoing surgical VSD repair. From January 2008 to September 2017 we retrospectively enrolled 141 patients, divided into two groups: 97 patients were subjected to TVI (68.8%) and 44 patients (31.2%) were not subjected to TVI. All patients received an echocardiogram upon discharge from the hospital and after 1 month, 3 months, 6 months and a year from the treatment. No perioperative or late deaths resulted in our dataset. TVI was associated with a slightly longer cardio-pulmonary bypass and cross-clamp time but there were no differences in the surgical outcome between the two groups. Moreover, no differences occurred concerning residual VSD, atrioventricular block or tricuspid regurgitation at discharge. Echocardiograms at follow-up were available for 134 patients (95%) with a median of 5.3 years (range 0.5-9.3) and the degree of tricuspid regurgitation did not differ between groups. No patient required reoperation for tricuspid regurgitation or residual interventricular shunt. Finally, no difference was found even when comparing the two TVI subgroups. TVI should be used whenever intraoperative exposure of VSD is compromised in order to avoid a residual shunt and atrioventricular block. Here we show that this procedure does not significantly compromise the tricuspid function although a large, multicenter, randomized controlled trial is advised to validate this hypothesis. Copyright © 2018. Published by Elsevier Inc.

  16. Characterisation of three-dimensional mapping in Wolff-Parkinson-White syndrome with septal aneurysmal dyskinesis.

    Science.gov (United States)

    Okada, Seigo; Muneuchi, Jun; Origuchi, Hideki

    2018-01-01

    A 21-year-old man with Wolff-Parkinson-White syndrome and aneurysmal septal dyskinesis underwent radiofrequency catheter ablation of the accessory pathways. Before radiofrequency catheter ablation, the activation wavefront arose from the aneurysmal septum, whereas the propagation of the left ventricle was normalised after radiofrequency catheter ablation. These findings demonstrate the importance of the electro-mechanical interaction in patients with Wolff-Parkinson-White syndrome and ventricular dysfunction.

  17. Characterization of SMAD3 Gene Variants for Possible Roles in Ventricular Septal Defects and Other Congenital Heart Diseases.

    Directory of Open Access Journals (Sweden)

    Fei-Feng Li

    Full Text Available Nodal/TGF signaling pathway has an important effect at early stages of differentiation of human embryonic stem cells in directing them to develop into different embryonic lineages. SMAD3 is a key intracellular messenger regulating factor in the Nodal/TGF signaling pathway, playing important roles in embryonic and, particularly, cardiovascular system development. The aim of this work was to find evidence on whether SMAD3 variations might be associated with ventricular septal defects (VSD or other congenital heart diseases (CHD.We sequenced the SMAD3 gene for 372 Chinese Han CHD patients including 176 VSD patients and evaluated SNP rs2289263, which is located before the 5'UTR sequence of the gene. The statistical analyses were conducted using Chi-Square Tests as implemented in SPSS (version 13.0. The Hardy-Weinberg equilibrium test of the population was carried out using the online software OEGE.Three heterozygous variants in SMAD3 gene, rs2289263, rs35874463 and rs17228212, were identified. Statistical analyses showed that the rs2289263 variant located before the 5'UTR sequence of SMAD3 gene was associated with the risk of VSD (P value=0.013 <0.05.The SNP rs2289263 in the SMAD3 gene is associated with VSD in Chinese Han populations.

  18. Virtual reality 3D echocardiography in the assessment of tricuspid valve function after surgical closure of ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Kappetein A Pieter

    2007-02-01

    Full Text Available Abstract Background This study was done to investigate the potential additional role of virtual reality, using three-dimensional (3D echocardiographic holograms, in the postoperative assessment of tricuspid valve function after surgical closure of ventricular septal defect (VSD. Methods 12 data sets from intraoperative epicardial echocardiographic studies in 5 operations (patient age at operation 3 weeks to 4 years and bodyweight at operation 3.8 to 17.2 kg after surgical closure of VSD were included in the study. The data sets were analysed as two-dimensional (2D images on the screen of the ultrasound system as well as holograms in an I-space virtual reality (VR system. The 2D images were assessed for tricuspid valve function. In the I-Space, a 6 degrees-of-freedom controller was used to create the necessary projectory positions and cutting planes in the hologram. The holograms were used for additional assessment of tricuspid valve leaflet mobility. Results All data sets could be used for 2D as well as holographic analysis. In all data sets the area of interest could be identified. The 2D analysis showed no tricuspid valve stenosis or regurgitation. Leaflet mobility was considered normal. In the virtual reality of the I-Space, all data sets allowed to assess the tricuspid leaflet level in a single holographic representation. In 3 holograms the septal leaflet showed restricted mobility that was not appreciated in the 2D echocardiogram. In 4 data sets the posterior leaflet and the tricuspid papillary apparatus were not completely included. Conclusion This report shows that dynamic holographic imaging of intraoperative postoperative echocardiographic data regarding tricuspid valve function after VSD closure is feasible. Holographic analysis allows for additional tricuspid valve leaflet mobility analysis. The large size of the probe, in relation to small size of the patient, may preclude a complete data set. At the moment the requirement of an I

  19. Altered left ventricular vortex ring formation by 4-dimensional flow magnetic resonance imaging after repair of atrioventricular septal defects.

    Science.gov (United States)

    Calkoen, Emmeline E; Elbaz, Mohammed S M; Westenberg, Jos J M; Kroft, Lucia J M; Hazekamp, Mark G; Roest, Arno A W; van der Geest, Rob J

    2015-11-01

    During normal left ventricular (LV) filling, a vortex ring structure is formed distal to the left atrioventricular valve (LAVV). Vortex structures contribute to efficient flow organization. We aimed to investigate whether LAVV abnormality in patients with a corrected atrioventricular septal defect (AVSD) has an impact on vortex ring formation. Whole-heart 4D flow MRI was performed in 32 patients (age: 26 ± 12 years), and 30 healthy subjects (age: 25 ± 14 years). Vortex ring cores were detected at peak early (E-peak) and peak late filling (A-peak). When present, the 3-dimensional position and orientation of the vortex ring was defined, and the circularity index was calculated. Through-plane flow over the LAVV, and the vortex formation time (VFT), were quantified to analyze the relationship of vortex flow with the inflow jet. Absence of a vortex ring during E-peak (healthy subjects 0%, vs patients 19%; P = .015), and A-peak (healthy subjects 10% vs patients 44%; P = .008) was more frequent in patients. In 4 patients, this was accompanied by a high VFT (5.1-7.8 vs 2.4 ± 0.6 in healthy subjects), and in another 2 patients with abnormal valve anatomy. In patients compared with controls, the vortex cores had a more-anterior and apical position, closer to the ventricular wall, with a more-elliptical shape and oblique orientation. The shape of the vortex core closely resembled the valve shape, and its orientation was related to the LV inflow direction. This study quantitatively shows the influence of abnormal LAVV and LV inflow on 3D vortex ring formation during LV inflow in patients with corrected AVSD, compared with healthy subjects. Copyright © 2015. Published by Elsevier Inc.

  20. Disturbed Intracardiac Flow Organization After Atrioventricular Septal Defect Correction as Assessed With 4D Flow Magnetic Resonance Imaging and Quantitative Particle Tracing

    NARCIS (Netherlands)

    Calkoen, Emmeline E.; de Koning, Patrick J. H.; Blom, Nico A.; Kroft, Lucia J. M.; de Roos, Albert; Wolterbeek, Ron; Roest, Arno A. W.; Westenberg, Jos J. M.

    2015-01-01

    Objectives Four-dimensional (3 spatial directions and time) velocity-encoded flow magnetic resonance imaging with quantitative particle tracing analysis allows assessment of left ventricular (LV) blood flow organization. Corrected atrioventricular septal defect (AVSD) patients have an abnormal left

  1. Nasal septal hematoma

    Science.gov (United States)

    ... medlineplus.gov/ency/article/001292.htm Nasal septal hematoma To use the sharing features on this page, please enable JavaScript. A nasal septal hematoma is a collection of blood within the septum ...

  2. Cardiac Complications of Fukuyama-Type Congenital Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2006-07-01

    Full Text Available The course of left ventricular function was evaluated using M-mode and Doppler echocardiography in 34 patients with Fukuyama-type congenital muscular dystrophy (FCMD, in a study at the Tokyo Women’s Medical University, Tokyo, Japan.

  3. Swiss cheese ventricular septal defect with myocarditis - A rare coexistence in a neonate

    Directory of Open Access Journals (Sweden)

    A R Saboo

    2012-01-01

    Full Text Available Myocarditis is defined as acute inflammation of the myocardium, usually following a non-specific flu-like illness, and encompasses a wide range of clinical presentations ranging from mild or subclinical disease to heart failure. We report a 12-day-old healthy full-term neonate who presented with abrupt onset of congestive cardiac failure (CCF following a viral prodrome. Examination revealed persistent sinus tachycardia, lymphocytosis, gross cardiomegaly, nonspecific electrocardiogram changes with echocardiography showing Swiss cheese ventricular septal defect (VSD. VSD alone very rarely presents as early-onset cardiac failure in the absence of other precipitating factors like anemia, sepsis, hypoglycemia etc. Myocarditis, however, can mimic VSD and can present as fulminant cardiac failure in an otherwise healthy newborn. Myocarditis is usually diagnosed based on circumstantial evidence such as a recent viral infection and the sudden onset of cardiac dysfunction while ruling out other diagnostic possibilities. Elevated troponin T level is one of the most crucial noninvasive diagnostic modalities. Several trials have concluded that levels >0.055 ng/ml are statistically significant for diagnosing myocarditis in children. In our case an abrupt onset of cardiac failure following a viral prodrome and markedly elevated cardiac troponin T without sepsis and in the presence of normal coronary anatomy clinched the diagnosis of myocarditis. An early and aggressive treatment for CCF along with regular long-term follow-up plays a key role in the management of myocarditis. Role of high-dose Intravenous immunoglobulin in myocarditis has been studied by many trials with different outcomes. This is the first case report showing coexistence of VSD with myocarditis in a neonate presenting as early-onset acute cardiac failure. The report highlights the importance of screening for myocarditis in all previously normal babies presenting primarily with cardiogenic

  4. Characterization of circulating microRNA expression in patients with a ventricular septal defect.

    Directory of Open Access Journals (Sweden)

    Dong Li

    Full Text Available Ventricular septal defect (VSD, one of the most common types of congenital heart disease (CHD, results from a combination of environmental and genetic factors. Recent studies demonstrated that microRNAs (miRNAs are involved in development of CHD. This study was to characterize the expression of miRNAs that might be involved in the development or reflect the consequences of VSD.MiRNA microarray analysis and reverse transcription-polymerase chain reaction (RT-PCR were employed to determine the miRNA expression profile from 3 patients with VSD and 3 VSD-free controls. 3 target gene databases were employed to predict the target genes of differentially expressed miRNAs. miRNAs that were generally consensus across the three databases were selected and then independently validated using real time PCR in plasma samples from 20 VSD patients and 15 VSD-free controls. Target genes of validated 8 miRNAs were predicted using bioinformatic methods.36 differentially expressed miRNAs were found in the patients with VSD and the VSD-free controls. Compared with VSD-free controls, expression of 15 miRNAs were up-regulated and 21 miRNAs were downregulated in the VSD group. 15 miRNAs were selected based on database analysis results and expression levels of 8 miRNAs were validated. The results of the real time PCR were consistent with those of the microarray analysis. Gene ontology analysis indicated that the top target genes were mainly related to cardiac right ventricle morphogenesis. NOTCH1, HAND1, ZFPM2, and GATA3 were predicted as targets of hsa-let-7e-5p, hsa-miR-222-3p and hsa-miR-433.We report for the first time the circulating miRNA profile for patients with VSD and showed that 7 miRNAs were downregulated and 1 upregulated when matched to VSD-free controls. Analysis revealed target genes involved in cardiac development were probably regulated by these miRNAs.

  5. Characterization of circulating microRNA expression in patients with a ventricular septal defect.

    Science.gov (United States)

    Li, Dong; Ji, Long; Liu, Lianbo; Liu, Yizhi; Hou, Haifeng; Yu, Kunkun; Sun, Qiang; Zhao, Zhongtang

    2014-01-01

    Ventricular septal defect (VSD), one of the most common types of congenital heart disease (CHD), results from a combination of environmental and genetic factors. Recent studies demonstrated that microRNAs (miRNAs) are involved in development of CHD. This study was to characterize the expression of miRNAs that might be involved in the development or reflect the consequences of VSD. MiRNA microarray analysis and reverse transcription-polymerase chain reaction (RT-PCR) were employed to determine the miRNA expression profile from 3 patients with VSD and 3 VSD-free controls. 3 target gene databases were employed to predict the target genes of differentially expressed miRNAs. miRNAs that were generally consensus across the three databases were selected and then independently validated using real time PCR in plasma samples from 20 VSD patients and 15 VSD-free controls. Target genes of validated 8 miRNAs were predicted using bioinformatic methods. 36 differentially expressed miRNAs were found in the patients with VSD and the VSD-free controls. Compared with VSD-free controls, expression of 15 miRNAs were up-regulated and 21 miRNAs were downregulated in the VSD group. 15 miRNAs were selected based on database analysis results and expression levels of 8 miRNAs were validated. The results of the real time PCR were consistent with those of the microarray analysis. Gene ontology analysis indicated that the top target genes were mainly related to cardiac right ventricle morphogenesis. NOTCH1, HAND1, ZFPM2, and GATA3 were predicted as targets of hsa-let-7e-5p, hsa-miR-222-3p and hsa-miR-433. We report for the first time the circulating miRNA profile for patients with VSD and showed that 7 miRNAs were downregulated and 1 upregulated when matched to VSD-free controls. Analysis revealed target genes involved in cardiac development were probably regulated by these miRNAs.

  6. Ambient Air Pollution and Traffic Exposures and Congenital Heart Defects in the San Joaquin Valley of California

    Science.gov (United States)

    Padula, Amy M.; Tager, Ira B.; Carmichael, Suzan L.; Hammond, S. Katharine; Yang, Wei; Lurmann, Frederick; Shaw, Gary M.

    2013-01-01

    Background Congenital anomalies are a leading cause of infant morbidity and mortality. Studies suggest associations between environmental contaminants and some anomalies, although evidence is limited. Methods We used data from the California Center of the National Birth Defects Prevention Study and the Children's Health and Air Pollution Study to estimate the odds of 27 congenital heart defects with respect to quartiles of 7 ambient air pollutant and traffic exposures in California during the first two months of pregnancy, 1997–2006 (N=813 cases and N=828 controls). Results Particulate matter <10 microns (PM10) was associated with pulmonary valve stenosis (aOR4th Quartile=2.6; 95% CI: 1.2, 5.7) and perimembranous ventricular septal defects (aOR3rd Quartile=2.1; 95% CI: 1.1, 3.9) after adjusting for maternal race-ethnicity, education and multivitamin use. PM2.5 was associated with transposition of the great arteries (aOR3rd Quartile=2.6; 95% CI: 1.1, 6.5) and inversely associated with perimembranous ventricular septal defects (aOR4th Quartile=0.5; 95% CI: 0.2, 0.9). Secundum atrial septal defects were inversely associated with carbon monoxide (aOR4th Quartile=0.4; 95% CI: 0.2, 0.8) and PM2.5 (aOR4th Quartile=0.5; 95% CI: 0.3, 0.8). Traffic density was associated with muscular ventricular septal defects (aOR4th Quartile=3.0, 95% CI: 1.2, 7.8) and perimembranous ventricular septal defects (aOR3rd Quartile =2.4; 95% CI: 1.3, 4.6), and inversely associated with transposition of the great arteries (aOR4th Quartile=0.3; 95% CI: 0.1, 0.8). Conclusions PM10 and traffic density may contribute to the occurrence of pulmonary valve stenosis and ventricular septal defects, respectively. The results were mixed for other pollutants and had little consistency with previous studies. PMID:23772934

  7. Postural Tachycardia Syndrome and Vasovagal Syncope: A Hidden Case of Obstructive Cardiomyopathy without Severe Septal Hypertrophy.

    Science.gov (United States)

    Mayuga, Kenneth A; Ho, Natalie; Shields, Robert W; Cremer, Paul; Rodriguez, L Leonardo

    2018-01-01

    A 36-year-old female with symptoms of orthostatic intolerance and syncope was diagnosed with vasovagal syncope on a tilt table test and with postural tachycardia syndrome (POTS) after a repeat tilt table test. However, an echocardiogram at our institution revealed obstructive cardiomyopathy without severe septal hypertrophy, with a striking increase in left ventricular outflow tract gradient from 7 mmHg at rest to 75 mmHg during Valsalva, with a septal thickness of only 1.3 cm. Cardiac MRI showed an apically displaced multiheaded posteromedial papillary muscle with suggestion of aberrant chordal attachments to the anterior mitral leaflet contributing to systolic anterior motion of the mitral valve. She underwent surgery with reorientation of the posterior medial papillary muscle head, resection of the tethering secondary chordae to the A1 segment of the mitral valve, chordal shortening and tacking of the chordae to the A1 and A2 segments of the mitral valve, and gentle septal myectomy. After surgery, she had significant improvement in her prior symptoms. To our knowledge, this is the first reported case of obstructive cardiomyopathy without severe septal hypertrophy with abnormalities in papillary muscle and chordal attachment, in a patient diagnosed with vasovagal syncope and POTS.

  8. Postural Tachycardia Syndrome and Vasovagal Syncope: A Hidden Case of Obstructive Cardiomyopathy without Severe Septal Hypertrophy

    Directory of Open Access Journals (Sweden)

    Kenneth A. Mayuga

    2018-01-01

    Full Text Available A 36-year-old female with symptoms of orthostatic intolerance and syncope was diagnosed with vasovagal syncope on a tilt table test and with postural tachycardia syndrome (POTS after a repeat tilt table test. However, an echocardiogram at our institution revealed obstructive cardiomyopathy without severe septal hypertrophy, with a striking increase in left ventricular outflow tract gradient from 7 mmHg at rest to 75 mmHg during Valsalva, with a septal thickness of only 1.3 cm. Cardiac MRI showed an apically displaced multiheaded posteromedial papillary muscle with suggestion of aberrant chordal attachments to the anterior mitral leaflet contributing to systolic anterior motion of the mitral valve. She underwent surgery with reorientation of the posterior medial papillary muscle head, resection of the tethering secondary chordae to the A1 segment of the mitral valve, chordal shortening and tacking of the chordae to the A1 and A2 segments of the mitral valve, and gentle septal myectomy. After surgery, she had significant improvement in her prior symptoms. To our knowledge, this is the first reported case of obstructive cardiomyopathy without severe septal hypertrophy with abnormalities in papillary muscle and chordal attachment, in a patient diagnosed with vasovagal syncope and POTS.

  9. Cardiac function associated with home ventilator care in Duchenne muscular dystrophy.

    Science.gov (United States)

    Lee, Sangheun; Lee, Heeyoung; Eun, Lucy Youngmin; Gang, Seung Woong

    2018-02-01

    Cardiomyopathy is becoming the leading cause of death in patients with Duchenne muscular dystrophy because mechanically assisted lung ventilation and assisted coughing have helped resolve respiratory complications. To clarify cardiopulmonary function, we compared cardiac function between the home ventilator-assisted and non-ventilator-assisted groups. We retrospectively reviewed patients with Duchenne muscular dystrophy from January 2010 to March 2016 at Gangnam Severance Hospital. Demographic characteristics, pulmonary function, and echocardiography data were investigated. Fifty-four patients with Duchenne muscular dystrophy were divided into 2 groups: home ventilator-assisted and non-ventilator-assisted. The patients in the home ventilator group were older (16.25±1.85 years) than those in the nonventilator group (14.73±1.36 years) ( P =0.001). Height, weight, and body surface area did not differ significantly between groups. The home ventilator group had a lower seated functional vital capacity (1,038±620.41 mL) than the nonventilator group (1,455±603.12 mL). Mean left ventricular ejection fraction and fractional shortening were greater in the home ventilator group, but the data did not show any statistical difference. The early ventricular filling velocity/late ventricular filling velocity ratio (1.7±0.44) was lower in the home ventilator group than in the nonventilator group (2.02±0.62). The mitral valve annular systolic velocity was higher in the home ventilator group (estimated β, 1.06; standard error, 0.48). Patients with Duchenne muscular dystrophy on a ventilator may have better systolic and diastolic cardiac functions. Noninvasive ventilator assistance can help preserve cardiac function. Therefore, early utilization of noninvasive ventilation or oxygen may positively influence cardiac function in patients with Duchenne muscular dystrophy.

  10. MicroRNA expression, target genes, and signaling pathways in infants with a ventricular septal defect.

    Science.gov (United States)

    Chai, Hui; Yan, Zhaoyuan; Huang, Ke; Jiang, Yuanqing; Zhang, Lin

    2018-02-01

    This study aimed to systematically investigate the relationship between miRNA expression and the occurrence of ventricular septal defect (VSD), and characterize the miRNA target genes and pathways that can lead to VSD. The miRNAs that were differentially expressed in blood samples from VSD and normal infants were screened and validated by implementing miRNA microarrays and qRT-PCR. The target genes regulated by differentially expressed miRNAs were predicted using three target gene databases. The functions and signaling pathways of the target genes were enriched using the GO database and KEGG database, respectively. The transcription and protein expression of specific target genes in critical pathways were compared in the VSD and normal control groups using qRT-PCR and western blotting, respectively. Compared with the normal control group, the VSD group had 22 differentially expressed miRNAs; 19 were downregulated and three were upregulated. The 10,677 predicted target genes participated in many biological functions related to cardiac development and morphogenesis. Four target genes (mGLUR, Gq, PLC, and PKC) were involved in the PKC pathway and four (ECM, FAK, PI3 K, and PDK1) were involved in the PI3 K-Akt pathway. The transcription and protein expression of these eight target genes were significantly upregulated in the VSD group. The 22 miRNAs that were dysregulated in the VSD group were mainly downregulated, which may result in the dysregulation of several key genes and biological functions related to cardiac development. These effects could also be exerted via the upregulation of eight specific target genes, the subsequent over-activation of the PKC and PI3 K-Akt pathways, and the eventual abnormal cardiac development and VSD.

  11. Novel mutation of GATA4 gene in Kurdish population of Iran with nonsyndromic congenital heart septals defects.

    Science.gov (United States)

    Soheili, Fariborz; Jalili, Zahra; Rahbar, Mahtab; Khatooni, Zahed; Mashayekhi, Amir; Jafari, Hossein

    2018-03-01

    The mutations in GATA4 gene induce inherited atrial and ventricular septation defects, which is the most frequent forms of congenital heart defects (CHDs) constituting about half of all cases. We have performed High resolution melting (HRM) mutation scanning of GATA4 coding exons of nonsyndrome 100 patients as a case group including 39 atrial septal defects (ASD), 57 ventricular septal defects (VSD) and four patients with both above defects and 50 healthy individuals as a control group. Our samples are categorized according to their HRM graph. The genome sequencing has been done for 15 control samples and 25 samples of patients whose HRM analysis were similar to healthy subjects for each exon. The PolyPhen-2 and MUpro have been used to determine the causative possibility and structural stability prediction of GATA4 sequence variation. The HRM curve analysis exhibit that 21 patients and 3 normal samples have deviated curves for GATA4 coding exons. Sequencing analysis has revealed 12 nonsynonymous mutations while all of them resulted in stability structure of protein 10 of them are pathogenic and 2 of them are benign. Also we found two nucleotide deletions which one of them was novel and one new indel mutation resulting in frame shift mutation, and 4 synonymous variations or polymorphism in 6 of patients and 3 of normal individuals. Six or about 50% of these nonsynonymous mutations have not been previously reported. Our results show that there is a spectrum of GATA4 mutations resulting in septal defects. © 2018 Wiley Periodicals, Inc.

  12. Exercises in anatomy: holes between the ventricles.

    Science.gov (United States)

    Anderson, Robert H; Sarwark, Anne E; Spicer, Diane E; Backer, Carl L

    2014-01-01

    Holes between the ventricles are the commonest congenital cardiac malformations. As yet, however, there is no consensus as to how they can best be described and categorized. In this, our third exercise in cardiac anatomy, we address the issue of classification of ventricular septal defects. We begin our demonstration by analysing the normal heart. We show that the larger part of the ventricular septum is made up of its muscular component. The membranous part accounts for only a small portion, which is located centrally within the cardiac base. This small membranous part forms a boundary between the right-sided chambers and the aortic root. Holes at this site, therefore, which account for the commonest defects closed surgically, will open centrally in the cardiac base, being located postero-inferiorly relative to the supraventricular crest. We then show that the larger part of the crest itself is a free-standing muscular sleeve, which lifts the leaflets of the pulmonary valve away from the cardiac base. Only a very small part of the muscle forming the right ventricular outlet is located in the septal position. Turning our attention to malformed hearts, we show how holes between the ventricles can open centrally at the cardiac base, open to the inlet or outlet of the right ventricle or open within the substance of the apical muscular septum. We demonstrate, however, that description of such geographical location of the defects does not paint the full picture, since lesions with markedly different phenotypic features can open in comparable geographic locations. We illustrate how it is the phenotypic features, as seen from the right ventricle, which convey the crucial information for the surgeon with regard to the location of the atrioventricular conduction axis, using hearts with holes opening to the inlet of the right ventricle with muscular as opposed to partially fibrous borders to emphasize this point. We continue by showing how holes with different phenotypes can

  13. Management of myocardial damage in muscular dystrophy

    International Nuclear Information System (INIS)

    Tamura, Takuhisa

    2011-01-01

    Heart failure (HF) is a fatal complication in many muscular dystrophy cases and has become the most common cause of death in Duchenne muscular dystrophy (DMD) since 2001. HF deaths in DMD occur in young patients and increase, along with respiratory failure, in older patients. Managing HF, therefore, is the most important component of DMD treatment. Management of HF is necessary in DMD patients of all ages because myocardial damage progresses regardless of age and disability. Electrocardiography, echocardiography, myocardial single-photon emission computed tomography (SPECT), and natriuretic peptides are used for the diagnosis of myocardial damage and chronic HF. Tissue Doppler echocardiography is in particularly useful for early detection of minute myocardial damage and dysfunction in DMD. The first-line drugs for chronic HF are angiotensin-converting enzyme inhibitors, and the prognosis of DMD patients has been improved using these drugs and beta-blockers. Diuretics are added in the presence of pulmonary congestion. Digoxin is most effective at a blood level of 0.5-0.8 ng/mL because of its pharmacokinetics in DMD. Surgical treatment may be necessary in cases of intractable HF. Cardiac resynchronization therapy (biventricular pacing), a treatment with an artificial pacemaker, is indicated for cases that meet specific criteria, including HF with ventricular dyssynchrony. Applications of partial left ventriculectomy (Batista procedure) and left ventricular assist devices in muscular dystrophy are likely in the near future. (author)

  14. Myocardial glucose metabolism is different between hypertrophic cardiomyopathy and hypertensive heart disease associated with asymmetrical septal hypertrophy

    International Nuclear Information System (INIS)

    Shiba, Nobuyuki; Kagaya, Yutaka; Ishide, Nobumasa; Takeyama, Daiya; Yamane, Yuriko; Chida, Masanobu; Otani, Hiroki; Shirato, Kunio; Ido, Tatsuo.

    1997-01-01

    Myocardial glucose metabolism has been shown to be heterogeneous in patients with hypertrophic cardiomyopathy (HCM). We tested the hypothesis that myocardial glucose metabolism differs between patients with HCM and those with hypertensive heart disease (HHD) associated with asymmetrical septal hypertrophy. We studied 12 patients with HCM, 7 HHD patients associated with asymmetrical septal hypertrophy using 18 F 2-deoxyglucose (FDG) and positron emission tomography. We calculated % FDG fractional uptake in the interventricular septum and posterolateral wall. Heterogeneity of FDG uptake was evaluated by % interregional coefficient of variation of FDG fractional uptake in each wall segment. In both the interventricular septum and posterolateral wall, % FDG fractional uptake was not significantly different between the two groups. The % interregional coefficient of variation for both interventricular septum (10.6±1.6 vs. 4.1±0.5, p<0.01) and posterolateral wall (5.9±0.7 vs. 3.8±0.5, p< 0.05) was significantly larger in patients with HCM than in HHD patients associated with asymmetrical septal hypertrophy. Echocardiography demonstrated that the degree of asymmetrical septal hypertrophy was similar between the two groups. These results suggest that myocardial glucose metabolism may be more heterogeneous in patients with HCM compared to HHD patients associated with asymmetrical septal hypertrophy, although the left ventricular shape is similar. The difference in the heterogeneity might have resulted from differences in the pathogeneses of the two diseases. (author)

  15. Atrial septal defect closure with the new Cardia Ultrasept II™ device with interposed Goretex patch: Mexican experience - has the perforation of Ivalon's membrane been solved?

    Science.gov (United States)

    Mijangos-Vázquez, Roberto; García-Montes, Antonio J; Soto-López, Elena M; Guarner-Lans, Verónica; Zabal, Carlos

    2018-05-01

    The objective of this study was to demonstrate the safety and feasibility of using the new Cardia Ultrasept II™ device with interposed Goretex patch referring to the perforation of polyvinyl alcohol membrane. Great advances have been made in the development of devices for closure of atrial septal defect. The Cardia Ultrasept II™ with interposed Goretex patch is the modified last generation of Cardia devices, having the advantage of a super-low profile within the atria and an integral locking delivery-retrieval mechanism that ensures safe deployment. In addition, with the interposition of the Goretex, it has been possible to abolish perforation of Ivalon's membrane as a complication.Methods and resultsPatients with ostium secundum atrial septal defect with surrounding rims with a minimum length of 5 mm and who underwent atrial septal defect closure with the new Ultrasept II™ with Goretex patch were included from two paediatric cardiac centres. Primary end point was to determine perforation of the Goretex membrane at follow-up; secondary end point included right ventricular diastolic diameter. In total, 30 patients underwent atrial septal defect closure at a median age of 6 (1-29) years. At follow-up for 6 (range, 1-15) months, freedom from perforations was 100%. A continuous decrease in right ventricular diastolic diameter was found with an initial median of 30 (25-49) mm and after catheterisation of 27.5 (18-33) mm, p=0.01, and Z-score of 2.6 (1.7-3.6) versus 1.9 (1-2.9) after procedure, p=0.01. The new modified generation of the Ultrasept II™ device with interposed Goretex patch is a good alternative to achieve atrial septal defect closure safely and feasibly with no membrane perforation at follow-up.

  16. Combining rhinoplasty with septal perforation repair.

    Science.gov (United States)

    Foda, Hossam M T; Magdy, Emad A

    2006-11-01

    A combined septal perforation repair and rhinoplasty was performed in 80 patients presenting with septal perforations (size 1 to 5 cm) and external nasal deformities. The external rhinoplasty approach was used for all cases and the perforation was repaired using bilateral intranasal mucosal advancement flaps with a connective tissue interposition graft in between. Complete closure of the perforation was achieved in 90% of perforations of size up to 3.5 cm and in only 70% of perforations that were larger than 3.5 cm. Cosmetically, 95% were very satisfied with their aesthetic result. The external rhinoplasty approach proved to be very helpful in the process of septal perforation repair especially in large and posteriorly located perforations and in cases where the caudal septal cartilage was previously resected. Our results show that septal perforation repair can be safely combined with rhinoplasty and that some of the routine rhinoplasty maneuvers, such as medial osteotomies and dorsal lowering, could even facilitate the process of septal perforation repair.

  17. Intraoperative device closure of perimembranous ventricular septal defects in the young children under transthoracic echocardiographic guidance; initial experience

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    Cao Hua

    2011-12-01

    Full Text Available Abstract Objectives This study aimed to assess the safety and feasibility of intraoperative device closure of perimembranous ventricular septal defects (VSD in young children guided by transthoracic echocardiography (TTE. Methods We enrolled 18 patients from our hospital to participate in the study from June 2011 to September 2011. A minimal inferior median incision was performed after full evaluation of the perimembranous VSD by real-time TTE, and a domestically made device was inserted to occlude the perimembranous VSD. The proper size of the device was determined by means of transthoracic echocardiographic analysis. Results Implantation was ultimately successful in 16 patients using TTE guidance. In these cases, the complete closure rate immediately following the operation and on subsequent follow-up was 100%. Symmetric devices were used in 14 patients, and asymmetric devices were used in two patients. Two patient were transformed to surgical treatment, one for significant residual shunting, and the other for unsuccessful wire penetration of the VSD. The follow-up periods were less than nine months, and only one patient had mild aortic regurgitation. There were no instances of residual shunt, noticeable aortic regurgitation, significant arrhythmia, thrombosis, or device failure. Conclusions Minimally invasive transthoracic device closure of perimembranous VSDs is safe and feasible, using a domestically made device under transthoracic echocardiographic guidance, without the need for cardiopulmonary bypass. This technique should be considered an acceptable alternative to surgery or device closure guided by transesophageal echocardiography in selected young children. However, a long-term evaluation of outcomes is necessary.

  18. Noninvasive assessment of right ventricular wall motion by radionuclide cardioangiography

    International Nuclear Information System (INIS)

    Nishimura, Tsunehiko; Uehara, Toshiisa; Naito, Hiroaki; Hayashida, Kohei; Kozuka, Takahiro

    1981-01-01

    Radionuclide cardioangiography is a useful method to evaluate the left ventricular wall motion in various heart diseases. It has been also attempted to assess the right ventricular wall motion simultaneously by radionuclide method. In this study, using the combination of first-pass (RAO 30 0 ) and multi-gate (LAO 40 0 ) method, the site of right vetricle was classified in five. (1 inflow, 2 sinus, 3 outflow, 4 septal, 5 lateral) and the degree of wall motion was classified in four stages (dyskinesis, akinesis, hypokinesis, normal) according to the AHA committee report. These methods were applied clinically to forty-eight patients with various heart diseases. In the cases with right ventricular pressure or volume overload such as COLD, pulmonary infarction, the right ventricle was dilated and the wall motion was reduced in all portions. Especially, in the cases with right ventricular infarction, the right ventricular wall motion was reduced in the infarcted area. The findings of radionuclide method were in good agreement with those of contrast right ventriculography or echocardiography. In conclusion, radionuclide cardioangiography is a useful and noninvasive method to assess not only the left but also the right ventricular wall motion. (author)

  19. Case report: an unstable wide QRS complexes tachycardia after ablation of a poster-septal accessory pathway: What is the mechanism?

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    Wang, Huan; Che, Xiaoru

    2018-03-01

    Differentiation of wide QRS complex tachycardia required repeated electrophysiological stimuli and mapping. However, instability of tachycardia would increase the difficulty in differential diagnosis. In this paper, we reported a wide QRS tachycardia following ablation of an atrioventricular reentrant tachycardia participated by a poster-septal accessory pathway. Limited differentiation strategy was performed because the wide QRS tachycardia was self-limited and with unstable hemodynamics. We analyzed the mechanism of the wide QRS tachycardia by only 4 beats ventricular overpacing. On the basis of the last ventricular pacing, an atypical atrioventricular nodal reentrant tachycardia was confirmed. After slow-pathway modification, the wide QRS tachycardia was eliminated. It was an atypical atrial-ventricular node reentrant tachycardia with right bundle branch block. Reasonable analysis based on electrophysiological electrophysiologic knowledge was the basis of successful diagnosis and treatment.

  20. Effect of right ventricular electrode location (outflow tract vs. apex) on mechanical Ventricular synchrony in patients that underwent pacemaker implant therapy

    International Nuclear Information System (INIS)

    Rincon, Oscar S; Saenz, Luis C; Salazar, Gabriel; Hernandez, Edgar

    2008-01-01

    Objective: to assess in depth the effect of ventricular stimulation from the right ventricular outflow tract and the apex on mechanical ventricular synchrony. Materials And Methods: cohort analytical study. 20 patients with indication of definitive pacemaker indication underwent trans thoracic echocardiogram before and after pacemaker implant with electrode implantation in the right ventricular outflow tract and in the apex (10 patients in each group). There was no structural cardiopathy, ejection fraction was ? 50%, QRS and AV conduction were normal. Mechanical ventricular asynchrony (M mode and tissue doppler) and implant and device parameters were evaluated. Statistical Analysis: results are given as mean values, standard deviation or percentages.Continuous variables were compared using Chi-square test and ANOVA. A p <0.05 value was considered statistically significant. Results: in five patients (25%) a pre-implant ventricular asynchrony was found; in seven (70%) ventricular asynchrony post-implant in the right ventricle outflow tract and in 5 (50%) in the apex. Mean interventricular pot-implant delay was 21,6 ms in the right ventricular outflow tract and 11,5 ms in the apex (p = 0,8); mean septal to lateral wall delay was 73 ms in the right ventricular outflow tract and 26 ms in the apex (p = 0,8). QRS post-implant delay was 134 ms in the right ventricular outflow tract and 140 ms in the apex (p = 0,1). No differences between implant parameters and device programming were found. Conclusions: presence of ventricular asynchrony was evidenced in patients with normal QRS and structurally healthy heart. Ventricular stimulation with pacemaker from the apex or the right ventricular outflow tract suggests acute ventricular asynchrony at least in 60% of the cases, without statistically significant difference between both groups.

  1. Evolution and development of ventricular septation in the amniote heart.

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    Robert E Poelmann

    Full Text Available During cardiogenesis the epicardium, covering the surface of the myocardial tube, has been ascribed several functions essential for normal heart development of vertebrates from lampreys to mammals. We investigated a novel function of the epicardium in ventricular development in species with partial and complete septation. These species include reptiles, birds and mammals. Adult turtles, lizards and snakes have a complex ventricle with three cava, partially separated by the horizontal and vertical septa. The crocodilians, birds and mammals with origins some 100 million years apart, however, have a left and right ventricle that are completely separated, being a clear example of convergent evolution. In specific embryonic stages these species show similarities in development, prompting us to investigate the mechanisms underlying epicardial involvement. The primitive ventricle of early embryos becomes septated by folding and fusion of the anterior ventricular wall, trapping epicardium in its core. This folding septum develops as the horizontal septum in reptiles and the anterior part of the interventricular septum in the other taxa. The mechanism of folding is confirmed using DiI tattoos of the ventricular surface. Trapping of epicardium-derived cells is studied by transplanting embryonic quail pro-epicardial organ into chicken hosts. The effect of decreased epicardium involvement is studied in knock-out mice, and pro-epicardium ablated chicken, resulting in diminished and even absent septum formation. Proper folding followed by diminished ventricular fusion may explain the deep interventricular cleft observed in elephants. The vertical septum, although indistinct in most reptiles except in crocodilians and pythonidsis apparently homologous to the inlet septum. Eventually the various septal components merge to form the completely septated heart. In our attempt to discover homologies between the various septum components we aim to elucidate the

  2. The History of Left Septal Fascicular Block: Chronological Considerations of a Reality Yet to be Universally Accepted

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    Andres Ricardo Perez Riera

    2008-04-01

    Full Text Available There are several papers in literature that prove in a conclusive and incontestable way, that the left branch of the His bundle, in most instances (85% of the cases splits into three fascicles of variable morphological pattern, and not into two: left anterior fascicle (LAF, left posterior fascicle (LPF, and left septal fascicle (LSF. The abovementioned papers have anatomical, histological, anatomo-pathological, electrocardiographic, and vectocardiographic, body surface potential mapping or ECG potential mapping and electrophysiological foundation. Additionally, the mentioned papers have been performed both in animal models (dogs and in the human heart. Several clinical papers have shown that the left septal fascicular block (LSFB may occur intermittently or transitorily as a consequence of a temporary dromotropic alteration, constituting an aberrant ventricular conduction, rate-dependent or by the application of atrial extra-stimuli, or naturally during the acute phase of infarction when this involves the anterior descending artery, before the septal perforating artery that supplies the central portion of the septum, where the mentioned LSF runs. The ECG/VCG manifestation of LSFB consists in anterior shift of electromotive forces, known as Prominent Anterior Forces (PAF, which can hardly be diagnosed in the clinical absence of other causes capable of causing PAF, such as the normal variant by counterclockwise rotation of the heart on its longitudinal axis, in right ventricular enlargement, in the dorsal or lateral infarction of the new nomenclature, in type-A WPW, in CRBBB, and others. In this historical manuscript, we review in a sequential fashion, the main findings that confirmed the unequivocal existence of this unjustifiably "forgotten" dromotropic disorder. In the developed countries, its most important cause is coronary insufficiency, particularly the proximal involvement of the left anterior descending coronary artery, and in Latin

  3. Long-term Associations of an Early Corrected Ventricular Septal Defect and Stress Systems of Child and Mother at Primary School Age.

    Science.gov (United States)

    Stonawski, Valeska; Vollmer, Laura; Köhler-Jonas, Nicola; Rohleder, Nicolas; Golub, Yulia; Purbojo, Ariawan; Moll, Gunther H; Heinrich, Hartmut; Cesnjevar, Robert A; Kratz, Oliver; Eichler, Anna

    2017-01-01

    Ventricular septal defect (VSD) is the most common congenital heart defect, with larger VSDs typically being corrected with an open-heart surgery during infancy. Long-term consequences of a VSD-corrective surgery on stress systems of child and mother are still unknown. The aim of the present study is to investigate the associations of an early corrected VSD and diurnal cortisol release of child and mother. 26 children (12 boys) between 6 and 9 years old, who underwent surgery for an isolated VSD within the first 3 years of life, and their mothers participated in the study. Their diurnal cortisol profiles were compared to a sex-, age-, and socioeconomic status-matched healthy control group. Within the VSD group, associations between cortisol and characteristics of surgery and hospitalization were investigated. Child and mother psychopathological symptoms were considered as a possible interfering mechanism of altered cortisol profiles. Diurnal cortisol profiles of children with an early corrected VSD did not differ from those of controls. However, mothers of affected children exhibited higher cortisol levels in the morning ( p  early corrected VSD, in terms of comparable diurnal cortisol profiles with healthy controls, according to a comparable mother-rated psychopathology. Mothers of affected children reveal altered diurnal cortisol levels, without differences in self-rated psychopathology. This divergence should be clarified in future research.

  4. Early diastolic mitral annular velocity at the interventricular septal annulus correctly reflects left ventricular longitudinal myocardial relaxation.

    Science.gov (United States)

    Okada, Kazunori; Mikami, Taisei; Kaga, Sanae; Onozuka, Hisao; Inoue, Mamiko; Yokoyama, Shinobu; Nishino, Hisao; Nishida, Mutsumi; Matsuno, Kazuhiko; Iwano, Hiroyuki; Yamada, Satoshi; Tsutsui, Hiroyuki

    2011-12-01

    Early diastolic mitral annular velocity (e') obtained by tissue Doppler imaging (TDI) is widely used to evaluate left ventricular (LV) diastolic function based on the assumption that it reflects myocardial relaxation in the long-axis direction. In this study, we aimed to determine whether or not e' truly reflects early diastolic longitudinal myocardial relaxation, and which is the most useful for evaluating LV diastolic function among e' measured at the interventricular-septal annulus (IS-e'), that measured at the lateral annulus (LW-e') or their mean value (M-e'). IS-e', LW-e', and M-e' were measured using colour TDI in 15 patients with hypertrophic cardiomyopathy, 13 patients with hypertension, and 19 control subjects. Using two-dimensional speckle-tracking imaging, early diastolic myocardial strain rates (SR(E)) were measured for the IS (IS-SR(E)), LW (LW-SR(E)), and entire LV myocardium (G-SR(E)). IS-e' was excellently correlated with IS-SR(E) (r = 0.90, P < 0.001); the correlation was better than that between LW-e' and LW-SR(E) (r = 0.75, P < 0.001). IS-e' and M-e' were well correlated with G-SR(E) (r = 0.88, P < 0.001 and r = 0.86, P< 0.001, respectively) and with LV early diastolic flow propagation velocity (FPV) (r = 0.77, P < 0.001 and r = 0.78, P < 0.001, respectively). The correlations of LW-e' to G-SR(E) (r = 0.80, P < 0.001) and FPV (r = 0.75, P < 0.001) did not reach this level. IS-e' well reflected LV longitudinal myocardial relaxation and LV diastolic function, and was found to be more useful in evaluating LV diastolic function than LW-e'.

  5. Bone recycling in nasal septal reconstruction.

    Science.gov (United States)

    Apaydin, Fazil

    2013-12-01

    Septal reconstruction alone or together with rhinoplasty can be a very challenging operation. In situations where septal cartilage is used for grafting or is not enough, bony implants taken from the perpendicular plate of the ethmoid and vomer can be used as a filler material between the mucoperichondrial flaps to avoid from unwanted mucosal atrophy, flapping, and septal perforation. These bony implants can also be used for splinting the dorsal and/or caudal segment of the septal cartilage after reshaping by rongeurs. On rare occasions, they can even be used for subtotal reconstruction of the septum. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  6. Left Ventricular Dysfunction Caused by Unrecognized Surgical AV block in a Patient with a Manifest Right Free Wall Accessory Pathway

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    Rakesh Gopinathannair, MD, MA

    2013-05-01

    Full Text Available A 24-year-old male with Wolff-Parkinson-White syndrome developed systolic cardiomyopathy and severe heart failure following membranous ventricular septal defect repair and tricuspid valve replacement. Following successful catheter ablation of a right anterolateral accessory pathway (AP, complete AV block with junctional escape rhythm was noted. Patient subsequently underwent implantation of a biventricular ICD. Heart failure symptoms significantly improved soon after and left ventricular systolic function normalized 3 months post-procedure. In this case, surgically acquired AV block likely explains development of postoperative cardiomyopathy by facilitating ventricular activation solely via the AP and thereby increasing the degree of ventricular dyssynchrony.

  7. Echocardiographic findings in infants with presumed congenital Zika syndrome: Retrospective case series study

    Science.gov (United States)

    Santos, Cleusa C.; Feitosa, Fabiana G.; Ribeiro, Maria C.; Menge, Paulo; Lira, Izabelle M.

    2017-01-01

    Objective To report the echocardiographic evaluation of 103 infants with presumed congenital Zika syndrome. Methods An observational retrospective study was performed at Instituto de Medicina Integral Prof. Fernando Figueira (IMIP), Recife, Brazil. 103 infants with presumed congenital Zika syndrome. All infants had microcephaly and head computed tomography findings compatible with congenital Zika syndrome. Zika IgM antibody was detected in cerebrospinal fluid samples of 23 infants. In 80 infants, the test was not performed because it was not available at that time. All infants had negative serology for HIV, syphilis, rubella, cytomegalovirus and toxoplasmosis. A complete transthoracic two-dimensional, M-mode, continuous wave and pulsed wave Doppler and color Doppler echocardiographic (PHILIPS HD11XE or HD15) examination was performed on all infants. Results 14/103 (13.5%) echocardiograms were compatible with congenital heart disease: 5 with an ostium secundum atrial septal defect, 8 had a hemodynamically insignificant small apical muscular ventricular septal defect and one infant with dyspnea had a large membranous ventricular septal defect. The echocardiograms considered normal included 45 infants with a persistent foramen ovale and 16 with a minimum patent ductus arteriosus. Conclusions Preliminarily this study suggests that congenital Zika syndrome may be associated with an increase prevalence of congenital heart disease. However the types of defects noted were septal defects, a proportion of which would not be hemodynamically significant. PMID:28426680

  8. Echocardiographic findings in infants with presumed congenital Zika syndrome: Retrospective case series study.

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    Danielle Di Cavalcanti

    Full Text Available To report the echocardiographic evaluation of 103 infants with presumed congenital Zika syndrome.An observational retrospective study was performed at Instituto de Medicina Integral Prof. Fernando Figueira (IMIP, Recife, Brazil. 103 infants with presumed congenital Zika syndrome. All infants had microcephaly and head computed tomography findings compatible with congenital Zika syndrome. Zika IgM antibody was detected in cerebrospinal fluid samples of 23 infants. In 80 infants, the test was not performed because it was not available at that time. All infants had negative serology for HIV, syphilis, rubella, cytomegalovirus and toxoplasmosis. A complete transthoracic two-dimensional, M-mode, continuous wave and pulsed wave Doppler and color Doppler echocardiographic (PHILIPS HD11XE or HD15 examination was performed on all infants.14/103 (13.5% echocardiograms were compatible with congenital heart disease: 5 with an ostium secundum atrial septal defect, 8 had a hemodynamically insignificant small apical muscular ventricular septal defect and one infant with dyspnea had a large membranous ventricular septal defect. The echocardiograms considered normal included 45 infants with a persistent foramen ovale and 16 with a minimum patent ductus arteriosus.Preliminarily this study suggests that congenital Zika syndrome may be associated with an increase prevalence of congenital heart disease. However the types of defects noted were septal defects, a proportion of which would not be hemodynamically significant.

  9. Atrial septal stenting - How I do it?

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    Kothandam Sivakumar

    2015-01-01

    Full Text Available A wide atrial communication is important to maintain hemodynamics in certain forms of congenital and acquired heart defects. In comparison to balloon septostomy or blade septostomy, atrial septal stenting provides a controlled, predictable, and long-lasting atrial communication. It often needs a prior Brockenbrough needle septal puncture to obtain a stable stent position. A stent deployed across a previously dilated and stretched oval foramen or tunnel form of oval foramen carries higher risk of embolization. This review provides technical tips to achieve a safe atrial septal stenting. Even though this is a "How to do it article," an initial discussion about the indications for atrial septal stenting is vital as the resultant size of the atrial septal communication should be tailored for each indication.

  10. Dyssynchronous ventricular contraction in Wolff-Parkinson-White syndrome: a risk factor for the development of dilated cardiomyopathy.

    Science.gov (United States)

    Dai, Chen-Cheng; Guo, Bao-Jing; Li, Wen-Xiu; Xiao, Yan-Yan; Jin, Mei; Han, Lin; Sun, Jing-Ping; Yu, Cheuk-Man; Dong, Jian-Zeng

    2013-11-01

    Emerging evidence suggests that significant left ventricular dysfunction may arise in right-sided septal or paraseptal accessory pathways (APs) with Wolff-Parkinson-White syndrome, even in the absence of recurrent or incessant tachycardia. During 1 year and 9 months, we identified four consecutive female children with median age of 8 years diagnosed as having dilated cardiomyopathy (DCM) combined with overt right-sided APs several years ago. Incessant or recurrent tachycardia as the cause of DCM could be excluded. Anti-heart failure chemotherapy did not produce satisfactory effects. The patients underwent radiofrequency ablations (RFCAs). This report describes the clinical and echocardiographic characteristics of the cases before and after the ablation. Dyssynchronous ventricular contraction was observed in all patients. The locations of the APs were the right-sided anteroseptum and the free wall (n = 2 each). All patients received successful RFCAs. Their physical activities and growth improved greatly, and the echocardiographic data demonstrated that their left ventricular (LV) contraction recovered to synchrony shortly after the ablation and that their LV function recovered to normal gradually during the follow-up. A causal relationship between overt ventricular preexcitation and the development of DCM is supported by the complete recovery of LV function and reversed LV remodeling after the loss of ventricular preexcitation. Preexcitation-related dyssynchrony was probably the crucial mechanism. Not only right-sided septal or paraseptal but also free wall overt APs may induce LV dysfunction and even DCM. AP-induced DCM is an indication for ablation with a good prognosis.

  11. Tratamento cirúrgico da comunicação interventricular pós infarto agudo do miocárdio: conduta atual Present surgical management of post-myocardial infarction ventricular septal rupture

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    Luís Alberto Dallan

    1989-04-01

    Full Text Available Os autores descrevem sua experiência com o tratamento cirúrgico de 42 pacientes portadores de comunicação interventricular (CIV pós infarto agudo do miocárdio (IAM. Destacam a elevada mortalidade cirúrgica nos pacientes com choque cardiogênico instalado (66,6% em relação aos demais (9,5%. A observação de descompensações súbitas em pacientes hemodinâmicamente estáveis tem levado à indicação cirúrgica precoce, se possível, assim que estabelecido o diagnóstico da rotura do septo interventricular (RSI. O comprometimento de múltiplas artérias coronárias e faixas etárias elevadas foram considerados fatores agravantes no prognóstico cirúrgico. O mesmo não ocorreu com a relação fluxo pulmonar/sistêmico e shunt E-D, que não guardaram relação com a mortalidade. A técnica de exposição de ambas as cavidades ventriculares e reforço das 2 faces do septo roto com tecido biológico tem fornecido resultados gratificantes na correção da RSI, especialmente de localização posterior.The authors describe the experience with surgical treatment of 42 patients with ventricular septal rupture (VSR in post acute myocardial infarction (AMI. They showed increased mortality in patients with cardiogenic shock (66.6% with respect to others (9.5%. Sudden descompensations in patients hemodinamically stable were indications to early surgeries, if possible when the diagnosis of ventricular septum rupture (VSR was made. The compromise of multiple coronary arteries and advanced age were considered as aggravating factors in the prognosis of surgery. The same prognosis did not occur regarding pulmonary/systemic flow and shunt E-D. The technique of exposure in both ventricular cavity and reinforcement in the both sides of the ruptured septum with biological tissue offered good results in the correction of posterior VSR.

  12. Congenital left ventricular wall abnormalities in adults detected by gated cardiac multidetector computed tomography: Clefts, aneurysms, diverticula and terminology problems

    International Nuclear Information System (INIS)

    Erol, Cengiz; Koplay, Mustafa; Olcay, Ayhan; Kivrak, Ali Sami; Ozbek, Seda; Seker, Mehmet; Paksoy, Yahya

    2012-01-01

    Objectives: Our aim was to evaluate congenital left ventricular wall abnormalities (clefts, aneurysms and diverticula), describe and illustrate imaging features, discuss terminology problems and determine their prevalence detected by cardiac CT in a single center. Materials and methods: Coronary CT angiography images of 2093 adult patients were evaluated retrospectively in order to determine congenital left ventricular wall abnormalities. Results: The incidence of left ventricular clefts (LVC) was 6.7% (141 patients) and statistically significant difference was not detected between the sexes regarding LVC (P = 0.5). LVCs were single in 65.2% and multiple in 34.8% of patients. They were located at the basal to mid inferoseptal segment of the left ventricle in 55.4%, the basal to mid anteroseptal segment in 24.1%, basal to mid inferior segment in 17% and septal–apical septal segment in 3.5% of cases. The cleft length ranged from 5 to 22 mm (mean 10.5 mm) and they had a narrow connection with the left ventricle (mean 2.5 mm). They were contractile with the left ventricle and obliterated during systole. Congenital left ventricular septal aneurysm that was located just under the aortic valve was detected in two patients (0.1%). No case of congenital left ventricular diverticulum was detected. Conclusion: Cardiac CT allows us to recognize congenital left ventricular wall abnormalities which have been previously overlooked in adults. LVC is a congenital structural variant of the myocardium, is seen more frequently than previously reported and should be differentiated from aneurysm and diverticulum for possible catastrophic complications of the latter two.

  13. An Asymptomatic Case of Wolff-Parkinson-White Syndrome with Right-sided Free-wall Accessory Pathway and Left Ventricular Dysfunction

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    Takanao Mine, MD

    2011-01-01

    Full Text Available A 16-year-old girl with a known history of asymptomatic Wolff-Parkinson-White syndrome exhibited signs of left ventricular (LV septal akinesia and LV dysfunction during routine follow-up. A 12-lead surface ECG showed pre-excitation, a predominantly negative delta wave in V1 and left axis deviation, which was consistent with the presence of a right free-wall accessory pathway. Radiofrequency ablation of the anterolateral right atrium around the local shortest atrium-to-ventricle interval created the accessory pathway block. An echocardiogram taken one month after the procedure revealed that LV septal wall motion had normalized and that LV ejection fraction had improved from 50% before the ablation to 64% after the ablation. Most previous reports of asymptomatic patients of WPW with LV septal dyskinesia and dysfunction have described right septal or posteroseptal accessory pathways. This patient reported here represents a rare case with right free-wall accessory pathway and LV dysfunction without tachycardia.

  14. Life-threatening Arrhythmias in a Becker Muscular Dystrophy Family due to the Duplication of Exons 3-4 of the Dystrophin Gene.

    Science.gov (United States)

    Ishizaki, Masatoshi; Fujimoto, Akiko; Ueyama, Hidetsugu; Nishida, Yasuto; Imamura, Shigehiro; Uchino, Makoto; Ando, Yukio

    2015-01-01

    We herein present a report of three patients with Becker muscular dystrophy in the same family who developed complete atrioventricular block or ventricular tachycardia with severe cardiomyopathy. Our cases became unable to walk in their teens, and were introduced to mechanical ventilation due to respiratory muscle weakness in their twenties and thirties. In all three cases, a medical device such as a permanent cardiac pacemaker or an implantable cardiac defibrillator was considered to be necessary. The duplication of exons 3-4 in the dystrophin gene was detected in two of the patients. In patients with Becker muscular dystrophy, complete atrioventricular block or ventricular tachycardia within a family has rarely been reported. Thus attention should be paid to the possibility of severe arrhythmias in the severe phenotype of Becker muscular dystrophy.

  15. ECG-gated blood pool tomography in the determination of left ventricular volume, ejection fraction, and wall motion

    International Nuclear Information System (INIS)

    Underwood, S.R.; Ell, P.J.; Jarritt, P.H.; Emanuel, R.W.; Swanton, R.H.

    1984-01-01

    ECG-gated blood pool tomography promises to provide a ''gold standard'' for noninvasive measurement of left ventricular volume, ejection fraction, and wall motion. This study compares these measurements with those from planar radionuclide imaging and contrast ventriculography. End diastolic and end systolic blood pool images were acquired tomographically using an IGE400A rotating gamma camera and Star computer, and slices were reconstructed orthogonal to the long axis of the heart. Left ventricular volume was determined by summing the areas of the slices, and wall motion was determined by comparison of end diastolic and end systolic contours. In phantom experiments this provided an accurate measurement of volume (r=0.98). In 32 subjects who were either normal or who had coronary artery disease left ventricular volume (r=0.83) and ejection fraction (r=0.89) correlated well with those using a counts based planar technique. In 16 of 18 subjects who underwent right anterior oblique X-ray contrast ventriculography, tomographic wall motion agreed for anterior, apical, and inferior walls, but abnormal septal motion which was not apparent by contrast ventriculography, was seen in 12 subjects tomographically. All 12 had disease of the left anterior descending coronary artery and might have been expected to have abnormal septal motion. ECG-gated blood pool tomography can thus determine left ventricular volume and ejection fraction accurately, and provides a global description of wall motion in a way that is not possible from any single planar image

  16. Left Ventricular Dysfunction and Dilated Cardiomyopathy in Infants and Children with Wolff-Parkinson-White Syndrome in the Absence of Tachyarrhythmias

    Science.gov (United States)

    2012-01-01

    Left ventricular (LV) dysfunction and dilated cardiomyopathy (DCM) are rarely attributable to sustained or incessant tachyarrhythmias in infants and children with Wolff-Parkinson-White (WPW) syndrome. However, several recent reports suggested that significant LV dysfunction may develop in WPW syndrome in the absence of tachyarrhythmias. It is assumed that an asynchronous ventricular activation over the accessory pathway, especially right-sided, induces septal wall motion abnormalities, ventricular remodeling and ventricular dysfunction. The prognosis of DCM associated with asymptomatic WPW is excellent. Loss of ventricular pre-excitation results in mechanical resynchronization and reverse remodeling where LV function recovers completely. The reversible nature of LV dysfunction after loss of ventricular pre-excitation supports the causal relationship between LV dysfunction and ventricular pre-excitation. This review summarizes recent clinical and electrophysiological evidence for development of LV dysfunction or DCM in asymptomatic WPW syndrome, and discusses the underlying pathophysiological mechanism. PMID:23323117

  17. Horizontal right axillary minithoracotomy: aesthetic and effective option for atrial and ventricular septal defect repair in infants and toddlers

    Directory of Open Access Journals (Sweden)

    Luciana da Fonseca da Silva

    2014-04-01

    Full Text Available Introduction: Congenital heart defects treatment shows progressive reduction in morbidity and mortality, however, the scar, resulting from ventricular (VSD and atrial septal defect (ASD repair, may cause discomfort. Right axillary minithoracotomy approach, by avoiding the breast growth region, is an option for correction of these defects that may provide better aesthetic results at low cost. Since October 2011, we have been using this technique for repairing VSD and ASD defects as well as associated defects. Objectives: To evaluate the efficacy of this method in children undergoing correction of VSD and ASD, to compare perioperative clinical outcomes with those repaired by median sternotomy, and to evaluate the aesthetic result. Methods: Perioperative clinical data of 25 patients submitted to axillary thoracotomy were compared with data from a paired group of 25 patients with similar heart defects repaired by median sternotomy, from October 2011 to August 2012. Results: Axillary approach was possible even in infants. There was no mortality and the main perioperative variables were similar in both groups, except for lower use of blood products in the axillary group (6/25 vs. control (13/25, with statistical difference (P =0.04. The VSD size varied from 7 to 15 mm in axillary group. Cannulation of the aorta and vena cavae was performed through the main incision, whose size ranged from 3 to 5 cm in the axillary group, with excellent aesthetic results. Conclusion: The axillary thoracotomy was effective, allowing for a heart defect repair similar to the median sternotomy, with more satisfactory aesthetic results and reduced blood transfusion, and it can be safely used in infants.

  18. Predictors of right ventricular function as measured by tricuspid annular plane systolic excursion in heart failure

    DEFF Research Database (Denmark)

    Kjaergaard, Jesper; Iversen, Kasper K; Akkan, Dilek

    2009-01-01

    in heart failure patients, in particular with reduced septal longitudinal motion. TAPSE is decreased in patients with heart failure of ischemic etiology. However, the absolute reduction in TAPSE is small and seems to be of minor importance in the clinical utilization of TAPSE whether applied as a measure...... of right ventricular systolic function or as a prognostic factor....

  19. Resolving the True Ventricular Mural Architecture

    Directory of Open Access Journals (Sweden)

    Robert S. Stephenson

    2018-06-01

    Full Text Available The precise nature of packing together of the cardiomyocytes within the ventricular walls has still to be determined. The spiraling nature of the chains of interconnected cardiomyocytes has long been recognized. As long ago as the end of the nineteenth century, Pettigrew had emphasized that the ventricular cone was not arranged on the basis of skeletal muscle. Despite this guidance, subsequent anatomists described entities such as “bulbo-spiral muscles”, with this notion of subunits culminating in the suggestion that the ventricular cone could be unwrapped so as to produce a “ventricular myocardial band”. Others, in contrast, had suggested that the ventricular walls were arranged on the basis of “sheets”, or more recently “sheetlets”, with investigators seeking to establishing the angulation of these entities using techniques such as magnetic resonance imaging. Our own investigations, in contrast, have shown that the cardiomyocytes are aggregated together within the supporting fibrous matrix so as to produce a three-dimensional myocardial mesh. In this review, we summarize the previous accounts, and provide the anatomical evidence we have thus far accumulated to support the model of the myocardial mesh. We show how these anatomic findings underscore the concept of the myocardial mesh functioning in antagonistic fashion. They lend evidence to support the notion that the ventricular myocardium works as a muscular hydrostat.

  20. Impact of aortic prosthesis-patient mismatch on left ventricular mass regression.

    Science.gov (United States)

    Alassal, Mohamed A; Ibrahim, Bedir M; Elsadeck, Nabil

    2014-06-01

    Prostheses used for aortic valve replacement may be small in relation to body size, causing prosthesis-patient mismatch and delaying left ventricular mass regression. This study examined the effect of prosthesis-patient mismatch on regression of left ventricular mass after aortic valve replacement. We prospectively studied 96 patients undergoing aortic valve replacement between 2007 and 2012. Mean and peak gradients and indexed effective orifice area were measured by transthoracic echocardiography at 3 and 6 months postoperatively. Patient-prosthesis mismatch was defined as indexed effective orifice area ≤0.85 cm(2)·m(-2). Moderate prosthesis-patient mismatch was present in 25% of patients. There were no significant differences in demographic and operative data between patients with and without prosthesis-patient mismatch. Left ventricular dimensions, posterior wall thickness, transvalvular gradients, and left ventricular mass decreased significantly after aortic valve replacement in both groups. The interventricular septal diameter and left ventricular mass index regression, and left ventricular ejection fraction were better in patients without prosthesis-patient mismatch. There was a significant positive correlation between the postoperative indexed effective orifice area of each valve prosthesis and the rate of left ventricular mass regression. Prosthesis-patient mismatch leads to higher transprosthetic gradients and impaired left ventricular mass regression. A small-sized valve prosthesis does not necessarily result in prosthesis-patient mismatch, and may be perfectly adequate in patient with small body size. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  1. Study on Tei index of right ventricular by tissue doppler imaging and the observation point selection

    International Nuclear Information System (INIS)

    Luo Yinli; Wu Ji; Guo Shenglan; Zhang Di; Li Zhixian

    2009-01-01

    Objective: To explore the right ventricular (RV) Tei index in patients with pulmonary hypertension (PH) by tissue Doppler imaging (TDI), and to explore more accurate observation point to obtain Tei index of right ventricular. Methods: Assessment of RV Tei index values was performed in 95 patients with PH and 32 normal subjects. The 95 patients were grouped into 3 groups according to the severity of PH. Tei index values were obtained by TDI measurement from three observation points, the anterior tricuspid and septal tricuspid attachment points in the apical 4-chamber view and the posterior tricuspid attachment point in parasternal right heart 2-chamber review. Results: (1) RV Tel index values were measured at the three points of PH was higher than the normal significantly (P<0.05). (2) RV Tei index values of the three PH groups at he anterior tricuspid attachment had significant difference each other (P<0.05). RV Tei index values of low-grade and medium-grade PH groups at septal tricuspid and posterior tricuspid had no significant difference, but that of high-grade PH group were higher than the low-grade and medium-grade PH group. Conclusion: RV Tei index value was significantly increased in PH patients. The Tei index value measured by TDI at anterior tricuspid attachment point in apical 4-chamber view was better than that at septal tricuspid attachment point in the apical 4-chamber view and posterior' attachment of parasternal right heart 2-chamber. (authors)

  2. Idiopathic premature ventricular contractions and ventricular tachycardias originating from the vicinity of tricuspid annulus: Results of radiofrequency catheter ablation in thirty-five patients

    Directory of Open Access Journals (Sweden)

    Yue-Chun Li

    2012-07-01

    Full Text Available Abstract Background In recent years, catheter ablation has increasingly been used for ablation of idiopathic premature ventricular complexes (PVCs or ventricular tachycardias (IVTs. However, the mapping and catheter ablation of the arrhythmias originating from the vicinity of tricuspid annulus (TA may not be fully understood. This study aimed to investigate electrophysiologic characteristics and effects of radiofrequency catheter ablation (RFCA for patients with symptomatic PVCs and IVTs originating from the vicinity of TA. Methods Characteristics of body surface electrocardiogram (ECG and electrophysiologic recordings were analyzed in 35 patients with symptomatic PVCs/ IVTs originating from the vicinity of TA. RFCA was performed using pace mapping and activation mapping. Results Among the 35 patients with PVCs/IVTs arising from the vicinity of TA, complete elimination of PVCs/IVTs could be achieved by RFCA in 32 patients (success rate 91.43% during a median follow-up period of 21 months. PVCs/IVTs originating from the vicinity of TA had distinctive ECG characteristics that were useful for identifying the precise origin. An rS pattern was recorded in lead V1 in 93.1% of patients with PVCs/IVTs from the free wall of TA, vs 16.7% of patients with PVCs/IVTs from the septal TA, whereas a QS pattern in lead V1 occurred in 83.3% of patients with PVCs/IVTs from the septal TA vs 6.9% of patients with PVCs from the free wall of the TA. The precordial R wave transition occurred by lead V3 or earlier in all patients with PVCs/IVTs originating from the septal portion of the TA, as compared to transition beyond V3 in all patients with PVCs/IVTs from the free wall of the TA. Conclusions RFCA is an effective curative therapy for symptomatic PVCs/IVTs originating from the vicinity of TA. There are specific characteristics in ECG and the ablation site could be located by ECG analysis.

  3. Utilizing Three-Dimensional Printing Technology to Assess the Feasibility of High-Fidelity Synthetic Ventricular Septal Defect Models for Simulation in Medical Education.

    Science.gov (United States)

    Costello, John P; Olivieri, Laura J; Krieger, Axel; Thabit, Omar; Marshall, M Blair; Yoo, Shi-Joon; Kim, Peter C; Jonas, Richard A; Nath, Dilip S

    2014-07-01

    The current educational approach for teaching congenital heart disease (CHD) anatomy to students involves instructional tools and techniques that have significant limitations. This study sought to assess the feasibility of utilizing present-day three-dimensional (3D) printing technology to create high-fidelity synthetic heart models with ventricular septal defect (VSD) lesions and applying these models to a novel, simulation-based educational curriculum for premedical and medical students. Archived, de-identified magnetic resonance images of five common VSD subtypes were obtained. These cardiac images were then segmented and built into 3D computer-aided design models using Mimics Innovation Suite software. An Objet500 Connex 3D printer was subsequently utilized to print a high-fidelity heart model for each VSD subtype. Next, a simulation-based educational curriculum using these heart models was developed and implemented in the instruction of 29 premedical and medical students. Assessment of this curriculum was undertaken with Likert-type questionnaires. High-fidelity VSD models were successfully created utilizing magnetic resonance imaging data and 3D printing. Following instruction with these high-fidelity models, all students reported significant improvement in knowledge acquisition (P 3D printing technology to create high-fidelity heart models with complex intracardiac defects. Furthermore, this tool forms the foundation for an innovative, simulation-based educational approach to teach students about CHD and creates a novel opportunity to stimulate their interest in this field. © The Author(s) 2014.

  4. Clinical long-term outcome of septal myectomy for obstructive hypertrophic cardiomyopathy in infants.

    Science.gov (United States)

    Schleihauf, Julia; Cleuziou, Julie; Pabst von Ohain, Jelena; Meierhofer, Christian; Stern, Heiko; Shehu, Nerejda; Mkrtchyan, Naira; Kaltenecker, Emanuel; Kühn, Andreas; Nagdyman, Nicole; Hager, Alfred; Seidel, Heide; Lange, Rüdiger; Ewert, Peter; Wolf, Cordula M

    2018-03-01

    Surgical septal myectomy is performed to relieve left ventricular outflow tract narrowing in severe drug-refractory obstructive hypertrophic cardiomyopathy. The objective of this study was to assess the perioperative and long-term clinical outcome of this procedure performed during infancy. Clinical, transthoracic echocardiographic, electrocardiographic, 24-h Holter, cardiopulmonary exercise test and genetic data were extracted by medical record review. A subset of patients underwent additional prospective detailed clinical evaluation including cardiac magnetic resonance imaging with contrast. Surgery was performed in 23 paediatric patients between 1978 and 2015 at the German Heart Centre Munich. Twelve patients had undergone surgery during infancy (≤ 1 year) (Group A), 11 between 1 and 18 years of age (Group B). The underlying genetic diagnosis was Noonan syndrome spectrum and non-syndromic hypertrophic cardiomyopathy. As compared to Group B, patients in Group A showed more concomitant cardiac procedures and received more homologous transfusions. One perioperative death occurred in Group A, and none in Group B. Two patients in Group A but no patient in Group B required redo septal myectomy. The long-term clinical outcome was similar between the 2 groups. One patient in Group B required cardioverter-defibrillator/pacemaker implantation for higher degree atrioventricular block and none in Group A. There was no evidence of differences in myocardial fibrosis between groups on long-term follow-up magnetic resonance imaging. Surgical septal myectomy can be performed safely during infancy with favourable perioperative and long-term clinical outcome but with a trend towards a higher reoperation rate later in life. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  5. Anuloplastia de homoenxerto pulmonar criopreservado com anel de Delrin na atresia pulmonar com comunicação interventricular Annuloplasty of cryopreserved pulmonary homograft with Delrin stent in pulmonary atresia with ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Ulisses Alexandre Croti

    2007-05-01

    Full Text Available Criança de seis anos portadora de atresia pulmonar com comunicação interventricular, submetida a correção total com um ano, empregando monoválvula de pericárdio bovino na reconstrução da via de saída do ventrículo direito. Evoluiu com importante regurgitação valvar pulmonar (RVP e disfunção do ventrículo direito. Na reoperação foi implantado homoenxerto pulmonar criopreservado (HPC com anuloplastia, utilizando anel de Delrin com o intuito de evitar distorção geométrica do conduto. Após dois anos, o ecocardiograma, semelhante ao pós-operatório imediato, demonstra RVP discreta e função ventricular direita normal, sugerindo que essa manobra pode ser utilizada como coadjuvante para otimizar o resultado do implante do HPC.A six-year-old child was suffering from pulmonary atresia with ventricular septal defect after a total correction at 1 year of age using a bovine pericardial monocusp valve in the reconstruction of the right ventricle outflow tract. The infant evolved with significant pulmonary valve regurgitation (PVR and right ventricle dysfunction. On reoperation, a cryopreserved pulmonary homograft (CPH was implanted with annuloplasty utilizing a Delrin ring with the aim of avoiding geometric distortion of the vessel. After two years, an echocardiogram proved a similar state to the immediate postoperative period with slight pulmonary valve regurgitation and normal right ventricular function suggesting that this maneuver may be used as coadjuvant treatment to optimize the result of CPH implantation.

  6. The cardiac proteome in patients with congenital ventricular septal defect: A comparative study between right atria and right ventricles.

    Science.gov (United States)

    Bond, A R; Iacobazzi, D; Abdul-Ghani, S; Ghorbel, M T; Heesom, K J; George, S J; Caputo, M; Suleiman, M-S; Tulloh, R M

    2018-03-20

    Right ventricle (RV) remodelling occurs in neonatal patients born with ventricular septal defect (VSD). The presence of a defect between the two ventricles allows for shunting of blood from the left to right side. The resulting RV hypertrophy leads to molecular remodelling which has thus far been largely investigated using right atrial (RA) tissue. In this study we used proteomic and phosphoproteomic analysis in order to determine any difference between the proteomes for RA and RV. Samples were therefore taken from the RA and RV of five infants (0.34 ± 0.05 years, mean ± SEM) with VSD who were undergoing cardiac surgery to repair the defect. Significant differences in protein expression between RV and RA were seen. 150 protein accession numbers were identified which were significantly lower in the atria, whereas none were significantly higher in the atria compared to the ventricle. 19 phosphorylation sites (representing 19 phosphoproteins) were also lower in RA. This work has identified differences in the proteome between RA and RV which reflect differences in contractile activity and metabolism. As such, caution should be used when drawing conclusions based on analysis of the RA and extrapolating to the hypertrophied RV. RV hypertrophy occurs in neonatal patients born with VSD. Very little is known about how the atria responds to RV hypertrophy, especially at the protein level. Access to tissue from age-matched groups of patients is very rare, and we are in the unique position of being able to get tissue from both the atria and ventricle during reparative surgery of these infants. Our findings will be beneficial to future research into heart chamber malformations in congenital heart defects. Copyright © 2018. Published by Elsevier B.V.

  7. Influence of hypertensive left ventricular hypertrophy on detection of ischemic area with exercise thallium-201 myocardial scintigraphy

    International Nuclear Information System (INIS)

    Toyama, Takuji; Nishimura, Tsunehiko; Uehara, Toshiisa

    1992-01-01

    Sixty-four patients with single left anterior descending artery disease having effort angina (group A: 40 patients with hypertrophic hypertension, group B: 10 patients with hypertrophic hypertension, group C: 14 patients with non-hypertrophic hypertension) were assessed to determine the influence of hypertensive left ventricular (LV) hypertrophy on detection of ischemic area. The criterion of hypertrophy by two-dimensional echocardiography was >12 mm in the wall thickness of interventricular septal or posterior wall. Population in Group B might show low detectability in ischemic area by 201 Tl myocardial scintigraphy (positive thallium rate 60%, defect score 2.7±3.6), and high lung thallium uptake and high frequence of ECG positive among three groups. In semiquantitative analysis, the washout rate of the posterolateral wall and %RD (delayed %uptake-initial %uptake) of the septal wall in patients with Group B were lowest among three groups. However, the washout rate in the septal wall against the posterior wall, and the initial %uptake and the delayed %uptake of the septal wall were not significantly different among three groups. We could conclude that the decreased washout rate in nonischemic area with hypertensive LV hypertrophy might make the ischemic area masked. (author)

  8. Eplerenone for early cardiomyopathy in Duchenne muscular dystrophy: a randomised, double-blind, placebo-controlled trial.

    Science.gov (United States)

    Raman, Subha V; Hor, Kan N; Mazur, Wojciech; Halnon, Nancy J; Kissel, John T; He, Xin; Tran, Tam; Smart, Suzanne; McCarthy, Beth; Taylor, Michael D; Jefferies, John L; Rafael-Fortney, Jill A; Lowe, Jeovanna; Roble, Sharon L; Cripe, Linda H

    2015-02-01

    Cardiomyopathy is a leading cause of death in patients with Duchenne muscular dystrophy and myocardial damage precedes decline in left ventricular systolic function. We tested the efficacy of eplerenone on top of background therapy in patients with Duchenne muscular dystrophy with early myocardial disease. In this randomised, double-blind, placebo-controlled trial, boys from three centres in the USA aged 7 years or older with Duchenne muscular dystrophy, myocardial damage by late gadolinium enhancement cardiac MRI and preserved ejection fraction received either eplerenone 25 mg or placebo orally, every other day for the first month and once daily thereafter, in addition to background clinician-directed therapy with either angiotensin-converting enzyme inhibitors (ACEI) or angiotensin receptor blockers (ARB). Computer-generated randomisation was done centrally using block sizes of four and six, and only the study statistician and the investigational pharmacy had the preset randomisation assignments. The primary outcome was change in left ventricular circumferential strain (Ecc) at 12 months, a measure of contractile dysfunction. Safety was established through serial serum potassium levels and measurement of cystatin C, a non-creatinine measure of kidney function. This trial is registered with ClinicalTrials.gov, number NCT01521546. Between Jan 26, 2012, and July 3, 2013, 188 boys were screened and 42 were enrolled. 20 were randomly assigned to receive eplerenone and 22 to receive placebo, of whom 20 in the eplerenone group and 20 in the placebo group completed baseline, 6-month, and 12-month visits. After 12 months, decline in left ventricular circumferential strain was less in those who received eplerenone than in those who received placebo (median ΔEcc 1·0 [IQR 0·3-2·2] vs 2·2 [1·3-3·1]; p=0·020). Cystatin C concentrations remained normal in both groups, and all non-haemolysed blood samples showed normal potassium concentrations. One 23-year-old patient in

  9. Stenting of right ventricular outflow tract in Tetralogy of Fallot with subarterial ventricular septal defect: A word of caution

    Directory of Open Access Journals (Sweden)

    Jonathan Lee

    2017-01-01

    Full Text Available We report a case of Tetralogy of Fallot with severe cyanosis who underwent a successful right ventricular outflow tract stenting. Follow-up echocardiography revealed moderate aortic regurgitation due to the impingement of the stent on the aortic valve. The patient underwent successful surgical correction at which time the stent was removed completely with a resolution of the aortic regurgitation.

  10. Assessment of left ventricular regional function in affected and carrier dogs with duchenne muscular dystrophy using speckle tracking echocardiography

    Directory of Open Access Journals (Sweden)

    Yugeta Naoko

    2011-05-01

    Full Text Available Abstract Background Two-dimensional speckle tracking echocardiography (STE is a relatively new method to detect regional myocardial dysfunction. To assess left ventricular (LV regional myocardial dysfunction using STE in Duchenne muscular dystrophy model dogs (CXMDJ without overt clinical signs of heart failure. Methods Six affected dogs, 8 carrier dogs with CXMDJ, and 8 control dogs were used. Conventional echocardiography, systolic and diastolic function by Doppler echocardiography, tissue Doppler imaging (TDI, and strain indices using STE, were assessed and compared among the 3 groups. Results Significant differences were seen in body weight, transmitral E wave and E' wave derived from TDI among the 3 groups. Although no significant difference was observed in any global strain indices, in segmental analysis, the peak radial strain rate during early diastole in posterior segment at chordae the tendineae level showed significant differences among the 3 groups. Conclusions The myocardial strain rate by STE served to detect the impaired cardiac diastolic function in CXMDJ without any obvious LV dilation or clinical signs. The radial strain rate may be a useful parameter to detect early myocardial impairment in CXMDJ.

  11. Evaluation of myocardial and skeletal muscular involvement with thallium-201 myocardial emission computed tomography and whole body scintigraphy

    International Nuclear Information System (INIS)

    Yamamoto, Shuhei; Matsushima, Hideo; Sotobata, Iwao; Suzuki, Akio; Indo, Toshikatsu; Matsuoka, Yukihiko

    1986-01-01

    Thallium-201 (Tl-201) myocardial emission computed tomography and whole body scintigraphy were performed using a rotating gamma camera in 64 patients with neurologic disease and 14 normal subjects. Thallium-201 myocardial perfusion defects were seen in 40 % of the muscular involvement in 47 patients with muscular dystrophy (MD), in whom morphological abnormality of the heart was common. There was strong relationship between the degree of left ventricular perfusion defects and the degree of pulmonary uptake of Tl-201. Thallium-201 whole body scintigraphy showed homogeneous distribution of Tl-201 in the extremities in normal subjects, and perfusion defects in 73 % of the muscular lesions in MD patients. Muscular and skeletal lesions for MD appear to progress independently. Thallium-201 imaging seems to be of clinical value in assessing the muscular and skeletal lesions. (Namekawa, K.)

  12. Multiplanar transesophageal echocardiography for the evaluation and percutaneous management of ostium secundum atrial septal defects in the adult.

    Science.gov (United States)

    Sobrino, Ayax; Basmadjian, Arsène J; Ducharme, Anique; Ibrahim, Reda; Mercier, Lise-Andrée; Pelletier, Guy B; Marcotte, François; Garceau, Patrick; Burelle, Denis; O'Meara, Eileen; Dore, Annie

    2012-01-01

    The purpose of this paper is to review the usefulness of multiplanar transesophageal echocardiography before, during and after percutaneous transcatheter closure of secundum atrial septal defects. Transesophageal echocardiography imaging techniques,including their role in patient selection, procedural guidance and immediate assessment of technical success and complications are described and discussed in this review. Percutaneous transcatheter closure is indicated for ostium secundum atrial septal defects of less than 40 mm in maximal diameter. The defect must have a favorable anatomy, with adequate rims of at least 5 mm to anchor the prosthesis. Transesophageal echocardiography plays a critical role before the procedure in identifying potential candidates for percutaneous closure and to exclude those with unfavorable anatomy or associated lesions, which could not be addressed percutaneously. Transesophageal echocardiography is also important during the procedure to guide the deployment of the device. After device deployment, the echocardiographer must assess the device (integrity, position and stability), residual shunt, atrio-ventricular valve regurgitation, obstruction to systemic or venous return and pericardial effusion, in order to determine procedural success and diagnose immediate complications.

  13. Nasal septum configuration as a basis for novel septal splints.

    Science.gov (United States)

    Furbish, Nina; Kühnel, Thomas S

    2017-03-01

    The objective is to use anatomical determinations of nasal septum shape and surface area in adults as a design basis for silastic septal splints of universal size and fit and offering maximum possible surface coverage. The objective is also to devise a method of securing the septal splints, so that surface pressure on septal mucosa is distributed as evenly as possible while not interfering with capillary perfusion. Nasal septum area was determined in 21 Caucasian body donors, and nasal septum thickness was measured in 20 CT scans. Septal splints of universal size and shape were prepared from silastic sheeting. The holding force of various neodymium-iron-boron (NdFeB) magnets, and the surface pressure exerted by magnet-containing septal splints was calculated. These septal splints of novel design offer a satisfactory fit in routine clinical practice. The splints can be securely attached with built-in NdFeB magnets, and surface pressure can be distributed evenly across the nasal septum while not interfering with mucosal tissue perfusion. With their simple intranasal insertion, these magnet-containing septal splints of universal size and optimised shape offer maximum possible septum coverage following septoplasty/septorhinoplasty. The absence of interference with septal tissue perfusion means that they are likely to be associated with fewer postoperative complications and better outcomes.

  14. Detection of regional derangements in myocardial metabolism by positron computed tomography in Duchenne's muscular dystrophy

    International Nuclear Information System (INIS)

    Henze, E.; Schelbert, H.R.; Perloff, J.K.; Schwaiger, M.; Phelps, M.E.

    1982-01-01

    Duchenne's Muscular Dystrophy is unique in genetically targeting for disease a specific region of myocardium: the postero-basal left ventricular wall. Postmortem examinations revealed focal fibrous degenerations in the postero-basal segment, while the coronary arteries were usually not affected. A predystrophic metabolic fault has been postulated for this region. This hypothesis was tested with positron computed tomography as a new means for the noninvasive study of regional myocadial perfusion and metabolism and to determine the incidence of regional and global left ventricular dysfunction and perfusion abnormalities using Thallium-201 and gated blood pool imaging. Myocardial perfusion was evaluated with N-13 ammonia while regional myocardial glucose uptake was studied with the glucose analog F-18 DG. The sensitivity of each diagnostic test for detecting cardiac involvement in Duchenne's Muscular Dystrophy was evaluated. It was highest for ammonia and glucose imaging and it was low for Thallium and radionuclide blood pool imaging

  15. Right ventricular dysfunction following continuous flow left ventriccular assist device placement in 51 patients: predicators and outcomes

    Directory of Open Access Journals (Sweden)

    Neragi-Miandoab Siyamek

    2012-06-01

    Full Text Available Abstract Background Right ventricular (RV dysfunction following implantation of a left ventricular assist device (LVAD is a serious condition and is associated with increased mortality. Methods The aim of the study is to investigate the significance of pre-existing RV dysfunction, tricuspid valve (TV insufficiency, and the severity of septal deviation following LVAD implantation on RV dysfunction, as well as the outcome and short-term complications in 51 patients from June 2006 to August 2010. Student t test was used to compare the data and estimate the p value. Results Mean age was 55.1 ± 13, with a male to female ratio of 3.25. The 30-day mortality was 13.7% (7/51 patients, and the overall mortality was 23.5% (12/51 patients. Meanwhile, 21 patients (21/51; 41.2% have undergone orthotopic heart transplantation. The mean time of support was 314.5±235 days with a median of 240 days at the time of closing this study. Echocardiographic evaluation of RV function pre- and post-implantation of an LVAD demonstrated septal deviation towards the left ventricle in immediate postoperative phase, which correlated with acute RV dysfunction (p = 0.002. Preoperative RV dysfunction was a significant predictor of postoperative right heart dysfunction following implantation of an LVAD (p = 0.001. Conclusion Preoperative RV dysfunction is a predictor of RV failure in LVAD patients. The adjustment of septal deviation through gradual increase of the LVAD flow can prevent the acute RV dysfunction following LVAD placement.

  16. Effect of Left Ventricular Outflow Tract Obstruction on Left Atrial Mechanics in Hypertrophic Cardiomyopathy

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    Lynne K. Williams

    2015-01-01

    Full Text Available Left atrial (LA volumes are known to be increased in hypertrophic cardiomyopathy (HCM and are a predictor of adverse outcome. In addition, LA function is impaired and is presumed to be due to left ventricular (LV diastolic dysfunction as a result of hypertrophy and myocardial fibrosis. In the current study, we assess the incremental effect of outflow tract obstruction (and concomitant mitral regurgitation on LA function as assessed by LA strain. Patients with HCM (50 obstructive, 50 nonobstructive were compared to 50 normal controls. A subset of obstructive patients who had undergone septal myectomy was also studied. Utilising feature-tracking software applied to cardiovascular magnetic resonance images, LA volumes and functional parameters were calculated. LA volumes were significantly elevated and LA ejection fraction and strain were significantly reduced in patients with HCM compared with controls and were significantly more affected in patients with obstruction. LA volumes and function were significantly improved after septal myectomy. LVOT obstruction and mitral regurgitation appear to further impair LA mechanics. Septal myectomy results in a significant reduction in LA volumes, paralleled by an improvement in function.

  17. Comparative study of interventricular phase difference and pressure gradient in cases of isolated ventricular septal defect

    Energy Technology Data Exchange (ETDEWEB)

    Elhaddad, SH; Moustafa, H; Ziada, G; Seleem, Z; Elsabban, KH; Mahmoud, F [Nuclear medicine department and pediatric cardiology department Faculty of medicine, Cairo university, Cairo, (Egypt)

    1995-10-01

    One hundred and fifty patients with isolated VSD were evaluated by radionuclide MUGA study and Echo-Doppler. Difference between phase angle of the right and left ventricles as detected by MUGA had been divided into main four groups according to pressure gradient between the two ventricles : group I (with pressure gradient {<=}30 mmHg and phase difference 80.10 degree{+-}34.1), group III (with pressure gradient > 70 mmHg and phase difference -0.5 degree {+-} 8.4). It has been found that there was a significant difference between the 4 groups as regards right - to - left ventricular phase difference (P<0.0001). There was significant delay in emptying of right ventricle in groups with pressure gradient < 50 mmHg. Regression analysis revealed inverse correlation between right -to- left ventricular phase difference with changes in pressure gradient (r= 0.81). Similarly, significant correlation had been found between right -to-left ventricular phase difference in relation Qp/Qs (r=0.85); conclusion: interventricular phase difference can be used to evaluate interventricular pressure gradient in cases of isolated VSD. 4 figs., 2 tabs.

  18. Small atrial septal defect associated with heart failure in an infant with a marginal left ventricle

    Directory of Open Access Journals (Sweden)

    Sandra D.K. Kingma

    2012-07-01

    Full Text Available Atrial septal defect (ASD is usually asymptomatic in infancy, unless pulmonary hypertension or severe co-morbidity is present. We report a case of a 4-week-old infant with moderate- sized ASD, small patent ductus arteriosus (PDA, and a borderline sized left ventricle that developed heart failure. Despite the relatively small diameter of the ASD, this defect influenced the mechanism of heart failure significantly. After surgical closure of both PDA and ASD, the signs of pulmonary hypertension resolved and the patient developed a normal sized left ventricle. This report illustrates that the presence of a small ASD in combination with a marginal left ventricle may result in inadequate left ventricular filling, pulmonary hypertension and heart failure.

  19. Left Ventricular Function in Patients with Pulmonary Arterial Hypertension: The Role of Two-Dimensional Speckle Tracking Strain.

    Science.gov (United States)

    de Amorim Corrêa, Ricardo; de Oliveira, Fernanda Brito; Barbosa, Marcia M; Barbosa, Jose Augusto A; Carvalho, Taís Soares; Barreto, Michele Campos; Campos, Frederico Thadeu A F; Nunes, Maria Carmo Pereira

    2016-09-01

    Pulmonary arterial hypertension (PAH) is characterized by elevated mean pulmonary arterial pressure with abnormal right ventricular (RV) pressure overload that may alter left ventricular (LV) function. The aim of this study was to assess the impact of RV pressure overload on LV function in PAH patients using two-dimensional (2D) speckle tracking strain. The study enrolled 37 group 1 PAH patients and 38 age- and gender-matched healthy controls. LV longitudinal and radial 2D strains were measured with and without including the ventricular septum. Six-minute walk test (6MWT) and brain natriuretic peptide (BNP) levels were also obtained in patients with PAH. The mean age of patients was 46.4 ± 14.8 years, 76% women, and 16 patients (43%) had schistosomiasis. Sixteen patients (43%) were in WHO class III or IV under specific treatment for PAH. The overall 6MWT distance was 441 meters, and the BNP levels were 80 pg/mL. Patients with PAH more commonly presented with LV diastolic dysfunction and impairment of RV function when compared to controls. LV global longitudinal and radial strains were lower in patients than in controls (-17.9 ± 2.8 vs. -20.5 ± 1.9; P < 0.001 and 30.8 ± 10.5 vs. 49.8 ± 15.4; P < 0.001, respectively). After excluding septal values, LV longitudinal and radial strains remained lower in patients than in controls. The independent factors associated with global LV longitudinal strain were LV ejection fraction, RV fractional area change, and tricuspid annular systolic motion. This study showed impaired LV contractility in patients with PAH assessed by speckle tracking strain, irrespective of ventricular septal involvement. Global LV longitudinal strain was associated independently with RV fractional area change and tricuspid annular systolic motion, after adjustment for LV ejection fraction. © 2016, Wiley Periodicals, Inc.

  20. Increased pulmonary artery pressures during exercise are related to persistent tricuspid regurgitation after atrial septal defect closure.

    Science.gov (United States)

    De Meester, Pieter; Van De Bruaene, Alexander; Herijgers, Paul; Voigt, Jens-Uwe; Vanhees, Luc; Budts, Werner

    2013-08-01

    Although closure of an atrial septal defect type secundum often normalizes right heart dimensions and pressures, mild tricuspid insufficiency might persist. This study aimed at (1) identification of determinants explaining the persistence of tricuspid insufficiency after atrial septal defect closure, and (2) evaluation of functional capacity of patients with persistent mild tricuspid insufficiency. Twenty-five consecutive patients (age 42+17 y) were included from the outpatient clinic of congenital heart disease at the University Hospitals of Leuven. All underwent transthoracic echocardiography, semi-supine bicycle stress echocardiography and cardio-pulmonary exercise testing. Six patients (24%) had mild tricuspid insufficiency (2/4) compared to 19 patients (76%) with no or minimal tricuspid insufficiency ( 1/4) as assessed by semi-quantitative colour Doppler echocardiography. Mann-Whitney U and Fisher's exact tests were performed where applicable. Patients with persistent mild tricuspid insufficiency were significantly older than those with no or minimal tricuspid insufficiency (P = 0.042). At rest, no differences in right heart configuration, mean pulmonary artery pressure or right ventricular function were found. At peak exercise, mean pulmonary artery pressure was significantly higher in patients with mild persistent tricuspid insufficiency (P = 0.026). Peak oxygen uptake was significantly lower in patients with mild persistent tricuspid insufficiency (P = 0.019). Mild tricuspid insufficiency after atrial septal defect repair occurs more frequently in older patients and in patients with higher mean pulmonary artery pressure at peak exercise. In patients with mild tricuspid insufficiency, functional capacity was more reduced. Mild tricuspid insufficiency could be a marker of subclinical persistent pressure load on the right ventricle.

  1. Diagnosis of atrial septal defect using magnetic resonance imaging

    International Nuclear Information System (INIS)

    Sakakibara, Makoto; Kobayashi, Shiro; Imai, Hitoshi; Watanabe, Shigeru; Masuda, Yoshiaki; Inagaki, Yoshiaki

    1987-01-01

    The capability of magnetic resonance imaging (MRI) to detect atrial septal defect (ASD) was evaluated in 28 ASD patients of diagnostic confirmation, including five with complicated anomaly. Findings of MRI obtained from 7 normal volunteers and 142 patients with acquired heart diseases were used as controls. Non-gated MRI, performed in three ASD patients, failed to reveal defect areas ; gated MRI, in the other 25 patients, depicted defects in the interatrial septum corresponding to findings of surgery and cardiac catheterizatioin, which allowed the site and size of ASD to be determined. Other findings of MRI included right atrial dilatation, right ventricular hypertrophy and dilatation, and pulmonary artery dilatation in majority of ASD patients. Complex anomalies associated with ADS were also clearly shown on MRI. The interatrial septum was shown on gated-MRI in 124 control subjects, 17 of whom had an extremely faint signal from the central portion of the interatrial septum. The results indicate that gated MRI is a valuable noninvasive method in the diagnosis of ASD and complicating anomalies. (Namekawa, K.)

  2. Long-term outcomes of septal reduction for obstructive hypertrophic cardiomyopathy.

    Science.gov (United States)

    Sedehi, Daniel; Finocchiaro, Gherardo; Tibayan, Yen; Chi, Jeffrey; Pavlovic, Aleksandra; Kim, Young M; Tibayan, Frederick A; Reitz, Bruce A; Robbins, Robert C; Woo, Joseph; Ha, Richard; Lee, David P; Ashley, Euan A

    2015-07-01

    Surgical myectomy and alcohol septal ablation (ASA) aim to decrease left ventricular outflow tract (LVOT) gradient in hypertrophic cardiomyopathy (HCM). Outcome of myectomy beyond 10 years has rarely been described. We describe 20 years of follow-up of surgical myectomy and 5 years of follow-up for ASA performed for obstructive HCM. We studied 171 patients who underwent myectomy for symptomatic LVOT obstruction between 1972 and 2006. In addition, we studied 52 patients who underwent ASA for the same indication and who declined surgery. Follow-up of New York Heart Association (NYHA) functional class, echocardiographic data, and vital status were obtained from patient records. Mortality rates were compared with expected mortality rates of age- and sex-matched populations. Surgical myectomy improved NYHA class (2.74±0.65 to 1.54±0.74, phistorical data from non-operated HCM patients. Survival after ASA at 2 and 5 years was 97.8% and 94.7%, respectively. Short-term (5 year) survival after ASA (SMR=0.61, p=0.48) was comparable to that of the general population. Long-term follow-up of septal reduction strategies in obstructive HCM reveals that surgical myectomy and ASA are effective for symptom relief and LVOT gradient reduction and are associated with favorable survival. While overall prognosis for the community HCM population is similar to the general population, the need for surgical myectomy may identify a sub-group with poorer long-term prognosis. We await long-term outcomes of more extensive myectomy approaches adopted in the past 10 years at major institutions. Copyright © 2015. Published by Elsevier Ltd.

  3. Mitral valve replacement complicated by iatrogenic left ventricular outflow obstruction and paravalvular leak: case report and review of literature.

    Science.gov (United States)

    Lee, Justin Z; Tey, Kai R; Mizyed, Ahmad; Hennemeyer, Charles T; Janardhanan, Rajesh; Lotun, Kapildeo

    2015-10-09

    Left ventricular outflow tract (LVOT) obstruction and paravalvular leak (PVL) are relatively uncommon, but are serious complications of prosthetic valve replacement. We present a case that displays the unique therapeutic challenges of treating a patient who developed both LVOT obstruction and mitral PVL after undergoing surgical aortic and mitral valve replacement (MVR). We also describe the use of alcohol septal ablation and albumin-glutaraldehyde (BioGlue) for septal ablation to percutaneously treat the patient's LVOT obstruction, followed by use of an Amplatzer vascular plug for percutaneous closure of an antero-medial mitral PVL associated with severe regurgitation. Percutaneous interventional management of these entities may be considered as an initial therapeutic option, especially in high-risk patients with significant morbidity and mortality of repeat surgical operations.

  4. Removing intranasal splints after septal surgery.

    Science.gov (United States)

    Aksoy, Elif; Serin, Gediz Murat; Polat, Senol; Kaytaz, Asm

    2011-05-01

    The aim of this retrospective chart review of the patients who had septal surgery with or without turbinate surgery was to compare the postoperative complication rates according to the time of intranasal-splint (INS) removal. The data of 137 patients who underwent septal surgery with or without turbinate surgery at 2 different hospitals of Acıbadem Health Care Group between January 2007 and March 2009 were retrospectively evaluated. The patients who had these risk factors were eliminated, and 96 patients were included in this study. The patients were divided into 2 groups according to splint-removal time. The first group comprises patients whose nasal splints were removed in 24 hours after surgery, and the second group comprises patients whose splints were removed 5 days after the surgery. Any bleeding, septal hematoma, and synechia after pack removal were recorded. Analysis of the rate of complications was done with the χ test. Sixty-five male and 31 female patients with a mean age of 32.4 years (range, 18-57 years) were included in the study groups. Septal surgeries were performed in association with turbinate surgery in all 96 patients. These patients were divided into 2 groups. In the first group (n = 50), INSs were removed in 24 hours after surgery. In the second group (n = 46), INSs were removed 5 days after surgery. Bleeding within the first postoperative week was not recorded in both groups. Late bleeding was recorded in 2% (n = 1) of group 1 and in 2.17% (n = 1) in group 2. Septal hematoma and synechia were not recorded in none of the groups. The results were not statistically significant (P = 1). The routine use of INSs after septoplasty and removing them 24 hours after septoplasty are sufficient to avoid postoperative complications, and it minimizes postoperative discomfort.

  5. Differential diagnosis of left ventricular hypertrophy: usefulness of multimodality imaging and tissue characterization with cardiac magnetic resonance.

    Science.gov (United States)

    Izgi, Cemil; Vassiliou, Vassilis; Baksi, A John; Prasad, Sanjay K

    2016-11-01

    Differential diagnosis of asymmetrical left ventricular hypertrophy may be challenging, particularly in patients with history of hypertension. A middle-aged man underwent an echocardiographic examination during workup for hypertension, which unexpectedly showed significant asymmetrical septal hypertrophy and raised suspicion for hypertrophic cardiomyopathy. Cardiovascular magnetic resonance confirmed the asymmetrical hypertrophy. No myocardial late gadolinium contrast enhancement was seen. However, precontrast T1 mapping revealed a low native myocardial T1 value. This was highly suggestive of Anderson-Fabry disease, which was subsequently proved with very low alpha galactosidase enzyme levels and mutation analysis. The case illustrates clinical usefulness of multimodality imaging and the novel tissue characterization techniques for assessment of left ventricular hypertrophy. © 2016, Wiley Periodicals, Inc.

  6. Detection and quantification by deformation imaging of the functional impact of septal compared to free wall preexcitation in the Wolff-Parkinson-White syndrome.

    Science.gov (United States)

    De Boeck, Bart W L; Teske, Arco J; Leenders, Geert E; Mohamed Hoesein, Firdaus A A; Loh, Peter; van Driel, Vincent J; Doevendans, Pieter A; Prinzen, Frits W; Cramer, Maarten J

    2010-08-15

    Pacing experiments in healthy animal hearts have suggested a larger detrimental effect of septal compared to free wall preexcitation. We investigated the intrinsic relation among the site of electrical preexcitation, mechanical dyssynchrony, and dysfunction in human patients. In 33 patients with Wolff-Parkinson-White (WPW) syndrome and 18 controls, regional myocardial deformation was assessed by speckle tracking mapping (ST-Map) to assess the preexcitation site, shortening sequences and dyssynchrony, and the extent of local and global ejecting shortening. The ST-Map data in patients with accessory atrioventricular pathways correctly diagnosed as located in the interventricular septum (IVS) (n = 11) or left ventricular free wall (LFW) (n = 12) were compared to the corresponding control values. A local ejecting shortening of <2 SD of the control values identified hypokinetic segments. The localization of the atrioventricular pathways by ST-Map matched with the invasive electrophysiology findings in 23 of 33 patients and was one segment different in 5 of 33 patients. In both WPW-IVS and WPW-LFW, local ejecting shortening was impaired at the preexcitation site (p <0.01). However, at similar electrical and mechanical dyssynchrony, WPW-IVS had more extensive hypokinesia than did WPW-LFW (3.6 +/- 0.9 vs 1.8 +/- 1.3 segments, p <0.01). Compared to controls, the left ventricular function was significantly reduced only in WPW-IVS (global ejecting shortening 17 +/- 2% vs 19 +/- 2%, p = 0.01; ejection fraction 55 +/- 5% vs 59 +/- 3%, p = 0.02). In conclusion, preexcitation is associated with local hypokinesia, which at comparable preexcitation is more extensive in WPW-IVS than in WPW-LFW and could adversely affect ventricular function. ST-Map might have a future role in detecting and guiding treatment of septal pathways with significant mechanical effects.

  7. Cardiac involvement of progressive muscular dystrophy (Becker type, Limb-girdle type and Fukuyama type) evaluated by radionuclide method

    International Nuclear Information System (INIS)

    Nagamachi, Shigeki; Inoue, Kenjiro; Jinnouchi, Seishi; Hoshi, Hiroaki; Ono, Seiji; Ohnishi, Takashi; Futami, Shigemi; Watanabe, Katsushi; Hayashi, Tohru

    1994-01-01

    Tl-201 SPECT and Tc-99m-Human serum albumin (HSA) multigated radionuclide ventriculography were performed on 11 patients with progressive muscular dystrophy (Becker type 2, Fukuyama type 2, Limb-girdle type 7) to evaluate myocardial involvement. Hypoperfusion was detected in 8 patients on Tl-201 SPECT. Decreases in both systolic function (left ventricular ejection fraction; LVEF) and diastolic function (peak filling rate; PFR) were also seen in these patients. A high incidence of myocardial involvement of these kinds of progressive muscular dystrophy was suggested. (author)

  8. Cardiac involvement of progressive muscular dystrophy (Becker type, Limb-girdle type and Fukuyama type) evaluated by radionuclide method

    Energy Technology Data Exchange (ETDEWEB)

    Nagamachi, Shigeki; Inoue, Kenjiro; Jinnouchi, Seishi; Hoshi, Hiroaki; Ono, Seiji; Ohnishi, Takashi; Futami, Shigemi; Watanabe, Katsushi; Hayashi, Tohru [Miyazaki Medical Coll., Kiyotake (Japan)

    1994-02-01

    Tl-201 SPECT and Tc-99m-Human serum albumin (HSA) multigated radionuclide ventriculography were performed on 11 patients with progressive muscular dystrophy (Becker type 2, Fukuyama type 2, Limb-girdle type 7) to evaluate myocardial involvement. Hypoperfusion was detected in 8 patients on Tl-201 SPECT. Decreases in both systolic function (left ventricular ejection fraction; LVEF) and diastolic function (peak filling rate; PFR) were also seen in these patients. A high incidence of myocardial involvement of these kinds of progressive muscular dystrophy was suggested. (author).

  9. Right ventricular bifocal stimulation in the treatment of dilated cardiomyopathy with heart failure

    Directory of Open Access Journals (Sweden)

    José Carlos Pachón Mateos

    1999-12-01

    Full Text Available OBJECTIVE: To describe a new more efficient method of endocardial cardiac stimulation, which produces a narrower QRS without using the coronary sinus or cardiac veins. METHODS: We studied 5 patients with severe dilated cardiomyopathy, chronic atrial fibrillation and AV block, who underwent definitive endocardial pacemaker implantation, with 2 leads, in the RV, one in the apex and the other in the interventricular septum (sub pulmonary, connected, respectively, to ventricular and atrial bicameral pacemaker outputs. Using Doppler echocardiography, we compared, in the same patient, conventional (VVI, high septal ("AAI" and bifocal ("DDT" with AV interval ~ 0 stimulation. RESULTS: The RV bifocal stimulation had the best results with an increase in ejection fraction and cardiac output and reduction in QRS duration, mitral regurgitation and in the left atrium area (p <= 0.01. The conventional method of stimulation showed the worst result. CONCLUSION: These results suggest that, when left ventricular stimulation is not possible, right ventricular bifocal stimulation should be used in patients with severe cardiomyopathy where a pacemaker is indicated.

  10. Maternal alcohol drinking pattern during pregnancy and the risk for an offspring with an isolated congenital heart defect and in particular a ventricular septal defect or an atrial septal defect

    DEFF Research Database (Denmark)

    Strandberg-Larsen, Katrine; Skov-Ettrup, Lise Skrubbeltrang; Grønbaek, Morten

    2011-01-01

    (VSD) or of an atrial septal defect (ASD). METHODS: Participants were 80,346 pregnant women who were enrolled into the Danish National Birth Cohort in 1996-2002 and gave birth to a live-born singleton without any chromosome anomalies. Twice during pregnancy these women were asked about their intake...... the early part of pregnancy was not statistical significantly associated with the prevalence of isolated VSD and ASD in offspring. Birth Defects Research (Part A), 2011. © 2011 Wiley-Liss, Inc....

  11. Thallium-201 myocardial imaging in children with heart disease. Quantitative assessment of right ventricular pressure

    Energy Technology Data Exchange (ETDEWEB)

    Mitomori, Toshihiro; Ono, Yasuo; Kamiya, Tetsuro; Nishimura, Tsunehiko; Kozuka, Takahiro (National Cardiovascular Center, Suita, Osaka (Japan)); Kohata, Tohru; Tanimoto, Takeshi

    1985-02-01

    201-TlCl myocardial imaging studies were performed to evaluate systolic pressure of right ventricle on 107 patients including 89 patients with congenital cardiac disease, patients with primary pulmonary hypertension and 13 patients with history of MCLS with the age range of 2 months to 17 years. The congenital cardiac disease group included 30 patients with tetralogy of Fallot, 8 patients with pulmonary stenosis (included 1 patient with double chambered right ventricle), 20 patients with ventricular septal defect, 10 patients with patent ductus arteriosus, 9 patients with atrial septal defect and 12 patients with complete transposition of great arteries. None of the patients with history of MCLS had coronary involvement. 50 ..mu..Ci/kg of 201-TlCl was infused intravenously and after 15 minutes the images were obtained from 5 directions (anterior, LAO 30, LAO 45, LAO 60 and lateral). The angle was determined to demonstrate the intraventricular septum and ventricular free walls cleary separated. The image of end-diastolic phase was obtained with ECG synchronized gated method from that direction. The ROI (region of interest) was defined as a slice line by drawing two lines perpendicular to the septum and the counts of the left and right ventricular free wall (Cl and Cr) were analyzed to evaluate the pressure of right ventricle. The pressure of ventricles were obtained by cardiac catheterization performed at the same time with myocardial imaging. The ratio of Cl and Cr (Cr/Cl) had good correlation with the ratio of peak systolic pressure of left ventricle and right ventricle (regression equation Y=0.84X + 0.08, r=0.87). In each congenital heart disease group, very good correlation was also demonstrated (Y=1.46X - 0.30, r=0.96 in PS; Y=0.70X0.20, r=0.91 in VSD; Y=0.86X + 0.15, r=0.78 in ASD and Y=0.72X + 0.24, r=0.80 in PDA). Thus 201-TlCl myocardial imaging was useful method to evaluate right ventricular pressure noninvasively in congenital cardiac disease.

  12. Pattern of left ventricular hypertrophy seen on transthoracic echo in patients with hypertensive cardiomyopathy when compared with idiopathic hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Mirza, S. J.; Radaideh, G. A.

    2013-01-01

    Objective: To explore the pattern of left ventricular hypertrophy caused by hypertension and to compare it with idiopathic hypertrophic cardiomyopathy. Methods: The retrospective study was conducted at the echocardiography lab of Rashid Hospital, Dubai, from January 2009 to January 2010. Cases of 11 patients with significant left ventricular hypertrophy (septum >15mm) due to underlying hypertension were analysed and compared with 11 cases of idiopathic hypertrophic cardiography (septum >15mm) to assess the two groups with similar baseline echocardiographic features. Minitab software was used for statistical analysis. Results: Although the pattern of hypertrophy in hypertensive patients was more concentric (n=5; 45%), there was also asymmetrical septal hypertrophy in 4 (36%) cases, particularly the elderly with sigmoid shape septum. There was evidence of resting mid-cavity gradient due to reduced left ventricular end-systolic diameter in 4 (36%) cases. Conclusion: Although the equation between hypertension and left ventricular hypertrophy is more concentric, but it can be associated with left ventricular outflow tract obstruction and significant mid-cavity gradients similar to that seen in idiopathic hypertrophic cardiomyopathy. (author)

  13. Radionuclide stroke count ratios for assessment of right and left ventricular volume overload in children

    International Nuclear Information System (INIS)

    Parrish, M.D.; Graham, T.P. Jr.; Born, M.L.; Jones, J.P.; Boucek, R.J. Jr.; Artman, M.; Partain, C.L.

    1983-01-01

    The ratio of left ventricular to right ventricular stroke counts measured by radionuclide angiography has been used in adults to estimate the severity of left-sided valvular regurgitation. The validation of this technique in children for assessment of right and left ventricular volume overload is reported herein. Radionuclide stroke count ratios in 60 children aged 0.5 to 19 years (mean 11) were determined. Based on their diagnoses, the patients were divided into 3 groups: (1) normal--40 patients with no shunts or valvular regurgitation, (2) left ventricular volume overload--13 patients with mitral or aortic regurgitation, or both, and (3) right ventricular volume overload--7 patients, 2 with severe tricuspid regurgitation, 3 with atrial septal defects, and 2 with total anomalous pulmonary venous drainage. The radionuclide stroke count ratio clearly differentiated these groups (p less than 0.05): normal patients had a stroke count ratio of 1.04 +/- 0.17 (mean +/- 1 standard deviation), the left ventricular volume overload group had a stroke count ratio of 2.43 +/- 0.86, and the right ventricular volume overload group had a stroke count ratio of 0.44 +/- 0.17. In 22 of our 60 patients, radionuclide stroke count ratios were compared with cineangiographic stroke volume ratios, resulting in a correlation coefficient of 0.88. It is concluded that radionuclide ventriculography is an excellent tool for qualitative and quantitative assessment of valvular regurgitation in children

  14. Sensitivity and specificity of radionuclide equilibrium angiocardiography for detection of hemodynamically significant secundum atrial septal defect

    Energy Technology Data Exchange (ETDEWEB)

    Brunotte, F.; Laurens, M.H.; Itty, C.; Robert, J.; Cloez, J.L.; Marcon, F.; Pernot, C.

    1986-12-01

    To determine the value, of gated equilibrium angiography in secundum atrial septal defect (ASD) in children, the first pass pulmonic/systemic flow ratio (Qp/Qs) was compared with diastolic count ratio (DCR) and stroke count ratio (SCR) of the two ventricles. In 50 children we have found a correlation between Qp/Qs and DCR (r=0.71) and between Qp/Qs and SCR (r=0.66). For detection of significant atrial shunt (Qp/Qs>1.5) the sensitivity of DCR>2 was 0.81 and the specificity 0.75. For SCR>1.5 we sensitivity and specificity values of 0.87 and 0.71 respectively. Left and right ventricular ejection fractions were normal (0.67+-0.08 and 0.50+-0.07).

  15. Zero net flux estimates of septal extracellular glucose levels and the effects of glucose on septal extracellular GABA levels

    OpenAIRE

    Krebs-Kraft, Desiree L.; Rauw, Gail; Baker, Glen B.; Parent, Marise B.

    2009-01-01

    Although hippocampal infusions of glucose enhance memory, we have found repeatedly that septal glucose infusions impair memory when γ-aminobutyric acid (GABA) receptors are activated. For instance, hippocampal glucose infusions reverse the memory-impairing effects of co-infusions of the GABA agonist muscimol, whereas septal glucose infusions exacerbate memory deficits produced by muscimol. One potential explanation for these deleterious effects of glucose in the septum is that there are highe...

  16. Phase image characterization of ventricular contraction in left anterior hemiblock

    International Nuclear Information System (INIS)

    Ono, Akifumi; Mizuno, Haruyoshi; Tahara, Yorio; Ishikawa, Kyozo

    1991-01-01

    We investigated whether or not left anterior hemiblock is present in patients with left axis deviation using first-harmonic Fourier analysis of gated blood-pool images. Gated blood-pool images were taken in 50 patients without contraction abnormality. They included 14 normal subjects, 8 patients with right bundle branch block (RBBB), 20 with left axis deviation (LAD) and 8 with both RBBB and LAD (RBBB+LAD). ECG gated blood-pool scans were acquired in the anterior and 'best septal' left anterior oblique projections. First, the phase images were displayed cinematically as a continuous-loop movie. Next, for quantitative analysis of the phase image, the whole left ventricular and left ventricular high lateral regions of interest were drawn. The 'regional phase shift' (RPS) was then defined as {RPS=A-a} where 'A' is the mean value of the whole left ventricular phase angles and 'a' is that of phase angles in the high lateral region. The left ventricular phase changes and the RPSs in the RBBB and LAD groups were similar to those in the normal group. In the RBBB+LAD group, the latest phase changes occurred in the high anterolateral region. The RPSs of this group were significantly lower than those in the other 3 groups (p<0.01). These data suggest that left anterior hemiblock might coexist with RBBB in patients with RBBB+LAD, whereas left anterior hemiblock might not exist in the majority of patients with LAD alone. (author)

  17. Molecular Diffusion through Cyanobacterial Septal Junctions.

    Science.gov (United States)

    Nieves-Morión, Mercedes; Mullineaux, Conrad W; Flores, Enrique

    2017-01-03

    Heterocyst-forming cyanobacteria grow as filaments in which intercellular molecular exchange takes place. During the differentiation of N 2 -fixing heterocysts, regulators are transferred between cells. In the diazotrophic filament, vegetative cells that fix CO 2 through oxygenic photosynthesis provide the heterocysts with reduced carbon and heterocysts provide the vegetative cells with fixed nitrogen. Intercellular molecular transfer has been traced with fluorescent markers, including calcein, 5-carboxyfluorescein, and the sucrose analogue esculin, which are observed to move down their concentration gradient. In this work, we used fluorescence recovery after photobleaching (FRAP) assays in the model heterocyst-forming cyanobacterium Anabaena sp. strain PCC 7120 to measure the temperature dependence of intercellular transfer of fluorescent markers. We find that the transfer rate constants are directly proportional to the absolute temperature. This indicates that the "septal junctions" (formerly known as "microplasmodesmata") linking the cells in the filament allow molecular exchange by simple diffusion, without any activated intermediate state. This constitutes a novel mechanism for molecular transfer across the bacterial cytoplasmic membrane, in addition to previously characterized mechanisms for active transport and facilitated diffusion. Cyanobacterial septal junctions are functionally analogous to the gap junctions of metazoans. Although bacteria are frequently considered just as unicellular organisms, there are bacteria that behave as true multicellular organisms. The heterocyst-forming cyanobacteria grow as filaments in which cells communicate. Intercellular molecular exchange is thought to be mediated by septal junctions. Here, we show that intercellular transfer of fluorescent markers in the cyanobacterial filament has the physical properties of simple diffusion. Thus, cyanobacterial septal junctions are functionally analogous to metazoan gap junctions

  18. Assessment of left ventricular function using 201Tl electrocardiogram-gated myocardial single photon emission computed tomography

    International Nuclear Information System (INIS)

    Nishikubo, Naotsugu; Tamai, Hiroyuki

    2013-01-01

    Advances in computed tomography (CT) technology make it possible to obtain left ventricular wall motion using 3D reconstruction. In this study, we compared the images obtained from CT and 201 Tl electrocardiogram (ECG) gated single photon emission computed tomography (SPECT). In 20 patients with ischemic heart disease, we performed 201 Tl ECG gated SPECT (GE Healthcare Millennium VG) and ECG gated CT (Philips Medical Systems Brilliance iCT) to evaluate of left ventricular wall motion during the resting phase. In SPECT, left ventricular images were reconstructed using quantitative gated SPECT (QGS) software. In CT, the images were reconstructed using Virtual Place (AZE Software). The left ventricle was classified into five regions (anterior, lateral, inferior, septal, and apical). The amplitude of the wall motion was classified into five grades according to AHA classification. The values of the wall motion were separately checked by two radiographers. Assessment of left ventricular function myocardial wall movement using the three-dimensional movie display with ECG gated myocardial SPECT data was in agreement with the evaluation by cardiac CT inspection, and corresponded with wall motion in 88 of all 100 segments. SPECT analysis has the same quantity as that of obtained from CT for evaluation of left ventricular wall motion. (author)

  19. Radiographic and ultrasonographic features of hypertrophic feline muscular dystrophy in two cats

    International Nuclear Information System (INIS)

    Berry, C.R.; Gaschen, F.P.; Ackerman, N.

    1992-01-01

    Hypertrophic fellne musculer dystrophy has been reported as an X-linked inherited deficiency of a cytoskeletal myofiber protein called dystrophin. This report deserlbes the radiographic and ultrasonographic abnormalities of two male littermate domestic short-hair cats and reviews the previous reported findings assoclated with hypertrophic feline muscular dystrophy. The thoracic radiographic abnormalities included: progressive cardiomegaly, large convex, scalloped irregularities associated with the vetral aspect of the diaphragm, and variable degrees of esophageal dilation (megaesophagus) with associated cranioventral aspiration pneumonia. Echocardiographic features included: concentric left vetricular wall thickening, increased left ventricular and diastolic and systolic dimensions, and an increase in endocardial echogenicity. Abdominal radiographic abnormalities included: hepatosplenomegaly, peritoneal effusion, renomegaly, adrenal gland mineralization, and paralumbar and diaphragmatic musculature enlargement. Abdomlnal ultrasonographic abnormalities included: irregularly thickened muscular portion of the diaphragm; hypoechogenicity of the liver; peritoneal effusion; hepatosplenomegaly; renomegaly with hyperechoic cortex and medulla; and adrenal gland mineralization. The irregular scalloped appearance of the diaphragm (particularly along the ventral/sternal margin) was a consistenl radiographic abnormlity in the two cats with hypertrophic feline muscular dystrophy after the age of 7 months. This finding was confirmed by ultrasound as a thickened irregular, hyperechoic diaphragm. A diagnosis of hypertrophic feline muscular dystrophy should be strongly suspected if this abnormality is identified

  20. Cost-Effectiveness of Ventricular Assist Device Destination Therapy for Advanced Heart Failure in Duchenne Muscular Dystrophy.

    Science.gov (United States)

    Magnetta, Defne A; Kang, JaHyun; Wearden, Peter D; Smith, Kenneth J; Feingold, Brian

    2018-05-17

    Destination ventricular assist device therapy (DT-VAD) is well accepted in select adults with medically refractory heart failure (HF) who are not transplant candidates; however, its use in younger patients with progressive diseases is unclear. We sought to evaluate the cost-effectiveness of DT-VAD in Duchenne muscular dystrophy (DMD) patients with advanced HF. We created a Markov-state transition model (5-year horizon) to compare survival, costs, and quality of life (QOL) between medical management and DT-VAD in DMD with advanced HF. Model input parameters were derived from the literature. We used sensitivity analyses to explore uncertainty around model assumptions. DT-VAD had higher costs ($435,602 vs. $125,696), survival (3.13 vs. 0.60 years), and quality-adjusted survival (1.99 vs. 0.26 years) than medical management. The incremental cost-effectiveness ratio (ICER) for DT-VAD was $179,086 per quality-adjusted life year (QALY). In sensitivity analyses that were widely varied to account for uncertainty in model assumptions, the DT-VAD strategy generally remained more costly and effective than medical management. Only when VAD implantation costs were <$113,142 did the DT-VAD strategy fall below the $100,000/QALY willingness-to-pay threshold commonly considered to be "cost-effective." In this exploratory analysis, DT-VAD for patients with DMD and advanced HF exceeded societal expectations for cost-effectiveness but had an ICER similar to the accepted practice of DT-VAD in adult HF patients. While more experience and research in this population is needed, our analysis suggests that DT-VAD for advanced HF in DMD should not be dismissed solely based on cost.

  1. Clinical and echocardiographic features of primary infundibular stenosis with intact ventricular septum in dogs.

    Science.gov (United States)

    Minors, Sandra L; O'Grady, Michael R; Williams, Regan M; O'Sullivan, M Lynne

    2006-01-01

    Primary infundibular stenosis is a rare congenital defect in which the right ventricle is divided into a proximal "high-pressure" chamber and a distal "low-pressure" chamber. The condition can be misdiagnosed as ventricular septal defect or valvular pulmonic stenosis and the disease severity underestimated. The purpose of this study was to provide a detailed clinical and echocardiographic description of this anomaly in a series of dogs. Several anatomic forms of infundibular stenosis exist. High resolution two-dimensional echocardiography could differentiate 3 gross anatomic substrates. Knowledge of the anatomy of the obstructing lesion could influence options for corrective interventions. Thirteen dogs examined at the Ontario Veterinary College teaching hospital from 1994 to 2005 with an ultrasound diagnosis of subpulmonic stenasis. A retrospective review was made of case records from 1994 to 2005. Thirteen dogs were identified as having primary infundibular stenosis, with apparent increased prevalence in Golden Retrievers (8/13, 62%) and Siberian Huskies (3/13, 23%). Three types of infundibular lesions were identified by ultrasound in 11/13 dogs: a fibrous diaphragm (6), fibromuscular (4), and muscular obstruction (1). Two dogs with a fibrous diaphragm underwent direct surgical dilation without the use of cardiopulmonary bypass or inflow occlusion, resulting in substantial reduction of the severity of stenosis. Accurate determination of the severity of the stenosis and the anatomy of the obstructing lesion are important in devising a treatment strategy. Recognition of the fibrous diaphragm by echocardiography identifies a subset of dogs potentially amenable to surgical dilation without the need for cardiopulmonary bypass.

  2. Cost-effectiveness of procedures for treatment of ostium secundum atrial septal defects occlusion comparing conventional surgery and septal percutaneous implant.

    Science.gov (United States)

    da Costa, Márcia Gisele Santos; Santos, Marisa da Silva; Sarti, Flávia Mori; Simões e Senna, Kátia Marie; Tura, Bernardo Rangel; Correia, Marcelo Goulart; Goulart, Marcelo Correia

    2014-01-01

    The study performs a cost-effectiveness analysis of procedures for atrial septal defects occlusion, comparing conventional surgery to septal percutaneous implant. A model of analytical decision was structured with symmetric branches to estimate cost-effectiveness ratio between the procedures. The decision tree model was based on evidences gathered through meta-analysis of literature, and validated by a panel of specialists. The lower number of surgical procedures performed for atrial septal defects occlusion at each branch was considered as the effectiveness outcome. Direct medical costs and probabilities for each event were inserted in the model using data available from Brazilian public sector database system and information extracted from the literature review, using micro-costing technique. Sensitivity analysis included price variations of percutaneous implant. The results obtained from the decision model demonstrated that the percutaneous implant was more cost effective in cost-effectiveness analysis at a cost of US$8,936.34 with a reduction in the probability of surgery occurrence in 93% of the cases. Probability of atrial septal communication occlusion and cost of the implant are the determinant factors of cost-effectiveness ratio. The proposal of a decision model seeks to fill a void in the academic literature. The decision model proposed includes the outcomes that present major impact in relation to the overall costs of the procedure. The atrial septal defects occlusion using percutaneous implant reduces the physical and psychological distress to the patients in relation to the conventional surgery, which represent intangible costs in the context of economic evaluation.

  3. Cost-effectiveness of procedures for treatment of ostium secundum atrial septal defects occlusion comparing conventional surgery and septal percutaneous implant.

    Directory of Open Access Journals (Sweden)

    Márcia Gisele Santos da Costa

    Full Text Available OBJECTIVES: The study performs a cost-effectiveness analysis of procedures for atrial septal defects occlusion, comparing conventional surgery to septal percutaneous implant. METHODS: A model of analytical decision was structured with symmetric branches to estimate cost-effectiveness ratio between the procedures. The decision tree model was based on evidences gathered through meta-analysis of literature, and validated by a panel of specialists. The lower number of surgical procedures performed for atrial septal defects occlusion at each branch was considered as the effectiveness outcome. Direct medical costs and probabilities for each event were inserted in the model using data available from Brazilian public sector database system and information extracted from the literature review, using micro-costing technique. Sensitivity analysis included price variations of percutaneous implant. RESULTS: The results obtained from the decision model demonstrated that the percutaneous implant was more cost effective in cost-effectiveness analysis at a cost of US$8,936.34 with a reduction in the probability of surgery occurrence in 93% of the cases. Probability of atrial septal communication occlusion and cost of the implant are the determinant factors of cost-effectiveness ratio. CONCLUSIONS: The proposal of a decision model seeks to fill a void in the academic literature. The decision model proposed includes the outcomes that present major impact in relation to the overall costs of the procedure. The atrial septal defects occlusion using percutaneous implant reduces the physical and psychological distress to the patients in relation to the conventional surgery, which represent intangible costs in the context of economic evaluation.

  4. Pulmonary atresia with intact ventricular septum and agenesis of the ductus arteriosus in a pup

    International Nuclear Information System (INIS)

    Brown, D.J.; Patterson, D.F.

    1989-01-01

    A 7-week-old Wire Fox Terrier was admitted with pulmonary atresia with intact ventricular septum. The right ventricle and tricuspid valve were hypoplastic, and venous return to the right atrium reached the left side through an atrial septal defect. Oxygenation was via hyperplastic bronchial arteries. There was no evidence of the ductus arteriosus. Physical examination, plain and contrast radiography, and electrocardiography were performed. Clinical findings for this combination of defects were similar to those of more common defects (tetralogy of Fallot, patent ductus arteriosus

  5. A giant cardiac hydatid cyst presenting with chest pain and ventricular tachycardia in a pregnant woman undergoing cesarean section

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    Mehmet Yaman

    2016-09-01

    Full Text Available Cyst hydatid disease is an infectious disease caused by development of the larval form of Echinococcus granulosus in humans. Cardiac involvement of this disease is a rare condition, and if present, it is most commonly located in the left ventricle. Interventricular septal involvement is observed only in 4% of these cases. Herein, we report a case of cyst hydatid located at interventricular septum causing chest pain and ventricular tachycardia during cesarean section.

  6. Left Ventricular Diastolic Function and Characteristics in Fetal Aortic Stenosis

    Science.gov (United States)

    Friedman, Kevin G.; Schidlow, David; Freud, Lindsay; Escobar-Diaz, Maria; Tworetzky, Wayne

    2014-01-01

    Fetal aortic valvuloplasty (FAV) has shown promise in averting progression of mid-gestation aortic stenosis (AS) to hypoplastic left heart syndrome in a subset of patients. Patients who achieve biventricular circulation after FAV frequently have left ventricular (LV) diastolic dysfunction (DD). This study evaluates DD in fetuses with AS by comparing echocardiographic indices of LV diastolic function in fetuses undergoing FAV (n=20) to controls (n=40) and evaluates for LV factors associated with DD in FAV patients. We also compared pre- and post-FAV DD variables (n=16). Median gestational age (24 weeks, range 18–29 weeks) and fetal heart rate were similar between FAV and controls. Compared to controls, FAV patients had universally abnormal LV diastolic parameters including fused mitral inflow E and A waves (p=0.008), higher E velocity(p<0.001), shorter mitral inflow time (p=0.001), lower LV lateral annulus E′ (p<0.001), septal E′ (p=0.003) and higher E/E′ (p<0.001) than controls. FAV patients had abnormal right ventricular mechanics with higher tricuspid inflow E velocity (p<0.001), and shorter tricuspid inflow time (p=0.03). Worse LV diastolic function (lower LV E′) was associated with higher endocardial fibroelastosis (EFE) grade (r=0.74, p<0.001), large LV volume (r=0.55, p=0.013) and sphericity (r=0.58, P=0.009) and with lower LV pressure by mitral regurgitation jet (r=−0.68, p<0.001). Post-FAV, fewer patients had fused mitral inflow E and A than pre-FAV (p=0.05) and septal E′ was higher (=0.04). In conclusion, fetuses with mid-gestation AS have evidence of marked DD. Worse DD is associated with larger, more spherical LV, with more extensive EFE and lower LV pressure. PMID:24819899

  7. Double Outlet Right Ventricle With Intact Ventricular Septum: Avulsion or Exclusion.

    Science.gov (United States)

    Menon, Sabarinath; Kumar, C J Ashok; Mathew, Thomas; Venkateshwarn, S; Jayakumar, K; Dharan, Baiju S

    2016-03-01

    Double outlet right ventricle (DORV) is almost always associated with a ventricular septal defect. The variant of DORV with intact ventricular septum is very uncommon and may be associated with fetal demise or death immediately after birth. Reports of successful palliation of these patients to the stage of superior cavopulmonary anastomosis (bidirectional Glenn shunt) are rare. We describe the case of a child with DORV with intact ventricular septum who underwent successful palliation. This condition often provides a diagnostic, interventional, and surgical challenge. Patients generally require either balloon atrial septostomy or surgical atrial septectomy for survival, with the addition of a Blalock-Tausig shunt or pulmonary artery band depending on the pulmonary blood flow. Very few patients survive to a Glenn procedure. For those who do survive, a decision must be made regarding the management of the hypertensive and hypoplastic left ventricle (LV) and associated mitral regurgitation. The LV can be excluded by either mitral valve avulsion or closure (exclusion) of the mitral valve with a patch. The choice of the technique should be carefully made and depends in part on the size of the LV. In our patient, who was successfully palliated by bidirectional Glenn shunt, the LV was managed by means of mitral valve avulsion. © The Author(s) 2015.

  8. Right atrial isolation associated with atrial septal closure in patients with atrial septal defect and chronic atrial fibrillation.

    Science.gov (United States)

    Minzioni, G; Graffigna, A; Pagani, F; Vigano, M

    1993-12-01

    To restore sinus rhythm in the remaining heart chambers of six adult patients with atrial septal defect and chronic or paroxysmal atrial fibrillation, electrical, right atrial isolation associated with surgical correction of the defect was performed. All but one patient was free from atrial fibrillation without medication 2-25 months after operation. The isolated right atrial appendages showed intrinsic rhythmical activity in five patients and no electrical activity in one. Right atrial isolation is a safe and effective procedure that abolishes atrial fibrillation in patients with arrhythmia after surgical correction of atrial septal defect.

  9. Assessment of congenital heart disease by a thallium-201 SPECT study in children; Accuracy of estimated right to left ventricular pressure ratio

    Energy Technology Data Exchange (ETDEWEB)

    Ishii, Iwao; Nakajima, Kenichi; Taki, Junichi; Taniguchi, Mitsuru; Bunko, Hisashi; Tonami, Norihisa; Hisada, Kinichi; Ohno, Takashi (Kanazawa Univ. (Japan). School of Medicine)

    1993-01-01

    The characteristics of correlation between the right-to-left ventricular systolic pressure ratios (RVp/LVp) and the thallium-201 right-to-left ventricular ([sup 201]Tl R/L) count ratios was investigated in children with various congenital heart diseases. High-resolution three-headed SPECT system equipped with either parallel-hole or fan-beam collimators was used. In a total of 102 patients, the correlation between RVp/LVp and [sup 201]Tl R/L average count ratios was good in both planar (r=0.89, p=0.0001) and SPECT studies (r=0.80, p=0.0001). Quantitative analysis of myocardial uptake by SPECT demonstrated the characteristic pattern of each disease as well as the differences in the right ventricular overload types. When the linear regression analysis was performed in each heart disease, ventricular septal defect showed most excellent correlation. Complex heart anomalies also showed positive correlation (r=0.51, p=0.05) with RVp/LVp, and it can be used to estimate right ventricular pressure. After surgical treatment of tetralogy of Fallot and pulmonary stenosis, the decrease of [sup 201]Tl R/L count ratio was in accordance with improvement of right ventricular overload. We conclude that [sup 201]Tl SPECT study can be a good indicator for estimation of right ventricular pressure. (author).

  10. Atrial septal defect in a Korean wild raccoon dog.

    Science.gov (United States)

    Yim, Soomi; Choi, Sooyoung; Kim, Jongtaek; Chung, Jin-Young; Park, Inchul

    2017-10-07

    An approximately two-year-old, male 6.1 kg body weight, Korean wild raccoon dog (Nyctereutes procyonoides koreensis) was captured by the wildlife medical rescue center of Kangwon National University. Upon physical examination, the heart rate was 87 beats per min and there were no clinical signs. The hematological, and blood biochemical profiles revealed no remarkable findings; however, thoracic radiographs showed cardiac enlargement, especially in the right atrium. On electrocardiogram, sinus node dysfunction and bradyarrhythmia were revealed. Echocardiography showed a left-to-right shunting atrial septal defect. Based on these findings, this Korean wild raccoon dog was diagnosed with atrial septal defect. This is the rare case report of atrial septal defect in wildlife.

  11. Transcatheter closure of secundum atrial septal defect with cardio SEAL septal occluder. A preliminary result of clinical application

    International Nuclear Information System (INIS)

    Zhang Gejun; Dai Ruping; Liu Yanling; Jiang Shiliang; Zeng Zheng; Huang Lianjun; Xie Ruolan

    2001-01-01

    Objective: To evaluate the efficiency and preliminary results of transcatheter closure of secundum atrial septal defect (ASD) with CardioSEAL septal occluder. Methods: There were 12 patients in this study. Trans-esophageal echocardiography (TEE) before the interventions confirmed the ASDs with a mean diameter of *13.14 +- 3.48) mm (ranging from 8 to 20 mm). There were 11 isolated ASDs and 1 multi-defects ASD in the group. Each ASD was occluded with CardioSEAL septal occluder through the percutaneous procedure. The closure procedure was guided by fluoroscopy and trans-esophageal echocardiography. The TEE was done immediately after the procedure to find whether there was residual shunt. Trans-thoracic-echocardiography (TTE), ECG, and X-ray examination were done 24 hours, 1 month, 3 months, and 1 year after the procedures as follow-up to evaluate the efficiency. Results: The placements of the occluders were successful in 11 cases. There were no mortality and no emergent surgery during the procedures. TEE confirmed that there were trivial and small residual shunts in 3 cases immediately after the procedures. TTE confirmed small residual shunts in 2 cases 24 hour after the procedures, and in 1 case 1 month, 3 month, and 1 year after the procedures. Conclusion: Transcatheter closure of secundum ASD with CardioSEAL septal occluder was an efficient nonsurgical method. It could be the method of choice in treating the ASDs with special anatomic variations. It had a high successful rate of device placement and satisfied preliminary results, but the long-term follow-up was needed

  12. Contribution of mitral valve leaflet length and septal wall thickness to outflow tract obstruction in patients with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Morant, Kareem; Mikami, Yoko; Nevis, Immaculate; McCarty, David; Stirrat, John; Scholl, David; Rajchl, Martin; Giannoccaro, Peter; Kolman, Louis; Heydari, Bobby; Lydell, Carmen; Howarth, Andrew; Grant, Andrew; White, James A

    2017-08-01

    We sought to examine whether elongation of the mitral valve leaflets in patients with hypertrophic cardiomyopathy (HCM) is synergistic to septal wall thickness (SWT) in the development of left ventricular outflow tract obstruction (LVOTO). HCM is a common genetic cardiac disease characterized by asymmetric septal hypertrophy and predisposition towards LVOTO. It has been reported that elongation of the mitral valve leaflets may be a primary phenotypic feature and contribute to LVOTO. However, the relative contribution of this finding versus SWT has not been studied. 152 patients (76 with HCM and 76 non-diseased age, race and BSA-matched controls) and 18 young, healthy volunteers were studied. SWT and the anterior mitral valve leaflet length (AMVLL) were measured using cine MRI. The combined contribution of these variables (SWT × AMVLL) was described as the Septal Anterior Leaflet Product (SALP). Peak LVOT pressure gradient was determined by Doppler interrogation and defined as "obstructive" if ≥ 30 mmHg. Patients with HCM were confirmed to have increased AMVLL compared with controls and volunteers (p SALP were significantly higher in patients with LVOTO (N = 17) versus without. SALP showed modest improvement in predictive accuracy for LVOTO (AUC = 0.81) among the HCM population versus SWT alone (AUC = 0.77). However, in isolated patients this variable identified patients with LVOTO despite modest SWT. Elongation of the AMVLL is a primary phenotypic feature of HCM. While incremental contributions to LVOTO appear modest at a population level, specific patients may have dominant contribution to LVOTO. The combined marker of SALP allows for maintained identification of such patients despite modest increases in SWT.

  13. Efeitos da estimulação ventricular convencional em pacientes com função ventricular normal Efectos de la estimulación ventricular convencional en pacientes con función ventricular normal Conventional ventricular stimulation effects on patients with normal ventricular function

    Directory of Open Access Journals (Sweden)

    Luiz Antonio Batista de Sá

    2009-08-01

    (CF, test de marcha, dosificación de BNP, ecocardiograma (convencional y parámetros de desincronía intraventricular y prueba de calidad de vida (SF36. Esas mediciones se hicieron con 10 días(d (t1, 120d(t2 y 240 d(t3. Los datos se compararon a lo largo del tiempo según el método ANOVA. Comparaciones múltiples de promedios se efectuaron utilizándose el método de Tukey. RESULTADOS: Desde los datos evaluados, los siguientes no presentaron variación estadística significante (p>0,05: clase funcional, dosificación de BNP, parámetros ecocardiográficos convencionales, desincronía intraventricular (Doppler tisular. Presentaron empeoramiento (pBACKGROUND: The stimulation of the right ventricle (RV may be deleterious in patients with ventricular dysfunction; however there is little evidence about the impact of this stimulation in patients with normal ventricular function. OBJECTIVES: To assess the clinical and laboratory evolution of patients with normal ventricular function submitted to implant of artificial cardiac pacemaker (PM. METHODS: 16 patients enrolled according to the following inclusion criteria: normal ventricular function defined by echocardiogram and presence of upper ventricular stimulation > 90% (generator telemetry assessment submitted to a PM implant were prospectively studied. The following parameters were assessed: Functional Class (FC, walk test, BNP levels, echocardiography evaluation (conventional and intraventricular dyssynchrony and quality of life test (SF36. The patients were assessed after 10 (t1, 120 (t2 and 240 days (t3. Data was compared throughout time according to ANOVA. Multiple comparisons of means were performed through Tukey's test. RESULTS: Among the assessed data, the following did not present significant statistic variation (p> 0.05: functional class, BNP levels, conventional echocardiographic parameters, intraventricular dyssynchrony (tissue Doppler. The walk test (between t2 and t3 and the time between septal contraction

  14. Stenting of the right ventricular outflow tract in 2 dogs for palliation of dysplastic pulmonary valve stenosis and right-to-left intracardiac shunting defects.

    Science.gov (United States)

    Scansen, Brian A; Kent, Agnieszka M; Cheatham, Sharon L; Cheatham, John P; Cheatham, John D

    2014-09-01

    Two dogs with severe dysplastic pulmonary valve stenosis and right-to-left shunting defects (patent foramen ovale, perimembranous ventricular septal defect) underwent palliative stenting of the right ventricular outflow tract and pulmonary valve annulus using balloon expandable stents. One dog received 2 over-lapping bare metal stents placed 7 months apart; the other received a single covered stent. Both procedures were considered technically successful with a reduction in the transpulmonary valve pressure gradient from 202 to 90 mmHg in 1 dog and from 168 to 95 mmHg in the other. Clinical signs of exercise intolerance and syncope were temporarily resolved in both dogs. However, progressive right ventricular concentric hypertrophy, recurrent stenosis, and erythrocytosis were observed over the subsequent 6 months leading to poor long-term outcomes. Stenting of the right ventricular outflow tract is feasible in dogs with severe dysplastic pulmonary valve stenosis, though further study and optimization of the procedure is required. Copyright © 2014 Elsevier B.V. All rights reserved.

  15. Autoshaping a leverpress in rats with lateral, medial, or complete septal lesions.

    Science.gov (United States)

    Poplawsky, A; Phillips, C L

    1986-05-01

    Rats with either control operations or lateral, medial, or complete septal lesions received 600 trials of leverpress training using an autoshaping procedure, i.e., food delivery followed a 10 s illuminated lever presentation, or occurred immediately after a leverpress. Rats with complete septal lesions acquired the leverpress faster than controls and had more food-tray entries per minute during the first 100 trials than the other groups. Rats with lateral or medial septal lesions had leverpress and food-tray entries equivalent to controls. The facilitation of autoshaping a leverpress may partially be explained by the general increase in motor reactivity to stimuli found following septal lesions.

  16. [Effect of down-regulation of IKs repolarization-reserve on ventricular arrhythmogenesis in a guinea pig model of cardiac hypertrophy].

    Science.gov (United States)

    Wang, Hegui; Huang, Ting; Wang, Zheng; Ge, Nannan; Ke, Yongsheng

    2018-04-28

    To observe the changes of rapidly activated delayed rectifier potassium channel (IKr) and slowly activated delayed rectifier potassium channel (IKs) in cardiac hypertrophy and to evaluate the effects of IKr and IKs blocker on the incidence of ventricular arrhythmias in guinea pigs with left ventricular hypertrophy (LVH).
 Methods: Guinea pigs were divided into a sham operation group and a left ventricular hypertrophy (LVH) group. LVH model was prepared. Whole cell patch-clamp technique was used to record IKr and IKs tail currents in a guinea pig model with LVH. The changes of QTc and the incidence rate of ventricular arrhythmias in LVH guinea pigs were observed by using the IKr and IKs blockers.
 Results: Compared with cardiac cells in the control group, the interventricular septal thickness at end systole (IVSs), left ventricular posterior wall thickness at end systole (LVPWs), QTc interval and cell capacitance in guinea pigs with LVH were significantly increased (Pguinea pigs with LVH compared with the control guinea pigs. In contrast, IKs blocker produced modest increase in QTc interval in guinea pigs of control group with no increase in LVH animals. IKs blocker did not induce ventricular arrhythmias incidence in either control or LVH animals.
 Conclusion: The cardiac hypertrophy-induced arrhythmogenesis is due to the down-regulation 
of IKs.

  17. Efecto de la localización del electrodo ventricular derecho (tracto de salida vs. ápex sobre la sincronía ventricular mecánica, en pacientes sometidos a terapia de implante de marcapaso cardiaco Effect of right ventricular electrode location (outflow tract vs. apex on mechanical ventricular synchrony in patients that underwent pacemaker implant therapy

    Directory of Open Access Journals (Sweden)

    Oscar S Rincón

    2008-12-01

    post-implant in the right ventricle outflow tract and in 5 (50% in the apex. Mean interventricular pot-implant delay was 21,6 ms in the right ventricular outflow tract and 11,5 ms in the apex (p = 0,8; mean septal to lateral wall delay was 73 ms in the right ventricular outflow tract and 26 ms in the apex (p = 0,8. QRS post-implant delay was 134 ms in the right ventricular outflow tract and 140 ms in the apex (p = 0,1. No differences between implant parameters and device programming were found. Conclusions: presence of ventricular asynchrony was evidenced in patients with normal QRS and structurally healthy heart. Ventricular stimulation with pacemaker from the apex or the right ventricular outflow tract suggests acute ventricular asynchrony at least in 60% of the cases, without statistically significant difference between both groups.

  18. Effect of right ventricular pacing lead site on left ventricular function in patients with high-grade atrioventricular block: results of the Protect-Pace study.

    Science.gov (United States)

    Kaye, Gerald C; Linker, Nicholas J; Marwick, Thomas H; Pollock, Lucy; Graham, Laura; Pouliot, Erika; Poloniecki, Jan; Gammage, Michael

    2015-04-07

    Chronic right ventricle (RV) apical (RVA) pacing is standard treatment for an atrioventricular (AV) block but may be deleterious to left ventricle (LV) systolic function. Previous clinical studies of non-apical pacing have produced conflicting results. The aim of this randomized, prospective, international, multicentre trial was to compare change in LV ejection fraction (LVEF) between right ventricular apical and high septal (RVHS) pacing over a 2-year study period. We randomized 240 patients (age 74 ± 11 years, 67% male) with a high-grade AV block requiring >90% ventricular pacing and preserved baseline LVEF >50%, to receive pacing at the RVA (n = 120) or RVHS (n = 120). At 2 years, LVEF decreased in both the RVA (57 ± 9 to 55 ± 9%, P = 0.047) and the RVHS groups (56 ± 10 to 54 ± 10%, P = 0.0003). However, there was no significant difference in intra-patient change in LVEF between confirmed RVA (n = 85) and RVHS (n = 83) lead position (P = 0.43). There were no significant differences in heart failure hospitalization, mortality, the burden of atrial fibrillation, or plasma brain natriutetic peptide levels between the two groups. A significantly greater time was required to place the lead in the RVHS position (70 ± 25 vs. 56 ± 24 min, P function requiring a high percentage of ventricular pacing, RVHS pacing does not provide a protective effect on left ventricular function over RVA pacing in the first 2 years. ClinicalTrials.gov number NCT00461734. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.

  19. Decreased inward rectifier potassium current IK1 in dystrophin-deficient ventricular cardiomyocytes.

    Science.gov (United States)

    Rubi, Lena; Koenig, Xaver; Kubista, Helmut; Todt, Hannes; Hilber, Karlheinz

    2017-03-04

    Kir2.x channels in ventricular cardiomyocytes (most prominently Kir2.1) account for the inward rectifier potassium current I K1 , which controls the resting membrane potential and the final phase of action potential repolarization. Recently it was hypothesized that the dystrophin-associated protein complex (DAPC) is important in the regulation of Kir2.x channels. To test this hypothesis, we investigated potential I K1 abnormalities in dystrophin-deficient ventricular cardiomyocytes derived from the hearts of Duchenne muscular dystrophy mouse models. We found that I K1 was substantially diminished in dystrophin-deficient cardiomyocytes when compared to wild type myocytes. This finding represents the first functional evidence for a significant role of the DAPC in the regulation of Kir2.x channels.

  20. Anomalous muscle bundle in the right atrium; Implication to trans atrial device closure

    Directory of Open Access Journals (Sweden)

    Saji Philip

    2017-09-01

    Full Text Available Intracavitary muscle bands or aberrant bands have been well described in all four chambers of the heart but rarely seen thick muscular band crossing right atrium. We report a case of devisable secundum atrial septal defect with an intra-atrial anomalous muscular band, crossing right atrial wall to the rim of the secundum atrial septal defect warranting surgical closure.

  1. NMDA-dependent phase synchronization between septal and temporal CA3 hippocampal networks.

    Science.gov (United States)

    Gu, Ning; Jackson, Jesse; Goutagny, Romain; Lowe, Germaine; Manseau, Frédéric; Williams, Sylvain

    2013-05-08

    Increasing evidence suggests that synchronization between brain regions is essential for information exchange and memory processes. However, it remains incompletely known which synaptic mechanisms contribute to the process of synchronization. Here, we investigated whether NMDA receptor-mediated synaptic plasticity was an important player in synchronization between septal and temporal CA3 areas of the rat hippocampus. We found that both the septal and temporal CA3 regions intrinsically generate weakly synchronized δ frequency oscillations in the complete hippocampus in vitro. Septal and temporal oscillators differed in frequency, power, and rhythmicity, but both required GABAA and AMPA receptors. NMDA receptor activation, and most particularly the NR2B subunit, contributed considerably more to rhythm generation at the temporal than the septal region. Brief activation of NMDA receptors by application of extracellular calcium dramatically potentiated the septal-temporal coherence for long durations (>40 min), an effect blocked by the NMDA antagonist AP-5. This long-lasting NMDA-receptor-dependent increase in coherence was also associated with an elevated phase locking of spikes locally and across regions. Changes in coherence between oscillators were associated with increases in phase locking between oscillators independent of oscillator amplitude. Finally, although the septal CA3 rhythm preceded the oscillations in temporal regions in control conditions, this was reversed during the NMDA-dependent enhancement in coherence, suggesting that NMDA receptor activation can change the direction of information flow along the septotemporal CA3 axis. These data demonstrate that plastic changes in communication between septal and temporal hippocampal regions can arise from the NMDA-dependent phase locking of neural oscillators.

  2. One-stop shop assessment for atrial septal defect closure using 256-slice coronary CT angiography

    Energy Technology Data Exchange (ETDEWEB)

    Yamasaki, Yuzo; Kamitani, Takeshi; Sagiyama, Koji; Yamanouchi, Torahiko; Honda, Hiroshi [Kyushu University, Department of Clinical Radiology, Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka (Japan); Nagao, Michinobu; Kawanami, Satoshi [Kyushu University, Department of Molecular Imaging and Diagnosis, Graduate School of Medical Sciences, Fukuoka (Japan); Sakamoto, Ichiro [Kyushu University, Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Fukuoka (Japan); Yamamura, Kenichiro [Kyushu University, Department of Pediatrics, Graduate School of Medical Sciences, Fukuoka (Japan); Yabuuchi, Hidetake [Kyushu University, Department of Health Sciences, Graduate School of Medical Sciences, Fukuoka (Japan)

    2017-02-15

    To investigate the feasibility and accuracy of measurement of the pulmonary to systemic blood flow ratio (Qp/Qs) and defect and rim sizes in secundum atrial septal defects (ASDs) using 256-slice CT, compared to the reference transoesophageal echocardiography (TEE) and right heart catheterization (RHC) measurements. Twenty-three consecutive adult patients with secundum ASDs who underwent retrospective ECG-gated coronary CT angiography (CCTA), TEE and RHC were enrolled in this study. Right ventricular (RV) and left ventricular (LV) stroke volumes (SV) were calculated by biventricular volumetry of CCTA. Qp/Qs-CT was defined as RVSV/LVSV. The sizes of the defect and rim were measured by multi-planar reconstruction CT images. Correlations between Qp/Qs-CT and Qp/Qs-RHC and between the defect diameter obtained by CT and TEE were analyzed by Pearson's coefficient analysis. Rim sizes by CT and TEE were compared by paired t-test. Qp/Qs-CT was significantly correlated with Qp/Qs-RHC (r = 0.83, p < 0.0001), and the defect diameter by CT was significantly correlated with that by TEE (r = 0.95, p < 0.0001). There was no significant difference between CT and TEE in measurements of rim size. 256-slice CCTA allows measuring Qp/Qs and size of defects and rims in patients with secundum ASDs, accomplishing pretreatment evaluation non-invasively and comprehensively. (orig.)

  3. One-stop shop assessment for atrial septal defect closure using 256-slice coronary CT angiography

    International Nuclear Information System (INIS)

    Yamasaki, Yuzo; Kamitani, Takeshi; Sagiyama, Koji; Yamanouchi, Torahiko; Honda, Hiroshi; Nagao, Michinobu; Kawanami, Satoshi; Sakamoto, Ichiro; Yamamura, Kenichiro; Yabuuchi, Hidetake

    2017-01-01

    To investigate the feasibility and accuracy of measurement of the pulmonary to systemic blood flow ratio (Qp/Qs) and defect and rim sizes in secundum atrial septal defects (ASDs) using 256-slice CT, compared to the reference transoesophageal echocardiography (TEE) and right heart catheterization (RHC) measurements. Twenty-three consecutive adult patients with secundum ASDs who underwent retrospective ECG-gated coronary CT angiography (CCTA), TEE and RHC were enrolled in this study. Right ventricular (RV) and left ventricular (LV) stroke volumes (SV) were calculated by biventricular volumetry of CCTA. Qp/Qs-CT was defined as RVSV/LVSV. The sizes of the defect and rim were measured by multi-planar reconstruction CT images. Correlations between Qp/Qs-CT and Qp/Qs-RHC and between the defect diameter obtained by CT and TEE were analyzed by Pearson's coefficient analysis. Rim sizes by CT and TEE were compared by paired t-test. Qp/Qs-CT was significantly correlated with Qp/Qs-RHC (r = 0.83, p < 0.0001), and the defect diameter by CT was significantly correlated with that by TEE (r = 0.95, p < 0.0001). There was no significant difference between CT and TEE in measurements of rim size. 256-slice CCTA allows measuring Qp/Qs and size of defects and rims in patients with secundum ASDs, accomplishing pretreatment evaluation non-invasively and comprehensively. (orig.)

  4. A new "twist" on right heart failure with left ventricular assist systems.

    Science.gov (United States)

    Houston, Brian A; Shah, Keyur B; Mehra, Mandeep R; Tedford, Ryan J

    2017-07-01

    Despite significant efforts to predict and prevent right heart failure, it remains a leading cause of morbidity and mortality after implantation of left ventricular assist systems (LVAS). In this Perspective, we review the underappreciated anatomic and physiologic principles that govern the relationship between left and right heart function and contribute to this phenomenon. This includes the importance of considering the right ventricle (RV) and pulmonary arterial circuit as a coupled system; the contribution of the left ventricle (LV) to RV contractile function and the potential negative impact of acutely unloading the LV; the influence of the pericardium and ventricular twist on septal function; the role of RV deformation in reduced mechanical efficiency after device placement; and the potential of ongoing stressors of an elevated right-sided preload. We believe an appreciation of these complex issues is required to fully understand the expression of the unique phenotypes of right heart failure after LVAS implantation and for developing better prognostic and therapeutic strategies. Copyright © 2017 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.

  5. Translating golden retriever muscular dystrophy microarray findings to novel biomarkers for cardiac/skeletal muscle function in Duchenne muscular dystrophy.

    Science.gov (United States)

    Galindo, Cristi L; Soslow, Jonathan H; Brinkmeyer-Langford, Candice L; Gupte, Manisha; Smith, Holly M; Sengsayadeth, Seng; Sawyer, Douglas B; Benson, D Woodrow; Kornegay, Joe N; Markham, Larry W

    2016-04-01

    In Duchenne muscular dystrophy (DMD), abnormal cardiac function is typically preceded by a decade of skeletal muscle disease. Molecular reasons for differences in onset and progression of these muscle groups are unknown. Human biomarkers are lacking. We analyzed cardiac and skeletal muscle microarrays from normal and golden retriever muscular dystrophy (GRMD) dogs (ages 6, 12, or 47+ mo) to gain insight into muscle dysfunction and to identify putative DMD biomarkers. These biomarkers were then measured using human DMD blood samples. We identified GRMD candidate genes that might contribute to the disparity between cardiac and skeletal muscle disease, focusing on brain-derived neurotropic factor (BDNF) and osteopontin (OPN/SPP1, hereafter indicated as SPP1). BDNF was elevated in cardiac muscle of younger GRMD but was unaltered in skeletal muscle, while SPP1 was increased only in GRMD skeletal muscle. In human DMD, circulating levels of BDNF were inversely correlated with ventricular function and fibrosis, while SPP1 levels correlated with skeletal muscle function. These results highlight gene expression patterns that could account for differences in cardiac and skeletal disease in GRMD. Most notably, animal model-derived data were translated to DMD and support use of BDNF and SPP1 as biomarkers for cardiac and skeletal muscle involvement, respectively.

  6. Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature

    Directory of Open Access Journals (Sweden)

    Lalita Nemani

    2014-01-01

    Full Text Available We report a case of type-A Coffin-Siris syndrome (CSS with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD, subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.

  7. Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature.

    Science.gov (United States)

    Nemani, Lalita; Barik, Ramachandra; Patnaik, Amar Narayana; Mishra, Ramesh C; Rao, Amaresh M; Kapur, Pragati

    2014-09-01

    We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.

  8. Evaluation of cardiac function in patients with Duchenne's muscular dystrophy by single photon emission computed tomography (SPECT)

    International Nuclear Information System (INIS)

    Tamura, Takuhisa; Motomura, Masakatsu; Kanazawa, Hajime; Shibuya, Noritoshi

    1989-01-01

    The extent of myocardial ischemia was evaluated in 20 patients with Duchenne's muscular dystrophy (DMD) by using Bull's eye method of thallium-201 myocardial SPECT. It was examined in relation to skeletal muscle involvement, age, left ventricular (LV) ejection fraction and ventricular premature contractions (VPCs). Myocardial ischemia was detected in all of patients with DMD. Ischemic lesion was mostly detected in the apical side of the LV lateral wall and interventricular septum, while the extent of myocardial ischemia had no correlations with either the stage of functional disability of skeletal muscle or age. The more ischemic ratio was higher, the more LV ejection fraction decreased. The total number of VPCs was relatively small and it did not have any relation to myocardial ischemic ratio. These results suggest that younger DMD patients having extensive myocardial ischemia and/or ventricular tachycardia will have a high risk of cardiac death. (author)

  9. Evaluating the cardiac function of duchenne muscular dystrophy with Doppler Tei index

    International Nuclear Information System (INIS)

    Yao Fengjuan; Zheng Ju; Lu Kun; Liu Donghong; Wu Miaoling; Lin Hong; Zhang Cheng; Yu Hongkui

    2007-01-01

    Objective: To evaluate the cardiac function of early Duchenne muscular dystrophy (DMD) by left ventricular ejection fraction (LVEF) and pulse Doppler Tei index. Methods: Twenty-eight DMD patients and fifteen normal people were studied. LVEF, E/A and Tei index were measured and calculated by M-mode and Pulse wave Doppler respectively. Results: Compared with control group, Tei index and IRT were significantly high, and there were not significant difference in LVEF(%) and E/A. Conclusion: Tei index was valuable in assessing cardiac function of early DMD. (authors)

  10. Budget impact analysis of the percutaneous septal occluder for treatment of ostium secundum atrial septal defects in the Brazilian Unified National Health System.

    Science.gov (United States)

    Senna, Kátia Marie Simões e; Sarti, Flavia Mori; Costa, Márcia Gisele Santos da; Nita, Marcelo Eidi; Santos, Marisa da Silva; Tura, Bernardo Rangel; Correia, Marcelo Goulart

    2015-08-01

    The aim of this study was to perform a budget impact analysis on the adoption of percutaneous occlusion of ostium secundum atrial septal defects in the Brazilian Unified National Health System. Costs were collected using micro-costing technique from medical records for each treatment technique (conventional surgery versus percutaneous septal occluder) at a public federal hospital specialized in high-complexity cardiology. The analysis showed that expenditures associated with percutaneous occlusion were lower than with conventional surgery, and sensitivity analysis confirmed the cost reduction in several scenarios, showing a significant budget impact with a 30% adoption rate for the percutaneous occluder (savings of approximately 1.5 million dollars per year). The study indicates that the adoption of the percutaneous septal occluder would mean cost savings of approximately 3.5 million dollars for the Brazilian public health system.

  11. Evolutionary changes in left and right ventricular function in acute myocardial infarction

    International Nuclear Information System (INIS)

    Ohsuzu, Fumitaka; Yasuda, Tsunehiro; Gold, H.K.; Leinbach, R.C.; Rosenthal, S.V.; Alpert, N.M.; Boucher, C.A.; McKusick, K.A.; Strauss, H.W.

    1987-01-01

    To determine the evolutionary changes in right and left ventricular function in acute myocardial infarction, 3 serial gated blood pool scans were performed in 76 patients within 24 hours (24 H), at 10 days (10 D) and 3 months (3 M) following the onset of myocardial infarction. The patients were divided into 3 groups: ANT (anterior MI), INF (inferior MI without right ventricular dysfunction) and RVF (inferior MI with right ventricular dysfunction). LVEF in ANT was significantly lower than that of INF and RVF at 24 H, 10 D and 3 M. The ratio of right ventricular volume to LV volume (RVV/LVV) was compared among 3 groups. The mean values of RVV/LVV in RVF were 1.3 through 24 H and 3 M and they were significantly higher than the other two groups. The RVV/LVV in ANT and INF were around 1.0. LVEDVI in RVF was rather smaller than that of ANT and INF. LVESVI in ANT at 24 H was significantly larger than that of INF and RVF and the mean value of LVESVI in ANT were around 60 ml/M 2 from 24 H to 3 M. LVEF in ANT, RVF and INF did not increase significantly during peak exercise at 3 M. However, quantitative regional wall motion analysis revealed that regional wall motion of R2 (posterolateral wall motion) in ANT and R5 (septal wall motion) in INF decreased significantly during peak exercise. These impairments in regional wall motion might be due to the exacerbation of ischemia of non-infarcted area. (author)

  12. Evolutionary changes in left and right ventricular function in acute myocardial infarction

    Energy Technology Data Exchange (ETDEWEB)

    Ohsuzu, Fumitaka; Yasuda, Tsunehiro; Gold, H K; Leinbach, R C; Rosenthal, S V; Alpert, N M; Boucher, C A; McKusick, K A; Strauss, H W

    1987-09-01

    To determine the evolutionary changes in right and left ventricular function in acute myocardial infarction, 3 serial gated blood pool scans were performed in 76 patients within 24 hours (24 H), at 10 days (10 D) and 3 months (3 M) following the onset of myocardial infarction. The patients were divided into 3 groups: ANT (anterior MI), INF (inferior MI without right ventricular dysfunction) and RVF (inferior MI with right ventricular dysfunction). LVEF in ANT was significantly lower than that of INF and RVF at 24 H, 10 D and 3 M. The ratio of right ventricular volume to LV volume (RVVLVV) was compared among 3 groups. The mean values of RVVLVV in RVF were 1.3 through 24 H and 3 M and they were significantly higher than the other two groups. The RVVLVV in ANT and INF were around 1.0. LVEDVI in RVF was rather smaller than that of ANT and INF. LVESVI in ANT at 24 H was significantly larger than that of INF and RVF and the mean value of LVESVI in ANT were around 60 mlM/sup 2/ from 24 H to 3 M. LVEF in ANT, RVF and INF did not increase significantly during peak exercise at 3 M. However, quantitative regional wall motion analysis revealed that regional wall motion of R2 (posterolateral wall motion) in ANT and R5 (septal wall motion) in INF decreased significantly during peak exercise. These impairments in regional wall motion might be due to the exacerbation of ischemia of non-infarcted area

  13. Transcatheter closure, mini-invasive closure and open-heart surgical repair for treatment of perimembranous ventricular septal defects in children: a protocol for a network meta-analysis.

    Science.gov (United States)

    You, Tao; Yi, Kang; Ding, Zhao-Hong; Hou, Xiao-Dong; Liu, Xing-Guang; Wang, Xin-Kuan; Ge, Long; Tian, Jin-Hui

    2017-06-21

    Both transcatheter device closure and surgical repair are effective treatments with excellent midterm outcomes for perimembranous ventricular septal defects (pmVSDs) in children. The mini-invasive periventricular device occlusion technique has become prevalent in research and application, but evidence is limited for the assessment of transcatheter closure, mini-invasive closure and open-heart surgical repair. This study comprehensively compares the efficacy, safety and costs of transcatheter closure, mini-invasive closure and open-heart surgical repair for treatment of pmVSDs in children using Bayesian network meta-analysis. A systematic search will be performed using Chinese Biomedical Literature Database, China National Knowledge Infrastructure, PubMed, EMBASE.com and the Cochrane Central Register of Controlled Trials to include random controlled trials, prospective or retrospective cohort studies comparing the efficacy, safety and costs of transcatheter closure, mini-invasive closure and open-heart surgical repair. The risk of bias for the included prospective or retrospective cohort studies will be evaluated according to the risk of bias in non-randomised studies of interventions (ROBINS-I). For random controlled trials, we will use risk of bias tool from Cochrane Handbook version 5.1.0. A Bayesian network meta-analysis will be conducted using R-3.3.2 software. Ethical approval and patient consent are not required since this study is a network meta-analysis based on published trials. The results of this network meta-analysis will be submitted to a peer-reviewed journal for publication. CRD42016053352. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  14. Myotonic Muscular Dystrophy

    Science.gov (United States)

    ... Marie-Tooth Disease (CMT) Congenital Muscular Dystrophy (CMD) Duchenne Muscular Dystrophy (DMD) Emery-Dreifuss Muscular Dystrophy Endocrine Myopathies Metabolic Diseases of Muscle Mitochondrial Myopathies (MM) Myotonic Dystrophy (DM) Spinal-Bulbar ...

  15. Myocardial Fibrosis Progression in Duchenne and Becker Muscular Dystrophy: A Randomized Clinical Trial.

    Science.gov (United States)

    Silva, Marly Conceição; Magalhães, Tiago Augusto; Meira, Zilda Maria Alves; Rassi, Carlos Henrique Reis Esselin; Andrade, Amanda Cristina de Souza; Gutierrez, Paulo Sampaio; Azevedo, Clerio Francisco; Gurgel-Giannetti, Juliana; Vainzof, Mariz; Zatz, Mayana; Kalil-Filho, Roberto; Rochitte, Carlos Eduardo

    2017-02-01

    In Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), interventions reducing the progression of myocardial disease could affect survival. To assess the effect of early angiotensin-converting enzyme (ACE) inhibitor therapy in patients with normal left ventricular function on the progression of myocardial fibrosis (MF) identified on cardiovascular magnetic resonance (CMR). A randomized clinical trial conducted in 2 centers included 76 male patients with DMD or BMD undergoing 2 CMR studies with a 2-year interval for ventricular function and MF assessment. In a non-intent-to-treat trial, 42 patients with MF and normal left ventricular ejection fraction (LVEF) were randomized (1:1) to receive or not receive ACE inhibitor therapy. The study was conducted from June 26, 2009, to June 30, 2012. Data analysis was performed from June 30, 2013, to October 3, 2016. Randomization (1:1) to receive or not receive ACE inhibitor therapy. Primary outcome was MF progression from baseline to the 2-year CMR study. Of the 76 male patients included in the study, 70 had DMD (92%) and 6 had BMD (8%); mean (SD) age at baseline was 13.1 (4.4) years. Myocardial fibrosis was present in 55 patients (72%) and LV systolic dysfunction was identified in 13 patients (24%). Myocardial fibrosis at baseline was an independent indicator of lower LVEF at follow-up (coefficient [SE], -0.16 [0.07]; P = .03). Among patients with MF and preserved LVEF (42 [55%]), those randomized (21 patients in each arm) to receive ACE inhibitors demonstrated slower MF progression compared with the untreated group (mean [SD] increase of 3.1% [7.4%] vs 10.0% [6.2%] as a percentage of LV mass; P = .001). In multivariate analysis, ACE inhibitor therapy was an independent indicator of decreased MF progression (coefficient [SE], -4.51 [2.11]; P = .04). Patients with MF noted on CMR had a higher probability of cardiovascular events (event rate, 10 of 55 [18.2%] vs 0 of 21 [0%]; log-rank P = .04

  16. Response to Cardiac Resynchronization Therapy: The Muscular Metabolic Pathway

    Directory of Open Access Journals (Sweden)

    Jérémie Jaussaud

    2011-01-01

    245±140 seconds (=.01. Peak VO2, VE/VCO2, peak circulatory power and NYHA were improved after CRT (13±4 to16±5 ml/kg/min (<.05, 45±16 to 39±13 (<.01, 1805±844 to 2225±1171 mmHg.ml/kg/min (<.01 and 3±0.35 to 1.88±0.4 (=.01. In addition, left ventricular ejection fraction and end-systolic volumes were improved from 24±8 to 29±7% (<.01 and from 157±69 to 122±55 ml (<.01. Conclusion. We suggest that CRT leads to an increase in oxidative muscular metabolism and postponed anaerobic threshold reducing exaggerated hyperventilation during exercise.

  17. Serial changes in anatomy and ventricular function on dual-source cardiac computed tomography after the Norwood procedure for hypoplastic left heart syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Seoul (Korea, Republic of)

    2017-12-15

    Accurate evaluation of anatomy and ventricular function after the Norwood procedure in hypoplastic left heart syndrome is important for treatment planning and prognostication, but echocardiography and cardiac MRI have limitations. To assess serial changes in anatomy and ventricular function on dual-source cardiac CT after the Norwood procedure for hypoplastic left heart syndrome. In 14 consecutive patients with hypoplastic left heart syndrome, end-systolic and end-diastolic phase cardiac dual-source CT was performed before and early (average: 1 month) after the Norwood procedure, and repeated late (median: 4.5 months) after the Norwood procedure in six patients. Ventricular functional parameters and indexed morphological measurements including pulmonary artery size, right ventricular free wall thickness, and ascending aorta size on cardiac CT were compared between different time points. Moreover, morphological features including ventricular septal defect, endocardial fibroelastosis and coronary ventricular communication were evaluated on cardiac CT. Right ventricular function and volumes remained unchanged (indexed end-systolic and end-diastolic volumes: 38.9±14.0 vs. 41.1±21.5 ml/m{sup 2}, P=0.7 and 99.5±30.5 vs. 105.1±33.0 ml/m{sup 2}, P=0.6; ejection fraction: 60.1±7.3 vs. 63.8±7.0%, P=0.1, and indexed stroke volume: 60.7±18.0 vs. 64.0±15.6 ml/m{sup 2}, P=0.5) early after the Norwood procedure, but function was decreased (ejection fraction: 64.2±2.6 vs. 58.1±7.1%, P=0.01) and volume was increased (indexed end-systolic and end-diastolic volumes: 39.2±14.9 vs. 68.9±20.6 ml/m{sup 2}, P<0.003 and 107.8±36.5 vs. 162.9±36.2 ml/m{sup 2}, P<0.006, and indexed stroke volume: 68.6±21.7 vs. 94.0±21.3 ml/m{sup 2}, P=0.02) later. Branch pulmonary artery size showed a gradual decrease without asymmetry after the Norwood procedure. Right and left pulmonary artery stenoses were identified in 21.4% (3/14) of the patients. Indexed right ventricular free wall

  18. Comparison of human septal nuclei MRI measurements using automated segmentation and a new manual protocol based on histology

    Science.gov (United States)

    Butler, Tracy; Zaborszky, Laszlo; Pirraglia, Elizabeth; Li, Jinyu; Wang, Xiuyuan Hugh; Li, Yi; Tsui, Wai; Talos, Delia; Devinsky, Orrin; Kuchna, Izabela; Nowicki, Krzysztof; French, Jacqueline; Kuzniecky, Rubin; Wegiel, Jerzy; Glodzik, Lidia; Rusinek, Henry; DeLeon, Mony J.; Thesen, Thomas

    2014-01-01

    Septal nuclei, located in basal forebrain, are strongly connected with hippocampi and important in learning and memory, but have received limited research attention in human MRI studies. While probabilistic maps for estimating septal volume on MRI are now available, they have not been independently validated against manual tracing of MRI, typically considered the gold standard for delineating brain structures. We developed a protocol for manual tracing of the human septal region on MRI based on examination of neuroanatomical specimens. We applied this tracing protocol to T1 MRI scans (n=86) from subjects with temporal epilepsy and healthy controls to measure septal volume. To assess the inter-rater reliability of the protocol, a second tracer used the same protocol on 20 scans that were randomly selected from the 72 healthy controls. In addition to measuring septal volume, maximum septal thickness between the ventricles was measured and recorded. The same scans (n=86) were also analysed using septal probabilistic maps and Dartel toolbox in SPM. Results show that our manual tracing algorithm is reliable, and that septal volume measurements obtained via manual and automated methods correlate significantly with each other (pautomated methods detected significantly enlarged septal nuclei in patients with temporal lobe epilepsy in accord with a proposed compensatory neuroplastic process related to the strong connections between septal nuclei and hippocampi. Septal thickness, which was simple to measure with excellent inter-rater reliability, correlated well with both manual and automated septal volume, suggesting it could serve as an easy-to-measure surrogate for septal volume in future studies. Our results call attention to the important though understudied human septal region, confirm its enlargement in temporal lobe epilepsy, and provide a reliable new manual delineation protocol that will facilitate continued study of this critical region. PMID:24736183

  19. Nasal septal angiofibroma, a subclass of extranasopharyngeal angiofibroma.

    Science.gov (United States)

    Garcia-Rodriguez, Laura; Rudman, Kelli; Cogbill, Christopher H; Loehrl, Todd; Poetker, David M

    2012-01-01

    Extranasopharyngeal angiofibromas (ENA) arising from the nasal septum or nasal septal angiofibromas are extremely rare; only 13 such cases have been reported in the international literature. Our objective is to describe the presentation, workup, and surgical management of these lesions. Case reports were done. The setting was a tertiary care referral center and the Veterans Affairs Medical Center. PATIENTS, INTERVENTIONS, AND RESULTS: We present 2 cases of extranasopharyngeal angiofibroma occurring on the nasal septum. In this report, we discuss the occurrence, the histopathologic findings, and the treatment of nasal septal angiofibroma. Copyright © 2012 Elsevier Inc. All rights reserved.

  20. Detection of impaired fatty acid metabolism in right ventricular hypertrophy. Assessment by I-123 {beta}-methyl iodophenyl pentadecanoic acid (BMIPP) myocardial single-photon emission computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yong-ih; Goto, Hideki; Kobayashi, Katsuhiro; Sawada, Yoshihiro; Miyake, Yoshitaka; Fujiwara, Go [Nishiyodo Hospital, Osaka (Japan); Chiba, Hiroshi; Okada, Tomoya; Nishimura, Tsunehiko

    1997-08-01

    The subjects consisted of 6 patients with chronic obstructive pulmonary disease, 4 with primary pulmonary hypertension, 2 each with refractory pulmonary tuberculosis, tricuspid insufficiency, pulmonary embolism, 1 each with atrial septal defect, ventricular septal defect (Eisenmenger complex), Ebstein anomaly, and endocardial defect, and 7 healthy controls. SPECT imaging with Tl-201 (Tl) and I-123 BMIPP, and Tc-99m RBC first pass and gated blood pool scintigraphy were performed. Based on Tl planar images, the subjects were classified into 3 groups: 7 patients with no RV visualization (Group A), 11 with moderate RV visualization (Group B) and 9 with marked RV visualization (Group C). As a semi-quantitative evaluation by a myocardial SPECT, 3 regions in 3 representative short axial images were divided into 9 segments, each of which was graded from 0 to +3, and their sum was calculated as the RV score. The right ventricular ejection fraction (RVEF) and the left ventricular ejection fraction were obtained by Tc-99m RBC cardiac scintigraphy. The groups with marked visualization of the right ventricle had lower RVEF, and there was a good correlation between the RVEF and the RV score with both a and BMIPP. Although a good correlation was demonstrated between the RV score with Tl and BMIPP in Groups A and B, in Group C, in which there was marked RV Tl visualization, the RV score with BMIPP was significantly smaller than with Tl. These findings suggest that impaired fatty acid metabolism may exist in severely hypertrophic right ventricle due to RV overload. (K.H.)

  1. Detection of impaired fatty acid metabolism in right ventricular hypertrophy. Assessment by I-123 β-methyl iodophenyl pentadecanoic acid (BMIPP) myocardial single-photon emission computed tomography

    International Nuclear Information System (INIS)

    Kim, Yong-ih; Goto, Hideki; Kobayashi, Katsuhiro; Sawada, Yoshihiro; Miyake, Yoshitaka; Fujiwara, Go; Chiba, Hiroshi; Okada, Tomoya; Nishimura, Tsunehiko.

    1997-01-01

    The subjects consisted of 6 patients with chronic obstructive pulmonary disease, 4 with primary pulmonary hypertension, 2 each with refractory pulmonary tuberculosis, tricuspid insufficiency, pulmonary embolism, 1 each with atrial septal defect, ventricular septal defect (Eisenmenger complex), Ebstein anomaly, and endocardial defect, and 7 healthy controls. SPECT imaging with Tl-201 (Tl) and I-123 BMIPP, and Tc-99m RBC first pass and gated blood pool scintigraphy were performed. Based on Tl planar images, the subjects were classified into 3 groups: 7 patients with no RV visualization (Group A), 11 with moderate RV visualization (Group B) and 9 with marked RV visualization (Group C). As a semi-quantitative evaluation by a myocardial SPECT, 3 regions in 3 representative short axial images were divided into 9 segments, each of which was graded from 0 to +3, and their sum was calculated as the RV score. The right ventricular ejection fraction (RVEF and the left ventricular ejection fraction were obtained by Tc-99m RBC cardiac scintigraphy. The groups with marked visualization of the right ventricle had lower RVEF, and there was a good correlation between the RVEF and the RV score with both a and BMIPP. Although a good correlation was demonstrated between the RV score with Tl and BMIPP in Groups A and B, in Group C, in which there was marked RV Tl visualization, the RV score with BMIPP was significantly smaller than with Tl. These findings suggest that impaired fatty acid metabolism may exist in severely hypertrophic right ventricle due to RV overload. (K.H.)

  2. An Introduction to the Septal Extension Graft

    Directory of Open Access Journals (Sweden)

    Myung-Hoon Kim

    2014-01-01

    Full Text Available The septal extension graft is a very useful method of controlling nasal lengthening and tip projection, rotation, and shape by fixing a graft to the septum, which leads to a strong supporting structure. Enhancing graft stability is important for better long-term outcomes and minimizing complications or relapse, and even more efficient application of these methods is needed for East Asians who lack enough cartilage to be harvested in addition to possessing a weak cartilage framework. In this paper, the methods for overcoming the drawbacks of the septal extension graft, such as instability, a fixed tip, and insufficiency of cartilage, are presented, and the applications of each method for greater satisfaction with surgical outcomes are also discussed.

  3. Left ventricular remodelling in chronic primary mitral regurgitation: implications for medical therapy.

    Science.gov (United States)

    McCutcheon, Keir; Manga, Pravin

    Surgical repair or replacement of the mitral valve is currently the only recommended therapy for severe primary mitral regurgitation. The chronic elevation of wall stress caused by the resulting volume overload leads to structural remodelling of the muscular, vascular and extracellular matrix components of the myocardium. These changes are initially compensatory but in the long term have detrimental effects, which ultimately result in heart failure. Understanding the changes that occur in the myocardium due to volume overload at the molecular and cellular level may lead to medical interventions, which potentially could delay or prevent the adverse left ventricular remodelling associated with primary mitral regurgitation. The pathophysiological changes involved in left ventricular remodelling in response to chronic primary mitral regurgitation and the evidence for potential medical therapy, in particular beta-adrenergic blockers, are the focus of this review.

  4. Thallium-201 myocardial imaging for evaluation of right-ventricular overloading

    International Nuclear Information System (INIS)

    Kondo, M.; Kubo, A.; Yamazaki, H.; Ohsuzu, F.; Handa, S.; Tsugu, T.; Masaki, H.; Kinoshita, F.; Hashimoto, S.

    1978-01-01

    This study evaluated the specificity and sensitivity of Tl-201 myocardial imaging in the detection of right-ventricular (RV) overloading. Right-ventricular visualization (RVV) after administration of Tl-201 chloride was studied on 99 patients with various heart diseases. Tracer uptake in the free wall of the RV was graded in four degrees. The degree of RVV was compared with the findings of cardiac catheterization. The comparisons indicated that the uptake increased in step with the inreases in RV systolic pressure, RV end-diastolic pressure, mean pulmonary arterial pressure, total pulmonary vascular resistance, and stroke-work index of the right ventricle (P < 0.05--P < 0.001). Of the patients with visible RV, all but three had RV overloading, and all but three of those without RVV had normal RV systolic pressure. Myocardial images also reflect the type of RV overloading. In patients with RV pressure overloading, the septum showed a tendency to appear straight. In patients with atrial septal defect leading to RV volume overloading, the RV cavity was dilated, the LV image small, and the septum convex toward the RV cavity. These results indicate that Tl-201 myocardial imaging is a sensitive and specific method for the study of RV overloading

  5. Classificação anatômica e correção cirúrgica da atresia pulmonar com comunicação interventricular Anatomical classification and surgical repair of the pulmonary atresia with ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Ulisses Alexandre CROTI

    2001-12-01

    Full Text Available OBJETIVO: Analisar as características anatômicas, o resultado das técnicas empregadas na correção cirúrgica de acordo com o número de procedimentos, assim como a mortalidade em cada grupo da classificação de Barbero-Marcial para atresia pulmonar com comunicação interventricular. CASUÍSTICA E MÉTODOS: De janeiro de 1990 a novembro de 1999, 73 pacientes que foram submetidos a estudo cineangiocardiográfico previamente à primeira intervenção cirúrgica, foram analisados. As características anatômicas das artérias pulmonares e artérias colaterais sistêmico-pulmonares, assim como as técnicas cirúrgicas que propiciaram tratamento paliativo, "paliativo definitivo" e definitivo foram estudadas. As causas de mortalidade também foram descritas. RESULTADOS: Dezenove pacientes apresentavam os segmentos pulmonares supridos por artérias pulmonares (grupo A, 45 por artérias pulmonares e artérias colaterais sistêmico-pulmonares (grupo B e 9 somente por artérias colaterais sistêmico-pulmonares (grupo C. O grupo A apresentou maior proporção de tratamentos definitivos, o grupo B maior proporção de paliativos e o grupo C, maior proporção de "paliativos definitivos" (pOBJECTIVE: To analyze the morphological aspects, the surgical results obtained according to the number of procedures, and the mortality in each group of Barbero-Marcial´s classification of the pulmonary atresia with ventricular septal defect. MATERIAL E METHODS: From January 1990 to November 1999, 73 patients submitted to cardiac catheterization and detailed pulmonary angiographic study before the first surgical intervention were analyzed. The anatomical characteristics of the pulmonary arteries and major aorticopulmonary collaterals, as the surgical techniques of definitive, palliative and "definitive palliative" were studied. The causes of mortality were also described. RESULTS: Nineteen patients had all the pulmonary segments supplied by pulmonary arteries (group

  6. Brain natriuretic peptide is not predictive of dilated cardiomyopathy in Becker and Duchenne muscular dystrophy patients and carriers.

    Science.gov (United States)

    Schade van Westrum, Steven; Dekker, Lukas; de Haan, Rob; Endert, Erik; Ginjaar, Ieke; de Visser, Marianne; van der Kooi, Anneke

    2013-07-16

    Cardiomyopathy is reported in Duchenne and Becker muscle dystrophy patients and female carriers. Brain Natriuretic peptide (BNP) is a hormone produced mainly by ventricular cardiomyocytes and its production is up regulated in reaction to increased wall stretching. N-terminal-proBNP (NT-proBNP) has been shown to be a robust laboratory parameter to diagnose and monitor cardiac failure, and it may be helpful to screen for asymptomatic left ventricular dysfunction. Therefore we tested whether NT-proBNP can distinguish patients with Duchenne or Becker muscular dystrophy patients and carriers of a dystrophin mutation with a dilated cardiomyopathy from those without. In a cohort of Duchenne and Becker muscle dystrophy patients (n = 143) and carriers (n = 219) NT-proBNP was measured, and echocardiography was performed to diagnose dilated cardiomyopathy (DCM). In total sixty-one patients (17%) fulfilled the criteria for DCM, whereas 283 patients (78%) had an elevated NT-pro BNP. The sensitivity of NT-proBNP for DCM in patients or carriers was 85%, the specificity 23%, area under the ROC-curve = 0.56. In the specified subgroups there was also no association. Measurement of NT-pro BNP in patients suffering from Duchenne or Becker muscular dystrophy and carriers does not distinguish between those with and without dilated cardiomyopathy.

  7. Systolic Compression of Epicardial Coronary and Intramural Arteries

    Science.gov (United States)

    Mohiddin, Saidi A.; Fananapazir, Lameh

    2002-01-01

    It has been suggested that systolic compression of epicardial coronary arteries is an important cause of myocardial ischemia and sudden death in children with hypertrophic cardiomyopathy. We examined the associations between sudden death, systolic coronary compression of intra- and epicardial arteries, myocardial perfusion abnormalities, and severity of hypertrophy in children with hypertrophic cardiomyopathy. We reviewed the angiograms from 57 children with hypertrophic cardiomyopathy for the presence of coronary and septal artery compression; coronary compression was present in 23 (40%). The left anterior descending artery was most often affected, and multiple sites were found in 4 children. Myocardial perfusion abnormalities were more frequently present in children with coronary compression than in those without (94% vs 47%, P = 0.002). Coronary compression was also associated with more severe septal hypertrophy and greater left ventricular outflow gradient. Septal branch compression was present in 65% of the children and was significantly associated with coronary compression, severity of septal hypertrophy, and outflow obstruction. Multivariate analysis showed that septal thickness and septal branch compression, but not coronary compression, were independent predictors of perfusion abnormalities. Coronary compression was not associated with symptom severity, ventricular tachycardia, or a worse prognosis. We conclude that compression of coronary arteries and their septal branches is common in children with hypertrophic cardiomyopathy and is related to the magnitude of left ventricular hypertrophy. Our findings suggest that coronary compression does not make an important contribution to myocardial ischemia in hypertrophic cardiomyopathy; however, left ventricular hypertrophy and compression of intramural arteries may contribute significantly. (Tex Heart Inst J 2002;29:290–8) PMID:12484613

  8. Septo-Hippocampo-Septal Loop and Memory Formation

    Directory of Open Access Journals (Sweden)

    Fatemeh Khakpai

    2013-01-01

    Full Text Available   Cholinergic and GABAergic fibers in the medial septal/diagonal band of Broca (MS/DB area project to the hippocampus and constitute the septo-hippocampal pathway, which has been proven in learning and memory. In addition, the hippocampus has bidirectional connections with the septum, which use this relation for self-regulation of cholinergic input.   The activity of septal and hippocampal neurons is modulated by several neurotransmitters including glutamatergic neurons from the entorhinal cortex, serotonergic fibers from the raphe nucleus, dopaminergic neurons from the ventral tegmental area (VTA, histaminergic cells from the tuberomammillary nucleus and adrenergic fibers from the locus coeruleus (LC. Thus, changes in the glutamatergic, serotonergic and etc. mediated transmission in the MS/DB may influence cholinergic or GABAergic transmission in the hippocampus.

  9. Septo-Hippocampo-Septal Loop and Memory Formation

    Directory of Open Access Journals (Sweden)

    Fatemeh Khakpai

    2013-02-01

    Full Text Available The Cholinergic and GABAergic .bers of the medial septal/diagonal band of Broca (MS/DB area project to the hippocampus and constitute the septo-hippocampal pathway, which has been proven to play a role in learning and memory. In addition, the hippocampus has bidirectional connections with the septum so that to self-regulate of cholinergic input. The activity of septal and hippocampal neurons is modulated by several neurotransmitter systems including glutamatergic neurons from the entorhinal cortex, serotonergic .bers from the raphe nucleus, dopaminergic neurons from the ventral tegmental area (VTA, histaminergic cells from the tuberomammillary nucleus and adrenergic .bers from the locus coeruleus (LC. Thus, changes in the glutamatergic, serotonergic and other systems- mediated transmission in the MS/DB may in.uence cholinergic or GABAergic transmission in the hippocampus.

  10. Ultrastructural aspects in perithecia hyphae septal pores of Glomerella cingulata F. SP. Phaseoli

    Directory of Open Access Journals (Sweden)

    Roca M. María Gabriela

    2000-01-01

    Full Text Available Glomerella cingulata (Stonem. Spauld. & Schrenk f. sp. phaseoli, better known in its anamorphic state Colletotrichum lindemuthianum (Sacc. & Magn. Briosi & Cav., is a causal agent of anthracnose in beans (Phaseolus vulgaris L.. Ultrastructural aspects of the perithecial hyphae of this pathogen were studied. The perithecia hyphae septal pores were found either plugged by a vesicle or unplugged. Some perithecia hyphae septa presented no pore. The Woronin bodies, close to the septal pores, appeared as globose structures which were more electron dense than the occlusions plugging the septal pore.

  11. Evaluation of left ventricular function in patient with old myocardial infarction by 201-thallium myocardial scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Takahashi, Kanji; Shimohara, Yasuaki; Ito, Misao; Okada, Keisei [Kure Kyosai Hospital, Hiroshima (Japan); Kodama, Kazunori

    1984-08-01

    Correlation between the ratio of myocardial defect calculated by 201-thallium myocardial scintigraphy and the left ventricular ejection fraction (LVEF) obtained by gated blood pool scintigraphy and the maximum level of creatine phosphokinase (CPK) was studied in 70 patients with old myocardial infarction. There was a significant correlation between the defect ratio and the LVEF or CPK level in patients with anterior or septal myocardial infarction. In all patients with inferior myocardial infarction in whom no defect was seen, the LVEF was more than 40%. However, no distinct correlation between the defect ratio and the LVEF or CPK level was obtained in cases of inferior myocardial infarction.

  12. Association of the beta-1 adrenergic receptor carboxyl terminal variants with left ventricular hypertrophy among diabetic and non-diabetic survivors of acute myocardial infarction

    Directory of Open Access Journals (Sweden)

    Hakalahti Anna E

    2010-08-01

    Full Text Available Abstract Background The beta-1 adrenergic receptor (β1AR plays a fundamental role in the regulation of cardiovascular functions. It carries a nonsynonymous single nucleotide polymorphism in its carboxyl terminal tail (Arg389Gly, which has been shown to associate with various echocardiographic parameters linked to left ventricular hypertrophy (LVH. Diabetes mellitus (DM, on the other hand, represents a risk factor for LVH. We investigated the possible association between the Arg389Gly polymorphism and LVH among non-diabetic and diabetic acute myocardial infarction (AMI survivors. Methods The study population consisted of 452 AMI survivors, 20.6% of whom had diagnosed DM. Left ventricular parameters were measured with two-dimensional guided M-mode echocardiography 2-7 days after AMI, and the Arg389Gly polymorphism was determined using a polymerase chain reaction-restriction fragment length polymorphism assay. Results The Arg389 homozygotes in the whole study population had a significantly increased left ventricular mass index (LVMI when compared to the Gly389 carriers (either Gly389 homozygotes or Arg389/Gly389 heterozygotes [62.7 vs. 58.4, respectively (p = 0.023]. In particular, the Arg389 homozygotes displayed thicker diastolic interventricular septal (IVSd measures when compared to the Gly389 carriers [13.2 vs. 12.3 mm, respectively (p = 0.004]. When the euglycemic and diabetic patients were analyzed separately, the latter had significantly increased LVMI and diastolic left ventricular posterior wall (LVPWd values compared to the euglycemic patients [LVMI = 69.1 vs. 58.8 (p = 0.001 and LVPWd = 14.2 vs. 12.3 mm (p Conclusions The data suggest an association between the β1AR Arg389Gly polymorphism and LVH, particularly the septal hypertrophy. The Arg389 variant appears to confer a higher risk of developing LVH than the corresponding Gly389 variant among patients who have suffered AMI. This association cannot be considered to be universal

  13. Preoperative Pulmonary Valvuloplasty in Tetralogy of Fallot with Right-To-Left Shunt

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    Baris Bugan

    2014-12-01

    Full Text Available Tetralogy of Fallot is the most common cyanotic congenital heart disease and characterized by right ventricular outflow tract obstruction, ventricular septal defect, overriding aorta, and right ventricular hypertrophy. Right ventricular outflow tract obstruction and ventricular septal defect are the major clinical components of the syndrome. Although most have undergone a corrective operation, an important minority of patients with tetralogy of Fallot have had a preoperative palliative procedure. Herein we reported a succesful palliative percutaneous balloon valvuloplasty of pulmonary stenosis at an 19-year-old female patient with tetralogy of Fallot who was considered as inoperable for corrective surgery due to right -to-left shunt.

  14. Caudal Septal Stabilization Suturing Technique to Treat Crooked Noses.

    Science.gov (United States)

    Baykal, Bahadir; Erdim, Ibrahim; Guvey, Ali; Oghan, Fatih; Kayhan, Fatma Tulin

    2016-10-01

    To rotate the nasal axis and septum to the midline using an L-strut graft and a novel caudal septal stabilization suturing technique to treat crooked noses. Thirty-six patients were included in the study. First, an L-strut graft was prepared by excising the deviated cartilage site in all patients. Second, multiple stabilization suturing, which we describe as a caudal septal stabilization suturing technique with a "fishing net"-like appearance, was applied between the anterior nasal spine and caudal septum in all patients. This new surgical technique, used to rotate the caudal septum, was applied to 22 I-type and 14 C-type crooked noses. Correction rates for the crooked noses were compared between the 2 inclination types with angular estimations. Deviation angles were measured using the AutoCAD 2012 software package and frontal (anterior) views, with the Frankfurt horizontal line parallel to the ground. Nasal axis angles showing angle improvement graded 4 categories as excellent, good, acceptable, and unsuccessful for evaluations at 6 months after surgery in the study. The success rate in the C-type nasal inclination was 86.7% (±21.9) and 88% (±16.7) in the I-type. The overall success rate of L-strut grafting and caudal septal stabilization suturing in crooked nose surgeries was 87.5% (±18.6). "Unsuccessful" results were not reported in any of the patients. L-strut grafting and caudal septal stabilization suturing techniques are efficacious in crooked noses according to objective measurement analysis results. However, a longer follow-up duration in a larger patient population is needed.

  15. Clinical impact of left ventricular eccentricity index using cardiac MRI in assessment of right ventricular hemodynamics and myocardial fibrosis in congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Yamasaki, Yuzo; Kamitani, Takeshi; Yamanouchi, Torahiko; Honda, Hiroshi [Kyushu University, Departments of Clinical Radiology, Graduate School of Medical Sciences, Fukuoka (Japan); Nagao, Michinobu; Kawanami, Satoshi [Kyushu University, Molecular Imaging and Diagnosis, Graduate School of Medical Sciences, Fukuoka (Japan); Yamamura, Kenichiro [Kyushu University, Pediatrics, Graduate School of Medical Sciences, Fukuoka (Japan); Sakamoto, Ichiro [Kyushu University, Cardiovascular Medicine, Graduate School of Medical Sciences, Fukuoka (Japan); Yabuuchi, Hidetake [Kyushu University, Health SciencesGraduate School of Medical Sciences, Fukuoka (Japan)

    2016-10-15

    To investigate the utility of eccentricity index (EI) using cardiac cine MRI for the assessment of right ventricular (RV) hemodynamics in congenital heart disease (CHD). Fifty-five patients with CHD (32 women; mean age, 40.7 ± 20.9 years) underwent both cardiac MRI and right heart catheterization. EI was defined as the ratio of the distance between the anterior-posterior wall and the septal-lateral wall measured in the short-axis of mid-ventricular cine MRI. Correlations between EIs and RV hemodynamic parameters were analyzed. EIs were compared between patients with and without late gadolinium enhancement (LGE). A strong correlation between mean pulmonary artery pressure (PAP) and systolic EI (r = 0.81, p < 0.0001) and a moderate negative correlation between diastolic EI and RV ejection fraction (EF) (r = -0.62, p < 0.0001) were observed. Receiver operating characteristic analysis revealed optimal EI thresholds for detecting patients with mean PAP ≥40 mmHg with C-statistics of 0.90 and patients with RVEF <40 % with C-statistics of 0.78. Systolic EIs were significantly greater for patients with LGE (1.45 ± 0.05) than for those without LGE (1.15 ± 0.07; p < 0.001). EI offers a simple, comprehensive index that can predict pulmonary hypertension and RV dysfunction in CHD. (orig.)

  16. Evaluation of cardiac function in patients with Duchenne's muscular dystrophy by single photon emission computed tomography (SPECT)

    Energy Technology Data Exchange (ETDEWEB)

    Tamura, Takuhisa; Motomura, Masakatsu; Kanazawa, Hajime; Shibuya, Noritoshi (Kawatana Byoin National Sanatorium, Nagasaki (Japan))

    1989-06-01

    The extent of myocardial ischemia was evaluated in 20 patients with Duchenne's muscular dystrophy (DMD) by using Bull's eye method of thallium-201 myocardial SPECT. It was examined in relation to skeletal muscle involvement, age, left ventricular (LV) ejection fraction and ventricular premature contractions (VPCs). Myocardial ischemia was detected in all of patients with DMD. Ischemic lesion was mostly detected in the apical side of the LV lateral wall and interventricular septum, while the extent of myocardial ischemia had no correlations with either the stage of functional disability of skeletal muscle or age. The more ischemic ratio was higher, the more LV ejection fraction decreased. The total number of VPCs was relatively small and it did not have any relation to myocardial ischemic ratio. These results suggest that younger DMD patients having extensive myocardial ischemia and/or ventricular tachycardia will have a high risk of cardiac death. (author).

  17. Hybrid closure of atrial septal defect: A modified approach

    Directory of Open Access Journals (Sweden)

    Kshitij Sheth

    2015-01-01

    Full Text Available A 3.5-year-old girl underwent transcatheter closure of patent ductus arteriosus in early infancy during which time her secundum atrial septal defect (ASD was left alone. When she came for elective closure of ASD, she was found to have bilaterally blocked femoral veins. The defect was successfully closed with an Amplatzer septal occluder (ASO; St. Jude Medical, Plymouth, MN, USA using a hybrid approach via a sub-mammary mini-thoracotomy incision without using cardiopulmonary bypass. At the end of 1-year follow-up, the child is asymptomatic with device in a stable position without any residual shunt.

  18. Survival and sudden cardiac death after septal ablation for hypertrophic obstructive cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten Kvistholm; Havndrup, Ole; Hassager, Christian

    2011-01-01

    Reports of long-term survival and the risk of sudden cardiac death (SCD) after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) are sparse.......Reports of long-term survival and the risk of sudden cardiac death (SCD) after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) are sparse....

  19. Cardiac involvement in Beagle-based canine X-linked muscular dystrophy in Japan (CXMDJ: electrocardiographic, echocardiographic, and morphologic studies

    Directory of Open Access Journals (Sweden)

    Machida Noboru

    2006-12-01

    Full Text Available Abstract Background Cardiac mortality in Duchenne muscular dystrophy (DMD has recently become important, because risk of respiratory failure has been reduced due to widespread use of the respirator. The cardiac involvement is characterized by distinctive electrocardiographic abnormalities or dilated cardiomyopathy, but the pathogenesis has remained obscure. In research on DMD, Golden retriever-based muscular dystrophy (GRMD has attracted much attention as an animal model because it resembles DMD, but GRMD is very difficult to maintain because of their severe phenotypes. We therefore established a line of dogs with Beagle-based canine X-linked muscular dystrophy in Japan (CXMDJ and examined the cardiac involvement. Methods The cardiac phenotypes of eight CXMDJ and four normal male dogs 2 to 21 months of age were evaluated using electrocardiography, echocardiography, and histopathological examinations. Results Increases in the heart rate and decreases in PQ interval compared to a normal littermate were detected in two littermate CXMDJ dogs at 15 months of age or older. Distinct deep Q-waves and increase in Q/R ratios in leads II, III, and aVF were detected by 6–7 months of age in all CXMDJ dogs. In the echocardiogram, one of eight of CXMDJ dogs showed a hyperechoic lesion in the left ventricular posterior wall at 5 months of age, but the rest had not by 6–7 months of age. The left ventricular function in the echocardiogram indicated no abnormality in all CXMDJ dogs by 6–7 months of age. Histopathology revealed myocardial fibrosis, especially in the left ventricular posterobasal wall, in three of eight CXMDJ dogs by 21 months of age. Conclusion Cardiac involvement in CXMDJ dogs is milder and has slower progression than that described in GRMD dogs. The distinct deep Q-waves have been ascribed to myocardial fibrosis in the posterobasal region of the left ventricle, but our data showed that they precede the lesion on echocardiogram and

  20. Myocardial metabolism, perfusion, wall motion and electrical activity in Duchenne muscular dystrophy

    International Nuclear Information System (INIS)

    Perloff, J.K.; Henze, E.; Schelbert, H.R.

    1982-01-01

    The cardiomyopathy of Duchenne's muscular dystrophy originates in the posterobasal left ventricle and extends chiefly to the contiguous lateral wall. Ultrastructural abnormalities in these regions precede connective tissue replacement. We postulated that a metabolic fault coincided with or antedated the subcellular abnormality. Accordingly, regional left ventricular metabolism, perfusion and wall motion were studied using positron computed tomography and metabolic isotopes supplemented by thallium perfusion scans, equilibrium radionuclide angiography and M-mode and two-dimensional echocardiography. To complete the assessment, electrocardiograms, vectorcardiograms, 24 hour taped electrocardiograms and chest x-rays were analyzed. Positron computed tomography utilizing F-18 2-fluoro 2-deoxyglucose (FDG) provided the first conclusive evidence supporting the hypothesis of a premorphologic regional metabolic fault. Thus, cardiac involvement in duchenne dystrophy emerges as a unique form of heart disease, genetically targeting specific regions of ventricular myocardium for initial metabolic and subcellular changes. Reported ultrastructural abnormalities of the impulse and conduction systems provide, at least in part, a basis for the clinically observed sinus node, intraatrial, internodal, AV nodal and infranodal disorders

  1. Muscular Dystrophy

    Science.gov (United States)

    ... sets of muscles and cause different degrees of muscle weakness. Duchenne muscular dystrophy is the most common and the most severe ... can walk independently. Prednisone If a child has Duchenne muscular ... to help slow the rate of muscle deterioration. By doing so, the child may be ...

  2. [Nasal septal abscess].

    Science.gov (United States)

    Barril, María F; Ferolla, Fausto M; José, Pablo; Echave, Cecilia; Tomezzoli, Silvana; Fiorini, Sandra; López, Eduardo Luis

    2008-12-01

    A nasal septal abscess (NA) is defined as a collection of pus between the cartilage or bony septum and its normally applied mucoperichondrium or mucoperiostium. It is an uncommon disease which should be suspected in a patient with acute onset of nasal obstruction and recent history of nasal trauma, periodontal infection or an inflammatory process of the rhinosinusal region. We report a case of an 8-year-old boy with bilateral NA caused by community-acquired methicillin-resistant Staphylococcus aureus(MR-CO) in order to emphasize the importance of prompt diagnosis and adequate treatment to prevent the potentially dangerous spread of infection and the development of severe functional and cosmetic sequelae.

  3. Apical ballooning syndrome complicated by acute severe mitral regurgitation with left ventricular outflow obstruction – Case report

    Directory of Open Access Journals (Sweden)

    Celermajer David S

    2007-02-01

    Full Text Available Abstract Background Apical ballooning syndrome (or Takotsubo cardiomyopathy is a syndrome of transient left ventricular apical ballooning. Although first described in Japanese patients, it is now well reported in the Caucasian population. The syndrome mimicks an acute myocardial infarction but is characterised by the absence of obstructive coronary disease. We describe a serious and poorly understood complication of Takotsubo cardiomyopathy. Case Presentation We present the case of a 65 year-old lady referred to us from a rural hospital where she was treated with thrombolytic therapy for a presumed acute anterior myocardial infarction. Four hours after thrombolysis she developed acute pulmonary oedema and a new systolic murmur. It was presumed she had acute mitral regurgitation secondary to a ruptured papillary muscle, ischaemic dysfunction or an acute ventricular septal defect. Echocardiogram revealed severe mitral regurgitation, left ventricular apical ballooning, and systolic anterior motion of the mitral valve with significant left ventricular outflow tract gradient (60–70 mmHg. Coronary angiography revealed no obstructive coronary lesions. She had an intra-aortic balloon pump inserted with no improvement in her parlous haemodynamic state. We elected to replace her mitral valve to correct the outflow tract gradient and mitral regurgitation. Intra-operatively the mitral valve was mildly myxomatous but there were no structural abnormalities. She had a mechanical mitral valve replacement with a 29 mm St Jude valve. Post-operatively, her left ventricular outflow obstruction resolved and ventricular function returned to normal over the subsequent 10 days. She recovered well. Conclusion This case represents a serious and poorly understood association of Takotsubo cardiomyopathy with acute pulmonary oedema, severe mitral regurgitaton and systolic anterior motion of the mitral valve with significant left ventricular outflow tract obstruction. The

  4. Nasal airway and septal variation in unilateral and bilateral cleft lip and palate.

    Science.gov (United States)

    Starbuck, John M; Friel, Michael T; Ghoneima, Ahmed; Flores, Roberto L; Tholpady, Sunil; Kula, Katherine

    2014-10-01

    Cleft lip and palate (CLP) affects the dentoalveolar and nasolabial facial regions. Internal and external nasal dysmorphology may persist in individuals born with CLP despite surgical interventions. 7-18 year old individuals born with unilateral and bilateral CLP (n = 50) were retrospectively assessed using cone beam computed tomography. Anterior, middle, and posterior nasal airway volumes were measured on each facial side. Septal deviation was measured at the anterior and posterior nasal spine, and the midpoint between these two locations. Data were evaluated using principal components analysis (PCA), multivariate analysis of variance (MANOVA), and post-hoc ANOVA tests. PCA results show partial separation in high dimensional space along PC1 (48.5% variance) based on age groups and partial separation along PC2 (29.8% variance) based on CLP type and septal deviation patterns. MANOVA results indicate that age (P = 0.007) and CLP type (P ≤ 0.001) significantly affect nasal airway volume and septal deviation. ANOVA results indicate that anterior nasal volume is significantly affected by age (P ≤ 0.001), whereas septal deviation patterns are significantly affected by CLP type (P ≤ 0.001). Age and CLP type affect nasal airway volume and septal deviation patterns. Nasal airway volumes tend to be reduced on the clefted sides of the face relative to non-clefted sides of the face. Nasal airway volumes tend to strongly increase with age, whereas septal deviation values tend to increase only slightly with age. These results suggest that functional nasal breathing may be impaired in individuals born with the unilateral and bilateral CLP deformity. © 2014 Wiley Periodicals, Inc.

  5. Assessment of cardiac blood pool imaging in patients with left ventricular outflow tract stenosis

    International Nuclear Information System (INIS)

    Nakamura, Yutaka; Ono, Yasuo; Kohata, Tohru; Tsubata, Shinichi; Kamiya, Tetsuroh.

    1993-01-01

    We performed cardiac blood pool imagings with Tc-99m at rest and during supine ergometer exercise to evaluate left ventricular performance in 14 patients with left ventricular outflow tract stenosis. All catheterized patients were divided into two subgroups: 8 patients with peak systolic left ventricular to descending aortic pressure gradients of less than 50 mmHg (LPG group) and 6 patients with peak systolic gradients of more than 50 mmHg (HPG group). Control group included 10 patients without stenotic coronary lesions after Kawasaki disease. Left ventricular ejection fraction (LVEF) was obtained as systolic index; both filling fraction during the first third of diastole (1/3FF) and mean filling rate during the first third of diastole (1/3FR mean) were obtained as diastolic indices. None of the patients had abnormal findings on 201 Tl imaging. LVEF at rest in HPG group was significantly higher than those in control group, but LVEF in HPG group did not increase after exercise. It increased significantly in control group and LPG group. 1/3 FF in HPG group was significantly lower not only at rest but also during exercise. 1/3 FR mean at rest was not different significantly among the 3 groups. However, 1/3FR mean during exercise in LPG group was significantly lower; and 1/3 FR mean during exercise was significantly lower in HPG group than LPG group. The ratio of left ventricular muscular mass to left ventricular end-diastolic volume (M/V) calculated from left ventricular cineangiograms was different significantly among the 3 groups. The M/V ratio showed a correlation with LVEF and 1/3 FF both at rest and during exercise. These results would indicate that systolic function was impaired on exercise in severe left ventricular outflow tract stenosis and diastolic function was impaired on exercise in mild and severe left ventricular outflow tract stenosis. This may correlate with left ventricular hypertrophy and interaction of systolic function. (author)

  6. Predictors of transient left ventricular dysfunction following transcatheter patent ductus arteriosus closure in pediatric age.

    Science.gov (United States)

    Agha, Hala Mounir; Hamza, Hala S; Kotby, Alyaa; Ganzoury, Mona E L; Soliman, Nanies

    2017-10-01

    To evaluate the left ventricular function before and after transcatheter percutaneous patent ductus arteriosus (PDA) closure, and to identify the predictors of myocardial dysfunction post-PDA closure if present. Transcatheter PDA closure; conventional, Doppler, and tissue Doppler imaging; and speckle tracking echocardiography. To determine the feasibility and reliability of tissue Doppler and myocardial deformation imaging for evaluating myocardial function in children undergoing transcatheter PDA closure. Forty-two children diagnosed with hemodynamically significant PDA underwent percutaneous PDA closure. Conventional, Doppler, and tissue Doppler imaging, and speckle-derived strain rate echocardiography were performed at preclosure and at 48 hours, 1 month, and 6 months postclosure. Tissue Doppler velocities of the lateral and septal mitral valve annuli were obtained. Global and regional longitudinal peak systolic strain values were determined using two-dimensional speckle tracking echocardiography. The median age of the patients was 2 years and body weight was 15 kg, with the mean PDA diameter of 3.11 ± 0.99 mm. M-mode measurements (left ventricular end diastolic diameter, left atrium diameter to aortic annulus ratio, ejection fraction, and shortening fraction) reduced significantly early after PDA closure ( p  closure causes a significant decrease in left ventricular performance early after PDA closure, which recovers completely within 1 month. Preclosure global longitudinal strain can be a predictor of postclosure myocardial dysfunction.

  7. Identification and Assessment of Paradoxical Ventricular Wall Motion Using ECG Gated Blood Pool Scan - Comparison of cine Loop , Phase Analysis and Paradox Image -

    International Nuclear Information System (INIS)

    Lee, Jae Tae; Kim, Gwang Weon; Lee, Kyu Bo; Chung, Byung Chun; Whang, Kee Suk; Chae, Sung Chul; Paek, Wee Hyun; Cheon, Jae Eun; Lee, Hyong Woo; Chung, Jin Hong

    1990-01-01

    Sixty-four patients with paradoxical ventricular wall motion noticed both in angiocardiography or 2-dimensional echocardiography were assessed by ECG gated blood pool scan (GBPS). Endless cine loop image, phase and amplitude images and paradox image obtained by visual inspection of each cardiac beat or Fourier transformation of acquired raw data were investigated to determine the incremental value of GBPS with these processing methods for identification of paradoxical ventricular wall motion. The results were as follows:1) Paradoxical wall motions were observed on interventricular septum in 34 cases, left ventricular free wall in 26 and right ventricular wall in 24. Underlying heart diseases were is chemic (23 cases) valvular(9), congenital heart disease (12), cardiomyopathy (5), pericardial effusion(5), post cardiac surgery(3), corpulmonale (2), endocarditis (l) and right ventricular tumor(l). 2) Left ventricular ejection fractions of patients with paradoxical left ventricular wall motion were significantly lower than those with paradoxical septal motion (p <0.005). 3) The sensitivity of each processing methods for detecting paradoxical wall motion was 76.9% by phase analysis, 74.6% by endless cine loop mapping and 68.4% by paradox image manipulation respectively. Paradoxial motions visualized only in phase, paradox or both images were appeared as hypokinesia or akinesia in cine loop image. 4) All events could be identified by at least one of above three processing methods, however only 34 cases (48.4%) showed the paradoxical motions in all of the three images. By these findings, we concluded that simultaneous inspection of all above three processing methods-endless cine loop, phase analysis and paradox image is necessary for accurate identification and assessment of paradoxical ventricular wall motion when performing GBPS.

  8. Identification and Assessment of Paradoxical Ventricular Wall Motion Using ECG Gated Blood Pool Scan - Comparison of cine Loop , Phase Analysis and Paradox Image -

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jae Tae; Kim, Gwang Weon; Lee, Kyu Bo; Chung, Byung Chun; Whang, Kee Suk; Chae, Sung Chul; Paek, Wee Hyun; Cheon, Jae Eun [Kyungpook National University School of Medicine, Daegu (Korea, Republic of); Lee, Hyong Woo; Chung, Jin Hong [Yeongnam National University College of Medicine, Daegu (Korea, Republic of)

    1990-07-15

    Sixty-four patients with paradoxical ventricular wall motion noticed both in angiocardiography or 2-dimensional echocardiography were assessed by ECG gated blood pool scan (GBPS). Endless cine loop image, phase and amplitude images and paradox image obtained by visual inspection of each cardiac beat or Fourier transformation of acquired raw data were investigated to determine the incremental value of GBPS with these processing methods for identification of paradoxical ventricular wall motion. The results were as follows:1) Paradoxical wall motions were observed on interventricular septum in 34 cases, left ventricular free wall in 26 and right ventricular wall in 24. Underlying heart diseases were is chemic (23 cases) valvular(9), congenital heart disease (12), cardiomyopathy (5), pericardial effusion(5), post cardiac surgery(3), corpulmonale (2), endocarditis (l) and right ventricular tumor(l). 2) Left ventricular ejection fractions of patients with paradoxical left ventricular wall motion were significantly lower than those with paradoxical septal motion (p <0.005). 3) The sensitivity of each processing methods for detecting paradoxical wall motion was 76.9% by phase analysis, 74.6% by endless cine loop mapping and 68.4% by paradox image manipulation respectively. Paradoxial motions visualized only in phase, paradox or both images were appeared as hypokinesia or akinesia in cine loop image. 4) All events could be identified by at least one of above three processing methods, however only 34 cases (48.4%) showed the paradoxical motions in all of the three images. By these findings, we concluded that simultaneous inspection of all above three processing methods-endless cine loop, phase analysis and paradox image is necessary for accurate identification and assessment of paradoxical ventricular wall motion when performing GBPS.

  9. Effects of exercise training on pulmonary vessel muscularization and right ventricular function in an animal model of COPD

    OpenAIRE

    Hassel, Erlend; Berre, Anne Marie; Skjulsvik, Anne Jarstein; Steinshamn, Sigurd

    2014-01-01

    Background Right ventricular dysfunction in COPD is common, even in the absence of pulmonary hypertension. The aim of the present study was to examine the effects of high intensity interval training (HIIT) on right ventricular (RV) function, as well as pulmonary blood vessel remodeling in a mouse model of COPD. Methods 42 female A/JOlaHsd mice were randomized to exposure to either cigarette smoke or air for 6 hours/day, 5 days/week for 14 weeks. Mice from both groups were further randomized t...

  10. [Primary, single-stage arterial switch operations at a newly-established, comprehensive congenital cardiac center performed in the neonatal age and beyond].

    Science.gov (United States)

    Király, László; Tamás, Csaba

    2015-06-21

    Outcome of arterial switch operation for transposition of the great arteries with/without ventricular septal defect is a service key-performance-indicator. The aim of the authors was to assess patient characteristics and parameters in the perioperative course. In the setting of a newly-established, comprehensive tertiary-care center, primary complete repair was performed including associated anomalies, e.g. transverse arch repairs. Patients with d-transposition were grouped according to coexistence of ventricular septal defect. 118 arterial switch operations were performed between 2007 and 2014 with 96.62% survival (114/118). Ventricular septal defect and repair of associated anomalies did not yield worse outcome. Left ventricular re-training with late presentation necessitated mechanical circulatory support for 4.5±1.5 days. D-transposition is suitable for standardization of clinical algorithm and surgical technique. Quality standards contribute to excellent outcomes, minimize complications, and serve as blueprint for other neonatal open-heart procedures. Availability of mechanical circulatory support is key for single-stage left ventricular re-training beyond the neonatal period.

  11. A case of Becker muscular dystrophy with early manifestation of cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Ki Hyun Doo

    2012-09-01

    Full Text Available An 18-year-old boy was admitted with chest discomfort, nausea, and dyspnea at rest. At the age of 3 years, he underwent muscle biopsy and dystrophin gene analysis owing to an enlarged calf muscle and elevated serum kinase level (6,378 U/L without overt weakness; based on the results, Becker muscular dystrophy (BMD was diagnosed. The dystrophin gene showed deletion of exons 45 to 49. He remained ambulant and could step upstairs without significant difficulties. A chest roentgenogram showed cardiomegaly (cardiothoracic ratio, 54%, and his electrocardiogram (ECG showed abnormal ST-T wave, biatrial enlargement, and left ventricular hypertrophy. The 2-dimensional and M-mode ECGs showed a severely dilated left ventricular cavity with diffuse hypokinesis. The systolic indices were reduced, including fractional shortening (9% and ejection fraction (19%. Despite receiving intensive medical treatment, he died from congestive heart failure 5 months after the initial cardiac symptoms. We report a case of BMD with early-onset dilated cardiomyopathy associated with deletion of exons 45 to 49. Early cardiomyopathy can occur in BMD patients with certain genotypes; therefore, careful follow-up is required even in patients with mild phenotypes of BMD.

  12. Anatomy of a wrong diagnosis: false Sinus Venosus Atrial Septal Defect

    Directory of Open Access Journals (Sweden)

    Montresor Graziano

    2003-11-01

    Full Text Available Abstract In contrast with transthoracic echocardiography, transesophageal echocardiography provides a sure way to make the diagnosis of sinus venosus atrial septal defect; on the other hand this abnormality is more complex than that seen with the secundum atrial septal defect, and inexperienced operators may fail to recognize properly the defect. In front of a high reported sensitivity using transesophageal echocardiography, specificity is difficult to assess, due to possible underreporting of diagnostic errors. We describe a false positive diagnosis of sinus venosus atrial septal defect, in the setting of enlarged right chambers of the heart because of pressure overload. Modified anatomy of the heart, together with the presence of a prominent linear structure(probably Eustachian Valve and an incomplete examination in this case made image interpretation very prone to misinterpretation. In this anatomical setting transesophageal longitudinal "bicaval" view may be sub-optimal for examining the atrial septum, potentially showing false images that need to be known for correct image interpretation. Nonetheless, a scan plane taken more accurately at the superior level would have demonstrated/excluded the pathognomonic feature of sinus venosus atrial septal defect in the high atrial septum, between the fatty limbus and the inferior aspect of the right pulmonary artery; moreover TEE allows morphological information about the posterior structures of the heart that need to be investigated in detail for a complete diagnosis.

  13. Scar Homogenization Versus Limited-Substrate Ablation in Patients With Nonischemic Cardiomyopathy and Ventricular Tachycardia.

    Science.gov (United States)

    Gökoğlan, Yalçın; Mohanty, Sanghamitra; Gianni, Carola; Santangeli, Pasquale; Trivedi, Chintan; Güneş, Mahmut F; Bai, Rong; Al-Ahmad, Amin; Gallinghouse, G Joseph; Horton, Rodney; Hranitzky, Patrick M; Sanchez, Javier E; Beheiry, Salwa; Hongo, Richard; Lakkireddy, Dhanunjaya; Reddy, Madhu; Schweikert, Robert A; Dello Russo, Antonio; Casella, Michela; Tondo, Claudio; Burkhardt, J David; Themistoclakis, Sakis; Di Biase, Luigi; Natale, Andrea

    2016-11-01

    Scar homogenization improves long-term ventricular arrhythmia-free survival compared with standard limited-substrate ablation in patients with post-infarction ventricular tachycardia (VT). Whether such benefit extends to patients with nonischemic cardiomyopathy and scar-related VT is unclear. The aim of this study was to assess the long-term efficacy of an endoepicardial scar homogenization approach compared with standard ablation in this population. Consecutive patients with dilated nonischemic cardiomyopathy (n = 93), scar-related VTs, and evidence of low-voltage regions on the basis of pre-defined criteria on electroanatomic mapping (i.e., bipolar voltage homogenization and standard ablation, respectively (p = 0.01). During a mean follow-up period of 14 ± 2 months, single-procedure success rates were 63.9% after scar homogenization and 38.6% after standard ablation (p = 0.031). After multivariate analysis, scar homogenization and left ventricular ejection fraction were predictors of long-term success. During follow-up, the rehospitalization rate was significantly lower in the scar homogenization group (p = 0.035). In patients with dilated nonischemic cardiomyopathy, scar-related VT, and evidence of low-voltage regions on electroanatomic mapping, endoepicardial homogenization of the scar significantly increased freedom from any recurrent ventricular arrhythmia compared with a standard limited-substrate ablation. However, the success rate with this approach appeared to be lower than previously reported with ischemic cardiomyopathy, presumably because of the septal and midmyocardial distribution of the scar in some patients. Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  14. Effect of Mitral Annular Calcium on Left Ventricular Diastolic Parameters.

    Science.gov (United States)

    Codolosa, Jose N; Koshkelashvili, Nikoloz; Alnabelsi, Talal; Goykhman, Igor; Romero-Corral, Abel; Pressman, Gregg S

    2016-03-01

    Assessment of left ventricular (LV) diastolic function by Doppler flow imaging and tissue Doppler is an integral part of the echocardiographic examination. Mitral annular calcium (MAC) is frequently encountered on echocardiography. The aim of this study was to assess the impact of MAC, quantitatively measured by computed tomography scan, on echocardiographic LV diastolic parameters. We included 155 patients aged ≥65 years. Computed tomography reconstructions of the mitral annulus were created, and calcium identified and quantified by Agatston technique. Calcium locations were assigned using an overlaid template depicting the annular segments in relation to surrounding anatomic structures. Echocardiographic assessment of diastolic function was performed in standard fashion. Mean age was 77 years; 49% were men; and 43% were black. Patients with MAC had lower septal e' (p = 0.003), lateral e' (p = 0.04), and average e' (p = 0.01) compared with those without MAC. They also had a higher E-wave velocity (p = 0.01) and E/e' ratio (p <0.001). When evaluated by severity of MAC, and after adjustment for multiple clinical factors, there was a graded (inverse) relation between MAC severity and septal e' (p = 0.01), lateral e' (p = 0.01), and average e' (p = 0.01). In conclusion, LV diastolic parameters, as measured by Doppler echocardiography, are altered in the presence of MAC. This could be due to direct effects of MAC on annular function or might reflect truly reduced diastolic function. Interpretation of diastolic parameters in patients with MAC should be performed with caution. Copyright © 2016 Elsevier Inc. All rights reserved.

  15. Growth activity in human septal cartilage: age-dependent incorporation of labeled sulfate in different anatomic locations

    International Nuclear Information System (INIS)

    Vetter, U.; Pirsig, W.; Heinze, E.

    1983-01-01

    Growth activity in different areas of human septal cartilage was measured by the in vitro incorporation of 35 S-labeled NaSO 4 into chondroitin sulfate. Septal cartilage without perichondrium was obtained during rhinoplasty from 36 patients aged 6 to 35 years. It could be shown that the anterior free end of the septum displays high growth activity in all age groups. The supra-premaxillary area displayed its highest growth activity during prepuberty, showing thereafter a continuous decline during puberty and adulthood. A similar age-dependent pattern in growth activity was found in the caudal prolongation of the septal cartilage. No age-dependent variations could be detected in the posterior area of the septal cartilage

  16. Clinical evaluation of left ventricular wall thickness by combined technique with gated planer 201Tl image and gated cardiac pool image

    International Nuclear Information System (INIS)

    Nakai, Kenji; Katsuragawa, Shigehiko; Takahashi, Tsuneo; Matsushita, Kazuo; Kawamura, Akiyoshi

    1983-01-01

    To evaluate the left ventricular (LV) wall thickness, combined technique with gated planer 201-Thallium image and gated cardiac pool image was applied to 6 patients with hypertrophic cardiomyopathy (HCM) and 4 patients with secondary hypertrophy due to hypertension (HHD) proven by electrocardiography and ultrasonic-echocardiography. Scintigraphic pattern of hypertrophy on reconstructed planer 201 Tl image showed diffuse or asymmetrical apical hypertrophy in HHD, asymmetrical septal hypertrophy in HCM. It was very interesting that abnormal perfusion was shown in 201 Tl image, despite symmetrical hypertrophy in echocardiography. This techniques provided useful information for evaluating the LV wall thickness and cardiac performance. (author)

  17. The Role of Heart Team Approach in Penetrating Cardiac Trauma: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Marzia Cottini

    Full Text Available Abstract Penetrating cardiac trauma has been increasing in clinical experience and is joined to important morbidity and mortality. A case of a 38-year-old female with history of postpartum depression was reported, admitted to our department for cardiac tamponade due to penetrating self-inflicted multiple stab wound of the chest complicated by rupture of anterior left ventricular wall and traumatic ventricular septal defect. Following the unstable hemodynamic instability, a combined therapeutic strategy was chosen: surgery and transcatheter implantation to correct free wall ventricle damage and traumatic ventricular septal defect, respectively.

  18. Device and method for treatment of openings in vascular and septal walls

    Science.gov (United States)

    Singhal, Pooja; Wilson, Thomas S.; Cosgriff-Hernandez, Elizabeth; Maitland, Duncan J.

    2017-06-06

    A device, system and method for treatment of an opening in vascular and/or septal walls including patent foramen ovale. The device has wings/stops on either end, an axis core covered in a shape memory foam and is deliverable via a catheter to the affected opening, finally expanding into a vascular or septal opening where it is held in place by the expandable shape memory stops or wings.

  19. Atrial Septal Aneurysm Presenting as Clubbing without Clinically Apparent Cyanosis.

    Science.gov (United States)

    Goyal, Laxmi Kant; Banerjee, S; Yadav, R N; Singh, Gajraj; Ganguli, Sujata; Isran, Rohit

    2015-09-01

    Atrial septal aneurysm (ASA) is a localised "saccular" deformity which protrudes to the right or the left atrium or on both sides. It is a rare, but well recognised cardiac abnormality. It is usually an incidental finding or may presents as atrial arrhythmias or arterial embolism. Though it is an acyanotic congenital heart disease but it may result in significant right to left shunt and cyanosis. We describe a patient of ASA with atrial septal defect who presented with clubbing and right to left shunt without clinically apparent cyanosis. © Journal of the Association of Physicians of India 2011.

  20. Limb-Girdle Muscular Dystrophy (LGMD)

    Science.gov (United States)

    ... Marie-Tooth Disease (CMT) Congenital Muscular Dystrophy (CMD) Duchenne Muscular Dystrophy (DMD) Emery-Dreifuss Muscular Dystrophy Endocrine Myopathies Metabolic Diseases of Muscle Mitochondrial Myopathies (MM) Myotonic Dystrophy (DM) Spinal-Bulbar ...

  1. Evaluation of Limb-Girdle Muscular Dystrophy

    Science.gov (United States)

    2014-03-06

    Becker Muscular Dystrophy; Limb-Girdle Muscular Dystrophy, Type 2A (Calpain-3 Deficiency); Limb-Girdle Muscular Dystrophy, Type 2B (Miyoshi Myopathy, Dysferlin Deficiency); Limb-Girdle Muscular Dystrophy, Type 2I (FKRP-deficiency)

  2. Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation

    Directory of Open Access Journals (Sweden)

    Rupesh Kumar

    2014-01-01

    Full Text Available Holt-Oram syndrome is a rare inherited disorder involving the hands, arms, and the heart. The defects involve carpal bones of the wrist and the thumb and the associated cardiac anomalies like atrial or ventricular septal defects. Congenital cardiac and upper-limb malformations frequently occur together and are classified as heart-hand syndromes. The most common amongst the heart-hand disorders is the Holt-Oram syndrome, which is characterized by septal defects of the heart and preaxial radial ray abnormalities. Its incidence is one in 100,000 live births. Approximately three out of four patients have some cardiac abnormality with common associations being either an atrial septal defect or ventricular septal defect. Herein, we report a rare sporadic case of Holt-Oram syndrome with atrial septal defect with symptoms of heart failure in a forty-five-year-old lady who underwent emergency cardiac surgery for the symptoms.

  3. Cor triatriatum dexter and atrial septal defect in a 43-year-old woman.

    Science.gov (United States)

    Vukovic, Petar M; Kosevic, Dragana; Milicic, Miroslav; Jovovic, Ljiljana; Stojanovic, Ivan; Micovic, Slobodan

    2014-08-01

    Cor triatriatum dexter is a rare congenital heart anomaly in which a membrane divides the right atrium into 2 chambers. We report the case of a 43-year-old woman who had cor triatriatum dexter and a large atrial septal defect. During attempted percutaneous closure, the balloon disrupted the membrane and revealed that the defect had no inferior rim, precluding secure placement of an Amplatzer Septal Occluder. Surgical treatment subsequently proved to be successful. In patients with an incomplete membrane and a septal defect with well-defined rims, percutaneous treatment can be the first choice. In patients who have cor triatriatum dexter and unfavorable anatomic features or concomitant complex heart anomalies, open-heart surgery remains the gold standard for treatment.

  4. Molecular Diffusion through Cyanobacterial Septal Junctions

    Directory of Open Access Journals (Sweden)

    Mercedes Nieves-Morión

    2017-01-01

    Full Text Available Heterocyst-forming cyanobacteria grow as filaments in which intercellular molecular exchange takes place. During the differentiation of N2-fixing heterocysts, regulators are transferred between cells. In the diazotrophic filament, vegetative cells that fix CO2 through oxygenic photosynthesis provide the heterocysts with reduced carbon and heterocysts provide the vegetative cells with fixed nitrogen. Intercellular molecular transfer has been traced with fluorescent markers, including calcein, 5-carboxyfluorescein, and the sucrose analogue esculin, which are observed to move down their concentration gradient. In this work, we used fluorescence recovery after photobleaching (FRAP assays in the model heterocyst-forming cyanobacterium Anabaena sp. strain PCC 7120 to measure the temperature dependence of intercellular transfer of fluorescent markers. We find that the transfer rate constants are directly proportional to the absolute temperature. This indicates that the “septal junctions” (formerly known as “microplasmodesmata” linking the cells in the filament allow molecular exchange by simple diffusion, without any activated intermediate state. This constitutes a novel mechanism for molecular transfer across the bacterial cytoplasmic membrane, in addition to previously characterized mechanisms for active transport and facilitated diffusion. Cyanobacterial septal junctions are functionally analogous to the gap junctions of metazoans.

  5. Tissue doppler echocardiography for evaluating left ventricular functions in obese

    Directory of Open Access Journals (Sweden)

    Mecnun Çetin

    2013-03-01

    Full Text Available Objective: The aim of this study was to investigate theimpact of childhood obesity on ventricular functions usingtissue Doppler echocardiography.Materials and methods: The mitral tissue Doppler signalswere recorded in the apical four-chamber view, with thesample volume placed at the lateral walls of mitral annulus.The mitral flow was examined with the sample Pulse Dopplerpositioned parallely to flow just at the orifice of the mitralleaflets. Twenty obese (mean age, 161.5±25.8 monthsand 20 healthy children (mean age, 151.2±33.5 monthswere included to this study.Results: Body mass index (BMI was significantly higherin obese group (30.92±6.87. Isovolumic relaxation time(IVRT which is one of the left ventricular (LV diastolicfunction parameters was 66.10±7.30 ms in obese group,and 58.70±9.06 ms in the control group. IVRT was significantlyhigher in the obese group (p=0.007. In obesegroup, the mitral annulus tissue Doppler E velocity wasdecreased, flow velocity was increased and decrement inEm/Am ratio was significant. We found significant negativecorrelation between BMI and LV Em/Am ratio. Myocardialperformance index (MPI in obese group and control groupwas 0.50±0.07 and 0.41±0.04, respectively (p<0.001.Between MPI and LV Em/Am ratio there was a significantnegative correlation while MPI showed positive correlationwith BMI. In obese group septal and LV posterior wall thicknesswas shown to be significantly increased compared tothe control group (p<0.001.Conclusion: Our findings, may be important for determinationof the relationship between obesity and cardiovascularrisk factors in children. Tissue Doppler echocardiographicimaging may be useful in revealing this relationship.Key words: obesity, doppler echocardiography, ventricular dysfunction

  6. Identifying Septal Support Reconstructions for Saddle Nose Deformity: The Cakmak Algorithm.

    Science.gov (United States)

    Cakmak, Ozcan; Emre, Ismet Emrah; Ozkurt, Fazil Emre

    2015-01-01

    The saddle nose deformity is one of the most challenging problems in nasal surgery with a less predictable and reproducible result than other nasal procedures. The main feature of this deformity is loss of septal support with both functional and aesthetic implications. Most reports on saddle nose have focused on aesthetic improvement and neglected the reestablishment of septal support to improve airway. To explain how the Cakmak algorithm, an algorithm that describes various fixation techniques and grafts in different types of saddle nose deformities, aids in identifying saddle nose reconstructions that restore supportive nasal framework and provide the aesthetic improvements typically associated with procedures to correct saddle nose deformities. This algorithm presents septal support reconstruction of patients with saddle nose deformity based on the experience of the senior author in 206 patients with saddle nose deformity. Preoperative examination, intraoperative assessment, reconstruction techniques, graft materials, and patient evaluation of aesthetic success were documented, and 4 different types of saddle nose deformities were defined. The Cakmak algorithm classifies varying degrees of saddle nose deformity from type 0 to type 4 and helps identify the most appropriate surgical procedure to restore the supportive nasal framework and aesthetic dorsum. Among the 206 patients, 110 women and 96 men, mean (range) age was 39.7 years (15-68 years), and mean (range) of follow-up was 32 months (6-148 months). All but 12 patients had a history of previous nasal surgeries. Application of the Cakmak algorithm resulted in 36 patients categorized with type 0 saddle nose deformities; 79, type 1; 50, type 2; 20, type 3a; 7, type 3b; and 14, type 4. Postoperative photographs showed improvement of deformities, and patient surveys revealed aesthetic improvement in 201 patients and improvement in nasal breathing in 195 patients. Three patients developed postoperative infection

  7. Effect of myocardial viability in the infarct area on regional left ventricular function

    International Nuclear Information System (INIS)

    Ishii, Toshihiko; Watanabe, Takeshi; Usui, Mikio; Nagai, Yoshikazu

    1990-01-01

    This study explored the relationship between global left ventricular ejection fraction (LVEF), regional LVEF of infarct myocardium, and the viability of the myocardium due to the development of collaterals in the infarct area. Each was evaluated by exercise thallium-201 myocardial SPECT, multigated blood pool scintigraphy and coronary angiography. A total of 68 patients with old anteroseptal myocardial infarction and 23 normal persons were studied. The patients were found to have septal defect on exercise thallium images. According to the appearance of redistribution (RD) on delayed images, the patients were classified as having positive RD (n=42, 62%) or negative RD (n=26, 38%). The global LVEF was compared with regional LVEF of the basal, middle and apical areas within septum, as calculated by multigated blood pool scintigraphy. There was no significant difference in global LVEF between the groups of positive and negative RD patients. However, regional LVEF of the basal and middle areas was significantly larger in the group of positive RD than the group of negative RD. Collaterals were significantly observed in the group of positive RD, as compared with the group of negative RD (53% vs 25%). It was more frequent in cases of higher degree of stenosis. In the group of negative RD, none of the patients had collaterals when coronary stenosis was 90% or less. Redistribution may reflect myocardial viability. Myocardial viability within the infarct area had favorable influences on the regional left ventricular function. Therefore, not only global but also regional left ventricular function is necessary for better understanding of pathophysiology of myocardial infarction.(N.K.)

  8. Clinical validation of the planar radionuclide ventriculography in patients with right ventricular dysfunction.

    Science.gov (United States)

    Bontemps, L; Merabet, Y; Chevalier, P; Itti, R

    2013-01-01

    Gated radionuclide ventriculography (RNV) may be used for the evaluation of the right ventricular function. However, the accuracy of the method should be clinically validated in patients suffering from diseases with specific pathology of the right ventricle (RV) and with possible left ventricular (LV) interaction. Three groups of 15 patients each, diagnosed with arrhythmogenic right ventricular dysplasia (ARVD), pulmonary artery hypertension (PAH) or atrial septal defect (ASD) were compared to a group of normal subjects. The parameters for both ventricles were evaluated separately (ejection fractions: LVEF and RVEF, and intraventricular synchronism quantified as phase standard deviation: LVPSD and RVPSD) as well as the relation or interdependence of the right to left ventricle (RV/LV volume ratio, LV/RV ejection fraction and stroke volume ratios, and interventricular synchronism). All the variables as a whole were analyzed to identify groups of patients according to their functional behaviour. Significant differences were found between the patients and control group for the RV function while the LV function remained mostly within normal limits. When the RV function was considered, the control group and ASD patient group showed differences regarding the ARVD and PAH patients. On evaluating the RV/LV ratios, differences were found between the control group and the ASD group. In the PAH patients, LV function showed differences in relation to the rest of the groups. RNV is a reliable clinical tool to evaluate RV function in patients with RV abnormality. Copyright © 2013 Elsevier España, S.L. and SEMNIM. All rights reserved.

  9. Transposition of the great vessels

    Science.gov (United States)

    ... as atrial septal defect, ventricular septal defect, or patent ductus arteriosus) and how much the blood can ... develop, get worse, or continue after treatment. Prevention Women who plan to become pregnant should be immunized ...

  10. Evaluation of cardiomyopathy in Duchenne muscular dystrophy by Tl-201 myocardial SPECT

    International Nuclear Information System (INIS)

    Jinnouchi, Seishi; Asai, Junko; Inoue, Kenjirou; Hoshi, Hiroaki; Watanabe, Katsushi.

    1985-01-01

    Clinical evaluation of Tl-201 myocardial SPECT in patients with cardiomyopathy of Duchenne type progressive muscular dystrophy (DMD) was reported. Cases subjected to study were 14 DMD and 8 normal. Using a rotating gamma camera system (ZLC7500 and Scintipac 70A), SPECT data were collected for 16 minutes (32 angles x 30 sec/angle, 180 0 ). The hypoperfusion areas of the left ventricular muscle were observed in 10 patients (71 %), which was in posterior wall (71 %), inferior wall (57 %), lateral and anterior wall (43 %) in the order of frequency. The hypoperfusion area became wider with age and stage, exept for some patients. It was suggested that myocardial degeneration in DMD started in posterior wall and then spread in other areas. For the purpose of quantitative evaluation of myocardial damage, Tl-201 myocardial uptake ratio (MUR) and left ventricular muscle volume (LVMV) were calculated using the results obtained by phantom studies. MUR and LVMV were higher in DMD than those of normal individuals (3.6 +- 1.0 %: 2.7 +- 0.3 %, p < 0.05, 190.2 +- 67.5 ml: 157.8 +- 21.5 ml, n.s., respectively). (author)

  11. Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy.

    Science.gov (United States)

    Maron, Martin S; Finley, John J; Bos, J Martijn; Hauser, Thomas H; Manning, Warren J; Haas, Tammy S; Lesser, John R; Udelson, James E; Ackerman, Michael J; Maron, Barry J

    2008-10-07

    Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease characterized by a diverse clinical and phenotypic spectrum. This study reports the prevalence, morphology, clinical course, and management of an underrecognized subgroup of HCM patients with left ventricular apical aneurysms. Of 1299 HCM patients, 28 (2%) were identified with left ventricular apical aneurysms, including a pair of identical twins. Aneurysms were recognized at a wide age range (26 to 83 years), including 12 patients (43%) who were rims, and were associated with transmural (and often more extensive) myocardial scarring identified by late gadolinium enhancement cardiovascular magnetic resonance. Apical aneurysms were recognized by echocardiography in only 16 of 28 patients (57%) but by cardiovascular magnetic resonance in the 12 patients undetected by echocardiography. Left ventricular chamber morphology varied; however, 19 patients (68%) showed an "hourglass" contour, with midventricular hypertrophy producing muscular narrowing and intracavitary gradients in 9 patients (74+/-42 mm Hg). Sarcomeric protein missense mutations known to cause other phenotypic expressions of HCM were present in 3 patients. Over 4.1+/-3.7 years of follow-up, 12 patients (43%) with left ventricular apical aneurysms experienced adverse disease complications (event rate, 10.5%/y), including sudden death, appropriate implantable cardioverter-defibrillator discharges, nonfatal thromboembolic stroke, and progressive heart failure and death. Patients with left ventricular apical aneurysms represent an underappreciated subset in the heterogeneous HCM disease spectrum with important clinical implications, often requiring a high index of suspicion and cardiovascular magnetic resonance for identification. Apical aneurysms in HCM are associated with substantial cardiovascular morbidity and mortality and raise novel treatment considerations.

  12. Testing of RPMI-1640 as a Nutrient Medium for Fresh Semilunar Valve Storage.

    Science.gov (United States)

    1979-01-01

    Tetralogy of Fallot, pulmonary atresia, ventricular septal defect, truncus arteriosis , transposition of the great arteries with intact ventricular septum and...a method of overcoming severe developmental abnormalities such as ventricular atresia, truncus arteriosis , and Tetrology of Fallot. 3 Experimental

  13. Aortic intracardiac echocardiography-guided septal puncture during mitral valvuloplasty.

    Science.gov (United States)

    Akkaya, Emre; Vuruskan, Ertan; Zorlu, Ali; Sincer, Isa; Kucukosmanoglu, Mehmet; Ardic, Idris; Yilmaz, Mehmet Birhan

    2014-01-01

    Transoesophageal echocardiography (TEE) and venous intracardiac echocardiography (ICE) are traditionally used to visualize the interatrial septum (IAS) and the tenting effect of the fossa ovalis in patients undergoing percutaneous balloon mitral valvuloplasty (PBMV). The aim of the present study was to assess the comparative efficacy and safety of arterial (intra-aortic) ICE and venous ICE, compared with TEE (traditional approach), in the patients undergoing PBMV. TEE, aortic ICE, and venous ICE were consecutively performed in 50 patients (40 ± 9 years, 86% female). The images of intracardiac structures were obtained from both aortic and right atrial loci. The IAS was visualized using TEE, aortic ICE, and venous ICE. The mean mitral valve area was 1.14 ± 0.2 cm(2), and the mean left atrial volume index was 57.5 ± 12 mL/m(2). The mean size of the visualized septal length was 48 ± 5 mm by TEE, 51 ± 5 mm by aortic ICE, and 33 ± 6 mm by venous ICE. The Bland-Altman test indicated that the 95% limits of agreement for the measurement of septal diameter ranged from -11.0 to +5.9 mm (mean -2.5 mm) between TEE and aortic ICE, -2.8 to +33.5 mm (mean +15.3 mm) between TEE and venous ICE, and -36.6 to +0.8 mm (mean -17.9 mm) between venous and aortic ICE. Standard venous ICE generally tended to yield smaller values compared with TEE and aortic ICE for the measurement of septal length. Furthermore, the view of fossa ovalis and 'tenting effect' was optimal in 11 patients on venous ICE; however, the fossa ovalis and tip of the needle were well visualized in all patients on aortic ICE (P < 0.001). There were no major complications with the use of aortic ICE. Aortic ICE is a superior alternative to venous ICE and facilitates trans-septal puncture in patients with mitral stenosis.

  14. The characteristics of myocardial fatty acid metabolism in patients with left ventricular hypertrophy

    International Nuclear Information System (INIS)

    Isobe, Naoki; Toyama, Takuji; Hoshizaki, Hiroshi

    1999-01-01

    We evaluated the characteristics of myocardial fatty acid metabolism in patients with left ventricular hypertrophy (LVH). Myocardial imaging with 123 I-beta-methyl iodophenyl pentadecanoic acid (BMIPP) was performed in 28 patients with hypertrophic cardiomyopathy (HCM), 15 patients with hypertensive heart disease (HHD), 13 patients with aortic stenosis (AS) and 8 normal controls (NC). The patients with HCM consisted of 13 patients of asymmetric septal hypertrophy (ASH), 7 patients of diffuse hypertrophy (Diffuse-HCM) and 8 patients of apical hypertrophy (APH). Planar and SPECT images of BMIPP were acquired 15 minutes and 4 hours after tracer injection. Resting 201 Tl SPECT images and echocardiography were also performed on other days. We calculated heart/mediastinum count ratio and washout rate of BMIPP by using planar image. In patients with LVH, the incidence of reduced BMIPP uptake was more frequent than that of reduced 201 Tl uptake. In delayed images, more than 60% of patients with LVH reduced BMIPP uptake, especially remarkable for patients with ASH and APH. The washout rate of all cardiac hypertrophic disorders was tended to be higher than that of normal subjects. Reduced BMIPP uptake was frequently found in septal portion of anterior and inferior wall in patients with ASH, in inferior wall in patients with Diffuse-HCM and HHD, in apex in patients with APH and AS. These results suggest that BMIPP scintigraphy can differentiate three types of cardiac hypertrophy. (author)

  15. Ultrasound tissue characterization does not differentiate genotype, but indexes ejection fraction deterioration in becker muscular dystrophy.

    Science.gov (United States)

    Giglio, Vincenzo; Puddu, Paolo Emilio; Holland, Mark R; Camastra, Giovanni; Ansalone, Gerardo; Ricci, Enzo; Mela, Julia; Sciarra, Federico; Di Gennaro, Marco

    2014-12-01

    The aims of the study were, first, to assess whether myocardial ultrasound tissue characterization (UTC) in Becker muscular dystrophy (BMD) can be used to differentiate between patients with deletions and those without deletions; and second, to determine whether UTC is helpful in diagnosing the evolution of left ventricular dysfunction, a precursor of dilated cardiomyopathy. Both cyclic variation of integrated backscatter and calibrated integrated backscatter (cIBS) were assessed in 87 patients with BMD and 70 controls. The average follow-up in BMD patients was 48 ± 12 mo. UTC analysis was repeated only in a subgroup of 40 BMD patients randomly selected from the larger overall group (15 with and 25 without left ventricular dysfunction). Discrimination between BMD patients with and without dystrophin gene deletion was not possible on the basis of UTC data: average cvIBS was 5.2 ± 1.2 and 5.5 ± 1.4 dB, and average cIBS was 29.9 ± 4.7 and 29.6 ± 5.8, respectively, significantly different (p < 0.001) only from controls (8.6 ± 0.5 and 24.6 ± 1.2 dB). In patients developing left ventricular dysfunction during follow-up, cIBS increased to 31.3 ± 5.4 dB, but not significantly (p = 0.08). The highest cIBS values (34.6 ± 5.3 dB, p < 0.09 vs. baseline, p < 0.01 vs BMD patients without left ventricular dysfunction) were seen in the presence of severe left ventricular dysfunction. Multivariate statistics indicated that an absolute change of 6 dB in cIBS is associated with a high probability of left ventricular dysfunction. UTC analysis does not differentiate BMD patients with or without dystrophin gene deletion, but may be useful in indexing left ventricular dysfunction during follow-up. Copyright © 2014 World Federation for Ultrasound in Medicine & Biology. Published by Elsevier Inc. All rights reserved.

  16. A Case of Refractory Heart Failure in Becker Muscular Dystrophy Improved With Corticosteroid Therapy.

    Science.gov (United States)

    Nakamura, Makiko; Sunagawa, Osahiko; Hokama, Ryo; Tsuchiya, Hiroyuki; Miyara, Takafumi; Taba, Yoji; Touma, Takashi

    2016-09-28

    The patient was a 26 year-old man who was referred to our hospital in June 2011 because of severe heart failure. At age 24 years, he was found to have Becker muscular dystrophy. He received enalapril for cardiac dysfunction; however, he had worsening heart failure and was thus referred to our hospital. Echocardiography showed enlargement of the left ventricle, with a diastolic dimension of 77 mm and ejection fraction of 19%. His condition improved temporarily after an infusion of dobutamine and milrinone. He was then administered amiodarone for ventricular tachycardia; however, he subsequently developed hemoptysis. Amiodarone was discontinued and corticosteroid pulse therapy was administered followed by oral prednisolone (PSL). His creatinine phosphokinase (CPK) level and cardiomegaly improved after the corticosteroid therapy. The PSL dose was reduced gradually, bisoprolol was introduced, and the catecholamine infusion was tapered. A cardiac resynchronization device was implanted; however, the patient's condition gradually worsened, which necessitated dobutamine infusion for heart failure. We readministered 30 mg PSL, which decreased the CPK level and improved the cardiomegaly. The dobutamine infusion was discontinued, and the patient was discharged. He was given 7.5 mg PSL as an outpatient, and he returned to normal life without exacerbation of the heart failure. There are similar reports showing that corticosteroids are effective for skeletal muscle improvement in Duchenne muscular dystrophy; however, their effectiveness for heart failure has been rarely reported. We experienced a case of Becker muscular dystrophy in which corticosteroid therapy was effective for refractory heart failure.

  17. Usefulness of electrocardiography-gated dual-source computed tomography for evaluating morphological features of the ventricles in children with complex congenital heart defects

    International Nuclear Information System (INIS)

    Nakagawa, Motoo; Hara, Masaki; Sakurai, Keita; Asano, Miki; Shibamoto, Yuta; Ohashi, Kazuya

    2011-01-01

    Improved time resolution using dual-source computed tomography (DSCT) enabled adaptation of electrocardiography (ECG)-gated cardiac CT for children with a high heart rate. In this study, we evaluated the ability of ECG-gated DSCT (ECG-DSCT) to depict the morphological ventricular features in patients with congenital heart disease (CHD). Between August 2006 and March 2010, a total of 66 patients with CHD (aged 1 day to 9 years, median 11 months) were analyzed using ECG-DSCT. The type of anomaly was ventricular septal defect (VSD) in 32 (malaligned type in 20, perimembranous type in 7, supracristal type in 3, muscular type in 2), single ventricle (SV) in 11, and corrected transposition of the great arteries (cTGA) in 3. All patients underwent ECG-DSCT and ultrasonography (US). We evaluated the accuracy of diagnosing the type of VSD. For the cases with SV and cTGA, we evaluated the ability to depict anatomical ventricular features. In all 32 cases of VSD, DSCT could confirm the VSD defects, and the findings were identical to those obtained by US. Anatomical configurations of the SV and cTGA were correctly diagnosed, similar to that on US. Our study suggests that ECG-DSCT can clearly depict the configuration of ventricles. (author)

  18. Biomass fuel smoke exposure was associated with adverse cardiac remodeling and left ventricular dysfunction in Peru.

    Science.gov (United States)

    Burroughs Peña, M S; Velazquez, E J; Rivera, J D; Alenezi, F; Wong, C; Grigsby, M; Davila-Roman, V G; Gilman, R H; Miranda, J J; Checkley, W

    2017-07-01

    While household air pollution from biomass fuel combustion has been linked to cardiovascular disease, the effects on cardiac structure and function have not been well described. We sought to determine the association between biomass fuel smoke exposure and cardiac structure and function by transthoracic echocardiography. We identified a random sample of urban and rural residents living in the high-altitude region of Puno, Peru. Daily biomass fuel use was self-reported. Participants underwent transthoracic echocardiography. Multivariable linear regression was used to examine the relationship of biomass fuel use with echocardiographic measures of cardiac structure and function, adjusting for age, sex, height, body mass index, diabetes, physical activity, and tobacco use. One hundred and eighty-seven participants (80 biomass fuel users and 107 non-users) were included in this analysis (mean age 59 years, 58% women). After adjustment, daily exposure to biomass fuel smoke was associated with increased left ventricular internal diastolic diameter (P=.004), left atrial diameter (P=.03), left atrial area (four-chamber) (P=.004) and (two-chamber) (P=.03), septal E' (P=.006), and lateral E' (P=.04). Exposure to biomass fuel smoke was also associated with worse global longitudinal strain in the two-chamber view (P=.01). Daily biomass fuel use was associated with increased left ventricular size and decreased left ventricular systolic function by global longitudinal strain. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  19. Muscular dystrophy in a dog resembling human becker muscular dystrophy.

    Science.gov (United States)

    Baroncelli, A B; Abellonio, F; Pagano, T B; Esposito, I; Peirone, B; Papparella, S; Paciello, O

    2014-05-01

    A 3-year-old, male Labrador retriever dog was presented with clinical signs of progressive exercise intolerance, bilateral elbow extension, rigidity of the forelimbs, hindlimb flexion and kyphosis. Microscopical examination of muscle tissue showed marked variability in myofibre size, replacement of muscle with mature adipose tissue and degeneration/regeneration of muscle fibres, consistent with muscular dystrophy. Immunohistochemical examination for dystrophin showed markedly reduced labelling with monoclonal antibodies specific for the rod domain and the carboxy-terminal of dystrophin, while expression of β-sarcoglycan, γ-sarcoglycan and β-dystroglycan was normal. Immunoblotting revealed a truncated dystrophin protein of approximately 135 kDa. These findings supported a diagnosis of congenital canine muscular dystrophy resembling Becker muscular dystrophy in man. Copyright © 2014 Elsevier Ltd. All rights reserved.

  20. Radial bone graft usage for nasal septal reconstruction

    Directory of Open Access Journals (Sweden)

    Yakup Cil

    2011-01-01

    Full Text Available Background: Although various techniques have been described for correction of crooked and saddle nose deformities, these problems are challenging with high recurrence and revision rates. Conventional septal surgery may not be adequate for nose reconstruction in crooked and saddle nose deformities. Materials and Methods: Between December 2005 and October 2009, six patients with crooked nose and five patients with saddle nose deformities underwent corrective surgery in our clinic. All patients were male, and the mean age was 21 years (range, 19-23 years. We used rigid radial bone graft to prevent redeviation and recurrence following corrective nasal septal surgery. Results: The mean follow-up period was 28 months, ranging from 18 to 46 months. Mean operation time was 4 hours (3-4.5. All patients healed uneventfully. None of the patients required secondary surgery. Conclusions: We believe that radial bone grafts offer a long lasting support in treatment of challenging cases with crooked and saddle nose deformities.

  1. Ventricular Septal Defect (For Teens)

    Science.gov (United States)

    ... of the heart and surrounding organs an electrocardiogram (EKG) , which records the electrical activity of the heart ... couch doing quiet activities like watching TV or reading. Everyone heals differently. Your doctor will let you ...

  2. Facts about Ventricular Septal Defect

    Science.gov (United States)

    ... of the Aorta D-Transposition of the Great Arteries Hypoplastic Left Heart Syndrome Pulmonary Atresia Tetralogy of ... Privacy FOIA No Fear Act OIG 1600 Clifton Road Atlanta , GA 30329-4027 USA 800-CDC-INFO ( ...

  3. Rhabdomyolysis featuring muscular dystrophies.

    Science.gov (United States)

    Lahoria, Rajat; Milone, Margherita

    2016-02-15

    Rhabdomyolysis is a potentially life threatening condition of various etiology. The association between rhabdomyolysis and muscular dystrophies is under-recognized in clinical practice. To identify muscular dystrophies presenting with rhabdomyolysis at onset or as predominant feature. We retrospectively reviewed clinical and laboratory data of patients with a genetically confirmed muscular dystrophy in whom rhabdomyolysis was the presenting or main clinical manifestation. Thirteen unrelated patients (males=6; females=7) were identified. Median age at time of rhabdomyolysis was 18 years (range, 2-47) and median duration between the first episode of rhabdomyolysis and molecular diagnosis was 2 years. Fukutin-related protein (FKRP) muscular dystrophy (n=6) was the most common diagnosis, followed by anoctaminopathy-5 (n=3), calpainopathy-3 (n=2) and dystrophinopathy (n=2). Four patients experienced recurrent rhabdomyolysis. Eight patients were asymptomatic and 3 reported myalgia and exercise intolerance prior to the rhabdomyolysis. Exercise (n=6) and fever (n=4) were common triggers; rhabdomyolysis was unprovoked in 3 patients. Twelve patients required hospitalization. Baseline CK levels were elevated in all patients (median 1200 IU/L; range, 600-3600). Muscular dystrophies can present with rhabdomyolysis; FKRP mutations are particularly frequent in causing such complication. A persistently elevated CK level in patients with rhabdomyolysis warrants consideration for underlying muscular dystrophy. Copyright © 2015 Elsevier B.V. All rights reserved.

  4. Various manifestations of hypertrophic cardiomyopathy on ultrafast computed tomography

    International Nuclear Information System (INIS)

    Sekiya, Tohru; Karikomi, Masahito; Ohshiro, Masaya; Iwakami, Masayoshi; Takamoto, Toshihiko; Sakamoto, Tsuguya

    1992-01-01

    Ultrafast computed tomography was performed in 30 patients with hypertrophic cardiomyopathy and images were assessed on variability of left ventricular hypertrophy, the pattern of left ventricular contraction, right ventricular hypertrophy, dilatation of the left atrium, and thickening of the mitral valve. Fifteen (50.0%) of 30 patients had asymmetric septal hypertrophy, six (20.0%) had diffuse hypertrophy, and nine (30.0%) had apical hypertrophy. In eleven patients with asymmetric septal hypertrophy and two with apical hypertrophy, non-hypertrophied segments in end-diastole showed vigorous contraction. Sixteen patients showed homogeneous left ventricular contraction and one showed partial apical contraction. Right ventricular hypertrophy was noted in 12 patients (40.0%), dilatation of the left atrium in 13 patients (43.3%), and mitral valve thickening in three (10.0%). Ultrafast computed tomography was useful in the evaluation of apical hypertrophy and right ventricular hypertrophy, which could be difficult to obtain by echocardiography. (author)

  5. Força e arquitetura muscular do gémeo interno na bomba muscular venosa

    OpenAIRE

    Peixoto, Flávia; Pinto, Ângela; Kozlova, Veronika; Crisóstomo, Rute

    2015-01-01

    Objetivo: Avaliar e comparar a Força Muscular (FM), Amplitude de Movimento (ADM) e Arquitetura Muscular da bomba muscular venosa em sujeitos com e sem Insuficiência Venosa Crónica (IVC). Relevância: A IVC provoca alterações na função da bomba muscular venosa, no entanto, pouco se conhece acerca das suas repercussões físicas e funcionais. Amostra: Sujeitos com IVC (alterações da tróficas, e úlcera ativa/cicatrizada) e saudáveis. Foram avaliados 33 sujeitos dos quais foram analis...

  6. Cor triatriatum dexter associated with atrial septal defect: Management in a complex clinical case.

    Science.gov (United States)

    Sozzi, Fabiola B; Montanaro, Claudia; Bacà, Laura; Viani, Giacomo M; Zilocchi, Massimo; Canetta, Ciro; Meazza, Roberto; Pavone, Laura; Lombardi, Federico

    2017-11-01

    The coexistence of an atrial septal defect and a prominent eustachian valve is a rare congenital anomaly, rarely reported in literature. Differentiation between a giant eustachian valve and cor triatriatum dexter can be difficult. A case of a large atrial septal defect associated with cor triatriatum dexter diagnosed by echocardiography in an asymptomatic woman is reported. A watchful waiting strategy was adopted. © 2017, Wiley Periodicals, Inc.

  7. There Is No Structural Relationship between Nasal Septal Deviation, Concha Bullosa, and Paranasal Sinus Fungus Balls

    Directory of Open Access Journals (Sweden)

    Tung-Lung Tsai

    2012-01-01

    Full Text Available This study aims to determine the relationship between nasal septal deviation, concha bullosa, and chronic rhinosinusitis by using a definitive pathological and simplified model. Fifty-two consecutive sinus computed tomography scans were performed on patients who received endoscopic sinus surgery and whose final diagnosis was paranasal sinus fungus balls. The incidences of nasal septal deviation and concha bullosa for patients diagnosed with paranasal sinus fungus balls among the study group were 42.3% and 25%, respectively. About 63.6% sinuses with fungus balls were located on the ipsilateral side of the nasal septal deviation, and 46.2% were located on the ipsilateral side of the concha bullosa. When examined by Pearson’s chi-square test and the chi-squared goodness-of-fit test, no significant statistical difference for the presence of paranasal sinus fungus balls between ipsilateral and contralateral sides of nasal septal deviation and concha bullosa was noted (P=0.292 and P=0.593, resp.. In conclusion, we could not demonstrate any statistically significant correlation between the location of infected paranasal sinus, the direction of nasal septal deviation, and the location of concha bullosa, in location-limited rhinosinusitis lesions such as paranasal sinus fungal balls. We conclude that the anatomical variants discussed herein do not predispose patients to rhinosinusitis.

  8. Clinical evaluation of left ventricular wall thickness by combined technique with gated planer /sup 201/Tl image and gated cardiac pool image

    Energy Technology Data Exchange (ETDEWEB)

    Nakai, Kenji; Katsuragawa, Shigehiko; Takahashi, Tsuneo; Matsushita, Kazuo; Kawamura, Akiyoshi

    1983-11-01

    To evaluate the left ventricular (LV) wall thickness, a combined technique with gated planer 201-thallium image and gated cardiac pool image was applied to 6 patients with hypertrophic cardiomyopathy (HCM) and 4 patients with secondary hypertrophy due to hypertension (HHD) proven by electrocardiography and ultrasonic-echocardiography. Scintigraphic pattern of hypertrophy on reconstructed planer /sup 201/Tl image showed diffuse or asymmetrical apical hypertrophy in HHD, asymmetrical septal hypertrophy in HCM. It was very interesting that abnormal perfusion was shown in /sup 201/Tl image, despite symmetrical hypertrophy in echocardiography. This techniques provided useful information for evaluating the LV wall thickness and cardiac performance.

  9. Neurogenesis in the septal and temporal part of the adult rat dentate gyrus.

    Science.gov (United States)

    Bekiari, Chryssa; Giannakopoulou, Aggeliki; Siskos, Nikistratos; Grivas, Ioannis; Tsingotjidou, Anastasia; Michaloudi, Helen; Papadopoulos, Georgios C

    2015-04-01

    Structural and functional dissociation between the septal and the temporal part of the dentate gyrus predispose for possible differentiations in the ongoing neurogenesis process of the adult hippocampus. In this study, BrdU-dated subpopulations of the rat septal and temporal dentate gyrus (coexpressing GFAP, DCX, NeuN, calretinin, calbindin, S100, caspase-3 or fractin) were quantified comparatively at 2, 5, 7, 14, 21, and 30 days after BrdU administration in order to examine the successive time-frames of the neurogenesis process, the glial or neuronal commitment of newborn cells and the occurring apoptotic cell death. Newborn neurons' migration from the neurogenic subgranular zone to the inner granular cell layer and expression of glutamate NMDA and AMPA receptors were also studied. BrdU immunocytochemistry revealed comparatively higher numbers of BrdU(+) cells in the septal part, but stereological analysis of newborn and total granule cells showed an identical ratio in the two parts, indicating an equivalent neurogenic ability, and a common topographical pattern along each part's longitudinal and transverse axis. Similarly, both parts exhibited extremely low levels of newborn glial and apoptotic cells. However, despite the initially equal division rate and pattern of the septal and temporal proliferating cells, their later proliferative profile diverged in the two parts. Dynamic differences in the differentiation, migration and maturation process of the two BrdU-incorporating subpopulations of newborn neurons were also detected, along with differences in their survival pattern. Therefore, we propose that various factors, including developmental date birth, local DG microenvironment and distinct functionality of the two parts may be the critical regulators of the ongoing neurogenesis process, leading the septal part to a continuous, rapid, and less-disciplined genesis rate, whereas the quiescent temporal microenvironment preserves a quite steady, less

  10. Treatment of septal hematomas and abscesses in children

    NARCIS (Netherlands)

    Menger, Dirk Jan; Tabink, Ivar; Nolst Trenité, Gilbert J.

    2007-01-01

    The cartilaginous part of the nasal septum of a child with a septal hematoma or abscess is at risk of destruction. Consequently, the noses of these children can collapse, causing a saddle nose deformity, and in time, the normal outgrowth of both the nose and maxilla win be disturbed. In adulthood,

  11. Stable right ventricular size and function during short-term follow-up in patients with pulmonary regurgitation after tetralogy of Fallot repair

    International Nuclear Information System (INIS)

    Śpiewak, M.; Małek, Ł.A.; Petryka, J.; Biernacka, E.K.; Hoffman, P.; Demkow, M.; Miśko, J.; Rużyłło, W.

    2013-01-01

    Aim: To assess changes in ventricular size and function over time in conservatively treated adult patients with repaired tetralogy of Fallot (TOF) and significant pulmonary regurgitation (PR). Materials and methods: Patients with repaired TOF who had undergone more than one cardiac magnetic resonance study were identified. To confine the cause of ventricular size and function deviation to PR, patients with residual ventricular septal defect, more than mild regurgitation at a valve other than the pulmonary valve, and known coronary artery disease were excluded. Results: The final analysis included 27 adults with PR fraction >20%. During a follow-up of mean 2.1 ± 0.8 years, there was no change in right ventricular (RV) end-diastolic volume (EDV; 162.1 ± 27.6 versus 164 ± 29.6 ml/m 2 , p = 0.5). Left ventricular (LV) EDV showed a small decrease (85.1 ± 16.2 versus 81.5 ± 14.1 ml/m 2 , p = 0.02). The mean PR fraction, PR volume, and peak RV outflow tract gradient did not change. Additionally, both RV ejection fraction (EF) and LVEF remained stable over the follow-up period (48.1 ± 6.5 versus 48.4 ± 6.7%, p = 0.83, and 57.3 ± 5.4 versus 57.2 ± 5.1 %, p = 0.91, respectively). Only two asymptomatic patients (7.4% of the study group) developed symptoms and the remaining did not deteriorate. Conclusion: The RVEDV, RVEF, and LVEF remained stable over a mean follow-up of approximately 2 years in the majority of adult patients after TOF repair with significant PR and a wide range of RVEDV

  12. An Echocardiography Training Program for Improving the Left Ventricular Function Interpretation in Emergency Department; a Brief Report

    Directory of Open Access Journals (Sweden)

    Mary S. Jacob

    2017-06-01

    Full Text Available Introduction: Focused training in transthoracic echocardiography enables emergency physicians (EPs to accurately estimate the left ventricular function. This study aimed to evaluate the efficacy of a brief training program utilizing standardized echocardiography video clips in this regard. Methods: A before and after design was used to determine the efficacy of a 1 hour echocardiography training program using PowerPoint presentation and standardized echocardiography video clips illustrating normal and abnormal left ventricular ejection fraction (LVEF as well as video clips emphasizing the measurement of mitral valve E-point septal separation (EPSS. Pre- and post-test evaluation used unique video clips and asked trainees to estimate LVEF and EPSS based on the viewed video clips. Results: 21 EPs with no prior experience with the echocardiographic technical methods completed this study. The EPs had very limited prior echocardiographic training. The mean score on the categorization of LVEF estimation improved from 4.9 (95% CI: 4.1-5.6 to 7.6 (95%CI: 7-8.3 out of a possible 10 score (p<0.0001. Categorization of EPSS improved from 4.1 (95% CI: 3.1-5.1 to 8.1 (95% CI: 7.6- 8.7 after education (p<0.0001. Conclusions: The results of this study demonstrate a statistically significant improvement of EPs’ ability to categorize left ventricular function as normal or depressed, after a short lecture utilizing a commercially available DVD of standardized echocardiography clips.

  13. The effect of milrinone on right and left ventricular function when used as a rescue therapy for term infants with pulmonary hypertension.

    Science.gov (United States)

    James, Adam T; Corcoran, John D; McNamara, Patrick J; Franklin, Orla; El-Khuffash, Afif F

    2016-01-01

    Milrinone may be an appropriate adjuvant therapy for infants with persistent pulmonary hypertension of the newborn. We aimed to describe the effect of milrinone administration on right and left ventricular function in infants with persistent pulmonary hypertension not responding to inhaled nitric oxide after 4 hours of administration. This is a retrospective review of infants born after or at 34 weeks of gestation with persistent pulmonary hypertension who received milrinone treatment. The primary endpoint was the effect of milrinone on myocardial performance and haemodynamics, including right and left ventricular outputs, tissue Doppler velocities, right ventricle and septal strain, and strain rate. Secondary endpoints examined included duration of inhaled nitric oxide and oxygen support. A total of 17 infants with a mean (standard deviation) gestation and birth weight of 39.8 (2.0) weeks and 3.45 (0.39) kilograms, respectively, were included in the study. The first echocardiogram was performed 15 hours after the commencement of nitric oxide inhalation. Milrinone treatment was started at a median time of 1 hour after the echocardiogram and was associated with an increase in left ventricular output (p=0.04), right ventricular output (p=0.004), right ventricle strain (p=0.01) and strain rate (p=0.002), and left ventricle s` (pmilrinone as an adjunct to nitric oxide is worth further exploration, with preliminary evidence suggesting an improvement in both oxygenation and myocardial performance in this group of infants.

  14. Structural Organization of Muscular Elements of a Skin-Muscular Sac of Trematodes: Literature Survey

    OpenAIRE

    Kanat Kambarovich Akhmetov; Irina Yurievna Chidunchi

    2015-01-01

    The issue of structural organization of muscular elements of a trematodes’ skin-muscular sac is considered in the study. Special attention is paid to an analysis of materials of preceding researches, study of foreign authors and also to additional literature reflecting peculiarities of structure of a trematodes’ body muscular system. The stated issue is insufficiently studied and calls for further researches. A comparative analysis of places of trematodes’ localization, taking into considerat...

  15. [Neonatal anatomical repair of transposition of great vessels associated with atrial septal defect. Apropos of 42 cases].

    Science.gov (United States)

    Planché, C; Serraf, A; Bruniaux, J; Lacour-Gayet, F; Bouchart, F; Losay, J; Touchot, A

    1991-05-01

    The good results obtained by anatomic correction of simple transposition of the great arteries (TGA) in the neonatal period have incited some surgical teams to widen the indications to neonates with TGA associated with ventricular septal defect (VSD). The classical management of these patients is a two stage procedure: banding of the pulmonary artery followed by detransposition, which carries a certain risk. Between January 1985 and June 1990, 42 neonates with TGA and VSD underwent a combined procedure consisting in anatomic correction of the TGA and closure of the VSD. The average age of these patients was 16 days, and the average weight was 3.3 kg. Ten patients had coarctation and 6 underwent a complete one stage correction by an anterior approach. The surgical technique consisted in closing the VSD from the right atrium in 20 patients, from the right ventricle in 11 patients and from the pulmonary artery in 11 patients, associated with detransposition of the great arteries and coronary artery reimplantation. Three children died in the preoperative period (7.1%). In two cases, death was related to malposition of the coronary artery. The third fatality was the result of haemorrhage. There has been one late death three years after surgery. Four patients have been reoperated for stenosis of the right ventricular outflow tract (1 case), recurrence of coarctation (2 cases) and stenosis of the superior vena cava (1 case) and have survived. All patients were followed up for an average period of 26.4 +/- 19 months. They are all in the NYHA Class I without treatment. One patient has mild aortic regurgitation.(ABSTRACT TRUNCATED AT 250 WORDS)

  16. Association of Right Ventricular Pressure and Volume Overload with Non-Ischemic Septal Fibrosis on Cardiac Magnetic Resonance.

    Directory of Open Access Journals (Sweden)

    Jiwon Kim

    Full Text Available Non-ischemic fibrosis (NIF on cardiac magnetic resonance (CMR has been linked to poor prognosis, but its association with adverse right ventricular (RV remodeling is unknown. This study examined a broad cohort of patients with RV dysfunction, so as to identify relationships between NIF and RV remodeling indices, including RV pressure load, volume and wall stress.The population comprised patients with RV dysfunction (EF 6-fold more common in the highest, vs. the lowest, common tertile of PASP and RV size (p<0.001.Among wall stress components, NIF was independently associated with RV chamber dilation and afterload, supporting the concept that NIF is linked to adverse RV chamber remodeling.

  17. Spinal Muscular Atrophy FAQ

    Science.gov (United States)

    ... as ALS (Lou Gehrig’s Disease), cystic fibrosis and Duchenne muscular dystrophy. Approximately 1 in 50 Americans, or about 6 ... Pediatric Neuromuscular Clinical Research Network ( PNCR ) and the Muscular ... is the SMN2 gene? Muscle weakness and atrophy in SMA results from the ...

  18. Cardiac systolic function in cirrhotic patients’ candidate of liver trans-plantation compared with control group

    Directory of Open Access Journals (Sweden)

    Roya Sattarzadeh-Badkoubeh

    2017-02-01

    Full Text Available Background: We assessed different systolic cardiac indices to describe left and right ventricular dysfunction in cirrhotic patients before liver transplantation. Methods: In this case-control study, eighty-one consecutive individuals with the confirmed hepatic cirrhosis and candidate for liver transplantation in the Imam Khomeini Hospital between March 2008 and March 2010 were selected. Thirty-two age and gender cross-matched healthy volunteers were also selected as the control group. A detailed two-dimensional and Doppler echocardiography was obtained in all patients and controls performed by the same operator on the day of admission. Results: Dimensions of both left and right atriums as well as left ventricular end-diastolic volume and basal right ventricular dimension in the cirrhotic group were significantly higher than control group. Left ventricular end-systolic dimensions as well as aortic annulus diameter were not different between the two study groups. Left ventricular outflow tract velocity time integral, isovolumic pre-ejection time, isovolumic relaxation time, stroke volume, left ventricular ejection fraction, IVCT+IVRT+ET, systolic velocity of tricuspid annulus, systolic velocity of basal segment of RV free wall, systolic velocity of basal segment of septal wall, peak strain of septal margin (base, peak strain of septal margin (midpoint, peak strain of lateral margin (midpoint, strain rate of septal margin (base, strain rate of septal margin (midpoint, strain rate of lateral margin (base, strain rate of lateral margin (midpoint, Tei index (left and right ventricles, systolic time interval and tricuspid annular plane systolic excursion were higher in cirrhotic group, significantly, (P< 0.05. Left ventricular ejection time and systolic velocity of mid segment of lateral wall were lower in cirrhotic group, significantly, (P< 0.05. Conclusion: In this study, the effects of liver on heart were volume overload, hyperdynamic state and

  19. [The crooked nose: correction of dorsal and caudal septal deviations].

    Science.gov (United States)

    Foda, H M T

    2010-09-01

    The deviated nose represents a complex cosmetic and functional problem. Septal surgery plays a central role in the successful management of the externally deviated nose. This study included 800 patients seeking rhinoplasty to correct external nasal deviations; 71% of these suffered from variable degrees of nasal obstruction. Septal surgery was necessary in 736 (92%) patients, not only to improve breathing, but also to achieve a straight, symmetric external nose. A graduated surgical approach was adopted to allow correction of the dorsal and caudal deviations of the nasal septum without weakening its structural support to the nasal dorsum or nasal tip. The approach depended on full mobilization of deviated cartilage, followed by straightening of the cartilage and its fixation in the corrected position by using bony splinting grafts through an external rhinoplasty approach.

  20. Dental management of pediatric patients affected by pulmonary atresia with ventricular septal defect: A scoping review.

    Science.gov (United States)

    Garrocho-Rangel, A; Echavarría-García, A-C; Rosales-Bérber, M-A; Flores-Velázquez, J; Pozos-Guillén, A

    2017-07-01

    Congenital Heart Diseases (CHD) involves a wide range of pathological conditions, such as Pulmonary Atresia with Ventricle Septal Defect (PA/VSD). This disorder leads to the systemic circulation of oxygen-poor blood (cyanosis), with associated features and consequences in the oral cavity. Using scoping review methodology for screening and article selection, the primary objectives of this paper were as follows: first, to pose a research question; second, to identify relevant studies in order to answer the research question; third, to select and retrieve the studies; fourth, to chart the critical data, and finally, to collate, summarize, and report the results from the most important articles on the dental management of children affected with PA/VSD. Relevant articles (Randomized Controlled Trials [RCT], reviews, observational studies, and clinical case reports) published over a 10-year period were identified and retrieved from four Internet databases: PubMed; Embase/Ovid; Cochrane Library, and Google Scholar. By title and abstract screening and after removing duplicates, 24 articles were finally included in the present scoping review. According to the extracted data, the following are the most important clinical issues to be considered when treating children with PA/VSD in the dental setting: prevalence of dental caries; prevention of dental disease (oral hygiene and diet); bacteremia and infective endocarditis risk, and child behavior control and treatment under general anesthesia. Pediatric Dentists should bear in mind that early diagnosis and treatment, together a long-term follow-up of children with PA/VSD, continue to be the best approaches for achieving enhanced patient psychological well-being and, in consequence, their good quality of life.

  1. Genetics Home Reference: Fukuyama congenital muscular dystrophy

    Science.gov (United States)

    ... with mental retardation Muscular dystrophy, congenital, Fukuyama type Muscular dystrophy, congenital, with central nervous system involvement Polymicrogyria with muscular dystrophy Related Information How ...

  2. Structure of Masera's Septal Olfactory Organ in Cat (Felis silvestris f. catus - Light Microscopy in Selected Stages of Ontogeny

    Directory of Open Access Journals (Sweden)

    I. Kociánová

    2006-01-01

    Full Text Available The septal organ /SO/ (Masera's organ /MO/ is a chemoreceptor presently considered one of three types of olfactory organs (along with the principal olfactory region and vomeronasal organ. Notwithstanding the septal organ having been first described by Rodolfo Masera in 1943, little is known of the properties of sensory neurons or of its functional significance in chemoreception. Until now the septal organ has been described only in laboratory rodents and some marsupials. This work refers to its existence in the domestic cat (Felis silvestris f. catus. The septal organ can be identified at the end of embryonic period - 27 or 28 days of ontogenesis in cats (the 6th developmental stage of Štěrba - coincident with formation of the principal olfactory region in nasal cavity. At 45 days of ontogenesis (the 9th developmental stage of Štěrba, this septal olfactory organ is of circular or oval shape, 120 μm in diameter, in ventral part of septum nasi, lying caudally to the opening of ductus incisivus. The structure of the epithelium of septal olfactory organ is clearly distinct from the respiratory epithelium of the nasal cavity. It varies in thickness, cellular composition, as well as free surface appearance, and even lack the typical structure of sensory epithelium, in this developmental period. Nerve bundles and glandular acini are lacking in the lamina propria mucosae of the septal organ and in the adjacent tissues. Glands appear as the single non-luminized cords of epithelia extending from the surface. The adjacent respiratory epithelium contains numerous goblet cells.

  3. Ablación miocárdica septal transluminal percutánea en pacientes con miocardiopatía hipertrófica septal obstructiva: Resultados agudos y seguimiento a 3 años

    OpenAIRE

    Llamas-Esperón,Guillermo A.; Loera Pinales,Armando; Sandoval Navarrete,Santiago; Zamora Muciño,Alberto; Ramírez Robledo,Miguel A.; Varela Ortiz,Samuel; Casas Juárez,Ulices; Fuantos Delgado,María de la Luz; Albarrán Domínguez,Javier; Muñoz Sandoval,Rocío; Sandoval Rodríguez,Eufracino; Ruiz Esparza,M. Eugenia

    2009-01-01

    Objetivo: Reportar los resultados agudos y a largo plazo de la ablación miocárdica septal transluminal percutánea (AMSTP) como tratamiento de la miocardiopatía hipertrófica septal obstructiva (MHSO). La AMSTP se considera una alternativa del tratamiento quirúrgico en pacientes con MHSO y sintomatología refractaria a otras opciones de tratamiento. La respuesta aguda suele ser satisfactoria, pero los resultados a largo plazo no se han descrito de forma suficiente. Métodos: Realizamos AMSTP en 8...

  4. Distrofia muscular de Emery-Dreifuss: relato de caso Emery-Dreifuss muscular dystrophy: case report

    Directory of Open Access Journals (Sweden)

    Ana Lucila Moreira Carsten

    2006-06-01

    Full Text Available A distrofia muscular de Emery-Dreifuss é uma forma de distrofia muscular freqüentemente associada a contraturas articulares e defeitos de condução cardíaca, que pode ser causada pela deficiência da proteína emerina na membrana nuclear interna das fibras musculares. Descrevemos o caso de um homem de 19 anos com diminuição de força muscular, hipotrofia nas cinturas escapular e pélvica, disfagia, contraturas articulares em cotovelos e tornozelos, apresentando história familiar compatível com herança ligada ao cromossomo X. A investigação mostrou creatinaquinase sérica elevada, eletrocardiograma com bloqueio atrioventricular de primeiro grau e bloqueio de ramo direito, eletroneuromiografia normal, biópsia muscular com alterações miopáticas e a análise por imuno-histoquímica mostrou deficiência de emerina. São discutidas as manifestações clínicas e genéticas, alterações laboratoriais e eletroneuromiográficas, bem como, a importância do estudo do padrão de herança no aconselhamento genético destas famílias.The Emery-Dreifuss muscular dystrophy is a form of muscular dystrophy that frequently presents early contractures and cardiac conduction defects, caused by emerin deficiency in the inner nuclear membrane of the muscular fibers. A 19-years-old man it presented muscle weakness and hypotrophy in the proximal upper and lower limbs, dysphagia and early contractures in elbows and ankles, with familiar history compatible with X-linked inheritance form. The investigation showed increased serum creatinekinase levels electrocardiogram had a first degree atrioventricular block and right bundle branch block normal electromyography and nerve conduction study muscle biopsy disclosed myopathic characteristics and nuclear protein immunohystochemical analysis showed deficiency of emerin. The clinical and genetics manifestations, laboratorial and electromyography changes, as well as, the study of the pattern of inheritance for

  5. Relaci??n entre la masa muscular, la densidad mineral ??sea, la fuerza muscular, la aptitud funcional y la calidad muscular en personas mayores

    OpenAIRE

    Pati??o Villada, Fredy Alonso

    2015-01-01

    294 p. La tesis pretende determinar la frecuencia de la sarcopenia y osteporosis , problemas que afectan a la salud de las personas mayores, y analizar la relaci??n entre la masa muscular, la densidad mineral ??sea (DMO), la fuerza muscular, la aptitud funcional y la calidad muscular (CM). La muestra estudiada fue la formada por un grupo de 83 hombres y 175 mujeres mayores de Le??n (Espa??a). El estudio transversal eval??a ??ndices de masa grasa y densidad mineral ??sea y niveles d...

  6. Para-muscular and trans-muscular approaches to the lumbar inter-vertebral foramen: an anatomical comparison.

    Science.gov (United States)

    Poetscher, Arthur Werner; Ribas, Guilherme Carvalhal; Yasuda, Alexandre; Nishikuni, Koshiro

    2005-03-01

    Foraminal and extra-foraminal disc herniations comprise up to 11.7% of all lumbar disc herniations. Facetectomy, which had been the classic approach, is now recognized as cause of pain and instability after surgery. Otherwise, posterior lateral approaches through a trans-muscular or a para-muscular technique offer no significant damage to key structures for spinal stability. The surgical anatomy of these approaches has already been described, but they were not compared. In order to quantify the angle of vision towards the intervertebral foramen offered by each technique, 12 fresh cadavers were dissected and studied regarding these approaches. The angle presented by trans-muscular approach was wider in all studied lumbar levels. Surgery through the trans-muscular approach is performed with a better working angle, requiring a smaller resection of surrounding tissues. Therefore, minor surgical trauma can be expected. Our measurements support previously published data that point the trans-muscular approach as the best surgical option.

  7. Orocaecal transit time in Duchenne muscular dystrophy.

    OpenAIRE

    Korman, S H; Bar-Oz, B; Granot, E; Meyer, S

    1991-01-01

    Smooth muscle degeneration may occur in Duchenne muscular dystrophy. We measured fasting orocaecal transit time in patients with advanced Duchenne muscular dystrophy and other muscular dystrophies and in healthy controls. No significant differences were found. In contrast to reports of gastric hypomotility in Duchenne muscular dystrophy, we found no evidence of impaired small intestinal motility.

  8. Septal penetration correction in I-131 imaging following thyroid cancer treatment

    Science.gov (United States)

    Barrack, Fiona; Scuffham, James; McQuaid, Sarah

    2018-04-01

    Whole body gamma camera images acquired after I-131 treatment for thyroid cancer can suffer from collimator septal penetration artefacts because of the high energy of the gamma photons. This results in the appearance of ‘spoke’ artefacts, emanating from regions of high activity concentration, caused by the non-isotropic attenuation of the collimator. Deconvolution has the potential to reduce such artefacts, by taking into account the non-Gaussian point-spread-function (PSF) of the system. A Richardson–Lucy deconvolution algorithm, with and without prior scatter-correction was tested as a method of reducing septal penetration in planar gamma camera images. Phantom images (hot spheres within a warm background) were acquired and deconvolution using a measured PSF was applied. The results were evaluated through region-of-interest and line profile analysis to determine the success of artefact reduction and the optimal number of deconvolution iterations and damping parameter (λ). Without scatter-correction, the optimal results were obtained with 15 iterations and λ  =  0.01, with the counts in the spokes reduced to 20% of the original value, indicating a substantial decrease in their prominence. When a triple-energy-window scatter-correction was applied prior to deconvolution, the optimal results were obtained with six iterations and λ  =  0.02, which reduced the spoke counts to 3% of the original value. The prior application of scatter-correction therefore produced the best results, with a marked change in the appearance of the images. The optimal settings were then applied to six patient datasets, to demonstrate its utility in the clinical setting. In all datasets, spoke artefacts were substantially reduced after the application of scatter-correction and deconvolution, with the mean spoke count being reduced to 10% of the original value. This indicates that deconvolution is a promising technique for septal penetration artefact reduction that

  9. Comparison between minimal right vertical infra-axillary thoracotomy and standard median sternotomy for repair of atrial septal defects

    Directory of Open Access Journals (Sweden)

    Hafize Yaliniz

    2015-10-01

    Conclusion: Minimal right vertical infra-axillary thoracotomy can be performed with favorable cosmetic and clinical results for atrial septal defects closure. Infra-axillary thoracotomy provides a good alternative to standard median sternotomy for patients with atrial septal defects.

  10. Relation of isovolumic times after cardiac resynchronization therapy to improvement in exercise capacity

    NARCIS (Netherlands)

    Jansen, A.H.M.; Peels, C.H.; Bracke, F.A.L.E.; Dantzig, van J.M.; Meijer, A.; van der Voort, P.H.; Gelder, van L.M.; Korsten, H.H.M.; Hemel, van N.M.

    2007-01-01

    M-mode echocardiography (using the septal-to-posterior wall motion delay [SPWMD]) and color-coded tissue Doppler imaging (TDI; using the septal-to-lateral delay in peak systolic velocity) have been proposed for assessment of left ventricular (LV) dyssynchrony and prediction of response to cardiac

  11. Cardiac complications relating to pregnancy and recurrence of disease in the offspring of women with atrioventricular septal defects

    NARCIS (Netherlands)

    Drenthen, W; Pieper, PG; van der Tuuk, K; Roos-Hesselink, JW; Voors, AA; Mostert, B; Mulder, BJM; Moons, P; Ebels, T; van Veldhuisen, DJ

    2005-01-01

    Aims In most pregnancy reports, atrioventricular septal defects (AVSD) are not differentiated from more simple septal defects, thus underestimating the risks of pregnancy. To investigate the magnitude and determinants of risk during pregnancy in female patients with balanced AVSD. Methods and

  12. Prevalence of muscular dystrophy in patients with muscular disorders in Tehran, Iran

    Directory of Open Access Journals (Sweden)

    Khadijeh Hajinaghi Tehrani

    2018-05-01

    Full Text Available Muscular dystrophy is a group of diseases that is characterized by progressive muscle wasting and the weakness of variable distribution and severity. On the basis of the distribution of predominant muscle weakness, there are many different kinds of muscular dystrophy. Some dystrophies are especially frequent in certain populations. There are no studies on the prevalence of muscular dystrophy in Iran. This study was aimed to survey the prevalence of muscular dystrophy among Iranian patients with muscular disorders. This analytical cross-sectional study was conducted on 1000 patients with musculoskeletal disorders who visited the dystrophy association of Bou-Ali Hospital (Tehran from June 2014 to June 2016. Patients’ data were extracted using a checklist that included age, gender, age of onset, family history, findings from clinical diagnostic tests and types of muscular dystrophy. The clinical findings were the results of genetic tests; EMG-NCV; para-clinical findings, including LDH and CPK; and pathological findings. All data were analyzed by SPSS V.22 (IBM Inc., NY with Chi Square and One way ANOVA tests. All analyses were performed with P = 0.05 considered as the threshold of statistical significant. Out of the 337 patients studied, 262 (77.7% were male and 75 (22.3% were female. Subjects had a mean (± SD age of 26.08 (± 11.86 years with an age range of 3 to 59 years. The most common types of muscular dystrophy were found to be Duchenne dystrophy (131 cases, 38.9%, limb-girdle dystrophy (91 cases, 27%, Becker dystrophy (58 cases, 17.2%, FSHD dystrophy (31 cases, 9.2%, and SMA (26 cases, 7.7%, respectively. The results showed that a statistically significant relationship between dystrophy types and gender, age, family history, age of diagnosis, CPK and LDH levels (P < 0.001. There were no statistical relationship between dystrophy types and pathological findings (P = 0.57, EMG-NCV test results (P = 0.062, and genetic findings (P = 0

  13. Detection of long-term progression of myocardial fibrosis in Duchenne muscular dystrophy in an affected family: A cardiovascular magnetic resonance study

    International Nuclear Information System (INIS)

    Walcher, Thomas; Steinbach, Peter; Spiess, Jochen; Kunze, Markus; Gradinger, Robert; Walcher, Daniel; Bernhardt, Peter

    2011-01-01

    Background: Detection of myocardial fibrosis and left ventricular dysfunction in Duchenne muscular dystrophy (DMD) is the corner stone for further therapeutic studies. Little is known about the ability of cardiac magnetic resonance imaging (CMR) to evaluate progression of myocardial fibrosis. Aim of our study was to provide CMR data in a previously genotyped DMD family and to evaluate whether progression of myocardial fibrosis could be visualized. Methods and results: DMD genotypes were available in 14 family members. CMR was performed in 4/5 carrier females, in 2/2 affected males and in one healthy family member with normal genotype. Functional images and late gadolinium enhanced (LGE) images in contiguous short-axis orientation were acquired at baseline and follow-up of 1231 days CMR examination could be repeated in three carrier females, in one affected male and in the healthy subject previously scanned. Mean decrease of left ventricular ejection fraction during the follow-up period was 10.5 ± 11.0%, mean progression of LGE volume 11.7 ± 9.5%. Conclusions: Myocardial fibrosis seems to occur prior to global left ventricular dysfunction in DMD diseased males and carrier females. CMR could be used to evaluate progression of myocardial fibrosis and left ventricular function and may thus serve as an important diagnostic tool in the evaluation of therapeutical options in DMD.

  14. Detection of long-term progression of myocardial fibrosis in Duchenne muscular dystrophy in an affected family: A cardiovascular magnetic resonance study

    Energy Technology Data Exchange (ETDEWEB)

    Walcher, Thomas [Department of Internal Medicine II, University of Ulm, Ulm (Germany); Steinbach, Peter [Institute of Human Genetics, University of Ulm, Ulm (Germany); Spiess, Jochen; Kunze, Markus; Gradinger, Robert; Walcher, Daniel [Department of Internal Medicine II, University of Ulm, Ulm (Germany); Bernhardt, Peter, E-mail: peter.bernhardt@uniklinik-ulm.de [Department of Internal Medicine II, University of Ulm, Ulm (Germany)

    2011-10-15

    Background: Detection of myocardial fibrosis and left ventricular dysfunction in Duchenne muscular dystrophy (DMD) is the corner stone for further therapeutic studies. Little is known about the ability of cardiac magnetic resonance imaging (CMR) to evaluate progression of myocardial fibrosis. Aim of our study was to provide CMR data in a previously genotyped DMD family and to evaluate whether progression of myocardial fibrosis could be visualized. Methods and results: DMD genotypes were available in 14 family members. CMR was performed in 4/5 carrier females, in 2/2 affected males and in one healthy family member with normal genotype. Functional images and late gadolinium enhanced (LGE) images in contiguous short-axis orientation were acquired at baseline and follow-up of 1231 days CMR examination could be repeated in three carrier females, in one affected male and in the healthy subject previously scanned. Mean decrease of left ventricular ejection fraction during the follow-up period was 10.5 {+-} 11.0%, mean progression of LGE volume 11.7 {+-} 9.5%. Conclusions: Myocardial fibrosis seems to occur prior to global left ventricular dysfunction in DMD diseased males and carrier females. CMR could be used to evaluate progression of myocardial fibrosis and left ventricular function and may thus serve as an important diagnostic tool in the evaluation of therapeutical options in DMD.

  15. Dismorfia muscular Muscle dysmorphia

    Directory of Open Access Journals (Sweden)

    Sheila Seleri Marques Assunção

    2002-12-01

    Full Text Available Preocupações mórbidas com a imagem corporal eram tidas até recentemente como problemas eminentemente femininos. Atualmente estas preocupações também têm sido encontradas no sexo masculino. A dismorfia muscular é um subtipo do transtorno dismórfico corporal que ocorre principalmente em homens que, apesar da grande hipertrofia muscular, consideram-se pequenos e fracos. Além de estar associada a prejuízos sociais, ocupacionais, recreativos e em outras áreas do funcionamento do indivíduo, a dismorfia muscular é também um fator de risco para o abuso de esteróides anabolizantes. Este artigo aborda aspectos epidemiológicos, etiológicos e padrões clínicos da dismorfia muscular, além de tecer comentários sobre estratégias de tratamento para este transtorno.Morbid concern over body image was considered, until recently, a female issue. Nowadays, it has been viewed as a common male disorder. Muscle dysmorphia, a subtype of a body dysmorphic disorder, affects men who, despite having clear muscular hypertroph,y see themselves as frail and small. Besides being associated to major social, leisure and occupational dysfunction, muscle dysmorphia is also a risk factor for the abuse of steroids. This article describes epidemiological, etiological and clinical characteristics of muscle dysmorphia and comments on its treatment strategy.

  16. A study of atriphos (ATP) action on muscular circulation in progressive muscular dystrophy by the radioactive xenon clearance technique

    International Nuclear Information System (INIS)

    Chakyrov, B.; Samardzhiev, A.

    1977-01-01

    The effect of intramuscularly and intravenously adminostered atriphos on the muscular circulation was studied with radioactive xenon in 12 children with progressive muscular dystrophy. After combined local intramuscular injection of ATP (atriphos) with the radioactive marker a 12-fold increment of muscular circulation ensues, lasting about 15 minutes. No vasodilatating effect on the muscular flow was oberved after intravenous injection of 20-40 mg of atriphos. It is believed that intramuscular administration of atriphos produced dilatation of capillaries and of the venous part of the muscular circulation. (author)

  17. Limited diagnostic accuracy of gated myocardial perfusion SPECT for wall motion analysis in patients with asymmetric septal hypertrophy

    International Nuclear Information System (INIS)

    Seo, J.H.; Ahn, B.C.; Bae, J.H.; Jeong, S.Y.; Lee, J.; Lee, K.B.

    2004-01-01

    Objective: Although gated SPECT(G-SPECT) using Tc-99m MIBI is well-known diagnostic modality in the evaluation of myocardial perfusion and wall motion analysis, there were limited reports for subjects with asymmetric septal hypertrophy (ASH). This study was performed to evaluate the clinical usefulness of G-SPECT for assessments of myocardial perfusion and wall motion analysis in patients with ASH on 2D-echocardiography(Echo). Methods: Thirty patients (male 18, 59 12 years) with ASH on Echo (septal wall thickness 13 mm and 1.3 times as thick as that of posterior wall) underwent Tc-99m MIBI G-SPECT. Two studies were performed within one month. No patient had experienced any significant cardiac event, nor had changed medical and surgical therapy during the studies. Functional parameters of the left ventricle were acquired with QGS software(AutoQUANTTM). Three experts performed visual interpretation for the presence of septal thickening and perfusion abnormalities on G-SPECT and two experienced cardiologists measured dimension, thickness and wall motion of the left ventricle on Echo. Results: Mean septum thickness measured by Echo was 1.90 0.50 cm, and the septum/posterior wall thickness ratio was 1.85 0.51. On visual SPECT analysis, 14 patients (46.7%) were interpreted as with thickened septum and 17 patients (57%) as with abnormal perfusion. All 3 patients who underwent coronary angiography showed significant luminal stenosis and also had perfusion abnormalities on SPECT. On Echo, only one patient showed septal hypokinesia, who showed anteroseptal infarction on SPECT, and the others showed normal septal wall motion. But 13 patients (54%) among 24 patients showed septal hypokinesia on G-SPECT. Patients with thickened septum on SPECT had thicker septum (2.3 vs 1.6 cm) and higher septum/posterior wall thickness ratio (2.2 vs 1.6) on Echo, compared with patients without septal thickening on SPECT. Conclusions: Although G-SPECT could proffer diagnostic accuracy for

  18. Early-progressive dilated cardiomyopathy in a family with Becker muscular dystrophy related to a novel frameshift mutation in the dystrophin gene exon 27.

    Science.gov (United States)

    Tsuda, Takeshi; Fitzgerald, Kristi; Scavena, Mena; Gidding, Samuel; Cox, Mary O; Marks, Harold; Flanigan, Kevin M; Moore, Steven A

    2015-03-01

    We report a family in which two male siblings with Becker muscular dystrophy (BMD) developed severe dilated cardiomyopathy (DCM) and progressive heart failure (HF) at age 11 years; one died at age 14 years while awaiting heart transplant and the other underwent left ventricular assist device implantation at the same age. Genetic analysis of one sibling showed a novel frameshift mutation in exon 27 of Duchenne muscular dystrophy (DMD) gene (c.3779_3785delCTTTGGAinsGG), in which seven base pairs are deleted and two are inserted. Although this predicts an amino-acid substitution and premature termination (p.Thr1260Argfs*8), muscle biopsy dystrophin immunostaining instead indicates that the mutation is more likely to alter splicing. Despite relatively preserved skeletal muscular performance, both the siblings developed progressive HF secondary to early-onset DCM. In addition, their 7-year-old nephew with delayed gross motor development, mild proximal muscle weakness and markedly elevated serum creatine kinase level (>13 000 IU l(-1)) at 16 months was recently demonstrated to have the familial DMD mutation. Here, we report a novel genotype of BMD with early-onset DCM and progressive lethal HF during early adolescence.

  19. Long-term follow up after transcatheter closure of atrial septal defect and patent foramen ovale in adults

    Directory of Open Access Journals (Sweden)

    Jure Dolenc

    2014-01-01

    Full Text Available Background: The aim of our study was to define long-term electrocardiographic and echocardiographic changes and complications after transcatheter closure of atrial septal defect and patent foramen ovale in adults.Methods: The clinical, electrocardiographic and echocardiographic follow-up of 137 consecutive patients that underwent transcatheter closure of atrial septal defect (51 patients or patent foramen ovale (86 patients in a 10-year period was analyzed retrospectively.Results: In the patent foramen ovale group, we observed no significant postprocedural changes. There were no changes in heart rate, heart rhythm and PR or QRS duration in both groups. In the atrial septal defect group, we observed a leftward shift in the heart axis (p = 0.017, a decrease in the estimated systolic pulmonary artery pressure (p = 0.024, decreased tricuspid early diastolic flow velocity (p = 0.002, a decrease in the right chamber dimensions (p = 0.0004 and interventricular septal movement normalization (p < 0.0001. Most of the complications were mild and occurred early after the procedure. Three early serious complications were documented.Conclusions: No electrocardiographic or echocardiographic changes occurred after patent foramen ovale closure. Atrial septal defect closure is related to significant early morphological and hemodynamic improvement. Postprocedural complications are usually early and mild but serious late complications can occur. For that reason, long-term follow up is recommended in these patients.

  20. Animal experimental research of the endothelialization of home-made atrial septal defect occluder device

    International Nuclear Information System (INIS)

    Chen Mingwu; Zhou Aiqing; Li Feng; Gao Wei; Yu Zhiqing; Tang Ning; Zhang Lan

    2003-01-01

    Objective: To evaluate the endothelialization of Chinese nitinol atrial septal defect occluder device. Methods: Atrial septal defect with controllable size was created by the Brockenborough needle and Rashkind balloon atrial septostomy, the occluder devices were implanted in six piglets (mean weight 7.5 kg). Two pigs were killed each time after 1 month, 3 months and 6 months after the device implantation and then the explanted devices were examined by scanning electron microscope (SEM). Results: The devices were found covering with collagen fibrosis together with diffuse endothelial cells spreading over the primer 1 month after implantation. The implants were covered mostly by neointima 3 months after implantation and completely covered by confluent endothelial cells 6 months after the implantation. Endothelial cells were not found on the smooth marker band at 3 months, however, did exist by 6 months. Conclusions: Home-made atrial septal defect occluder devices were mostly endothelialised 3 months after the implantation and did completely at 6 months

  1. Physiological differences between various types of Eisenmenger syndrome and relation to outcome.

    Science.gov (United States)

    Moceri, Pamela; Kempny, Aleksander; Liodakis, Emmanouil; Alonso Gonzales, Rafael; Germanakis, Ioannis; Diller, Gerhard-Paul; Swan, Lorna; Marino, Philip S; Wort, Stephen J; Babu-Narayan, Sonya V; Ferrari, Emile; Gatzoulis, Michael A; Li, Wei; Dimopoulos, Konstantinos

    2015-01-20

    Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) related to congenital heart disease. Several studies have suggested that the presence and location of the shunt defines the natural history of these patients by influencing right ventricular adaptation to PAH. We aimed to echocardiographically assess differences in cardiac physiology and outcome between various types of ES. In this longitudinal cohort study, 191 patients with ES and non-complex congenital heart disease were recruited, 36 with pre-tricuspid and 155 with post-tricuspid shunts. Patients with pre-tricuspid shunts were older, had higher BNP concentrations and lower exercise tolerance compared to patients with post-tricuspid shunts. Right ventricular (RV) function was impaired in patients with atrial septal defects, with larger right ventricles, impaired systolic function and adaptation. The left ventricular eccentricity index was significantly higher in pre-tricuspid defects. Within post-tricuspid shunts, patients with atrio-ventricular septal defects had better right ventricular function compared to ventricular septal defects, while in those with a patent ductus arteriosus this was worse. There was a trend towards lower mortality in patients with post versus pre-tricuspid shunts, which was significant for patients above the age of 48 years. The presence of a post-tricuspid shunt appears to carry physiological and possibly prognostic benefits in ES compared to patients with pre-tricuspid shunts. This should be borne in mind when management decisions and advanced therapies are considered. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  2. Anatomy of right superior septal artery demonstrated on the coronary CT scan

    Energy Technology Data Exchange (ETDEWEB)

    Takeguchi, Takaya (Mitsui Memorial Hospital, Department of Diagnostic Radiology, Tokyo (Japan); Tokyo Medical and Dental University, Department of Diagnostic Radiology, Tokyo (Japan)); Ibukuro, Kenji; Fukuda, Hozumi; Tobe, Kimiko; Abe, Shoko (Mitsui Memorial Hospital, Department of Diagnostic Radiology, Tokyo (Japan)), Email: kj-ibkr@qd6.so-net.ne.jp

    2012-02-15

    Background. A coronary CT scan allows for non-invasive visualization of the anatomy of a coronary artery in three dimensions compared to the two dimensions afforded by conventional angiography. The septal artery, the main blood source of the interventricular septum, is usually derived from the left anterior descending artery; however, it is occasionally derived from the right coronary artery. Purpose. To analyze the prevalence, origin, diameter, and length of the right superior septal artery (RSSA) demonstrated on a coronary CT scan. Material and Methods. The right superior septal artery was retrospectively reviewed on the reconstructed axial scan images (0.5-mm thickness, 0.25-mm interval) in 1290 consecutive patients who underwent coronary CT scans. All patients were scanned on a 320-row CT scanner. The images were transferred to a workstation to trace the vessel to analyze the origin, diameter, and length. We also compared the length of the RSSA between patients with and without coronary artery stenosis. Results. The RSSA was identified in 51 (3.9%) of 1290 patients. The origin was the proximal portion of the right coronary artery (n = 40) or the right sinus of Valsalva (n 11). The artery co-existed with the conus artery in 15 (29%) of 51 patients. The length was 16-62 mm (mean 31.2 mm +- 10.5), and the diameter was 0.8-2.0 mm (mean 1.3 mm +- 0.2). Longer RSSAs tended to be demonstrated in the patients with coronary artery stenosis rather than with normal coronary arteries (P < 0.05). Conclusion. The right superior septal artery and its anatomical variant could be analyzed with a coronary CT scan. The ability to demonstrate this artery on the coronary CT scan was the same as with coronary angiography. The recognition of this vessel is useful for physicians managing with the diagnosis and treatment of the coronary artery disease

  3. A comparison of swallowing dysfunction in Becker muscular dystrophy and Duchenne muscular dystrophy.

    Science.gov (United States)

    Yamada, Yuka; Kawakami, Michiyuki; Wada, Ayako; Otsuka, Tomoyoshi; Muraoka, Kaori; Liu, Meigen

    2018-06-01

    Swallowing dysfunction has been reported in Duchenne muscular dystrophy (DMD), but has not been studied in Becker muscular dystrophy (BMD). The aims of this study were to report the characteristics of swallowing dysfunction in BMD compared with DMD. The study participants were 18 patients with BMD and 18 patients with DMD. All the patients were examined using videofluorography during swallowing of 5 mL of fluid. The penetration-aspiration scale (P-A scale) and the videofluorographic dysphagia scale (VDS) were used to evaluate dysphagia. Swinyard functional ability stage was not significantly different between the BMD and DMD groups. Rate of aspiration, P-A scale score, and total VDS score did not differ across groups, but the VDS item score for laryngeal elevation was lower in the BMD group than in the DMD group (median scores 4.5 and 9, respectively; p Becker muscular dystrophy (BMD) was not well known. Eighteen patients with BMD and 18 patients with Duchenne muscular dystrophy were examined with videofluorography. Patients with BMD have swallowing problems similar to those observed in patients with DMD.

  4. Morphologic imaging in muscular dystrophies and inflammatory myopathies

    International Nuclear Information System (INIS)

    Degardin, Adrian; Lacour, Arnaud; Vermersch, Patrick; Morillon, David; Cotten, Anne; Stojkovic, Tanya

    2010-01-01

    To determine if magnetic resonance imaging (MR imaging) is useful in the diagnostic workup of muscular dystrophies and idiopathic inflammatory myopathies for describing the topography of muscle involvement. MR imaging was performed in 31 patients: 8 with dystrophic myotony types 1 (n = 4) or 2 (n = 4); 11 with limb-girdle muscular dystrophy, including dysferlinopathy, calpainopathy, sarcoglycanopathy, and dystrophy associated with fukutin-related protein mutation; 3 with Becker muscular dystrophy; and 9 with idiopathic inflammatory myopathies, including polymyositis, dermatomyositis, and sporadic inclusion body myositis. Analysis of T1 images enabled us to describe the most affected muscles and the muscles usually spared for each muscular disease. In particular, examination of pelvis, thigh, and leg muscles demonstrated significant differences between the muscular diseases. On STIR images, hyperintensities were present in 62% of our patients with muscular dystrophies. A specific pattern of muscular involvement was established for each muscular disease. Hyperintensities observed on STIR images precede fatty degeneration and are not specific for inflammatory myopathies. (orig.)

  5. Morphologic imaging in muscular dystrophies and inflammatory myopathies

    Energy Technology Data Exchange (ETDEWEB)

    Degardin, Adrian; Lacour, Arnaud; Vermersch, Patrick [CHU de Lille, Clinique neurologique, Lille (France); Morillon, David; Cotten, Anne [CHRU de Lille, Service de Radiologie Osteoarticulaire, Hopital Roger Salengro, Lille (France); Stojkovic, Tanya [G-H Pitie-Salpetriere, Institut de Myologie, Paris (France)

    2010-12-15

    To determine if magnetic resonance imaging (MR imaging) is useful in the diagnostic workup of muscular dystrophies and idiopathic inflammatory myopathies for describing the topography of muscle involvement. MR imaging was performed in 31 patients: 8 with dystrophic myotony types 1 (n = 4) or 2 (n = 4); 11 with limb-girdle muscular dystrophy, including dysferlinopathy, calpainopathy, sarcoglycanopathy, and dystrophy associated with fukutin-related protein mutation; 3 with Becker muscular dystrophy; and 9 with idiopathic inflammatory myopathies, including polymyositis, dermatomyositis, and sporadic inclusion body myositis. Analysis of T1 images enabled us to describe the most affected muscles and the muscles usually spared for each muscular disease. In particular, examination of pelvis, thigh, and leg muscles demonstrated significant differences between the muscular diseases. On STIR images, hyperintensities were present in 62% of our patients with muscular dystrophies. A specific pattern of muscular involvement was established for each muscular disease. Hyperintensities observed on STIR images precede fatty degeneration and are not specific for inflammatory myopathies. (orig.)

  6. Learning about Duchenne Muscular Dystrophy

    Science.gov (United States)

    ... protein. Often these boys are classified as having Becker muscular dystrophy. Genetic testing (looking at the body's genetic instructions) ... National Library of Medicine Web site Duchenne and Becker muscular dystrophy [ghr.nlm.nih.gov] From Genetics Home Reference ...

  7. Distrofia muscular de Duchenne: análise eletrocardiográfica de 131 pacientes Distrofia muscular de duchenne: análisis electrocardiográfica de 131 pacientes Duchenne muscular dystrophy: electrocardiographic analysis of 131 patients

    Directory of Open Access Journals (Sweden)

    Maria Auxiliadora Bonfim Santos

    2010-05-01

    Full Text Available FUNDAMENTO: É conhecido o envolvimento cardíaco em pacientes com distrofia muscular de Duchenne (DMD. O eletrocardiograma (ECG apresenta algumas alterações típicas na DMD, fato que o torna um exame útil no diagnóstico da lesão cardíaca nessa patologia. OBJETIVO: Avaliar as alterações eletrocardiográficas em pacientes portadores de DMD, correlacionando-as com a idade da população estudada. MÉTODOS: Foram analisados os ECG de 131 pacientes com diagnóstico de DMD. Avaliaram-se diversas variáveis eletrocardiográficas, sendo os pacientes separados em dois grupos: aqueles com e sem alterações, por variável estudada. Fez-se a correlação desses dois grupos com a idade dos pacientes. Foram utilizados os critérios de Garson para estabelecer os parâmetros eletrocardiográficos de normalidade. RESULTADOS: O ECG estava anormal em 78,6% dos pacientes. Todos apresentavam ritmo sinusal. Foram os seguintes os percentuais encontrados para as principais variáveis estudadas: PR curto = 18,3%, ondas R anormais em V1 = 29,7%, ondas Q anormais em V6 = 21,3%, alterações da repolarização ventricular = 54,9%, ondas QS anormais em paredes inferior e/ou lateral alta = 37,4%, distúrbios de condução pelo ramo direito = 55,7%, intervalo QT C prolongado = 35,8% e alargamento do QRS = 23,6%. O teste t não pareado foi utilizado para se estabelecer a correlação da idade com as variáveis eletrocardiográficas estudadas nos dois grupos e, apenas a variável alteração da repolarização mostrou diferença estatisticamente significante. CONCLUSÃO: As alterações eletrocardiográficas na DMD são frequentes, revelando comprometimento cardíaco precoce. Apenas a variável alteração da repolarização ventricular foi mais frequente, porém em faixa etária menor (p FUNDAMENTO: Se sabe de la afectación cardíaca en pacientes con distrofia muscular de Duchenne (DMD. El electrocardiograma (ECG presenta algunas alteraciones típicas en la DMD

  8. The role of septal surgery in management of the deviated nose.

    Science.gov (United States)

    Foda, Hossam M T

    2005-02-01

    The deviated nose represents a complex cosmetic and functional problem. Septal surgery plays a central role in the successful management of the externally deviated nose. This study included 260 patients seeking rhinoplasty to correct external nasal deviations; 75 percent of them had various degrees of nasal obstruction. Septal surgery was necessary in 232 patients (89 percent), not only to improve breathing but also to achieve a straight, symmetrical, external nose as well. A graduated surgical approach was adopted to allow correction of the dorsal and caudal deviations of the nasal septum without weakening its structural support to the dorsum or nasal tip. The approach depended on full mobilization of deviated cartilage, followed by straightening of the cartilage and its fixation in the corrected position by using bony splinting grafts through an external rhinoplasty approach.

  9. Cineangiographic analysis of T.O.F

    Energy Technology Data Exchange (ETDEWEB)

    Yeon, Kyung Mo; Kim, Jong Chul; Han, Man Chung [College of Medicine, Seoul National University, Seoul (Korea, Republic of)

    1985-06-15

    The total 223 cases of tetralogy of Fallot is analysed angiographically, in which selective biplane cinecardioangiography with axial projection has been done at the Department of Radiology, Seoul National University Hospital, in recent 2 year and 4 months, with specific reference to the location of pulmonic stenosis, and associated cardiac anomalies. The summary of the analysis is as follow; 1. Left ventriculogram (long axial oblique view) was necessary to detect the ventricular septal defects including anterior marginal VSD, and was helpful to identify the coronary artery anatomy and presence of PDA and evaluate the size of left ventricle. 2. Right ventriculogram (elongated RAO view) was the best projection for the demonstration of pulmonic infundibulum and was essential to differentiate and localize the ventricular septal defects. 3. In the right ventriculogram (4 chamber view), well demonstrated the right ventricular outflow tract, pulmonary valve, and pulmonary arteries (main, right, left and peripheral), and also permitted the detection of ASD in levophase. 4. The position of ventricular septal defects was perimembranous in 199 cases (89.2%), subarterial in 15 cases (6.8%), and infundibular (intracristal) in 9 cases (4%). Multiple ventricular septal defects (perimembranous and marginal) were found in 11 cases (4.5%). 5. In 35 cases (15.5%), pulmonary stenosis is found only in pulmonary infundibulum. The combination of its components was most commonly anterior wall and conus septum hypertrophy in 24 cases (68.6%). 6. Combined pulmonary stenosis was identified in 188 cases (84.3%). The most common combination was infundibular and pulmonary valvular stenosis in 56 cases (29.8%). 7. Associated cardiac anomalies were right aortic arch in 53 cases (23.8%), persistent left SVC in 17 cases (7.7%), ASD in 13 cases (5.9%), PDA in 9 cases (4,1%), aberrant subclavian artery in 5 cases (2.2%), IVC interruption with azygos continuation in 2 cases, single coronary artery in

  10. A lesão muscular na miastenia grave: estudo de 17 casos com histoquimica muscular

    Directory of Open Access Journals (Sweden)

    Lineu Cesar Werneck

    1982-03-01

    Full Text Available Estudo de 17 biópsias musculares de pacientes com miastenia grave, utilizando técnicas de coloração a fresco e histoquímica muscular. Foram encontradas 15 biópsias musculares anormais, sendo que as principais alterações foram fibras musculares angulares escuras atróficas, excesso de gotículas de gordura na membrana externa das fibras, variação no diâmetro das fibras e atrofia de fibras do tipo II. Os achados foram interpretados como denervação em 11 biópsias, atrofia de fibras do tipo II em 7, infiltrado linfocitário em 4, necrose de fibras musculares com fagocitose em 1 e em 2 biópsias não foi encontrada qualquer anormalidade. Quanto maior o tempo de doença, mais severa foi a anormalidade encontrada. Dois pacientes apresentavam timoma, um miastenia grave congênita, um artrite reumatoide, um neurite hipertrófica intersticial, um tireoidite de Hashimoto e um com síndrome miastênica concomitante. São discutidos os achados anatomopatológicos e sua possível explicação.

  11. Left Ventricular Function after Arterial Switch Operation as Assessed by Two-Dimensional Speckle-Tracking Echocardiography in Patients with Simple Transposition of the Great Arteries.

    Science.gov (United States)

    Malakan Rad, Elaheh; Ghandi, Yazdan; Kocharian, Armen; Mirzaaghayan, Mohammadreza

    2016-07-06

    Background: The late postoperative course for children with transposition of the great arteries (TGA) with an intact ventricular septum (IVS) is very important because the coronary arteries may be at risk of damage during arterial switch operation (ASO). We sought to investigate left ventricular function in patients with TGA/IVS by echocardiography. Methods: From March 2011 to December 2012, totally 20 infants (12 males and 8 females) with TGA/IVS were evaluated via 2-dimensional speckle-tracking echocardiography (2D STE) more than 6 months after they underwent ASO. A control group of age-matched infants and children was also studied. Left ventricular longitudinal strain (S), strain rate (SR), time to peak systolic longitudinal strain (TPS), and time to peak systolic longitudinal strain rate (TPSR) were measured and compared between the 2 groups. Results: Mean ± SD of age at the time of study in the patients with TGA/IVS was 15 ± 5 months, and also age at the time of ASO was 12 ± 3 days. Weight was 3.13 ± 0.07 kg at birth and 8.83 ± 1.57 kg at the time of ASO. Global strain (S), Time to peak strain rate (TPSR), and Time to peak strain (TPS) were not significantly different between the 2 groups, whereas global strain rate (SR) was significantly different (p value < 0.001). In the 3-chamber view, the values of S in the lateral, septal, inferior, and anteroseptal walls were significantly different between the 2 groups (p value < 0.001), and SR in the posterior wall was significantly different between the 2 groups (p value < 0.001). There were no positive correlations between S and SR in terms of the variables of heart rate, total cardiopulmonary bypass time, and aortic cross-clamp time. There were no statistically significant differences between the 2 groups regarding S, SR, TPS, and TPSR in the anteroseptal and posterior walls in the 3-chamber view and in the lateral and septal walls in the 4-chamber view. Conclusion: We showed that between 6 and 18 months after

  12. Exercise thallium-201 imaging in complete left bundle branch block and the prevalence of septal perfusion defects

    International Nuclear Information System (INIS)

    Jazmati, B.; Sadaniantz, A.; Emaus, S.P.; Heller, G.V.

    1991-01-01

    To determine the prevalence of septal defects in a generalized referral population, the records of 93 consecutive patients with complete left bundle branch block (BBB) who underwent symptom-limited treadmill exercise testing with thallium-201 myocardial imaging over a 3-year period were reviewed. Segmental analysis of the planar thallium-201 images was performed in a blinded fashion with agreement by consensus. Computerized quantitative analysis of the images also was independently performed, and was correlated with the visual interpretations. Forty-seven patients (51%) had normal images, and 46 (49%) had defects of greater than or equal to 2 segments. In the abnormal studies, only 13 patients (14% of the total population) had septal defects, while a much higher number of patients, 33 (39%), had inferior or apical defects, or both. Coronary angiography was performed in 6 patients with septal defects: Significant narrowing of the left anterior descending coronary artery was found in 4 patients, a narrowed right coronary artery was found in 1, and normal coronary arteries were seen in the other patient. In conclusion, whereas previous studies have suggested a high percentage of false-positive septal defects in patients with left BBB, this study demonstrates a low prevalence (14%) of septal defects in a large population of unselected patients presenting for exercise thallium-201 imaging. Therefore, exercise thallium-201 imaging remains a useful procedure for evaluating patients with complete left BBB

  13. Right ventricular mechanics in hypertrophic cardiomyopathy using feature tracking

    Science.gov (United States)

    Badran, Hala Mahfouz; Soliman, Mahmood; Hassan, Hesham; Abdelfatah, Raed; Saadan, Haythem; Yacoub, Magdi

    2013-01-01

    Objectives: Right ventricular (RV) mechanics in hypertrophic cardiomyopathy (HCM) are poorly understood. We investigate global and regional deformation of the RV in HCM and its relationship to LV phenotype, using 2D strain vector velocity imaging (VVI). Methods: 100 HCM patients (42% females, 41 ± 19 years) and 30 control patients were studied using VVI. Longitudinal peak systolic strain (ϵsys), strain rate (SR), time to peak (ϵ) (TTP), displacement of RV free wall (RVFW) and septal wall were analyzed. Similar parameters were quantified in LV septal, lateral, anterior and inferior segments. Intra-V-delay was defined as SD of TTP. Inter-V-delay was estimated from TTP difference between the most delayed LV segment & RVFW. Results: ϵsys and SR of both RV & LV, showed loss of base to apex gradient and significant decline in HCM (p < 0.001). Deformation variables estimated from RVFW were strongly correlated with each other (r = 0.93, p < 0.0001). Both were directly related to LV ϵsys, SRsys, SRe, ejection fraction (EF)%, RVFW displacement (P < 0.001) and inversely related to age, positive family history (p < 0.004, 0.005), RV wall thickness, maximum wall thickness (MWT), intra-V-delay, LA volume (P < 0.0001), LVOT gradient (p < 0.02, 0.005) respectively. ROC curves were constructed to explore the cut-off point that discriminates RV dysfunction. Global and RVFW ϵsys: − 19.5% shows 77, 70% sensitivity & 97% specificity, SRsys: − 1.3s− 1 shows 82, 70% sensitivity & 30% specificity. Multivariate analyses revealed that RVFW displacement (β = − 0.9, p < 0.0001) and global LV SRsys (β = 5.9, p < 0.0001) are independent predictors of global RV deformation. Conclusions: Impairment of RV deformation is evident in HCM using feature tracking. It is independently influenced by LV mechanics and correlated to the severity of LV phenotype. RVFW deformation analysis and global RV assessment are comparable. PMID:24689019

  14. Systolic left ventricular function according to left ventricular concentricity and dilatation in hypertensive patients

    DEFF Research Database (Denmark)

    Bang, Casper; Gerdts, Eva; Aurigemma, Gerard P

    2013-01-01

    Left ventricular hypertrophy [LVH, high left ventricular mass (LVM)] is traditionally classified as concentric or eccentric based on left ventricular relative wall thickness. We evaluated left ventricular systolic function in a new four-group LVH classification based on left ventricular dilatation...... [high left ventricular end-diastolic volume (EDV) index and concentricity (LVM/EDV)] in hypertensive patients....

  15. Left ventricular function in patients with ventricular arrhythmias and aortic valve disease

    International Nuclear Information System (INIS)

    Santinga, J.T.; Kirsh, M.M.; Brady, T.J.; Thrall, J.; Pitt, B.

    1983-01-01

    Forty patients having aortic valve replacement were evaluated preoperatively for ventricular arrhythmia and left ventricular ejection fraction. Arrhythmias were classified as complex or simple using the Lown criteria on the 24-hour ambulatory electrocardiogram; ejection fractions were determined by radionuclide gated blood pool analysis and contrast angiography. The ejection fractions determined by radionuclide angiography were 59.1 +/- 13.1% for 26 patients with simple or no ventricular arrhythmias, and 43.9 +/- 20.3% for 14 patients with complex ventricular arrhythmias (p less than 0.01). Ejection fractions determined by angiography, available for 31 patients, were also lower in patients with complex ventricular arrhythmias (61.1 +/- 16.3% versus 51.4 +/- 13.4%; p less than 0.05). Seven of 9 patients showing conduction abnormalities on the electrocardiogram had complex ventricular arrhythmias. Eight of 20 patients with aortic stenosis had complex ventricular arrhythmias, while 2 of 13 patients with aortic insufficiency had such arrhythmias. It is concluded that decreased left ventricular ejection fraction, intraventricular conduction abnormalities, and aortic stenosis are associated with an increased frequency of complex ventricular arrhythmias in patients with aortic valve disease

  16. Myocardial perfusion and left ventricular function during exercise evaluated by 201Tl myocardial scintigraphy and 99mTc radionuclide ventriculography in patients treated with PTCA

    International Nuclear Information System (INIS)

    Honda, Toshio; Jo, Tadafumi; Doiuchi, Junji

    1992-01-01

    To evaluate the effects of percutaneous transluminal coronary angioplasty (PTCA), we investigated myocardial ischemia and left ventricular function during exercise before and after successful PTCA in 30 patients. We used extent and severity scores of 201 thallium ( 201 Tl) exercise myocardial scintigraphy to assess myocardial ischemia and determined global and regional left ventricular ejection fraction (EF and REF) of 99m Tc-RBC exercise radionuclide ventriculography to assess left ventricular function. The extent and severity scores of stress images were significantly less after PTCA than before PTCA. The scores of the redistribution images were unchanged before and after PTCA. Global EF during exercise was significantly higher after PTCA than before PTCA. There was no difference in resting global EF between before and after PTCA. Myocardial ischemia induced by exercise was semi-quantitatively analyzed as transient perfusion defect with severity score. Severity score was significantly less after PTCA than before PTCA. ΔEF, which was obtained by subtraction of resting global EF from exercise one, was significantly higher after PTCA than before PTCA. However, the degree of improvement in myocardial ischemia and left ventricular function varied from patient to patient. In 17 patients with one-vessel left anterior descending artery disease, ΔREF, which was determined by subtracting resting regional EF from exercise one, was significantly higher in septal and apical segments after PTCA than before PTCA. Myocardial ischemia and left ventricular function under exercise were alleviated by PTCA. However, the degree of improvement varied from patient to patient and it might have been affected by various factors including coronary dissection, edema, thrombus, restenosis, spasm, side branch stenosis or occlusion, distal thrombus, and myocardial hibernation. (author)

  17. Congenital Heart Diseases in Adults: A Review of Echocardiogram ...

    African Journals Online (AJOL)

    The most common congenital anomalies were ventricular septal defects (VSD) ‑ 31.3%, (36/115), atrial septal defects ‑ 28.7% (33/115) and tetralogy of fallot ‑ 10.4% (12/115). Conclusion: VSD are the most common congenital heart diseases in adults presenting for echocardiographic examination in Enugu, Nigeria.

  18. Left ventricular diastolic filling in patients with systemic hypertension

    International Nuclear Information System (INIS)

    Narita, Michihiro; Kurihara, Tadashi; Murano, Kenichi; Usami, Masahisa; Honda, Minoru; Kanao, Keisuke

    1985-01-01

    To study the prevalence and significance of left ventricular (LV) diastolic dysfunction in mild to moderate systemic hypertension (HT), cardiac blood pool imagings with Tc-99 m were obtained in 10 normal subjects and 27 patients with HT. The patients with HT did not show any evidence of coronary heart disease, renal insufficiency, cerebrovascular accident or diabetes mellitus. They were divided into 3 groups; (1) HT-1 (n=10): without evidence of echocardiographic (UCG) and electrocardiographic (ECG) left ventricular hypertrophy (LVH), (2) HT-2 (n=8): with evidence of ECG-LVH without UCG-LVH, (3) HT-3 (n=9): with evidence of UCG-LVH. UCG-LVH was defined when posterior or interventricular septal tall thickness exceeded 13 mm at end-diastole. From UCG findings LV mass was calculated and from UCG findings and auscultating brachial systolic pressure LV peak-systolic wall stress (WS) was obtained. Cardiac blood pool imagings were performed at modified LAO at rest and during exercise stress. Indices of LV systolic function (rest ejection fraction, mean ejection rate during the first third of ejection and exercise ejection fraction response) were essentially similar in normal subjects and all HT groups. In contrast, LV diastolic filling rate during the first third of diastole (1/3 FR mean) decreased significantly in any group of HT, and it was prominent in HT-3. In patients with HT, 1/3 FR mean did not correlate with blood pressure, LV peak-systolic WS, LV systolic functions and LV end-diastolic volume. But it correlated inversely with LV mass (r=-0.84). These results suggest that impairment of early diastolic LV filling can be detected before systolic cardiac impairment and LVH develop, and it is, at least in part, relate to the LV mass. (author)

  19. Quantification of the right ventricular wall using stress myocardial emission computed tomography with thallium-201 in normal subjects

    International Nuclear Information System (INIS)

    Akanabe, Hiroshi; Oshima, Motoo; Sakuma, Sadayuki; Yamamoto, Shuhei; Kawai, Naoki; Sotobata, Iwao

    1985-01-01

    Although many studies of quantitative analysis of left ventricular myocardial wall (LVMW) have been reported using stress thallium-201 (Tl-201), few reports of right ventricular myocardial wall (RVMW) have been estimated. In this study we determined whether single photon emission computed tomography (SPECT) with Tl-201 could accurately define normal range of RVMW in normal subjects. Twelve persons who have no valvular disease, nor coronary artery disease were included in this study. Stress SPECT study was reconstructed to make a short axial images of ventricles. RVMW and LVMW were flagged by mamual. Each ventricle was divided into 36 parts at every 10 degree. Relative activity counts in each ventricle were calculated as a percent counts of maximum counts in left ventricle. The normal range of RVMW with stress SPECT was as follows: anterior wall (33.2 +- 11.4 %, mean +- 2 standard deviation, -62.7 +- 18.4 %), free wall (30.1 +- 12.4 % - 38.5 +- 8.8 %), inferior wall (40.4 +- 7.8 % - 60.0 +- 21.4 %), septal wall (65.2 +- 17.2 % - 71.1 +- 14.2 %). Above the results, SPECT with Tl-201 can accurately define the normal range of RVMW, and this method is usefull to quantify the degree of ischemia and hypertrophy in RVMW. (author)

  20. Arrhythmogenic Right Ventricular Cardiomyopathy in an Endurance Athlete Presenting with Ventricular Tachycardia and Normal Right Ventricular Function.

    Science.gov (United States)

    Hedley, Jeffrey S; Al Mheid, Ibhar; Alikhani, Zoubin; Pernetz, Maria A; Kim, Jonathan H

    2017-08-01

    Arrhythmogenic right ventricular cardiomyopathy, a genetically inherited disease that results in fibrofatty replacement of normal cardiac myocytes, has been associated with sudden cardiac death in athletes. Long-term participation in endurance exercise hastens the development of both the arrhythmic and structural arrhythmogenic right ventricular cardiomyopathy phenotypes. We describe the unusual case of a 34-year-old, symptomatic, female endurance athlete who had arrhythmogenic right ventricular cardiomyopathy in the presence of a structurally normal right ventricle. Clinicians should be aware of this infrequent presentation when evaluating athletic patients who have ventricular arrhythmias and normal findings on cardiac imaging studies.

  1. Prophylactic implantable defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation or sustained ventricular tachycardia.

    LENUS (Irish Health Repository)

    Corrado, Domenico

    2010-09-21

    The role of implantable cardioverter-defibrillator (ICD) in patients with arrhythmogenic right ventricular cardiomyopathy\\/dysplasia and no prior ventricular fibrillation (VF) or sustained ventricular tachycardia is an unsolved issue.

  2. Tricuspid annuloplasty with the MC3 ring and septal plication technique.

    Science.gov (United States)

    Isomura, Tadashi; Hirota, Masanori; Hoshino, Joji; Fukada, Yasuhisa; Kondo, Taichi; Takahashi, Yu

    2015-01-01

    Functional tricuspid regurgitation is caused by annular dilation mainly in the posterior annulus. However, ring annuloplasty does not always prevent the recurrence of tricuspid regurgitation due to dilation of the septal annulus. We developed a septal plication technique with a 3-dimensional MC3 ring. Between 2006 and 2011, 76 patients (male/female 30/46; mean age 68 ± 11 years) with functional tricuspid regurgitation received tricuspid ring annuloplasty. After placement of the annular sutures, the 3 commissural ring portions were fixed on the equivalent commissures to plicate the anterior and posterior annulus. The end of the septal ring portion was fixed at the optimal annular position to obtain minimal tricuspid regurgitation. All patients were followed-up for a mean of 47 ± 18 months; the longest duration was 79 months. Although there was no operative death, one patient died of sepsis during hospitalization (hospital mortality 1.3%). After implantation of the MC3 ring (mean size 31.0 ± 3.3 mm), additional edge-to-edge sutures were required for minor leakage in 5 (7%) patients. The degree of tricuspid regurgitation was significantly reduced at discharge (0.5 ± 0.6) and midterm (0.6 ± 0.6) compared to 2.5 ± 0.7 before the operation (p tricuspid ring annuloplasty with a 3-dimensional MC3 ring. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  3. Left ventricular outflow tract arrhythmias with divergent QRS morphology: mapping of different exits and ablation strategy.

    Science.gov (United States)

    Reithmann, Christopher; Fiek, Michael

    2018-01-01

    Ventricular arrhythmias (VAs) from the left ventricular outflow tract (LVOT) can have multiple exits exhibiting divergent ECG features. In a series of 131 patients with VAs with LVOT origin, 10 patients presented with divergent QRS morphologies. Multisite endo- and epicardial mapping of different exit sites was performed. The earliest ventricular activity of 23 LVOT VAs in 10 patients was detected in the endocardium of the LV in 7 patients, the aortic sinuses of Valsalva (SoV) in 3 patients, the distal coronary sinus in 6 patients, the anterior interventricular vein in 3 patients, and the posterior right ventricular outflow tract (RVOT) in 4 patients. Simultaneous elimination of two divergent QRS morphologies of LVOT VAs by ablation from a single site was achieved in 5 patients (aorto-mitral continuity in 3 patients, SoV and RVOT in each 1 patient) using a mean maximum ablation energy of 46 ± 5 W. Sequential ablation from two or three different sites, including trans-pericardial and distal coronary sinus ablation in each 2 patients, led to elimination of the divergent VA QRS morphologies in the other 5 patients. During the follow-up of 28 ± 29 months, 4 of the 10 patients had recurrence of at least one LVOT VA. A 43-year-old patient with muscular dystrophy Curschmann-Steinert had recurrence of sustained LVOT VTs and died of sudden cardiac death. Multisite mapping of different exit sites of LVOT VAs can guide ablation of intramural foci but the recurrence rate after initially successful ablation was high.

  4. Cardiac involvement in patients with limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy

    DEFF Research Database (Denmark)

    Sveen, Marie-Louise; Thune, Jens Jakob; Køber, Lars

    2008-01-01

    OBJECTIVE: To investigate the extent of cardiac involvement in patients with 1 of the 12 groups of recessively inherited limb-girdle muscular dystrophy type 2 (LGMD2A-L) and Becker muscular dystrophy (BMD). DESIGN: Prospective screening. SETTING: Neuromuscular Clinic and Department of Cardiology...

  5. Efeitos da fadiga muscular induzida por exercícios no tempo de reação muscular dos fibulares em indivíduos sadios Efectos de la fatiga muscular inducida por ejercicios sobre el tiempo de reacción muscular peronea en individuos sanos Effects of the exercise-induced muscular fatigue on the time of muscular reaction of the fibularis in healthy individuals

    Directory of Open Access Journals (Sweden)

    Bruno Araújo Rego Santos Silva

    2006-04-01

    Full Text Available A fadiga muscular (FM é um fenômeno comum nas atividades esportivas e diárias, resultando numa piora da performance motora. Ela é considerada um dos fatores causadores de lesões músculo-esqueléticas. A entorse de tornozelo é um exemplo: a FM afetaria tanto o sistema aferente quanto o eferente. Vários estudos têm analisado a influência da FM no controle neuromuscular (CNM; entretanto, existe pouca pesquisa sobre essa influência na velocidade de reação dos músculos. O objetivo deste estudo foi verificar os efeitos da FM no tempo de reação muscular (TRM dos músculos fibulares, que são os primeiros a responder a um estresse em inversão do tornozelo. Foram estudados 14 indivíduos saudáveis masculinos (idade: 20-35 anos, que tiveram seus TRM avaliados por meio de eletromiografia (EMG de superfície. O início da atividade muscular foi definido como a média de repouso + 3x o desvio-padrão (DP. O TRM dos fibulares foi mensurado após uma inversão súbita de 20º realizada numa plataforma. A inversão súbita foi realizada antes e depois da fadiga muscular, que foi induzida por exercícios localizados dos fibulares até a exaustão. Os resultados mostraram que houve um aumento significativo do tempo de reação muscular após a fadiga (p La fatiga muscular (FM es un fenómeno común en las actividades diarias, produciendo un empeoramiento de la actuación. Se la considera una de las causas de factores lesionantes musculares de esqueleto. El esguince del tobillo es un ejemplo: La FM afectaría tanto el sistema aferente cuanto el eferente. Varios estudios han estado analizando la influencia de FM en el comando neuromuscular (CNM, sin embargo, la existen pocas investigaciones sobre la influencia en la velocidad de reacción de los músculos. El objetivo de ese estudio era verificar los efectos de FM en el tiempo de reacción muscular (TRM de los músculos peroneos, que son los primeros en responder a una tensión en la inversi

  6. Exclusive double outlet right ventricle with atrioventricular concordance and pulmonary stenosis. Results of reconstructive surgery.

    Science.gov (United States)

    Busquet, J; Fontan, F; Choussat, A; Caianiello, G; Fernandez, G

    1988-01-01

    Double outlet right ventricle associated with atrioventricular concordance, pulmonary stenosis and situs solitus of the atria is a subset of double outlet right ventricle related through the surgical treatment. From 1974 to 1985, 14 patients, 5 males, 9 females (mean age 8.9 years, range 13 months-22 years) were operated upon. All patients had infundibular stenosis and normal or large pulmonary arteries. The apex of the heart was to the right in 2 patients, the right and left ventricles were superior and inferior in 2 patients and 1 patient had both anomalies. The ventricular septal defect was subaortic in 11 patients (aorto-mitral discontinuity in 5) and non-committed in 3 patients. Three patients had 2 ventricular septal defects. The aorta was anterior in 3 patients and to the right of the pulmonary artery in 11 patients. All patients, through a transventricular and transatrial approach, had a reconstructive surgery. In 3 patients, an aortic homograft valved conduit was used. One patient had the ventricular septal defect enlarged. There was one early death (7.1%) from high residual right ventricle pressure and no late death. One patient had a transient atrioventricular block. One patient was reoperated upon for a residual ventricular septal defect. All survivors had a good clinical result. Re-evaluation in 8 patients confirmed excellent haemodynamics: the right ventricle to pulmonary artery pressure gradient decreased from 80 mm Hg (range 60-95) preoperatively to 24 mm Hg (range 3-32) postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)

  7. Muscular dystrophy

    Science.gov (United States)

    ... are no known cures for the various muscular dystrophies. The goal of treatment is to control symptoms. Physical therapy may help maintain muscle strength and function. Leg braces and a wheelchair ...

  8. Surgical treatment of interrupted aortic arch associated with ventricular septal defect and patent ductus arteriosus in patients over one year of age.

    Science.gov (United States)

    Li, Zhiqiang; Li, Bin; Fan, Xiangming; Su, Junwu; Zhang, Jing; He, Yan; Liu, Yinglong

    2014-01-01

    Interrupted aortic arch (IAA) is a rare congenital anomaly affecting 1.5% of infants with congenital heart disease. Neonatal repair of IAA is required to avoid irreversible pulmonary vascular lesion. However, in China, patients with IAA associated with ventricular septal defect (VSD) and patent ductus arteriosus (PDA) over one year of age are common. So we investigated the outcome of surgical treatment of IAA with VSD and PDA in patients over one year of age. From January 2009 to December 2012, 19 patients with IAA have undergone complete single-stage repair. The patients' mean age was 4.4 years, ranging 1 to 15 years; and their mean weight was 12.8 kg, ranging 4.2 to 36.0 kg. Fifteen IAA were type A, four were type B. Preoperative cardiac catheterization data were available from all patients. Mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) were measured. The measurements of postoperative pulmonary artery pressure were taken in the operating room at the end of the case. All patients underwent echocardiographic examinations before discharged from the hospital. In addition, cardiac catheterization and echocardiographic examinations were performed during follow-up. Selective brain perfusion through the innominate artery during aortic arch reconstruction was used in all patients. Mean follow-up was (1.6±0.8) years. There were two hospital deaths (2/19, 11%). One patient died of pulmonary hypertension crisis, and another died of postoperative low cardiac output. Five cases had other main postoperative complications but no postoperative neurologic complications. Seventeen survivors were followed up, and there were no late deaths or reoperation. Mean cross-clamp duration was (85±22) minutes and selective brain perfusion duration was (34±11) minutes. Two patients required delayed sternal closure at two days postoperatively. Intensive care unit and hospital stays were (9±8) days and (47±24) days, respectively. Pressure gradients across

  9. Left septal atrial tachycardia after open-heart surgery: relevance to surgical approach, anatomical and electrophysiological characteristics associated with catheter ablation, and procedural outcomes.

    Science.gov (United States)

    Adachi, Toru; Yoshida, Kentaro; Takeyasu, Noriyuki; Masuda, Keita; Sekiguchi, Yukio; Sato, Akira; Tada, Hiroshi; Nogami, Akihiko; Aonuma, Kazutaka

    2015-02-01

    Septal atrial tachycardia (AT) can occur in patients without structural heart disease and in patients with previous catheter ablation of atrial fibrillation. We aimed to assess septal AT that occurs after open-heart surgery. This study comprised 20 consecutive patients undergoing catheter ablation of macroreentrant AT after open-heart surgery. Relevance to surgical approach, mechanisms, anatomic and electrophysiological characteristics, and outcomes were assessed. Septal AT was identified in 7 patients who had all undergone mitral valve surgery. All septal ATs were localized in the left atrial septum, whereas 10 of 13 nonseptal ATs originated from the right atrium. Patients with left septal AT had a thicker fossa ovalis (median, 4.0; 25th-75th percentile, 3.6-4.2 versus 2.3; 1.6-2.6 mm; P=0.006) and broader area of low voltage (open-heart surgery was characterized by a thicker septum, more scar burden in the septum, and repeated prolongations of the tachycardia cycle length during ablation. Such an arrhythmogenic substrate may interfere with transmural lesion formation by ablation and may account for higher likelihood of recurrence of left septal AT. © 2014 American Heart Association, Inc.

  10. Effectiveness of caudal septal extension graft application in endonasal septoplasty

    Directory of Open Access Journals (Sweden)

    Yunus Karadavut

    Full Text Available Abstract Introduction Septal deviation is a common disease seen in daily otorhinolaryngology practice and septoplasty is a commonly performed surgical procedure. Caudal septum deviation is also a challenging pathology for ear, nose, and throat specialists. Many techniques are defined for caudal septal deviation. Objective To evaluate the effectiveness of caudal septal extension graft (CSEG application in patients who underwent endonasal septoplasty for a short and deviated nasal septum. Methods Forty patients with nasal septal deviation, short nasal septum, and weak nasal tip support who underwent endonasal septoplasty with or without CSEG placement between August 2012 and June 2013 were enrolled in this study. Twenty patients underwent endonasal septoplasty with CSEG placement. The rest of the group, who rejected auricular or costal cartilage harvest for CSEG placement, underwent only endonasal septoplasty without any additional intervention. Using the Nasal Obstruction Symptom Evaluation (NOSE and Rhinoplasty Outcome Evaluation (ROE questionnaires, pre- and post-operative acoustic rhinometer measurements were evaluated to assess the effect of CESG placement on nasal obstruction. Results In the control group, preoperative and postoperative minimal cross-sectional areas (MCA1 were 0.44 ± 0.10 cm2 and 0.60 ± 0.11 cm2, respectively (p < 0.001. In the study group, pre- and postoperative MCA1 values were 0.45 ± 0.16 cm2 and 0.67 ± 0.16 cm2, respectively (p < 0.01. In the control group, the nasal cavity volume (VOL1 value was 1.71 ± 0.21 mL preoperatively and 1.94 ± 0.17 mL postoperatively (p < 0.001. In the study group, pre- and postoperative VOL1s were 1.72 ± 0.15 mL and 1.97 ± 0.12 mL, respectively (p < 0.001. Statistical analysis of postoperative MCA1 and VOL1 values in the study and the control groups could not detect any significant intergroup difference (p = 0.093 and 0.432, respectively. In the study group, mean nasolabial angles were

  11. Yield Strength Testing in Human Cadaver Nasal Septal Cartilage and L-Strut Constructs.

    Science.gov (United States)

    Liu, Yuan F; Messinger, Kelton; Inman, Jared C

    2017-01-01

    To our knowledge, yield strength testing in human nasal septal cartilage has not been reported to date. An understanding of the basic mechanics of the nasal septum may help surgeons decide how much of an L-strut to preserve and how much grafting is needed. To determine the factors correlated with yield strength of the cartilaginous nasal septum and to explore the association between L-strut width and thickness in determining yield strength. In an anatomy laboratory, yield strength of rectangular pieces of fresh cadaver nasal septal cartilage was measured, and regression was performed to identify the factors correlated with yield strength. To measure yield strength in L-shaped models, 4 bonded paper L-struts models were constructed for every possible combination of the width and thickness, for a total of 240 models. Mathematical modeling using the resultant data with trend lines and surface fitting was performed to quantify the associations among L-strut width, thickness, and yield strength. The study dates were November 1, 2015, to April 1, 2016. The factors correlated with nasal cartilage yield strength and the associations among L-strut width, thickness, and yield strength in L-shaped models. Among 95 cartilage pieces from 12 human cadavers (mean [SD] age, 67.7 [12.6] years) and 240 constructed L-strut models, L-strut thickness was the only factor correlated with nasal septal cartilage yield strength (coefficient for thickness, 5.54; 95% CI, 4.08-7.00; P cadaver nasal septal cartilage, L-strut thickness was significantly associated with yield strength. In a bonded paper L-strut model, L-strut thickness had a more important role in determining yield strength than L-strut width. Surgeons should consider the thickness of potential L-struts when determining the amount of cartilaginous septum to harvest and graft. NA.

  12. Muscular Oxygen Uptake Kinetics in Aged Adults.

    Science.gov (United States)

    Koschate, J; Drescher, U; Baum, K; Eichberg, S; Schiffer, T; Latsch, J; Brixius, K; Hoffmann, U

    2016-06-01

    Pulmonary oxygen uptake (V˙O2) kinetics and heart rate kinetics are influenced by age and fitness. Muscular V˙O2 kinetics can be estimated from heart rate and pulmonary V˙O2. In this study the applicability of a test using pseudo-random binary sequences in combination with a model to estimate muscular V˙O2 kinetics was tested. Muscular V˙O2 kinetics were expected to be faster than pulmonary V˙O2 kinetics, slowed in aged subjects and correlated with maximum V˙O2 and heart rate kinetics. 27 elderly subjects (73±3 years; 81.1±8.2 kg; 175±4.7 cm) participated. Cardiorespiratory kinetics were assessed using the maximum of cross-correlation functions, higher maxima implying faster kinetics. Muscular V˙O2 kinetics were faster than pulmonary V˙O2 kinetics (0.31±0.1 vs. 0.29±0.1 s; p=0.004). Heart rate kinetics were not correlated with muscular or pulmonary V˙O2 kinetics or maximum V˙O2. Muscular V˙O2 kinetics correlated with maximum V˙O2 (r=0.35; p=0.033). This suggests, that muscular V˙O2 kinetics are faster than estimates from pulmonary V˙O2 and related to maximum V˙O2 in aged subjects. In the future this experimental approach may help to characterize alterations in muscular V˙O2 under various conditions independent of motivation and maximal effort. © Georg Thieme Verlag KG Stuttgart · New York.

  13. Long-term survival in children with atrioventricular septal defect and common atrioventricular valvar orifice in Sweden.

    Science.gov (United States)

    Frid, Christina; Björkhem, Gudrun; Jonzon, Anders; Sunnegårdh, Jan; Annerén, Göran; Lundell, Bo

    2004-02-01

    The survival for patients with atrioventricular septal defect has improved markedly over the last decades and, during the same period, the survival of children with Down's syndrome has also increased. The aim of our study was to investigate long-term survival in patients having atrioventricular septal defect with common valvar orifice, but without associated significant congenital heart defects, in the setting of Down's syndrome, comparing the findings to those in chromosomally normal children with the same malformation. In a population-based retrospective study, we scrutinised the medical records from 801 liveborn children with atrioventricular septal defect born in Sweden during the period 1973 through 1997. Data on gender, presence or absence of Down's syndrome, associated congenital heart defects, date of birth, operation and death were recorded and followed up until 2001. An isolated atrioventricular septal defect with common atrioventricular valvar orifice was present in 502 children, of whom 86% had Down's syndrome. We found a significant reduc tion over time in age at operation, and in postoperative mortality at 30 days, from 28 to 1%. Using a multiple logistic regression model, we found no significant differences in mortality between genders, nor between those with or without Down's syndrome. Early corrective surgery could not be identified as a significant independent factor for survival. The 5-year postoperative survival in patients with Down's syndrome increased from 65% over the period from 1973 through 1977, to about 90% in the period 1993 through 1997, and the same trend was observed in chromosomally normal patients. Survival in uncomplicated atrioventricular septal defect with common atrioventricular valvar orifice has greatly increased, and surgical correction is now equally successful in patients with Down's syndrome and chromosomally normal patients, and for both genders. Death in connection with surgery is no longer the major threat, and focus

  14. Genetics Home Reference: spinal muscular atrophy

    Science.gov (United States)

    ... difficulty breathing. Children with this type often have joint deformities (contractures) that impair movement. In severe cases, ... Proximal spinal muscular atrophy Washington University, St. Louis: Neuromuscular Disease Center: Spinal Muscular Atrophy Patient Support and ...

  15. Radionuclide detection and differential diagnosis of left-to-right cardiac shunts by analysis of time-activity curves

    International Nuclear Information System (INIS)

    Kim, Ok-Hwa

    1986-01-01

    The noninvasive nature of the radionuclide angiocardiography provided a useful approach for the evaluation of left-to-right cardiac shunts (LRCS). While the qualitative information can be obtained by inspection of serial radionuclide angiocardiograms, the quantitative information of radionuclide angiocardiography can be obtained by the analysis of time-activity curves using advanced computer system. The count ratios method and pulmonary-to-systemic flow ratio (QP/QS) by gamma variate fit method were used to evaluate the accuracy of detection and localization of LRCS. One hundred and ten time-activity curves were analyzed. There were 46 LRCS (atrial septal defects 11, ventricular septal defects 22, patent ductus arteriosus 13) and 64 normal subjects. By computer analysis of time-activity histograms of the right atrium, ventricle and the lungs separately, the count ratios modified by adding the mean cardiac transit time were calculated in each anatomic site. In normal subjects the mean count ratios in the right atrium, ventricle and lungs were 0.24 on average. In atrial septal defects, the count ratios were high in the right atrium, ventricle and lungs, whereas in ventricular septal defects the count ratios were higher only in the right ventricle and lungs. Patent ductus arteriosus showed normal count ratios in the heart but high count ratios were obtained in the lungs. Thus, this count ratios method could be separated normal from those with intracardiac or extracardiac shunts, and moreover, with this method the localization of the shunts level was possible in LRCS. Another method that could differentiate the intracardiac shunts from extracardiac shunts was measuring QP/QS in the left and right lungs. In patent ductus arteriosus, the left lung QP/QS was hight than those of the right lung, whereas in atrial septal defects and ventricular septal defects QP/QS ratios were equal in both lungs. (J.P.N.)

  16. Anatomy of right superior septal artery demonstrated on the coronary CT scan

    International Nuclear Information System (INIS)

    Takeguchi, Takaya; Ibukuro, Kenji; Fukuda, Hozumi; Tobe, Kimiko; Abe, Shoko

    2012-01-01

    Background. A coronary CT scan allows for non-invasive visualization of the anatomy of a coronary artery in three dimensions compared to the two dimensions afforded by conventional angiography. The septal artery, the main blood source of the interventricular septum, is usually derived from the left anterior descending artery; however, it is occasionally derived from the right coronary artery. Purpose. To analyze the prevalence, origin, diameter, and length of the right superior septal artery (RSSA) demonstrated on a coronary CT scan. Material and Methods. The right superior septal artery was retrospectively reviewed on the reconstructed axial scan images (0.5-mm thickness, 0.25-mm interval) in 1290 consecutive patients who underwent coronary CT scans. All patients were scanned on a 320-row CT scanner. The images were transferred to a workstation to trace the vessel to analyze the origin, diameter, and length. We also compared the length of the RSSA between patients with and without coronary artery stenosis. Results. The RSSA was identified in 51 (3.9%) of 1290 patients. The origin was the proximal portion of the right coronary artery (n = 40) or the right sinus of Valsalva (n 11). The artery co-existed with the conus artery in 15 (29%) of 51 patients. The length was 16-62 mm (mean 31.2 mm ± 10.5), and the diameter was 0.8-2.0 mm (mean 1.3 mm ± 0.2). Longer RSSAs tended to be demonstrated in the patients with coronary artery stenosis rather than with normal coronary arteries (P < 0.05). Conclusion. The right superior septal artery and its anatomical variant could be analyzed with a coronary CT scan. The ability to demonstrate this artery on the coronary CT scan was the same as with coronary angiography. The recognition of this vessel is useful for physicians managing with the diagnosis and treatment of the coronary artery disease

  17. Genetics Home Reference: Duchenne and Becker muscular dystrophy

    Science.gov (United States)

    ... Conditions Duchenne and Becker muscular dystrophy Duchenne and Becker muscular dystrophy Printable PDF Open All Close All Enable Javascript ... dystrophy occur almost exclusively in males. Duchenne and Becker muscular dystrophies have similar signs and symptoms and are caused ...

  18. Identification of sleep hypoventilation in young individuals with Becker muscular dystrophy: A pilot study.

    Science.gov (United States)

    Nakamura, Yuko; Saito, Yoshiaki; Kubota, Norika; Matsumura, Wataru; Hosoda, Chika; Tamasaki-Kondo, Akiko; Nishimura, Yoko; Sunada, Yoshihide; Fukada, Masuyuki; Ohno, Takako; Maegaki, Yoshihiro; Matsuo, Masafumi; Tokita, Yasuko

    2018-03-08

    To report on sleep hypercapnia in Becker muscular dystrophy (BMD) at earlier stages than ever recognized. This retrospective study examined nocturnal hypercapnia in six young Becker muscular dystrophy (BMD) patients with deletions of one or more exons of DMD gene. Clinical information, consecutive data on forced vital capacity (FVC%), forced expiratory volume in one second (FEV1%), peak expiratory flow (PEF%), peak cough flow (PCF), average PCO 2 in all-night monitoring, and left ventricular ejection fraction (LVEF) were reviewed. In five BMD patients, including three who were still ambulant, nocturnal average PCO 2 was elevated to >45 mmHg at 12-31 years of age. Noninvasive positive pressure ventilation was initiated in four patients. Gradual declines in FVC% and PEF% were evident in one BMD patient with exon 3-7 deletion, whereas these functions did not change in the remaining BMD patients. PCF, FEV1%, and LVEF were less informative for the assessment of respiratory function in this patient series. Sleep hypercapnia was present in certain BMD patients, which was unexpected from the routine pulmonary function tests. Individualized assessment of nocturnal PCO 2 , partly based on the deletion types, should be further explored in the clinical practice of BMD patients. Copyright © 2018 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  19. Double-outlet right ventricle revisited.

    Science.gov (United States)

    Ebadi, Ameneh; Spicer, Diane E; Backer, Carl L; Fricker, F Jay; Anderson, Robert H

    2017-08-01

    Double-outlet right ventricle is a form of ventriculoarterial connection. The definition formulated by the International Society for Nomenclature of Paediatric and Congenital Heart Disease is based on hearts with both arterial trunks supported in their greater part by a morphologically right ventricle. Bilateral infundibula and ventricular septal defects are highly debated criteria. This study examines the anatomic controversies surrounding double-outlet right ventricle. We show that hearts with double-outlet right ventricle can have atrioventricular-to-arterial valvular continuity. We emphasize the difference between the interventricular communication and the zone of deficient ventricular septation. The hearts examined were from the University of Florida in Gainesville; Johns Hopkins All Children's Hospital, St Petersburg, Fla; and Lurie Children's Hospital, Chicago, Ill. Each specimen had at least 75% of both arterial roots supported by the morphologically right ventricle, with a total of 100 hearts examined. The morphologic method was used to assess anatomic features, including arterial-atrioventricular valvular continuity, subarterial infundibular musculature, and the location of the hole between the ventricles. Most hearts had fibrous continuity between one of the arterial valves and an atrioventricular valve, with bilateral infundibula in 23%, and intact ventricular septum in 5%. Bilateral infundibula are not a defining feature of double-outlet right ventricle, representing only 23% of the specimens in our sample. The interventricular communication can have a posteroinferior muscular rim or extend to become perimembranous (58%). Double-outlet right ventricle can exist with an intact ventricular septum. Copyright © 2017 The American Association for Thoracic Surgery. All rights reserved.

  20. Urgent surgical management for embolized occluder devices in childhood: single center experience

    Directory of Open Access Journals (Sweden)

    Gokaslan Gokhan

    2012-12-01

    Full Text Available Abstract Background In this study, we sought to analyze our experience in urgent surgical management for embolized cardiac septal and ductal occluder devices resulting from trans-catheter closure of atrial septal defect, ventricular septal defect and patent ductus arteriosus in childhood patient group. Methods We retrospectively reviewed 9 patients (aged 2–15 years who underwent urgent surgery due to cardiac septal and ductal occluder embolization between January 2007 and December 2010. Congenital defects were atrial septal defect (n = 6, ventricular septal defect (n = 1, and patent ductus arteriosus (n = 2. Risk factors for device embolization and urgent surgical management techniques for embolized device removal were discussed. Results Removal of embolized devices in all cases and repair of damaged tricuspid valve in 2 patients were performed. Inevitably, all congenital defects were closed or ligated up to the primary defect. Total circulator arrest necessitated in 1 patient with ascending aortic device embolization. All operations were completed successfully and no hospital mortality or morbidity was encountered. Conclusions Although closure of left to right shunting defects by percutaneous occluder devices has a lot of advantages, device embolization is still a major complication. If embolized device retrieval fails with percutaneous intervention attempts, surgical management is the only method to remove embolized devices. In this circumstance, to provide an uneventful perioperative course, urgent management strategies should be well planned.

  1. Muscular Imbalance Correction in the Power Fitness Training

    OpenAIRE

    Olga E. Aftimichuk; Alexander V. Varvarich

    2013-01-01

    Muscular imbalance is one of the manifestations of pathological-biomechanical changes in muscular-skeletal system. It is the result of tonus-power imbalance of short and relaxed muscles. Muscle shortening is the most striking sign of muscular imbalance. Hypodynamia and passive lifestyle can cause such results. The paper justifies the experimental technique of women muscular imbalances correction by means of power training. Selection of exercises, weights and machines was made, taking into acc...

  2. Stress induced right ventricular dysfunction: An indication of reversible right ventricular ischaemia

    International Nuclear Information System (INIS)

    Underwood, S.R.; Walton, S.; Emanuel, R.W.; Swanton, R.H.; Campos Costa, D.; Laming, P.J.; Ell, P.J.

    1987-01-01

    Stress induced changes in left ventricular ejection fraction are widely used in the detection and assessment of coronary artery disease. This study demonstrates that right ventricular dysfunction may also occur, and assesses its significance in terms of coronary artery anatomy. This study involved 14 normal subjects and 26 with coronary artery disease investigated by equilibrium radionuclide ventriculography, at rest and during maximal dynamic exercise. Mean normal resting right ventricular ejection fraction (RVEF) was 0.40 (SD 0.118), and all normal subjects increased RVEF with stress (mean ΔRVEF+0.13 SD 0.099). Mean ΔRVEF in the subjects with coronary artery disease was significantly lower at 0.00 (SD 0.080), but there was overlap between the two groups. The largest falls in RVEF were seen if the right coronary artery was occluded without retrograde filling. In this subgroup with the most severely compromised right ventricular perfusion (nine subjects), RVEF always fell with stress, and mean ΔRVEF was -0.08 (SD 0.050). There was no significant correlation between ΔLVEF and ΔRVEF, implying that the right ventricular dysfunction was due to right ventricular ischaemia, rather than secondary to left ventricular dysfunction. Stress induced right ventricular ischaemia can therefore be detected readily by radionuclide ventriculography. (orig.)

  3. [Comparison of ablation of left-sided accessory pathway by atrial septal and retrograde arterial approach].

    Science.gov (United States)

    Zhu, J G; Bao, Z Y; Gu, X

    2017-03-07

    Objective: To compare the advantages and disadvantages of radiofrequency ablation of left-sided accessory pathways by via atrial septal approach with retrograde through aortic approach. Methods: A total of 184 patients of left-side accessory pathways were treated in Taizhou People's Hospital and the Subei People's Hospital from March 2012 to August 2015.A total of 103 cases were treated by aortic retrograde approach as through arterial group, 81 cases were treated by punctured atrial septal to left atrial for mapping and ablation as through atrial septal group.Comparison of ablation procedure time, total and pathways of different parts(subgroup) at instant success and relapse rates, safety (serious complications), and statistics other complications in operation and postoperative. Results: Through arterial group and through atrial septal group were no significant difference ( P >0.05) in the ablation procedure time((25±18 ) vs (22±15)min ), instant success(98.1% vs 97.5%) and relapse rates(1.0% vs 1.2%), security(1 vs 0 case). There was no statistical difference in septal part subgroups (all P >0.05) in the ablation procedure time((22±18)vs (25±19)min), instant success(91.7% vs 89.9 %) and relapse rates(0 vs 11.1%); posterior wall subgroup had no statistical difference in the ablation procedure time((18±15)vs (16±12)min), instant success(100% vs 100 %) and relapse rates(0 vs 0)(all P >0.05); side wall subgroup had no statistical difference in the ablation procedure time((29±20)vs (21±18) min), instant success (98.3% vs 98.1%)and relapse rates(1.7% vs 0%)(all P >0.05). Conclusion: Ablation of left-sided accessory pathways by transseptal approach and transaortic approach has no statistical difference in the procedure time, instant success and relapse rates, security.In a particular case, there is a certain complementarity between the two methods.

  4. Left ventricular hypertrabeculation/noncompaction with epilepsy, other heart defects, minor facial anomalies and new copy number variants

    Directory of Open Access Journals (Sweden)

    Nagel Bert

    2012-07-01

    Full Text Available Abstract Background Left ventricular hypertrabeculation/noncompaction (LVHT is a cardiac abnormality of unknown etiology which has been described in children as well as in adults with and without chromosomal aberrations. LVHT has been reported in association with various cardiac and extracardiac abnormalities like epilepsy and facial dysmorphism. Case presentation A unique combination of LVHT, atrial septal defect, pulmonary valve stenosis, aortic stenosis, epilepsy and minor facial anomalies is presented in a 5.5 years old girl. Microarray-based genomic hybridization (array-CGH detected six previously not described copy number variants (CNVs inherited from a clinically unaffected father and minimally affected mother, thus, most likely, not clinically significant but rare benign variants. Conclusions Despite this complex phenotype de novo microdeletions or microduplications were not detected by array CGH. Further investigations, such as whole exome sequencing, could reveal point mutations and small indels as the possible cause.

  5. Muscular Dystrophy

    Science.gov (United States)

    ... Surveillance Tracking and Research Network , known as MD STAR net . Learn more about CDC’s other muscular dystrophy ... for Disease Control and Prevention Email Recommend Tweet YouTube Instagram Listen Watch RSS ABOUT About CDC Jobs ...

  6. Tissue characteristics in left ventricular hypertrophy using magnetic resonance imaging

    International Nuclear Information System (INIS)

    Yoshida, Shigeru; Ueno, Yuji; Arita, Mikio; Nishio, Ichiro; Masuyama, Yoshiaki

    1988-01-01

    For 15 normotensive patients with asymmetric septal hypertrophy (ASH), 10 hypertensive patients with concentric hypertrophy (CH), and five normal subjects (N), we examined changes in myocardial T 1 and T 2 values related to the cardiac cycle. The usefulness of those values in differentiating diseases with left ventricular hypertrophy was evaluated. Left ventricular (LV) short-axis spin echo images and inversion recovery images were obtained at endsystolic and diastolic cardiac phases, and T 1 and T 2 images were calculated. The regional wall thickness (WT) and T 1 and T 2 values were measured in the anterior septum, anterior wall, lateral wall, posterior wall and posterior septum. Myocardial T 1 and T 2 values were significantly decreased in systole (T 1 : 185.6±37.9 msec, T 2 : 24.4±6.3 msec, mean±SD) compared to those in diastole (T 1 : 249.2±56.7 msec, T 2 : 31.7±9.4 msec). In both the ASH and CH groups, significant correlations were observed between diastolic T 1 values and WT (ASH: r = 0.80, p 2 values and WT (ASH: r = 0.58, p 1 values in the ASH group (343.4±40.5 msec) were significantly higher than those of the CH group (247.3±21.4 msec), although the mean wall thickness values were similar in both groups. The T 1 /WT and T 2 /WT were significantly lower in the CH group than those in the ASH and N groups. In conclusion, myocardial T 1 and T 2 values were related not only to the cardiac cycle, but to wall thickness and to types of hypertrophy. The T 1 and T 2 values may be useful for distinguishing hypertrophic cardiomyopathy from hypertrophy due to hypertension. (author)

  7. Lateral septal vasopressin in rats : Role in social and object recognition?

    NARCIS (Netherlands)

    Everts, H.G J; Koolhaas, J.M.

    1997-01-01

    The capacity of male rats to remember familiar conspecifics is called social recognition. It is a form of short-term memory modulated by lateral septal (LS) vasopressin (VP). The specificity of this phenomenon was studied by examining whether recognition of previously investigated objects is also

  8. One and a half ventricular repair as an alternative for hypoplastic right ventricle.

    Science.gov (United States)

    Maluf, Miguel Angel; Carvalho, Antonio Carlos; Carvalho, Werther Brunow

    2010-01-01

    Patients with complex congenital heart disease, characterized by right ventricle hypoplasia, had a palliative surgical option with one and a half ventricular repair. From July 2001 to March 2009, nine patients (mean age 5.2 years, range 3 to 9 years) with hypoplastic right ventricle, underwent correction with one and a half ventricle technique. Preoperative diagnoses included: pulmonary atresia with intact ventricular septum, in six and Ebstein's anomaly, in three cases. Six patients had bidirectional cavo-pulmonary shunt (Glenn operation) previously. The surgical approach was performed with cardiopulmonary bypass to correct intracardiac defects: atrial septal defect closure (nine cases); right ventricle outlet tract reconstruction with porcine pulmonary prosthesis (seven cases); tricuspid valvuloplasty (three cases). There was one (11.1%) hospital death. All the patients left the hospital in good clinical conditions. One patient presented pulmonary stenosis at distal prosthesis anastomosis and needed surgical correction. There was one (12.5%) late deaths after reoperation. At mean follow-up of 39.8 months (range 16 months to 8.4 years) seven patients are alive in functional class I (NYHA). Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic right ventricle by means of one and a half ventricle repair has the advantages of reducing the surgical risk of biventricular repair compared to the Fontan circulation; it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation with short and medium-term promising results. Longer follow-up is needed to prove the efficacy of such a repair in the long term.

  9. Caffeine ingestion acutely enhances muscular strength and power but not muscular endurance in resistance-trained men.

    Science.gov (United States)

    Grgic, Jozo; Mikulic, Pavle

    2017-09-01

    The goal of this randomized, double-blind, cross-over study was to assess the acute effects of caffeine ingestion on muscular strength and power, muscular endurance, rate of perceived exertion (RPE), and pain perception (PP) in resistance-trained men. Seventeen volunteers (mean ± SD: age = 26 ± 6 years, stature = 182 ± 9 cm, body mass = 84 ± 9 kg, resistance training experience = 7 ± 3 years) consumed placebo or 6 mg kg -1 of anhydrous caffeine 1 h before testing. Muscular power was assessed with seated medicine ball throw and vertical jump exercises, muscular strength with one-repetition maximum (1RM) barbell back squat and bench press exercises, and muscular endurance with repetitions of back squat and bench press exercises (load corresponding to 60% of 1RM) to momentary muscular failure. RPE and PP were assessed immediately after the completion of the back squat and bench press exercises. Compared to placebo, caffeine intake enhanced 1RM back squat performance (+2.8%; effect size [ES] = 0.19; p = .016), which was accompanied by a reduced RPE (+7%; ES = 0.53; p = .037), and seated medicine ball throw performance (+4.3%, ES = 0.32; p = .009). Improvements in 1RM bench press were not noted although there were significant (p = .029) decreases in PP related to this exercise when participants ingested caffeine. The results point to an acute benefit of caffeine intake in enhancing lower-body strength, likely due to a decrease in RPE; upper-, but not lower-body power; and no effects on muscular endurance, in resistance-trained men. Individuals competing in events in which strength and power are important performance-related factors may consider taking 6 mg kg -1 of caffeine pre-training/competition for performance enhancement.

  10. Experiences with surgical treatment of ventricle septal defect as a post infarction complication

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    Stich Kathrin

    2009-01-01

    Full Text Available Abstract Background Complications of acute myocardial infarction (AMI with mechanical defects are associated with poor prognosis. Surgical intervention is indicated for a majority of these patients. The goal of surgical intervention is to improve the systolic cardiac function and to achieve a hemodynamic stability. In this present study we reviewed the outcome of patients with post infarction ventricular septal defect (PVSD who underwent cardiac surgery. Methods We analysed retrospectively the hospital records of 41 patients, whose ages range from 48 to 81, and underwent a surgical treatment between 1990 and 2005 because of PVSD. Results In 22 patients concomitant coronary artery bypass grafting (CAGB was performed. In 15 patients a residual shunt was found, this required re-op in seven of them. The time interval from infarct to rupture was 8.7 days and from rupture to surgery was 23.1 days. Hospital mortality in PVSD group was 32%. The mortality of urgent repair within 3 days of intractable cardiogenic shock was 100%. The mortality of patients with an anterior VSD and a posterior VSD was 29.6% vs 42.8%, respectively. All patients who underwent the surgical repair later than day 36 survived. Conclusion Surgical intervention is indicated for a majority of patients with mechanical complications. Cardiogenic shock remains the most important factor that affects the early results. The surgical repair of PVSD should be performed 4–5 weeks after AMI. To improve surgical outcome and hemodynamics the choice of surgical technique and surgical timing as well as preoperative management should be tailored for each patient individually.

  11. Sequential change of cardiomyopathy of Duchenne muscular dystrophy by 201Tl myocardial SPECT

    International Nuclear Information System (INIS)

    Ono, Seiji; Sugimoto, Seiichirou; Inoue, Kenjirou; Jinnouchi, Seishi; Hoshi, Hiroaki; Watanabe, Katsushi.

    1989-01-01

    201 Tl myocardial SPECT were performed to evaluate of cardiomyopathy in Duchenne type of progressive muscular dystrophy (DMD). Follow up SPECT images of the same patients were also obtained about 1 year after the first scan. Cases subjected to study were 10 DMD. At the first study the hypoperfusion area of the left ventricular muscle were observed in 6 cases (60%) out of 10. At the second study the hypoperfusion areas became wider and lower in 4 out of 6 cases (66.7%). The new hypoperfusion area which was not demonstrated at the first study was observed at the second study in one case of these cases. These results suggested that the positive rate of cardiomyopathy in DMD by 201 Tl myocardial SPECT was high, and 201 Tl myocardial SPECT is a useful examination to detect the change of myocardial damage in DMD. (author)

  12. Decellularization of Human Nasal Septal Cartilage for the Novel Filler Material of Vocal Fold Augmentation.

    Science.gov (United States)

    Kang, Dae-Woon; Shin, Sung-Chan; Jang, Jeon-Yeob; Park, Hee-Young; Lee, Jin-Choon; Wang, Soo-Geun; Lee, Byung-Joo

    2017-01-01

    The clinical application of allogenic and/or xenogenic cartilage for vocal fold augmentation requires to remove the antigenic cellular component. The objective of this study was to assess the effect of cartilage decellularization and determine the change in immunogenicity after detergent treatment in human nasal septal cartilage flakes made by the freezing and grinding method. Human nasal septal cartilages were obtained from surgical cases. The harvested cartilages were treated by the freezing and grinding technique. The obtained cartilage flakes were treated with 1% Triton X-100 or 2% sodium dodecyl sulfate (SDS) for decellularization of the cartilage flakes. Hematoxylin and eosin stain (H&E stain), surface electric microscopy, immunohistochemical stain for major histocompatibility complex I and II, and ELISA for DNA contents were performed to assess the effect of cartilage decellularization after detergent treatment. A total of 10 nasal septal cartilages were obtained from surgical cases. After detergent treatment, the average size of the cartilage flakes was significantly decreased. With H&E staining, the cell nuclei of decellularized cartilage flakes were not observed. The expression of major histocompatibility complex (MHC)-I and II antigens was not identified in the decellularized cartilage flakes after treatment with detergent. DNA content was removed almost entirely from the decellularized cartilage flakes. Treatment with 2% SDS or 1% Triton X-100 for 1 hour appears to be a promising method for decellularization of human nasal septal cartilage for vocal fold augmentation. Copyright © 2017 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

  13. Reliability and limitation of various diagnostic methods including nuclear medicine in myocardial disease

    International Nuclear Information System (INIS)

    Tokuyasu, Yoshiki; Kusakabe, Kiyoko; Yamazaki, Toshio

    1981-01-01

    Electrocardiography (ECG), echocardiography, nuclear method, cardiac catheterization, left ventriculography and endomyocardial biopsy (biopsy) were performed in 40 cases of cardiomyopathy (CM), 9 of endocardial fibroelastosis and 19 of specific heart muscle disease, and the usefulness and limitation of each method was comparatively estimated. In CM, various methods including biopsy were performed. The 40 patients were classified into 3 groups, i.e., hypertrophic (17), dilated (20) and non-hypertrophic.non-dilated (3) on the basis of left ventricular ejection fraction and hypertrophy of the ventricular wall. The hypertrophic group was divided into 4 subgroups: 9 septal, 4 apical, 2 posterior and 2 anterior. The nuclear study is useful in assessing the site of the abnormal ventricular thickening, perfusion defect and ventricular function. Echocardiography is most useful in detecting asymmetric septal hypertrophy. The biopsy gives the sole diagnostic clue, especially in non-hypertrophic.non-dilated cardiomyopathy. ECG is useful in all cases but correlation with the site of disproportional hypertrophy was not obtained. (J.P.N.)

  14. Suture fixation of migrated septal occluder device to prevent further migration: a simple surgical technique

    Directory of Open Access Journals (Sweden)

    Mohite Prashant N

    2013-01-01

    Full Text Available Abstract As the use of percutaneous intervention is increasing for the closure of the atrial septal defect, the procedure related complications are also on rise, migration of the device being most common. The migrated devices with failed percutaneous retrieval must be removed surgically under cardiopulmonary bypass. During establishment of cardiopulmonary bypass, the handling of heart may cause further migration of the device into other chambers of heart which leads to difficulty in finding and retrieval of the device. The authors propose a simple and unique technique to prevent further migration of the septal occluder device.

  15. Three-dimensional echocardiographic assessment of atrial septal defects

    Directory of Open Access Journals (Sweden)

    Charles German

    2015-01-01

    Full Text Available Echocardiography provides a useful tool in the diagnosis of many congenital heart diseases, including atrial septal defects, and aids in further delineating treatment options. Although two-dimensional echocardiography has been the standard of care in this regard, technological advancements have made three-dimensional echocardiography possible, and the images obtained in this new imaging modality are able to accurately portray the morphology, location, dimensions, and dynamic changes of defects and many other heart structures during the cardiac cycle.

  16. Association between septal deviation and sinonasal papilloma.

    Science.gov (United States)

    Nomura, Kazuhiro; Ogawa, Takenori; Sugawara, Mitsuru; Honkura, Yohei; Oshima, Hidetoshi; Arakawa, Kazuya; Oshima, Takeshi; Katori, Yukio

    2013-12-01

    Sinonasal papilloma is a common benign epithelial tumor of the sinonasal tract and accounts for 0.5% to 4% of all nasal tumors. The etiology of sinonasal papilloma remains unclear, although human papilloma virus has been proposed as a major risk factor. Other etiological factors, such as anatomical variations of the nasal cavity, may be related to the pathogenesis of sinonasal papilloma, because deviated nasal septum is seen in patients with chronic rhinosinusitis. We, therefore, investigated the involvement of deviated nasal septum in the development of sinonasal papilloma. Preoperative computed tomography or magnetic resonance imaging findings of 83 patients with sinonasal papilloma were evaluated retrospectively. The side of papilloma and the direction of septal deviation showed a significant correlation. Septum deviated to the intact side in 51 of 83 patients (61.4%) and to the affected side in 18 of 83 patients (21.7%). Straight or S-shaped septum was observed in 14 of 83 patients (16.9%). Even after excluding 27 patients who underwent revision surgery and 15 patients in whom the papilloma touched the concave portion of the nasal septum, the concave side of septal deviation was associated with the development of sinonasal papilloma (p = 0.040). The high incidence of sinonasal papilloma in the concave side may reflect the consequences of the traumatic effects caused by wall shear stress of the high-velocity airflow and the increased chance of inhaling viruses and pollutants. The present study supports the causative role of human papilloma virus and toxic chemicals in the occurrence of sinonasal papilloma.

  17. Three novel serum biomarkers, miR-1, miR-133a, and miR-206 for Limb-girdle muscular dystrophy, Facioscapulohumeral muscular dystrophy, and Becker muscular dystrophy.

    Science.gov (United States)

    Matsuzaka, Yasunari; Kishi, Soichiro; Aoki, Yoshitsugu; Komaki, Hirofumi; Oya, Yasushi; Takeda, Shin-Ichi; Hashido, Kazuo

    2014-11-01

    Muscular dystrophies are a clinically and genetically heterogeneous group of inherited myogenic disorders. In clinical tests for these diseases, creatine kinase (CK) is generally used as diagnostic blood-based biomarker. However, because CK levels can be altered by various other factors, such as vigorous exercise, etc., false positive is observed. Therefore, three microRNAs (miRNAs), miR-1, miR-133a, and miR-206, were previously reported as alternative biomarkers for duchenne muscular dystrophy (DMD). However, no alternative biomarkers have been established for the other muscular dystrophies. We, therefore, evaluated whether these miR-1, miR-133a, and miR-206 can be used as powerful biomarkers using the serum from muscular dystrophy patients including DMD, myotonic dystrophy 1 (DM1), limb-girdle muscular dystrophy (LGMD), facioscapulohumeral muscular dystrophy (FSHD), becker muscular dystrophy (BMD), and distal myopathy with rimmed vacuoles (DMRV) by qualitative polymerase chain reaction (PCR) amplification assay. Statistical analysis indicated that all these miRNA levels in serum represented no significant differences between all muscle disorders examined in this study and controls by Bonferroni correction. However, some of these indicated significant differences without correction for testing multiple diseases (P < 0.05). The median values of miR-1 levels in the serum of patients with LGMD, FSHD, and BMD were approximately 5.5, 3.3 and 1.7 compared to that in controls, 0.68, respectively. Similarly, those of miR-133a and miR-206 levels in the serum of BMD patients were about 2.5 and 2.1 compared to those in controls, 1.03 and 1.32, respectively. Taken together, our data demonstrate that levels of miR-1, miR-133a, and miR-206 in serum of BMD and miR-1 in sera of LGMD and FSHD patients showed no significant differences compared with those of controls by Bonferroni correction. However, the results might need increase in sample sizes to evaluate these three miRNAs as

  18. Atrial Septal Aneurysm and Patent Foramen Ovale as Risk Factors for Cryptogenic Stroke in Patients Less Than 55 Years of Age: A Study using Transesophageal Echocardiography

    Science.gov (United States)

    Cabanes, L.; Mas, J. L.; Cohen, A.; Amarenco, P.; Cabanes, P. A.; Oubary, P.; Chedru, F.; Guerin, F.; Bousser, M. G.; deRecondo, J.

    1993-01-01

    Background and Purpose: An association between atrial septal aneurysm and embolic events has been suggested. Atrial septal aneurysm has been shown to be associated with patent foramen ovale and,.in some reports, with mitral valve prolapse. These two latter cardiac disorder; have been identified as potential risk factors for ischemic stroke. The aim of this prospective study was to assess the role of atrial septal aneurysm as an independent risk factor for stroke, especially for cryptogenic stroke. Methods: We studied the prevalence of atrial septal aneurysm, patent foramen ovale, and mitral valve prolapse in 100 consecutive patients ischemic stroke who underwent extensive etiological investigations. We compared these results with those in a control group of 50 consecutive patients. The diagnosis of atrial septal aneurysm and patent foramen ovale relied on transesophageal echocardiography with a contrast study and that of mitral valve prolapse, on two-dimensional transthoracic echocardiography. Results: Stepwise logistic regression analysis showed that atrial septal aneurysm (odds ratio, 4.3; 95% confidence interval, 1.3 to 14.6; P=.01) and patent foramen ovale (odds ratio, 3.9; 95% confidence interval, 1.5 to 10; P=.003) but not mitral valve prolapse were significantly associated with the diagnosis of cryptogenic stroke. The stroke odds of a patient with both atrial septal aneurysm and patent foramen ovale were 33.3 times (95% confidence interval, 4.1 to 270) the stroke odds of a patient with neither of these cardiac disorders. For a patient with atrial septal aneurysm of >lo-mm excursion, the stroke odds were approximately 8 times the stroke odds of a patient with atrial septal aneurysm of stroke and that their association has a marked synergistic effect. Atrial septal aneurysms of >lo-mm excursion are associated with a higher risk of stroke. (Stroke. 1993;24:1865-1873.) KEY WORDS aneurysm echocardiography foramen ovale, patent mitral valve prolapse o young adults

  19. [Right patent ductus arteriosus with an ipsilateral aortic arch: percutaneous closure with amplatzer devices].

    Science.gov (United States)

    Santiago, Justo; Acuña, Manuel; Arispe, Elizabeth; Camargo, Ronaldo; Neves, Juliana; Arnoni, Daniel; Fontes, Valmir F; Pedra, Carlos A

    2007-03-01

    The association of a right aortic arch with an ipsilateral patent ductus arteriosus is rare, especially when there are no other intracardiac anomalies. We report three female patients aged 26, 35 and 9 years with this combination in whom previous attempts at surgical closure by thoracotomy and sternotomy were unsuccessful and who subsequently underwent successful percutaneous closure of the defects using Amplatzer devices. In two patients, although angiography demonstrated the presence of type-A patent ductus arteriosus, it was not possible to determine the minimum diameter accurately and it was necessary to measure it using a sizing balloon. An Amplatzer duct occluder was used in two patients and an Amplatzer muscular ventricular septal defect occluder, in the other. In all patients, full closure was confirmed in the catheterization laboratory and the patients were discharged on the same day with no complications. Percutaneous closure of a right patent ductus arteriosus associated with a right aortic arch is feasible, safe and effective.

  20. Altered myofilament structure and function in dogs with Duchenne muscular dystrophy cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Ait Mou, Younss; Lacampagne, Alain; Irving, Thomas; Scheuermann, Valérie; Blot, Stéphane; Ghaleh, Bijan; de Tombe, Pieter P.; Cazorla, Olivier

    2018-01-01

    Aim Duchenne Muscular Dystrophy (DMD) is associated with progressive depressed left ventricular (LV) function. However, DMD effects on myofilament structure and function are poorly understood. Golden Retriever Muscular Dystrophy (GRMD) is a dog model of DMD recapitulating the human form of DMD. Objective The objective of this study is to evaluate myofilament structure and function alterations in GRMD model with spontaneous cardiac failure. Methods and results We have employed synchrotron X-rays diffraction to evaluate myofilament lattice spacing at various sarcomere lengths (SL) on permeabilized LV myocardium. We found a negative correlation between SL and lattice spacing in both sub-epicardium (EPI) and sub-endocardium (ENDO) LV layers in control dog hearts. In the ENDO of GRMD hearts this correlation is steeper due to higher lattice spacing at short SL (1.9 μm). Furthermore, cross-bridge cycling indexed by the kinetics of tension redevelopment (ktr) was faster in ENDO GRMD myofilaments at short SL. We measured post-translational modifications of key regulatory contractile proteins. S-glutathionylation of cardiac Myosin Binding Protein-C (cMyBP-C) was unchanged and PKA dependent phosphorylation of the cMyBP-C was significantly reduced in GRMD ENDO tissue and more modestly in EPI tissue. Conclusions We found a gradient of contractility in control dogs' myocardium that spreads across the LV wall, negatively correlated with myofilament lattice spacing. Chronic stress induced by dystrophin deficiency leads to heart failure that is tightly associated with regional structural changes indexed by increased myofilament lattice spacing, reduced phosphorylation of regulatory proteins and altered myofilament contractile properties in GRMD dogs.

  1. The septal bulge--an early echocardiographic sign in hypertensive heart disease.

    Science.gov (United States)

    Gaudron, Philipp Daniel; Liu, Dan; Scholz, Friederike; Hu, Kai; Florescu, Christiane; Herrmann, Sebastian; Bijnens, Bart; Ertl, Georg; Störk, Stefan; Weidemann, Frank

    2016-01-01

    Patients in the early stage of hypertensive heart disease tend to have normal echocardiographic findings. The aim of this study was to investigate whether pathology-specific echocardiographic morphologic and functional parameters can help to detect subclinical hypertensive heart disease. One hundred ten consecutive patients without a history and medication for arterial hypertension (AH) or other cardiac diseases were enrolled. Standard echocardiography and two-dimensional speckle-tracking-imaging analysis were performed. Resting blood pressure (BP) measurement, cycle ergometer test (CET), and 24-hour ambulatory BP monitoring (ABPM) were conducted. Patients were referred to "septal bulge (SB)" group (basal-septal wall thickness ≥ 2 mm thicker than mid-septal wall thickness) or "no-SB" group. Echocardiographic SB was found in 48 (43.6%) of 110 patients. In this SB group, 38 (79.2%) patients showed AH either by CET or ABPM. In contrast, in the no-SB group (n = 62), 59 (95.2%) patients had no positive test for AH by CET or ABPM. When AH was solely defined by resting BP, SB was a reasonable predictive sign for AH (sensitivity 73%, specificity 76%). However, when AH was confirmed by CET or ABPM the echocardiographic SB strongly predicted clinical AH (sensitivity 93%, specificity 86%). In addition, regional myocardial deformation of the basal-septum in SB group was significantly lower than in no-SB group (14 ± 4% vs. 17 ± 4%; P heart disease. Sophisticated BP evaluation including resting BP, ABPM, and CET should be performed in all patients with an accidental finding of a SB in echocardiography. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

  2. The Performance of the Upper Limb scores correlate with pulmonary function test measures and Egen Klassifikation scores in Duchenne muscular dystrophy.

    Science.gov (United States)

    Lee, Ha Neul; Sawnani, Hemant; Horn, Paul S; Rybalsky, Irina; Relucio, Lani; Wong, Brenda L

    2016-01-01

    The Performance of the Upper Limb scale was developed as an outcome measure specifically for ambulant and non-ambulant patients with Duchenne muscular dystrophy and is implemented in clinical trials needing longitudinal data. The aim of this study is to determine whether this novel tool correlates with functional ability using pulmonary function test, cardiac function test and Egen Klassifikation scale scores as clinical measures. In this cross-sectional study, 43 non-ambulatory Duchenne males from ages 10 to 30 years and on long-term glucocorticoid treatment were enrolled. Cardiac and pulmonary function test results were analyzed to assess cardiopulmonary function, and Egen Klassifikation scores were analyzed to assess functional ability. The Performance of the Upper Limb scores correlated with pulmonary function measures and had inverse correlation with Egen Klassifikation scores. There was no correlation with left ventricular ejection fraction and left ventricular dysfunction. Body mass index and decreased joint range of motion affected total Performance of the Upper Limb scores and should be considered in clinical trial designs. Copyright © 2016 Elsevier B.V. All rights reserved.

  3. Investigating the Role of Interventricular Interdependence in Development of Right Heart Dysfunction During LVAD Support: A Patient-Specific Methods-Based Approach

    Directory of Open Access Journals (Sweden)

    Kevin L. Sack

    2018-05-01

    Full Text Available Predictive computation models offer the potential to uncover the mechanisms of treatments whose actions cannot be easily determined by experimental or imaging techniques. This is particularly relevant for investigating left ventricular mechanical assistance, a therapy for end-stage heart failure, which is increasingly used as more than just a bridge-to-transplant therapy. The high incidence of right ventricular failure following left ventricular assistance reflects an undesired consequence of treatment, which has been hypothesized to be related to the mechanical interdependence between the two ventricles. To investigate the implication of this interdependence specifically in the setting of left ventricular assistance device (LVAD support, we introduce a patient-specific finite-element model of dilated chronic heart failure. The model geometry and material parameters were calibrated using patient-specific clinical data, producing a mechanical surrogate of the failing in vivo heart that models its dynamic strain and stress throughout the cardiac cycle. The model of the heart was coupled to lumped-parameter circulatory systems to simulate realistic ventricular loading conditions. Finally, the impact of ventricular assistance was investigated by incorporating a pump with pressure-flow characteristics of an LVAD (HeartMate II™ operating between 8 and 12 k RPM in parallel to the left ventricle. This allowed us to investigate the mechanical impact of acute left ventricular assistance at multiple operating-speeds on right ventricular mechanics and septal wall motion. Our findings show that left ventricular assistance reduces myofiber stress in the left ventricle and, to a lesser extent, right ventricle free wall, while increasing leftward septal-shift with increased operating-speeds. These effects were achieved with secondary, potentially negative effects on the interventricular septum which showed that support from LVADs, introduces unnatural bending

  4. Septal membrane localization by C-terminal amphipathic α-helices of MinD in Bacillus subtilis mutant cells lacking MinJ or DivIVA.

    Science.gov (United States)

    Ishikawa, Kazuki; Matsuoka, Satoshi; Hara, Hiroshi; Matsumoto, Kouji

    2017-10-18

    The Min system, which inhibits assembly of the cytokinetic protein FtsZ, is largely responsible for positioning the division site in rod-shaped bacteria. It has been reported that MinJ, which bridges DivIVA and MinD, is targeted to the cell poles by an interaction with DivIVA, and that MinJ in turn recruits MinCD to the cell poles. MinC, however, is located primarily at active division sites at mid-cell when expressed from its native promoter. Surprisingly, we found that Bacillus subtilis MinD is located at nascent septal membranes and at an asymmetric site on lateral membranes between nascent septal membranes in filamentous cells lacking MinJ or DivIVA. Bacillus subtilis MinD has two amphipathic α-helices rich in basic amino acid residues at its C-terminus; one of these, named MTS1 here, is the counterpart of the membrane targeting sequence (MTS) in Escherichia coli MinD while the other, named MTS-like sequence (MTSL), is the nearest helix to MTS1. These amphipathic helices were located independently at nascent septal membranes in cells lacking MinJ or DivIVA, whereas elimination of the helices from the wild type protein reduced its localization considerably. MinD variants with altered MTS1 and MTSL, in which basic amino acid residues were replaced with proline or acidic residues, were not located at nascent septal membranes, indicating that the binding to the nascent septal membranes requires basic residues and a helical structure. The septal localization of MTSL, but not of MTS1, was dependent on host cell MinD. These results suggest that MinD is targeted to nascent septal membranes via its C-terminal amphipathic α-helices in B. subtilis cells lacking MinJ or DivIVA. Moreover, the diffuse distribution of MinD lacking both MTSs suggests that only a small fraction of MinD depends on MinJ for its localization to nascent septal membranes.

  5. Utility of pulmonary venous flow diastolic deceleration time in an adult patient undergoing surgical closure of atrial septal defect and coronary artery bypass grafting

    Directory of Open Access Journals (Sweden)

    Dharmesh R Agrawal

    2013-01-01

    Full Text Available Acute left ventricular (LV failure has been reported after surgical closure of atrial septal defect (ASD in adult patients. We report acute LV failure in a 56 year old gentleman following coronary artery bypass grafting (CABG and surgical closure of ASD. Transesophageal echocardiography examination of the patient following closure of ASD and CABG showed a residual ASD and a shunt (Qp :Qs = 1.5. The residual ASD was closed after re-institution of cardiopulmonary bypass (CPB under cardioplegic cardiac arrest. However, the patient did not tolerate closure of the residual ASD. The CPB was re-established and under cardioplegic cardiac arrest residual ASD was reopened to create a fenestration. This time patient was weaned easily from CPB. Postoperatively, 16 hours after extubation, patient became hemodynamically unstable, the patient was electively put on ventilator and intra-aortic balloon pump. Later the patient was weaned off successfully from ventilator. Retrospective analysis of pulmonary venous flow diastolic deceleration time (PVDT D recorded during prebypass period measured 102 msec suggestive of high left atrial pressure which indicate possibility of LV failure after ASD closure.

  6. VACTERL association with double-chambered left ventricle: A rare occurrence

    Directory of Open Access Journals (Sweden)

    Abdulla Al-Farqani

    2013-01-01

    Full Text Available VACTERL association is a non-random association of birth defects of unknown etiology derived from structures of embryonic mesoderm. The common cardiac defects seen with VACTERL association are ventricular septal defects, atrial septal defects, and tetralogy of Fallot. We present a 2-year-old child with VACTERL association in whom we detected double-chambered left ventricle on transthoracic echocardiography.

  7. Atrofia muscular espinal en el niño Spinal muscular atrophy present in children

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    Nicolás Garófalo Gómez

    2009-09-01

    Full Text Available INTRODUCCIÓN. Las atrofias musculares espinales en la infancia (AME son trastornos genéticos autosómicos recesivos, caracterizados por degeneración de las motoneuronas espinales y bulbares. El presente estudio tuvo el objetivo principal de describir las principales características clínicas en una serie de niños con AME. MÉTODOS. Se realizó un estudio retrospectivo de los pacientes con AME atendidos en el Instituto de Neurología y Neurocirugía de Cuba, entre enero de 1997 y diciembre de 2001. Se recopilaron los datos de 35 pacientes, 4 de ellos, fetos con confirmación prenatal de AME. Se precisaron las principales características clínicas, electromiográficas, de la biopsia muscular y de los estudios genéticos moleculares realizados en cada caso. RESULTADOS. La AME de tipo II resultó la forma clínica más frecuente (58 %, seguida por la AME de tipo I (42 %. Las principales manifestaciones clínicas resultaron la debilidad muscular generalizada con predominio proximal en extremidades, asociada a hipotonía y arreflexia osteotendinosa. La deleción de los exones 7 y 8 del gen SMN1 se detectó en 20 de 23 casos estudiados (87 %.INTRODUCTION: Spinal muscular atrophies (SMA in childhood are autosomal recessive genetic disorders, characterized by spinal and bulbar motoneurons degenerations. Aim of present paper was to describe the main clinical features in a series of children presenting SMA. METHODS: A retrospective study of patients with SMA seen in the Neurology and Neurosurgery Institute of Cuba from January, 2997 and December, 2001 was made. Data from 35 patients were available; four of them were fetus with prenatal confirmation of SMA. Main clinical, electromyography, muscular biopsy, and of molecular genetic studies performed in each case were determined. RESULTS: Type II SMA was the more frequent clinical presentation (58%, followed by type I SMA (42,%. Main clinical manifestations were a systemic muscular weakness with

  8. Radiographic features of Golden Retriever muscular dystrophy.

    Science.gov (United States)

    Brumitt, Jason W; Essman, Stephanie C; Kornegay, Joe N; Graham, John P; Weber, William J; Berry, Clifford R

    2006-01-01

    Golden Retriever muscular dystrophy is an inherited, degenerative myopathy due to the absence of dystrophin and is used as a model of Duchenne muscular dystrophy of young boys. This report describes the radiographic abnormalities of Golden Retriever muscular dystrophy in 26 dogs. The thoracic abnormalities included diaphragmatic asymmetry (18/26), diaphragmatic undulation (18/26), and gastro-esophageal hiatal hernia (6/26). Pelvic abnormalities included narrowing of the body of the ilia (14/19), ventral deviation and curvature of the tuber ischii (14/19), elongation of the obturator foramen with a decrease in opacity of the surrounding bone (12/19), and lateral flaring of the wings of the ilia (12/19). Abdominal abnormalities consisted of hepatomegaly (14/22) and poor serosal detail (12/22). The unique thoracic abnormalities were a consistent finding in affected Golden Retriever muscular dystrophy dogs. The diagnosis of muscular dystrophy should be included in the differential list if the combination of diaphragm undulation and asymmetry, and gastro-esophageal hiatal hernia are identified. These diaphragmatic abnormalities are related to hypertrophy and hyperplasia of the diaphragm. Additionally, the skeletal changes of pelvic tilt, elongation of the pelvis, widening of the obturator foramina and thinning of the ischiatic tables appear to be specific to Golden Retriever muscular dystrophy in dogs. These pelvic abnormalities are most likely secondary to bone remodeling associated with the progressive skeletal myopathy and subsequent contracture/fibrosis.

  9. What Are the Types of Muscular Dystrophy?

    Science.gov (United States)

    ... muscular dystrophy? There are more than 30 forms of muscular dystrophy (MD), with information on the primary types included in the table below. 1 Duchenne (DMD) What It Is Common Symptoms How It ...

  10. Parental attitudes toward newborn screening for Duchenne/Becker muscular dystrophy and spinal muscular atrophy.

    Science.gov (United States)

    Wood, Molly F; Hughes, Sarah C; Hache, Lauren P; Naylor, Edwin W; Abdel-Hamid, Hoda Z; Barmada, M Michael; Dobrowolski, Steven F; Stickler, David E; Clemens, Paula R

    2014-06-01

    Disease inclusion in the newborn screening (NBS) panel should consider the opinions of those most affected by the outcome of screening. We assessed the level and factors that affect parent attitudes regarding NBS panel inclusion of Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), and spinal muscular atrophy (SMA). The attitudes toward NBS for DMD, BMD, and SMA were surveyed and compared for 2 categories of parents, those with children affected with DMD, BMD, or SMA and expectant parents unselected for known family medical history. The level of support for NBS for DMD, BMD, and SMA was 95.9% among parents of children with DMD, BMD, or SMA and 92.6% among expectant parents. There was strong support for NBS for DMD, BMD, and SMA in both groups of parents. Given advances in diagnostics and promising therapeutic approaches, discussion of inclusion in NBS should continue. Copyright © 2013 Wiley Periodicals, Inc.

  11. Mitochondrial disorders in progressive muscular dystrophies

    Directory of Open Access Journals (Sweden)

    D. A. Kharlamov

    2014-01-01

    Full Text Available The literature review gives data on the role of mitochondrial disorders in the pathogenesis of different progressive muscular dystrophies. It describes changes in Duchenne, limb-girdle, facial scapulohumeral (Landuzi—Degerina muscular dystrophies. The review is based on both clinical and experimental animal studies. Along with the implication of mitochondria in the pathogenesis of the diseases, it describes muscular dystrophy treatment options compensating for energy disorders and overcoming oxidative stress and mitochondrial dysfunction. Mitochondrial studies in different muscle diseases hand physicians treatment modalities that fail to lead to recovery, but compensate for disorders caused by mutations in the genetic apparatus. 

  12. Three-dimensional transesophageal echocardiography of the atrial septal defects

    Directory of Open Access Journals (Sweden)

    Romero-Cárdenas Ángel

    2008-07-01

    Full Text Available Abstract Transesophageal echocardiography has advantages over transthoracic technique in defining morphology of atrial structures. Even though real time three-dimensional echocardiographic imaging is a reality, the off-line reconstruction technique usually allows to obtain higher spatial resolution images. The purpose of this study was to explore the accuracy of off-line three-dimensional transesophageal echocardiography in a spectrum of atrial septal defects by comparing them with representative anatomic specimens.

  13. Late bacterial endocarditis of an Amplatzer atrial septal device

    Directory of Open Access Journals (Sweden)

    Bhavith Aruni

    2013-07-01

    Full Text Available A 59-year-old male with an secundum atrial septal defect status post repair with an Amplatzer occluder in 2001 was admitted with sepsis and MRSA bacteremia. Transesophageal Echocardiography (TEE showed presence of an overlying mobile echogenic structure on the left atrial surface of the device suggestive of a vegetation/infected thrombus. This is only the 3rd case description of late endocarditis involving the Amplatzer ASD closure device in an adult.

  14. MR tomography of tetralogy of Fallot. Kernspintomographie der Fallot-Tetralogie

    Energy Technology Data Exchange (ETDEWEB)

    Stark, P.; Agness, M.; Holshouser, B.; Hinshaw, D. Jr. (Loma Linda Univ., CA (USA). Dept. of Radiology)

    1991-08-01

    We present four patients with Tetralogy of Fallot, imaged with magnetic resonance. MRI is useful in clearly depicting the dilated ascending aorta, the infundibular stenosis, ventricular septal defect, right ventricular hypertrophy and dilated bronchial arteries. The pulmonary outflow tract can be visualized better with MR imaging than with ultrasound. (orig.).

  15. MR tomography of tetralogy of Fallot

    International Nuclear Information System (INIS)

    Stark, P.; Agness, M.; Holshouser, B.; Hinshaw, D. Jr.

    1991-01-01

    We present four patients with Tetralogy of Fallot, imaged with magnetic resonance. MRI is useful in clearly depicting the dilated ascending aorta, the infundibular stenosis, ventricular septal defect, right ventricular hypertrophy and dilated bronchial arteries. The pulmonary outflow tract can be visualized better with MR imaging than with ultrasound. (orig.) [de

  16. Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet): case definition in surveillance for childhood-onset Duchenne/Becker muscular dystrophy.

    Science.gov (United States)

    Mathews, Katherine D; Cunniff, Chris; Kantamneni, Jiji R; Ciafaloni, Emma; Miller, Timothy; Matthews, Dennis; Cwik, Valerie; Druschel, Charlotte; Miller, Lisa; Meaney, F John; Sladky, John; Romitti, Paul A

    2010-09-01

    The Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet) is a multisite collaboration to determine the prevalence of childhood-onset Duchenne/Becker muscular dystrophy and to characterize health care and health outcomes in this population. MD STARnet uses medical record abstraction to identify patients with Duchenne/Becker muscular dystrophy born January 1, 1982 or later who resided in 1 of the participating sites. Critical diagnostic elements of each abstracted record are reviewed independently by >4 clinicians and assigned to 1 of 6 case definition categories (definite, probable, possible, asymptomatic, female, not Duchenne/Becker muscular dystrophy) by consensus. As of November 2009, 815 potential cases were reviewed. Of the cases included in analysis, 674 (82%) were either ''definite'' or ''probable'' Duchenne/Becker muscular dystrophy. These data reflect a change in diagnostic testing, as case assignment based on genetic testing increased from 67% in the oldest cohort (born 1982-1987) to 94% in the cohort born 2004 to 2009.

  17. Sequential change of cardiomyopathy of Duchenne muscular dystrophy by /sup 201/Tl myocardial SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Ono, Seiji; Sugimoto, Seiichirou; Inoue, Kenjirou; Jinnouchi, Seishi; Hoshi, Hiroaki; Watanabe, Katsushi.

    1989-03-01

    /sup 201/Tl myocardial SPECT were performed to evaluate of cardiomyopathy in Duchenne type of progressive muscular dystrophy (DMD). Follow up SPECT images of the same patients were also obtained about 1 year after the first scan. Cases subjected to study were 10 DMD. At the first study the hypoperfusion area of the left ventricular muscle were observed in 6 cases (60%) out of 10. At the second study the hypoperfusion areas became wider and lower in 4 out of 6 cases (66.7%). The new hypoperfusion area which was not demonstrated at the first study was observed at the second study in one case of these cases. These results suggested that the positive rate of cardiomyopathy in DMD by /sup 201/Tl myocardial SPECT was high, and /sup 201/Tl myocardial SPECT is a useful examination to detect the change of myocardial damage in DMD. (author).

  18. Becker muscular dystrophy: an unusual presentation.

    OpenAIRE

    Thakker, P B; Sharma, A

    1993-01-01

    A 15 year old boy who presented with passing painless dark urine was found to have myoglobinuria. His creatine phosphokinase was raised, and a muscle biopsy specimen showed non-specific dystrophic changes. Subsequent DNA analysis led to the diagnosis of Becker muscular dystrophy. Myoglobinuria may be a presenting symptom of Becker muscular dystrophy.

  19. Successful anesthetic management of a child with blepharophimosis syndrome and atrial septal defect for reconstructive ocular surgery

    Directory of Open Access Journals (Sweden)

    Dalim Kumar Baidya

    2011-01-01

    Full Text Available Blepharophimosis syndrome is an autosomal dominant disorder characterized by eyelid malformation, involvement of reproductive system and abnormal facial morphology leading to difficult airway. We report a rare association of blepharophimosis syndrome and atrial septal defect in a 10-year-old girl who came for reconstruction surgery of eyelid. The child had dyspnea on exertion. Atrial septal defect was identified preoperatively by clinical examination and echocardiography. Anesthesia management was complicated by failure in laryngeal mask airway placement and Cobra perilaryngeal airway was subsequently used.

  20. What Is a Cardiothoracic Surgeon?

    Science.gov (United States)

    ... Tumors Mediastinal Tumors Achalasia and Esophageal Motility Disorders Pleural Diseases Mesothelioma Heart and Lung Transplantation Organ failure ... the hollow, muscular tube that moves food and liquid from the mouth to the stomach Atrial Septal ...

  1. Acute oral administration of low doses of methylphenidate targets calretinin neurons in the rat septal area.

    Directory of Open Access Journals (Sweden)

    Alvaro eGarcía-Aviles

    2015-03-01

    Full Text Available Methylphenidate (MPD is a commonly administered drug to treat children suffering from attention deficit hyperactivity disorder (ADHD. Alterations in septal driven hippocampal theta rhythm may underlie attention deficits observed in these patients. Amongst others, the septo-hippocampal connections have long been acknowledged to be important in preserving hippocampal function. Thus, we wanted to ascertain if methylphenidate administration, which improves attention in patients, could affect septal areas connecting with hippocampus. We used low and orally administered methylphenidate doses (1.3; 2.7 and 5mg/Kg to rats what mimics the dosage range in humans. In our model, we observed no effect when using 1.3mg/Kg methylphenidate; whereas 2.7 and 5 mg/Kg induced a significant increase in c-fos expression specifically in the medial septum, an area intimately connected to the hippocampus. We analyzed dopaminergic areas such as nucleus accumbens and striatum, and found that only 5mg/Kg induced c-fos levels increase. In these areas tyrosine hydroxylase correlated well with c-fos staining, whereas in the medial septum the sparse tyrosine hydroxylase fibres did not overlap with c-fos positive neurons. Double immunofluorescence of c-fos with neuronal markers in the septal area revealed that co-localization with choline acethyl transferase, parvalbumin, and calbindin with c-fos did not change with MPD treatment; whereas, calretinin and c-fos double labeled neurons increased after MPD administration. Altogether, these results suggest that low and acute doses of methylphenidate primary target specific populations of caltretinin medial septal neurons.

  2. Autonomic, locomotor and cardiac abnormalities in a mouse model of muscular dystrophy: targeting the renin-angiotensin system.

    Science.gov (United States)

    Sabharwal, Rasna; Chapleau, Mark W

    2014-04-01

    New Findings What is the topic of this review? This symposium report summarizes autonomic, cardiac and skeletal muscle abnormalities in sarcoglycan-δ-deficient mice (Sgcd-/-), a mouse model of limb girdle muscular dystrophy, with emphasis on the roles of autonomic dysregulation and activation of the renin-angiotensin system at a young age. What advances does it highlight? The contributions of the autonomic nervous system and the renin-angiotensin system to the pathogenesis of muscular dystrophy are highlighted. Results demonstrate that autonomic dysregulation precedes and predicts later development of cardiac dysfunction in Sgcd-/- mice and that treatment of young Sgcd-/- mice with the angiotensin type 1 receptor antagonist losartan or with angiotensin-(1-7) abrogates the autonomic dysregulation, attenuates skeletal muscle pathology and increases spontaneous locomotor activity. Muscular dystrophies are a heterogeneous group of genetic muscle diseases characterized by muscle weakness and atrophy. Mutations in sarcoglycans and other subunits of the dystrophin-glycoprotein complex cause muscular dystrophy and dilated cardiomyopathy in animals and humans. Aberrant autonomic signalling is recognized in a variety of neuromuscular disorders. We hypothesized that activation of the renin-angiotensin system contributes to skeletal muscle and autonomic dysfunction in mice deficient in the sarcoglycan-δ (Sgcd) gene at a young age and that this early autonomic dysfunction contributes to the later development of left ventricular (LV) dysfunction and increased mortality. We demonstrated that young Sgcd-/- mice exhibit histopathological features of skeletal muscle dystrophy, decreased locomotor activity and severe autonomic dysregulation, but normal LV function. Autonomic regulation continued to deteriorate in Sgcd-/- mice with age and was accompanied by LV dysfunction and dilated cardiomyopathy at older ages. Autonomic dysregulation at a young age predicted later development of

  3. Relationship between Fibrosis and Ventricular Arrhythmias in Chagas Heart Disease Without Ventricular Dysfunction

    Energy Technology Data Exchange (ETDEWEB)

    Tassi, Eduardo Marinho, E-mail: etassi@ibest.com.br [Instituto de Cardiologia Edson Saad - Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ (Brazil); Continentino, Marcelo Abramoff [Hospital Frei Galvão, Guaratinguetá, SP (Brazil); Nascimento, Emília Matos do; Pereira, Basílio de Bragança [Instituto de Cardiologia Edson Saad - Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ (Brazil); Coppe - Instituto Alberto Luiz Coimbra de Pós-Graduação e Pesquisa de Engenharia - UFRJ, Rio de Janeiro, RJ (Brazil); Pedrosa, Roberto Coury [Instituto de Cardiologia Edson Saad - Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ (Brazil)

    2014-05-15

    Patients with Chagas disease and segmental wall motion abnormality (SWMA) have worse prognosis independent of left ventricular ejection fraction (LVEF). Cardiac magnetic resonance (CMR) is currently the best method to detect SWMA and to assess fibrosis. To quantify fibrosis by using late gadolinium enhancement CMR in patients with Chagas disease and preserved or minimally impaired ventricular function (> 45%), and to detect patterns of dependence between fibrosis, SWMA and LVEF in the presence of ventricular arrhythmia. Electrocardiogram, treadmill exercise test, Holter and CMR were carried out in 61 patients, who were divided into three groups as follows: (1) normal electrocardiogram and CMR without SWMA; (2) abnormal electrocardiogram and CMR without SWMA; (3) CMR with SWMA independently of electrocardiogram. The number of patients with ventricular arrhythmia in relation to the total of patients, the percentage of fibrosis, and the LVEF were, respectively: Group 1, 4/26, 0.74% and 74.34%; Group 2, 4/16, 3.96% and 68.5%; and Group 3, 11/19, 14.07% and 55.59%. Ventricular arrhythmia was found in 31.1% of the patients. Those with and without ventricular arrhythmia had mean LVEF of 59.87% and 70.18%, respectively, and fibrosis percentage of 11.03% and 3.01%, respectively. Of the variables SWMA, groups, age, LVEF and fibrosis, only the latter was significant for the presence of ventricular arrhythmia, with a cutoff point of 11.78% for fibrosis mass (p < 0.001). Even in patients with Chagas disease and preserved or minimally impaired ventricular function, electrical instability can be present. Regarding the presence of ventricular arrhythmia, fibrosis is the most important variable, its amount being proportional to the complexity of the groups.

  4. Relationship between Fibrosis and Ventricular Arrhythmias in Chagas Heart Disease Without Ventricular Dysfunction

    International Nuclear Information System (INIS)

    Tassi, Eduardo Marinho; Continentino, Marcelo Abramoff; Nascimento, Emília Matos do; Pereira, Basílio de Bragança; Pedrosa, Roberto Coury

    2014-01-01

    Patients with Chagas disease and segmental wall motion abnormality (SWMA) have worse prognosis independent of left ventricular ejection fraction (LVEF). Cardiac magnetic resonance (CMR) is currently the best method to detect SWMA and to assess fibrosis. To quantify fibrosis by using late gadolinium enhancement CMR in patients with Chagas disease and preserved or minimally impaired ventricular function (> 45%), and to detect patterns of dependence between fibrosis, SWMA and LVEF in the presence of ventricular arrhythmia. Electrocardiogram, treadmill exercise test, Holter and CMR were carried out in 61 patients, who were divided into three groups as follows: (1) normal electrocardiogram and CMR without SWMA; (2) abnormal electrocardiogram and CMR without SWMA; (3) CMR with SWMA independently of electrocardiogram. The number of patients with ventricular arrhythmia in relation to the total of patients, the percentage of fibrosis, and the LVEF were, respectively: Group 1, 4/26, 0.74% and 74.34%; Group 2, 4/16, 3.96% and 68.5%; and Group 3, 11/19, 14.07% and 55.59%. Ventricular arrhythmia was found in 31.1% of the patients. Those with and without ventricular arrhythmia had mean LVEF of 59.87% and 70.18%, respectively, and fibrosis percentage of 11.03% and 3.01%, respectively. Of the variables SWMA, groups, age, LVEF and fibrosis, only the latter was significant for the presence of ventricular arrhythmia, with a cutoff point of 11.78% for fibrosis mass (p < 0.001). Even in patients with Chagas disease and preserved or minimally impaired ventricular function, electrical instability can be present. Regarding the presence of ventricular arrhythmia, fibrosis is the most important variable, its amount being proportional to the complexity of the groups

  5. Oxidative muscular injury and its relevance to hyperthyroidism.

    Science.gov (United States)

    Asayama, K; Kato, K

    1990-01-01

    In experimental hyperthyroidism, acceleration of lipid peroxidation occurs in heart and slow-oxidative muscles, suggesting the contribution of reactive oxygen species to the muscular injury caused by thyroid hormones. This article reviews various models of oxidative muscular injury and considers the relevance of the accompanying metabolic derangements to thyrotoxic myopathy and cardiomyopathy, which are the major complications of hyperthyroidism. The muscular injury models in which reactive oxygen species are supposed to play a role are ischemia/reperfusion syndrome, exercise-induced myopathy, heart and skeletal muscle diseases related to the nutritional deficiency of selenium and vitamin E and related disorders, and genetic muscular dystrophies. These models provide evidence that mitochondrial function and the glutathione-dependent antioxidant system are important for the maintenance of the structural and functional integrity of muscular tissues. Thyroid hormones have a profound effect on mitochondrial oxidative activity, synthesis and degradation of proteins and vitamin E, the sensitivity of the tissues to catecholamine, the differentiation of muscle fibers, and the levels of antioxidant enzymes. The large volume of circumstantial evidence presented here indicates that hyperthyroid muscular tissues undergo several biochemical changes that predispose them to free radical-mediated injury.

  6. Muscular Dystrophy (MD)

    Science.gov (United States)

    ... patients may need assisted ventilation to treat respiratory muscle weakness and a pacemaker for cardiac abnormalities. View Full Treatment Information Definition The muscular dystrophies (MD) are a group of more than 30 ...

  7. Self-expanding nanoplatinum-coated nitinol devices for atrial septal defect and patent ductus arteriosus closure: a swine model.

    Science.gov (United States)

    Lertsapcharoen, Pornthep; Khongphatthanayothin, Apichai; La-orkhun, Vidhavas; Supachokchaiwattana, Pentip; Charoonrut, Phingphol

    2006-01-01

    Our purpose was to evaluate self-expanding nanoplatinum-coated nitinol devices for transcatheter closure of atrial septal defects and patent ductus arteriosus in a swine model. The devices were braided from platinum-activated nitinol wires and filled with polyester to enhance thrombogenicity. The platinum activation of the nitinol wires was carried out with the help of Nanofusion technology. The coating of platinum covers the exposed surface of the nitinol wires and prevents the release of nickel into the blood stream after the implantation of the device but does not affect its shape memory, which makes the device self-expanding after it is loaded from the catheter. Atrial septal defects were created in 12 piglets by balloon dilation of the patent foramen ovale. The size of the device was selected on the basis of the diameter of the balloon and the size of the defect, measured by transthoracic echocardiography. The devices were successfully deployed in all 12 piglets under fluoroscopic study. Transthoracic color Doppler echocardiograms showed complete closure of the atrial septal defect within 15 minutes of device implantation. Twelve patent ductus arteriosus closure devices were deployed in the right or left subclavian arteries in 10 piglets. Angiograms showed complete occlusion of the subclavian arteries within a few minutes of device deployment. In the atrial septal defect cases, the autopsy findings showed complete organizing fibrin thrombus formation and complete neo-endothelialization on the outer surface of the devices within one week and six weeks of implantation, respectively. The use of self-expanding nanoplatinum-coated nitinol devices for the transcatheter closure of atrial septal defects and patent ductus arteriosus is feasible. The excellent occlusion result and complete neo-endothelialization of the devices in the swine model is an indication of the potential of these devices in human application.

  8. Respiratory function in facioscapulohumeral muscular dystrophy 1

    NARCIS (Netherlands)

    Wohlgemuth, M.; Horlings, G.C.; Kooi, E.L. van der; Gilhuis, H.J.; Hendriks, J.C.M.; Maarel, S.M. van der; Engelen, B.G.M. van; Heijdra, Y.F.; Padberg, G.W.A.M.

    2017-01-01

    To test the hypothesis that wheelchair dependency and (kypho-)scoliosis are risk factors for developing respiratory insufficiency in facioscapulohumeral muscular dystrophy, we examined 81 patients with facioscapulohumeral muscular dystrophy 1 of varying degrees of severity ranging from ambulatory

  9. [Human myopathy and animal muscular dystrophy].

    Science.gov (United States)

    Schapira, G; Dreyfus, J C; Schapira, F

    1977-08-01

    Two hereditary muscular dystrophies similar to human progressive muscular dystrophy (P.M.D. Duchenne type) have been isolated in animals, one in mouse, the other in chicken. The decrease in the activity of glycogenolytic enzymes is similar to that observed in denervated muscle. Isozymic fetal types for several muscular enzymes have been observed as well in chicken as in man, but this fetal type may also be found in neurogenic atrophy. The release in circulation of muscle enzymes seems more specific. But the origin of the genetic lesion is still unknown. We describe here the three different theories about this problem: i.e. neurogenic, vascular, or myogenic. This last theory implies a trouble of membrane permeability.

  10. Dual myocardial scintigraphic imaging using 123I-BMIPP and 201Tl in patients with Duchenne's progressive muscular dystrophy

    International Nuclear Information System (INIS)

    Sasaki, Akira

    1996-01-01

    Dual single photon emission computed tomography (SPECT) was performed in 30 patients with Duchenne's muscular dystrophy (DMD) using 201 thallium (Tl) for myocardial perfusion 123 I-β-methyl-p-iodophenylpentadecanoic acid (BMIPP) for myocardial fatty acid metabolism scintigraphy. The left ventricle was divided into 9 regions, and accumulation of the radiotracers was assessed visually for each region to calculate defect score for each tracer. There was some degree of decrease in the myocardial accumulation of both tracers in all DMD patients. Reduced accumulation was most common at the apex (BMIPP, 70%; Tl, 63%), followed by the posterior wall, lateral wall, and anterior wall. It was less common on the apical side of the ventricular septum for both tracers (BMIPP, 3%; Tl, 17%). Accumulation of Tl was lower than BMIPP in 18/30 patients (60%) and higher in 9 (30%), while both tracers showed equal accumulation in 3 (10%). BMIPP showed higher accumulation than Tl in all regions but the septum. A significant negative correlation was found between the defect scores for both tracers and the left ventricular ejection fraction (LVEF) (r=-0.629 for Tl; r=-0.567 for BMIPP). The strongest negative correlation was that between the sum of the Tl and BMIPP defect scores and the LVEF (r=-0.681). Dual SPECT myocardial scintigraphy with Tl and BMIPP provided an accurate evaluation of the progression of cardiac lesions in DMD by detecting abnormalities of the myocardial metabolism of each substance thereby enabling the assessment of left ventricular function. (author)

  11. Amiodarone for the treatment and prevention of ventricular fibrillation and ventricular tachycardia

    Directory of Open Access Journals (Sweden)

    Hugo Van Herendael

    2010-06-01

    Full Text Available Hugo Van Herendael, Paul DorianDivision of Cardiology, St. Michael’s Hospital, University of Toronto, Toronto, CanadaAbstract: Amiodarone has emerged as the leading antiarrhythmic therapy for termination and prevention of ventricular arrhythmia in different clinical settings because of its proven efficacy and safety. In patients with shock refractory out-of-hospital cardiac arrest and hemodynamically destabilizing ventricular arrhythmia, amiodarone is the most effective drug available to assist in resuscitation. Although the superiority of the transvenous implantable cardioverter defibrillator (ICD over amiodarone has been well established in the preventive treatment of patients at high risk of life-threatening ventricular arrhythmias, amiodarone (if used with a beta-blocker is the most effective antiarrhythmic drug to prevent ICD shocks and treat electrical storm. Both the pharmacokinetics and the electrophysiologic profile of amiodarone are complex, and its optimal and safe use requires careful patient surveillance with respect to potential adverse effects.Keywords: amiodarone, ventricular fibrillation, unstable ventricular tachycardia

  12. Longitudinal evaluation of P-wave dispersion and P-wave maximum in children after transcatheter device closure of secundum atrial septal defect.

    Science.gov (United States)

    Grignani, Robert Teodoro; Tolentino, Kim Martin; Rajgor, Dimple Dayaram; Quek, Swee Chye

    2015-06-01

    Transcatheter device closure of the secundum atrial septal defect (ASD) in children prevents atrial arrhythmias in older age. However, the benefits of favourable atrial electrocardiographic markers in these children remain elusive. We aimed to review the electrocardiographic markers of atrial activity in a longitudinal fashion. We retrospectively reviewed longitudinal data of all children who underwent transcatheter device closure at the National University Hospital between 2004 and 2013. The inclusion criteria included the presence of a secundum-type ASD with left to right shunt and evidence of increased right ventricular volume load (Q p/Q s ratio >1.5 and/or right ventricular dilatation). A total of 25 patients with a mean follow-up of 44.7 ± 33.47 (7.3-117.4) months were included. P maximum and P dispersion decreased at 2 months, P amplitude at 1 week and remained so until last follow-up. A positive trend was seen with a correlation coefficient of +0.12 for P maximum, +0.08 for P dispersion and 0.34 for P amplitude. There was a higher baseline P amplitude and P dispersion in patients who were older than 10 years and a non-significant trend to support an increase in both P maximum (71.0 ± 8.8 vs. 73.2 ± 12.7), P dispersion (17.0 ± 6.5 vs. 22.0 ± 11.3) and P amplitude (0.88 ± 0.25 vs. 1.02 ± 0.23) in patients with an ASD more than 15 mm compared with an ASD <15 mm. There is reduction in both P maximum and P dispersion as early as 2 months, which persisted on follow-up. Earlier closure may result in more favourable electrocardiographic results.

  13. Recent developments in the treatment of Duchenne muscular dystrophy and spinal muscular atrophy

    Science.gov (United States)

    Liew, Wendy K. M.

    2013-01-01

    Pediatric neuromuscular disorders comprise a large variety of disorders that can be classified based on their neuroanatomical localization, patterns of weakness, and laboratory test results. Over the last decade, the field of translational research has been active with many ongoing clinical trials. This is particularly so in two common pediatric neuromuscular disorders: Duchenne muscular dystrophy and spinal muscular atrophy. Although no definitive therapy has yet been found, numerous active areas of research raise the potential for novel therapies in these two disorders, offering hope for improved quality of life and life expectancy for affected individuals. PMID:23634188

  14. The overloaded right heart and ventricular interdependence.

    Science.gov (United States)

    Naeije, Robert; Badagliacca, Roberto

    2017-10-01

    The right and the left ventricle are interdependent as both structures are nested within the pericardium, have the septum in common and are encircled with common myocardial fibres. Therefore, right ventricular volume or pressure overloading affects left ventricular function, and this in turn may affect the right ventricle. In normal subjects at rest, right ventricular function has negligible interaction with left ventricular function. However, the right ventricle contributes significantly to the normal cardiac output response to exercise. In patients with right ventricular volume overload without pulmonary hypertension, left ventricular diastolic compliance is decreased and ejection fraction depressed but without intrinsic alteration in contractility. In patients with right ventricular pressure overload, left ventricular compliance is decreased with initial preservation of left ventricular ejection fraction, but with eventual left ventricular atrophic remodelling and altered systolic function. Breathing affects ventricular interdependence, in healthy subjects during exercise and in patients with lung diseases and altered respiratory system mechanics. Inspiration increases right ventricular volumes and decreases left ventricular volumes. Expiration decreases both right and left ventricular volumes. The presence of an intact pericardium enhances ventricular diastolic interdependence but has negligible effect on ventricular systolic interdependence. On the other hand, systolic interdependence is enhanced by a stiff right ventricular free wall, and decreased by a stiff septum. Recent imaging studies have shown that both diastolic and systolic ventricular interactions are negatively affected by right ventricular regional inhomogeneity and prolongation of contraction, which occur along with an increase in pulmonary artery pressure. The clinical relevance of these observations is being explored. Published on behalf of the European Society of Cardiology. All rights

  15. Genetic modifiers of Duchenne and facioscapulohumeral muscular dystrophies.

    Science.gov (United States)

    Hightower, Rylie M; Alexander, Matthew S

    2018-01-01

    Muscular dystrophy is defined as the progressive wasting of skeletal muscles that is caused by inherited or spontaneous genetic mutations. Next-generation sequencing has greatly improved the accuracy and speed of diagnosis for different types of muscular dystrophy. Advancements in depth of coverage, convenience, and overall reduced cost have led to the identification of genetic modifiers that are responsible for phenotypic variability in affected patients. These genetic modifiers have been postulated to explain key differences in disease phenotypes, including age of loss of ambulation, steroid responsiveness, and the presence or absence of cardiac defects in patients with the same form of muscular dystrophy. This review highlights recent findings on genetic modifiers of Duchenne and facioscapulohumeral muscular dystrophies based on animal and clinical studies. These genetic modifiers hold great promise to be developed into novel therapeutic targets for the treatment of muscular dystrophies. Muscle Nerve 57: 6-15, 2018. © 2017 Wiley Periodicals, Inc.

  16. Decreased coronary reserve in patients with hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Yamaguchi, Ryutaro

    1987-01-01

    To assess coronary flow reserve in patients with hypertrophic cardiomyopathy (HCM), split dose thallium-201 dipyridamole (DP) myocardial scintigraphy was performed. Subjects included 30 HCM patients with asymmetric septal hypertrophy and normal coronary angiogram, 10 hypertensive patients with left ventricular hypertrophy (posterior wall thickness ≥ 15 mm) and 13 healthy controls. Coronary reserve index (CRI) was calculated as a ratio of myocardial thallium uptake after dipyridamole (0.5 mg/kg) to the baseline value. HCM patients showed a significantly lower CRI (177 %) as compared with controls (281 %) or hypertensive patients (214 %), and 17 of them had abnormally decreased CRI below the normal range (mean-2SD of controls). These HCM patients with abnormal CRI showed significantly more frequent family histrory of HCM (71 vs 31 %), and a greater degree of systolic narrowing of the septal perforator as compared with those normal CRI. Maximal work loads were significantly lower (82 vs 106 watts) in those with abnormal CRI, 31 % developed ST depression at 80 watts. However, patients with abnormal CRI did not differ from those with normal CRI in septal and posterior wall thickness, left ventricular end-diastolic pressure, and in the degree of systolic narrowing of the left anterior descending artery. In the segmental CRI analysis, 13 HCM patients showed abnormal CRI in the septal and/or apical segments, while 8 patients presented diffuse CRI decrease, including the non-hypertrophied posterior segment. These findings indicate that 57 % of HCM patients have impaired coronary vasodilatory reserve, which may not only be a consequence of left ventricular hypertrophy, elevated left ventricular end-diastolic pressure, and systolic narrowing of the coronary artery, but may be related to small vessel coronary disease. (J.P.N.)

  17. Physical Fitness and Metabolic Syndrome in Children with Repaired Congenital Heart Disease Compared with Healthy Children.

    Science.gov (United States)

    Zaqout, Mahmoud; Vandekerckhove, Kristof; Michels, Nathalie; Bove, Thierry; François, Katrien; De Wolf, Daniel

    2017-12-01

    To determine whether children who underwent surgery for congenital heart disease (CHD) are as fit as their peers. We studied 66 children (6-14 years) who underwent surgery for ventricular septal defect (n = 19), coarctation of aorta (n = 10), tetralogy of Fallot (n = 15), and transposition of great arteries (n = 22); and 520 healthy children (6-12 years). All children performed physical fitness tests: cardiorespiratory fitness, muscular strength, balance, flexibility, and speed. Metabolic score was assessed through z-score standardization using 4 components: waist circumference, blood pressure, blood lipids, and insulin resistance. Assessment also included self-reported and accelerometer-measured physical activity. Linear regression analyses with group (CHD vs control) as a predictor were adjusted for age, body mass index, physical activity, and parental education. Measured physical activity level, body mass index, cardiorespiratory fitness, flexibility, and total metabolic score did not differ between children with CHD and controls, whereas reported physical activity was greater in the CHD group than control group. Boys with CHD were less strong in upper muscular strength, speed, and balance, whereas girls with CHD were better in lower muscular strength and worse in balance. High-density lipoprotein was greater in boys and girls with CHD, whereas boys with CHD showed unhealthier glucose homeostasis. Appropriate physical fitness was achieved in children after surgery for CHD, especially in girls. Consequently, children with CHD were not at increased total metabolic risk. Lifestyle counseling should be part of every patient interaction. Copyright © 2017 Elsevier Inc. All rights reserved.

  18. Efficacy and Safety of Transthoracic Echocardiography Alone in Transcatheter Closure of Secundum-Type Atrial Septal Defects in Adults.

    Science.gov (United States)

    Ding, Cheng; Chang, Jia-Kan; Lin, Chang-Chyi; Wu, Yong-Jian; Hsieh, Kai-Sheng

    2016-04-01

    On-site transthoracic echocardiography (TTE) to guide the transcutaneous closure of secundum-type atrial septal defects (ASDs) in the catheterization laboratory remains unclear, especially in adults. Between 2005 and 2012, a total of 82 adults underwent transcutaneous closure of ASDs. The initial 15 cases underwent the procedure with both on-site transesophageal echocardiography (TEE) and TTE monitoring. Since January 2008, a total of 67 patients underwent on-site TTE alone to guide the procedure. Among the 82 adult patients who underwent a transcutaneous closure of the secundum-type ASD procedure, all had successful closure of the defects, and no periprocedural adverse complications occurred. No statistical significance was observed in the successful complete shunt closure rate between the TEE plus TTE and TTE groups during sequential follow-up (postprocedure 24 hour [87% vs. 92%],1 month [93% vs. 95%], 3 month [93% vs. 97%], and 12 month [93% vs. 97%], P > 0.05, respectively) nor was a significant difference observed between the two groups, including decreased right ventricular dimension (29.5 ± 3.3 vs. 32.0 ± 4.9 mm, 26.5 ± 3.0 vs. 28.7 ± 4.6 mm, 26.2 ± 3.1 vs. 28.2 ± 4.8 mm, and 25.6 ± 2.8 vs. 27.7 ± 4.7 mm, P > 0.05, respectively) or increased left ventricular end-diastolic dimension (41.1 ± 2.0 vs. 42.6 ± 3.0 mm, 44.3 ± 2.7 vs. 45.5 ± 3.1 mm, 44.2 ± 2.8 vs. 45.4 ± 3.1 mm, 44.9 ± 2.7 vs. 45.8 ± 2.6 mm, P > 0.05, respectively) before the procedure, and at the 3-, 6-, and 12-month follow-up evaluations. This study showed that TTE guidance alone may be considered efficacious and safe as TEE during a transcutaneous ASD occlusion procedure in select adults. © 2015, Wiley Periodicals, Inc.

  19. BEEF CATTLE MUSCULARITY CANDIDATE GENES

    Directory of Open Access Journals (Sweden)

    Irida Novianti

    2010-04-01

    Full Text Available Muscularity is a potential indicator for the selection of more productive cattle. Mapping quantitative trait loci (QTL for traits related to muscularity is useful to identify the genomic regions where the genes affecting muscularity reside. QTL analysis from a Limousin-Jersey double backcross herd was conducted using QTL Express software with cohort and breed as the fixed effects. Nine QTL suggested to have an association with muscularity were identified on cattle chromosomes BTA 1, 2, 3, 4, 5, 8, 12, 14 and 17. The myostatin gene is located at the centromeric end of chromosome 2 and not surprisingly, the Limousin myostatin F94L variant accounted for the QTL on BTA2. However, when the myostatin F94L genotype was included as an additional fixed effect, the QTL on BTA17 was also no longer significant. This result suggests that there may be gene(s that have epistatic effects with myostatin located on cattle chromosome 17. Based on the position of the QTL in base pairs, all the genes that reside in the region were determined using the Ensembl data base (www.ensembl.org. There were two potential candidate genes residing within these QTL regions were selected. They were Smad nuclear interacting protein 1 (SNIP1 and similar to follistatin-like 5 (FSTL5. (JIIPB 2010 Vol 20 No 1: 1-10

  20. Radionuclide Angiocardiographic Evaluation of Left-to-Right Cardiac Shunts: Analysis of Time-Active Curves

    International Nuclear Information System (INIS)

    Kim, Ok Hwa; Bahk, Yong Whee; Kim, Chi Kyung

    1987-01-01

    The noninvasive nature of the radionuclide angiocardiography provided a useful approach for the evaluation of left-to-right cardiac shunts (LRCS). While the qualitative information can be obtained by inspection of serial radionuclide angiocardiograms, the quantitative information of radionuclide angiocardiography can be obtained by the analysis of time-activity curves using advanced computer system. The count ratios method and pulmonary-to-systemic flow ratio (QP/QS) by gamma variate fit method were used to evaluate the accuracy of detection and localization of LRCS. One hundred and ten time-activity curves were analyzed. There were 46 LRCS (atrial septal defects 11, ventricular septal defects 22, patent ductus arteriosus 13) and 64 normal subjects. By computer analysis of time-activity curves of the right atriurn, ventricle and the lungs separately, the count ratios modified by adding the mean cardiac transit time were calculated in each anatomic site. In normal subjects the mean count ratios in the right atrium, ventricle and lungs were 0.24 on average. In atrial septal defects, the count ratios were high in the right atrium, ventricle and lungs, whereas in ventricular septal defects the count ratios were higher only in the right ventricle and lungs. Patent ductus arteriosus showed normal count ratios in the heart but high count ratios were obtained in the lungs. Thus, this count ratios method could be separated normal from those with intracardiac or extracardiac shunts, and moreover, with this method the localization of the shunt level was possible in LRCS. Another method that could differentiate the intracardiac shunts from extracardiac shunts was measuring QP/QS in the left and right lungs. In patent ductus arteriosus, the left lung QP/QS was higher than those of the right lung, whereas in atrial septal defects and ventricular septal defects QP/ QS ratios were equal in both lungs. From this study, it was found that by measuring QP/QS separately in the lungs