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Sample records for muscular ventricular septal

  1. Muscular ventricular septal defects: A reappraisal of the anatomy

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    Wenink, A.C.G.; Oppenheimer-Dekker, A.; Moulaert, A.J.

    1979-01-01

    Among 79 autopsy specimens of hearts with an isolated ventricular septal defect, there were 29 cases of muscular defect. Among 60 hearts with complete transposition of the great arteries and a ventricular septal defect, there were 13 cases with a muscular defect. All muscular defects could be classi

  2. A case of hybrid closure of a muscular ventricular septal defect: anatomical complexity and surgical management.

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    Karimi, Mohsen; Hulsebus, Elise; Murdison, Kenneth; Wiles, Henry

    2012-06-01

    Complex muscular ventricular septal defect poses difficult surgical management and is associated with high morbidity and mortality despite advancements in surgical therapy. Device closure of muscular ventricular septal defect has been encouraging and has been used in hybrid approach at a few centres. However, device closure has some limitations in patients with complex muscular ventricular septal defect. We report a case of perventricular device closure of a complex muscular ventricular septal defect in a beating heart with entrapped right ventricular disc and its surgical management.

  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. The hybrid perventricular closure of apical muscular ventricular septal defect with Amplatzer duct occluder

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    Soo Jin Kim

    2013-04-01

    Full Text Available <b>Purpose:</b> Apical muscular ventricular septal defects (MVSDs, especially in small infants, can be difficult to manage using surgical and percutaneous closure. An intraoperative perventricular procedure is a good option for closing apical MVSDs in small children with or without associated cardiac anomalies.We evaluated the results of hybrid perventricular closure of apical MVSDs performed using an Amplatzer duct occluder (ADO. <b>Methods:</b> We retrospectively reviewed the medical records of 5 patients who underwent hybrid perventricular closure of MVSDs with ADOs, from March 2006 to May 2011. The median patient age at the time of the procedure was 12 months (range, 25 days to 25 months, and the median body weight was 9.1 kg (range, 4.3 to 15 kg. Two patients had multiple ventricular septal defects (VSDs; additional perimembranous VSD in 1 patient and multiple MVSDs in the other and 3 patients had associated cardiac anomalies; complete transposition of the great arteries in 1 patient and an atrial septal defect in 2 patients. All the procedures were performed on beating hearts, exception in 1 case. The ADO selected for the aortic side was at least 1 to 2 mm larger than the largest VSD in the left ventricle side. <b>Results:</b> The procedure was successful in all patients and each device was well positioned. During the median follow-up of 2.4 years, a small residual VSD was noted in 2 patients who had multiple VSDs and no leakage was seen in the other 3 patients. <b>Conclusion:</b> Perventricular closure of MVSD with an ADO is a good option for patients with apical MVSD. However, careful manipulation is important, especially in the case of small infants.

  5. Ventricular septal defect (image)

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    Ventricular septal defect is a congenital defect of the heart, that occurs as an abnormal opening in ... wall that separates the right and left ventricles. Ventricular septal defect may also be associated with other ...

  6. Perventricular device closure of residual muscular ventricular septal defects after repair of complex congenital heart defects in pediatric patients.

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    Zhu, Da; Tao, Kaiyu; An, Qi; Luo, Shuhua; Gan, Changping; Lin, Ke

    2013-01-01

    Residual muscular ventricular septal defects are surgical challenges, especially after the repair of complex congenital heart defects. We investigated perventricular device closure as a salvage technique in pediatric patients who had postoperative residual muscular ventricular septal defects. From February 2009 through June 2011, 14 pediatric patients at our hospital had residual muscular ventricular septal defects after undergoing surgical repair of complex congenital heart defects. Ten patients met our criteria for perventricular device closure of the residual defects: significant left-to-right shunting (Qp/Qs >1.5) or substantial hemodynamic instability (a defect ≥2 mm in size). The patients' mean age was 20.4 ± 13.5 months, and their mean body weight was 10 ± 3.1 kg. The median diameter of the residual defects was 4.2 mm (range, 2.5-5.1 mm). We deployed a total of 11 SQFDQ-II Muscular VSD Occluders (Shanghai Shape Memory Alloy Co., Ltd.; Shanghai, China) in the 10 patients, in accord with conventional techniques of perventricular device closure. The mean procedural duration was 31.1 ±9.1 min. We recorded the closure and complication rates perioperatively and during a 12-month follow-up period. Complete closure was achieved in 8 patients; 2 patients had persistent trivial residual shunts. No deaths, conduction block, device embolism, or other complications occurred throughout the study period. We conclude that perventricular device closure is a safe, effective salvage treatment for postoperative residual muscular ventricular septal defects in pediatric patients. Long-term studies with larger cohorts might further confirm this method's feasibility.

  7. A Complication following the Transcatheter Closure of a Muscular Ventricular Septal Defect

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    Mustafa Karaçelik

    2015-12-01

    Full Text Available Today, congenital heart diseases may be treated without surgery through advances in interventional cardiology. However, complications such as infection and thrombus formation may develop due to foreign materials used during these procedures. Surgical intervention may be required for the removal of the device utilized for the procedure. In this case report, we present the surgical treatment of a residual ventricular septal defect (VSD that had developed in a 6-year-old patient with an apical muscular VSD closed with the Amplatzer muscular VSD device. The patient was admitted to the emergency room with complaints of abdominal pain and high fever 5 days after discharge without any cardiac symptoms. When she arrived at our clinic, she had a heart rate of 95 bpm, blood pressure of 110/70 mmHg, and temperature of 38.5ºC. Examinations of the other systems were normal, except for a 3/6 pan-systolic murmur at the mesocardiac focus on cardiac auscultation. Echocardiography showed a residual VSD, and the total pulmonary blood flow to the total systemic blood flow ratio (Qp/Qs of the residual VSD was 1.8. In the operating room, the Amplatzer device was removed easily with a blunt dissection. The VSD was closed with an autologous fresh pericardial patch. Following the pulmonary artery debanding procedure, the postoperative period was uneventful. The condition of the patient at the time of discharge and in the first postoperative month’s follow-up was good. There was no residual VSD or infection. 

  8. Concurrent transcatheter closure of an apical muscular ventricular septal defect and a patent ductus arteriosus in a child with severe hyperkinetic pulmonary hypertension.

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    Joseph, G; Muthunayagam, J V; Mandalay, A

    2003-01-01

    Definitive treatment of congenital apical muscular ventricular septal defect (VSD) with large left-to-right shunt, severe pulmonary hypertension, and major associated lesions such as patent ductus arteriosus (PDA) has so far been possible only by surgery that has significant attendant morbidity and mortality [2]. Transcatheter device closure of both shunt lesions, if feasible, is a potentially simpler and safer alternative to surgery.

  9. Percutaneous closure of huge patent ductus arterious associated with anomalous inferior vein cava drainage and dextrocardia with muscular ventricular septal defect occluder

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    @@ Transcatheter occlusion of patent ductus arterious (PDA) using various occluders and coils has been a well-established method1-5 since Porstmann and colleagues6 reported the first case in 1967. However, when patients associated with anomalous inferior vein cava drainage or/and huge high pulmonary artery pressure ductus (HPAP-PDA), the method is not suitable. First, it is unfeasible to carry out the procedure via femoral vein. Second, in the presence of high pulmonary artery pressure such devices including the Amplatzer ductu occluder carry the risk of embolising into the aorta.7 The muscular ventricular septal defect occluder (MVSDO), which is a device for transcatheter closure of muscular ventricular septal defect, may be more suitable for using with HPAP-PDA as its double disk tends to anchor the device, preventing embolisation into the aorta. We present a patient, who is suffering from huge PDA associated with anomalous inferior vein cava drainage and dextrocardia, in whom percutaneous closure of PDA using MVSDO was successfully accomplished via transjugular approach.

  10. Atrial – Ventricular Septal Defect

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    T Panagiotopoulos

    2009-05-01

    Full Text Available Atrial and ventricular septal defect constitute the most common congenital heart disease.Aim: Τhe aim of the present retrospective study was to record data and factors that affect atrial and ventricular septal defect.Method and material: The sample study included patients of both sexes who were hospitalized with diagnosis atrial and ventricular septal defect in a Cardiac Surgery hospital of Athens. A specially constructed printed form was used for data collection, where were recorded the demographic and personal variables, the pathological, surgical, cardiology and obstetric history, the habits of adults, as well as the personal characteristics of mothers. Analysis of data was performed by descriptive statistical analysis.Results: The sample study consisted of 101 individuals with diagnosis atrial or ventricular Septal Defect, of which 40% were boys and 60% girls. The 70% of the sample study suffered from atrial Septal Defect and the 30% suffered from ventricular Septal Defect. Regarding age, 12% of the sample study was 0-1 years old, 35% was >1 years old, 8% was >12-18 years old and 45% over than 18 years old. Regarding educational status of the adult participants, 9% was of 0-6 years education, 22%>6 -12 years, 13%>12 years. 14% of the adult paticipants smoked, 4% consumed alcohol and 5% smoked in conjunction with alcohol. In terms of the obstetric history of the sample studied, 32% of the cases had normal birth, 4% had a twin birth and 1% had a triplet one. According to the variables related to mothers, the mean age of the mother was 30 years and 3 months, 10% were smokers at pregnancy and 3% used chemical substance and mainly hair color. Also, the results of the present study showed that individuals of 12-18 and >18 years old did not suffer from ventricular Septal Defect, whereas the infants 0-1 years old did not suffer from Atrial Septal Defect. The mean value of age at the admission in intensive care unit was 7 months (12% for the infants

  11. Facts about Ventricular Septal Defect

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    ... Living With Heart Defects Data & Statistics Tracking & Research Articles & Key Findings Free Materials Multimedia and Tools Links to Other Websites Information For... Media Policy Makers Facts about Ventricular Septal Defect Language: English Español (Spanish) Recommend on Facebook Tweet Share Compartir ...

  12. [Surgical Treatment of Large Muscular Ventricular Septal Defect nearby the Moderator Band Using the Sandwich Method;Report of a Case].

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    Motokawa, Mamika; Sasahara, Akihiro; Terakawa, Katsunari; Miyamoto, Takashi

    2016-09-01

    We describe the rare case of a 1-year-old girl who had large muscular ventricular defect (VSD) nearby the moderator band. We experienced the patch closure using sandwich method. A 1-month-old girl was referred to our institution for treatment of muscular VSD. At the age of 2 month, she underwent the pulmonary artery banding to control the pulmonary high flow. After follow up, the patient have reached 70 cm tall and weighed 7 kg. One year after the "sandwich operation", cardiac catheterization revealed the tiny residual shunt. Nevertheless, the cardiac function was good and the growth was in fine fettle. Sandwich method is a useful surgical technique to close the muscular VSD without resect the right ventricular trabeculation.

  13. Ventricular Septal Defect (For Parents)

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    ... Child What Kids Say About: Handling Stress Anxiety, Fears, and Phobias Community Service: A Family's Guide to ... septal defect (VSD) — sometimes referred to as a hole in the heart — is a type of congenital ...

  14. MULTIPLE VENTRICULAR SEPTAL DEFECTS: A NEW STRATEGY

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    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

  15. Ventricular Septal Defect from Takotsubo Syndrome

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    Caplow, Julie; Quatromoni, Neha

    2016-01-01

    Takotsubo Syndrome is a transient condition characterized by left ventricular systolic dysfunction with apical akinesis/dyskinesis and ballooning. Although the prognosis with medical management is excellent in most cases, rare cases of serious complications can occur. We present here a case of a 71-year-old woman presenting with acute decompensated heart failure with initial findings consistent with a myocardial infarction, who was found instead to have an acute ventricular septal defect as a complication of Takotsubo Syndrome. PMID:27563471

  16. Ventricular Septal Defect from Takotsubo Syndrome

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    Daniel Y. Lu

    2016-01-01

    Full Text Available Takotsubo Syndrome is a transient condition characterized by left ventricular systolic dysfunction with apical akinesis/dyskinesis and ballooning. Although the prognosis with medical management is excellent in most cases, rare cases of serious complications can occur. We present here a case of a 71-year-old woman presenting with acute decompensated heart failure with initial findings consistent with a myocardial infarction, who was found instead to have an acute ventricular septal defect as a complication of Takotsubo Syndrome.

  17. 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.

  18. 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.

  19. Echocardiographic evaluation of ventricular septal defect haemodynamics

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    Miranović Vesna

    2007-01-01

    Full Text Available Introduction Ventricular septal defect (VSD is an opening in the interventricular septum. 30-50% of patients with congenital heart disease have VSD. Objective The aim of the study was to determine the dependence of the left ventricular diastolic dimension (LVD, left ventricular systolic dimension (LVS, shortening fraction (SF, left atrium (LA, pulmonary artery truncus (TPA on the body surface and compare their values among experimental, control and a group of healthy children. Values of maximal systolic gradient pressure (Pvsd of VSD were compared with children from one experimental and control group. Method Children were divided into three groups: experimental (32 children with VSD that were to go to surgery, control (20 children with VSD who did not require surgery and 40 healthy children. Measurements of LVD, LVS, SF, LA, TPA were performed in accordance to recommendations of the American Echocardiographic Association. The value of Pvsd was calculated from the maximal flow velocity (V in VSD using the following formula: Pvsd=4xVІ (mm Hg. Results For children from the experimental group, the relationship between the body surface and the variability of the LVD was explained with 56.85%, LVS with 66.15%, SF with 4.9%, TPA with 58.92%. For children from the control group, the relationship between the body surface and the variability of LVD was explained with 88.8%, LVS with 72.5%, SF with 0.42%, PA with 58.92%. For healthy children, the relationship between the body surface and the variabilitiy of the LVD was explained with 88.8%, LVS with 88.78%, SF with 5.25% and PA with 84.75%. There was a significant statistical difference between average values of Pvsd in the experimental and control group (p<0.02. Conclusion The presence of the large VSD has an influence on the enlargement of LVD, LVS, SF, TPA. The enlargement of the size of the pulmonary artery depends on the presence of VSD and there is a direct variation in the magnitude of the shunt

  20. 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.

  1. Percutaneous closure of postinfarction ventricular septal defect: cardiac magnetic resonance-guided case selection and postprocedure evaluation.

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    Artis, Nigel J; Thomson, John; Plein, Sven; Greenwood, John P

    2011-01-01

    Despite modern surgical techniques, complications and early mortality remain high following postinfarction ventricular septal defect (VSD) repair. It is now possible to close these acquired defects percutaneously using, for example, the Amplatzer postinfarct muscular VSD device. Cardiovascular magnetic resonance is an important tool in determining appropriate case selection and device sizing as it can provide a multicomponent assessment of the VSD anatomy, ventricular volumes and function, infarct extent, and left-to-right shunt calculations.

  2. Ventricular septal rupture and right ventricular intramyocardial dissection secondary to acute inferior myocardial infarction.

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    Bittencourt, Márcio Sommer; Seltmann, Martin; Muschiol, Gerd; Achenbach, Stephan

    2010-01-01

    A 61-year-old female patient presented with sub acute myocardial infarction with an occluded right coronary artery on invasive evaluation and a ventricular septal rupture on echocardiogram. Cardiac computed tomography (CT) was performed to better define the septal anatomy. As the anatomy on cardiac CT was considered unfavorable for percutaneous intervention, the patient underwent successful surgical repair.

  3. Rupture of Right Ventricular Free Wall Following Ventricular Septal Rupture in Takotsubo Cardiomyopathy with Right Ventricular Involvement.

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    Sung, June Min; Hong, Sung Jin; Chung, In Hyun; Lee, Hye Young; Lee, Jae Hoon; Kim, Hyun Jung; Byun, Young Sup; Kim, Byung Ok; Rhee, Kun Joo

    2017-01-01

    Most patients diagnosed with takotsubo cardiomyopathies are expected to almost completely recover, and their prognosis is excellent. However, complications can occur in the acute phase. We present a case of a woman with takotsubo cardiomyopathy with right ventricular involvement who developed a rupture of the right ventricular free wall following ventricular septal rupture, as a consequence of an acute increase in right ventricular afterload by left-to-right shunt. Our case report illustrates that takotsubo cardiomyopathy can be life threatening in the acute phase. Ventricular septal rupture in biventricular takotsubo cardiomyopathy may be a harbinger of cardiac tamponade by right ventricular rupture.

  4. The road to right ventricular septal pacing: techniques and tools.

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    Mond, Harry G

    2010-07-01

    Prolonged right ventricular (RV) apical pacing is associated with progressive left ventricular dysfunction due to dysynchronous ventricular activation and contraction. RV septal pacing allows a narrower QRS compared to RV apical pacing, which might reflect a more physiological and synchronous ventricular activation. Previous clinical studies, which did not consistently achieve RV septal pacing, were not confirmatory and need to be repeated. This review summarizes the anatomy of the RV septum, the radiographic appearances of pacing leads in the RV, the electrocardiograph correlates of RV septal lead positioning, and the techniques and tools required for implantation of an active-fixation lead onto the RV septum. Using the described techniques and tools, conventional active-fixation leads can now be reliably secured to either the RV outflow tract septum or mid-RV septum with very low complication rates and good long-term performance. Even though physiologic and hemodynamic studies on true RV septal pacing have not been completed, the detrimental effects of long-term RV apical pacing are significant enough to suggest that it is now time to leave the RV apex and secure all RV leads onto the septum.

  5. Aspects of surgery for congenital ventricular septal defect

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    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 ech

  6. Prenatal diagnosis of fetal aortopulmonary septal defect with ventricular septal defect by two-dimension echocardiography

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    @@ Fetal aortopulmonary septal defect (APSD) is an extremely rare condition, accounting for 0.1%-0.2% of all cardiac defects in live births world wide.1 Hospital mortality is 13% and 33% for simple and complex APSD, respectively.2 This rare cardiac defect refers to a congenital malformation in the development of the arteriosus truncus septum, and is usually associated with a wide variety of other structural cardiac anomalies such as ventricular septal defect (VSD), pulmonary valve stegnosis and so on.3 Prenatal diagnosis of an APSD is possible by echocardiography.

  7. MR demonstration of septal involvement in arrhythmogenic right ventricular dysplasia

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    Malhaire, Caroline; Rahmouni, Alain [Centre Hospitalo-Universitaire Henri Mondor, Service de Radiologie et d' Imagerie Medicale, Creteil Cedex (France); Garot, Jerome [Centre Hospitalo-Universitaire Henri Mondor, Service de Cardiologie, Creteil Cedex (France)

    2005-05-01

    Arrhythmogenic right ventricular dysplasia (ARVD) is a heart muscle disease of unknown origin. Although MR imaging is regarded as the best technique for the demonstration of functional and structural abnormalities in ARVD, fat deposits in the interventricular septum have never been documented on MR imaging. We report the case of interventricular septal fatty deposition demonstrated by fat-suppressed MR imaging in a 48-year-old man. (orig.)

  8. Iron deficiency and hemolytic anemia reversed by ventricular septal myectomy

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    Costa, Steven M.; Cable, Christian

    2015-01-01

    Hemolytic anemia has been reported to occur in the setting of aortic stenosis and prosthetic heart valves, but much more rarely in association with obstructive hypertrophic cardiomyopathy (HC). Of the few descriptions of hemolytic anemia secondary to HC, all but one case involved bacterial endocarditis contributing to left ventricular outflow tract obstruction. We present the case of a 67-year-old man with recurrent hemolytic anemia and HC, without infective endocarditis. Attempts at iron repletion and augmentation of beta-blocker therapy proved his anemia to be refractory to medical management. Ventricular septal myectomy led to the resolution of hemolysis, anemia, and its coexisting symptoms. PMID:26424952

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

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    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.

  10. Transcatheter closure of perimembranous ventricular septal defects: single centre experience in China

    Institute of Scientific and Technical Information of China (English)

    LI Tian-chang; HU Da-yi; BIAN Hong; WANG Guo-hong; WANG Xian; ZHU Zheng-yan; XU Yu-yun

    2005-01-01

    @@ Ventricular septal defect (VSD) is one of the most common congenital heart malformation. As an isolated lesion, it accounts for 20%-30% of patients with congenital heart disease.1 Eighty percent of these defects are perimembranous involving the membranous septum and the adjacent area of muscular septum. At least 80% of these defects are small and close spontaneously,2,3 the larger defects often persist to cause significant shunt and right ventricular hypertension. Although conventional surgical repair of perimembranous ventricular septal defects (PMVSDs) is a safe, widely accepted procedure with negligible mortality. It is associated with morbidity, discomfort and a thoracotomy scar.1 As an alternative to surgery, a variety of devices for transcatheter closure of VSD have been developed. However, these devices were not specifically designed for this purpose and none has gained wide acceptance. Large delivery sheaths, inability to recapture and reposition, structural failure, dislodgement and embolization of the device, interference with the aortic valve resulting in aortic insufficiency and a high rate of residual shunting are the major limitations of the previously described techniques.2,3 The initial experiences with transcatheter closure of PMVSDs in patients with a new device that was especially designed for non-surgical occlusion of these defects, the Amplatzer asymmetric VSD occluder (AAVSDO, AGA Medical Co., USA) were encouraging.4-6 But the long-term results are not known.

  11. Acute left ventricular dysfunction secondary to right ventricular septal pacing in a woman with initial preserved contractility: a case report

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    Gribaa Rim

    2011-10-01

    Full Text Available Abstract Introduction Right ventricular apical pacing-related heart failure is reported in some patients after long-term pacing. The exact mechanism is not yet clear but may be related to left ventricular dyssynchrony induced by right ventricular apical pacing. Right ventricular septal pacing is thought to deteriorate left ventricular function less frequently because of a more normal left ventricular activation pattern. Case presentation We report the case of a 55-year-old Tunisian woman with preserved ventricular function, implanted with a dual-chamber pacemaker for complete atrioventricular block. Right ventricular septal pacing induced a major ventricular dyssynchrony, severe left ventricular ejection fraction deterioration and symptoms of congestive heart failure. Upgrading to a biventricular device was associated with a decrease in the symptoms and the ventricular dyssynchrony, and an increase of left ventricular ejection fraction. Conclusion Right ventricular septal pacing can induce reversible left ventricular dysfunction and heart failure secondary to left ventricular dyssynchrony. This complication remains an unpredictable complication of right ventricular septal pacing.

  12. Risk factors for ventricular septal defect in Finland.

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    Tikkanen, J; Heinonen, O P

    1991-03-01

    The possible effect of genetic and environmental factors during pregnancy on the occurrence of ventricular septal defect (VSD) in the offspring was studied in 150 cases and 756 controls. The cases represented all verified VSDs in Finland during 1982-1983. The controls were randomly selected from all babies born during the same period. Case and control mothers were interviewed by midwives approximately three months after delivery using a structured questionnaire. Congenital heart disease was more prevalent among parents of cases than those of controls. Maternal alcohol consumption during the first trimester of pregnancy was more common among the mothers of VSD infants (47.0%) than among those of controls (38.0%, P less than 0.05). Exposure to organic solvents at work showed in logistic regression analysis an adjusted relative odds ratio of 1.8 (95% confidence interval 1.0-3.4). The risk of VSD was not associated with any of the maternal habits monitored, e.g. smoking, or coffee, tea, cola, acetosalicylic acid or diazepam consumption. Whether the mother was employed during the first trimester of pregnancy, and her exposure to anesthetic gases, disinfectants, pecticides, wood preservatives or video display terminals were not factors associated with the risk of ventricular septal defect.

  13. Surgical closure of apical multiple muscular septal defects via right ventriculotomy using a single patch with intermediate fixings

    Institute of Scientific and Technical Information of China (English)

    LI Gang; WANG Qiang; LIU Ying-long; XU Yu-lin; GUO Yan

    2013-01-01

    Background Multiple apical muscular ventricular septal defects (VSDs) remain a challenge for surgeons because of their anatomical features.We used single patch with intermediate fixations to repair multiple apical muscular VSDs through right ventriculotomy.Methods We analysed the data of 16 children (median age 8 months,range 2 months to 144 months) with multiple apical muscular VSDs who underwent a single patch technique via apical right ventriculotomy.Perioperative data were collected and analysed,and the patients were followed up for three months to 66 months (median,46 months) to investigate the outcomes.Results All patients recovered from cardiopulmonary bypass easily with median of cardiopulmonary bypass time 87 minutes and of aortic crossclamp time 53 minutes.No surgically related death occurred and no patient required reoperation.One patient died of pseudomonas pyocyanea infection on day 11 postoperatively.Residual shunt happened in one patient with a diameter of 2 mm and spontaneously closed in 12 months after operation.Two patients presented slightly reduced right ventricular volume and apical hypokinesia postoperatively and recovered 24 and 36 months later respectively.No other complication was found during the three months to 66 months follow-up.Conclusion Our experiences using a single patch technique with intermediate fixations via right ventriculotomy in the management of multiple muscular VSDs had favourable outcomes.

  14. Leiomyosarcoma with coronary fistulae and ventricular septal perforation:A case study

    Institute of Scientific and Technical Information of China (English)

    Dang-Sheng HUANG; Yu-Mei WANG; Yu CHEN

    2014-01-01

    Coronary fistulae and ventricular septal perforation are very rare clinically, and even less caused by cardiac leiomyosarcoma. A case is reported that a 67-year-old female had cardiac leiomyosarcoma with progressive heart failure and coronary fistulae and ventricular septal perforation. This case was special since all ante-mortem examinations and cardiac surgery failed to detect the presence of any abnormal car-diac mass. Therefore, the malignant cardiac tumors could appear in an invasive form without mass and be one of the causes of the coronary fistulae and ventricular septal perforation.

  15. Familial ventricular aneurysms and septal defects map to chromosome 10p15.

    Science.gov (United States)

    Tremblay, Nicolas; Yang, Shi Wei; Hitz, Marc-Phillip; Asselin, Géraldine; Ginns, Jonathan; Riopel, Kathleen; Gendron, Roxanne; Montpetit, Alexandre; Duhig, Edwina; Dubé, Marie-Pierre; Radford, Dorothy; Andelfinger, Gregor

    2011-03-01

    Although ventricular septal defects (VSD) are the most common congenital heart lesion, familial clustering has been described only in rare instances. The aim of this study was to identify genetic factors and chromosomal regions contributing to VSD. A unique, large kindred segregating various forms of septal pathologies-including VSD, ventricular septal aneurysms, and atrial septal defects (ASD)-was ascertained and characterized clinically and genetically. Eighteen family members in three generations could be studied, out of whom 10 are affected (2 ASD, 3 septal aneurysm, 4 VSD, and 1 tetralogy of Fallot). Parametric multipoint LOD scores reach significance on chromosome 10p15.3-10p15.2 (max. 3.29). The LOD score support interval is in a gene-poor region where deletions have been reported to associate with septal defects, but that is distinct from the DiGeorge syndrome 2 region on 10p. Multiple linkage analysis scenarios suggest that tetralogy of Fallot is a phenocopy and genetically distinct from the autosomal dominant form of septal pathologies observed in this family. This study maps a rare familial form of VSD/septal aneurysms to chromosome 10p15 and extends the spectrum of the genetic heterogeneity of septal pathologies. Fine mapping, haplotype construction, and resequencing will provide a unique opportunity to study the pathogenesis of septal defects and shed light on molecular mechanisms of septal development.

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

    Directory of Open Access Journals (Sweden)

    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.

  17. Myocardial infarction with ventricular septal rupture complicating elective aortic valve replacement.

    Science.gov (United States)

    Milhous, J Gerrit-Jan; Heijmen, Robin H; Ball, Egbert T; Plokker, H W Thijs

    2006-07-01

    Herein is described the case of a 79-year-old woman who underwent elective aortic valve replacement. The procedure was complicated by a particulate embolism into the left anterior descending artery leading to a myocardial infarction, complicated by ventricular septal rupture. Subsequently, the patient was reoperated on and the septal defect closed successfully, with an uneventful recovery.

  18. Short-term prognosis and risk factors of ventricular septal rupture following acute myocardial infarction

    Institute of Scientific and Technical Information of China (English)

    胡小莹

    2013-01-01

    Objective To analyze the short-term prognosis and risk factors of ventricular septal rupture(VSR)following acute myocardial infarction(AMI).Methods A total of 70 consecutive VSR patients following AMI hospitalized in

  19. Short-term prognosis and risk factors of ventricular septal rupture following acute myocardial infarction

    Institute of Scientific and Technical Information of China (English)

    胡小莹

    2013-01-01

    Objective To analyze the short-term prognosis and risk factors of ventricular septal rupture(VSR)following acute myocardial infarction(AMI).Methods A total of 70 consecutive VSR patients following AMI

  20. Ventricular Septal Defect Spontaneous Close Induced by Transcatheter: A Case Report

    Institute of Scientific and Technical Information of China (English)

    Qilian Xie; Jun Wang; Lei Gao; Zhen Wang; Milin Zhang; Kunshen Liu

    2007-01-01

    Congenital ventricular septal defect (VSD) spontaneous close induced by transcatheter treatment is rare and has not yet been reported.We report on one case of VSD spontaneous close induced by transcatheter treatment in a 10 years old girl.

  1. Pregnancy after biventricular repair for pulmonary atresia with ventricular septal defect.

    NARCIS (Netherlands)

    Drenthen, W.; Pieper, P.G.; Zoon, N.; Roos-Hesselink, J.W.; Voors, A.A.; Mulder, B.J.M.; Dijk, A.P.J. van; Sollie, K.M.; Vliegen, H.W.; Ebels, T.; Veldhuisen, D.J. van

    2006-01-01

    Information on pregnancy and delivery in women with biventricular repair for isolated noncomplex pulmonary atresia with a ventricular septal defect (PAVSD) is limited. Using a nationwide congenital heart disease registry (CONgenital CORvitia [CONCOR]), 9 women with biventricular repair for PAVSD

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

    Directory of Open Access Journals (Sweden)

    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.

  3. Repetitive Delay in Diagnosis of Ventricular Septal Defect

    Directory of Open Access Journals (Sweden)

    Zahra Nikyar

    2011-12-01

    Full Text Available Objective: Although ventricular septal defect (VSD is the most common congenital heart disease, it is usually diagnosed late. The presentation of the disease is variable; sometimes it is so quiet and silent that might even improve and heal spontaneously, and in some certain cases if the appropriate, on time and early treatment is not done, this would lead to irreparable complications and mortality even in the early life period. This study reviews the diagnostic process, treatment and follow-up of the patients. It is hoped that the results of the present study be used to improve the patients' condition.Methods: This was a cross-sectional study done on 145 patients with VSD during 54 months in Isfahan. The disease was identified through color Doppler echocardiogram, cardiac catheterization and angiography if necessary. The required data were collected at the time of definite diagnosis.Findings: Mean age at initial and definite diagnosis of the disease was 17 months and 44 months, respectively. Heart murmur led to initial diagnosis in 85% of the cases. In 27.5% VSD was associated with other cardiac anomalies. Pulmonary artery hypertension existed in 16.5% of the cases. Fifty nine surgeries were performed on 40 patients.Conclusion: In routine physical examination of the infants, the probability of heart disease should be considered; conducting echocardiogram in suspected cases would lead to early diagnosis and eventually timely treatment. Appropriate follow-up of the patients will provide optimal care and treatment at proper time.

  4. Device Closure of Small Ventricular Septal Defects: When and Why?

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    IB Vijayalakshmi

    2014-10-01

    Full Text Available Ventricular septal defect (VSD accounts for approximately 20-30 % of all forms of congenital heart disease (CHD. They were traditionally closed surgically in the past. The surgery though safe carries the risk of morbidity, complete heart block, wound infection and thoracotomy scar on the chest. On weighing the risk and benefit, the small VSDs were not submitted to surgery in the past. So for many years it has been taught that the small VSDs are to be left alone and surgery is not recommended. Many clinicians believed in spontaneous closure of VSDs. Hence they advised the parents to wait for spontaneous closure till the child is 9 years old. But what if the VSD does not close by 10 years is the question. Are they normal as grown up congenital heart (GUCH. The Jane Somerville GUCH unit showed that spontaneous closure occurred only in 10% between the age of 17 and 45 (mean - 27 years in small VSDs in adults. What is worth noting is about 25% had serious complications: infective endocarditis (11%, progressive aortic regurgitation (5%, age-related symptomatic arrhythmias (8.5% like atrial fibrillation. This means that asymptomatic small VSDs in childhood is not necessarily benign during adult life. This raises the question, when we have a safe non-surgical device closure available to close the small VSDs, should we put the future lives of the young in danger by not giving the benefit of technology to them?

  5. 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.

  6. Incidental Discovery of a Membranous Ventricular Septal Aneurysm in Two Dissimilar Patients

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    Abhishek Naidu

    2012-01-01

    Full Text Available A ventricular septal aneurysm (VSA is a rare cardiac anomaly, and an accurate statistic of its prevalence has not been reported in the literature. True incidence is likely underestimated as most patients are thought to be asymptomatic. As a result, most VSAs are discovered incidentally on echocardiography, during angiography, or at autopsy. Potential complications include rupture, bacterial endocarditis, right ventricular outflow tract obstruction, and thromboembolic disease. It has been proposed that VSAs occur in association with ventricular septal defects (VSDs and other congenital cardiac abnormalities. It is uncommon for a VSA to exist in the absence of a known prior ventricular septal defect. We present two cases, each highlighting an incidental intact aneurysm involving the membranous interventricular septum. We discuss the contrast in the two patients with regard to their age, accompanying cardiac anomalies and cardiovascular fitness. Clinical implications of the condition are reviewed.

  7. 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例,

  8. 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.

  9. Evaluation of Ventricular Septal Defect with Special Reference to the Spontaneous Closure Rate, Subaortic Ridge, and Aortic Valve Prolapse II.

    Science.gov (United States)

    Eroglu, Ayse Guler; Atik, Sezen Ugan; Sengenc, Esma; Cig, Gulnaz; Saltik, Irfan Levent; Oztunc, Funda

    2017-06-01

    The medical records of 2283 patients with ventricular septal defect (VSD) were reviewed to determine spontaneous closure, left ventricular-to-right atrial shunt, subaortic ridge, and aortic valve prolapse. One thousand eight hundred and twenty-three patients had been followed 1 month to 26 years (median 4 years) by echocardiography. Most of 460 patients could not be followed due to transportation of the institution. VSD was perimembranous in 68.8% (1255), trabecular muscular in 21.7% (395), muscular outlet in 6% (109), muscular inlet in 2.6% (48), and doubly committed subarterial in 0.9% (16). Defect size was classified in 66.8% (1218) as small, in 15.7% (286) as moderate, and in 17.5% (319) as large. VSD closed spontaneously in 18.8% (343 of 1823 patients) by ages 40 days to 24.9 years (median, 1.8 years). One hundred fifty-seven of 1255 perimembranous defects (12.5%) and 167 of 395 trabecular muscular defects (42%) closed spontaneously (p < 0.001). Defect size became small in 306 (16.8%) of patients with VSD at a median of 2.5 years. Aneurysmal transformation was detected in 32.9% (600), left ventricular-to-right atrial shunt in 9.7% (176), subaortic ridge in 2.6% (48) of 1823 patients who were followed. In 381 (20.9%) of the 1823 patients, the VSD had been closed by a surgical or transcatheter technique. Surgery is required in one-fifth of patients with subaortic ridge or aortic valve prolapse. In conclusion, isolated VSDs are usually benign abnormalities that tend to shrink and close spontaneously.

  10. Percutaneous septal ablation for left mid-ventricular obstructive hypertrophic cardiomyopathy: a case report

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    Alioglu Emin

    2006-04-01

    Full Text Available Abstract Background Mid-ventricular obstructive hypertrophic cardiomyopathy (MVOHC is a rare type of cardiomyopathy. The diagnosis is based on the hourglass appearance on the left ventriculogram and the presence of pressure gradient between apical and basal chamber of the ventriculum on the hemodynamic assessment. Case presentation The present case represents successful percutaneous treatment with septal ablation to patient with MVOHC associated with systolic anterior motion of the mitral valve and obstruction at both the mid-ventricular and outflow levels. Conclusion Alcohol septal ablation has been proposed as less invasive alternatives to surgery in patients with MVOHC.

  11. Right ventricular septal pacing: Safety and efficacy in a long term follow up

    Institute of Scientific and Technical Information of China (English)

    Eraldo; Occhetta; Gianluca; Quirino; Lara; Baduena; Rosaria; Nappo; Chiara; Cavallino; Emanuela; Facchini; Paolo; Pistelli; Andrea; Magnani; Miriam; Bortnik; Gabriella; Francalacci; Gabriele; Dell’Era; Laura; Plebani; Paolo; Marino

    2015-01-01

    AIM: To evaluate the safety and efficacy of the permanent high interventricular septal pacing in a long term follow up, as alternative to right ventricular apical pacing. METHODS: We retrospectively evaluated:(1) 244 patients(74 ± 8 years; 169 men, 75 women) implanted with a single(132 pts) or dual chamber(112 pts) pacemaker(PM) with ventricular screw-in lead placed at the right ventricular high septal parahisian site(SEPTAL pacing);(2) 22 patients with permanent pacemaker and low percentage of pacing(< 20%)(NO pacing);(3) 33 patients with high percentage(> 80%) right ventricular apical pacing(RVA). All patients had a narrow spontaneous QRS(101 ± 14 ms). We evaluated New York Heart Association(NYHA) class, quality of life(Qo L), 6 min walking test(6MWT) and left ventricular function(end-diastolic volume, LV-EDV; end-systolic volume, LVESV; ejection fraction, LV-EF) with 2D-echocardiography. RESULTS: Pacing parameters were stable duringfollow up(21 mo/patient). In SEPTAL pacing group we observed an improvement in NYHA class, Qo L score and 6MWT. While LV-EDV didn’t significantly increase(104 ± 40 m L vs 100 ± 37 m L; P = 0.35), LV-ESV slightly increased(55 ± 31 m L vs 49 ± 27 m L; P = 0.05) and LV-EF slightly decreased(49% ± 11% vs 53% ± 11%; P = 0.001) but never falling < 45%. In the RVA pacing control group we observed a worsening of NYHA class and an important reduction of LV-EF(from 56% ± 6% to 43% ± 9%, P < 0.0001).CONCLUSION: Right ventricular permanent high septal pacing is safe and effective in a long term follow up evaluation; it could be a good alternative to the conventional RVA pacing in order to avoid its deleterious effects.

  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 clos

  13. Staged repair of pentalogy of Cantrell with ectopia cordis and ventricular septal defect.

    Science.gov (United States)

    Sakasai, Yoshie; Thang, Bui Quoc; Kanemoto, Shinya; Takahashi-Igari, Miho; Togashi, Shinji; Kato, Hideyuki; Hiramatsu, Yuji

    2012-05-01

    Pentalogy of Cantrell is a rare congenital anomaly characterized by a combination of severe defects in the middle of the chest and abdomen including intracardiac defects. Survival rate after cardiac surgery is extremely low. We present a successful staged complete repair of an omphalocele, a ventricular septal defect and a sternal defect in a case of pentalogy of Cantrell.

  14. [Permanent cardiac stimulation in a patient with isolated dextrocardia and ventricular septal defect].

    Science.gov (United States)

    Słowiński, S; Derlaga, B; Kapusta, J

    A case of pacemaker implantation because of acquired atrioventricular block third degree in woman aged 39 years with rare isolated dextrocardia with inversion of cardiac ventricles, compensatory transposition of both aorta and pulmonary artery, and ventricular septal defect. The end of electrode has wedged in the apex of the arterial ventricle. Following pacemaker implantation, patients clinical course of gynaecological operation was uneventful.

  15. Repair of a ventricular septal defect in a patient with left lung agenesis.

    Science.gov (United States)

    Guo, Hong-Wei; Pan, Shi-Wei; Song, Yun-Hu; Hu, Sheng-Shou

    2011-09-01

    Congenital heart disease combined with lung agenesis is extremely rare. We report a case of a 5-year-old female with a ventricular septal defect (VSD) and left lung agenesis with severe pulmonary hypertension who underwent successful closure of the VSD. 

  16. Robotic trans-atrial and trans-mitral ventricular septal resection

    Science.gov (United States)

    2017-01-01

    Localized ventricular septal hypertrophy, also known as idiopathic hypertrophic subaortic stenosis or idiopathic hypertrophic subaortic septal obstruction (IHSS), can create severe ventricular outflow obstruction. This often results in a high sub-aortic pressure gradient with potentially lethal symptoms. In 1960, Braunwald described the hemodynamic characteristics of IHSS, and thereafter, Morrow developed a trans-aortic approach to resect a large part of the ventricular septum, enlarging the outflow tract. The Morrow operation has remained the gold standard for surgically treating this pathology. However, in patients with a small aortic annulus, a severely hypertrophied septum, and a long anterior mitral leaflet, the trans-aortic approach may be more difficult, resulting in an inadequate resection and/or systolic anterior leaflet motion. The latter usually increases the obstruction and can cause mitral regurgitation. Herein, we describe a minimally invasive trans-left atrial robotic approach to treat IHSS. First, the anterior mitral leaflet is incised radially to reveal the aortic outflow tract and ventricular septum, which are located posteriorly. Thereafter, a deep “block” of septum is excised, beginning at the right valve cusp nadir and continued counterclockwise toward the left fibrous trigone. This excision is extended to the anterior papillary muscle base, where any septal connections must be divided. Subsequently, the anterior leaflet is re-suspended and the repair is completed with a flexible annuloplasty band. If necessary, the anterior leaflet can be augmented with a pericardial patch. The “wristed” robotic instruments and magnified 3-D vision definitely facilitate an adequate septal resection and anterior papillary muscle mobilization. Moreover, it is possible that this fine control helps to reduce complications, such as heart block or a ventricular septal defect. PMID:28203542

  17. Association of TBX5 gene polymorphism with ventricular septal defect in the Chinese Han population

    Institute of Scientific and Technical Information of China (English)

    LIU Cai-xia; SHEN A-dong; LI Xiao-feng; JIAO Wei-wei; BAI Song; YUAN Feng; GUAN Xiao-lei; ZHANG Xin-gen; ZHANG Gui-rong; LI Zhong-zhi

    2009-01-01

    Background Congenital heart disease is a diverse group of diseases determined by genetic and environmental factors. Considerable research has been done on genes associated with development of the heart. A recent focus is the role of transcription factor TBX5 in the development of atria, left ventricle and conduction system. As part of a larger study, high density, single nucleotide polymorphism (SNP) scanning was used to explore the relationship between TBX5 gene polymorphism and susceptibility to ventricular septal defect not associated with forelimb malformation in the Chinese Han population. Methods One hundred and ninety two paediatric patients with congenital ventricular septal defect and 192 matched healthy control subjects were studied. The haplotype reconstructions were calculated by PHASE2.0 software. Haploview software was used to 15erform linkage disequilibrium assessment and defining of haplotype blocks. The algorithm used for defining of blocks was the confidence interval method. Results The TBX5 gene region can be divided into 3 haplotype blocks of 27, 15 and 2 SNPs. Strong linkage disequilibrium exists within each block. SNP rs11067075 within the TBX5 gene had significant correlation with ventricular septal defect (P=0.0037) by single marker association analysis. In addition, a 20 kb haplotype composed of 27 SNPs correlated with ventricular septal defect (P=0.05, multiple loci regression analyses based on reconstructed haplotype blocks). Conclusions TBX5 is associated with the occurrence of ventricular septal defect and may be a predisposing gene to congenital heart disease in Hart Chinese. This finding has set a direction for further genetic and functional studies.

  18. Transcatheter closure of atrial septal defect associated with arrhythmogenic right ventricular cardiomyopathy: a case report and literature review.

    Science.gov (United States)

    Wang, Haiyan; Ding, Hongyu; Lei, Lei; Zhang, Xiaohong; Gong, Yuling; Hou, Yinglong

    2015-03-01

    Arrhythmogenic right ventricular cardiomyopathy is characterised by progressive, fibrofatty replacement of myocardium, and ventricular arrhythmias, and its prognosis is usually poor. Arrhythmogenic right ventricular cardiomyopathy associated with atrial septal defect is very rare, and this combination may make the diagnosis, treatment, and prognosis difficult. We present a case of a patient with this association who underwent interventional treatment with a septal defect occluder. Transcatheter closure of atrial septal defect in a patient with arrhythmogenic right ventricular cardiomyopathy is hitherto unreported. During a 3-year follow-up he remained relatively stable. We also review the cases reported in the medical literature describing this uncommon association between arrhythmogenic right ventricular cardiomyopathy and atrial septal defect or patent foramen ovale.

  19. Lessons learnt from a series of hemodynamic and interventional complications during pulmonary valvotomy and device closure of ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Anil Kumar Singhi

    2015-01-01

    Full Text Available In an adolescent girl with coexistent valvar pulmonary stenosis (PS and muscular ventricular septal defect (VSD causing right to left shunt and severe cyanosis, a series of complications were encountered during transcatheter intervention. After balloon pulmonary valvotomy (BPV and device closure of the VSD, dynamic infundibular hypercontractility elevated the right ventricular (RV systolic pressures leading to embolization of the device into the left ventricle. During retrieval of the device from the left ventricular outflow tract (LVOT, there was injury to the atrioventricular nodal tissue resulting in transient complete heart block. Even though the device was successfully retrieved out from the right femoral arterial access, there was a retroperitoneal hematoma causing severe anemia. A combination of dynamic infundibular obstruction, intravascular volume depletion caused by hemorrhage, low oxygen carrying capacity due to anemia, and inotropes resulted in a life-threatening hypercyanotic spell. Once this combination of suicidal hypercontractile right ventricle, anemia, and dehydration was recognized; management of the pathophysiology resulted in recovery of the patient. On a subsequent day, device closure of the VSD was complicated by unstable device position, but was finally achieved by an innovative device stabilization technique. We propose to explain the basis of hemodynamic and procedural complications, their recognition, and management. During preparatory stage before interventions in patients with multiple defects, which are linked by strong hemodynamic interdependence, meticulous planning should be done and multiple untoward events should be foreseen. While a few complications were completely unanticipated, some others could have been predicted.

  20. Ectopia cordis with a double outlet right ventricle, large ventricular septal defect, malposed great arteries and left ventricular hypoplasia.

    Science.gov (United States)

    Malik, Rabiya; Zilberman, Mark V; Tang, Liwen; Miller, Susan; Pandian, Natesa G

    2015-03-01

    Ectopia cordis, defined as partial or complete displacement of the heart outside of the thoracic cavity, is a rare congenital malformation. If not surgically corrected during the early years of life, ectopia cordis can prove to be a fatal abnormality. However, due to the presence of multiple intracardiac and extracardiac malformations, a corrective surgery might not always be successful. The pathology of ectopia cordis with a double outlet right ventricle, large ventricular septal defect, malposed great arteries and left ventricular hypoplasia is discussed, highlighting the complexities involved in such a rare disorder.

  1. Hemodynamic improvement by right ventricular septal pacing in elderly patients with chronic atrial fibrillation and slow ventricular response

    Institute of Scientific and Technical Information of China (English)

    Wei HUA; Shidong GUO; Shu ZHANG; Fangzheng WANG; Lida ZHI; Hongxia NIU; Xin CHEN

    2005-01-01

    Background and objectives Right ventricular apical (RVA) pacing has been reported impairing left ventricular (LV)performance. Alternative pacing sites in right ventricle (RV) has been explored to obtain better cardiac function. Our study was designed to compare the hemodynamic effects of right ventricular septal (RVS) pacing with RVA pacing. Methods Ten elderly patients with chronic atrial fibrillation (AF) and long RR interval or slow ventricular response (VR) received VVI pacing. The hemodynamic difference between RVS and RVA pacing were examined by transthoracic echocardiography (TTE). Results Pacing leads were implanted successfully at the RVA and then RVS in all patients without complication. The left ventricular (LV) parameters,measured during RVA pacing including left ventricular ejection fraction (LVEF), FS, stroke volume (SV) and peak E wave velocity (EV) were decreased significantly compared to baseline data, while during RVS pacing, they were significantly better than those during RVA pacing. However, after 3-6 weeks there was no statistical significant difference between pre- and post- RVS pacing.Conclusions The LV hemodynamic parameters during RVA pacing were significantly worse than baseline data. The short term LV hemodynamic parameters of RVS pacing were significantly better than those of RVA pacing; RVS pacing could improve the hemodynamic effect through maintaining normal ventricular activation sequence and biventricular contraction synchrony in patients with chronic AF and slow ventricular response.

  2. Infective endocarditis affecting both systemic and pulmonary circulations predisposed by a ventricular septal defect.

    Science.gov (United States)

    Ando, M; Sakai, A; Nakamura, K; Iwata, Y; Sanae, T

    2000-07-01

    A 39-year-old woman was admitted to our hospital presenting persisting fever. An echocardiographic examination showed severe aortic and mitral valve regurgitation with moderate tricuspid regurgitation. Small left-to-right shunt through the ventricular septal defect was identified. Vegetation was also detected on the tricuspid, mitral, and aortic valves. At one month after admission, the patient showed sudden onset of headache and abdominal pain. A computed tomographic scan demonstrated cerebral and splenic infarction. A pulmonary perfusion scintigram demonstrated perfusion defects in left-S1 and right-S6 regions. At 4 months after admission, as operation was performed. The aortic valve was replaced with a #23 mm CarboMedics prosthesis and the mitral valve with a #29 mm Carbo Medics prosthesis. Tricuspid valve plasty was performed, with closure of He laceration and perforation of the anterior leaflet combined with a commissuroplasty, according to Kay's method. Ventricular septal defect was closed with a bovine pericardial patch. She was discharged at 19 days after the operation, and is leading a good life. Pervasion of the organism seemed to be initiated from the mitral valve which was conveyed by the blood stream to the aortic valve, and to the tricuspid valve through the ventricula septal defect. Left heart evaluation may be important in cases with infective endocarditis and ventricula septal defect.

  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. [Left ventricular synchrony with septum stimulation vs. septal ventricular outflow tract in complete atrioventricular block].

    Science.gov (United States)

    Rodríguez-Serrano, Gustavo; Lara-Vaca, Susano; Pereyra-Nobara, Texar; Bernal-Ruiz, Enrique

    2016-01-01

    Introducción: el objetivo del presente estudio fue determinar si existe diferencia en la función sistólica y la sincronía del ventrículo izquierdo con estimulación del septum medio o tracto de salida del ventrículo derecho en pacientes con bloqueo auriculoventricular portadores de marcapaso. Métodos: estudio observacional, analítico, transversal. Se seleccionaron todos los pacientes mayores de 18 años portadores de marcapaso con diagnóstico de bloqueo auriculoventricular. Se analizaron dos grupos acorde al sitio de colocación del electrodo de estimulación en región septal media o tracto de salida del ventrículo derecho. Se determinó la fracción de expulsión y sincronía del ventrículo izquierdo. Resultados: se incluyeron 54 pacientes por cada grupo, siendo las características de ambos similares, excepto el tiempo de colocación del electrodo de estimulación ventricular (p = < 0.001). No hubo diferencia significativa en la fracción de expulsión o sincronía del ventrículo izquierdo. Conclusiones: no se encontró diferencia en la fracción de expulsión o sincronía del ventrículo izquierdo independientemente del sitio de colocación del electrodo de estimulación.

  5. 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.

  6. 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.

  7. Device closure of post-myocardial infarction ventricular septal defect three weeks after coronary angioplasty

    Science.gov (United States)

    Patnaik, A. N.; Barik, Ramachandra; Kumari, N. Rama; Gulati, A. S.

    2012-01-01

    Percutaneus device closure appears to be safe and effective in patients treated for a residual shunt after initial surgical closure, as well as after two to three weeks of index myocardial infarction. The index case presented with a ventricular septal defect on second of acute myocardial infarction thrombolysed with streptokinase. The general condition of the patient was fairly stable. Cardiac catheterization and coronary angiography showed significant left to right shunt and there was 90 % proximal stenosis of left anterior descending coronary artery. Other coronary arteries were normal. Angioplasty and stenting to the coronary artery lesion was done using drug eluting stent (DES) with very good angiographic result. Patient was discharged after four days in stable condtion. After 3 weeks his ventricular septal defect was closed percutaneusly using cardio -O-fix device with tiny residual shunt. The procedure was uneventful and of brief duration. He was discharged after 5 days of the post procedure in very stable condition with minimal residual shunt. A staged procedure is a better option if the condition of the patient allows strengthening ventricular septal defect border. PMID:22629038

  8. A New Coated Nitinol Occluder for Transcatheter Closure of Ventricular Septal Defects in a Canine Model

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    Yong Zhou

    2013-01-01

    Full Text Available Aims. This study evaluated feasibility and safety of implanting the polyester-coated nitinol ventricular septal defect occluder (pcVSDO in the canine model. Methods and Results. VSD models were successfully established by transseptal ventricular septal puncture via the right jugular vein in 15 out of 18 canines. Two types of VSDOs were implanted, either with pcVSDOs (n=8 as the new type occluder group or with the commercial ventricular septal defect occluders (VSDOs, n=7, Shanghai Sharp Memory Alloy Co. Ltd. as the control group. Sheath size was 10 French (10 Fr in two groups. Then the general state of the canines was observed after implantation. ECG and TTE were performed, respectively, at 7, 30, 90 days of follow-up. The canines were sacrificed at these time points for pathological and scanning electron microscopy examination. The devices were successfully implanted in all 15 canines and were retrievable and repositionable. There was no thrombus formation on the device or occurrence of complete heart block. The pcVSDO surface implanted at day 7 was already covered with neotissue by gross examination, and it completed endothelialization at day 30, while the commercial VSDO was covered with the neotissue in 30th day and the complete endothelialization in 90th day. Conclusion. The study shows that pcVSDO is feasible and safe to close canine VSD model and has good biocompatibility and shorter time of endothelialization.

  9. Altered right ventricular contractile pattern after cardiac surgery: monitoring of septal function is essential.

    Science.gov (United States)

    Nguyen, Tin; Cao, Long; Movahed, Assad

    2014-10-01

    Assessment of right ventricular (RV) function is important in the management of various forms of cardiovascular disease. Accurately assessing RV volume and systolic function is a challenge in day-to-day clinical practice due to its complex geometry. Tricuspid annular plane systolic excursion (TAPSE) and systolic excursion velocity (S') have been reviewed to further assess their suitability and objectivity in evaluating RV function. Multiple studies have validated their diagnostic and prognostic values in numerous pathologic conditions. Diminished longitudinal contraction after cardiothoracic surgery is a well-known phenomenon, but it is not well validated. Despite significant reduction in RV performance along the long-axis assessed by TAPSE and S' after cardiac surgery, RV ejection fractions did not change as well as the left ventricular parameters and exercise capacity. RV contractile patterns were markedly altered with decreased longitudinal shortening and increased transverse shortening, which are likely resulted from the septal damage during cardiac surgery. The septum is essential for RV performance due to its oblique fiber orientation. This allows ventricular twisting, which is a vital mechanism against increased pulmonary vascular resistance. The septum function along with TAPSE and S' should be adequately assessed during cardiac surgery, and evidence of septal dysfunction should lead to reevaluation of myocardial protection methods.

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

    OpenAIRE

    Ori Shen; Sari Lieberman; Benjamin Farber; Daniel Terner; Amnon Lahad; Ephrat Levy-Lahad

    2014-01-01

    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 ...

  11. "Not-so-identical" twins with trisomy 21 and perimembranous ventricular septal defects.

    Science.gov (United States)

    Batlivala, Sarosh P; Courtney, Kendra S; Ebeid, Makram R; Parnell, Aimee S

    2016-06-01

    While trisomy 21 is a common genetic disorder in singletons, the incidence among identical twins is very rare, occurring in approximately 1-2 per 1000 twin gestations. Trisomy 21 is associated with high incidence of congenital heart defects, and commonly occurs with ventricular septal defects (VSDs). Physiologic burden of VSDs depends on prevalence of anatomic and other circulatory factors. A case of identical twins with trisomy 21 and large VSDs is described in the present report. Though genetically identical, phenotypes varied significantly. One twin was managed medically, while the other developed more significant heart failure, requiring operative repair.

  12. Further insights into the syndrome of prolapsing non-coronary aortic cusp and ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Akhlaque N Bhat

    2012-03-01

    Full Text Available Ventricular septal defect ( VSD with prolapse of the right coronary cusp and aortic regurgitation can be managed surgically with the anatomical correction technique . However when the VSD is located underneath the non coronary cusp surgical management differs due to anatomical constraints and secondary pathological changes seen in the non coronary cusp. It is therefore important that the location of the VSD and the morphology of prolapsing cusp be characterised preoperatively in order to plan appropriate surgical repair. We present a case study in which we discuss the salient differences in the surgical management of the prolapsing right and the prolapsing non coronary cusps.

  13. Percutaneous closure of atrial septal defects leads to normalisation of atrial and ventricular volumes

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    Worthley Matthew I

    2008-12-01

    Full Text Available Abstract Background Percutaneous closure of atrial septal defects (ASDs should potentially reduce right heart volumes by removing left-to-right shunting. Due to ventricular interdependence, this may be associated with impaired left ventricular filling and potentially function. Furthermore, atrial changes post-ASD closure have been poorly understood and may be important for understanding risk of atrial arrhythmia post-ASD closure. Cardiovascular magnetic resonance (CMR is an accurate and reproducible imaging modality for the assessment of cardiac function and volumes. We assessed cardiac volumes pre- and post-percutaneous ASD closure using CMR. Methods Consecutive patients (n = 23 underwent CMR pre- and 6 months post-ASD closure. Steady state free precession cine CMR was performed using contiguous slices in both short and long axis views through the ASD. Data was collected for assessment of left and right atrial, ventricular end diastolic volumes (EDV and end systolic volumes (ESV. Data is presented as mean ± SD, volumes as mL, and paired t-testing performed between groups. Statistical significance was taken as p Results There was a significant reduction in right ventricular volumes at 6 months post-ASD closure (RVEDV: 208.7 ± 76.7 vs. 140.6 ± 60.4 mL, p Conclusion ASD closure leads to normalisation of ventricular volumes and also a reduction in right atrial volume. Further follow-up is required to assess how this predicts outcomes such as risk of atrial arrhythmias after such procedures.

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

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    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.

  15. Urgent hybrid approach in treatment of the acute myocardial infarction complicated by the ventricular septal rupture

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    Radosavljević-Radovanović Mina

    2014-01-01

    Full Text Available Introduction. Ventricular septal rupture (VSR in the acute myocardial infarction (AMI is a rare but very serious complication, still associated with high mortality, despite significant improvements in pharmacological and surgical treatment. Therefore, hybrid approaches are introduced as new therapeutical options. Case Outline. We present an urgent hybrid approach, consisting of the initial percutaneous coronary intervention (PCI of the infarct-related artery, followed by immediate surgical closure of the ventricular septal rupture, for treatment of high risk, hemodynamically unstable female patient with AMI caused by one-vessel disease and complicated by VSR and cardiogenic shock. Since the operative risk was also very high (EUROSCORE II 37%, this therapeutic decision was based on the assumption that preoperative PCI could promptly establish blood flow and thereby lessen the risks, duration and complexity of urgent cardiosurgical intervention, performed on the same day. This approach proved to be successful and the patient was discharged from the hospital on the fifteenth postoperative day in stable condition. Conclusion. In selected cases, with high operative risk and unstable hemodynamic state due to AMI complicated by VSR, urgent hybrid approach consisting of the initial PCI followed by surgical closure of VSR may represent an acceptable treatment option and contribute to the treatment of this complex group of patients.

  16. Utilization of 3 amplatzer occluders for closure of post-myocardial infarction ventricular septal defect.

    Science.gov (United States)

    Kar, Saibal; Ibebuogu, Uzoma N; Conte, Antonio Hernandez

    2012-05-01

    This case report describes a patient who sustained a post-myocardial infarction ventricular septal defect (VSD) with an associated left ventricular aneurysm who developed cardiogenic shock and required an intra-aortic balloon pump for hemodynamic stabilization. After deployment of a single Amplatzer occluder (AGA Medical), a residual VSD measuring 0.5 cm was noted. Therefore, a second Amplatzer occluder was deployed and a minimal residual VSD remained. The patient remained hemodynamically stable throughout the procedure and was subsequently extubated with removal of intra-aortic balloon pump. Post-discharge, the patient was readmitted with congestive heart failure. A third Amplatzer device was deployed to ameliorate the recurrent VSD shunt. At 9-week follow-up, transthoracic echocardiogram was performed and findings included: 1) left ventricular ejection fraction of 62%; 2) appearance of 3 Amplatzer devices along the interventrcular septum seated well with no motion and residual shunt; 3) moderate diastolic dysfunction with pseudonormal left ventricular filling pattern; and 4) no valvular abnormalities. The patient had increased exercise tolerance with no shortness of breath at rest or with exertion. This case demonstrates the utility and viability of multiple Amplatzer device deployment as a means of repairing a large post-myocardial infarction VSD and recurrent VSDs.

  17. 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 ...

  18. 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 da

  19. 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, penergy loss were significantly correlated with BNP (r=0.75, 0.69 and 0.49, penergy 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.

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

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    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. Onychomycosis due to Candida parapsilosis in a Child with Ventricular Septal Defect: An Unusual Predisposition

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    Supram Hosuru Subramanya

    2016-01-01

    Full Text Available Candida parapsilosis is emerging as a potential pathogen for onychomycosis. A 4-year-old male child with perimembranous ventricular septal defect (VSD was admitted with features of cystitis and was treated with broad spectrum antibiotics. Two weeks later, he developed yellowish discoloration of nails of both hands. The sloughed out nail, on microscopy, showed numerous yeast forms that were identified as Candida parapsilosis by both phenotypic and genotypic methods. Antifungal sensitivity testing of the isolate was performed by microbroth dilution method in accordance with CLSI guidelines. Patient was successfully treated with topical amphotericin B and oral fluconazole. Thus, one should have a high index of suspicion of C. parapsilosis onychomycosis, especially when the patient is in the paediatric age group, presenting with unusual predisposing condition like congenital heart disease, and is on broad spectrum antibiotics.

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

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    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.

  3. Ventricular septal rupture imaged with real-time three-dimensional transesophageal echocardiography: diagnosis at a glance.

    Science.gov (United States)

    Squeri, Angelo; Conti, Rita; Bosi, Stefano

    2014-01-01

    Ventricular septal rupture is a rare complication of acute myocardial infarction and its diagnosis can be really challenging especially in the case of complex lesions. Echocardiography is the technique of choice for the detection of mechanical complications following myocardial infarction. The introduction of three-dimensional echocardiography offers new imaging possibilities with precise localization and easiest definition of the defect anatomy. This information is of paramount importance in the setting of a percutaneous closure procedure. We describe a case where real-time three-dimensional transesophageal echocardiography correctly defined the complex anatomy of a postmyocardial infarction septal defect with an associated pseudoaneurysm.

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

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    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.

  5. Hepatic and Renal Failure after Anterior Myocardial Infarction Induced Apical Ventricular Septal Defect

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    Dirk Lossnitzer

    2010-01-01

    Full Text Available We report the case of a 68-year-old man suffering from incremental hepatic and renal failure one month after anterior myocardial infarction. Cardiac MRI showed a pronounced apical post-AMI aneurysm, a moderate to severe mitral and tricuspid regurgitation as well as a hemodynamically highly significant 12 mm apical ventricular septal defect with a left-to-right ventricular shunt of almost 63% as the underlying cause. Heart X-ray revealed a severe LAD in-stent restenosis. CAPD catheter drainage of hydroperitoneum due to congestive liver and renal failure was provided in combination with intensified CAPD hemodialysis. Heart surgery was performed where the apical aneurysm was excised, the mitral valve was reconstructed, the IVSD was closed and the subtotally in-stent occluded LAD was bypassed. Post-surgery, the ascites were significantly reduced, and CAPD hemodialysis therapy could be terminated since the renal function gradually improved (MDRD = 25 mL/min. To our knowledge, for the first time we report successful CAPD catheter drainage of hydroperitoneum in combination with CAPD hemodialysis.

  6. SUCCESSFUL ANAESTHETIC MANAGEMENT OF A PATIENT WITH PARADOXICAL VENTRICULAR SEPTAL MOTION (PVSM POSTED FOR MODIFIED RADICAL NECK DISSECTION WITH RADIAL FREE FLAP

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    Amruta

    2015-04-01

    Full Text Available BACKGROUND : Patients with paradoxical ventricular septal motion are a challenge to anaesthesiologist due to risk of perioperative myocardial ischaemia and sudden cardiac arrest. CASE DESCRIPTION : We present anaesthetic management of a 45year old lady with a diagnosed case of carcinoma left buccal mucosa with paradoxical ventricular septal motion posted for modified radical neck disse ction with radial free flap. CONCLUSION : Although clinical manifestations of this cardiac condition may be mild, there is certainly associated pathology of direct relevance, which carries importance in the anaesthetic management in the peri - operative perio d. Patients with paradoxical ventricular septal motion of any etiology are more prone for perioperative myocardial ischaemia and sudden cardiac arrest, because if cardiac conduction is not maintained properly then it may result in further increase in the p aradoxical ventricular septal motion

  7. Retraction: Selective cerebro-myocardial perfusion under mild hypothermia during primary repair for aortic coarctation with ventricular septal defect.

    Science.gov (United States)

    2013-04-01

    The following article from Artificial Organs, "Selective Cerebro-Myocardial Perfusion Under Mild Hypothermia During Primary Repair for Aortic Coarctation With Ventricular Septal Defect" by Huiwen Chen, Haifa Hong, Zhongqun Zhu and Jinfen Liu, published online on 2 November 2012 in Wiley Online Library (wileyonlinelibrary.com), has been retracted by agreement between the authors, the journal Editor-in-Chief, Paul S. Malchesky, the International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc. The retraction has been agreed due to simultaneous publication of a substantially similar article, "Continuous Cerebral and Myocardial Perfusion During One-Stage Repair for Aortic Coarctation With Ventricular Septal Defect", by Huiwen Chen, Haifa Hong, Zhongqun Zhu and Jinfen Liu, in Pediatric Cardiology 7 November 2012 [Epub ahead of print].

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

    OpenAIRE

    Luciana da Fonseca da Silva; José Pedro da Silva; Turquetto,Aida L R; Sonia Meiken Franchi; Cascudo,Cybelle M; Rodrigo Moreira Castro; Walter José Gomes; Christian Schreiber

    2014-01-01

    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 as...

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

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    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.

  10. Conduction Disorders in Continuous Versus Interrupted Suturing Technique in Ventricular Septal Defect Surgical Repair

    Directory of Open Access Journals (Sweden)

    Gholampour-Dehaki

    2016-01-01

    Full Text Available Background Ventricular septal defects (VSD is one of the most frequent congenital cardiac malformations and cardiac conduction disorders are still one of the serious postoperative complications in this surgery. Objectives This study aimed to compare the incidence of conduction disorders with the use of continuous compared to interrupted suturing techniques in VSD surgical repair. Patients and Methods Previously recorded data of 231 patients who underwent surgical closure of VSD between January 2009 and January 2012 at the Rajaie cardiovascular medical and research center were retrospectively reviewed. VSD surgical repair was performed using continues suturing technique in group A patients (n = 163, 70.6% and interrupted suturing technique in group B patients (n = 68, 29.4%. Results The most common concomitant congenital anomaly was Tetralogy of Fallot (27.3%. Twenty-four (10.4% patients had intraoperative cardiac arrhythmia, including 19 (8.2% transient and 5 (2.2 % permanent arrhythmia. During their ICU stay, ventricular arrhythmia and complete heart block were observed in 34 (14.7% and 5 patients (2.2%, respectively. At the time of the last follow-up, incomplete right bundle branch block (RBBB, complete RBBB, RBBB with left anterior hemi-block, and complete heart block were identified in 84 (36.4%, 42 (18.2%, 29 (12.6%, and 5 patients (2.2%, respectively. The results revealed that group A patients were most likely to have had cardiac arrhythmias during their ICU stay and at the time of last follow-up (P < 0.001, while the intraoperative incidence of cardiac arrhythmia during surgery was not statistically significant between the two groups (P = 0.06. Conclusions In the absence of any statistical differences in the other risk factors between the two groups, the difference in the incidence of conduction disorders can be attributed to the type of suturing used during the procedure.

  11. An evaluation of the left atrial/aortic root ratio in children with ventricular septal defect.

    Science.gov (United States)

    Lester, L A; Vitullo, D; Sodt, P; Hutcheon, N; Arcilla, R

    1979-08-01

    Echocardiograms were performed in 80 infants and children with isolated ventricular septal defect (VSD) who underwent cardiac catheterization. The pulmonary-to-systemic flow ratio (Qp/Qs) was correlated with the echocardiographic left atrial-to-aortic root diameter ratio (LA/Ao), and a relatively poor correlation (r = 0.62) was found. The end-systolic diameters of the left atrium and aorta at the level of the aortic root, obtained from lateral cineangiograms of 55 of the 80 patients, were compared with the corresponding echocardiographic dimensions. To assess the possible effect of transducer beam angulation upon the echocardiographic determinations, the angiographic measurements were made at 0 degrees position (perpendicular to the frontal plane) and at angles of 5 degrees, 10 degrees, 15 degrees and 20 degrees from zero, using the aortic root center as the point of intersection. The echocardiographic and angiographic aortic root measurements were comparable (r = 0.95), and the angiographically derived aortic diameter did not vary with different angle projections. However, the left atrial angiographic dimensions were significantly influenced by the angle of projection. We conclude that the echocardiographic LA/Ao ratio cannot reliably estimate the severity of the shunt flow in VSD.

  12. 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.

  13. Association of NFATc1 gene polymorphism with ventricular septal defect in the Chinese Han population

    Institute of Scientific and Technical Information of China (English)

    SHEN Lei; LI Zhong-zhi; SHEN A-dong; LIU Hui; BAI Song; GUO Jian; YUAN Feng

    2013-01-01

    Background Congenital heart disease (CHD) is a diverse group of diseases determined by genetic and environmental factors.Considerable research has been done on genes associated with the development of the heart.Recently,focus is on the role of transcription factor NFATc1 in the development of proper valve and septa.As part of a larger study,high density single nucleotide polymorphism (SNP) scanning was used to explore the relationship between NFATc1 gene polymorphism and susceptibility to ventricular septal defect (VSD) in the Chinese Han population.Methods One hundred and ninety-two pediatric patients with congenital VSD and 192 matching healthy control subjects were studied.The haplotype reconstructions were calculated by PHASE2.0 software.Haploview software was used to perform linkage disequilibrium assessment and define haplotype blocks.The algorithm used for defining the blocks was the confidence interval method.Results The NFATc1 gene region can be divided into 11 haplotype blocks.Strong linkage disequilibrium existed within blocks 6,8,9,and 11.Three SNPs (rs7240256,rs11665469,and rs754505) within the NFATc1 gene had significant correlation with VSD by single marker association analysis.In addition,two haplotypes correlated with VSD.Conclusions NFATc1 is associated with the occurrence of VSD and it may be a predisposing gene to CHD in Han Chinese.This finding has set a direction for further genetic and functional studies.

  14. Novel and functional DNA sequence variants within the GATA5 gene promoter in ventricular septal defects

    Institute of Scientific and Technical Information of China (English)

    Ji-Ping Shan; Xiao-Li Wang; Yuan-Gang Qiao; Hong-Xin Wan Yan; Wen-Hui Huang; Shu-Chao Pang; Bo Yan

    2014-01-01

    Background: Congenital heart disease (CHD) is the most common human birth defect. Genetic causes for CHD remain largely unknown. GATA transcription factor 5 (GATA 5) is an essential regulator for the heart development. Mutations in the GATA5 gene have been reported in patients with a variety of CHD. Since misregulation of gene expression have been associated with human diseases, we speculated that changed levels of cardiac transcription factors, GATA5, may mediate the development of CHD. Methods: In this study, GATA5 gene promoter was genetically and functionally analyzed in large cohorts of patients with ventricular septal defect (VSD) (n=343) and ethnic-matched healthy controls (n=348). Results: Two novel and heterozygous DNA sequence variants (DSVs), g.61051165A>G and g.61051463delC, were identified in three VSD patients, but not in the controls. In cultured cardiomyocytes, GATA5 gene promoter activities were significantly decreased by DSV g.61051165A>G and increased by DSV g.61051463delC. Moreover, fathers of the VSD patients carrying the same DSVs had reduced diastolic function of left ventricles. Three SNPs, g.61051279C>T (rs77067995), g.61051327A>C (rs145936691) and g.61051373G>A (rs80197101), and one novel heterozygous DSV, g.61051227C>T, were found in both VSD patients and controls with similar frequencies. Conclusion: Our data suggested that the DSVs in the GATA5 gene promoter may increase the susceptibility to the development of VSD as a risk factor.

  15. Clinical Study of 147 Cases with Ventricular Septal Defect and Aortic Valve Insufficiency in Children

    Institute of Scientific and Technical Information of China (English)

    李渝芬; 李江林; 王树水; 庄建; 陈欣欣

    2004-01-01

    Objectives To introduce our experience in treatment of ventricular septal defects with aortic valve insufficiency. Methods A total of 147 cases was involved in a nine-year retrospective study. Age ranged from 5 months to 15 years (mean,7.60 ± 4.12 years). All had been diagnosed by thorough history, physical examinations, chest roentgenogram, two-dimensional echocardiogram.Some 103 patients had taken catheterization and angiocardiography. 91 (61.9%) cases underwent the procedure of VSD closure, 31 (21.08%) had aortic valvuloplasty simultaneously, and 25 (17.02%) were performed VSD closure plus aortic valve replacement.Results Among the 147 patients, 137 (93.19%) have fully recovered, 6 (4.08%) improved, and 4 patients died (2.73%). Conclusions As soon as being diagnosed as subarterial VSD, surgical intervention should be recommended at an early date. The cases of perimembranous VSD also need close follow-up. Once AI occurs, operations should be taken in time. The cases without AI ought to accept treatment during school age.

  16. Peptidomic Analysis of Amniotic Fluid for Identification of Putative Bioactive Peptides in Ventricular Septal Defect

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    Xing Li

    2016-05-01

    Full Text Available Background: Ventricular septal defect (VSD is one of the most common congenital heart diseases and to date the role of peptides in human amniotic fluid in the pathogenesis of VSD have been rarely investigated. Methods: To gain insight into the mechanisms of protein and peptides in cardiovascular development, we constructed a comparative peptidomic profiling of human amniotic fluid between normal and VSD fetuses using a stable isobaric labeling strategy involving tandem mass tag reagents, followed by nano liquid chromatography tandem mass spectrometry. Results: We identified and quantified 692 non-redundant peptides, 183 of which were differentially expressed in the amniotic fluid of healthy and VSD fetuses; 69 peptides were up regulated and 114 peptides were down regulated. These peptides were imported into the Ingenuity Pathway Analysis (IPA and identified putative roles in cardiovascular system morphogenesis and cardiogenesis. Conclusion: We concluded that 35 peptides located within the functional domains of their precursor proteins could be candidate bioactive peptides for VSD. The identified peptide changes in amniotic fluid of VSD fetuses may advance our current understanding of congenital heart disease and these peptides may be involved in the etiology of VSD.

  17. Towards high-throughput mouse embryonic phenotyping: a novel approach to classifying ventricular septal defects

    Science.gov (United States)

    Liang, Xi; Xie, Zhongliu; Tamura, Masaru; Shiroishi, Toshihiko; Kitamoto, Asanobu

    2015-03-01

    The goal of the International Mouse Phenotyping Consortium (IMPC, www.mousephenotype.org) is to study all the over 23,000 genes in the mouse by knocking them out one-by-one for comparative analysis. Large amounts of knockout mouse lines have been raised, leading to a strong demand for high-throughput phenotyping technologies. Traditional means via time-consuming histological examination is clearly unsuitable in this scenario. Biomedical imaging technologies such as CT and MRI therefore have started being used to develop more efficient phenotyping approaches. Existing work however primarily rests on volumetric analytics over anatomical structures to detect anomaly, yet this type of methods generally fail when features are subtle such as ventricular septal defects (VSD) in the heart, and meanwhile phenotypic assessment normally requires expert manual labor. This study proposes, to the best of our knowledge, the first automatic VSD diagnostic system for mouse embryos. Our algorithm starts with the creation of an atlas using wild-type mouse images, followed by registration of knockouts to the atlas to perform atlas-based segmentation on the heart and then ventricles, after which ventricle segmentation is further refined using a region growing technique. VSD classification is completed by checking the existence of an overlap between left and right ventricles. Our approach has been validated on a database of 14 mouse embryo images, and achieved an overall accuracy of 90.9%, with sensitivity of 66.7% and specificity of 100%.

  18. Clinical analysis and risk stratification of ventricular septal rupture following acute myocardial infarction

    Institute of Scientific and Technical Information of China (English)

    HU Xiao-ying; QIU Hong; QIAO Shu-bin; KANG Lian-ming; SONG Lei; ZHANG Jun; TAN Xiao-yan

    2013-01-01

    Background Ventricular septal rupture (VSR) remains an infrequent but devastating complication of acute myocardial infarction (AMI).The best time to undergo surgical repair is controversial and there is currently no risk stratification for patients with VSR to guide treatment.The purpose of this study was to review the clinical outcomes of 70 patients with VSR,to analyze the short-term prognosis factors of VSR following AMI,and to make a risk stratification for patients with VSR.Methods A total of 70 consecutive VSR patients following AMI treated in our hospital from January 2002 to October 2010 were enrolled in this study retrospectively.The difference of clinical characteristics were observed between patients with VSR who survived <30 days and survived >30 days.We analyzed the short-term prognosis factors of VSR and established the short-term prognosis index of VSR (SPIV) based on the Logistic regression analysis to stratify patients with VSR.Results Among 12 354 patients with acute ST-segment elevation myocardial infarction,70 (0.57%) patients (33 males and 37 females) were found to have VSR.The average age was (68.1±8.5) years.Fifty-four (77.1%) patients were diagnosed with an acute anterior infarction.Patients with VSR selected for surgical repair had better outcomes than patients treated conservatively; 1-year mortality 9.5% versus 87.8%,P <0.005.Logistic regression analysis revealed that female (P=-0.013),anterior AMI (P=0.023),non-ventricular aneurysm (P=0.023),non-diabetes (P=0.009),Killip class 3 or 4 (P=0.022) and time from AMI to VSR less than 4 days (P=0.027) were independent risk determinants for shortterm mortality.SPIV >9 indicates a high risk as the 30-day mortality is 77.4%; SPIV <8 indicates a low risk as the 30-day mortality is 28.6%; SPIV between 8 and 9 indicates a moderate risk.Conclusions VSR remains a rare but devastating complication of AMI.The independent risk determinants for short-term mortality of VSR were female

  19. QUANTIFICATION OF RIGHT VENTRICULAR FUNCTION IN ATRIAL SEPTAL DEFECT USING ULTRASOUND-BASED STRAIN RATE IMAGING

    Institute of Scientific and Technical Information of China (English)

    MENG Xiang-chun; SUN Kun; ZHANG Yu-qi; HUANG Mei-rong; GAO Wei; ZHANG Zhi-fang; SHEN Rong; CHEN Shu-bao

    2005-01-01

    Objective To study the validation of ultrasound-based strain rate imaging in the quantitative assessment of right ventricular (RV) function in atrial septal defect (ASD). Methods Tissue Doppler images (TDI) of RV longitudinal and short axes were recorded from the apical 4-chamber view and the subcostal short-axis view in 18 normal controls, 28 children with ASD and 14 children after Amplazter closure of ASD respectively. Peak systolic velocities (V), peak systolic strain rates (SR), peak systolic strains (S) at the basal segment, middle segment of RV lateral wall and the basal septum from the longitudinal axis, the middle segment of RV free wall from the short axis were quantitatively measured using QLAB TM tissue velocity quantification software system respectively. Peak dp/dt from the RV isovolumic contraction determined during the right cardiac catheterization in 28 ASD patients was used as the gold standard of RV contractility. Peak systolic indices were compared against max dp/dt by linear correlation. Results Peak systolic indices at the basal and middle segments of RV lateral wall from the longitudinal axis increased significantly in 28 ASD patients.Peak systolic indices at the basal septum also increased in patient group, but not significantly. Significant decreases in peak systolic indices at the basal and middle segments of RV lateral wall were observed after the Amplatzer closure in 14 ASD patients. There was no significant difference at the middle segment of RV free wall from the short axis between patient group and normal control. A strong correlation was found between max dp/dt and peak systolic indices at the basal and middle segments of RV lateral wall (P<0.05). Conclusion Ultrasound-based strain rate imaging can assess quantitatively RV function in CHD. Peak systolic strains determined at the basal and middle segments of RV lateral wall are strong noninvasive indices of RV contractility.

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

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    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

  1. 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

    2017-08-18

    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.

  2. One-year cardiac morphological and functional evolution following permanent pacemaker implantation in right ventricular septal position in chagasic patients

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    Otaviano da Silva Júnior

    2012-06-01

    Full Text Available INTRODUCTION: The septal position is an alternative site for cardiac pacing (CP that is potentially less harmful to cardiac function. METHODS: Patients with Chagas disease without heart failure submitted to permanent pacemaker (PP implantation at the Clinics Hospital of the Triângulo Mineiro Federal University (UFTM, were selected from February 2009 to February 2010. The parameters analyzed were ventricular remodeling, the degree of electromechanical dyssynchrony (DEM, exercise time and VO2 max during exercise testing (ET and functional class (NYHA. Echocardiography was performed 24 to 48h following implantation and after one year follow-up. The patients were submitted to ET one month postprocedure and at the end of one year. RESULTS: Thirty patients were included. Patient mean age was 59±13 years-old. Indication for PP implantation was complete atrioventricular (AV block in 22 (73.3% patients and 2nd degree AV block in the other eight (26.7%. All patients were in NYHA I and no changes occurred in the ET parameters. No variations were detected in echocardiographic remodeling measurements. Intraventricular dyssynchrony was observed in 46.6% of cases and interventricular dyssynchrony in 33.3% of patients after one year. CONCLUSIONS: The findings of this work suggest that there is not significant morphological and functional cardiac change following pacemaker implantation in septal position in chagasic patients with normal left ventricular function after one year follow-up. Thus, patients may remain asymptomatic, presenting maintenance of functional capacity and no left ventricular remodeling.

  3. Post-traumatic ventricular septal defect: a rare indication for extracorporeal membrane oxygenation as a bridge to transplant

    Science.gov (United States)

    Hernández-Pérez, Francisco José; Gómez-Bueno, Manuel Francisco; Escudier-Villa, Juan Manuel; Castedo, Evaristo; Segovia, Javier; Alonso-Pulpón, Luis Antonio

    2017-01-01

    A ventricular septal defect (VSD) is a rare complication of blunt chest trauma. This report presents the case of a 44-year-old man who developed a VSD as a result of high-energy closed chest trauma. We describe the initial surgical and medical management of the cardiac rupture. After failed repair surgery, extracorporeal membrane oxygenation (ECMO) was used as a bridge to heart transplantation. We discuss the successful use of ECMO to improve the prognosis results in this rare and complex entity. PMID:28164015

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

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    Abdul-rahman R. Abdel-karim

    2016-01-01

    Full Text Available 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 favorable outcome.

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

    Science.gov (United States)

    Abdel-karim, Abdul-rahman R.; Main, Michael L.

    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 favorable outcome. PMID:27668097

  6. High-septal pacing reduces ventricular electrical remodeling and proarrhythmia in chronic atrioventricular block dogs

    DEFF Research Database (Denmark)

    Winckels, Stephan K G; Thomsen, Morten Bækgaard; Oosterhoff, Peter

    2007-01-01

    This study was designed to analyze the relevance of ventricular activation patterns for ventricular electrical remodeling after atrioventricular (AV) block in dogs.......This study was designed to analyze the relevance of ventricular activation patterns for ventricular electrical remodeling after atrioventricular (AV) block in dogs....

  7. [Serial Pathological Changes Between Pre- and Post-pulmonary Artery Banding in a 21-trisomy Patient with Ventricular Septal Defect].

    Science.gov (United States)

    Sato, Ken; Misawa, Yoshio; Kawada, Masaaki; Maekawa, Yoshiyuki

    2016-05-01

    Although many patients with a ventricular septa defect undergo primary intra-cardiac repair surgery, some patients require 2-stage repair with pulmonary artery banding. Patients with 21-trisomy, who exhibit fragility of the pulmonary vasculature and respiratory systems, may be candidates for this approach. Herein, we report the case of a 21-trisomy patient with ventricular septal defect associated with pulmonary hypertension. She underwent pulmonary artery banding at 4 months of age, and a subsequent radical operation at 27 months. Serial pathological findings between pre- and post-pulmonary artery banding revealed that changes of pulmonary vasculature and respiratory systems were reversible. Her Heath-Edwards grade changed from 0 from II and index of pulmonary vasculature disease changed from 1.1 to 1.0. In conclusion, pulmonary artery banding in a case with pulmonary hypertension prevented the lung from becoming irreversible pathological changes.

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

    Science.gov (United States)

    Kopic, S; Stephensen, S S; Heiberg, E; Arheden, H; Bonhoeffer, P; Ersbøll, M; Vejlstrup, N; Søndergaard, L; Carlsson, M

    2017-06-05

    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 this is a consequence of the surgical repair, or whether it is inherent to PR. The aim of this study was to assess the relationship between longitudinal, lateral and septal pumping in a porcine model of isolated PR. Piglets were divided into a control (n = 8) group and a treatment (n = 12) group, which 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 contributions to stroke volume (SV) were quantified by measuring volumetric displacements from end-diastole to end-systole in the cardiac short axis and long axis. PR resulted in a lower longitudinal contribution to RV stroke volume, compared to controls (60.0 ± 2.6% vs. 73.6 ± 3.8%; P = 0.012). Furthermore, a compensatory increase in septal contribution to RVSV was observed (11.0 ± 1.6% vs. -3.1 ± 1.5%; P function were reversed by PPVR. These findings suggest that PR contributes to decreased RV longitudinal function in the absence of scarring from cardiac surgery. Measurement of longitudinal RVSV may aid risk stratification and timing for interventional correction of PR in TOF patients. © 2017 The Authors. Acta Physiologica published by John Wiley & Sons Ltd on behalf of Scandinavian Physiological Society.

  9. Ventricular Septal Perforation after Biventricular Takotsubo Cardiomyopathy Successfully Repaired with an Amplatzer Device: First Report in the Literature

    Science.gov (United States)

    Rodríguez, Alfredo E.; Fernandez-Pereira, Carlos; Mieres, Juan; Ascarrunz, Diego; Gabe, Eduardo; Rodríguez-Granillo, Alfredo Matías; Frattini, Romina; Stuzbach, Pablo

    2016-01-01

    A 79-year-old female was admitted with sudden onset dyspnea, mild oppressive chest pain, and severe anxiety disorder. Patient had history of hypertension, dyslipidemia, smoking, and chronic obstructive pulmonary disease. On admission blood pressure was 160/90 and heart rate was 130 bpm. Transthoracic echocardiography (TE) and contrast tomography showed a thin septum with an abnormal left and right ventricular contraction with an “apical ballooning” pattern and mild increase of cardiac enzymes. At the 4th day of admission, the patient presented symptoms and signs of congestive heart failure and developed cardiogenic shock. EKG showed an inversion of T waves in all precordial leads. In a new TE, a ventricular septal perforation (VSP) in the apical portion of the septum was seen. Coronary angiogram showed angiographically “normal” coronary arteries. With a diagnosis of VSP in takotsubo cardiomyopathy, a percutaneous procedure to repair the VSP was performed 11 days after admission. The VSP was closed with an Amplatzer device. TE performed 24 hours after showed significant improvement of ventricular function and good apposition of the Amplatzer device. Three days later she was discharged from the hospital. To our knowledge, this is the first reported case of a VSP in a TCM repaired percutaneously with an occluder device. PMID:27190656

  10. Ventricular Septal Perforation after Biventricular Takotsubo Cardiomyopathy Successfully Repaired with an Amplatzer Device: First Report in the Literature

    Directory of Open Access Journals (Sweden)

    Alfredo E. Rodríguez

    2016-01-01

    Full Text Available A 79-year-old female was admitted with sudden onset dyspnea, mild oppressive chest pain, and severe anxiety disorder. Patient had history of hypertension, dyslipidemia, smoking, and chronic obstructive pulmonary disease. On admission blood pressure was 160/90 and heart rate was 130 bpm. Transthoracic echocardiography (TE and contrast tomography showed a thin septum with an abnormal left and right ventricular contraction with an “apical ballooning” pattern and mild increase of cardiac enzymes. At the 4th day of admission, the patient presented symptoms and signs of congestive heart failure and developed cardiogenic shock. EKG showed an inversion of T waves in all precordial leads. In a new TE, a ventricular septal perforation (VSP in the apical portion of the septum was seen. Coronary angiogram showed angiographically “normal” coronary arteries. With a diagnosis of VSP in takotsubo cardiomyopathy, a percutaneous procedure to repair the VSP was performed 11 days after admission. The VSP was closed with an Amplatzer device. TE performed 24 hours after showed significant improvement of ventricular function and good apposition of the Amplatzer device. Three days later she was discharged from the hospital. To our knowledge, this is the first reported case of a VSP in a TCM repaired percutaneously with an occluder device.

  11. Mid-septal hypertrophy and apical ballooning; potential mechanism of ventricular tachycardia storm in patients with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Hwang, Eui-Seock; Pak, Hui-Nam

    2012-01-01

    Medically refractory ventricular tachycardia (VT) storm can be controlled with radiofrequency catheter ablation (RFCA), however, it may be difficult to control in some patients with hemodynamic overload. We experienced a patient with intractable VT storm controlled by hemodynamic unloading. The patient had mid-septal hypertrophic cardiomyopathy with an implantable cardioverter defibrillator (ICD) back-up. Because of the severe mid-septal hypertrophy, his left ventricle (LV) had an hourglass-like morphology and showed apical ballooning; the focus of VT was at the border of apical ballooning. Although we performed VT ablation because of electrical storm with multiple ICD shocks, VT recurred 1 hour after procedure. As the post-RFCA monomorphic VT was refractory to anti-tachycardia pacing or ICD shock, we reduced the hemodynamic overload of LV with β-blockade, hydration, and sedation. VT spontaneously stopped 1.5 hours later and the patient has remained free of VT for 24 months with β-blockade alone. In patients with VT storm refractory to antiarrhythmic drugs or RFCA, the mechanism of mechano-electrical feedback should be considered and hemodynamic unloading may be an essential component of treatment.

  12. Parasitic rachipagus conjoined twins with spina bifida, diplomyelia, scoliosis, tethered cord syndrome, and ventricular septal defect--case report.

    Science.gov (United States)

    Zhang, Jiayong; Duan, Hongzhou; Zhang, Yang; Yi, Zhiqiang; Bao, Shengde

    2011-01-01

    A 17-year-old girl presented with a rare case of parasitic rachipagus conjoined twins associated with spina bifida, diplomyelia, scoliosis, tethered cord syndrome, and ventricular septal defect. Physical examination found a well developed breast and an apophysis on the back of the patient, and neuroimaging demonstrated scoliosis, spina bifida from T8 to L5, butterfly-shaped vertebra of T6, abnormal bone behind T7, diplomyelia, and tethered cord. Successful surgical excision of the parasitic mass was performed. Histological examination discovered uterine tube, Muller's duct, bone, cartilage, and nerve tissue besides the mammary gland. Rachipagus conjoined twins are extremely rare, but should be considered if well developed abnormal tissue is found in the dorsal midline of the spine. However, the possibility of malformations in other organs in the autosite should be considered. Careful preoperative examination and refined microsurgery may provide good outcome for the patient.

  13. 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.

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

    Science.gov (United States)

    Kuwelker, Saatchi Mahesh; Shetty, Devi Prasad; Dalvi, Bharat

    2017-01-01

    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. PMID:28163430

  15. [Isolated left ventricular muscular diverticulum in an adult. Value of non-invasive examinations].

    Science.gov (United States)

    Holeman, A; Bellorini, M; Lefevre, T; Lévy, M; Loiret, J; Huerta, F; Thébault, B; Funck, F

    1997-10-01

    The authors report a case of ventriculum in a 45 year old women investigated for chest pain. This was a congenital muscular left ventricular diverticulum confirmed by a complete imaging series including echocardiography, magnetic resonance imaging, angio-scintigraphy and conventional angiography. This diverticulum was unusual due to the fact that there was no associated congenital disease and that it was discovered in an adult. The authors review the literature and discuss the value of non-invasive imaging procedures.

  16. Acute left ventricular failure after transcatheter closure of a secundum atrial septal defect in a patient with hypertrophic cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    ZHANG Cao-jin; HUANG Yi-gao; HUANG Xin-sheng

    2011-01-01

    We report a case of acute left ventricular failure at one hour after transcatheter closure of a secundum atrial septal defect (ASD) in a 28-year-old man with hypertrophic cardiomyopathy. Afforded noninvasive mechanical ventilation and the administration of intravenous morphine and high doses of furosemide, the patient exhibited improvement of his clinical condition, redtion of pulmonary congestion at chest X-ray, and satisfactory blood gas analyses in twelve hours. Twentyfour hours later, the patient received oral administration of furosemide and metoprolol. After 7 days the patient was discharged in good clinical condition. At follow-up at 12 months, the patient had remained symptomatically improved from NYHA Class Ⅲ symptoms before the procedure to Class Ⅱ symptoms. There was no latent arrhythmia at the follow-up examination. Follow-up transthoracic echocardiography estimated an improvement of the left ventricular function. So,transcatheter closure of a secundum ASD in a patient with hypertrophic cardiomyopathy is feasible, and a thorough understanding of the hemodynamic condition of ASD and hypertrophic cardiomyopathy will reduce the complication of ASD closure.

  17. A novel two-stage complete repair method for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

    Institute of Scientific and Technical Information of China (English)

    MEI Ju; DING Fang-bao; ZHU Jia-quan; BAO Chun-rong; XIE Xiao; ZHANG Yun-jiao

    2010-01-01

    Background Pulmonary atresia with ventricular septal defect (PA-VSD) and major aortopulmonary collateral arteries (MAPCAs) remains a challenging complex congenital heart disease nowadays. In the present study, we aimed to develop a two-stage surgical method and to evaluate outcomes of this method in managing PA-VSD and MAPCAs.Methods Between December 2003 and December 2008, 7 female and 4 male patients between the age of 5 and 10 years who were suffering from PA-VSD and MAPCAs were selected and recruited. The native pulmonary artery trunks were absent in all patients; the lungs were solely supplied by major aortopulmonary collaterals, and the numbers of supplied lung segments ranged from 15 to 20 (17.9±1.6). There were a total of 43 MAPCAs in all the patients (3-5 (3.9±0.7) MAPCAs per patient). The accumulated Nakata index was (222.9±29.9) mm~2/m~2 (ranged from 182 to 272). All the patients underwent two sequential operations. Stage one included left major aortopulmonary collateral unifocalization and modified Blalock-Taussig shunt from left posterior lateral thoracotomy; stage two comprised right unifocalization, ligation of the shunt, followed by ventricular septal defect closure and right ventricular outflow tract reconstruction assisted with cardiopulmonary bypass from midline stemotomy.Results All the patients survived the initial surgery, but one of them died of low cardiac output syndrome on the third day after the second operation. Postoperative complications included pneumonia in one case and capillary leak syndrome in another. Postoperative oxygen saturation maintained about 95%-100%, which was significantly higher than pre-operation (P <0.01). During the follow-up period of 3-51 (25.4±15.2) months, there were no late death and no need for re-intervention. All the patients enjoyed their lives with good conditions.Conclusions This two-stage complete repair strategy was well-tolerated and effective with good outcome, thus offering an alternative

  18. [Right ventricular outflow tract reconstruction using monocusp valved outflow patch for pulmonary atresia with ventricular septal defect: influence of the presence of major aorto-pulmonary collateral arteries].

    Science.gov (United States)

    Hisamochi, K; Ishino, K; Kawada, M; Ohshima, Y; Aoki, A; Arai, S; Sano, S

    2001-07-01

    We have preferably utilized monocusp valved outflow patch (MVOP) for right ventricular outflow tract (RVOT) reconstruction in pulmonary atresia with ventricular septal defect (PA + VSD). The purpose of this study was to evaluate the influence of the presence of major aorto-pulmonary collateral arteries (MAPCAs) on probability of MVOP reconstruction and development of RVOT restenosis in midterm. 49 patients underwent complete repair (either MVOP reconstruction or Rastelli procedure) of PA + VSD in our service. These patients were divided into 2 groups: group 1; 21 patients with MAPCAs, group 2; 28 patients without MAPCAs. There was one operative death (group 1). The probably of MVOP reconstruction was similar between group 1 and group 2 (71 vs 79%, p = 0.57, chi 2 test). Follow-up was completed for 48 survivors with the period ranged 3-108 months (mean 47 months). In group 1, one patient died suddenly at home 10 months after surgery. For 47 long-term patients, the ratio of freedom from RVOT restenosis was 72% (95% CI: 52-92%, Kaplan-Meier method) at 5 year. There was no difference between 2 groups (group 1; 73%, 95% CI: 45-100%, group 2; 74%, 95% CI: 48-99%, respectively, p = 0.85 by Log-Rank test). The presence of MAPCAs in PA + VSD was not a risk factor for either the probably of MVOP reconstruction or development of RVOT restenosis in midterm.

  19. Left ventricular T2 distribution in Duchenne Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Hagenbuch Sean

    2010-03-01

    Full Text Available Abstract Background Although previous studies have helped define the natural history of Duchenne Muscular Dystrophy (DMD-associated cardiomyopathy, the myocardial pathobiology associated with functional impairment in DMD is not yet known. The objective of this study was to assess the distribution of transverse relaxation time (T2 in the left ventricle (LV of DMD patients, and to determine the association of myocardial T2 heterogeneity to the severity of cardiac dysfunction. DMD patients (n = 26 and normal control subjects (n = 13 were studied by Cardiovascular Magnetic Resonance (CMR. DMD subject data was stratified based on subject age and LV Ejection Fraction (EF into the following groups: A (12 years, n = 5. LV mid-slice circumferential myocardial strain (εcc was calculated using tagged CMR imaging. T2 maps of the LV were generated for all subjects using a black blood dual spin echo method at two echo times. The Full Width at Half Maximum (FWHM was calculated from a histogram of LV T2 distribution constructed for each subject. Results In DMD subject groups, FWHM of the T2 histogram rose progressively with age and decreasing EF (Group A FWHM= 25.3 ± 3.8 ms; Group B FWHM= 30.9 ± 5.3 ms; Group C FWHM= 33.0 ± 6.4 ms. Further, FWHM was significantly higher in those with reduced circumferential strain (|εcc| ≤ 12% (Group B, and C than those with |εcc| > 12% (Group A. Group A FWHM was not different from the two normal groups (N1 FWHM = 25.3 ± 3.5 ms; N2 FWHM= 24.0 ± 7.3 ms. Conclusion Reduced EF and εcc correlates well with increased T2 heterogeneity quantified by FWHM, indicating that subclinical functional impairments could be associated with pre-existing abnormalities in tissue structure in young DMD patients.

  20. Differences in global and regional left ventricular myocardial mechanics in various morphologic subtypes of patients with obstructive hypertrophic cardiomyopathy referred for ventricular septal myotomy/myectomy.

    Science.gov (United States)

    Kobayashi, Taisei; Dhillon, Ashwat; Popovic, Zoran; Bhonsale, Aditya; Smedira, Nicholas G; Thamilarasan, Maran; Lytle, Bruce W; Lever, Harry M; Desai, Milind Y

    2014-06-01

    Patients with obstructive hypertrophic cardiomyopathy (HC) have various left ventricular (LV) shapes: reverse septal curvature (RSC, commonly familial), sigmoid septum (SS, common in hypertensives), and concentric hypertrophy (CH). Longitudinal (systolic and early diastolic) strain rate (SR) is sensitive in detecting regional myocardial dysfunction. We sought to determine differences in longitudinal SR of patients with obstructive HC, based on LV shapes. We studied 199 consecutive patients with HC (50% men) referred for surgical myectomy. Clinical and echocardiographic parameters were recorded. LV shapes were classified on echocardiography, using basal septal 1/3 to posterior wall ratio: RSC = ratio >1.3 (extending to mid and distal septum), SS = ratio >1.3 (extending only to basal 1/3), and concentric = ratio ≤1.3. Longitudinal systolic and early diastolic SRs were measured from apical 4- and 2-chamber views (VVI 2.0; Siemens, Erlangen). Distribution of RSC, SS, and CH was 50%, 28%, and 22%, respectively. Patients with RSC were significantly younger (47 ± 12 vs 64 ± 10 and 57 ± 11, respectively) with lower hypertension (40% vs 71% and 67%, respectively) than patients with SS or CH (both p RSC had lower global systolic (-0.99 ± 0.3 vs -1.05 ± 0.3 and -1.17 ± 0.3) and early diastolic SR (0.95 ± 0.4 vs 0.98 ± 0.3 and 1.16 ± 0.4) versus patients with SS and CH (in 1/s, both p RSC was associated with abnormal global LV systolic (beta 0.16) and early diastolic (beta -0.17) SR (both p <0.01). In conclusion, patients with HC with RCS have significantly abnormal LV mechanics, despite being younger and less hypertensive. A combination of LV mechanics and shapes could help differentiate between genetically mediated and other causes of obstructive HC.

  1. 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

    BACKGROUND: This cohort study examines the possible association between maternal alcohol intake, including binge drinking, during pregnancy, and the subsequent risk of having a child with an isolated congenital heart defect and, more specifically, with the isolated form of ventricular septal defect...... of alcohol. Few (if any) women with an excessive/abusive intake of alcohol were enrolled into the Danish National Birth Cohort. RESULTS: Through linkage with the National Hospital Discharge Registry, we identified 477 infants with a diagnosis of isolated congenital heart defect registered at any time during...... (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...

  2. Comparison of the offset distance of the tricuspid septal leaflet in neonates with Ebstein's anomaly and neonates with pulmonary atresia with intact ventricular septum.

    Science.gov (United States)

    Kim, Min Jeong; Yu, Jeong Jin; Kang, So Yeon; Seo, Chang Deok; Baek, Jae Suk; Kim, Young-Hwue; Ko, Jae-Kon

    2015-01-01

    An indexed offset distance of the tricuspid septal leaflet ⩾8 mm/m2 is a quantitative criterion for the diagnosis of Ebstein's anomaly. The purpose of this study was to investigate the validity of this criterion for the discrimination of Ebstein's anomaly from pulmonary atresia with intact ventricular septum in neonatal patients. A total of 122 neonatal patients, 56 with Ebstein's anomaly and 66 with pulmonary atresia with intact ventricular septum, were enrolled. Diagnosis of each anomaly was based on typical morphologic features. Echocardiographic variables, including the offset distance of the tricuspid septal leaflet, were measured via an offline analysis of images recorded before 1 month of age. The offset distance of the tricuspid septal leaflet was indexed by the body surface area, and the indexed offset distances in the Ebstein's anomaly and pulmonary atresia with intact ventricular septum groups were 34.2 mm/m2 (7.1-119.1 mm/m2) and 7.2 mm/m2 (0.0-25.6 mm/m2), respectively. The indexed offset distance was ⩾8 mm/m2 in 29 (43.9%) of the patients with pulmonary atresia with intact ventricular septum; clinical and echocardiographic characteristics were comparable between these 29 patients and the remaining 37 patients with pulmonary atresia with intact ventricular septum. When an indexed offset distance ⩾8 mm/m2 was applied as a cut-off for the diagnosis of Ebstein's anomaly, the sensitivity was 0.963 and the specificity was 0.561. In conclusion, indexed offset distance ⩾8 mm/m2 cannot be used as a cut-off for the diagnosis of complicated Ebstein's anomaly in neonatal patients with pulmonary atresia with intact ventricular septum.

  3. Usefulness of myocardial performance index for assessing right ventricular function after percutaneous closure of atrial septal defect

    Institute of Scientific and Technical Information of China (English)

    Jingdong Ding; Genshan Ma; Yaoyao Huang; Xiaoli Zhang; Jian Zhu; Rong Yang; Fengxiang Lu

    2007-01-01

    Objective Assessment of right ventricular function in patients with atrial septal defect (ASD) is difficult. The Doppler myocardial performance index (MPI) may provide a method of assessing function in these patients. The purposes of this study were to evaluate the right ventricular function and its changes in patients with ASD after transcatheter closure of ASD. Methods MPI, defined as the sum of isovolumic relaxation time and isovolumic contraction time derived by ejection time, was measured from tricuspid inflow and right ventricular outflow; Doppler velocity profiles recorded during routine echocardiography. Twenty nine patients ( 13 men, 16 women;mean age 25.28 ± 12.69, range 6 to 57 years) were diagnosed to secundum ASD [the stretched diameters of ASD were from 9 To 36 (24.91 ±7.98) mm], and had a successfully placed Amplatzer septal occluder (ASO) (the sizes of ASO were from 11 to 40 mm ); there were 81 sex-matched, age-matched healthy people (control group 41men, 40 women; mean age 29.02 ± 14.22, range 4 to 45 years).MPI was measured again on 3 days and 1 month after closure of ASD. Change in the study group was assessed and compared to the control subjects with structurally normal hearts. A complete 2- dimensional and Doppler echocardiographic examination was performed in all study groups. Results 1) The isovolumic relaxation and isovolumic contraction times [respectively(77.59 ± 14.39)ms vs (60.93 ± 12.94)ms, P<0.0001; (28.28 ± 10.88)ms vs (23.64 ± 9.01)ms, P=0.027] were prolonged, and ejection time [(260.65 ± 21.86 )ms vs (271.85 ± 21.92)ms, P=0.033] was shortened in patients with ASD compared with that in control subjects, resulting in a marked increase in the MPI(0.40 ± 0.07 vs 0.31 ± 0.05, P<0.0001) from normal values; 2) by Pearson's correlations, the MPI had no correlation with heart rate and blood pressure in control subjects and patients with ASD, but it correlated positively with age in patients with ASD; 3) by Pearson

  4. RARE ASSOCIATION OF POSTERIOR EMBRYOTOXON WITH MAXILLARY HYPOPLASIA, VENTRICULAR SEPTAL DEFECT, PULMONARY ATRESIA AND PATENT DUCTUS ARTERIOSUS

    Directory of Open Access Journals (Sweden)

    Pandey

    2014-12-01

    Full Text Available BACKGROUND: Posterior embryotoxon is a congenital anomaly, considered to be a relatively mild disorder and can occur in 15% of normal eyes. Bilateral posterior embryotoxon associated with maxillary hypoplasia, Ventricular Septal Defect (VSD, Pulmonary Atresia (PA with Patent Ductus Arteriosus (PDA is of rare occurrence and hasn’t been reported in literature till date. CASE: We report a case of 12 year old female who came to us in eye Out Patient Department for routine eye checkup and on detailed ocular examination we found anteriorly displaced Schwalbe’s line. On detailed physical examination and investigations she was found to have pan-systolic murmur and continuous machinery murmur. On Echocardiography, the patient was found to have large peri-membranous VSD with PA and PDA. On Oro-dental examination she was found to have maxillary hypoplasia. CONCLUSION: The present case is reported due to the rarity and sporadic character of the condition and its rare association with cardiac defect and maxillary hypoplasia.

  5. 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.

  6. Left Ventricular Tonic Contraction as a Novel Biomarker of Cardiomyopathy in Duchenne Muscular Dystrophy.

    Science.gov (United States)

    Su, Jennifer A; Ramos-Platt, Leigh; Menteer, JonDavid

    2016-04-01

    Dilated cardiomyopathy (DCM) inevitably afflicts patients with Duchenne muscular dystrophy (DMD) as a consequence of cell death induced by unguarded calcium influx into cardiomyocytes. This mechanism may also inhibit muscle relaxation in early stages of cardiomyopathy. ACE inhibition (ACEi) is known to delay the onset and slow the progression of DCM in DMD. The objective of this study is to use echocardiography to assess for preclinical cardiac changes consistent with intracellular calcium dysregulation before the onset of overt ventricular dysfunction, and to evaluate how prophylactic ACEi may alter these pre-cardiomyopathic changes in the pediatric DMD population. We examined 263 echocardiograms from 70 pediatric patients with DMD. We defined abnormal tonic contraction (TC) as left ventricular internal dimension in diastole (LVIDd) Z-score < -1.5. In our cohort, we found that TC is detectable as early as 8 years of age, and most commonly affects patients between 11 and 15 years. This effect was independent of LV mass and systolic function. Prophylactic ACEi decreased the incidence of TC (p = 0.007) and preserved cardiac function (p < 0.0001). Left ventricular TC often precedes DCM in DMD, most commonly affecting the 11- to 15-year-old age range. TC is not related to ventricular hypertrophy, but rather may be a clinical correlate of the "calcium hypothesis" of DMD pathophysiology. LV TC is thus a promising biomarker for early detection of cardiomyopathy in DMD. ACEi prophylaxis suppresses LV TC and delays the development of DCM in DMD.

  7. 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

  8. Dynamic characteristic mechanism of atrial septal defect using real-time three-dimensional echocardiography and evaluation of right ventricular functions.

    Science.gov (United States)

    Sharen, Gao-Wa; Zhang, Jun; Qin, Chuan; Lv, Qing

    2017-02-01

    The dynamic characteristics of the area of the atrial septal defect (ASD) were evaluated using the technique of real-time three-dimensional echocardiography (RT 3DE), the potential factors responsible for the dynamic characteristics of the area of ASD were observed, and the overall and local volume and functions of the patients with ASD were measured. RT 3DE was performed on the 27 normal controls and 28 patients with ASD. Based on the three-dimensional data workstations, the area of ASD was measured at P wave vertex, R wave vertex, T wave starting point, and T wave terminal point and in the T-P section. The right atrial volume in the same time phase of the cardiac cycle and the motion displacement distance of the tricuspid annulus in the corresponding period were measured. The measured value of the area of ASD was analyzed. The changes in the right atrial volume and the motion displacement distance of the tricuspid annulus in the normal control group and the ASD group were compared. The right ventricular ejection fractions in the normal control group and the ASD group were compared using the RT 3DE long-axis eight-plane (LA 8-plane) method. Real-time three-dimensional volume imaging was performed in the normal control group and ASD group (n=30). The right ventricular inflow tract, outflow tract, cardiac apex muscular trabecula dilatation, end-systolic volume, overall dilatation, end-systolic volume, and appropriate local and overall ejection fractions in both two groups were measured with the four-dimensional right ventricular quantitative analysis method (4D RVQ) and compared. The overall right ventricular volume and the ejection fraction measured by the LA 8-plane method and 4D RVQ were subjected to a related analysis. Dynamic changes occurred to the area of ASD in the cardiac cycle. The rules for dynamic changes in the area of ASD and the rules for changes in the right atrial volume in the cardiac cycle were consistent. The maximum value of the changes in the

  9. Ventricular Septal Defect: a Literature Review Comunicación interventricular: revisión de la literatura

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    Luis Ángel Osorio Góngora

    2011-12-01

    Full Text Available Congenital heart defects occur in approximately eight of every 1000 live births. Ventricular septal defect occupies a large percentage of them and is the most commonly associated with chromosomal syndromes presenting multifactorial etiology. In 1879, Roger was the first to define the underlying clinical signs. New milestones in its history are marked by Einsenmenger, Dalrymple, Heath and Edwards. In 1979, the use of two-dimensional echocardiography in real time, dramatically improved non-invasive anatomical diagnosis. The main pathophysiologic alteration is part of the injuries that produce volume overload and left to right short-circuit. It is a model for the study of anatomical and physiological relationship in the different injuries that allow communication between the pulmonary and systemic circuits. The imperfections of the sites of origin of the interventricular septum result in different types of ventricular septal defects. The most commonly used classification is the anatomical, from Soto. Homodynamic effects after birth depend on the physiological changes of systemic and pulmonary circulation, as well as on the diameter of the hole. The clinical case depends on the defect and the pulmonary and vascular resistance. The diagnosis is clinical and echocardiographic. Treatment is medical, palliative, surgical and with rehabilitation. Currently, rehabilitation is approached as an essential part of care and therapy.Las cardiopatías congénitas se presentan aproximadamente en 8 de cada 1000 recién nacidos vivos. La comunicación interventricular ocupa un gran porcentaje entre todas, y es la que más se asocia a los síndromes cromosómicos con etiología multifactorial. En 1879 Roger fue el primero en definir los signos clínicos subyacentes. Nuevos hitos en su historia marcan: Einsenmenger, Dalrymple, Heath y Edwards, hasta 1979 en que, con el uso de la ecocardiografía bidimensional en tiempo real, se mejora dramáticamente el diagn

  10. Comparison of left ventricular diastolic function in obstructive hypertrophic cardiomyopathy in patients undergoing percutaneous septal alcohol ablation versus surgical myotomy/myectomy

    Science.gov (United States)

    Sitges, Marta; Shiota, Takahiro; Lever, Harry M.; Qin, Jian Xin; Bauer, Fabrice; Drinko, Jeannie K.; Agler, Deborah A.; Martin, Maureen G.; Greenberg, Neil L.; Smedira, Nicholas G.; Lytle, Bruce W.; Tuzcu, E. Murat; Garcia, Mario J.; Thomas, James D.

    2003-01-01

    Both percutaneous transcoronary alcohol septal reduction (ASR) and surgical myectomy are effective treatments to relieve left ventricular (LV) outflow tract obstruction in obstructive hypertrophic cardiomyopathy (HC). LV diastolic function was assessed by echocardiography in 57 patients with obstructive HC at baseline and 5 +/- 4 months after ASR (n = 37) or surgical myectomy (n = 20). LV outflow tract pressure gradient decreased from 65 +/- 40 to 23 +/- 21 mm Hg (p Patients had a significant improvement in New York Heart Association functional class and in exercise performance. When comparing ASR with myectomy, no difference was found in the degree of change in any parameter of diastolic function. Thus, diastolic function indexes obtained by echocardiography changed after septal reduction interventions in patients with obstructive HC; this change was similar to that after surgical myectomy and ASR.

  11. Association of coronary to left ventricular microfistulae (vessels of Wearn) with atrial septal defect in an adult without cyanotic heart disease.

    Science.gov (United States)

    Hussain, Munem; Roberts, Elved Bryn

    2015-07-02

    Vessels of Wearn are rare findings during coronary angiography in adults. They are known to be associated with forms of cyanotic congenital heart disease in infants but we are not aware of any published cases of association with non-cyanotic left to right shunts in adults. We present the case of a 69-year-old man with angiographically evident vessels of Wearn draining from the left and right coronary arteries into the left ventricle associated with an asymptomatic atrial septal defect. We postulate a developmental phase association between atrial septal maturation and closure of perfusing microchannels from the ventricular cavities to the epicardial coronary arteries on the same spectrum as that which leads to more widespread defects in infants. We also highlight a common medication side effect that might have been mistaken as a manifestation of the congenital anomalies.

  12. Mid-term Follow-up of the Transcatheter Closure of Perimembranous Ventricular Septal Defects in Children Using the Amplatzer

    Directory of Open Access Journals (Sweden)

    Mehdi Ghaderian

    2016-03-01

    Full Text Available Background: The ventricular septal defect (VSD is the most common form of congenital heart defects. The purpose of this study was to evaluate the results of the early complications and mid-term follow-up of the transcatheter closure of the VSD using the Amplatzer VSD Occluder.Methods: Between April 2012 and October 2013, 110 patients underwent the percutaneous closure of the perimembranous VSD. During the procedure, the size and type of the VSD were obtained via ventriculography. A device at least 2 mm larger than the VSD diameter measured via ventriculography was deployed. The size of the VSD, size of the Amplatzer, and device-size to VSD-size ratio were calculated. After the confirmation of the suitable position of the device via echocardiography and left ventriculography, the device was released. Follow-up evaluations were done at discharge as well as at 1, 6, and 12 months and yearly thereafter for the VSD occlusion and complete heart block.Results: The study population comprised 62 females and 48 males. The mean age and weight of the patients at procedure were 4.3 ± 5.6 years (range: 2 to 14 and 14.9 ± 10.8 kg (range: 10 to 43. The average device size was 7.0 ± 2.5 mm (range: 4 to 14. The VSD occlusion rate was 72.8% at the completion of the procedure and rose up to 99.0% during the follow-up. The most serious significant complication was complete atrioventricular block, which was seen in 2 patients. The mean follow-up duration was 10.9 ± 3.6 months.Conclusion: The transcatheter closure of the perimembranous VSD was a safe and effective treatment with excellent closure rates in our study population. This procedure had neither mortality nor serious complications. 

  13. 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.)

  14. Role of hybrid operating room in surgery for the right atrial thrombus, pulmonary thrombi, and ventricular septal rupture after myocardial infarction

    Science.gov (United States)

    Singh, Ajmer; Mehta, Yatin; Parakh, Rajiv; Kohli, Vijay; Trehan, Naresh

    2016-01-01

    Free-floating right heart thrombi are uncommon and need emergency treatment in view of their tendency to dislodge and cause pulmonary embolism. We report a successful surgical management of a patient who had large mobile right atrial thrombus, bilateral pulmonary thrombi, coronary artery disease, and postmyocardial infarction ventricular septal rupture (VSR). The patient underwent coronary angiography, inferior vena cava filter placement, removal of thrombi from the right atrium and pulmonary arteries, repair of VSR, and coronary artery bypass graft surgery in a hybrid operating room. PMID:27716704

  15. Right ventricular electrical and mechanical synchronization by properly timed septal pacing in a patient with right bundle branch block and first degree AV block--a case report.

    Science.gov (United States)

    Siliste, Calin; Suran, Maria-Claudia-Berenice; Margulescu, Andrei-Dumitru; Vinereanu, Dragos

    2015-03-01

    We present a case of near-normalization of the QRS by septal pacing in a patient with dual-chamber pacemaker and underlying complete right bundle branch block and first degree atrioventricular block. The right ventricular mechanical synchronization suggested by the ECG was validated as such by strain echo. To the best of our knowledge, this is the first time it has been shown that the narrowing of the QRS corresponds to mechanical synchronization in a case of this seldom-recognized phenomenon.

  16. Permanent Pacing in Patients with Recurrence of Symptoms and Relapse of Left Ventricular Obstruction at Midcavity Level after Alcohol Septal Ablation

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    Vasil Velchev

    2012-01-01

    Full Text Available Treatment of symptom recurrence after initially successful alcohol septal ablation (ASA in hypertrophic obstructive cardiomyopathy (HOCM when accompanied by relapse of intracavitary left ventricular pressure gradient (LVG is guided by the underlying mechanism. We describe our experience with permanent pacing in three patients with relapse of both LVG and symptoms 7 to 12 months after successful ASA. Even though pressure gradient recurrence was observed at midventricular level, we were able to achieve symptomatic improvement and LVG reduction after right ventricular apex pacing in all three cases. The effect on symptoms was long lasting—the 6-month followup echo-stress tests confirmed good exercise capacity and lack of provocable LVG. We found pacing to be a safe and effective treatment option in this clinical scenario. Based on our overall observations, we propose pacing as a niche treatment for patients with recurrence of LVG at midventricular level after ASA.

  17. Timing in resolution of left heart dilation according to the degree of mitral regurgitation in children with ventricular septal defect after surgical closure

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    Hwa Jin Cho

    2014-01-01

    Full Text Available OBJECTIVE: Children with ventricular septal defects (VSD can have chronic volume overload, which can result in changes of left heart echocardiographic parameters. To evaluate the changes before and after surgical closure, the children were divided into three groups according to the degree of mitral regurgitation (MR, and their echocardiographic characteristics were reviewed at serial follow-up after surgical closure. METHODS: The preoperative, and one-, three-, and 12-month postoperative echocardiographic data of 40 children who underwent surgical closure of VSD were retrospectively reviewed. Left ventricular end-diastolic volume (LVEDV, left ventricular end-diastolic dimension (LVEDD, left ventricular end-systolic dimension (LVESD, mitral valvular characteristics, including degree of MR and mitral valve annulus, and left atrial (LA characteristics, including volume and dimensions, were observed. RESULTS: Preoperative LVEDV, LVEDD, LVESD, mitral valvular annulus, LA volume, and LA dimensions were significantly larger in children with MR. Additionally, there were significant decreases in LVEDV, LVEDD, LA volume, and LA dimensions at one, three, and 12 months postoperatively. The degree of MR also improved to a lower grade after surgical closure of the VSD without additional mitral valve repair. CONCLUSION: The echocardiographic parameters of left heart dilation and MR in children with VSD improved within the first year after surgical closure without additional mitral valve repair. Furthermore, in all of the patients with VSD, regardless of MR, LA dilation was reduced within three months after surgical closure of the VSD; however, LV and mitral valve annular dilatation decreased within 12 months.

  18. Ventricular septal defect

    Science.gov (United States)

    ... insufficiency Arrhythmias Delayed growth Failure to thrive Heart failure - ... MD, Assistant Professor of Medicine, Harvard Medical School, Cardiovascular Disease and Clinical Outcomes Research, Watertown, MA. ...

  19. 室间隔缺损介入封堵治疗失败原因分析%Analysing on failure in transcatheter closure of ventricular septal defect

    Institute of Scientific and Technical Information of China (English)

    李军; 张军; 刘利勋; 朱霆; 刘丽文; 朱永胜; 李梅

    2010-01-01

    目的 分析室间隔缺损介入封堵失败病例,提高术前超声心动图筛选患者的手术成功率.方法 对50例室间隔缺损介入封堵手术失败患者的超声心动图检查结果进行分析.结果 14例术中出现房室传导阻滞,13例术后有残余分流,19例无法建立封堵轨道,2例嵴内置人封堵器后出现较明显的主动脉瓣反流,1例冠心病、1例急性心肌梗死伴室间隔穿孔,以上患者均未能完成手术.结论 应用超声心动图注意观察室间隔缺损的左右室侧大小及周边结构.对年龄较小、缺损复杂或年龄较大合并冠心病的患者,应慎用封堵方法.%Objective To analyse the failure reason in transcatheter closure of ventricular septal defect(VSD) ,in order to improve the succeeding rate of occlusive operation on screening VSD patients with echocardiography. Methods Echocardiographic datas were summarized in 50 cases who were failed in VSD occlusion. Results Atrio-ventricular block was occurred in 14 patients,residual shunt was visualized in 13 cases,transcatheter occlusive track could not be set up in 19 cases,arotic valve regurgitation in two cases were screened obviously after occlusive device was released into the intracristal VSD. One case was suffered from cononary heart disease(CAD) and another one was suffered from acute myocardial infarction with ventricular septal perforation. All these patients did not succeed in closure procedure. Conclusions The defect sizes of left and right sides of ventricular septal, structure close to defect must be considered in selecting patients before operation by echocardiography. Occlusion operation should be strictly chosen in cases who were children,or suffered from complex VSD,or senior patients with CAD.

  20. Amplatzer封堵器治疗儿童伴有假性室隔瘤的膜周部室间隔缺损%Transcatheter closure of aneurysm of perimembrane ventricular septal defect with Amplatzer duct occluder in children

    Institute of Scientific and Technical Information of China (English)

    唐礼江; 高伟; 方崇峰; 林仙方; 江建军; 刘志国

    2004-01-01

    我们应用Amplatzer不对称的膜周部室间隔缺损封堵器介入治疗伴有假性室隔瘤的膜周部室间隔缺损(Aneurysm of perimembrane ventricular septal defect)28例,现报告如下。

  1. A Rare Case of Pulmonary Atresia with Ventricular Septal Defect with a Right Sided Aortic Arch and a Calcified Pulmonary AVM Presenting in an Adult without Cyanosis

    Directory of Open Access Journals (Sweden)

    Devendra V. Kulkarni

    2014-01-01

    Full Text Available Pulmonary atresia with ventricular septal defect (PA-VSD with pulmonary arterial supply arising from the aorta representing large MAPCAs associated with a right sided aortic arch is an uncommon anomaly. Most of the patients succumb to severe respiratory compromise or congestive cardiac failure very early. We report the clinical details and imaging findings of a case of PA-VSD with a right sided aortic arch and a calcified pulmonary arteriovenous malformation (AVM in a 21-year-old postpartum female with no previous episodes of cyanosis who was diagnosed as having a cardiac anomaly on echocardiography when she presented with breathlessness during the 8th month of the pregnancy.

  2. [Is minimal skin incision and partial sternotomy approach for congenital heart defects less invasive?; evaluation of SIRS on ventricular septal defect].

    Science.gov (United States)

    Nishi, H; Nishigaki, K; Kume, Y; Miyamoto, K

    2002-03-01

    Minimally invasive cardiac surgery (MICS) has been developed to offer patients the benefits of open heart operations with limited skin incision. But it is unclear whether this procedure is less invasive. We evaluate postoperative duration of systemic inflammatory response syndrome (SIRS) on ventricular septal defect (VSD). From August 1997 to March 2000, 47 patients VSD underwent total repair by the minimal skin incision and lower partial median sternotomy (MICS group). We compared duration of SIRS between MICS and conventional method group (n = 14) and between early MICS and recent MICS group. We also evaluated the relationship between MICS and postoperative clinical course. Duration of SIRS of MICS group were obviously shorter than that of conventional method group (p SIRS. The results of this study indicate that MICS for VSD may be less invasive method.

  3. Hypothermia During Cardiopulmonary Bypass Increases Need for Inotropic Support but Does Not Impact Inflammation in Children Undergoing Surgical Ventricular Septal Defect Closure.

    Science.gov (United States)

    Schmitt, Katharina Rose Luise; Fedarava, Katsiaryna; Justus, Georgia; Redlin, Mathias; Böttcher, Wolfgang; Delmo Walter, Eva Maria; Hetzer, Roland; Berger, Felix; Miera, Oliver

    2016-05-01

    Minimizing the systemic inflammatory response caused by cardiopulmonary bypass is a major concern. It has been suggested that the perfusion temperature affects the inflammatory response. The aim of this prospective study was to compare the effects of moderate hypothermia (32°C) and normothermia (36°C) during cardiopulmonary bypass on markers of the inflammatory response and clinical outcomes (time on ventilator) after surgical closure of ventricular septal defects. During surgical closure of ventricular septal defects under cardiopulmonary bypass, 20 children (median age 4.9 months, range 2.3-38 months; median weight 7.2 kg, range 5.2-11.7 kg) were randomized to a perfusion temperature of either 32°C (Group 1, n = 10) or 36°C (Group 2, n = 10). The clinical data and blood samples were collected before cardiopulmonary bypass, directly after aortic cross-clamp release, and 4 and 24 h after weaning from cardiopulmonary bypass. Time on ventilation as primary outcome did not differ between the two groups. Other clinical outcome parameters like fluid balance or length of stay in the intensive care were also similar in the two groups. Compared with Group 2, Group 1 needed significantly higher and longer inotropic support (P bypass temperature does not influence time on ventilation or inflammatory marker release. However, in the present study, with a small patient cohort, patients operated under hypothermic bypass needed higher and longer inotropic support. The use of hypothermic cardiopulmonary bypass in infants and children should be approached with care.

  4. LEFT VENTRICULAR INFLOW OBSTRUCTION BY GIANT ATRIAL SEPTAL ANEURYSM IN A NEONATE WITH HYPOPLASTIC RIGHT HEART SYNDROME: CASE REPORT.

    Science.gov (United States)

    Yuko-Jowi, C; Okello, C A

    2013-02-01

    Atrial septal aneurysm remains a rare congenital cardiac malformation. In the neonatal age group it can occur as an isolated cardiac malformation or in association with complex hypoplastic cardiac malformations of the right and left heart. In the adult population most aneurysms have been described in association with stroke. Baby H.N delivered on 10/05/2008 by C/S, was cyanosed at birth with systemic desaturation. Chest X-ray showed oligaemic lung fields while two dimensional echocardiograms showed tricuspid atresia with hypoplastic right ventricle, large secundum atrial septal defect, and highly mobile gigantic aneurysms of the atrial septum obstructing the inflow of the mitral valve and entering the left ventricle in diastole. Surgical intervention was not possible and child died on second day.

  5. Implante de marcapasso ventricular esquerdo no tratamento da miocardiopatia dilatada e bloqueio de ramo esquerdo associado a discinesia de contração septal

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    MOREIRA NETO Francisco Fernandes

    1998-01-01

    Full Text Available O Bloqueio de Ramo (BRE pode apresentar-se como entidade isolada, inclusive em corações normais, nos quais aparentemente não causa importante prejuízo à função ventricular esquerda. Estudos realizados com a indução de BRE pelo implante de marcapasso ventricular direito (1 mostraram, no entanto, efeitos deletérios tanto na função sistólica como diastólica do ventrículo esquerdo, inclusive com aparecimento e/ou acentuação de insuficiência mitral. O BRE pode também vir acompanhado de contração discinética do septo interventricular, que causa um prejuízo adicional à função ventricular esquerda e está, em geral, associada a um quadro de miocardiopatia e insuficiência cardíaca de mau prognóstico (2. Estimulados pelo trabalho de CAZEAU et al. (3, em 1994, estudaram pacientes miocardiopatas e demonstraram que a utilização de marcapasso nas quatro câmaras cardíacas causava agudamente redução da pressão capilar pulmonar e aumento do débito cardíaco, provavelmente pela sincronização de contração ventricular estimulando os dois átrios e ventrículos sincronicamente. Nós investigamos a possibilidade da utilização apenas de estimulação ventricular esquerda através de um marcapasso bicameral, para a tentativa de sicronização de contração do ventrículo esquerdo e septo interventricular em pacientes portadores de miocardiopatia, bloqueio de ramo esquerdo e discinesia septal. O procedimento foi realizado em 3 pacientes que foram submetidos no pós-operatório, com o MP ligado e desligado, a estudo ecocardiográfico e cintilográfico para a análise da função ventricular esquerda através do cálculo da fração de ejeção. Os resultados mostraram importante melhora deste parâmetro: no primeiro caso indo de 17% para 25%; no segundo de 13% para 30% e no terceiro de 13% para 27%. A discinesia praticamente desapareceu em 2 dos casos e foi quantificada como +/4 no primeiro caso. A classe funcional e a insufici

  6. First successful repair of an aortico-to-right ventricular tunnel (ARVT) in d-transposition of the great arteries with aortic valve atresia and ventricular septal defect.

    Science.gov (United States)

    Wagner, Robert; Vollroth, Marcel; Daehnert, Ingo; Kostelka, Martin

    2015-04-01

    The presented case reports on the first successful complex biventricular repair in a neonate with an aortico-to-right ventricular tunnel and dextrotransposition of the great arteries complicated by aortic atresia.

  7. Postoperative Residual Shunt After Repair of Ventricular Septal Defect Among Children%小儿室间隔缺损术后残余漏

    Institute of Scientific and Technical Information of China (English)

    蒋雄刚

    1995-01-01

    8例小儿室间隔缺损修补术后残余漏再次手术病例分析:残余漏表现为5例术后残留心前区Ⅲ级以上收缩期杂音,7例血红蛋白尿,2例右心衰,8例超声心动图(UCG)证实心室水平残余左向右分流.均于术后2~112天行残余漏修补.术中见残余漏原因为不同部位缝针处组织撕裂6例,修补不全2例.8例均经带垫片褥式缝合加固,7例痊愈,1例再次发生残余漏.本文对残余漏特点、诊断、手术适应证及时机进行了讨论.%Eight cases of postoperative residual shunt after repair of venticular septal defect (VSD) were analysed in this paper.Their clinical features disclosed:hemofiubinuria in 7 cases,cardiac murmur in 5 and residual shunt signs on echocardiograph in 8.In the operation for residual shunt,it was found:the tear in septal leaflet in aneurysm-like VSD in 2 cases,the tear in septal leaflet of tricuspid in 4,infundibular muscular 2 and residual triangle defect under the patch repair in VSD 2.Residual shunt in all the 8 cases were repaired with mattress suture under cardiopulmonary bypass,seven patients recovered without hemogiobinuria or cardiac murmur,but one had residual shunt again and cerebral insufficiency,which was improved by medical therapy.The harmfulness,the diagnosis and prevention of postoperative residual shunt,the operative indication,optimal time and surgical procedures were discussed.

  8. Heterogeneous abnormalities of in-vivo left ventricular calcium influx and function in mouse models of muscular dystrophy cardiomyopathy

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    Greally Elizabeth

    2013-01-01

    Full Text Available Abstract Background Manganese-enhanced cardiovascular magnetic resonance (MECMR can non-invasively assess myocardial calcium influx, and calcium levels are known to be elevated in muscular dystrophy cardiomyopathy based on cellular studies. Methods Left ventricular functional studies and MECMR were performed in mdx mice (model of Duchenne Muscular Dystrophy, 24 and 40 weeks and Sgcd−/− mice (Limb Girdle Muscular Dystrophy 2 F, 16 and 32 weeks, compared to wild type controls (C57Bl/10, WT. Results Both models had left ventricular hypertrophy at the later age compared to WT, though the mdx mice had reduced stroke volumes and the Sgcd−/− mice increased heart rate and cardiac index. Especially at the younger ages, MECMR was significantly elevated in both models (both Pmdx mice (PSgcd−/− mice (PSgcd−/− mice had increased heart rates, to determine the role of heart rate in MECMR we studied the hyperpolarization-activated cyclic nucleotide-gated channel inhibitor ZD 7288 which selectively reduces heart rate. This reduced heart rate and MECMR in all mouse groups. However, when looking at the time course of reduction of MECMR in the Sgcd−/− mice at up to 5 minutes of the manganese infusion when heart rates were matched to the WT mice, MECMR was still significantly elevated in the Sgcd−/− mice (P Conclusions Despite both mouse models exhibiting increased in-vivo calcium influx at an early stage in the development of the cardiomyopathy before left ventricular hypertrophy, there are distinct phenotypical differences between the 2 models in terms of heart rates, hemodynamics and responses to calcium channel inhibitors.

  9. Association of Right Ventricular Pressure and Volume Overload with Non-Ischemic Septal Fibrosis on Cardiac Magnetic Resonance

    OpenAIRE

    Jiwon Kim; Medicherla, Chaitanya B.; Ma, Claudia L.; Attila Feher; Nina Kukar; Alexi Geevarghese; Parag Goyal; Evelyn Horn; Devereux, Richard B; Weinsaft, Jonathan W.

    2016-01-01

    Background 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. Methods and Results The population comprised patients with RV dysfunction (EF

  10. 供体先天性室间隔缺损心脏移植1例%Donor congenital ventricular septal defect heart transplantation in one case

    Institute of Scientific and Technical Information of China (English)

    仲崇俊; 王春生; 薛群; 陆晨希; 许一鸣; 高增栋

    2008-01-01

    本例心脏移植受者为终末期扩张性心肌病患者,供体为先天性室间隔缺损患者,进行原位双腔心脏移植,随访4年结果满意.在当前供体紧缺的情况下,对于一些存在先天性缺陷的供体,适当处理后应用也能取得满意的临床效果.%The receptor of the heart transplantation was a patient with terminal dilated cardiomyopathy, the donor was a patient with congenital ventricular septal defect, in situ double-chamber heart transplantation was performed, and the result of the four-year follow-up was satisfactory. At present, donor is deficient,and those donors with congenital defect can also obtain satisfactory clinical application effects after appropriate handling.

  11. Midterm results of diagnostic treatment and repair strategy in older patients presenting with nonrestrictive ventricular septal defect and severe pulmonary artery hypertension

    Institute of Scientific and Technical Information of China (English)

    Liu Aijun; Li Zhiqiang; Li Xiaofeng; Fan Xiangming; Su Junwu; Zhang Jing; He Yan

    2014-01-01

    Background Congenital heart disease with severe pulmonary arterial hypertension (SPAH),previously thought to have irreversible pulmonary vascular disease (PVD),has been recently successfully corrected using diagnostic treatment and repair strategy by our surgery team.This study aimed to evaluate the midterm results of a selected cohort of older patients with nonrestrictive ventricular septal defect (VSD) and SPAH using diagnostic treatment and repair strategy.Methods The records of 56 patients older than six years with nonrestrictive VSD and SPAH undergoing diagnostic treatment and repair strategy from 2006 to 2012 were retrospectively reviewed.All patients received advanced pulmonary arterial hypertension (PAH) therapy and radical repairs were performed when transcutaneous oxygen saturation (SPO2) increased up to 93%.Results There were no operative deaths.SPO2 and baseline six-minute walk test (SMWT) distance of all selected patients increased significantly and mean pulmonary artery pressure (MPAP) regressed significantly after diagnostic treatment and at late follow-up (P <0.01).The incidence of late postoperative PAH was seen in six (10.7%) patients and by Logistic regression analysis,early postoperative PAH was an independent risk factor related to late postoperative PAH.Conclusions Diagnostic treatment and repair strategy was effective and safe for treatment of nonrestrictive VSD and SPAH and the midterm results were excellent.Diagnostic treatment strategy was effective in assessing the reversibility of SPAH in older patients associated with nonrestrictive VSD and the PVD in these selective patients is generally reversible.

  12. Transcatheter closure of ventricular septal defect in patients with aortic valve prolapse and mild aortic regurgitation:feasibility and preliminary outcome

    Institute of Scientific and Technical Information of China (English)

    Guan-Liang Chen; Hai-Tao Li; Hai-Rong Li; Zhi-Wei Zhang

    2015-01-01

    Objective: To evaluate the feasibility, safety and efficacy of transcatheter closure of ventricular septal defect (VSD) in patients with aortic valve prolapse (AVP) and mild aortic regurgitation (AR). Methods: Between January 2008 and July 2014, transcatheter closure of VSD was attempted in 65 patients. Results: The total intermediate closure successful rate in all subjects was 96.9%. During the perioperative period, no death, major bleeding, pericardial tamponade, occluder dislodgement, residual shunt or hemolysis occurred. Two procedures had been forced to suspend due to significant aggregation of device related aortic regurgitation, three cases of transient complete left bundle branch block occurred but did not sustain. At 1-year follow-up, no patients had residual shunts and complications. Furthermore, grade of residual AR were relieved in 61.9% (39/63) cases and degree of AVP were ameliorated in 36.5% (23/63) patients; Conclusions: Transcatheter closure VSD in selected patients with AVP and mild AR is technically feasible and highly effective. Long term safety and efficacy needs to be assessed.

  13. Congenital Left Ventricular Diverticulum Associated with ASD, VSD, and Epigastric Hernia

    Directory of Open Access Journals (Sweden)

    Seyed Mohammad Dalili

    2008-12-01

    Full Text Available Congenital left ventricular diverticulum is a rare cardiac malformation. Two categories of congenital ventricular diverticulum have been identified with regard to their localization: apical and non-apical. Apical diverticula are always associated with midline thoraco-abdominal defects and other heart malformations. Non-apical diverticula are always isolated defects. Diagnosis is established by imaging studies such as echocardiography, magnetic resonance imaging, or left ventricular angiography. Mode of treatment has to be individually tailored and depends on clinical presentation, accompanying abnormalities, and possible complications. We report a 10-month-old girl with left ventricular apical diverticulum, large atrial septal defect, two small muscular ventricular septal defects, and pulmonary hypertension, associated with epigastric hernia. This patient underwent total surgical repair for intra-cardiac defects as well as diverticular resection.

  14. 胎儿先天性室间隔缺损的染色体核型分析%Study of chromosome karyotype in congenital ventricular septal defect of fetus

    Institute of Scientific and Technical Information of China (English)

    高金梅; 关云萍; 项宇识; 闫峰; 李冬梅

    2013-01-01

    目的:通过产前诊断分析先天性胎儿室间隔缺损与染色体核型的相关性。方法:回顾性分析我院产前超声诊断胎儿发育异常并进行脐静脉血穿刺染色体核型分析的患者387例,对其中单纯室间隔缺损或室间隔缺损伴发其他结构异常的病例74例进行分析。结果:74例病例中,脐血穿刺血细胞培养成功72例,培养失败2例。72例病例中,产前超声诊断单纯室间隔缺损12例,染色体核型结果均正常;产前超声诊断室间隔缺损伴发其他结构异常60例,其中染色体核型异常者17例(18-三8例、13-三体3例、21-三体4例、其他2例),染色体核型正常者43例。结论:胎儿心脏室间隔缺损伴发其他结构异常时较单纯室间隔缺损出现染色体异常的几率大。%Objective:To analyse the correlations between congenital ventricular septal defects and fetal karyotype through prenatal diagnosis.Methods:A retrospective analysis of 387 cases who diagnosed with fetal abnormalities and carried on umbilical vein puncture for karyotype analysis in our hospital through prenatal ultrasound diagnosis. among which, 74 cases with the ventricular septal defect or ventricular septal defect simply associated with other structural abnormalities were analyzed.Results:In the 74 cases, 72 cases were successful y cultured in the umbilical cord blood cells, 2 cases were cultured failure. 12 cases were diagnosed with ventricular septal defect simply in prenatal ultrasound diagnosis in the 72 cases, karyotype of the 12 cases were al normal; 60 cases were diagnosed with ventricular septal defect associated with other structural abnormalities in prenatal ultrasound diagnosisin the 72 cases, in which chromosome abnormalities were 17 cases (8 cases of 18-trisomy syndrome;3 cases of 13-trisomy syndrom;4 cases of 21-trisomy syndrom;2 cases of other chromosomal abnormalities), normal karyotype were 43 cases.Conclusion:Fetal cardiac

  15. Interventianal therapy of acute myocardial infarction complicated with ventricular septal rapture%急性心肌梗死并发室间隔穿孔六例介入治疗

    Institute of Scientific and Technical Information of China (English)

    唐学杰; 韩贞普; 李仕华; 夏碧华; 刘贤铭

    2009-01-01

    目的 评价Amplatzer肌部室间隔封堵器封堵室间隔穿孔(VSR)和同期经皮腔内冠状动脉成形术(PTCA)及冠脉支架术联合介入方法治疗急性心肌梗死(AMI)并发VSR的可行性及安全性.方法 6例患者术前均经超声心动图检查诊断为AMI并发心尖部VSR,出现心原性休克后均予主动脉球囊反搏(IABP)、机械通气支持.于3周后行室间隔封堵术,同期完成PTCA及支架术.结果 6例子Amplatzer肌部室间隔封堵器成功封堵VSR,2例有少量残余漏;同期冠状动脉造影3例患者为前降支中段及回旋支局限性狭窄,分别予植入1~2枚支架,另外3例患者为多支多段狭窄,未予以植入支架;3例并行支架的患者存活出院.结论 应用Amplatzer肌部室间隔封堵器封堵VSR同期行PTCA及冠状动脉支架术治疗AMI并发VSR是安全可行的.%Objective To evaluate the feasibility and safety of the treatment for ventricular septal rupture (VSR) by transcatheter interventional therapy coordinated with percutaneous coronary intervention (PCI) or percutaneous transluminal coronary angioplasty (PTCA).Methods Six patients who suffered from cardiogenic shock due to acute myocardial infarction (AMI) and VSR were given intra-aortic balloon pump (IABP) and mechanical ventilation support. After three weeks, they underwent transcatheter closure of ventricular septal rupture with Amplatzer ventricular septal occluder, and at the same time PCI and PTCA were performed. Results All the patients successfully underwent ventricular septal defect occlusion, including two cases of residual shunt. Segmental stenosis of the middle of anterior descending branch and circumflex artery were noted in three patients, and then stent implantation was used. The other three cases had multi-vessel lesion but were without stent implantation. All the patients with stent implantation survived and discharged. Conclusions It is safe and feasible to apply transcatheter closure of ventricular

  16. 隔瓣后室间隔缺损并假性室隔瘤的手术治疗%Surgical Treatment of Ventricular Septal Defect With False Aneurysm of Membrane Septum

    Institute of Scientific and Technical Information of China (English)

    毛志福; 高尚志; 陈俊堂; 姚震; 涂仲凡

    1996-01-01

    Objective:To introduce the procedure for better exposure of the ventricular septal defect(VSD)from fused septal leaflet pouch.Method:Among the 28 cases,the septal leaflet of tricuspid valve in 22 cases was incised and the papillary muscle in 6 cases was dissected to expose the rim of the hidden VSD for proper anatomic repair.Results:There was no death,no significant intraoperative or postoperative complications and tricuspid valve dysfunction.Conclusion: Opening the fused septal leaflet pouch is safe and beneficial to a better exposure and repair of the hidden defect.%采用隔瓣切开或圆锥乳头肌切断术治疗假性室隔瘤型VSD并予以评价.全组共28例,其中22例采用隔瓣切开,6例采用圆锥乳头肌切断片显露缺损,再以涤纶片或自体心包片修补VSD.全组无手术死亡,无重大并发症及三尖瓣功能不全.此法安全,有利于VSD的显露和对VSD准确可靠的修补,值得推荐使用.

  17. 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.

  18. Down 综合征患儿室间隔缺损的手术治疗%Surgical Treatment of Ventricular Septal Defect in Children with Down Syndrome

    Institute of Scientific and Technical Information of China (English)

    吴士强

    2014-01-01

    目的:总结 Down 综合征患儿室间隔缺损的外科治疗经验。方法48例 Down 综合征患儿室间隔缺损在全身麻醉低温体外循环下行一期根治术。术前对于合并肺部感染、心功能不全、肺动脉高压者,给予有效的抗感染、强心及利尿剂、降肺动脉压等治疗;加强静脉营养,提高机体免疫力。术中提高手术技巧,操作轻柔,选择合适的手术切口及修补材料,避免发生传导阻滞等并发症。术后入 CICU,加强呼吸道管理,合理使用呼吸机;合理使用血管活性药物,维持循环系统的稳定;合理使用抗生素,减少肺部感染的发生。结果48例中无1例死亡,并发呼吸机相关性肺炎10例,心律失常2例,气胸1例,心包填塞1例,低心排1例。术后随访6个月至6年,生长发育好,无死亡。结论Down 综合征室间隔缺损患儿应尽早手术,选择适当的手术时机,合理有效的围术期处理,可提高手术成功率,延长患儿寿命。%Objective To summarize the experience with the surgical treatment of ventricular septal defect in children with Down syndrome.Methods Forty-eight children with Down syn-drome underwent one-stage radical operation under general anesthesia during hypothermic car-diopulmonary bypass.Patients with lung infection,heart failure,and pulmonary hypertension were given anti-infection,cardiotonics and diuretics,and anti-pulmonary hypertension,respectively.In addition,intravenous nutrition was strengthened and immunity was improved in all patients.Dur-ing operation,gentle operation technique was carried out and appropriate surgical incision and re-pair material were selected to avoid conduction block and other complications.After operation,pa-tients were admitted to CICU,and respiratory tract management and rational use of breathing ma-chine were strengthened.Moreover,vasoactive drugs and antibiotics were rationally used to main-tain the stability of

  19. Effects of percutaneous transluminal septal myocardial ablation for obstructive hypertrophic cardiomyopathy on systolic and diastolic left ventricular function assessed by pressure-volume loops.

    Science.gov (United States)

    Meliga, Emanuele; Steendijk, Paul; Valgimigli, Marco; Ten Cate, Folkert J; Serruys, Patrick W

    2008-04-15

    The aim of the present study was to determine the long-term effects of percutaneous transluminal septal myocardial ablation (PTSMA) on systolic and diastolic left ventricular (LV) functions in patients with obstructive hypertrophic cardiomyopathy (HC). Ten consecutive patients with symptomatic HC despite optimal medical treatment were referred for PTSMA at our center. LV systolic and diastolic functions were assessed by online LV pressure-volume loops obtained by conductance catheter at baseline and at 6 months after the procedure. At follow-up, the mean gradients at rest and after extrasystole were significantly decreased compared with baseline (88 +/- 29 to 21 +/- 11 mm Hg and 130 +/- 50 to 35 +/- 22 mm Hg, respectively, p <0.01 for the 2 comparisons). End-systolic and end-diastolic pressures significantly decreased (p <0.01), whereas end-systolic and end-diastolic LV volumes significantly increased (p <0.01 for the 2 comparisons). Cardiac output and stroke volume were unchanged, as were ejection fraction (p = 0.25) and maximum dP/dt (p = 0.13). The slope of the end-systolic pressure-volume relation was not decreased, indicating a preserved contractility. The relaxation constant time, end-diastolic stiffness, projected volume of the end-diastolic pressure-volume relation at 30 mm Hg, and diastolic stiffness constant showed a significant improvement of active and passive myocardial diastolic properties. In conclusion, PTSMA is an effective method in the treatment of symptomatic patients with HC. At 6-month follow-up, the LV-aortic gradient was decreased and active and passive LV diastolic properties were increased. Myocardial contractility was not decreased and general hemodynamics was maintained.

  20. Achievements and Limitations of a Strategy of Rehabilitation of Native Pulmonary Vessels in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries.

    Science.gov (United States)

    Soquet, Jerome; Liava'a, Matthew; Eastaugh, Lucas; Konstantinov, Igor E; Brink, Johann; Brizard, Christian P; d'Udekem, Yves

    2017-05-01

    A strategy of rehabilitation for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) comprises repetitive shunting and patching procedures of the central pulmonary arteries. We wanted to determine the feasibility and limitations of a strategy of rehabilitation. The outcomes of 37 consecutive patients operated from June 2003 to December 2014 for PA/VSD/MAPCAs were reviewed. The patients were directed to a rehabilitation strategy, except when they presented in heart failure with very large collaterals. Four patients with very large MAPCAs underwent a one-stage repair with unifocalization of collateral vessels at a median age of 8.6 months. There was no mortality in this group after a median follow-up of 4.6 years. Following a strategy of staged rehabilitation, 33 patients had 2.01 ± 0.9 procedures before repair. Median age at primary shunting was 3.3 weeks (0.4 to 31.9 weeks). Repair rate was 73% (22 patients), at a median age of 1.7 years. Three patients (10%) were left palliated and 3 patients (10%) died. Median follow-up in this group was 4.5 years. Complementary procedures to the rehabilitation strategy consisted in pulmonary artery reconstruction in 25 patients (76%) and MAPCAs ligation in 7 patients (21%). Pulmonary balloon angioplasty was required in 12 patients (36%) and MAPCAs coil occlusion in 8 patients (24%). A strategy of rehabilitation can be implemented in almost 90% of the cases, with a low mortality rate. Following this strategy, 73% of the patients can be successfully repaired. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  1. Postprocedural Outcomes and Risk Factors for Arrhythmias Following Transcatheter Closure of Congenital Perimembranous Ventricular Septal Defect: A Single-center Retrospective Study

    Science.gov (United States)

    Zhao, Li-Jian; Han, Bo; Zhang, Jian-Jun; Yi, Ying-Chun; Jiang, Dian-Dong; Lyu, Jian-Li

    2017-01-01

    Background: Currently, transcatheter closure of perimembranous ventricular septal defect (pmVSD) is a widely accepted therapeutic modality. However, arrhythmias, especially postprocedural heart blocks, are a concern and outcomes are not very clear. This study explored the outcomes and risk factors of arrhythmias associated with transcatheter device closure of pmVSD. Methods: A total of 395 patients diagnosed with pmVSD who successfully underwent transcatheter intervention between January 2010 and December 2015 in our center were retrospectively reviewed. Electrocardiographic data before and after the procedure were collected and analyzed. We first evaluated the potential risk factors including gender, age, weight, inlet and outlet diameters of defect, subaortic rim length, occluder size, corrected occluder size into body surface area, fluoroscopy time, presence of aneurysm, and deployment position. We compared the potential risk factors between arrhythmia and nonarrhythmia groups using univariate analysis, followed by logistic analysis for independent risk factors. Results: Various arrhythmias were detected in 95 cases (24.1%) following transcatheter closure procedure. Logistic regression analysis revealed that eccentric (odds ratio [OR] 2.9, 95% confidence interval [CI]: 1.2–7.2) and large occluders (OR 2.0, 95% CI: 1.6–2.5), as well as long fluoroscopy time (OR 1.1, 95% CI: 1.1–1.2), were correlated with postprocedural arrhythmia. During 35.5 months (range: 9–80 months) of follow-up, most of the patients (74 out of 95) reverted to normal heart rhythm. Conclusions: The mid-term outcome of patients with arrhythmias after transcatheter closure of pmVSD was satisfactory as most of the patients recovered normal rhythm. Eccentric, large device and long fluoroscopy time increase the risk of arrhythmias after transcatheter closure of pmVSD. PMID:28229981

  2. Complete resolution of chronic pericardial effusion with an intensive course of inhaled iloprost in an adult patient with unrepaired ventricular septal defect, and life-threatening severe pulmonary arterial hypertension.

    Science.gov (United States)

    Alzahrani, Ahmed M; Alqahtani, Abdullah S; Alnajashi, Khalid S; Idrees, Majdy M

    2014-10-01

    A 38-year-old male was diagnosed with unrepaired ventricular septal defect associated with severe pulmonary arterial hypertension, cyanosis, and significant exercise intolerance. His echocardiogram showed right ventricular dysfunction and moderate pericardial effusion with no signs of cardiac tamponade. He was treated with an intensive course of inhaled iloprost and sildenafil. He showed a dramatic clinical response; his saturation went up from 60% on admission to 90% on minimal oxygen with significant improvement in his symptoms and signs of heart failure and total resolution of pericardial effusion. On follow up 3 and 6 weeks later, he was stable and could walk 360 meters in a 6 minutes walk test with disappearance of pericardial effusion. With unavailability of intravenous prostacyclin, we have shown in this case that intensive administration of inhaled iloprost could be used intensively as a rescue therapy in severe cases of pulmonary arterial hypertension with excellent results. 

  3. Relationships between left heart chamber dilatation on echocardiography and left-to-right ventricle shunting quantified by cardiac catheterization in children with ventricular septal defects.

    Science.gov (United States)

    Gokalp, Selman; Guler Eroglu, Ayse; Saltik, Levent; Koca, Bulent

    2014-04-01

    Left atrium and/or left ventricle dilatation on echocardiography is considered to be an indication for closure of ventricular septal defects (VSD). No study has addressed the accuracy of using dilated left heart chambers when defining significant left-to-right shunting quantified by cardiac catheterization in isolated small or moderate VSDs. In this study, the relation between dilated left heart chambers, measured by echocardiography, and left-to-right ventricle shunting, quantified by cardiac catheterization, was evaluated in patients with isolated VSD. The medical records of all patients with isolated VSD who had undergone catheterization from 1996 to 2010 were examined retrospectively. Normative data for left heart chambers adjusted for body weight (BW) and body surface area (BSA) were used. The pulmonary-to-systemic flow ratio (Qp:Qs) was calculated by an oximetry technique. A total of 115 patients (mean age 7.3 ± 5 years) fulfilled the inclusion criteria. There was a statistically significant difference in terms of Qp:Qs between the patient groups with normal and dilated left heart chambers, when adjusted for BW and BSA (p = 0.001 and p = 0.002, respectively). But the relationships between Qp:Qs and left heart chamber sizes on echocardiography were not strong enough to be useful for making surgical decisions, as left heart chamber dilatation was not significantly associated with Qp:Qs ≥ 2 (p = 0.349 when adjusted for BW, p = 0.107 when adjusted for BSA). Left heart chamber dilatation was significantly associated with Qp:Qs ≥ 1.5 only when it was adjusted for BSA (for BW p = 0.022, for BSA p = 0.006). As a result, left heart chamber dilatation measured by echocardiography does not show significant left-to-right ventricle shunting, as quantified by catheterization. We still advocate that catheter angiography should be undertaken when left heart chambers are dilated in echocardiography in order to make decisions about closing small- to moderate-sized VSD.

  4. Re-operation of residual ventricular septal defect in 12 cases%残余室间隔缺损的再手术治疗12例

    Institute of Scientific and Technical Information of China (English)

    胡晓峰; 周运乾; 曹庆亨; 邱兆崑

    2001-01-01

    Objective To analyze the causation, surgical indications and pivotal points in reoperation of residual ventricular septal defect (VSD). Methods Collecting the clinical data of 12 cases of reoperated residual VSD in this hospital from 1988 to 1998 and investigating into insight of their causation, surgical indications and keys elements of reoperation in residual VSD. Results This group consisted of 12 patients of whom 8 were operated in this hospital from 1977 to 1997, and 4 were operated in other hospital. The re-operative rate of VSD in our hospital was 0.18 %.The defects in the first operation were 8 cases of simple VSD and 3 tetralogy of Fallot. The mean interval between the two operations was 4.1 years (5 months-12 years). Residual shunting in 10 patients were adjacent to the base of anterior and septal leaflets of tricuspid valve. The mean longitudinal diameter of residual VSD was 1.2 cm(0.3~2.5 cm).Three patients died after reoperation. Causes of death included low cardiac output syndrome, cardiac arrhythmia and cardiac tamponade. Conclusion 1. The common location of residual VSD is around the base of anterior and septal leaflets of tricuspid valve, this area involves some important structures like aortic valve and AV bundle which are not easily exposed. 2. Adequate exposure of entire VSD margin is the key element to avoid leakage by laceration. 3. Early re-operation is required for all residual VSD with diameter above 0.5 cm. 4. The key points of reoperation are timely operation, atrial incisional approach, patch repairment and careful hemostasis.%目的分析残余室间隔缺损(VSD)的发生原因、防治措施、手术指征和手术要点。方法对12例施行残余VSD再手术治疗患者的发生原因、预防措施、手术指征和手术要点进行分析。结果 12例中8例首次手术于1977年至1997年在我院施行,4例在外院施行,我院VSD再手术率为0.18%。首次手术时单纯VSD 9例,法洛四联症(TOF)3

  5. Ventricular Septal Defect (For Teens)

    Science.gov (United States)

    ... now, either with cardiac catheterization or heart surgery. Heart Surgery The surgeon makes an incision in the chest ... The first few days at home after VSD surgery, your doctor will probably tell you to get lots of ... Catheterization Certain types of VSDs may be closed ...

  6. 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.

  7. 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

    Directory of Open Access Journals (Sweden)

    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

  8. Glue septal ablation: A promising alternative to alcohol septal ablation

    Directory of Open Access Journals (Sweden)

    Sercan Okutucu

    2016-03-01

    Full Text Available Hypertrophic cardiomyopathy (HCM is defined as myocardial hypertrophy in the absence of another cardiac or systemic disease capable of producing the magnitude of present hypertrophy. In about 70% of patients with HCM, there is left ventricular outflow tract (LVOT obstruction (LVOTO and this is known as obstructive type of hypertrophic cardiomyopathy (HOCM. Cases refractory to medical treatment have had two options either surgical septal myectomy or alcohol septal ablation (ASA to alleviate LVOT gradient. ASA may cause some life-threatening complications including conduction disturbances and complete heart block, hemodynamic compromise, ventricular arrhythmias, distant and massive myocardial necrosis. Glue septal ablation (GSA is a promising technique for the treatment of HOCM. Glue seems to be superior to alcohol due to some intrinsic advantageous properties of glue such as immediate polymerization which prevents the leak into the left anterior descending coronary artery and it is particularly useful in patients with collaterals to the right coronary artery in whom alcohol ablation is contraindicated. In our experience, GSA is effective and also a safe technique without significant complications. GSA decreases LVOT gradient immediately after the procedure and this reduction persists during 12 months of follow-up. It improves New York Heart Association functional capacity and decrease interventricular septal wall thickness. Further studies are needed in order to assess the long-term efficacy and safety of this technique.

  9. Surgical treatment of interrupted aortic arch associated with ventricular septal defect and patent ductus arteriosus in patients over one year of age

    Institute of Scientific and Technical Information of China (English)

    Li Zhiqiang; Li Bin; Fan Xiangming; Su Junwu; Zhang Jing; He Yan; Liu Yinglong

    2014-01-01

    Background 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.Methods 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.Results 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

  10. Perioperative management of the ventricular septal defect with moderate or severe pulmonary hypertension%室间隔缺损并中重度肺动脉高压患者围手术期处理

    Institute of Scientific and Technical Information of China (English)

    李占伟; 程兆云; 张国报; 权晓强; 乔刚; 刘传振

    2011-01-01

    目的:探讨室间隔缺损并中重度肺动脉高压患者的手术时机及围手术期处理.方法:28例室间隔缺损并中重度肺动脉高压患者,术前给予吸氧、前列腺素E1等药物治疗后均行室间隔缺损修补术,部分重度肺动脉高压患者应用单向活瓣补片修补室间隔缺损.结果:术后死亡2例,其中1例为低心排血量综合征并肺动脉高压危象,1例为低心排血量综合征并肺部感染.余26例安全度过围手术期并痊愈出院.结论:选择手术时机,术中完善手术修复和恰当的围术期处理是室间隔缺损并中重度肺动脉高压患者治疗的关键.%Objective To study the surgical opportunity and perioperative management of the ventricular septal defect with moderate or severe pulmonary hypertension. Methods After medicinal therapy, 28 cases of ventricular septal defect received operation. One-way valve patch and further myocardial protection were applied on patients with severe pulmonary hypertension. Results Two patients died after the operation, in which one died of low cardiac output syndrome and pulmonary hypertension crisis, and the other died of low cardiac output syndrome and pulmonary infection. Twenty-six patients went through perioperative period smoothly. Conclusion Proper choice of surgical opportunity, complete surgical repair and good perioperative management are the keys to the successful operation of ventricular septal defect with moderate or severe pulmonary hypertension.

  11. 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

    Directory of Open Access Journals (Sweden)

    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.

  12. 室间隔缺损介入术后并发三度房室阻滞%Third-degree atrioventricular block after transcatheter closure of ventricular septal defect

    Institute of Scientific and Technical Information of China (English)

    白元; 宗刚军; 秦永文

    2009-01-01

    目的 分析国内室间隔缺损患者介入封堵术后三度房室阻滞的发生特点和原因.方法通过中国生物医学文献数据库和清华同方医学期刊数据库文献检索系统,以"室间隔缺损"和"传导"为任意字段进行检索,在纳入的文献中,重点对患者年龄、性别、术后三度房室阻滞的发生时间、恢复时间、处理措施等进行总结分析.结果 总计纳入23篇文献,共报道了73例术后出现三度房室阻滞的患者,女性和儿童多见.88%的三度房室阻滞发生于术后3~7 d.80%的病例均在症状出现后的7~15 d恢复窦性心律.有3例患者植入了永久起搏器.结论 室间隔缺损介入术后的三度房室阻滞有其发生发展的规律,多数为一过性,需植入永久性起搏器的患者极少.%Objective To observe clinical characteristics of third-degree atrioventricular block (AVB Ⅲ) after transcatheter closure of ventricular septal defect in China. Methods All the data were collected from the Chinese Bio-Medical Data-base and the Chinese Journal Full-text Data-base. The search term (all fields) used was "ventricular septal defect and conduction". The major items included age, sex, size of ven-tricular septal defect and the features of postoperative AVB Ⅲ. Results Twenty-three articles were reviewed in which the emphasis of analysis was conduction disorders after transcatheter closure of ventricular septal de-fect. There were 73 cases of postoperative AVB Ⅲ of the total 73 patients, most of them were women and chil-dren. In 88% of patients the AVB Ⅲ occurred in the first 3~7 day after surgery. AVB Ⅲ reversed during the 7~15 day after the onset of it in 80% of patients. Three patients required permanent pacemaker implantation due to irreversible AVB Ⅲ. Conclusion Postoperative AVB Ⅲ was transient in most of cases. The require-ment of permanent pacemaker implantation after closure is a rare but serious complication.

  13. Investigation of Influence Factors and Nutritional Status in Ventricular Septal Defect Children before Surgery%室间隔缺损患儿术前的营养状况及其影响因素

    Institute of Scientific and Technical Information of China (English)

    焦文娟; 陈京立; 李庆印

    2014-01-01

    探讨室间隔缺损患儿术前的营养状况及其可能的影响因素.方法采用便利抽样法,选取2011年5-12月在北京阜外医院就诊并接受室间隔缺损修补术的患儿143例,采用人体测量法、访谈法和问卷法调查患儿营养不良发生的情况及其影响因素.结果(1)室间隔缺损修补术前患儿急性营养不良发生率为44.1%,慢性营养不良发生率为21.0%,总营养不良发生率为50.3%;(2)Logistic回归分析结果显示,患儿出生体质量、术前肺动脉压力水平、能量摄入情况及患儿母亲是否通过网络或图书获取营养知识是影响患儿术前营养状况的独立预测因素.结论室间隔缺损修补术前患儿营养不良发生率高,护理人员应全面评估患儿的出生体质量、术前肺动脉压力水平、能量摄入情况等,并有针对性地向高危患儿家属进行相关宣教,以改善患儿的营养状况,提高手术成功率.%Objective To investigate the nutritional status in ventricular septal defect children before surgery and to analyze its influence factors.Methods By convenience sampling,totally 1 4 3 ventricular septal defect children were selected and evaluated by using body measurement,interviews and ques-tionnaires to investigate the incidence of malnutrition and its influencing factors.Results ①The inci-dence of acute malnutrition and chronic malnutrition were 4 4 .1%,2 1%.The incidence of malnutrition was 5 0 .3%.②Logistic analysis showed that the influence factors of nutritional status in ventricular septal defect infants were birth weight,pulmonary artery pressures,dietary intake and whether the mother required nutritional information through e-books was the independent predictive factor.Conclu-sion There is a high incidence of malnutrition in ventricular septal defect children before surgery. Nurses should comprehensively evaluate the birth weight,pulmonary artery pressures,dietary intake before

  14. Septal rhinoscleroma

    Directory of Open Access Journals (Sweden)

    Shoeib Mohamed

    2010-01-01

    Full Text Available Rhinoscleroma is a chronic granulomatous condition of the nose and other structures of the upper respiratory tract. Infection by the bacterium Klebsiella rhinoscleromatis is said to be the cause. A female patient aged 45 years, presented with a past history of trauma to the nose and swelling on her nose since last 1 year. There was nasal asymmetry and internal nasal examination showed a septal swelling protruding to the right nasal cavity with hypertrophied nasal mucosa and inferior turbinate. Open tip rhinoplasty approach was used to excise the mass, which examined pathologically revealing a rhinoscleroma, fibrotic infiltrative stage.

  15. Interventional treatment of common congenital heart diseases the common view of Chinese medical experts.Part Two——Interventional treatment of ventricular septal defect%常见先天性心脏病介入治疗中国专家共识二、室间隔缺损介入治疗

    Institute of Scientific and Technical Information of China (English)

    中国医师协会心血管内科分会先心病工作委员会

    2011-01-01

    室间隔缺损(VSD)为最常见的先天性心脏畸形,占先天性心脏病的25% ~ 30%.介入治疗的关键在于适应证的选择,操作技巧和并发症的防治.根据左心室造影图像判断VSD的形态和类型,选择不同类型的封堵器.操作中要避免右房室瓣腱索的缠绕、瓣膜的损伤和心律紊乱的发生.术后应严密观察,及早处理房室传导阻滞等并发症.本文详细介绍了膜部和肌部VSD的诊断,规范化的操作方法,术中和术后可预测的情况处理.%Ventricular septal defect(VSD)is the most common congenital heart disease. it accounts for 25 ~ 30% of all congenital heart diseases. The key points of interventional treatment for VSD are the careful selection of indications. the rich experience in manipulating skill and the effective prevention of complications. According to the imaging f'indings on the selective left sided cardiography and echocardiography, the morphology and type of VSD, mainly including the precise size and location of the defect. can be accurately determined. on this account the proper transcatheter device closure can be rationally selected. During the interventional management, the twine and damage of the right atrio-ventricular valve as well as its tendinous cords should be avoided. and the ventricular arrhythmias and new aortic or tricuspid regurgitation should be prevented. After the treatment, the patient should be closely observed, the postoperative complications such as atrioventricular block should be promptly dealt with. In this chapter, the diagnosis of membranous and muscular VSD. the standard interventional procedure of transcatheter device closure implantation and the management of predictable events occurred during and after the procedure will be systemically described. (J Intervent Radiol, 2011 , 20 : 87-92)

  16. Fourier analysis of multi-gated blood-pool data in patients with congenital heart disease, (1). Assessment of disease with left to right shunt, especially ventricular septal defect

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    Takeda, Kan; Maeda, Hisato; Yamaguchi, Nobuo; Nakamura, Kazuyoshi; Nakagawa, Tsuyoshi (Mie Univ., Tsu (Japan). School of Medicine)

    1983-07-01

    The ventricular emptying performance in patients with congenital heart disease with left to right (L-to-R) shunt was investigated by temporal Fourier analysis of multi-gated cardiac blood-pool data. Functional images are constructed with parameters of each pixel's phase angle and amplitude at fundamental frequency. Using global time-activity curves of both ventricles, phase angle and amplitude of left and right ventricles (LV and RV) were computed. Values of interventricular phase difference (D (phase)) and amplitude ratio of RV to LV (R (amp)) were calculated in individual cases. In 18 subjects with normal cardiac function, mean ( +- standard deviation) values of D (phase) was 1.7 +- 5.8 degree and that of R (amp) was 0.54 +- 0.20, respectively regardless of heart rate. In 22 patients of ventricular septal defect (VSD) with L-to-R shunt, D (phase) became larger in proportion to the ratio of pulmonary to systemic blood flow (Qp/Qs) (r = 0.899, p < 0.001). Especially, in those with large L-to-R shunt (Qp/ Qs < 2.0), significant RV phase lag over 18 degrees was recognized and types of VSD might be possible to be differentiated by phase images. In 9 patients with patent ductus arteriosus (PDA), no RV phase delay was seen. Mean value of R (amp) was considerably smaller in patients with PDA and significantly larger in 11 patients with atrial septal defect (ASD), as compared with that of subjects with normal cardiac functions. However, cases with VSD took the values within normal range. This method is highly valuable for pathophysiologic investigation and differential diagnosis of congenital heart disease with L-to-R shunt.

  17. Percutaneous Interventional Treatment of Atrial Septal Defect Secundum in Macedonia

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    Ivan Milev

    2014-03-01

    Conclusion: In conclusion, the implantation of a septal occluder was found to be a safe procedure that resulted in improved hemodynamic parameters that result from right ventricular volume overload with favorable short- and mid-term results.

  18. 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 b...

  19. En bloc aortic and mitral valve replacement and left ventricular outflow tract enlargement using a combined transaortic and trans-septal atrial approach.

    Science.gov (United States)

    Hassan, Mohammed; Windsor, Jimmy; Ricci, Marco

    2015-12-01

    Aortic and mitral valve replacement with division and reconstruction of the inter-valvular fibrous body has been described in clinical situations involving infective endocarditis, extensive annular calcifications and diminutive valve annuli. Herein, we describe a combined transaortic and trans-septal approach with division of the inter-valvular fibrosa for combined aortic and mitral valve replacement. The reconstruction of the inter-valvular fibrous body, atrial walls and aortic root was carried out using a 'three-patch' technique with bovine pericardium.

  20. 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.

  1. 经胸微创封堵术治疗婴幼儿室间隔缺损的疗效评价%Evaluation of the efficacy of transcatheter closure of ventricular septal defect in infants and young children

    Institute of Scientific and Technical Information of China (English)

    宋毅; 钟前进; 陈建明; 李志平; 李福平; 胡义杰

    2015-01-01

    Objective To investigate the therapeutic effect of transcatheter closure of ventricular septal defect in infants oryoung children with minimally invasive surgery,and to explore the clinical application value. Methods 50 cases of children with ventricular septal defect were confirm by Colour Doppler Ultrasound. Then selected the appropriate occluder type spare and had an operation under general anesthesia and tracheal intubation.The transesophageal echocardiography probe is inserted through the mouth. Exposed the right ventricle with 3 ~ 4 cm small incision from the xiphoid to the third intercostal level on median sternotomy. And the transesophageal echocardiography was used for occluder closure of ventricular septal defect. In addition,Membranous ventricular septal defect choose occluder diameter larger than the defect diameter of 0 ~ 2 mm; with membranous aneurysm,the selection of the occluder diameter should be 3 ~ 4 mm larger than the diameter of the defect. Conventional heparin anticoagulation and oral aspirin anticoagulation were used after the surgery. Results The Operation time was(62.23±11.23)min. All the closures were successful with no residual shunt and no regurgitation in the aortic valve,mitral valve and tricuspid valve. And the Hospitalization were(9.11±1.84)days. Postoperative follow-up was(24.11±6.42)months; all follow-up to the patients,there were no occluder detachment,infective endocarditis,thromboembolism,sudden death and other serious complications occurred. Conclusion Minimally invasive closure is an effective method for the treatment of ventricular septal defect in infants and young children.%目的:探讨经胸微创婴幼儿室间隔缺损封堵术治疗效果,探讨其临床应用价值。方法室间隔缺损的患儿50例,彩超明确室间隔缺损情况,选取相应的封堵器型号备用,在全麻、气管插管下进行手术,经口腔插入食道超声探头,胸骨正中作上至第三肋间水平下至剑突3~4

  2. Comparative analysis of early and middle outcomes of the arterial switch operation in children with complete transposition of the great arteries with ventricular septal defect and severe pulmonary artery hypertension

    Institute of Scientific and Technical Information of China (English)

    LIU Cheng-hu; SU Jun-wu; LI Zhi-qiang; FAN Xiang-ming; CHEN Yan; HE Yan; LIU Ying-long

    2013-01-01

    Background The best age for the arterial switch operation (ASO) in complete transposition of great arteries with ventricular septal defect is usually considered to be within six months.This is because of severe pulmonary arterial hypertension and pulmonary arterial obstructive pathological changes.There are few reports on ASO surgery in children older than three years old.Methods We studied 41 children,including 24 males and 17 females,from January 2010 to December 2011.They were divided into three groups by operation age; 15 patients were <1 year old,13 were 1-3 years old,and 13 were >3 years old.Associated cardiac abnormalities included patent ductus arteriosus in six cases,atrial septal defect in five cases,and mitral regurgitation in two cases.All the patients had echocardiography before the operation.Seventeen patients underwent a coronary computed tomography examination and five patients underwent right heart catheterization.All ASO surgeries were performed under inhalation anesthesia and hypothermic cardiopulmonary bypass.Results Three operative deaths occurred.Two were in the <1 year old group,who died from severe postoperative low cardiac output.The other was two years old and died of postoperative multiple organ failure.There was no significant difference in postoperative mortality and the recent mid-term survival rate among the three groups.Thirty-eight cases were followed up for an average of 11.2 months,ranging 6-20 months.One seven years old patient died of acute diarrhea and electrolyte disturbance arrhythmia caused by food poisoning.Three patients more than three years old still had residual pulmonary arterial hypertension.Conclusion Children older than three years old can still undergo the ASO procedure,but residual pulmonary hypertension is present.

  3. Surgical repair of true left ventricular aneurysm in an infant: a rare complication after unsuccessful perventricular VSD closure.

    Science.gov (United States)

    Ozyilmaz, Isa; Saygi, Murat; Yildiz, Okan; Erek, Ersin; Guzeltas, Alper

    2014-10-01

    A 2.5-month-old female patient presented for closure of a ventricular septal defect (VSD). Transthoracic echocardiography showed a large muscular ventricular septal defect. After perventricular closure of the defect was performed with an Amplatzer muscular VSD occluder, peroperative transesophageal echocardiography revealed that the device had been implanted in the wrong area of the ventricular septum. The device was retrieved and a large mid-muscular defect with extension to the outlet septum was closed with a patch of Dacron which was secured with 5-0 sutures. A perforation in the ventricular septum due to attempted perventricular device delivery was seen, and it was repaired primarily. In the eighth month of follow-up, transthoracic echocardiography revealed an aneurysm in the posterior wall of the left ventricle. The patient's electrocardiogram showed pathological Q waves and ST-segment elevation in leads DII, DIII, and aVF consistent with subacute inferior myocardial infarction. At subsequent surgery, this was found to be a true aneurysm, located in area of distribution of the obtuse marginal branch of the left circumflex coronary artery in the posterior wall of the left ventricle. The aneurysm was closed off using a Dacron patch, and the sac was resected. Development of a true aneurysm is a rare but important complication of attempted perventricular VSD closure.

  4. Echocardiographic diagnosis of muscular ventricular septal aneurysm in infants%婴幼儿室间隔肌部瘤的超声诊断

    Institute of Scientific and Technical Information of China (English)

    郭楠; 陈娇; 朱琦

    2010-01-01

    @@ 室间隔肌部瘤是一种罕见的室壁瘤,由于多数患有单纯室间隔肌部瘤的病例无明显症状和体征,临床诊断较困难.超声心动图检查能清楚显示室间隔肌部瘤的形态、运动以及判断是否合并其他心脏结构及功能异常,对室间隔肌部瘤患者的诊断及监测具有重要的临床意义.

  5. VENTRICULAR SEPTAL DEFECT ASSOCIATED WITH AORTIC VALVE PROLAPSE IN CHINESE CHILDREN——A PROSPECTIVE ASCENDING AORTOGRAM STUDY OF 550 PATIENTS

    Institute of Scientific and Technical Information of China (English)

    朱铭; 黄廉溪

    1993-01-01

    From April 1985 to April 1991, ascending aortograms were peformed in 550consecutive children with I/SD. The locations of VSD were subpulmonary in 121,perimembranous in 420, and muscular in 9 cases. Seventy-eight cases (14.2%) were associ-ated with aortic valve prolapse (male 57,female 21), 38 without AR and 40 with AR,which occured in 54 (44.6%)subpulmonary VSD, 24 (5.7%) in perimembranous VSD.Most of the VSD with aortic prolapse were functionally small. Ascending aortogram andleft ventriculogram (RAO view) can demonstrate aortic prolapse.

  6. 婴幼儿室间隔缺损并肺动脉高压的外科治疗%Surgical treatment for ventricular septal defect infants with pulmonary hypertension

    Institute of Scientific and Technical Information of China (English)

    吕瑛; 李小兵; 张会军; 闫芳; 孙丽颖; 李志杰; 申红; 黄建成; 李红英

    2013-01-01

    Objective To evaluate the efficiency of urgent surgical correction for ventricular septal defect (VSD) infants with pulmonary hypertension(PH).Methods Two hundred and fifteen infants (109 male,106 female)with VSD and PH received surgical correction from Department of Cardiac Surgery,Center of Congenital Heart Disease,the First Hospital of Hebei Medical University.The age of patients ranged from 1 to 36 months on admission,the body mass ranged from 1.5 to 10.0 kg.Among all the cases,the perimembranous type was found in 156 children,the conal septal defect in 18 cases,and subpulmonary VSD in 41 cases.The diameter of defect was 0.8-2.1 cm.Sixteen patients were associated with atrial septal defect (ASD),8 cases with patent foramen ovale,15 cases with ductus arteriosus (PDA),14 cases with ASD and PDA,and 3 cases with PDA and coarctation of the aorta,14 cases with pulmonary valve stenosis,33 cases with stricture of right ventricular outflow tract,22 cases with incompetence of mitral valve directly ligation,42 cases with incompetence of tritral valve directly ligation,and the rest 12 cases with persistent left superior vena cava.The pressure of pulmonary artery was 4.66-11.31 kPa.All patients received cardiopulmonary bypass under hypothermic anaesthesia through extracorporeal blood circulation.Results Urgent operations were performed in all patients,but 3 patients died after surgery.Postoperative complications included low cardiac output syndrome (15 cases),among whom 2 cases died ; right bundle-branch block (10 cases),supraventricular tachycardia (6 cases),transient atrioventricular block of grade Ⅲ (5 cases),inhalation of nitric oxide was given to 5 cases for the management of significant postoperative PH,among whom 1 case died during surgery,3 cases were pneumothorax,2 cases were atelectasis.Chest radiography,echocardiography,and ECG were performed during this period.As a result,most children had good cardiac functions.Conclusions Low cardiac output syndrome and

  7. 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.

  8. 右心室心尖部与间隔部起搏对患者心功能的远期影响%Comparison of long-term effects on cardiac function of right ventricular septal pacing versus right ventricular apical pacing

    Institute of Scientific and Technical Information of China (English)

    彭珍云; 向力群; 曾向辉; 张良; 钟常青; 刘振芳; 张翼

    2013-01-01

    目的 探讨右心室心尖部与间隔部起搏对长期起搏依赖患者心功能的远期影响.方法 入选在湖南师范大学第一附属医院就诊并行双腔起搏器植入术的Ⅲ度房室传导阻滞108例,据心室电极固定部位分为间隔部起搏组(RVS组,57例)及心尖部起搏组(RVA组,51例);比较两组患者术前与术后3年血清氨基末端脑钠肽前体(NT-proBNP)浓度及左心室射血分数(LVEF)、左心室舒张末期内径(LVEDD)变化、左心房大小(LA)及心电图Ⅱ导联QRS波时程变化.对NT-proBNP水平与LA及QRS波时程进行直线相关分析.结果 术前NT-proBNP浓度、QRS波时程及LA大小两组间差异无统计学意义(P>0.05).术后两组患者NT-proBNP浓度、QRS波时程及LA大小均较术前明显增大,差异有统计学意义(P<0.05),但心尖部起搏组各观察指标值上升幅度较大.RVA组与RVS组比较显示,NT-proBNP浓度升高更明显[(367.4±38.5) pg/ml比(210.4±36.7)pg/ml,P<0.05];QRS波时程延长[(0.192 ±0.042)s比(0.151 ±0.091)s,P<0.05];LA明显扩大[(32.9±4.1) mm比(28.9±4.2)mm,P<0.05].组内及组间比较,LVEF及LVEDD差异均无统计学意义.双变量直线相关分析显示,NT-proBNP与QRS波时程呈直线正相关(r=0.701);与LA大小呈直线正相关(r=0.671).结论 心尖部起搏易引起心室收缩失同步及左心房不良重构,间隔部起搏更接近生理性起搏,是相对理想的起搏部位.%Objective To investigate and compare the efficiency of right ventricular apical pacing (RVA) with right ventricular septal pacing (RVS).Methods A total of one hundred and eight consecutive patients with Ⅲ ° atrio-ventricular block (Ⅲ° AVB) and normal ejection fraction undergoing dual-chamber pacemaker implantation were randomly divided into right ventricular apical pacing group (RVA,n =51) and right ventricular septal pacing group (RVS,n =57) according to the pacing electrode's position in the right ventricular apex.At baseline

  9. Cierre espontáneo de la comunicación interventricular muscular aislada y la perimembranosa con aneurisma del septum

    Directory of Open Access Journals (Sweden)

    Abdón Castro B

    2006-09-01

    Full Text Available Introducción y objetivo: La comunicación interventricular (CIV es la cardiopatía congénita más frecuente en todas las edades. El presente estudio se realizó con el fin de conocer el porcentaje de CIVs musculares aisladas y perimembranosas con aneurisma del septum que se cierran espontáneamente y en cuánto tiempo lo hacen. Métodos: De febrero 2003 a diciembre 2005 se recolectaron 75 casos de neonatos diagnosticados con CIV muscular aislada y 10 con CIV perimembranosa aislada y con formación de aneurisma, quienes fueron seguidos por un periodo de al menos 30 meses. Se usó como método de diagnóstico la ecocardiografía bidimensional y el mapeo con Doppler color Resultados: Al año de seguimiento, el 68% de las CIV musculares se cerró espontáneamente y 90% a los 2 años. Solo un 10% de las CIV perimembranosas con aneurisma del septum se cerraron a los 2 años de seguimiento. El tamaño de las comunicaciones varió de 1,5 a 6 mm en ambos grupos. Conclusión: Las CIV musculares cierran espontáneamente en el 90% de los casos a los 2 años de edad, no así las perimembranosas en que lo hacen solo en un 10% de los casos.Introduction and objective: Ventricular septal defect (VSD is the most frequent congenital heart anomaly. This study was planned to establish the percentage of spontaneous closure of isolated muscular and isolated perimembranous (with septal aneurysm VSD and their time of closure. Methods: From February 2003 to December 2005, we collected 75 neonates with muscular VSD and 10 with perimembranous VSD with septal aneurysm, who had a follow up period of at least 30 months. Two D echo and color Doppler mapping were utilized as diagnostic method. Results: after 1 year of follow-up, 68% of all muscular VSDs had closed and 90% had done so at the age of 2, however only 10% of perimembranous VSDs with septal aneurysm had closed at 2 years of age. The size of the defects varied from 1,5 to 6 mm in both groups. Conclusion: a muscular

  10. Surgical treatment of ventricular septal defect with pulmonary hypertension in infants younger than 1 yearold%1岁以下婴儿室间隔缺损伴肺动脉高压的外科治疗

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    许刚; 张镜芳; 庄建; 陈欣欣; 陈寄梅; 岑坚正

    2001-01-01

    目的探讨婴儿室间隔缺损(VSD)伴肺动脉高压(PH)外科治疗的结果和经验。方法对60例1岁以下婴儿室缺伴肺高压的手术治疗进行总结。年龄2~11个月,平均(6.23±2.60)个月,体重3~10 kg,平均(5.50±1.26)kg。术前中度以上肺高压53例(88.8%)。结果全组手术死亡3例(5%),手术死亡的主要原因是肺动脉高压危象和急性心包填塞。术后主要并发症为肺动脉高压危象,术后出血和肺部感染。结论婴儿室缺伴肺高压应尽早手术治疗。术前、术后支持疗法,术中加强心肌保护、提高手术技巧,术后注意呼吸、循环监测,加强呼吸道管理,防治可能出现的并发症是手术治疗成功的关键。%Objective To review the experience of surgical treatment of ventricular septal defect (VSD) with pulmonary hypertension (PH) in infants younger than 1 year old.Methods 60 infants with VSD+PH received operations. Their ages ranged from 2 to 11 months[(6.23±2.60) months]. The mean body weight was [(5.50±1.26)kg]. 53 patients (88.8%) had moderate or severe pulmonary hypertension before operation.Results Three patients died after operation. The operative mortality was 5%. The main reasons were pulmonary hypertensive crisis and acute cardiac tamponade. The main complications were pulmonary hypertensive crisis, bleeding and pulmonary infection. Conclusion Surgical treatment of VSD with PH in infants may have a satisfactory outcome. The improvement of surgical techniques and proper perioperative management are important factors influencing surgical outcome.

  11. The clinical application of symmetrical occluder in treating intercristal ventricular septal defect%嵴内型室间隔缺损介入封堵术中对称型封堵器的临床应用

    Institute of Scientific and Technical Information of China (English)

    许美珍; 罗来树; 李颐; 程应樟; 李运德; 吴延庆; 吴清华; 程晓曙; 彭强

    2014-01-01

    Objective To investigate the interventional treatment strategy for occluding the intercristal ventricular septal defect (VSD) in order to improve the surgical safety and success rate. Methods During the period from January 2012 to December 2013, a total of 31 patients with intercristal VSD were admitted to authors’ hospital to receive interventional catheter occlusion therapy. Preoperative color Doppler ultrasound echocardiography showed that on the short axis view of the aorta the VSD interrupted port was situated at 12:00 - 1:00 o’clock region. Left ventricular and above aortic valve angiography indicated that the VSD location, shape and size, the split vent size on the left ventricle side and its distance from the aortic valve could be correctly measured when the VSD shunt was visualized , which were very helpful in guiding the operator to select the suitable occluder as well as to adjust the release pattern of the occluder. Postoperative imaging findings of the left ventricular and above aortic valve angiography were compared with the preoperative ones. Results Successful occlusion of VSD was obtained in 22 patients , in 13 among them the left ventricular angiography showed that the direction of blood flow beam at the defect hole was from the left ventricle to the right ventricle in an obliquely upward direction. The basal width of the defect on the left ventricle side was (5.12 ± 1.38) mm, and(6 - 10) mm occluder was employed. In the remaining 9 patients the left ventricular angiography showed that the direction of blood flow beam at the defect hole was from the left ventricle to the right ventricle in a direction almost parallel to the aortic valve , and the basal width of the defect on the left ventricle side was (7.18 ± 1.26) mm, and (9 - 12) mm zero-bias occluder was adopted. Interventional occlusion of VSD was unsuccessful in 9 cases as the intercristal hole was rather larger, and two of them had coexisting aortic sinus aneurysm complicated by mid

  12. Application value of echocardiography in perventricular device closure of ventricular septal defect%超声心动图在微创外科室间隔缺损封堵治疗中的应用价值

    Institute of Scientific and Technical Information of China (English)

    康彧; 唐红; 白文娟; 安琪; 赁可; 宋海波

    2011-01-01

    目的 探讨超声心动图在微创外科室间隔缺损(VSD)封堵治疗中的应用价值.方法 经胸超声心动图(TTE)筛选拟行微创外科VSD封堵治疗患者169例,术中经食管超声心动图(TEE)再次评估,对具备封堵条件者进行实时引导和监控.术后3~5 d及3、6、12个月 TTE随访.结果 术中TEE评估对15例不适宜封堵者直接改行VSD修补术.行微创封堵术154例,成功137例,成功率88.9%;封堵失败改行VSD修补术17例.术中即刻观察10例有微量残余分流,3例主动脉瓣有微量反流,7例三尖瓣反流较术前减轻或消失,11例三尖瓣出现少量反流.术后TTE随访封堵器无移位,4例残余分流消失,1例三尖瓣反流较术前略有加重,左心室重构改善.结论 超声心动图在微创外科VSD封堵的术前诊断、术中监控及术后效果评价中均发挥了重要作用.%Objective To explore the application value of echocardiography in perventricular device closure of ventricular septal defect ( VSD ). Methods One hundred and sixty - nine patients with VSD were selected by transthoracic echocardiography ( TTE ) and prepared to receive off pump surgical treatment-perventricular device closure. All the patients were re-evaluated in cardiac surgical procedures by transesophageal echocardiography ( TEE ). The patients suitable to the perventricular device closure were guided and observed. After operation, 3 ~5d,3,6,12 months follow-up were performed by TTE. Results Fifteen cases were not suitable to the device closure by intraoperative TEE re-evaluation and were directly repaired under cardiopulmonary bypass. 137 of 154 cases treated with perventricular device closure were succeeded( 137/154, 88.9% ), 17 cases were failed and received the repair surgery. TEE was performed immediately after occluder release. The tricuspid regurgitation was decreased or disappeared in 7 patients, while the closure caused new mild tricuspid regurgitation in 11 patients, and there were 10

  13. 小儿膜周部室间隔缺损封堵术后心电图长期随访%Long-term follow-up of electrocardiogram after transcatheter closure of perimembranous ventricular septal defect in children

    Institute of Scientific and Technical Information of China (English)

    王慧深; 李淑娟; 林约瑟; 李轩狄; 李运泉; 孙芸芸

    2012-01-01

    目的 长期随访儿童经导管介入封堵膜周部室间隔缺损(ventricular septal defect,VSD)后心电图(ECG)各值,了解ECG改变规律及其转归.方法 对患膜周部VSD并成功介入封堵的290例患儿进行术后ECG随访.所有患儿术前术后均检查ECG,术后分别于1、3、6个月及1、2、3、5、8年复查ECG,电脑自动测算心率(V)、P-R间期、QRS间期、QT间期及校正的QT(QTc)值,观察心律及ST-T的变化.结果 随访时间:1~96个月[平均(40.16±29.2)个月].行多因素分析发现小儿膜周部VSD引起心电改变的危险因素是年龄在6周岁以内,膜部瘤样VSD,选择封堵器大于VSD直径3 mm以上者.封堵术后290例中115例(39.7%)出现各种轻重不等的ECG改变,进入5年以上长期随访的200例中持续ECG改变者43例(21.5%).但这些患儿均无症状,生长发育良好.随访80例经过应用激素和营养心肌治疗者中仅17例仍存在ECG的改变(21.3%),未经过治疗的35例,持续存在ECG改变者有26例(74.3%).随访5年以上仍需特别关注并定期随访的ECG改变者有25例(25/200例,12.5%).持续ECG改变组与术前组及无ECG改变组的各值对比,均有显著差异.对于出现重度房室传导阻滞(AVB)的患儿及时放置临时起搏器以及激素和营养心肌等治疗的病例预后较好.结论 本组随访提示封堵膜周部VSD慎重选择封堵器型号至关重要.对封堵术后ECG出现异常改变者,应用激素、果糖和维生素等营养心肌治疗,对心律失常的恢复或缓解有帮助.封堵术后早期发生ECG改变大部分轻微或可恢复,对一些明显的ECG改变,随访中未发现有转为三度AVB并影响患儿生长发育的病例,但对于一些ECG值的改变需要更长期随访以了解其转归和预后.%Objective To observe the long-term follow-up data of electrocardiographic (ECG) changes and prognosis after transcatheter closure of perimembranous ventricular septal defect (VSD)in children. Methods 290

  14. Pulmonary edema following transcatheter closure of atrial septal defect

    Directory of Open Access Journals (Sweden)

    Singhi Anil

    2010-01-01

    Full Text Available Pulmonary edema after device closure of atrial septal defect (ASD is a rare complication. We present illustrative images of a case of pulmonary edema after device closure of ASD in a 53 year old adult. Older patients undergoing ASD closure can benefit from their left atrial and left ventricular end diastolic pressures measurement before and after temporary balloon occlusion of ASD.

  15. Tratamento cirúrgico da rotura do septo interventricular pós infarto agudo do miocárdio Surgical treatment of ventricular septal defect post acute myocardial infart

    Directory of Open Access Journals (Sweden)

    Luiz Fernando Leite Tanajura

    1987-08-01

    Full Text Available A rotura do septo interventricular (RSI reduz a perspectiva de sobrevida do paciente com infarto agudo do miocárdio (IAM. Entre 1968 e 1987, atendemos 48 pacientes (p com este diagnóstico, sendo 16 (33% mantidos clinicamente e 32 (67% submetidos a cirurgia. Todos os p clínicos faleceram durante a internação. Dentre os p operados, o IAM se localizava na parede anterior em 18 (56% e na inferior em 14 (44%, com mortalidades respectivas de 6 (33% e 4 (29%. Disfunção ventricular esquerda severa (Killip III e IV foi encontrada em 26 (81%. Em 18 (56% p foi introduzido o cateter de Swan-Ganz. Dos 31 (97% p que se submeteram a cinecoronariografia, 22 (71% apresentavam lesão uniarterial e 9 (29%, doença multiarterial. Entre os p com lesões isoladas, a descendente anterior foi acometida em 15 (68%, a coronária direita em 6 (27% e a circunflexa em 1 (5%. A cirurgia foi realizada nas 2 primeiras semanas de evolução em 8 (25% e, após este período, em 24 (75%, com mortalidades de 6 (75% e 4 (17%, respectivamente. O balão intra-aórtico foi usado em 7 (22%. Os procedimentos cirúrgicos associados ao fechamento da RSI foram revascularização miocárdica em 10 (31%, aneurismectomia de VE em 17 (53% e infartectomia e 6 (19%. Na evolução tardia, dos 22 sobreviventes, 14 (64% encontram-se assintomáticos, 1 (5% sintomático, ocorreram 4 (18% óbitos e perdeu-se a evolução de 3 (13%. Concluímos que, apesar do risco cirúrgico, a cirurgia precoce ainda é a melhor opção terapêutica desta complicação.The ventricular septal rupture (VSR following acute myocardial infarction (AMI is a rare and catasthrophic complication with a high mortality rate. Surgery is the only effective procedure to try to control this complication. However the surgical mortality is elevated and is closely related to the post-infarct period thus making the indication for the precise time for surgery one the fundamental aspects for its success. In a population of 48

  16. 膜周部室间隔缺损介入治疗术后三尖瓣损伤11例%Tricuspid injury in 11 patients after interventional transcatheter closure for perimembranous ventricular septal defect

    Institute of Scientific and Technical Information of China (English)

    曹黎明; 王凤鸣; 秦玉明; 赵乃琤; 周凯; 莫绪明; 朱善良

    2016-01-01

    Objective To investigate the characteristics of the newly discovered tricuspid injury during the fol-low -up of the patients with interventional transcatheter closure for perimembranous ventricular septal defect (VSD). Methods A retrospective analysis of clinical data was performed in the successful completion of membranous VSD in-tervention from March 2003 to April 2015,and 11 cases of serious tricuspid injury in children were found during the fol-low -up.Results Among 11 children,10 cases underwent surgery again,and in 7 cases of them with pseudoaneurysm of perimembranous septum,the occluders were deviated toward the right ventricul which caused valvular inadequacy for plate winding closures,extrusion and wear of tricuspid chordae and /or leaflet by right ventricul(RV)disc.Among them,1 case was caused by leaflet fusion tear and tricuspid valve insufficiency;3 cases without pseudoaneurysm were caused by a spindle like RV disc recovery structure protruding into the right ventricle,and then the disc squeezed the tricuspid tendinous cord or leaflet and the right ventricular disc nut wrapped tendon of tricuspid valve finally leading to tricuspid valve insufficiency.There was a small amount of tricuspid regurgitation after surgery;the cardiac size and car-diac function returned to normal.One case did not receive surgery,and at present the right atrium and right ventricle were significantly enlarged,and the activity was limited.Conclusions Tricuspid injury discovered newly after perimem-branous VSD interventional therapy is more common in pseudoaneurysm with large tumor occluding the right ventricular disc resulting in poor formation,and it is important to consider these factors in choosing the size of the occluder and the operation.Long term follow -up mechanism should be established for the treatment of VSD after interventional therapy.%目的:探讨膜周部室间隔缺损(VSD)介入术后随访过程中新发现的三尖瓣损伤病例特点,以期发现其

  17. Evaluation on global left ventricular systolic function of children with ventricular septal defect by two-dimensional speckle tracking imaging%二维斑点追踪成像评价室间隔缺损患儿左室整体收缩功能

    Institute of Scientific and Technical Information of China (English)

    李莎; 张立敏; 马春燕; 刘爽; 张妍; 杨军

    2014-01-01

    目的:探讨应用二维斑点追踪成像(2D-STI)评价室间隔缺损(VSD)患儿左室整体收缩功能的价值。方法单纯VSD患儿42例,按缺损大小与主动脉瓣环比值分为小VSD组、中VSD组、大VSD组;30例体检健康儿童为对照组。超声心动图常规测量左房收缩末期内径(LAD)、左室舒张末期内径(LVEDD)、左室舒张末期容积(LVEDV)、左室收缩末期容积(LVESV)及左室射血分数(LVEF),计算左房容积。2D-STI测量左室整体纵向峰值应变(GLPS)、收缩期峰值应变率(GLSRS),左室整体径向峰值应变(GRPS)、收缩期峰值应变率(GRSRS)及左室整体圆周峰值应变(GCPS)、收缩期峰值应变率(GCSRS)。结果大VSD组和中VSD组的LAD、LAV、LVEDD、LVEDV、LVESV较小VSD组和对照组均增大(P<0.05),大VSD组LAD、LAV、LVEDD、LVEDV、LVESV较中VSD组均增大(P<0.05)。与对照组和小VSD组的GLPS、GRPS、GCPS、GLSRS、GRSRS、GCSRS比较,中VSD组均增大(P<0.05),大VSD组均减小(P<0.05);中VSD组GLPS、GRPS、GCPS、GLSRS、GRSRS、GCSRS亦大于大VSD组(P<0.05)。结论中度以上VSD影响左室整体收缩功能。2D-STI的应变、应变率对VSD患儿左室整体收缩功能的评价有重要意义。%Objective To explore the value of two-dimensional speckle tracking imaging (2D-STI) in evaluating the global left ventricular systolic function of children with ventricular septal defect. Methods Forty-two children with simple VSD were divided into small, moderate and large VSD group according to the ratio of VSD size to aortic annulus diameter. And 30 healthy children were enrolled as control group. The left atrial systolic diameter (LAD), left ventricular end-diastolic diameter (LVEDD), left ventricular end-diastolic volume (LVEDV), left ventricular end systolic volume (LVESV) and left ventricular ejection fraction (LVEF) were routinely

  18. Surgical Treatment of Large Ventricular Septal Defect Complicated with Pneumonia in Infants%大型室间隔缺损并肺炎婴幼儿的外科治疗

    Institute of Scientific and Technical Information of China (English)

    马千里; 陈子英; 石凤梧; 陈立华; 梁宜武; 李倩

    2011-01-01

    目的 探讨大型室间隔缺损(VSD)并肺炎婴幼儿早期手术治疗的可行性、手术时机、适应证以及术中术后的处理.方法 选择2009年10月-2010年7月本院收治的大型VSD并肺炎患儿19例.男11例,女8例;年龄3~15(7.3±2.8)个月;体质量5~12(7.6±1.4) kg .其中VSD并房间隔缺损5例、并动脉导管未闭1例;膜周部VSD 16例,肺动脉瓣下VSD 3例.患儿均在低温、体外循环、冷晶体停跳液灌注下行VSD直视修补术及并发畸形矫治.16例膜周部VSD患儿均经右房切口修补;3例肺动脉瓣下VSD患儿中,2例经肺动脉横切口修补,1例经右室流出道切口修补.19例患儿VSD修补均采用补片修补法,其中15例应用牛心包补片,4例应用自体心包补片(戊二醛固定).患儿术中均应用膜式氧合器和术中平衡超滤.结果 19例患儿手术均获成功,术后主要并发症为肺部感染3例,切口愈合不良2例,肺不张和室间隔少量残余分流各1例,无全身感染、肺动脉高压危象以及严重低心排病例,19例患儿均治愈出院.门诊随访2~6个月,上呼吸道感染显著减少,营养发育状况改善.结论 把握手术时机和适应证,早期手术、防治术中和术后肺损伤,是治疗大型VSD 并肺炎婴幼儿的有效方法.%Objective To investigate the feasibility,operation time,indication,management during and after operation of early repair in infants with large ventricular septal defect(VSD) complicated with pneumonia. Methods From Oct. 2009 to Jul. 2010,19 infants [ 11 male and 8 female, aged 3 - 15 (7.3 ± 2.8 ) months, weighted 5- 12 (7.6 ± 1.4) kg ] with large VSD complicated with pneumonia were admitted to the Second Hospital of Hebei Medical University. Five cases were associated with atrial septal defects , 1 case was associated with patent ductus arteriosus; 16 cases were membrana type,3 cases were subpulmonary valve type. All patients underwent cardiopulmonary bypass with systemic hypothermia

  19. 心室间隔膜部及嵴内型缺损动物模型的建立%Animal models of intracristal and membranous ventricular septal defect

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    周达新; 管丽华; 王晓彦; 王宜青; 黄国倩; 葛均波; 诸骏仁

    2008-01-01

    BACKGROUND:Surrounding structure of intracristal ventricular septal defects(IVSD) and membranous ventricular septal defects(MVSD)is complicated,and it is difficult to perform transcatheter closure treatment.Corresponding animal models should be established to define an optimal interventional therapy.OBJECTIVE:To prepare the animal models of IVSD and MVSD under echoeardiogram location.DESIGN:Animal modeling experiment.SETTING:Department of Cardiology,Zhongshan Hospital Affiliated to Fudan University.MATERIALS:Six healthy adult dogs,irrespective of gender,were purchased from Shanghai Experimental Animal Center.Aorta and great saphenous vein stapler-puncher(St.Jude corporation,USA)was used.The main improvement is to cut the plastic shell even,thin,or shorter in order to reduce the resistance when puncturing the"purse"in the fight ventficle and the interventficular septum.Sonos 5500 multifunction ultrasonoscope(Philips,USA)was used,and the frequency of the probe was between 2.5-3.5 MHz.METHODS:Experiments were performed at the Zhongshan Hospital Affiliated to Fudan University and Central Laboratory of Shanghai Institute of Cardiovascular Disease from April to July 2006(biosaflety level 2).After anaesthesia,6 adult dogs were fixed at decumbent position.The interventricular septum was punetured to make the preparation with the location of echocardiography(ECHO)by using the modified aorta and great saphenous vein proximal stapler-puncher.Animal intervention met the Animal Ethical Committee of Fudan University.MAIN OUTCOME MEASURES:One week after the surgery,transthoracic echocardiography examination was used to check the VSD site,the highest shunt flow rate,the pressure difference between both sides of the VSD,as well as pulmonary artenal pressure.RESULTS:The VSD models were successfully established in three survivals and included in the final result.Two models of IVSD and one model of MVSD were examined by ECHO one week after the surgery.The diameter of VSD ranges from 1

  20. Pulmonary edema following transcatheter closure of atrial septal defect

    Directory of Open Access Journals (Sweden)

    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.

  1. Pulmonary edema following transcatheter closure of atrial septal defect

    Science.gov (United States)

    Chigurupati, Keerthi; Reshmi, Liza Jose; Gadhinglajkar, Shrinivas; Venkateshwaran, S.; Sreedhar, Rupa

    2015-01-01

    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. PMID:26139760

  2. Muscular Dystrophy

    Science.gov (United States)

    ... Devices The Search for a Cure en español Distrofia muscular About MD Muscular dystrophy (MD) is a genetic ... muscles and cause different degrees of muscle weakness. Duchenne muscular dystrophy is the most common and the most ...

  3. Muscular Dystrophy

    Science.gov (United States)

    ... Devices The Search for a Cure en español Distrofia muscular About MD Muscular dystrophy (MD) is a ... muscles and cause different degrees of muscle weakness. Duchenne muscular dystrophy is the most common and the ...

  4. Estudio morfopatológico de 101 corazones portadores de defecto septal interventricular

    OpenAIRE

    Muñoz Castellanos,Luis; Martínez Asencio,Ma. Eugenia; Kuri Nivón,Magdalena

    2005-01-01

    Las diversas denominaciones y clasificaciones de los defectos septales ventriculares (DSV) motivó la realización de este estudio con el propósito de contribuir a la adopción de una terminología común y de una clasificación en la que se integren criterios morfológicos, topográficos y quirúrgicos. Se estudiaron 101 defectos septales ventriculares con el método secuencial segmentario de la colección patológica del Instituto Nacional de Cardiología "Ignacio Chávez". El septum ventricular (SV) se ...

  5. 小儿先天性室间隔缺损介入治疗心电改变及处理%Change of electrocardiogram and treatment in the children with congenital ventricular septal defect by interventional processes

    Institute of Scientific and Technical Information of China (English)

    王慧深; 李运泉; 陈国桢; 覃有振; 李淑娟; 李格丽; 李渝芬; 钱明阳

    2008-01-01

    目的 分析小儿先天性室间隔缺损(VSD)介入治疗后的心电图(ECG)变化,探索避免严重心律失常的发生.方法 记录260例介入封堵VSD术前后的ECG,据此分成术后心律失常组和无心律失常组(对照组),对发生完全性或重度房室传导阻滞(AVB)的患儿及时营养心肌和减轻心肌水肿治疗,必要时放置临时起搏器.结果 心律失常组82例(31.5%),对照组178例.心律失常组中4例(1.5%)为完全性或重度AVB.36例(13.8%)给予营养心肌等药物处理.两组VSD径比较无显著差异(P>0.05);心律失常组较对照组年龄小,体质量轻(P<0.05),选用封堵器较对照组大(P<0.05).心率、QRS时限、PR间期、QT间期和VTc封堵术前后差异有统计学意义(P<0.05).结论 VSD介入封堵术中和术后需密切注意ECG改变,避免选择过大封堵器.出现完全性右束支传导阻滞(CRBBB)、完全性左束支传导阻滞(CLBBB)、二度AVB者,应尽早营养心肌治疗;完全性或重度AVB者,尽早安装临时起搏器.%Objective To analysis the change of electrocardiogram(ECG)in the children with congenital ventricular septal defect(VSD)by interventional processes and to explore how to decrease the incidence of serious arrhythmias.Methods To record the ECG before and after interventional closure in the 260 patients that hey were divided into arrhythmia group and no arrhythmia group(control group).The patients were treated with nutrition of cardiac muscle and medicines of reducing the cardiac muscle dropsy when they suffered from complete or serious complete or severe atrioventricuhr block(AVB)even underwent the temporary pacemaker quickly.Results There were 178 csses in control group.82 cuses(31.5%)had arrhythmiag after the interventional,in which 4 cases(1.5%)were complete/serious AVB.36 cases(13.8%)were given medicine above.The age was smaller and the body weigh was less in the arrhythmia group than that of control group(P<0.05).The device occluder was bigger in

  6. Inlay Approach for the Infant with Ventricular Septal Defect%镶嵌技术治疗婴幼儿室间隔缺损的效果观察

    Institute of Scientific and Technical Information of China (English)

    姜明泽; 游昕; 莫绪明

    2015-01-01

    Objective To investigate the feasibility and safety of inlay approach treatment for infant with ventricular septal defect(VSD).Methods Retrospective analysis on 279 infant from PLA 454th Hospital with VSD who received inlay approach from Jun.2010 to Dec.2013,was done.According to whether cardiop-ulmonary bypass was involved, they were divided into off-pump group ( 201 cases ) , and on-pump group (78 cases) .And 65 cases who received conventional VSD repair on-pump during the same period were included as control group.Operation time, postoperative mechanical ventilation time, intensive care unit (ICU) stay time,postoperative hospital stay,the rate of complications in perioperative period and the total cost of hospitalization of the three groups were compared .Results All of the three groups had no death dur-ing perioperative period.Operation time,postoperative mechanical ventilation time,ICU stay time,postopera-tive hospital stay of off-pump inlay approach group were (1.3 ±0.4) h,(2.5 ±0.8) h,(17.8 ±3.8) h, (6.7 ±1.7) d,which were shorter than those in the control group [(3.0 ±0.5) h,(13.6 ±6.6) h, (38.4 ±4.2) h,(12.0 ±1.8) d] and inlay approach group with cardiopulmonary bypass [(2.7 ±0.4) h, (47.1 ±22.8) h,(58.9 ±26.0) h,(11.6 ±3.0) d],the difference were statistically significant(P <0.05).The rate of complications in perioperative period of off-pump inlay approach group were lower than control group and on-pump inlay approach group(P<0.05).Conclusion Inlay approach has the advanta-ges of good safety,fewer complications for the infant with VSD.%目的:探讨镶嵌技术治疗婴幼儿室间隔缺损( VSD)的可行性与安全性。方法回顾性分析2010年6月至2013年12月解放军第四五四医院心胸外科采用镶嵌技术治疗279例婴幼儿 VSD的临床资料,按是否有体外循环参与分为非体外循环组(201例)、体外循环组(78例)。以同期常规体外循环下VSD修补手术65例作为对照组。对比3

  7. Hybrid procedure for infants with ventricular septal defect and coarctation of aorta: a review of 20 cases%“杂交”手术治疗20例小婴儿主动脉缩窄合并室间隔缺损

    Institute of Scientific and Technical Information of China (English)

    陈纲; 贾兵; 刘芳; 吴琳; 叶明; 成梦遇

    2012-01-01

    Objective To evaluate the early clinical efficaoy of hybrid procedure for infants less than six months old with ventricular septal defect and coarctation of aorta.Methods From January 2010 to July 2011,20 patients with ventricular septal defect and coarctation of aorta received hybrid procedure in our center.The body weight was (4.5 ± 1.6) kg ( ranged from 1.9 kg to 6.5 kg) and the age was ( 56 ± 45 ) days ( ranged from 18 days to 6 months).The pressure gradient of the coarctation of the aorta ranged from 30 mm Hg to 56 mm Hg,5 patients of them were diagnosed as hypoplasty of aortic arch.The size of the ventricular septal defect ranged from 8 mm to 16 mm.Results The mortality was zero in all the 20 cases during the surgery,and the mobidity was 20% (4/20).The complications were pneumonia in 2 cases,infective endocarditis in 1 case and pneumothorax in 1 case.The diameter of coarctation of the aorta ranged from 1.5 mm to 3.4 mm,and the size of the balloon ranged from 4 mm to 12 mm.The pressure gradient of the coarctation of the aorta decreased to 0 to 27 mm Hg.The bypass time ranged from 40 minutes to 87 minutes,and the crossclamp time of the aorta ranged from 20 minutes to 41minutes.The atrial septal defects were repaired and the patent ductuses were ligated during the surgery without leaving the sternum open.The total operation time was (4.0 ± 0.7 ) hours ( ranging from 3.0 hours to 5.2 hours).The mean ventilation time was (2.2 ± 1.4) days and mean ICU stay time was (5 ± 3 ) days.All the patients were followed up for ( 10.0 ± 3.6) months without aneurysm in arch and obstruction in airway.The residual obstructive pressurc gradicnt in the aortic arch ranged from 12 mm Hg to 35 mm Hg and 2 patients received reintervention.One patient received re-balloon dilation and the other received surgery.The cardiac function reached NYHA Ⅰ - Ⅱ in all eases.Conclusion The early outcome of the hybrid procedure (balloon dilation of the coartation of the aorta and surgical

  8. An endoscopic and anatomical approach to the septal papillary muscle of the conus.

    Science.gov (United States)

    Loukas, Marios; Tubbs, R Shane; Louis, Robert G; Apaydin, Nihal; Bartczak, Artur; Vefali, Huseng; Huseng, Vefali; Alsaiegh, Nada; Fudalej, Martin

    2009-11-01

    Many authors have questioned the gross anatomy of the septal papillary muscle of the conus known as the papillary muscle complex (PMC) during the past century. An anatomical investigation was conducted to identify the morphology and the topography of the PMC. Our study involved 200 formalin fixed adult human hearts. The PMC was present in 82% of the hearts, while in the remaining 18% of specimens, it was replaced by tendinous chords. The PMC was connected with the septal (59.7%), anterior (20.7%), or both septal and anterior leaflets (19.5%) with single (29.8%) or multiple chordae tendinae (70.1%). The PMC was also found to be present as a single papilla (51.8%), double papilla (32.9%) or triple papilla (15.2%). In addition to the PMC, we observed accessory single septal papillary muscles 42 specimens, double septal papillary muscles 32 specimens and triple septal papillary muscles 26 specimens. In the right ventricular inflow tract, the location of the PMC was consistently found to be in a position below the junction of the anterior and septal leaflets of the tricuspid valve. In the right ventricular outflow tract, we were able to identify 73 specimens in which the PMC was located at the junction formed superiorly by the inferior border of the subpulmonary infundibulum and inferiorly by the superior-lateral border of the septal band, extending into the region of the subpulmonary infundibulum. In the remaining 27%, the PMC was located primarily at the area occupied by the superiolateral border of the septal band without extending to the subpulmonary infundibulum. The present study describes the topography of the PMC according to its surrounding anatomical structures such as the tricuspid valve, subpulmonary infundibulum and septal band of the right ventricle. This anatomical data could have important clinical significance for cardiac surgeons operating in this area.

  9. Down Syndrome with Complete Atrioventricular Septal Defect, Hypertrophic Cardiomyopathy, and Pulmonary Vein Stenosis.

    Science.gov (United States)

    Mahadevaiah, Guruprasad; Gupta, Manoj; Ashwath, Ravi

    2015-10-01

    The prevalence of congenital heart disease in infants with Down syndrome is 40%, compared with 0.3% in children who have normal chromosomes. Atrioventricular and ventricular septal defects are often associated with chromosomal aberrations, such as in trisomy 21, whereas hypertrophic cardiomyopathy is chiefly thought to be secondary to specific gene mutations. We found only one reported case of congenital hypertrophic cardiomyopathy and atrioventricular septal defect in an infant with Down syndrome. Here, we report atrioventricular septal defect, hypertrophic cardiomyopathy, and pulmonary vein stenosis in a neonate with Down syndrome-an apparently unique combination. In addition, we discuss the relevant medical literature.

  10. First experiences with the GORE(®) Septal Occluder in children and adults with atrial septal defects.

    Science.gov (United States)

    Nyboe, Camilla; Hjortdal, Vibeke E; Nielsen-Kudsk, Jens Erik

    2013-11-15

    In this study, we report the first experiences with the GORE(®) Septal Occluder in transcatheter treatment of patients with atrial septal defects (ASD). Twenty-two patients with secundum ASD were selected for GORE(®) Septal Occluder device closure. Mean age was 25.8 ± 4.6 years (range 4-60), 10 were children below the age of 15. Closure was guided by transeosophageal or intracardiac echocardiography and transthoracic echocardiography was used at follow-up (median time 3 months). All defects were balloon sized and device size was chosen according to an occluder/defect size ratio ≥2. The mean ASD size was 11.4 ± 0.5 mm (range 6-15). All GORE(®) Septal Occluder sizes (15, 20, 25 and 30 mm) were used. Six patients had additional defects. The aortic rim was deficient in twelve patients and four patients had a septal aneurysm. Symptoms were seen in 17 patients and 19 had dilated right ventricles. Implantation was successful in all patients with no complications. Delivery was easy and intuitive by the new delivery system. None of the patients had residual shunts at follow-up. Mean procedure time was 38 ± 5 min (range 20-120). All patients had resolution or improvement in symptoms. There were no new onset arrhythmias or AV valve regurgitation and right ventricular dimensions were normalized. The GORE(®) Septal Occluder is an efficient device for closing ASDs up to 15 mm. It is feasible for defects with different anatomy including a deficient aortic rim, septal aneurysm and multiple fenestrations and due to its compliance and conformability it has a presumed low risk of erosion. Copyright © 2013 Wiley Periodicals, Inc.

  11. 单向活瓣补片治疗合并重度肺动脉高压双向分流型室间隔缺损%One - way - valved patch for repair of ventricular septal defect with severe pulmonary hypertension with Bi-directional shunt

    Institute of Scientific and Technical Information of China (English)

    邵双印

    2009-01-01

    目的 总结本院自2003年至2008年用自制单向活瓣补片治疗合并重度肺动脉高压双向分流型室间隔缺损15例的经验.方法 对收治的15例合并重度肺动脉高压双向分流的室间隔缺损患者行单向活瓣补片修补室间隔缺损.结果 本组15例病人无手术死亡,疗效满意,1周时有7例右向左分流,3周时右向左分流消失.随访3个月病人自觉症状改善,肺动脉压下降.结论 单向活瓣补片能降低肺动脉高压危象的发生率,降低围术期死亡率,围术期的管理也非常重要.%Objective To summerize the surgical effect with one - way - valved patch to treat vetricular septal de-feet with severe pulmonary hypertension with bi - directional shunt since 2003. Methods The materials of 15 patients with ventricular septal defect and severe PH with bi- directional shunt who were treated by one- way- valved patch were ana-lyzed. Results No patient died in hospital after operation due to pulmonary hypertention crisis and the low output of left ventrical. Most patients felt better than preoperation and their pulmonary artery pressure decreased gradually. Conclusions One -way -valved patch is useful in decreasing the rate of perioparative pulmonary hypertension crisis and perioperation mortality. The perioperative therapy and prioperative evaluation are also essential to raise surgical operation effect.

  12. Oblique septal crossbar graft for anterior septal angle reconstruction.

    Science.gov (United States)

    Aziz, Zeeshan S; Brenner, Michael J; Putman, Harrison C

    2010-01-01

    Nasal septal deformity is a central feature of the crooked nose, contributing to functional and aesthetic problems. Straightening of the septum often requires resection, scoring, or incision of the septum--maneuvers that inevitably weaken the cartilaginous dorsal and caudal L-shaped struts, which together are known as the L-strut. Compromise of this L-strut predisposes to septal buckling, recurrent deviation, and saddle nose deformity. We describe our experience with the oblique septal crossbar, a structural graft that allows biomechanically sound anterior septal angle reconstruction during septorhinoplasty. The technique improves dorsal septal support and facilitates correction of the crooked nose. The open septorhinoplasty approach and swinging door maneuver are followed by placement of a diagonally oriented crossbar graft, obtained from cartilage or the perpendicular plate. The approach allows consistent midline correction and buttressing of the nasal dorsum, with no complications to date.

  13. 组织多普勒技术定量评价陈旧性室间隔心肌梗死的右室功能%Right ventricular regional and global function in patients with old septal myocardial infarction by different techniques of tissue Doppler imaging

    Institute of Scientific and Technical Information of China (English)

    赵玉; 周晓彦; 蒋银花; 张民

    2012-01-01

    目的:采用不同组织多普勒技术定量研究陈旧性室间隔心肌梗死患者的右室局部和整体功能.方法:20例陈旧性室间隔心肌梗死患者,30例健康成人.采用应变率及组织速度成像获取右室侧壁各节段的组织速度、位移、应变及应变率曲线.测量收缩功能参数:收缩期峰值速度(Vs)、最大位移(D)、收缩期峰值应变(S)及应变率(SR);测量舒张功能参数:舒张早期充盈速度(Ve)、心房收缩期充盈速度(Va)、舒张早期充盈应变率( Esr)、心房收缩期充盈应变率(Asr).脉冲组织多普勒获取三尖瓣环运动频谱曲线,计算右室Tei指数.结果:陈旧性室间隔心肌梗死组右室侧壁基底段的S和D以及心尖段的SR和D均较正常组明显减低;基底段及中间段的Ve和Esr均较正常组明显减低;右室Tei指数明显高于正常组.结论:陈旧性室间隔心肌梗死患者右室侧壁多个节段的局部收缩和舒张功能明显受损,此外右室整体功能也明显受损.%Objective:To evaluate right ventricular (RV) regional and global function in patients with old septal myocar-dial infarction by different techniques of tissue Doppler imaging. Methods: The curves of RV lateral wall myocardial Doppler velocity, displacement, strain and strain rate were acquired by strain rate and tissue velocity imaging in 20 patients with old septal myocardial infarction and 30 healthy volunteers. Measured systolic function parameters were as fo Hows: the peak systolic velocity (Vs), displacement (D), strain (S) and strain rate (SR). Measured diastolic function parameters were listed below: the early diastolic velocity (Ve) , atrial systolic velocity (Va) , early diastolic strain rate (Esr) . At-rial systolic strain rate (Asr). Pulsed tissue Doppler sample volume was placed in tricuspid annulus and RV Tei index was calculated. Results: In old septal myocardial infarction groups, the S and D in basal segment and the SR and D in apical

  14. Intraoperative blood oxygenation continuous pulmonary artery perfusion lung protection of ventricular septal defect combined children with pulmonary hypertension%术中氧合血持续肺动脉灌注对室间隔缺损合并肺动脉高压患儿的肺保护作用

    Institute of Scientific and Technical Information of China (English)

    章晔; 李春玲; 胡建明; 林庆; 陈艰

    2014-01-01

    目的:研究在体外循环手术中使用氧合血进行持续肺动脉灌注对合并肺动脉高压的室间隔缺损患儿的肺保护作用。方法30例室间隔缺损合并肺动脉高压的患儿,随机均分为试验组和对照组。均在体外循环下进行室间隔缺损修补手术。试验组在体外循环中采用氧合血持续肺动脉灌注,对照组未行肺动脉灌注。两组患儿在体外循环前,主动脉开放后6、12、24h时抽取动脉血3ml.采用酶联免疫吸附法(ELISA法)检测丙二醛(MDA)的水平。并抽取动脉血进行血气分析,计算体外循环前,主动脉开放后6、12、24h时的氧合指数(QI),并记录气道峰压和呼吸机辅助时间,进行统计分析。结果试验组术后12、24h的 QI高于对照组(P<0.05),试验组术后6、12h的气道峰压明显低于对照组(P<0.05)。试验组术后呼吸机辅助时间明显低于对照组(P<0.05)。试验组术后6、12、24h时MDA水平均低于对照组(P<0.05)。结论氧合血持续肺动脉灌注能减轻室间隔缺损合并肺动脉高压患儿在体外循环中的肺损伤。%Objective To study the effects of blood oxygenation in extracorporeal circulation operation on continuous pul-monary artery perfusion of merger pulmonary hypertension of ventricular septal defect with pulmonary protection. Methods 30 cases of ventricular septal defect combined pulmonary hypertension were randomly divided into experimental group and control group,15 cases in each group. Both ventricular septal defect repair surgery under extracorporeal circulation. Patients with blood oxygenation in extracorporeal circulation continuous pulmonary artery perfusion, the control group no pulmonary artery perfusion. Two groups of children in front of the extracorporeal circulation, aorta open after 6,12 and 24 h when extracting arterial blood 3 ml. By using enzyme-linked immunosorbent (ELISA) to detect malondialdehyde (MDA

  15. Atrial Septal Defect (For Teens)

    Science.gov (United States)

    ... septal defect (pronounced: AY-tree-ul SEP-tul DEE-fekt), or ASD for short, is sometimes referred ... can be treated with cardiac catheterization (pronounced: CAR-dee-ack cath-uh-turr-ih-ZAY-shun), in ...

  16. Use of Nasal Septal Bone for Septal Extension Graft after Jaw Surgery

    Directory of Open Access Journals (Sweden)

    Gui Rak Kim, MD

    2013-11-01

    Conclusions: Use of septal bone for septal extension graft may serve as a valuable option in septal cartilage–depleted patients. It gives a strong construct for tip surgery with patient satisfaction.

  17. 经胸与经食管超声心动图在室间隔缺损外科封堵中的应用价值%APPLICATION VALUE OF TTE AND TEE IN DEVICE CLOSURCE OF VENTRICULAR SEPTAL DEFECTS

    Institute of Scientific and Technical Information of China (English)

    张小杉; 哈斯; 吴晓萍; 王晓磊; 朱宪明; 刘志平

    2012-01-01

    目的:评价经胸与经食管超声心动图在室间隔缺损外科封堵中的价值.方法:2009 - 08~2011 - 06经我院心外科确诊的室间隔缺损病人29例.膜周型缺损21例,嵴内型缺损8例.缺损直径3~9mm,平均5.35±3.12mm.术前用经胸超声心动图观察缺损大小及缺损与主动脉瓣的距离.在经食管超声的引导下,经右室游离壁置入国产室缺封堵器.术后用经食管超声即刻评价封堵效果,出院前及封堵3 mo后用经胸超声心动图随访观察.结果:25例封堵成功,使用等边封堵器18例,7例偏心封堵器,术后即刻超声及术后超声观察均无分流信号,2例病人术后出现少量三尖瓣反流.2例膜周型缺损TEE发现缺损处有多处破口无法进行封堵.2例嵴内型封堵失败,即刻术中行开胸修补术.结论:经胸与经食管超声心动图在室间隔缺损外科封堵术前评估、术中监测及术后随访中发挥重要的价值.%objective: To access the application value of TTE and TEE in device closurce of ventricular septal defects. Methods: From August 2009 to June 2011, A total of 29 patients diagnosised with VSD(17 males and 12 females) underwent TTE preoperative assessment in the cardial surgical department in our hospital. 21 cases with Perimembranous VSD,other 8 cases with intracristal type VSD. The defect diameter ranges from 3mm to 9mm,the average value is 5. 35 ± 3.12mm. Used TTE to access defect size and distance from the aortic valve in preoperative. Introduce domestic ventricular septal defect occluder through right ventricular free wall under the guidance of TEE. Immediate evaluate postoperative effect with TEE, TTE was used to follow - up study in the prior to dischargr and three months after. Results:The procedures were successful in 25 cases. Equilateral occluders were used in 18 cases, eccentric occluders in 7 cases. There was no signal of shunt observed by immediate postoperative TEE in the 25 cases,2 patients had mild

  18. Muscular Dystrophy

    Science.gov (United States)

    ... muscular dystrophy. It's important to be vaccinated for pneumonia and to keep up to date with influenza shots. Dietary changes haven't been shown to slow the progression of muscular dystrophy. But proper nutrition is essential because limited mobility can contribute to ...

  19. Septal splint with wax plates.

    Directory of Open Access Journals (Sweden)

    Nayak D

    1995-07-01

    Full Text Available To pack or not to pack, has always been a debate, especially after septal and functional endoscopic sinus surgery. The authors have studied the symptoms of packing versus not packing in their series of 100 patients having undergone nasal surgery. They advocate the use of dental wax for the fashioning of septal splints, since they are easy to introduce, cheap and malleable. The patients postoperative comfort is greatly enhanced with the use of dental wax plate splints instead of nasal packing.

  20. Left ventricular regional and global diastolic function assessed using Quantitative Tissue velocity Imaging in patients with hypertrophic cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    王良玉; 王新房; 谢明星; 蔡志雄; 陈纪平

    2003-01-01

    Objectives The study was performed to assess the left ventricular (LV) regional and global diastolic function、 left ventricular wall motion features in patients with Hypertrophic cardiomyopathy by Quantitative Tissue Velocity Imaging (QTVI).Methods 42 patients with hypertrophic cardiomyopathy and 36 age-matched normal subjects underwent QTVI study. Off-line LV regional muscular tissue velocity Imaging along LV aplcal long-axis view were obtained. Regional diastolic function was assessed in using peak tissue velocities of LV regional muscular tissue during early diastole (Ve)and LA contraction (Va) , Ve/Va ratio, derived from Tissue Velocity Imaging. Global diastolic function was reflected by isovolumic relaxation time(IRT) and mitral valve peak flow velocity ( E/A ) calculated with pulsed wave doppler.The end-diastolic interventricular septal thickness (IVSt) was measured by conventional 2 - dimension the segments of hypertrophic interventricular septum (IVS) reduced wlhile E/A ratio significantly reduced and IRT markedly prolonged in HCM patients than in duced in the segments of hypertrophic interventricular septum compared with other LV segments in HCM paE/A in HCM patients with abnormal E/A ratio (r = 0.Va and IVSt in non- obstruction HCM patients (B group , r = - 0.61 ) Conclusions QTVI offers a newer method in clinical practice which has a higher sensibility and accuracy in evaluating the LV regional and global diastolic function in HCM patients.

  1. Facts about Atrial Septal Defect

    Science.gov (United States)

    ... Living With Heart Defects Data & Statistics Tracking & Research Articles & Key Findings Free Materials Multimedia and Tools Links to Other Websites Information For... Media Policy Makers Facts about Atrial Septal Defect Language: English Español (Spanish) Recommend on Facebook Tweet Share Compartir ...

  2. 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.

  3. The clinical value of spatio-temporal image correlation in the diagnosis of fetal ventricular septal defect%四维超声时间-空间关联成像对孕期胎儿室间隔缺损诊断的临床价值

    Institute of Scientific and Technical Information of China (English)

    贾立娟; 王智培

    2016-01-01

    Objective To explore the clinical value of four-dimensional ultrasound spatio-temporal correlation image (STIC) in the identification of the diagnosis room septal defect (VSD) in prenatal.Method Selected Court Ultrasound 2011 to 2015 by the two-dimensional ultrasound screening technology and STIC get 38 cases of fetal VSD were retrospectively analyzed to or after the final autopsy results or postnatal follow-up results as the gold standard to calculate two STIC-dimensional ultrasound and clinical diagnostic value of the VSD.Result STIC sample inspection time for each case, each image to obtain the required time were significantly shorter than the two-dimensional ultrasound (P<0.05); 36 patients were diagnosed VSD fetuses, STIC correctly diagnosed 35 cases, one case of missed diagnosis, misdiagnosis does not appear case; correct dimensional ultrasound fetal diagnosis VSD in 28 cases, 8 cases of missed diagnosis, one case of misdiagnosis; calculated according to table 2 to give STIC fetal diagnosis VSD sensitivity of 97.22%, specificity of 100%, misdiagnosis rate was 2.78%, misdiagnosis rate 0.00%; two-dimensional ultrasound diagnosis of fetal VSD sensitivity of 77.78% and a specificity of 50.00%, 22.22% rate of missed diagnosis, misdiagnosis rate was 50.00%.ConclusionPrenatal screening for VSD application STIC technology has the advantages of fast and accurate.%目的探讨四维超声时间-空间关联成像(spatio-temporal image correlation,STIC)技术在产前胎检中鉴别诊断室间隔缺损(ventricular septal defect,VSD)的临床价值。方法选取本院超声科2011年3月至2015年4月经二维超声和STIC技术筛查得到的38例VSD胎儿资料进行回顾性分析,以最终引产后尸体解剖结果或出生后随访结果作为金标准,分析二维超声和STIC诊断VSD的临床价值。结果 STIC对每例样本检查时间、获取每幅图像所需要时间均显著短于二维超声(P<0.05);36例

  4. 低龄低体重患儿经胸小切口封堵膜周部室间隔缺损的策略及早期疗效%Strategy and Short-term Outcomes of Transthoraic Occlusion via a Small Incision in Low Birth-weight Infants with Ventricular Septal Defect

    Institute of Scientific and Technical Information of China (English)

    李小波; 万亚红; 邬云龙; 肖明第

    2012-01-01

    To study the surgical techniques and efficacy of transthoracic occlusion through a small chest incision for low birth-weight infants with ventricular septal defect (VSD). Methods Totally 51 low birth-weight infants with VSD, who received transthoracic occlusion in our hospital during October 2010 to November 2011, were enrolled into this study. The shunt diameter was 4-8 mm, as shown by echocardiography, and was over 1 mm away from the aortic valve. The patients aged from 4 to 12 months with a mean of (8.6 ±2. 1) months; the mean body weight was (7.4 ±2. 1) kg (ranged from 6 to 11 kg). Via a 4- to 5-mm incision below the sternum, under the guidance by TEE, we introduced a guide wire through the right ventricular anterior wall, and then placed occlusion device at the VSD site. The surgical procedure and efficacy was analyzed retrospectively afterwards. Results No mortality occurred during peri-operative period. The success rate of transthoracic occlusion was 90% (46/51). The five patients, who failed in the procedure, were transferred to cardiopulmonary bypass (CPB) for a VSD repair in a direct surgical field. In the 46 successful cases, no residual leakage, dislocation of occlusion device, or arrhythmia occurred; echocardiography showed no aortic regurgitation, however, 5 (13%) patients developed mild tricuspid valve regurgitation. Three patients (5. 8%) received blood transfusion of 50 ml. The patients were discharged from hospital in a mean of (3. 9 ± 0. 7) days (ranged from 3 to 7 days). Afterwards, all the 46 successful cases were followed up for (3. 6 ± 1. 4) months ( ranged from 1 to 12 months) , during which no patient died,and ECG, echocardiography, and chest X-ray found nothing abnormal, the cardiac faction was in grade I in all the cases. Conclusions For low birth-weight infants with VSD with a shunt diameter of 4 - 8 mm, and > 1 mm away from the aortic valve, transthoracic occlusion through a small chest incision is feasible and effective.%目的

  5. Dismorfia muscular

    OpenAIRE

    Assunção,Sheila Seleri Marques

    2002-01-01

    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 dismo...

  6. Surgical correction of hypertrophic obstructive cardiomyopathy in a patient with severe hypertrophy and septal myocardial fibrosis.

    Science.gov (United States)

    Borisov, Konstantin Valentinovitch

    2012-10-01

    In patients with hypertrophic cardiomyopathy, myocardial fibrosis is an independent predictor of an adverse outcome. A new technique of hypertrophic obstructive cardiomyopathy (HOCM) surgical correction in patients with severe hypertrophy and septal myocardial fibrosis has been proposed. This approach avoids mechanical damage to the heart conduction system, and for the surgeon it improves visual inspection of the area to be resected. We present a case report of a 33-year old female patient with biventricular obstruction, extreme hypertrophy, septal myocardial fibrosis and episodes of ventricular tachycardia who underwent surgical correction according to this novel procedure. The advantage of the approach is an effective surgical treatment of HOCM in patients with severe hypertrophy and septal myocardial fibrosis who cannot be treated with the current surgical techniques.

  7. Dorsal Augmentation with Septal Cartilage

    OpenAIRE

    Murrell, George L.

    2008-01-01

    Deficiency of nasal dorsal projection may be inherent or acquired. Repair is most commonly performed with an onlay graft. When nasal septal cartilage is available, it is the author's preferred source for graft material. It is important to realize that dorsal augmentation is an operation performed for aesthetic not functional reasons. As such, patients understandably scrutinize their postoperative result, and attention to detail in all aspects of the surgery is critical in achieving a favorabl...

  8. Muscular Dystrophy

    Science.gov (United States)

    ... It Like for Teens With MD? en español Distrofia muscular Aside from seeing the telethon on Labor Day ... and rule out other diseases that affect the muscles or nerves. Some tests measure how nerves and muscles are working. Others ...

  9. Muscular Dystrophy

    Science.gov (United States)

    Muscular dystrophy (MD) is a group of more than 30 inherited diseases. They all cause muscle weakness and muscle loss. Some forms of MD appear in infancy ... types can vary in whom they affect, which muscles they affect, and what the symptoms are. All ...

  10. Is there any association between childhood cardiac septal defects and ROCK2 gene polymorphism?

    Science.gov (United States)

    Aksoy, M; Uygun, H; Baspinar, O; Demiryurek, S; Oztuzcu, S; Cengiz, B; Irdem, A; Araz, N C

    2014-03-17

    Rho/Rho-kinase pathway plays a critical role in the regulation of cellular functions such as proliferation and migration. One of the possible theories of the development of ventricular septal defects is cell migration disorder. The aim of this study was to analyze the genotype distributions and allele frequencies for the ROCK2 gene Thr431Asn polymorphisms in the development of cardiac septal defects in a Turkish population. In this case-control study, 300 patients with cardiac defects (150 patients with ventricular and 150 patients with atrial septal defects) and control group (150 healthy control subjects) were investigated. A single-nucleotide polymorphism in ROCK2 gene Thr431Asn was analyzed by real-time PCR using a Light-Cycler. Neither genotype distributions nor the allele frequencies for the Thr431Asn polymorphism showed a significant difference between the groups. These results suggest that there is no association of the ROCK2 gene Thr431Asn polymorphism with the development of cardiac septal defects in pediatric patients.

  11. Virtual Cardiac Surgery Using CFD: Application to Septal Myectomy in Obstructive Hypertrophic Cardiomyopathy

    Science.gov (United States)

    Vedula, Vijay; Mittal, Rajat; Abraham, Theodore

    2011-11-01

    Obstructive hypertrophic cardiomyopathy (HOCM) is characterized by ventricular wall thickening, diastolic dysfunction, and dynamic outflow tract obstruction, all of which strongly influence the vortex dynamics and pressure distribution in the left ventricle (LV). Severe cases of HCM are usually managed through septal myectomy where the surgeon resects the hypertrophic mass. Surgeons currently try to remove as much tissue as possible in order to optimize the post surgical result. However, excessive debulking increases the chance of ventricular septal defects, bundle branch block or complete heart block, and aneurysmal septal thinning. On the other hand, insufficient tissue removal also leads to unsatisfactory outcomes in terms of reduction of outflow tract pressure gradient. Knowing how much muscle to remove and where to remove it from could reduce the likelihood of complications and suboptimal outcomes. In the present study, we employ an immersed boundary solver to model the effect of septal myectomy for ventricles with HOCM and demonstrate the potential of such an approach for surgical planning. Computational resources were provided by the National Institute of Computational Science under Tergrid grant number TG-CTS100002.

  12. Short-and Mid-term Follow-up Study on Tricuspid Valve Detachment in Correction of Ventricular Septal Defect%三尖瓣适当切开修补室间隔缺损的近中期随访研究

    Institute of Scientific and Technical Information of China (English)

    陈仁伟; 罗金文; 刘剑; 黄鹏; 曾德斌; 刘平波; 周文武

    2012-01-01

    目的 探讨三尖瓣适当切开技术(tricuspid valve detachment,TVD)在室间隔缺损(ventricular septal defect,VSD)修补手术中的临床价值. 方法 回顾性分析我院2006年1月~2010年12月行VSD修补的1 021例患者的临床资料,其中211例采用TVD技术(TVD组),810例未采用TVD技术(非TVD组).所有患者在术前与术后3d、1个月、6个月及1年行超声心动图观察心脏结构,评估三尖瓣返流(tricuspic regurgitation,TR)情况. 结果 695例补片修补VSD,其中TVD组209例(128例使用单独缝线修补瓣叶切口,81例使用VSD补片缝线),非TVD组486例;另326例直接缝合VSD,其中TVD 组2例(使用VSD补片缝线直接修补瓣叶切口),非TVD组324例.671例进行了合并畸形手术,其中TVD组115例(占TVD组54.5%),非TVD组556例(占非TVD组68.6%).两组CPB时间、主动脉阻断时间及术后住院时间比较,均无统计学差异(P>0.05).两组均无围手术期死亡病例.TVD组无完全性房室传导阻滞出现,非TVD组出现3例,均于术后10 d内恢复窦性.术后3 d VSD残余漏11例,其中TVD组3例,非TVD组8例;TVD组轻度以上的TR病例14例,非TVD组有47例.两组均无因TR和残余漏而再手术者.两组术后并发症发生率比较无统计学差异(8.1% VS 7.2%,P>0.05).所有病人随访1年,无远期死亡病例,均无残余漏需远期再手术者.术后1年TVD组无轻度以上TR,非TVD组有8例轻度以上TR,组间比较差异无统计学意义(P>0.05).两组均无因TR再手术者.结论 TVD是一种安全、有效的技术,能改善VSD的暴露,与传导阻滞、手术时间延长以及TR无相关性.TVD能保护三尖瓣瓣叶结构,减少术后严重TR的发生.%Objective To investigate the clinical value of temporary tricuspid valve detachment (TVD) in the correlation of ventricular septal defect (VSD). Methods A total of 1 021 VSD patients admitted January 2006 to December 2010 in our hospital were analyzed retrospectively, including 211 of TVD

  13. Occurrence and outcome of tricuspid regurgitation after transcatheter closure of perimembranous ventricular septal defect%膜周部室间隔缺损经导管封堵术后三尖瓣反流的发生与转归

    Institute of Scientific and Technical Information of China (English)

    马进举; 姚青; 宋治远; 仝识非; 景涛; 李永华; 张倩; 张志辉; 李华康

    2013-01-01

    目的 探讨膜周部室间隔缺损(perimembranous ventricular septal defect,PMVSD)经导管封堵术后三尖瓣反流的发生与转归.方法 选取2007年1月至2012年3月在我院心内科诊断为PMVSD并成功行经导管介入封堵治疗的患者332例,其中男182例,年龄(15.4±12.3)岁.分别在经导管封堵术前、术后3天及1、3、6、12个月行经胸超声心动图(transthoracic echocardiography,TTE)检查,观察有无三尖瓣反流及其程度.并根据所用封堵器类型分为2组:对称型封堵器组和细腰型封堵器组,并比较两组三尖瓣反流的发生率.结果 332例患者中181例使用对称型封堵器,另151例使用细腰型封堵器.术后共有14例(4.2%)新出现三尖瓣反流或原有三尖瓣反流加重,其中对称型封堵器组8例(4.4%),细腰型封堵器组6例(4.0%),二者之间无明显差异(P=0.84);随访时间(12.1±7.9)个月,有3例患者三尖瓣反流程度较出院时减轻.无因三尖瓣腱索断裂或瓣膜毁损导致三尖瓣关闭不全的患者.结论 PMVSD经导管封堵术后少数患者可发生三尖瓣反流,其发生与封堵器类型无关,反流程度一般不随时间推移而加重,预后较好.%Objective To investigate the occurrence and outcome of tricuspid regurgitation after transcatheter closure of perimembranous ventricular septal defect (PMVSD).Methods From January 2007 to March 2012,332 patients diagnosed as PMVSD and successfully underwent transcatheter interventional therapy in the Southwest Hospital were enrolled,including 182 male patients with an average age of 15.4 ± 12.3 years.Transthoracic echocardiography (TTE) was applied to observe the tricuspid regurgitation and its degree before procedure,in 3 d after procedure,and during 1-,3-,6-and 12-month follow-up.The patients were divided into two groups based on the devices applied:a symmetric occluder group and a small-waist occluder group,and the incidence of tricuspid regurgitation in the two groups were

  14. Ebstein's Anomaly, Left Ventricular Noncompaction, and Sudden Cardiac Death

    Science.gov (United States)

    McGee, Michael; Warner, Luke; Collins, Nicholas

    2015-01-01

    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. PMID:26240764

  15. Natural history of ventricular septal defects in Nigerian children

    African Journals Online (AJOL)

    Paediatric Cardiology Unit, Department of Child Health, University of Benin Teaching Hospital, Benin City, Edo State, Nigeria .... low SES (26 – 42.6%) than middle (24 – 39.3%) or high SES. (11 – 18.0%). .... syndrome over time. Meanwhile ...

  16. Muscular disease

    Institute of Scientific and Technical Information of China (English)

    1993-01-01

    930186 The diagnostic value of MRI on neuro-muscular disease.CHEN Qingtang(陈清棠),etal.Dept Neurol,1st Hosp,Beijing Med Univ,100034.Chin J Neurol & Psychiat 1992;25(5):267-269.The article concentrated on the study ofskeletal muscles of four extremities in 12 casesof different kinds of neuromuscular diseases and4 volunteers with MRI.The results revealed:MRI could clearly display individual muscle,muscle groups or abnormal muscles morphologi-

  17. Right heart function assessment with real-time three-dimensional echocardiography before and after atrial septal defect surgery

    Institute of Scientific and Technical Information of China (English)

    Hua Zhao; Yi-Min Fu; Yong-Mei Jia

    2016-01-01

    Objective:To study the clinical value of right heart function assessment with real-time three-dimensional echocardiography before and after atrial septal defect surgery. Methods:Patients with atrial septal defect who received transcatheter closure in our hospital were selected for study and divided into non-pulmonary hypertension group and pulmonary hypertension group according to pulmonary artery systolic pressure (PASP), real-time three-dimensional echocardiography was conducted before and after operation, and right ventricular end-diastolic volume (RVEDV), right ventricular end-systolic volume (RVESV), right ventricular stroke volume (RVSV), right ventricular ejection fraction (RVEF) and right ventricular cardiac output (RVCO) were calculated;serum was collected, and brain natriuretic peptide (BNP), atrial natriuretic peptide (ANP), angiotensin I (AngI), angiotensin II (AngII) and endothelin (ET) contents were detected. Results:After operation, RVEDV, RVESV, RVSV, RVEF and RVCO as well as serum BNP, ANP, AngI, AngII and ET contents of both groups were lower than those before treatment;RVEDV, RVESV, RVSV, RVEF and RVCO were positively correlated with contents of BNP, ANP, AngI, AngII and ET. Conclusions:Using real-time three-dimensional echocardiography before and after atrial septal defect surgery can accurately assess right heart function, and it has good correlation with right heart volume load and pulmonary circulation blood flow.

  18. Atrial septal stenting - How I do it?

    Directory of Open Access Journals (Sweden)

    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.

  19. Effects of VAT and DDD pacing on left cardiac function in the patients of high right ventricular septal pacing with third degree atrioventricular block%Ⅲ度房室传导阻滞患者高位右室间隔部起搏VAT与DDD模式对左心功能的影响

    Institute of Scientific and Technical Information of China (English)

    王燕; 章蓉; 王旭; 邵一兵

    2012-01-01

    目的 通过研究Ⅲ度房室传导阻滞患者高位右室间隔部起搏( HRVS)时,VAT与DDD模式对心功能的影响,探讨心房的生理收缩和舒张对左心功能的作用.方法 32例Ⅲ度房室传导阻滞患者,在HRVS时,分别给予DDD模式或VAT模式工作,于调控即刻通过超声心动图测定二尖瓣口快速充盈期峰值血流速度(Ep),二尖瓣口左房收缩期峰值血流速度(Ap),二尖瓣环后壁处收缩期脉冲组织多普勒峰值速度平均值(Vs),二尖瓣环后壁处舒张早期脉冲组织多普勒峰值速度平均值(Ve),二尖瓣环后壁处舒张晚期脉冲组织多普勒峰值速度平均值(Va),二尖瓣血流频谱等容舒张时间(IVRT),通过Ep/Ap,Ve/Va,Ep/Ve和IVRT评价左室舒张功能,通过Vs,LVEF和LVFS评价收缩功能.结果 HRVS时DDD较VAT模式Ep/Ap、Ve/Va、Ep/Ve,IVRT差异有显著性(0.97 ±0.11 vs 1.01±0.11,0.89 ±0.09 vs 0.97 ±0.07,6.00 ±0.45 vs 6.24 ±0.36,100.4±14.32 vs 89.99 ±7.94;P均<0.01),Vs、左室射血分数和左室短轴缩短率无显著性差异.结论 Ⅲ度房室传导阻滞时,HRVS起搏时,DDD模式较VAT模式使左室舒张功能下降,收缩功能无影响,表明了心房生理性起搏的重要性.%Objective To analyze the effects of physiologic systole and diastole of atrium, through the influence of VAT and DDD pacing mode on left cardiac function in patients of high right ventricular septal( HRVS) pacing with third degree atrioventricular block. Methods Thirty-two patients of HRVS pacing with third degree atrioventricular block with pacemaker mode of VAT or DDD underwent complete standard echocardiography (2D,M-mode, pulsed Doppler) and PW-DTI. Transmitral inflow velocity Ep and Ap, peak systolic velocity ( Vs ) , peak early diastolic velocity ( Ve) , peak late diastolic velocity (Va) of posterior mitral annulus and the isovolumic relaxation time(IVRT) were obtained . Then left ventricular diastolic function were evaluated through Ep/Ap, Ve/Va and Ep

  20. 经食管超声心动图引导下微创小切口室间隔缺损封堵治疗%Transcatheter closure of ventricular septal defects through minimally invasive port -access surgery guided by transesophageal echocardiography

    Institute of Scientific and Technical Information of China (English)

    侯永波; 王国祥; 谭胜; 刘金东

    2015-01-01

    目的:总结经食管超声心动图(TEE)引导下,微创经胸室间隔缺损(VSD)封堵治疗的临床经验。方法11例 VSD 患者接受微创经胸封堵治疗,年龄3~6岁,体重12~65 kg,VSD 直径3~8 mm。手术在 TEE 引导,胸骨下段小切口微创下进行,于右心室表面选择适当的穿刺点,建立输送轨道并完成 VSD 封堵。术后密切随访1~6个月。结果11例中9例一次性安放成功,2例首次安放有残余分流,术中更换大号伞;心内操作时间10~36 min,平均(21±12)min。全部患者均于术后4~5天出院,随访1~6个月,效果良好。11例患者均无心律失常、残余分流等并发症发生。结论TEE 引导下微创经胸 VSD 封堵是一种安全有效的治疗方法,但远期结果有待进一步观察。%Abstrat: Objectve To summarize the clinical experience of transcatheter closure of ventricular septal defects (VSD) through minimally invasive port -access surgery guided by transesophageal echocardiography (TEE).Methods A total of 11 patients aged from 3 to 6, weighting 12 kg to 65 kg were subjected to transcatheter closure of VSD with a diameter ranging from 3 mm to 8 mm.The surgery was performed under the guidance of TEE, where a small transthoracic incision was made.A proper site was chosen at the surface of the right ventricle for punctuation, in order to establish de-livery pathway.Patients were closely followed up for 1 to 6 months.Results Among the patients were nice ones who successfully completed the surgery at one time.Residual shunts were seen in two cases which required the replacement of larger occluders.The time of intracardiac procedures was 10 -36 min [(21 ±12)min].All patients were discharged 4 to 5 days after operation.During fellow -up period, neither arrhythmia nor residual shunt occurred.Conclusion Tran-sthoracic minimally invasive closure is a safe and efficient method for the treatment of VSD.Meanwhile, long -term fel

  1. Cardiac function in types II and III spinal muscular atrophy: should we change standards of care?

    Science.gov (United States)

    Bianco, Flaviana; Pane, Marika; D'Amico, Adele; Messina, Sonia; Delogu, Angelica Bibiana; Soraru, Gianni; Pera, Maria Carmela; Mongini, Tiziana; Politano, Luisa; Baranello, Giovanni; Vita, Gianluca; Tiziano, Francesco Danilo; Morandi, Lucia; Bertini, Enrico; Mercuri, Eugenio

    2015-02-01

    In the last years, there has been increasing evidence of cardiac involvement in spinal muscular atrophy (SMA). Autonomic dysfunction has been reported in animal models and in several patients with types I and III SMA, these findings raising the question whether heart rate should be routinely investigated in all SMA patients. The aim of our study was to detect possible signs of autonomic dysfunction and, more generally, of cardiac involvement in types II and III SMA. We retrospectively reviewed 24-hour electrocardiography (ECG) in 157 types II and III SMA patients (age range, 2-74 years). Of them, 82 also had echocardiography. None of the patients had signs of bradycardia, atrial fibrillation, or the other previously reported rhythm disturbances regardless of the age at examination or the type of SMA. Echocardiography was also normal. There were no signs of congenital cardiac defects with the exception of one patient with a history of ventricular septal defects. Our results suggest that cardiac abnormalities are not common in type II and type III SMA. These findings provide no evidence to support a more accurate cardiac surveillance or changes in the existing standards of care. Georg Thieme Verlag KG Stuttgart · New York.

  2. Regional septal hinge-point injury contributes to adverse biventricular interactions in pulmonary hypertension.

    Science.gov (United States)

    Nielsen, Eva Amalie; Okumura, Kenichi; Sun, Mei; Hjortdal, Vibeke E; Redington, Andrew N; Friedberg, Mark K

    2017-07-01

    Death and morbidity in pulmonary arterial hypertension (PAH) are often due to right ventricular (RV) failure and associated left ventricular (LV) dysfunction. We investigated regional myocardial remodeling and function as the basis for adverse ventricular-ventricular interactions in experimental chronic RV pressure overload. Two distinct animal models were studied: A rabbit model of increased RV pressure-load through progressive pulmonary artery banding A rat model of monocrotaline (MCT)-induced pulmonary arterial hypertension (PAH). Regional myocardial function was assessed by speckle-tracking strain echocardiography and ventricular pressures measured by catheterization before termination. Regional RV and LV myocardium was analyzed for collagen content, apoptosis and pro-fibrotic signaling gene and protein expression. Although the RV developed more fibrosis than the LV; in both models the LV was substantially affected. In both ventricles, particularly the LV, fibrosis developed predominantly at the septal hinge-point regions in association with decreased regional and global circumferential strain, reduced global RV and LV function and up-regulation of regional transforming growth factor-β1 (TGFβ1) and apoptosis signaling. A group of PAH rats who received the TGFβ blocker SB431542 showed improved RV function and reduced regional hinge-point myocardial fibrosis. RV pressure-loading and PAH lead to biventricular TGFβ1 signaling, fibrosis and apoptosis, predominantly at the septal hinge-point regions, in association with regional myocardial dysfunction. This suggests that altered geometry and wall stress lead to adverse RV-LV interactions through the septal hinge-points to induce LV fibrosis and dysfunction. © 2017 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of The Physiological Society and the American Physiological Society.

  3. INFERIOR-SEPTAL MYOCARDIAL INFARCTION MISDIAGNOSED AS ANTERIOR-SEPTAL MYOCARDIAL INFARCTION: ELECTROCARDIOGRAPHIC, SCINTIGRAPHIC, AND ANGIOGRAPHIC CORRELATIONS

    Institute of Scientific and Technical Information of China (English)

    Ji-lin Chen; Zuo-xiang He; Zai-jia Chen; Jin-qing Yuan; Yue-qin Tian; Shu-bin Qiao; Rong-fang Shi; Yi-da Tang; Zong-lang Lu

    2007-01-01

    To explore the infarct sites in patients with inferior wall acute myocardial infarction (AMI) concomitant with ST segment elevation in leads V1-V3 and leads V3R-V5R.Methods Five patients diagnosed as inferior, right ventricular, and anteroseptal walls AMI at admission were enrolled. Electrocardiographic data and results of isotope 99mTc-methoxyisobutylisonitrile (MIBI) myocardial perfusion imaging and coronary angiography (CAG) were analyzed.Results Electrocardiogram showed that ST segment significantly elevated in standard leads Ⅱ, Ⅲ, aVF, and leads V1-V3 , V3R-V5R in all five patients. The magnitude of ST segment elevation was maximal in lead V1 and decreased gradually from lead V1 to V3 and from lead V1 to V3R-V5R. There was isotope 99mTc-MIBI myocardial perfusion imaging defect in inferior and basal inferior-septal walls. CAG showed that right coronary artery was infarct-related artery.Conclusions The diagnostic criteria for basal inferior-septal wall AMI can be formulated as follows: ( 1 ) ST segment elevates ≥2 mm in lead V1 in the clinical setting of inferior wall AMI; (2) the magnitude of ST segment elevation is the tallest in lead V1 and decreases gradually from lead V1 to V3 and from lead V1 to V3R-V5R. With two conditions above, the basal inferior-septal wall AMI should be diagnosed.

  4. Learning about Spinal Muscular Atrophy

    Science.gov (United States)

    ... News Release Fischbeck Group Learning About Spinal Muscular Atrophy What is spinal muscular atrophy? What are the ... for Spinal Muscular Atrophy What is spinal muscular atrophy? Spinal muscular atrophy is a group of inherited ...

  5. Septal Junctions in Filamentous Heterocyst-Forming Cyanobacteria.

    Science.gov (United States)

    Flores, Enrique; Herrero, Antonia; Forchhammer, Karl; Maldener, Iris

    2016-02-01

    In the filaments of heterocyst-forming cyanobacteria, septal junctions that traverse the septal peptidoglycan join adjacent cells, allowing intercellular communication. Perforations in the septal peptidoglycan have been observed, and proteins involved in the formation of such perforations and putative protein components of the septal junctions have been identified, but their relationships are debated.

  6. Spinal Muscular Atrophy (SMA)

    Science.gov (United States)

    ... Your 1- to 2-Year-Old Spinal Muscular Atrophy (SMA) KidsHealth > For Parents > Spinal Muscular Atrophy (SMA) A A A What's in this article? ... Outlook en español Atrofia muscular espinal Spinal muscular atrophy, or SMA, is an inherited condition that causes ...

  7. Cavopulmonary Anastomosis in a Patient With Arrhythmogenic Right Ventricular Cardiomyopathy With Severe Right Ventricular Dysfunction.

    Science.gov (United States)

    Vaidyanathan, Swaminathan; Kothandam, Sivakumar; Kumar, Rajesh; Indrajith, Sujatha Desai; Agarwal, Ravi

    2017-01-01

    A 26-year-old lady presented with exertional dyspnea, palpitations, central cyanosis, and oxygen saturations of 80% in room air. Her electrocardiogram, echocardiogram, and cardiac magnetic resonance were diagnostic of arrhythmogenic right ventricular dysplasia. There was no documented ventricular arrhythmia or syncopal episodes and Holter recordings were repeatedly normal. Cardiac hemodynamics showed right to left shunt through atrial septal defect, low pulmonary blood flow, normal atrial pressures, and minimally elevated right ventricular end-diastolic pressures. Since her presenting symptoms and cyanosis were attributed to reduced pulmonary blood flow, she underwent off-pump cavopulmonary anastomosis between right superior vena cava and right pulmonary artery. As we intended to avoid the adverse effect of extracorporeal circulation on the myocardial function and pulmonary vasculature, we did not attempt to reduce the size of the atrial septal defect. Her postoperative period was uneventful; oxygen saturation improved to 89% with significant improvement in effort tolerance. At 18-month follow-up, there were no ventricular arrhythmias on surveillance. The clinical presentation of this disease may vary from serious arrhythmias warranting defibrillators and electrical ablations at one end to right ventricular pump failure warranting cardiomyoplasty or right ventricular exclusion procedures at the other end. However, when the presentation was unusual with severe cyanosis through a stretched foramen ovale leading to reduced pulmonary blood flows, Glenn shunt served as a good palliation and should be considered as one of the options in such patients.

  8. PERCUTANEOUS CLOSURE OF SECUNDUM-TYPE ATRIAL SEPTAL DEFECTS USING AMPLATZER SEPTAL OCCLUDERS

    Directory of Open Access Journals (Sweden)

    Uroš Mazić

    2003-01-01

    Full Text Available Background. Percutaneous closure of secundumtype atrial septal defect (ASD II. is becoming an increasingly widespread alternative to surgical closure. We report our initial clinical experience with the percutaneous closure of ASD II. using Amplatzer Septal Occluders (ASO in Slovenia.Patients and methods. Fifty consecutive patients with ASD II. were evaluated for transcatheter closure with ASO using both transthoracic (TTE and transesophageal echocardiography (TEE. Transcatheter closure was performed under general endotracheal anesthesia with simultaneous fluoroscopy and TEE guidance. The stretch defect diameter was measured using an Amplatzer sizing balloon catheter. The ASO having a 2–4 mm larger diameter than the stretched defect diameter was selected for defect closure. Follow-up was scheduled 10 min, 24 hours, 1 month, 3 months, 6 months, 1 year and then annually after the procedure.Results. Eight patients (16% with deficiency of the posterior, inferior anterior, or inferior posterior rim were not deemed suitable for transcatheter closure and were referred for surgery. Fourteen patients (28% had centrally positioned defects, 23 patients (46% defects with a deficient superior anterior rim, 3 patients had multiple defects, while 2 patients presented with atrial septal aneurysm: 1 associated with a single perforation and 1 with multiple perforations. So far, cardiac catheterization has been performed in 24 patients, while the remaining 12 patients are waiting cardiac catheterization. During cardiac catheterization, 1 additional patient was excluded from percutaneous closure due to an additional defect unsuitable for percutaneous closure. Therefore, percutaneous closure was attempted in 23 patients. In a single patient we retrieved an inadequately positioned occluder and referred the patient for elective surgical closure. A large Eustachian valve caused this single failure of occluder positioning in our series. In the remaining 22 patients

  9. 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.

  10. Ventricular assist device

    Science.gov (United States)

    VAD; RVAD; LVAD; BVAD; Right ventricular assist device; Left ventricular assist device; Biventricular assist device; Heart pump; Left ventricular assist system; LVAS; Implantable ventricular assist device

  11. Influence of Septal Thickness on the Clinical Outcome After Alcohol Septal Alation in Hypertrophic Cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten K; Jacobsson, Linda; Almaas, Vibeke;

    2016-01-01

    BACKGROUND: We assessed the influence of interventricular septal thickness (IVSd) on the clinical outcome and survival after alcohol septal ablation (ASA) in patient with hypertrophic cardiomyopathy. METHODS AND RESULTS: We analyzed 531 patients with hypertrophic cardiomyopathy (age: 56±14 years...

  12. Asymmetric left ventricular hypertrophy associated with morbid obesity mimicking familial hypertrophic cardiomyopathy.

    Science.gov (United States)

    Wong, Raymond Ching-Chiew; Tan, Kong Bing

    2014-12-01

    Asymmetric septal hypertrophy with systolic anterior motion of the mitral valve is frequently a phenotypic, but not pathognomonic, expression of genetic hypertrophic cardiomyopathy (HCM) with or without obstruction. It can, however, be associated nonspecifically with other forms of increased left ventricular (LV) afterload. We herein report the case of a young man with obesity cardiomyopathy and heart failure who presented with asymmetric septal hypertrophy and marked LV hypertrophy, and endomyocardial biopsy ruled out genetic HCM.

  13. The pulmonary vascular blood supply in the pulmonary atresia with ventricular septal defect and its implications in surgical treatment O suprimento sangüíneo vascular pulmonar na atresia pulmonar com comunicação interventricular e suas implicações no tratamento cirúrgico

    Directory of Open Access Journals (Sweden)

    Ulisses Alexandre Croti

    2003-10-01

    Full Text Available OBJECTIVE: With base in the studies cineangiocardiography of pacients with pulmonary atresia (PA with ventricular septal defect (VSD, to identify in the groups proposed by BARBERO MARCIAL, subgroups with similar morphological characteristics, to measure their central pulmonary arteries (CPA and major aortopulmonary collateral arteries (MAPCA, thereby establishing their implications in surgical treatment. METHOD: Sixty three patients were classified in groups A (15, B (40 and C (8 between january 1990 and june 2001. Patients with complete cineangiocardiograms prior to the first surgical intervention were included in this study, being calculated the pulmonary arterial index (PAI, the major aortopulmonary collateral arterial index (MAPCAI and the total neopulmonary arterial index (TNPAI = PAI + MAPCAI. Surgical treatment was considered palliative (PT, definitively palliative (DPT and definitive (DT. RESULTS: Nine subgroups were identified, A (A1 and A2, B (B1, B2, B3, B4 and B5 and C (C1 and C2. In group A, the PAI of patients for DT was higher than for PT patients (p=0,0092. In group B, the TNPAI of DT patients was greater than for PT patients (p=0,0959. In group C, the MAPCAI in DPT patients was lower than in PT and DT patients. In the group A was not mortality, in the group B was of 17,5% and in the group C was of 12,5%. CONCLUSIONS:Among the groups A, B e C was possible to identifiy nine subgroups, the morphologic and morphometric characteristics allowed to suggest the surgical treatment in the patients of the group A had larger chance of TD, the group B of TP and the group C of TPD. The mortality presented larger correlation with the morphologic characteristics that with the morphometric.OBJETIVO: Com base nos estudos cineangiocardiográficos de pacientes portadores de atresia pulmonar (AP com comunicação interventricular (CIV, identificar nos grupos propostos pela classificação de BARBERO MARCIAL, subgrupos com suprimento sang

  14. Novas técnicas cirúrgicas para o tratamento da atresia pulmonar com comunicação interventricular e anomalias de artérias pulmonares incluindo o assim chamado truncus tipo IV New surgical techniques for treatment of pulmonary atresia with ventricular septal defect and pulmonary arteries anomalies including the so-called tipo IV truncus

    Directory of Open Access Journals (Sweden)

    Miguel Barbero-Marcial

    1987-04-01

    Full Text Available Entre janeiro de 1975 e outubro de 1986, 42 pacientes com atresia pulmonar e comunicação interventricular, com idade entre 2 e 18 anos, foram submetidos a correção parcial, ou total. Foram divididos em: tipo A com todos os segmentos broncopulmonares conectados às artérias pulmonares (AP's, 34 pacientes; tipo B com alguns dos segmentos broncopulmonares conectados às AP's, 6 pacientes; tipo C com todos os segmentos broncopulmonares conectados às colaterais sistêmico-pulmonares, 2 pacientes. A correção foi planejada em uma a três etapas. No tipo A, 17 foram corrigidos em uma etapa, com três óbitos; em 9, na primeira etapa, as AP's foram reconstruídas e o Blalock (BT, realizado, tendo ocorrido um óbito. Em 2, a segunda etapa de correção total foi realizada, sem óbitos. No tipo B, a primeira etapa de unificação das colaterais intra ou extra-hilares foi realizada em 6 casos, sem óbitos; em 2, a segunda etapa da correção total foi realizada, com um óbito. No tipo C, 2 pacientes foram operados; 1 em três etapas; a primeira constou de construção de segmento arterial intermediário entre as artérias lobares e o BT; a segunda compreendeu unificação das colaterais contralaterais e a terceira, restabelecimento da continuidade ventrículo direito - circulação pulmonar; o paciente teve boa evolução. No segundo caso, a correção foi realizada após somente uma intervenção prévia. A evolução foi satisfatória. Estudos hemodinâmicos seriados foram realizados em 32 pacientes. As técnicas propostas permitem obter condições para correção total com adequada relação pós-operatória das pressões ventrículo direito - ventrículo esquerdo.Fourty-two patients with pulmonary atresia and interventricular septal defect were submited to a partial or total correction, between January 1975 and October 1986, with a range of 2 months to 18 years of age. Three groups were identified: Group A: 34 patients with all bronco

  15. An Analysis of the Causes of the Emerging Tricuspid Regurgitation after Minimally Invasive Transthoracic Closure of the Perimembranous Ventricular Septal Defects%膜周部室间隔缺损微创封堵术后新发三尖瓣反流程度变化的原因分析

    Institute of Scientific and Technical Information of China (English)

    万浩; 段书华; 周彩萍; 邢泉生

    2016-01-01

    Objective To analyze the cause of changes of emerging tricuspid regurgitation after minimally invasive transthoracic device closure of perimembranous ventricular septal defects (pmVSD).Methods 281 patients with pmVSD were selected to assess for tricuspid regurgitation with transesophageal echocardiography during preoperative and postoperative periods. Transthoracic echocardiography was used postoperatively during the 1-wk, 1-mo, 3-mo, 6-mo, 12-mo, 24-mo, 48-mo, 60-mo follow-up to evaluate the effectiveness and to assess for any complication emerging after the surgery.ResultsThere were 19 cases with tricuspid regurgitation with varying degrees of aggravation emerging immediately after closure. During the follow-ups, in the mild-relfux group, 12 patients’ relfux symptom mitigated;one case aggravated to mild to moderate relfux. In the mild to moderate relfux group, 2 cases alleviated to mild relfux; the other case aggravated to moderate relfux.Conclusion The aggravation of tricuspid regurgitation after minimally invasive transthoracic device closure of pmVSD may be caused by the occluder oppression on the tricuspid chordae or lealfets, tendons damage resulted fromdelivery sheath, and abnormal origin of part of the anterior tricuspid valve chordate. Mitigation of tricuspid regurgitation after surgery may be caused by remodeling of the occluder, which leads to oppressed chordae or lealfets to restore to the physiological state. Preoperative and postoperative ultrasound allows precise assessment of the position of tricuspid valve chordae and helps with understanding of the construction of the structure of defect edge tissues, which provides guidance for selecting the appropriate occluder and thus reduce or mitigate tricuspid regurgitation and other complications.%目的:分析经胸微创封堵膜周部室间隔缺损(pmVSD)术后三尖瓣反流程度的变化原因。方法对经胸微创封堵成功的281例pmVSD患儿进行术前、术后即用食道超声心

  16. Cardiac involvement in Duchenne and Becker muscular dystrophy

    Institute of Scientific and Technical Information of China (English)

    Sophie; Mavrogeni; George; Markousis-Mavrogenis; Antigoni; Papavasiliou; Genovefa; Kolovou

    2015-01-01

    Duchenne and Becker muscular dystrophy(DMD/BMD) are X-linked muscular diseases responsible for over 80% of all muscular dystrophies. Cardiac disease is a common manifestation,not necessarily related to the degree of skeletal myopathy; it may be the predominant manifestation with or without any other evidence of muscular disease. Death is usually due to ventricular dysfunction,heart block or malignant arrhythmias. Not only DMD/BMD patients,but also female carriers may present cardiac involvement. Clinically overt heart failure in dystrophinopathies may be delayed or absent,due to relative physical inactivity. The commonest electrocardiographic findings include conduction defects,arrhythmias(supraventricular or ventricular),hypertrophy and evidence of myocardial necrosis. Echocardiography can assess a marked variability of left ventricular dysfunction,independently of age of onset or mutation groups. Cardiovascular magnetic resonance(CMR) has documented a pattern of epicardial fibrosis in both dystrophinopathies’ patients and carriers that can be observed even if overt muscular disease is absent. Recently,new CMR techniques,such as postcontrast myocardial T1 mapping,have been used in Duchenne muscular dystrophy to detect diffuse myocardial fibrosis. A combined approach using clinical assessment and CMR evaluation may motivate early cardioprotective treatment in both patients and asymptomatic carriers and delay the development of serious cardiac complications.

  17. Persistent truncus arteriosus with intact ventricular septum diagnosed by echocardiography

    Institute of Scientific and Technical Information of China (English)

    ZHANG Yu-qi; SHEN Rong; SUN Kun; ZHONG Shu-wen; WU Yu-rong

    2009-01-01

    Persistent truncus arteriosus (PTA) is a rare congenital cardiac anomaly, and has an incidence of about 0.5 to 0.9 per 10 000 live births. Almost all cases described in the literatures had a large ventricular septal defect, only few rare cases were reported with intact ventricular septum. From June 1998 to December 2008, cardiac angiography were performed in 10 880 patients with congenital heart disease in our hospital, 47 patients with PTA were diagnosed, one case with tricuspid atresia,hypoplastic right ventricle, and intact ventricular septum was encountered.

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

    Energy Technology Data Exchange (ETDEWEB)

    Shiba, Nobuyuki; Kagaya, Yutaka; Ishide, Nobumasa; Takeyama, Daiya; Yamane, Yuriko; Chida, Masanobu; Otani, Hiroki; Shirato, Kunio [Tohoku Univ., Sendai (Japan). School of Medicine; Ido, Tatsuo

    1997-06-01

    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 {sup 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)

  19. Anterior septal deviation and contralateral alar collapse.

    Science.gov (United States)

    Schalek, P; Hahn, A

    2011-01-01

    Septal deviation is often found in conjunction with other pathological conditions that adversely affect nasal patency. Anterior septal deviation, together with contralateral alar collapse, is a relatively rare type of anatomical and functional incompetence. In our experience, it can often be resolved with septoplasty, without the necessity of surgery involving the external valve. The aim of this paper was to verify this hypothesis prospectively. Twelve patients with anterior septal deviation and simultaneous alar collapse on the opposite side were prospectively enrolled in the study. Subjective assessment of nasal patency was made on post-operative day 1, and again 6 months after surgery, using a subjective evaluation of nasal breathing. The width of the nostril (alar-columellar distance) on the side with the alar collapse was measured during inspiration pre-operatively, 1 day after surgery and again 6 months after surgery. Immediately after surgery, all patients reported improved or excellent nasal breathing on the side of the original septal deviation. On the collapsed side, one patient reported no change in condition. With the exception of one patient, all measurements showed some degree of improvement in the extension of the alar-columellar distance. The average benefit 6 months after surgery was an improvement of 4.54 mm. In our group of patients (anterior septal deviation and simultaneous contralateral alar collapse and no obvious structural changes of the alar cartilage) we found septoplasty to be entirely suitable and we recommend it as the treatment of choice in such cases.

  20. Effects of urokinase type plasminogen activator and plasminogen activator inhibitor-1 expressions on the formation of aneurysm of perimembranous ventricular septal defect%尿激酶型纤溶酶原激活物及其抑制物表达在膜周型室间隔缺损自发闭合中的作用

    Institute of Scientific and Technical Information of China (English)

    钱娟; 李本尚; 殷敏智; 沈萍; 孙锟

    2015-01-01

    0.05).结论 uPA及抑制物系统在VSA形成过程中起重要作用,参与瘤体的形成和纤维增殖过程.%Objective The exact mechanisms of defect closure in patients with perimembranous ventricular septal defect (PMVSD) remain unknown.We hypothesized that the expression of urokinase type plasminogen activator (uPA) and plasminogen activator inhibitor-1 (PAI-1) may mediate extracellular matrix (ECM) remodeling in aneurysms.Method Seven normal heart tricuspid septal leaflet and 33 aneurysms were collected in Shanghai Renji Hospital and Shanghai Children's Medical Center from January 2008 to June 2010.Immunohistochemical expression of uPA and PAI-1 in 4 normal heart valvular tissues and 15 aneurysms was detected with immunohistochemical methods.The expression of uPA and PAI-1 mRNA in 3 normal heart valvular tissues and 7 aneurysms was studied by real time fluorescent PCR;the protein expression of uPA and PAI-1 in 4 normal heart valvular tissues and 11 aneurysms was tested with Western blotting.Result The surface of the aneurysms were completely covered by endothelial cells.Two types of granulation tissue,myxoid and fibrous,were associated with the aneurismal formation.uPA were recognized predominantly in valvar interstitial cells (VICs) which located mainly in regions adjacent to the endothelium and smooth muscle cells of blood vessels.PAI-1 was found in both VICs which located mainly in granulation tissue and endothelial cells.Nine aneurysms expressed a higher uPA activity than 4 normal valvular tissues ((74.6 ± 11.8) % vs.(49.5 ± 7.4) %;t =3.87,P =0.003) and six aneurysms expressed a low uPA activity ((10.3±3.1)% vs.(49.5±7.4)%;t=11.78,P=0.000) andahighPAI-1 activity ((55.2±1.7) % vs.(50.8 ± 3.8) %;t =2.55,P =0.034) using immunohistochemical methods.uPA / PAI-1 ratio of protein expression tested by Western blot was 0.88 ± 0.22 in four normal heart vavular tissues;five aneurysms expressed high uPA activity and low PAI-1 activity and u

  1. Spinal Muscular Atrophy (SMA)

    Science.gov (United States)

    ... Habits for TV, Video Games, and the Internet Spinal Muscular Atrophy (SMA) KidsHealth > For Parents > Spinal Muscular Atrophy (SMA) Print ... treatment for the disease's most troubling symptoms. About SMA Normally, healthy nerve cells in the brain called ...

  2. Alcohol septal ablation in patients with hypertrophic obstructive cardiomyopathy

    DEFF Research Database (Denmark)

    Jensen, Morten K; Prinz, Christian; Horstkotte, Dieter;

    2013-01-01

    The infarction induced by alcohol septal ablation (ASA) may predispose to arrhythmia and sudden cardiac death (SCD).......The infarction induced by alcohol septal ablation (ASA) may predispose to arrhythmia and sudden cardiac death (SCD)....

  3. [Asymptomatic myxoma of the tricuspid valve septal leaflet].

    Science.gov (United States)

    Jedliński, Ireneusz; Jamrozek-Jedlińska, Maria; Bugajski, Paweł; Kalawski, Ryszard; Poprawski, Kajetan; Słomczyński, Marek

    2012-01-01

    We presented a case of asymptomatic myxoma of the tricuspid valve septal leaflet. The tumour was diagnosed accidentally during rutine transthoracic echocardiography and confirmed by transesophageal echocardiography. It was resected and the septal leaflet repaired during surgery.

  4. Alcohol septal ablation in obstructive acromegalic hypertrophic cardiomyopathy - a first case report.

    Science.gov (United States)

    Viveiros Monteiro, André; Fiarresga, António; Cacela, Duarte; de Sousa, Lídia; Ramos, Ruben; Galrinho, Ana; Branco, Luísa; Cruz Ferreira, Rui

    2016-09-01

    Acromegaly is a rare disease, mostly caused by a growth hormone (GH)-secreting benign pituitary tumor, with an increased production of GH and insulin-like growth factor 1 (IGF-1). Cardiovascular complications are common and are associated with cardiomyocyte apoptosis and concentric cardiac hypertrophy. Suppression of GH and IGF-1 appears to improve cardiac function only in the short term, with little or no decrease in left ventricular mass or improvement in cardiac function after prolonged treatment. Alcohol septal ablation (ASA) has emerged as a minimally invasive alternative to septal myectomy, with significant improvement in symptoms, gradients and left ventricular remodeling. In this report, we describe the case of a 73-year-old woman with acromegaly due to a pituitary adenoma diagnosed and treated surgically at the age of 38 but with recurrence and reoperation at the age of 50. She was referred to our cardiology department due to a three-month history of progressively worsening exercise-induced dyspnea and orthopnea under optimal medical therapy. Echocardiography and magnetic resonance imaging revealed severe basal hypertrophy of the interventricular septum (19 mm), dynamic left ventricular outflow tract obstruction with a gradient of 70 mmHg at rest and 120 mmHg with Valsalva maneuver, and systolic anterior movement (SAM). Genetic testing excluded the most frequent forms of familial hypertrophic cardiomyopathy. ASA was performed with injection of 2 cc of alcohol in the first septal branch of the left coronary artery, without complications. At one-year reassessment, significant clinical and echocardiographic improvement was noted, with disappearance of SAM. To our knowledge, there have been no previously reported cases of ASA in hypertrophic cardiomyopathy due to acromegaly. We report a case of successful ASA in acromegalic cardiomyopathy.

  5. Echocardiographic assessment and percutaneous closure of multiple atrial septal defects

    Directory of Open Access Journals (Sweden)

    Timperley Jonathan

    2004-07-01

    Full Text Available Abstract Atrial septal defect closure is now routinely performed using a percutaneous approach under echocardiographic guidance. Centrally located, secundum defects are ideal for device closure but there is considerable morphological variation in size and location of the defects. A small proportion of atrial septal defects may have multiple fenestrations and these are often considered unsuitable for device closure. We report three cases of multiple atrial septal defects successfully closed with two Amplatzer septal occluders.

  6. Anesthetic management for reentry sternotomy in a patient with a full stomach and pericardial tamponade from left ventricular rupture

    Directory of Open Access Journals (Sweden)

    Bryan G Maxwell

    2013-01-01

    Full Text Available A 57-year-old man presented with chest pain and shortness of breath 1 month after left ventricular aneurysmectomy and ventricular septal defect closure for post-infarct left ventricular aneurysm and ventricular septal defect. Echocardiography revealed a large recurrent ruptured inferior left ventricular aneurysm with high-velocity flow into a 5 cm posterolateral pericardial effusion. Thirty minutes earlier, the patient had eaten a full meal. Rapid sequence induction was performed with midazolam, ketamine, and succinylcholine. Moderate hypotension was treated effectively and the patient tolerated controlled transition to cardiopulmonary bypass. The ventricular defect was oversewn and reinforced with bovine pericardium. The patient had a difficult but ultimately successful recovery. Options for anesthetic management in the setting of tamponade and a full stomach are discussed, with a brief review of the evidence relating to this clinical problem.

  7. Vector electrocardiographic alterations after percutaneous septal ablation in obstructive hypertrophic cardiomyopathy: possible anatomic causes

    Directory of Open Access Journals (Sweden)

    Pérez Riera Andrés Ricardo

    2002-01-01

    Full Text Available OBJECTIVE: Analyze the dromotropic disturbances (vector-electrocardiographic, and the possible anatomic causes, provoked by selective alcohol injection in the septal branch, for percutaneous treatment, of obstructive hypertrophic cardiomyopathy. METHODS: Ten patients with a mean age of 52.7 years underwent percutaneous septal ablation (PTSA from october 1998; all in functional class III/IV. Twelve-lead electrocardiogram was performed prior to and during PTSA, and later electrocardiogram and vectorcardiogram according to Frank's method. The patients were followed up for 32 months. RESULTS: On electrocardiogram (ECG prior to PTSA all patients had sinus rhythm and left atrial enlargement, 8 left ventricular hypertrophy of systolic pattern. On ECG immediately after PTSA, 8 had complete right bundle-branch block; 1 transient total atrioventricular block; 1 alternating transient bundle-branch block either right or hemiblock. On late ECG 8 had complete right bundle-branch block confirmed by vectorcardiogram, type 1 or Grishman. CONCLUSION: Septal fibrosis following alcohol injection caused a predominance of complete right bundle-branch block, different from surgery of myotomy/myectomy.

  8. [Management of myocardial damage in muscular dystrophy].

    Science.gov (United States)

    Tamura, Takuhisa

    2011-11-01

    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.

  9. Tissue Doppler Septal Tei Index Indicates Severity of Illness in Pediatric Patients with Congestive Heart Failure

    Science.gov (United States)

    Mejia, Aura A Sanchez; Simpson, Kathleen E.; Hildebolt, Charles F; Pahl, Elfriede; Matthews, Kathleen L; Rainey, Cheryl A; Canter, Charles E; Jay, Patrick Y; Johnson, Mark C

    2013-01-01

    Background: The Doppler Tei index is an independent predictor of outcomes in adult heart failure. Tissue Doppler imaging (TDI) may be a superior method to measure the Tei index in children, as it is less affected by heart rate variability. We hypothesized that the TDI Tei index reflects severity of illness in pediatric heart failure. Methods: Twenty-five pediatric heart failure patients were prospectively enrolled. Listing for heart transplantation or death were the outcomes used to define severity of illness. Baseline demographics, brain natriuretic peptide (BNP), standard echocardiographic and TDI-derived parameters were analyzed to determine outcome indicators. Results: Ten of the 25 patients (40%) were listed for transplantation. There were no deaths. Multivariate analysis combining age, heart rate, standard echocardiographic parameters, and BNP, resulted in shortening fraction (p=0.002) as the best indicator of listing for transplantation (R2 = 0.32). A second multivariate analysis combining age, heart rate, TDI parameters and BNP, resulted in age (p = 0.03) and septal Tei index (p = 0.03) as the best predictive model (R2 = 0.36). The area under the receiver operating characteristic (ROC) curve for septal Tei index was 0.84 (0.64-0.96, 95% confidence interval) and it was comparable to the ROC curve for shortening fraction, p=0.76. Optimal values of sensitivity (100%) and specificity (60%) were obtained with septal Tei index values > 0.51. Conclusion: The TDI septal Tei index is an indicator of disease severity in pediatric heart failure patients and offers potential advantages in comparison with standard echocardiographic measures of left ventricular ejection. PMID:24061276

  10. Prevalence and distribution of regional scar in dysfunctional myocardial segments in Duchenne muscular dystrophy

    Directory of Open Access Journals (Sweden)

    Crawford Thomas O

    2011-03-01

    Full Text Available Abstract Background The segmental relationship between cardiovascular magnetic resonance (CMR peak circumferential strain (Ecc and myocardial scar has not been well characterized in Duchenne muscular dystrophy (DMD, and it is unknown whether echocardiography accurately measures Ecc in DMD. We assessed segmental Ecc and scar using CMR with myocardial tissue tagging and late gadolinium enhancement (LGE in patients with DMD, then compared CMR with echocardiographic velocity vector imaging (VVI for regional Ecc based on independent observer assessments. Results Participants enrolled (n = 16; age 8-23 had median left ventricular (LV ejection fraction of 0.52 (range 0.28-0.69, and 156 basal and mid-cavity myocardial segments from the 13 patients completing the LGE protocol were analyzed for strain and scar. Segmental CMR Ecc in the most negative quartile (quartile 4 ruled out scar in that segment, but scar was present in 46% of segments in the least negative (most dysfunctional Ecc quartile 1, 33% of Ecc quartile 2 segments, and 15% of Ecc quartile 3 segments. Overall scar prevalence in inferior, inferolateral, and anterolateral segments was eight times higher than in inferoseptal, anteroseptal, and anterior segments (p Conclusions The relationship between scar and Ecc in DMD is complex. Among myocardial segments with depressed Ecc, scar prevalence was much higher in inferior, inferolateral, and anterolateral segments, indicating a regionally dependent association between abnormal Ecc and scar, with free wall segments commonly developing dysfunction with scar and septal segments developing dysfunction without scar. Although normal echocardiographic Ecc predicted absence of scar, regional echocardiographic Ecc by VVI has only a limited association with CMR Ecc in DMD.

  11. Atrofia muscular proximal familiar

    Directory of Open Access Journals (Sweden)

    José Antonio Levy

    1962-09-01

    Full Text Available Os autores relatam dois casos de atrofia muscular proximal familiar, moléstia caracterizada por déficit motor e atrofias musculares de distribuição proximal, secundárias a lesão de neurônios periféricos. Assim, como em outros casos descritos na literatura, foi feito inicialmente o diagnóstico de distrofia muscular progressiva. O diagnóstico correto foi conseguido com auxílio da eletromiografia e da biopsia muscular.

  12. Atrioventricular septal defects among infants in Europe

    DEFF Research Database (Denmark)

    Christensen, Nikolas; Andersen, Helle; Garne, Ester

    2013-01-01

    OBJECTIVE: To describe the epidemiology of chromosomal and non-chromosomal cases of atrioventricular septal defects in Europe. METHODS: Data were obtained from EUROCAT, a European network of population-based registries collecting data on congenital anomalies. Data from 13 registries for the period...... 2000-2008 were included. RESULTS: There was a total of 993 cases of atrioventricular septal defects, with a total prevalence of 5.3 per 10,000 births (95% confidence interval 4.1 to 6.5). Of the total cases, 250 were isolated cardiac lesions, 583 were chromosomal cases, 79 had multiple anomalies, 58...... of pregnancy owing to foetal anomaly. Among the groups, additional associated cardiac anomalies were most frequent in heterotaxia cases (38%) and least frequent in chromosomal cases (8%). Coarctation of the aorta was the most common associated cardiac defect. The 1-week survival rate for live births was 94...

  13. Could early septal involvement in the remodeling process be related to the advance hypertensive heart disease?

    Directory of Open Access Journals (Sweden)

    Fatih Yalçin

    2015-06-01

    Conclusions: LV diastolic dysfunction becomes more severe in septal wall than lateral wall in hypertensive LVH. Septal myocardial performance is more dominantly affected by hypertension possibly due to earlier septal involvement in disease course. Septal MPI is correlated moderately with septal wall thickness.

  14. Novel Mutations in the Transcriptional Activator Domain of the Human TBX20 in Patients with Atrial Septal Defect

    Directory of Open Access Journals (Sweden)

    Irma Eloisa Monroy-Muñoz

    2015-01-01

    Full Text Available Background. The relevance of TBX20 gene in heart development has been demonstrated in many animal models, but there are few works that try to elucidate the effect of TBX20 mutations in human congenital heart diseases. In these studies, all missense mutations associated with atrial septal defect (ASD were found in the DNA-binding T-box domain, none in the transcriptional activator domain. Methods. We search for TBX20 mutations in a group of patients with ASD or ventricular septal defect (VSD using the High Resolution Melting (HRM method and DNA sequencing. Results. We report three missense mutations (Y309D, T370O, and M395R within the transcriptional activator domain of human TBX20 that were associated with ASD. Conclusions. This is the first association of TBX20 transcriptional activator domain missense mutations with ASD. These findings could have implications for diagnosis, genetic screening, and patient follow-up.

  15. Cardiac involvement in facioscapulohumeral muscular dystrophy.

    Science.gov (United States)

    Finsterer, Josef; Stöllberger, Claudia; Meng, Gerhard

    2005-01-01

    Cardiac involvement (CI) in form of myocardial thickening in a patient with genetically confirmed facioscapulohumeral muscular dystrophy (FSHMD) has not been reported. The patient is a 50-year-old male with a tandem repeat size of 17 and 14 kb in the D4Z4 locus on chromosome 4q35. The clinical cardiologic investigation was normal. Blood pressure was 150/90 mm Hg. Funduscopy, 24-hour ambulatory ECG, and 24-hour blood pressure monitoring were normal. ECG showed incomplete right bundle branch block, ST elevation in V2-V4, tall T waves in V3-V5, and hypertrophy signs. Echocardiography revealed left ventricular myocardial thickening of the posterior wall (11.7 mm) and the septum (15.5 mm). In conclusion, CI in genetically confirmed FSHMD may manifest not only as ECG abnormalities but also as left ventricular myocardial thickening. Copyright 2005 S. Karger AG, Basel.

  16. Adverse ventricular-ventricular interactions in right ventricular pressure load: Insights from pediatric pulmonary hypertension versus pulmonary stenosis.

    Science.gov (United States)

    Driessen, Mieke M P; Hui, Wei; Bijnens, Bart H; Dragulescu, Andreea; Mertens, Luc; Meijboom, Folkert J; Friedberg, Mark K

    2016-06-01

    Right ventricular (RV) pressure overload has a vastly different clinical course in children with idiopathic pulmonary arterial hypertension (iPAH) than in children with pulmonary stenosis (PS). While RV function is well recognized as a key prognostic factor in iPAH, adverse ventricular-ventricular interactions and LV dysfunction are less well characterized and the pathophysiology is incompletely understood. We compared ventricular-ventricular interactions as hypothesized drivers of biventricular dysfunction in pediatric iPAH versus PS Eighteen iPAH, 16 PS patients and 18 age- and size-matched controls were retrospectively studied. Cardiac cycle events were measured by M-mode and Doppler echocardiography. Measurements were compared between groups using ANOVA with post hoc Dunnet's or ANCOVA including RV systolic pressure (RVSP; iPAH 96.8 ± 25.4 mmHg vs. PS 75.4 ± 18.9 mmHg; P = 0.011) as a covariate. RV-free wall thickening was prolonged in iPAH versus PS, extending beyond pulmonary valve closure (638 ± 76 msec vs. 562 ± 76 msec vs. 473 ± 59 msec controls). LV and RV isovolumetric relaxation were prolonged in iPAH (P < 0.001; LV 102.8 ± 24.1 msec vs. 63.1 ± 13.7 msec; RV 95 [61-165] vs. 28 [0-43]), associated with adverse septal kinetics; characterized by rightward displacement in early systole and leftward displacement in late RV systole (i.e., early LV diastole). Early LV diastolic filling was decreased in iPAH (73 ± 15.9 vs. PS 87.4 ± 14.4 vs. controls 95.8 ± 12.5 cm/sec; P = 0.004). Prolonged RVFW thickening, prolonged RVFW isovolumetric times, and profound septal dyskinesia are associated with interventricular mechanical discoordination and decreased early LV filling in pediatric iPAH much more than PS These adverse mechanics affect systolic and diastolic biventricular efficiency in iPAH and may form the basis for worse clinical outcomes. We used clinically derived data to study the pathophysiology of ventricular-ventricular

  17. Relationship of basal-septal fibrosis with LV outflow tract obstruction in hypertrophic cardiomyopathy: insights from cardiac magnetic resonance analysis.

    Science.gov (United States)

    Nakamura, Takashi; Iwanaga, Yoshitaka; Yasuda, Masakazu; Kawamura, Takayuki; Miyaji, Yuki; Morooka, Hanako; Miyazaki, Shunichi

    2016-04-01

    Myocardial fibrosis is frequently observed and may be associated with the prognosis in patients with hypertrophic cardiomyopathy (HCM); however, the clinical pathophysiological features, particularly in terms of fibrosis, of hypertrophic obstructive cardiomyopathy (HOCM) remain unclear. This study aimed to determine a role of local fibrosis in HOCM using cardiac magnetic resonance (CMR). 108 consecutive HCM patients underwent CMR. HOCM was defined as a left ventricular outflow tract (LVOT) pressure gradient ≥30 mmHg at rest. Myocardial mass and fibrosis mass by late gadolinium-enhancement CMR (LGE-CMR) were calculated and the distribution/pattern was analyzed using the AHA 17-segment model. LV ejection fraction (LVEF) was significantly higher in patients with HOCM (n = 19) than in those with nonobstructive HCM (n = 89) (P < 0.05). Both total myocardial and fibrosis masses in LV were similar in the two groups (P = 0.385 and P = 0.859, respectively). However, fibrosis in the basal septum was significantly less frequent in the HOCM group than in the nonobstructive HCM group (P < 0.01). The LVOT pressure gradient was significantly higher in the basal-septal non-fibrosis group than in the fibrosis group (23.6 ± 37.3 vs. 4.8 ± 11.4 mmHg, P < 0.01). Multivariate analysis revealed that basal-septal fibrosis was an independent negative predictor of LVOT obstruction in addition to the local wall thickness and LVEF as positive predictors in HCM patients. In conclusion, a significant association was observed between LVOT obstruction and basal septal fibrosis by LGE-CMR in HCM patients. In addition to negative impact of basal-septal fibrosis, basal-septal hypertrophy and preserved global LV contractility may be associated with the pathophysiological features of LVOT obstruction.

  18. Acute and mid-term results of pecutaneous transluminal septal myocardial ablation in patients with hypertrophic obstructive cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Tiebing Zhu; Zhijian Yang; Liansheng Wang; Hui Wang; Kejiang Cao; Jun Huang; Wenzhu Ma

    2007-01-01

    Objective: To assess the acute and mid-term results of cardiac function improvements and left ventricular outflow tract gradient (LVOTG)changes in 30 patients displaying hypertrophic obstructive cardiomyopathy (HOCM) treated with percutaneous transluminal septal myocardial ablation (PTSMA). Methods: PTSMA was intended for 32 patients comprising of 13 women and 19men (average years being 54.1 ± 15.5) to be treated in accordance with the following inclusion criteria: The New York Heart As-sociation(NYHA) definition for cardiac functional class Ⅲ or Ⅳ , or class Ⅱ but for whom medical therapies were not tolerated or with syncope; intraventricular septal (IVS) and left ventricular posterior wall (LVPW) hypertrophy asymmetrically associated with ratio of IVS to LVPW≥1.3 and LVOTG≥50 mm Hg at rest or ≥100 mm Hg at provocation (Valsalva maneuver). The target vessels were determined by coronary arteriography that demonstrated more than one septal branch and probatory balloon occlusion produced greater than 50% decrease of LVOTG. Once the target vessel established, the alcohol was administrated into septal ventricular via over-the-wire balloon. LVOTG was assessed by means of echocardiography measurements immediately after procedure and 3 months. Simultaneously, cardiac function class was also evaluated. Results: Two patients were abandoned prior to intervention due to inappropriate septal target vessels and DDD Pacemakers were chosed. Immediately after the procedure, resting LVOTG was reduced from 73.8 ± 35.5 to 16.6 ± 7.8 mmHg, at provocation LVOTG from 149.3 ± 42.5 to 61.9 ± 43.0 mmHg(P <0.0001 each) by echocardiography measurements. After 3 months, the mean New York Heart Association class was reduced from 2.8 ± 0.6 to 1.1 ± 1.0(P < 0.0001) and the LVOTG also remained decrease(28.5 ± 6.4 mmHg at rest and 75.3 ± 11.6 mmHg at provocation). Conclusion: PTSMA is a promising nonsurgical technique for relief of symptoms and reduction of LVOTG in

  19. Transcatheter closure of secundum atrial septal defect using an Amplatzer septal occluder

    Institute of Scientific and Technical Information of China (English)

    孔祥清; 曹克将; 杨荣; 许迪; 盛燕辉; 黄峻; 马文珠

    2002-01-01

    Objective To evaluate the safety and efficacy of the Amplatzer septal occluder for transcatheter closure in patients with secundum atrial septal defect (ASD Ⅱ). Methods Patients with clinically confirmed ASD Ⅱ were recommended for transcatheter closure of ASD Ⅱ. Results 30 ASD Ⅱ patients (20 females) underwent transcatheter closure at a median age of 18.4 years (5-55 years). Both the stretched diameters of ASDs and the sizes of the devices were from 18 to 34?mm (25±7?mm). The successful placement rate was 100%. The rest shunt documented by color Doppler, was immediately after implantation in 40% of patients, in 9.9% after 24 hours, and in 3.3% trace at 3 months. No serious complications were observed. There was improvement in symptoms and in cardiac size. Septal motion abnormalities normalized in all patients after 3 months follow-up. Conclusion The Amplatzer septal occluder is a safe and effective device for transcatheter closure of ASD Ⅱ. Long-term follow-up is still required before widespread clinical use can be recommended.

  20. Transcatheter Closure of Atrial Septal Defect with Amplatzer Septal Occluder in Adults: Immediate, Short, and Intermediate-Term Results

    Directory of Open Access Journals (Sweden)

    Majid Dehghani

    2011-05-01

    Full Text Available Background: The transcatheter closure of the atrial septal defect (ASD has become an alternative technique to surgical procedures. The aim of this study was to assess the immediate, short, and intermediate-term results of the transcatheter closure of the secundum ASD with the Amplatzer Septal Occluder (ASO in adult Iranian patients.Methods: Between December 2004 and July 2008, the transcatheter closure of the ASD using the ASO was attempted in 58consecutive, adult patients. The mean age of the patients was 37.1 ± 12.7 years (range = 19 - 75 years.All the procedures were performed under local anesthesia with transthoracic or transesophageal echocardiography and fluoroscopic guidance. The stretched diameter of the ASD was determined with a balloon sizing catheter, and device selection was based on and matched to the stretched diameter of the septal defect.Transthoracic echocardiography was performed immediately after the release of the device and before discharge.Further follow-up at one month, six months, and yearly thereafter included physical examination, electrocardiography, andtransthoracic echocardiography.Results: The mean ASD diameter, as measured by esophageal echocardiography, was 24.8 ± 5.4 mm (range = 13 - 34 mm.The mean stretched diameter, as measured by the balloon catheter, was 27.1 ± 6.4 mm (range = 12.5 - 39 mm. Deploymentof the ASO was successful in 52 (89.6% patients and failed in 6 (10.4%. Four patients experienced severe complications,1 had tamponade requiring drainage, 2 had device embolization to the left atrium and right ventricular outflow tract, and 1 had late wire fracture (surgical removal and repair of the ASD. The position of two large devices (34 mm and 36 mm was considered unsuitable and unstable after implantation and resulted in the removal of these devices.Minor complications included transient complete atrioventricular block in 1 patient, paroxysmal supra tachycardia in 3 patients, atrial flutter in 1 patient

  1. Initial experience in Brazil with the Helex septal occluder for percutaneous occlusion of atrial septal defects

    Directory of Open Access Journals (Sweden)

    Pedra Carlos A.C.

    2003-01-01

    Full Text Available OBJECTIVE: To evaluate the initial clinical experience with the Helex septal occluder for percutaneous closure of atrial septal defects. METHODS: Ten patients underwent the procedure, 7 patients with ostium secundum atrial septal defects (ASD with hemodynamic repercussions and 3 patients with pervious foramen ovale (PFO and a history of stroke. Mean age was 33.8 years and mean weight was 55.4 kg. Mean diameter by transesophageal echocardiography and mean stretched ASD diameter were 11.33 ± 3.3mm, and 15.2 ± 3.8mm, respectively. The Qp/Qs ratio was 1.9 ± 0.3 in patients with ASD. RESULTS: Eleven occluders were placed because a patient with 2 holes needed 2 devices. It was necessary to retrieve and replace 4 devices in 3 patients. We observed immediate residual shunt (< 2mm in 4 patients with ASD, and in those with patent foramen ovale total occlusion of the defect occurred. No complications were noted, and all patients were discharged on the following day. After 1 month, 2 patients with ASD experienced trivial residual shunts (1mm. In 1 patient, we observed mild prolapse in the proximal disk in the right atrium, without consequences. CONCLUSION: The Helex septal occluder was safe and effective for occluding small to moderate atrial septal defects. Because the implantation technique is demanding, it requires specific training of the operator. Even so, small technical failures may occur in the beginning of the learning curve, but they do not involve patient safety.

  2. Diagnostic value of plasma concentration of pro-brain natriuretic peptide in congestive heart failure in pediatric patients with ventricular septal defects%氨基末端脑利钠肽前体在室间隔缺损合并心力衰竭诊断中的价值

    Institute of Scientific and Technical Information of China (English)

    武育蓉; 陈树宝; 黄美蓉; 张玉奇; 孙锟; 陈笋

    2005-01-01

    目的探讨血浆氨基末端脑利钠肽前体(NT-proBNP)水平在室间隔缺损合并心力衰竭(简称心衰)诊断及心功能评估中的价值.方法 51例室间隔缺损患儿,按照小儿心衰改良Ross标准分为0~2分(无心衰)、3~6分(轻度心衰)、7~12分(中-重度心衰)三组;对照组15例.应用ELASA方法测定血浆NT-proBNP浓度.同时测定左室舒张末期容量指数(left ventricular end diastolic volume index,LVEDVI)、左室收缩末期室壁应力(left ventricular end systolic wall stress,LVESWS)、心率校正的平均周径缩短速率(heart rate corrected mean velocity of circumferential fiber shortening,mVcFc)、左室射血分数(left ventricular ejection fraction,LVEF)、左室缩短分数(left ventricular fractional shortening,LVFS)、心肌收缩力(contractility index,Con)等超声心动图指标.结果 (1)血浆NT-proBNP水平与临床评分之间呈明显正相关(r=0.75,P<0.01),中-重度心衰组[(2061±908)fmol/ml]高于轻度心衰组[(810±335)fmol/ml];轻度心衰组高于无心衰组[(309±68)fmol/ml];但是无心衰组与正常对照组间[(275±62)fmol/ml]差异无统计学意义.(2)血浆NT-proBNP水平与LVESWS、 LVEDVI呈正相关;与LVEF、LVFS、Con、mVcFc等无明显相关性.(3)血浆NT-proBNP水平≥400 fmol/ml的情况下诊断充血性心衰的敏感度为89.3%,特异度为91.2%,ROC曲线下面积为0.944.结论血浆NT-proBNP水平可用于室间隔缺损合并心衰患儿心功能的评估,可以用于该类患儿心衰的诊断及分度.

  3. Spinal muscular atrophy

    National Research Council Canada - National Science Library

    D'Amico, Adele; Mercuri, Eugenio; Tiziano, Francesco D; Bertini, Enrico

    2011-01-01

    Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease characterized by degeneration of alpha motor neurons in the spinal cord, resulting in progressive proximal muscle weakness and paralysis...

  4. Muscular Dystrophy Association

    Science.gov (United States)

    ... Families Live Unlimited Read More Deflazacort demonstrates significant muscle strength improvement in DMD Read More NDA Filing ... the Boot to Support Kids and Adults with Muscular Dystrophy, ALS and Related Diseases Read More Visit ...

  5. Spinal Muscular Atrophy

    Science.gov (United States)

    Spinal muscular atrophy (SMA) is a genetic disease that attacks nerve cells, called motor neurons, in the spinal cord. These cells communicate with your voluntary muscles - the ones you can control, like in your ...

  6. The one-stage rhinoplasty septal perforation repair.

    Science.gov (United States)

    Foda, H M

    1999-08-01

    A combined septal perforation repair and rhinoplasty was performed in 20 patients (12 males, eight females; age range 16-36, mean age 29.6) presenting with septal perforations (size 1-4 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. The perforation was totally closed in 18 cases (90 per cent) with complete resolution of the pre-operative symptoms occurring in 16 (80 per cent). Cosmetically, 19 cases (95 per cent) were very satisfied with their aesthetic result. The exposure provided by the external approach proved to be very helpful in the process of septal perforation repair. Our results show that septal perforation repair could safely be combined with rhinoplasty and that some of the rhinoplasty manoeuvres used could even facilitate the process of septal perforation repair.

  7. Cardiac function in muscular dystrophy associates with abdominal muscle pathology

    Science.gov (United States)

    Gardner, Brandon B.; Swaggart, Kayleigh A.; Kim, Gene; Watson, Sydeaka; McNally, Elizabeth M.

    2015-01-01

    Background The muscular dystrophies target muscle groups differentially. In mouse models of muscular dystrophy, notably the mdx model of Duchenne Muscular Dystrophy, the diaphragm muscle shows marked fibrosis and at an earlier age than other muscle groups, more reflective of the histopathology seen in human muscular dystrophy. Methods Using a mouse model of limb girdle muscular dystrophy, the Sgcg mouse, we compared muscle pathology across different muscle groups and heart. A cohort of nearly 200 Sgcg mice were studied using multiple measures of pathology including echocardiography, Evans blue dye uptake and hydroxyproline content in multiple muscle groups. Spearman rank correlations were determined among echocardiographic and pathological parameters. Findings The abdominal muscles were found to have more fibrosis than other muscle groups, including the diaphragm muscle. The abdominal muscles also had more Evans blue dye uptake than other muscle groups. The amount of diaphragm fibrosis was found to correlate positively with fibrosis in the left ventricle, and abdominal muscle fibrosis correlated with impaired left ventricular function. Fibrosis in the abdominal muscles negatively correlated with fibrosis in the diaphragm and right ventricles. Together these data reflect the recruitment of abdominal muscles as respiratory muscles in muscular dystrophy, a finding consistent with data from human patients. PMID:26029630

  8. Understanding atrioventricular septal defect: Anatomoechocardiographic correlation

    Directory of Open Access Journals (Sweden)

    Keirns Candace

    2008-06-01

    Full Text Available Abstract Objective Correlate the anatomic features of atrioventricular septal defect with echocardiographic images. Materials and methods Sixty specimen hearts were studied by sequential segmental analysis. Echocardiograms were performed on 34 patients. Specimen hearts with findings equivalent to those of echocardiographic images were selected in order to establish an anatomo-echocardiographic correlation. Results Thirty-three specimen hearts were in situs solitus, 19 showed dextroisomerism, 6 were in situs inversus and 2 levoisomerism. Fifty-eight had a common atrioventricular valve and 2 had two atrioventricular valves. Rastelli types were determined in 21 hearts. Nine were type A, 2 intermediate between A and B, 1 mixed between A and B, 4 type B and 5 type C. Associated anomalies included pulmonary stenosis, pulmonary atresia atrial septal defect, patent ductus arteriosus and anomalous connection of pulmonary veins. Echocardiograms revealed dextroisomerism in 12 patients, situs solitus in 11, levoisomerism in 7 and situs inversus in 4. Thirty-one patients had common atrioventricular valves and three two atrioventricular valves. Rastelli types were established in all cases with common atrioventricular valves; 17 had type A canal defects, 10 type B, 3 intermediate between A and B, 1 mixed between A and B and 3 type C. Associated anomalies included regurgitation of the atrioventricular valve, pulmonary stenosis, anomalous connection of pulmonary veins, pulmonary hypertension and pulmonary atresia. Conclusion Anatomo-echocardiographic correlation demonstrated a high degree of diagnostic precision with echocardiography.

  9. Transesophageal echocardiography for incremental value of Amplatezer cribriform septal occluder for percutaneous transcatheter closure of complex septal defects: Case series

    Directory of Open Access Journals (Sweden)

    Shen Kou Tsai

    2017-06-01

    Conclusion: Our patients with complex septal defects were successfully treated by transcatheter closure using an Amplazter cribriform septal occluder device with careful planning based on patient presentation and close interdisciplinary collaboration. RT 3D color Doppler TEE provided precise information for the selection of the appropriate occluder device and facilitated the procedure by guiding the catheter through the often challenging patient anatomy.

  10. Myotonic Dystrophy and Facioscapulohumeral Muscular Dystrophy Registry

    Science.gov (United States)

    2016-08-26

    Myotonic Dystrophy; Facioscapulohumeral Muscular Dystrophy; Muscular Dystrophy; Myotonic Dystrophy Type 1; Myotonic Dystrophy Type 2; Congenital Myotonic Dystrophy; PROMM (Proximal Myotonic Myopathy); Steinert's Disease; Myotonic Muscular Dystrophy

  11. Assessment of left ventricular systolic synchrony before and after percutaneous transluminal septal myocardial ablation of hypertrophic obstructive cardiomyopathy by two dimensional speckle tracking imaging%斑点追踪技术评价肥厚型梗阻性心肌病化学消融术前后左心室收缩同步性的研究

    Institute of Scientific and Technical Information of China (English)

    唐海林; 赵博文; 傅国胜; 王蓓; 黎鹏; 许立龙; 陈冉; 熊莉

    2012-01-01

    目的 应用二维斑点追踪显像(2D-STI)技术评价肥厚型梗阻性心肌病(HOCM)化学消融术前后患者左室收缩同步性,并探讨左室收缩同步性与左室流出道压差的关系.方法 21例HOCM患者于化学消融术前及术后6d,超声心动图测量左室流出道压差;获取左室短轴观(二尖瓣环、乳头肌和心尖水平)二维灰阶图像,应用2D-STI测量左室短轴观各节段收缩期径向应变达峰时间(TRS).计算左室整体径向应变达峰时间的标准差(TRS-SD).结果 21例HOCM患者左室流出道压差术后较术前明显下降,差异有统计学意义(P<0.01).术后6d,前间隔基底段及中段径向TRS较术前明显延迟(P<0.05),左室短轴观水平整体径向TRS-SD与术前比较差异无统计学意义(P>0.05).前间隔基底段TRS延迟与左室流出道压差下降呈中等相关(r =0.657,P<0.01).结论 2D-STI能准确检测HOCM患者化学消融术前后左室收缩同步性.%Objective To assess the characteristics of left ventrlcular ( LV) systolic syncnrony before and 6 days after percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) using two dimensional speckle tracking imaging(2DSTI),and to investigate correlation between the LV systolic synchrony and LV outflow-trace gradient (LVOTG).Methods LVOTG were measured before and 6 days after PTSMA in 21 patients with HOCM.The two dimensional loop-cinec were obtained in three levels of the short axis views of LV (mitral valve,papillary muscle and cardiac apex).The time from the onset of QRS complexes to systolic peak strain from the radial vectors (TRs) was recorded using 2D-STI.The standard deviation of the TRS of 18 segments (TRsSD) were calculated as indicator of LV systolic synchrony.Results Six days after PTSMA,LVOTG decreased significantly from (84.3 ± 19.1)mm Hg to (40.6 ± 8.3)mm Hg( P <0.01).TRS of the base and middle segments of anteroseptal were

  12. Efficacy and safety of alcohol septal ablation in elderly patients: one-year outcome

    Institute of Scientific and Technical Information of China (English)

    Hisham Dokainish; Antoine Abchee; Ariel Delarosa; Sherif F. Nagueh; Wdliam Spencer; Nasser Lakkis

    2005-01-01

    Objective The management of hypertrophic obstructive cardiomyopathy is not well-defined in the elderly.Medical therapy with 3-blockers and calcium-channel blockers are the mainstay therapy for symptomatic patients.Myomectomy is usually reserved for patients who fail medical therapy. Alcohol septal ablation has been recently introduced as an alternative therapy. Patients and Methods Ninety-five patients older than 65 years of age were included. All patients have completed one year of follow-up. The mean age was 72 ± 5 years, 47 patients were females, 10 patients with history of hypertension. Results The mean rise in CK post alcohol ablation was 1052 ± 430IU. The mean NYHA class decreased from 2.9 ± 0.6 to 1.2 ± 0.5 ( P < 0.001 ). The exercise duration on treadmill testing increased from 328 ± 260 s to 349 ± 39 s. The mean resting left ventricular outflow tract gradient decreased from 65 ± 37 mmHg to 16 ± 29 mmHg at one year. One patient died in the hospital after coronary artery bypass grafting that was done subsequent to spiral dissection of the left anterior descending artery during ablation. Thirteen patients developed complete heart block immediately after ablation requiring pacing therapy. Conclusions Alcohol septal ablation seems to be an effective alternative therapeutic option for elderly patients with hypertrophic obstructive cardiomyopathy. Larger studies with longer follow-up are needed.

  13. Premature Ventricular Contractions (PVCs)

    Science.gov (United States)

    Diseases and Conditions Premature ventricular contractions (PVCs) By Mayo Clinic Staff Premature ventricular contractions (PVCs) are extra, abnormal heartbeats that begin in one of your heart's two ...

  14. 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.

  15. Complications Related to Transcatheter Occlusion of Atrial Septal Defect with Amplatzer Septal Occluder in Children

    Institute of Scientific and Technical Information of China (English)

    Wang Shushui; Li Yufen; Zhang Zhiwei; Wang Huishen; Qian Mingyang

    2004-01-01

    Objectives To investigate the causes, theraputic and preventive methods of complications associated with transcatheter occlusion of atrial septal defect (ASD) using the Amplatzer septal occluder (ASO) in children. Methods 289 cases underwent transcatheter closure of ASD with ASO. Complications occurred in 9 cases. The complications in procedure included systemic circulatory systemic air embolism in 2, pulmonary air embolism in 1, pericardial tamponade in 1, ASO malposition requiring emergency surgical removal in 1, transient atrial extrasystoles in 1 and sizing balloon rupture in 1 case. 2 cases with postoperative complications were found in the follow-up studies. These included perforation of mitral valve and ASO partially dislodged. In this study, 5children with intraoperative complication received emergency therapy including surgical intervention, and others needed only follow-up, as the complications were transient or asymptomatic. Results There were no children death in this study. The 5 cases who received treatment were completely healing, and the others with intra - procedure complications were also had no sequela existed. Cases with mitral valve and ASO partially dislodged were still in follow-up studies, as the 2 patients having no symptoms. Conclutions Air embolism were occurred easily in atrial septal defect cases who received ASO therapy. Complications mainly caused by inappropriate operative procedure and some complications needed emergency treatments. Follow-up studies were important to cases with transcatheter occlusion therapy.

  16. 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)

  17. 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.

  18. Mammoth interatrial septal aneurysm in the ICE age

    Directory of Open Access Journals (Sweden)

    Timperley John

    2007-09-01

    Full Text Available Abstract Background Intracardiac echocardiography (ICE is a useful imaging modality that is now being used more widely to assist in the percutaneous closure of atrial septal defects (ASD and patent foramen ovales (PFO. Case presentation A 42 year old lady with a history of transient ischaemic attacks and migraine underwent percutaneous closure of an ASD. Intraprocedural ICE demonstrated a mammoth billowing multiperforated interatrial septal aneurysm in association with a secondum ASD. Conclusion ICE provides excellent adjuvant imaging during percutaneous closure of intracardiac shunts, in this case demonstrating a 'mammoth' interatrial septal aneurysm.

  19. Ullrich Congenital Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Goknur Haliloglu

    2011-09-01

    Full Text Available ObjectiveUllrich congenital muscular dystrophy is a rather severe type of congenitalmuscular dystrophy with early onset features related to motor development.In general it is inherited in autosomal recessive principles, however in theWestern world mostly seen with de novo dominant mutations in the collagenVI genes. Milder form of the condition is the Bethlem myopathy. There may beoverlap forms in the clinic resembling the Ehler-Danlos syndrome. There hasbeen some radical efforts for cure especially through the apoptosis cascades.Key words: Ullrich congenital muscular dystrophy, collgen VI genes, Bethlemmyopathy, autophagy.

  20. Ullrich Congenital Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Goknur Haliloglu

    2011-06-01

    Full Text Available ObjectiveUllrich congenital muscular dystrophy is a rather severe type of congenital muscular dystrophy with early onset features related to motor development.In general it is inherited in autosomal recessive principles, however in the Western world mostly seen with de novo dominant mutations in the collagen VI genes. Milder form of the condition is the Bethlem myopathy. There may be overlap forms in the clinic resembling the Ehler-Danlos syndrome. There has been some radical efforts for cure especially through the apoptosis cascades.

  1. Dismorfia muscular Muscle dysmorphia

    OpenAIRE

    Sheila Seleri Marques Assunção

    2002-01-01

    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 dismo...

  2. Cardiac MRI in a Patient with Coincident Left Ventricular Non-Compaction and Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Zahra Alizadeh-Sani

    2011-12-01

    Full Text Available Left ventricular non-compaction cardiomyopathy is a rare congenital cardiomyopathy that affects both children and adults. Since the clinical manifestations are not sufficient to establish diagnosis, echocardiography is the diagnostic tool that makes it possible to document ventricular non-compaction and establish prognostic factors. We report a 47-year-old woman with a history of dilated cardiomyopathy with unknown etiology. Echocardiography showed mild left ventricular enlargement with severe systolic dysfunction (EF = 20-25%. According to cardiac magnetic resonance imaging findings non-compaction left ventricle with hypertrophic cardiomyopathy was considered, and right ventricular septal biopsy was recommended. Right ventricular endomyocardial biopsy showed moderate hypertrophy of cardiac myocytes with foci of myocytolysis and moderate interstitial fibrosis. No evidence of infiltrative deposition was seen.

  3. "Repaired" tetralogy of fallot mimicking arrhythmogenic right ventricular cardiomyopathy (another phenocopy).

    Science.gov (United States)

    George, Betsy Ann; Ko, Jong Mi; Lensing, Forrester Dubus; Kuiper, Johannes Jacob; Roberts, William Clifford

    2011-07-15

    Described is a 41-year-old man who at age 6 had partial resection of an obstructed right ventricular outflow tract with insertion of a patch and closure of a ventricular septal defect (tetralogy of Fallot). At age 41, cardiac transplantation was performed because of right ventricular outflow patch aneurysm, numerous episodes of ventricular tachycardia, and chronic heart failure, all features of the familial form of arrhythmogenic right ventricular cardiomyopathy (ARVC). Additionally, the patient had bundle branch block and epsilon waves on electrocardiogram, other features of ARVC. The case is described to introduce the concept of acquired ARVC, because the patient had many of the clinically recognized features of familial ARVC. Copyright © 2011 Elsevier Inc. All rights reserved.

  4. Percutaneous transluminal septal myocardial ablation in patients with hypertrophic obstructive cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    姜腾勇; 吴学思; 贾长棋; 张芩; 吕强

    2002-01-01

    Objective To evaluate the immediate and follow-up results of percutaneous transluminal septal myocardial ablation (PTSMA) in patients with hypertrophic obstructive cardiomyopathy (HOCM). Methods Fifteen symptomatic, drug-refractory patients with HOCM underwent PTSMA procedures with application of a myocardial contrast echocardiography (MCE) intra-procedure. Before and after the procedure, clinical evaluations were obtained in all patients, who were followed up for a mean period of 8.6±3.8 (6-20) months.Results Immediate left ventricular outflow tract gradient (LVOTG) reduction was achieved (77.93±22?mm?Hg vs 14.8±15?mm?Hg, P<0.0001) after the procedure with a mean decrease of 5.75±2.87?mm?Hg of left ventricular end diastolic pressure (P<0.001). Follow up results revealed that ventricular remodelling occurred mainly 1-3 months after the procedure, but without evidence of ventricular dilation and contract dysfunction. Heart function (NYHA) was greatly improved (3.4±0.5 vs 1.1±0.4, P<0.001) and exercise endurance increased. A renewed increase of LVOTG was found in 2 patients during follow-up. Conclusions LVOTG was greatly decreased in HOCM patients undergoing a PTSMA procedure, and their symptoms were greatly improved without cardiac complications during follow-up. Sub-selection and re-opening of target vessels were the causes of renewed increase of LVOTG, and this can be avoided with the accumulation of experience. This is a promising method for the treatment of symptomatic patients with HOCM.

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

    Directory of Open Access Journals (Sweden)

    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

  6. Septal Myectomy Surgery to Treat Obstructive Hypertrophic Cardiomyopathy (HCM)

    Medline Plus

    Full Text Available Septal Myectomy Surgery to Treat Obstructive Hypertrophic Cardiomyopathy (HCM) Click Here to view the BroadcastMed, Inc. Privacy Policy and Legal Notice © 2017 BroadcastMed, Inc. All rights reserved.

  7. Amplatzer Septal Occluder-induced Transient Complete Atrioventricular Block

    Directory of Open Access Journals (Sweden)

    Shan-Miao Lin

    2007-12-01

    Full Text Available Percutaneous transcatheter atrial septal defect (ASD closure is a widely used technique that has replaced open-heart surgical closure in many centers. The most common implant is the Amplatzer septal occluder which seems to be a highly effective and safe device. However, there are reports of complications associated with its implantation. We report a 9-year-old boy who presented with complete atrioventricular block after undergoing percutaneous closure of a large secundum ASD with an Amplatzer septal occluder. We treated the patient with oral prednisolone. The patient's atrioventricular conduction improved to second-degree Mobitz type 1 block on post-procedure day 24 and first-degree block on day 35. We conclude that patients with Amplatzer septal occluder-induced complete atrioventricular block generally have a good outcome, although it may take several weeks for improvement.

  8. Septal Myectomy Surgery to Treat Obstructive Hypertrophic Cardiomyopathy (HCM)

    Science.gov (United States)

    Septal Myectomy Surgery to Treat Obstructive Hypertrophic Cardiomyopathy (HCM) Click Here to view the BroadcastMed, Inc. Privacy Policy and Legal Notice © 2017 BroadcastMed, Inc. All rights reserved.

  9. Limb girdle muscular dystrophies

    DEFF Research Database (Denmark)

    Vissing, John

    2016-01-01

    PURPOSE OF REVIEW: The aim of the study was to describe the clinical spectrum of limb girdle muscular dystrophies (LGMDs), the pitfalls of the current classification system for LGMDs, and emerging therapies for these conditions. RECENT FINDINGS: Close to half of all LGMD subtypes have been...

  10. Meaning of Muscular Dystrophy

    Science.gov (United States)

    ... MD Living With MD en español Qué significa distrofia muscular Over Labor Day, just as you're going ... blood test if a kid has Becker or Duchenne MD. Or the doctor might take a small piece of the muscle and look at it under a microscope to ...

  11. Meaning of Muscular Dystrophy

    Science.gov (United States)

    ... MD Living With MD en español Qué significa distrofia muscular Over Labor Day, just as you're ... grown-up. This article talks about two types: Duchenne and Becker MD. Generally, only boys get Duchenne ...

  12. 硝酸甘油对室间隔缺损合并心力衰竭婴幼儿血流动力学影响%Hemodynamic effects of intravenous nitroglycerin in infants and small children with ventricular septal defect and congestive heart failure

    Institute of Scientific and Technical Information of China (English)

    曾和平; 仇烨; 王冬玲; 韩梅; 李万镇; 陈永红; 马郁文; 杜军保

    1999-01-01

    目的:探讨静脉滴注硝酸甘油(nitroglycerin, NTG)对室间隔缺损合并充血性心力衰竭婴幼儿血流动力学影响.方法:15例室间隔缺损合并充血性心力衰竭婴幼儿,在连续监测心率和血压的情况下,静脉滴注NTG 0.4~0.8 μg.kg-1.min-1,利用超声心动图在用药前、用药后1 h、6 h分别测定左心室射血分数(ejection fraction, EF)、短轴缩短率(fractional shortening, FS)、肺动脉平均压(pulmonary artery mean pressure, PAMP)、肺毛细血管楔压(pulmonary capillary wedge pressure, PCWP)和左心室壁应力(left ventricular wall stress, LVWS)等.结果:PCWP用药前(2.8±0.4) kPa,用药后1 h降至(1.7±0.4) kPa,6 h降至(1.6±0.4) kPa(P<0.05); LVWS Ⅰ用药前(4.32±0.56) N/cm2,用药后1 h降至(3.65±0.59) N/cm2,6h降至(3.87±0.60) N/cm2(P<0.05);LVWS Ⅱ用药前(1.56±0.57) N/cm2,用药后1 h降至(1.33±0.55) N/cm2,6 h降至(1.30±0.57) N/cm2(P<0.05);LVWS Ⅲ用药前(2.64±0.54) N/cm2,用药后1h降至(2.26±0.57) N/cm2,6h降至(2.19±0.63) N/cm2(P<0.05),中期收缩应力用药前(1.48±0.43) N/cm2,用药后1 h降至(1.33±0.28) N/cm2,6 h为(1.37±0.38) N/cm2(P<0.01);静脉应用上述剂量NTG对心率、血压、EF、FS、PAMP及左向右分流量无明显影响.结论:小剂量静脉滴注NTG可降低PCWP和LVWS,而不影响其它血流动力学指标,具有改善小儿心力衰竭血流动力学的作用.

  13. Optimizing paced ventricular function in patients with and without repaired congenital heart disease by contractility-guided lead implant.

    Science.gov (United States)

    Karpawich, Peter P; Singh, Harinder; Zelin, Kathleen

    2015-01-01

    This study evaluates the concept of optimizing ventricular pacing in regard to functional cardiac response. Lead implant based on physiologic variables of contractility at various sites was performed in patients with and without congenital heart disease (CHD). Since right ventricular apical pacing may adversely alter contractility and myocellular function, septal and outflow tract pacing have been advocated. However, there are few studies in the young and essentially none in those with CHD. A total of 113 consecutive patients with and without repaired CHD, aged two to 51 (median 16), some with preexisting epicardial pacemakers, underwent transvenous pacemaker implant using standard sensing/pacing indices plus measurements of pressures, QRS, and contractility responses at each of five predefined potential ventricular implant sites: apex, inflow-, low-, mid-, and infundibular/outflow-septal with each patient serving as his/her own control. Implant was at the site of best contractility with active-fixation, low-threshold steroid-eluting leads. Measured contractility indices varied up to 31% (mean 12%) between sites per patient. Septal regions (mid-, inflow-, and low-) were associated with the most optimal and right ventricular epicardial showed the worst contractility (P implant sites are patient and CHD variable. Current lead designs ensure chronic stability/performance regardless of site. Proactive contractility-guided pacing implant can optimize chronic paced ventricular function. ©2014 Wiley Periodicals, Inc.

  14. 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.)

  15. Improved Right Ventricular Performance with Increased Tricuspid Annular Excursion in Athlete’s Heart

    Directory of Open Access Journals (Sweden)

    Himanshu eGupta

    2015-04-01

    Full Text Available Background: Marathon runners (MTH and patients with mitral regurgitation (MR exhibit left ventricular (LV overload, and LV geometric changes in these groups have been reported. In this study, right ventricular (RV adaptation to chronic volume overload was evaluated in MTH and MR and normal controls together with interventricular septal remodeling and tricuspid annulus (TA motion. Methods: A total of 60 age-matched subjects (including 19 MTH, 17 isolated chronic compensated MR patients and 24 normal subjects underwent conventional cine and tagged cardiac magnetic resonance imaging. Myocardial strain and curvature were computed on the interventricular septum and RV free wall. A dual-propagation technique was applied to construct RV volume-time curves for a single cardiac cycle. Similarly, the tricuspid annulus was tracked throughout the cardiac cycle to create displacement over time curve.Results: Septal curvature was significantly lower in MTH and MR compared to controls. No significant differences in RV free wall strain or RV ejection fraction were noted amongst the 3 groups. However, longitudinal TA excursion was significantly higher in MTH compared to controls (p=0.0061. The peak late diastolic TA velocity in MR was significantly faster than MTH (p=0.0031 and controls (p=0.020. Conclusions: Increased TA kinetics allows for improved RV performance in MTH. Septal remodeling was observed in both MR and MTH, therefore a direct relationship of septal remodeling to TA kinetics in athlete’s heart could not be elucidated in this study.

  16. Long-Term Outcomes of Ataluren in Duchenne Muscular Dystrophy

    Science.gov (United States)

    2017-07-11

    Muscular Dystrophy, Duchenne; Muscular Dystrophies; Muscular Disorders, Atrophic; Muscular Diseases; Musculoskeletal Disease; Neuromuscular Diseases; Nervous System Diseases; Genetic Diseases, X-Linked; Genetic Diseases, Inborn

  17. Ventricular torsional relation to ventricular fiber arrangement

    CERN Document Server

    Ranjbar, Saeed; Meybodi, Mahmood Emami

    2014-01-01

    Left ventricular torsion from helically oriented myofibers is a key parameter of cardiac performance. Physicians observing heart motion on echocardiograms, during cardiac catheterization, or in the operating room, are impressed by the twisting or rotary motion of the left ventricle during systole. Conceptually, the heart has been treated as a pressure chamber. The rotary or torsional deformation has been poorly understood by basic scientists and has lacked clinical relevance. The aim of this paper attempts to discuss about this question: Is ventricular twisting related to ventricular fiber arrangement? That is dependent to an assumed model of the left ventricular structure.

  18. [Fetal atrioventricular septal defect associated with Patau and Edwards syndromes, as well as trisomy 22].

    Science.gov (United States)

    Cesko, I; Hajdú, J; Marton, T; Tóth-Pál, E; Papp, C; Papp, Z

    1998-05-03

    The atrioventricular septal defect is usually associated with trisomy 21 and it may be observed in the heterotaxia syndromes. Atrioventricular septal defect may be associated with 8p deletion. There are reported cases of familial atrioventricular septal defect. Atrioventicular septal defect is rarely associated with other chromosomal abnormalities. We are reporting three unusual cases of atrioventricular septal defect that were associated with trisomy 13, 18 and 22. This association may be due to effect of genetic loci on the 13, 18 and 22 chromosome which could play the role in the development and fusion of endocardial cushion and atrioventricular septal defect.

  19. The superhealing MRL background improves muscular dystrophy

    Directory of Open Access Journals (Sweden)

    Heydemann Ahlke

    2012-12-01

    Full Text Available Abstract Background Mice from the MRL or “superhealing” strain have enhanced repair after acute injury to the skin, cornea, and heart. We now tested an admixture of the MRL genome and found that it altered the course of muscle pathology and cardiac function in a chronic disease model of skeletal and cardiac muscle. Mice lacking γ-sarcoglycan (Sgcg, a dystrophin-associated protein, develop muscular dystrophy and cardiomyopathy similar to their human counterparts with limb girdle muscular dystrophy. With disruption of the dystrophin complex, the muscle plasma membrane becomes leaky and muscles develop increased fibrosis. Methods MRL/MpJ mice were bred with Sgcg mice, and cardiac function was measured. Muscles were assessed for fibrosis and membrane leak using measurements of hydroxyproline and Evans blue dye. Quantitative trait locus mapping was conducted using single nucleotide polymorphisms distinct between the two parental strains. Results Introduction of the MRL genome reduced fibrosis but did not alter membrane leak in skeletal muscle of the Sgcg model. The MRL genome was also associated with improved cardiac function with reversal of depressed fractional shortening and the left ventricular ejection fraction. We conducted a genome-wide analysis of genetic modifiers and found that a region on chromosome 2 was associated with cardiac, diaphragm muscle and abdominal muscle fibrosis. Conclusions These data are consistent with a model where the MRL genome acts in a dominant manner to suppress fibrosis in this chronic disease setting of heart and muscle disease.

  20. Ullrich Congenital Muscular Dystrophy

    OpenAIRE

    2011-01-01

    ObjectiveUllrich congenital muscular dystrophy is a rather severe type of congenitalmuscular dystrophy with early onset features related to motor development.In general it is inherited in autosomal recessive principles, however in theWestern world mostly seen with de novo dominant mutations in the collagenVI genes. Milder form of the condition is the Bethlem myopathy. There may beoverlap forms in the clinic resembling the Ehler-Danlos syndrome. There hasbeen some radical efforts for cure espe...

  1. Cardiac Arrest due to Recurrent Ventricular Fibrillation Triggered by Unifocal Ventricular Premature Complexes in a Silent Myocardial Infarction

    Directory of Open Access Journals (Sweden)

    Dong Hyun Lee

    2014-11-01

    Suspecting idiopathic VF, emergency radiofrequency catheter ablation was planned for the VPCs. However, when coronary angiography was performed to exclude silent ischemia, the results showed a total occlusion of the right coronary artery posterolateral branch, which is thought to supply the left ventricular inferior and septal wall. After successful reperfusion, VF episodes and the triggering VPCs disappeared. We are documenting this case to emphasize the potential for silent myocardial infarction to cause out-of-hospital sudden cardiac arrest even in a patient without any symptom or sign of acute coronary syndrome.

  2. Morphometric analysis of septal aperture of humerus

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    Raghavendra K, Anil kumar Reddy Y, Shirol VS, Daksha Dixit, Desai SP

    2014-04-01

    Full Text Available Introduction: Lower end of humerus shows olecranon and coronoid fossae separated by a thin bony septum, sometimes it may deficient and shows foramen which communicates both the fossae called Septal aperture, which is commonly referred as supratrochlear foramen (STF. Materials & Methods: We have studied 260 humeri (126 right side and 134 left side, measurements were taken by using vernier caliper, translucency septum was observed by keeping the lower end of humerus against the x-ray lobby. Results: A clear cut STF was observed in 19.2% bones, translucency septum was observed in 99 (91.6% humeri on the right side and 95 (93.1% humeri on the left sides respectively (Table – 1. Clinical significance: The presence of STF is always associated with the narrow medullary canal at the lower end of humerus, Supracondylar fracture of humerus is most common in paediatric age group, medullary nailing is done to treat the fractures in those cases the knowledge about the STF is very important for treating the fractures. It has been observed in x-ray of lower end of the humerus the STF is comparatively radiolucent, it is commonly seen as a type of ‘pseudolesions’ in an x-ray of the lower end of humerus and it may mistake for an osteolytic or cystic lesions. Conclusion: The present study can add data into anthropology and anatomy text books regarding STF and it gives knowledge of understanding anatomical variation of distal end of the humerus, which is significant for anthropologists, orthopaedic surgeons and radiologists in habitual clinical practice.

  3. Nasal Septal Deviations: A Systematic Review of Classification Systems

    Directory of Open Access Journals (Sweden)

    Jeffrey Teixeira

    2016-01-01

    Full Text Available Objective. To systematically review the international literature for internal nasal septal deviation classification systems and summarize them for clinical and research purposes. Data Sources. Four databases (including PubMed/MEDLINE were systematically searched through December 16, 2015. Methods. Systematic review, adhering to PRISMA. Results. After removal of duplicates, this study screened 952 articles for relevance. A final comprehensive review of 50 articles identified that 15 of these articles met the eligibility criteria. The classification systems defined in these articles included C-shaped, S-shaped, reverse C-shaped, and reverse S-shaped descriptions of the septal deviation in both the cephalocaudal and anteroposterior dimensions. Additional studies reported use of computed tomography and categorized deviation based on predefined locations. Three studies graded the severity of septal deviations based on the amount of deflection. The systems defined in the literature also included an evaluation of nasal septal spurs and perforations. Conclusion. This systematic review ascertained that the majority of the currently published classification systems for internal nasal septal deviations can be summarized by C-shaped or reverse C-shaped, as well as S-shaped or reverse S-shaped deviations in the anteroposterior and cephalocaudal dimensions. For imaging studies, predefined points have been defined along the septum. Common terminology can facilitate future research.

  4. Nasal Septal Deviations: A Systematic Review of Classification Systems

    Science.gov (United States)

    Teixeira, Jeffrey; Certal, Victor; Chang, Edward T.; Camacho, Macario

    2016-01-01

    Objective. To systematically review the international literature for internal nasal septal deviation classification systems and summarize them for clinical and research purposes. Data Sources. Four databases (including PubMed/MEDLINE) were systematically searched through December 16, 2015. Methods. Systematic review, adhering to PRISMA. Results. After removal of duplicates, this study screened 952 articles for relevance. A final comprehensive review of 50 articles identified that 15 of these articles met the eligibility criteria. The classification systems defined in these articles included C-shaped, S-shaped, reverse C-shaped, and reverse S-shaped descriptions of the septal deviation in both the cephalocaudal and anteroposterior dimensions. Additional studies reported use of computed tomography and categorized deviation based on predefined locations. Three studies graded the severity of septal deviations based on the amount of deflection. The systems defined in the literature also included an evaluation of nasal septal spurs and perforations. Conclusion. This systematic review ascertained that the majority of the currently published classification systems for internal nasal septal deviations can be summarized by C-shaped or reverse C-shaped, as well as S-shaped or reverse S-shaped deviations in the anteroposterior and cephalocaudal dimensions. For imaging studies, predefined points have been defined along the septum. Common terminology can facilitate future research. PMID:26933510

  5. Concentric left ventricular morphology in aerobically trained kayak canoeists.

    Science.gov (United States)

    Gates, Phillip E; Campbell, Ian G; George, Keith P

    2004-09-01

    The aim of the present study was to test the hypothesis that upper body aerobically trained athletes (kayak canoeists) would have greater left ventricular wall thickness, but similar left ventricular diastolic chamber dimensions, compared with recreationally active and sedentary men. Ultrasound echocardiography was used to determine cardiac structure and function in highly trained kayak canoeists (n = 10), moderately active (n = 10) and sedentary men (n = 10). The septal and posterior left ventricular walls were approximately 0.2 cm thicker in kayak canoeists (P kayak canoeists had a concentric pattern of left ventricular adaptation to aerobic upper body training. Scaling the data to body composition indices had no effect on the outcome of the statistical analysis. There were no differences in resting Doppler left ventricular diastolic or systolic function among the groups. Ejection fraction was lower in the kayak canoeists, but the magnitude of the difference was within the normal variability for this measurement. Thus aerobically upper body trained athletes demonstrated a concentric pattern of cardiac enlargement, but resting left ventricle function was not different between athletes, moderately active and sedentary individuals.

  6. 57. Growth of left ventricular outflow tract after repair of ventricular septal defect and aortic arch obstruction

    Directory of Open Access Journals (Sweden)

    Abdulraouf Jijeh

    2015-10-01

    Conclusion: There is significant LVOT growth expected after repair of VSD and aortic arch obstruction. Small aortic valve and LVOT at diagnosis of those cases are not correlating with the need of surgical reintervention for LVOT obstruction.

  7. Evaluation of left ventricular systolic function in young adults with mitral valve prolapse

    Science.gov (United States)

    Malev, Eduard; Zemtsovsky, Eduard; Pshepiy, Asiyet; Timofeev, Eugeny; Reeva, Svetlana; Prokudina, Maria

    2012-01-01

    OBJECTIVE: To evaluate left ventricular function in young adults with mitral valve prolapse (MVP) without significant mitral regurgitation using two-dimensional strain imaging. METHODS AND RESULTS: A total of 58 asymptomatic young subjects (mean [± SD] age 19.7±1.6 years; 72% male) with MVP were compared with 60 sex- and age-matched healthy subjects. MVP was diagnosed by billowing one or both mitral leaflets >2 mm above the mitral annulus in the long-axis parasternal view. Longitudinal, radial and circumferential strain and strain rate were determined using speckle tracking with a grey-scale frame rate of 50 fps to 85 fps. There were no significant differences in the global systolic left ventricular function of the subjects with MVP compared with the control group. In the MVP group, most of the global myocardial systolic deformation indexes were not reduced. Only the global circumferential strain showed a decrease in the prolapse subjects. Regional, longitudinal, circumferential and radial strain and strain rate were decreased only in septal segments. A decrease in the rotation of the same septal segments at the basal level was also observed. CONCLUSION: Regional septal myocardial deformation indexes decrease in subjects with MVP. These changes may be the first sign indicating the deterioration of left ventricular systolic function as well as the existence of primary cardiomyopathy in asymptomatic young subjects with MVP. PMID:23592928

  8. Horizontal ventricular septum with dextroversion: hearts with and without aortic atresia.

    Science.gov (United States)

    Thilenius, O G; Bharati, S; Lev, M; Karp, R B; Arcilla, R A

    1987-01-01

    Two hearts with horizontal ventricular septum, dextroversion (situs solitus), ventricular septal defects, and malaligned great vessels are reported. One of the hearts had aortic atresia and the infant died; the other patient had a Fontan-type physiologic correction. Reviewing the literature, the following conclusions are drawn: (a) Hearts with horizontal ventricular septum and those with criss-cross atrioventricular connections may be the result of different degrees of rotation of the ventricular muscle mass. This rotation is not likely to be postseptational but preseptational. (b) Only those hearts with a complete 180 degrees rotation should be called criss-cross hearts. (c) Partial rotation results in a horizontal septum such that the right ventricle is invariably superior, regardless of atrioventricular concordance or discordance, situs solitus or inversus, or dextroversion. (d) Physiologic surgical correction is often possible but has to be tailored to the details of each heart.

  9. Intracoronary electrocardiogram during alcohol septal ablation for hypertrophic obstructive cardiomyopathy predicts myocardial injury size.

    Science.gov (United States)

    Meng, Jing; Qu, Xiaolong; Huang, Haiyun; Zhang, Shanwen; Zhao, Weibo; He, Guoxiang; Song, Zhiyuan; Hu, Houyuan

    2016-01-01

    Alcohol septal ablation (ASA) has been used widely to treat patients with hypertrophic obstructive cardiomyopathy (HOCM). During the routine ASA procedure, it is difficult to detect the septal injury in real-time. The aim of the present study is to assess myocardial injury during ASA by recording intracoronary electrocardiogram (IC-ECG). From 2012 to 2015, 31 HOCM patients were treated with ASA, and IC-ECG was recorded in 21 patients successfully before and after ethanol injection. The elevation of ST-segment on IC-ECG after ethanol injection was expressed as its ratio to the level before injection or the absolute increasing value. Blood samples were collected before and after ASA for measuring changes in cardiac biomarkers. The ratio value of ST-segment elevation was positively correlated with both the amount of ethanol injected (r = 0.645, P = 0.001) and the myocardial injury size (creatine kinase-MB area under the curve (AUC) of CK-MB) (r = 0.466, P = 0.017). The absolute increment of ST-segment was also positively associated with both the amount of ethanol (r = 0.665, P = 0.001) and AUC of CK-MB (0.685, P = 0.001). However, there was no statistical correlation between the reduction of left ventricular outflow tract gradient and ST-segment elevation. Additionally no severe ASA procedure-related complications were observed in our patients. In conclusion, myocardial injury induced by ethanol injection can be assessed immediately by ST-segment elevation on IC-ECG. This study is the first to show that IC-ECG is a useful method for predicting myocardial injury during ASA in real-time.

  10. Atrial Septal Defect in a Very Old Woman

    Science.gov (United States)

    Pinho, Elika; Gomes, Andre Amaral; Silva, Maria Joao; Torres, Tiago Pinheiro; Coelho, Andreia; Almeida, Pedro Bernardo; Lourenco, Patricia; Bettencourt, Paulo

    2013-01-01

    Atrial Septal Defect (ASD) is one of the most frequently congenital heart diseases in adults and it is often asymptomatic until adulthood. We report a case of a 90-year-old woman admitted to hospital with dyspnea and orthopnea insidiously progressing over the preceding 5 years and becoming severe with dyspnea on minimal activities, orthopnea and paroxysmal nocturnal dyspnea, in the last 2 weeks. The transthoracic echocardiogram revealed an atrial septal defect ostium secundum type, with left-to-right shunt, moderate to severe tricuspid insufficiency, severe pulmonary hypertension (72 mmHg) and preserved biventricular function. With diuretic therapy optimization the patient showed symptomatic improvement. This present case represents and unusual and very late presentation of an atrial septal defect ostium secundum type, which is usually diagnosed at the mild adult age. Our patient lived symptom-free for over 80 years.

  11. Platypnea-orthodeoxia induced by fenestrated atrial septal aneurysm.

    Science.gov (United States)

    Giombolini, Claudio; Notaristefano, Salvatore; Santucci, Stefano; Savino, Ketty; Pasquino, Stefano; Ragni, Temistocle; Ambrosio, Giuseppe

    2005-02-01

    Platypnea-orthodeoxia is a peculiar syndrome characterized by a right-to-left shunt, which occurs in the upright position. The diagnosis is made by contrast transesophageal echocardiography, paying attention to include contrast visualization in the orthostatic decubitus. The association of this syndrome with a fenestrated atrial septal aneurysm is rare and probably underlies a peculiar and also rare mechanism of shunting in presence of normal pulmonary pressure. We report of a case of a 58-year-old man with a fenestrated atrial septal aneurysm and platypnea-orthodeoxia syndrome treated by surgical closure of the atrial defect.

  12. 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.

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

    Science.gov (United States)

    Poelmann, Robert E; Gittenberger-de Groot, Adriana C; Vicente-Steijn, Rebecca; Wisse, Lambertus J; Bartelings, Margot M; Everts, Sonja; Hoppenbrouwers, Tamara; Kruithof, Boudewijn P T; Jensen, Bjarke; de Bruin, Paul W; Hirasawa, Tatsuya; Kuratani, Shigeru; Vonk, Freek; van de Put, Jeanne M M S; de Bakker, Merijn A; Richardson, Michael K

    2014-01-01

    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 evolution and development

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

    Directory of Open Access Journals (Sweden)

    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

  15. Assessment of ventricular coupling with real-time cine MRI and its value to differentiate constrictive pericarditis from restrictive cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Francone, Marco; Dymarkowski, Steven; Kalantzi, Maria; Rademakers, Frank E.; Bogaert, Jan [Gasthuisberg University Hospital, Department of Radiology and Cardiology, Leuven (Germany)

    2006-04-15

    The purpose of this study was to evaluate the use of respiratory-related ventricular coupling to differentiate patients with constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM). In 18 histologically proven cases of CP, 6 patients with inflammatory pericarditis (IP), 15 RCM patients and 17 normal subjects, real-time cine MRI was performed in the cardiac short-axis (basal half of the ventricles) during operator-guided deep respiration. The images were analyzed for ventricular septal position and shape during early ventricular filling. Early diastolic septal inversion (I) or flattening (F) was found in all CP (I:15,F:3), and in all IP (I:2,F:4), but seldom in normals (F:1) and not in RCM. The septal abnormalities occurred at the onset of inspiration and rapidly disappeared with the next heartbeats. The amount of ventricular coupling was evaluated by quantifying the difference in the maximal septal excursion between inspiration and expiration. This parameter, normalized to the biventricular diameter, was significantly larger in CP (20.0{+-}4.5%, P<0.0001) and IP (14.8{+-}3.2%, P<0.0001) patients than in normals (7.0{+-}2.4%), whereas RCM patients had a trend toward decreased excursion (4.2{+-}1.7%, P=0.11). A cut-off value of 11.8% (mean normals +2 SD) enabled to differentiate CP patients from normals and RCM patients completely. Real-time cine MRI can easily depict increased ventricular coupling, which may be helpful to better differentiate between CP and RCM patients, especially in patients with normal or minimally thickened pericardium. The increase in coupling in IP patients is likely caused by decreased compliance of the inflamed pericardial layers. (orig.)

  16. Ruptured Sinus of Valsalva Aneurysm Initially Misdiagnosed as Ventricular Septal Defect by Echocardiography

    Directory of Open Access Journals (Sweden)

    GR Rezaian

    2009-06-01

    Full Text Available Aneurysms of sinus of valsalva are rare cardiac lesions and most of them are in congenital origin. The malformation consists of a separation or lack of fusion between the media of the aorta and the annulus fibrosis of the aortic valve. The structure becomes aneurysmal and may rupture to form a fistula. We present a case of ruptured sinus of valasalva aneurysm in a 25-year-old man. The diagnosis was made by echocardiography and confirmed at operation.

  17. [Prognostic factors associated with postoperative morbidity in children with isolated ventricular septal defect].

    Science.gov (United States)

    Castro-Rodríguez, Claudia Olimpia; Rodríguez-Hernández, Lydia; Estrada-Loza, María Jesús; Herrera-Márquez, Julia Rocío; Gómez-Salvador, Maricela; Flores-Lujano, Janet; Núñez-Enríquez, Juan Carlos

    2015-01-01

    Introducción: la comunicación interventricular aislada (CIV) es una de las cardiopatías congénitas más frecuentes a nivel mundial. Se desconoce cuáles son los factores pronósticos asociados a la morbilidad postoperatoria de los pacientes con CIV aislada en el Hospital de Pediatría del Centro Médico Nacional Siglo XXI. Métodos: se realizó un estudio de cohorte retrospectivo del 1 de enero de 2009 al 31 de diciembre de 2013. Se utilizó estadística descriptiva y comparaciones entre los grupos mediante chi cuadrada, U de Mann Whitney y regresión lineal. Se calcularon riesgos relativos (RR) e intervalos de confianza al 95 % (IC 95%). Resultados: se incluyeron 64 pacientes a quienes se les realizó cierre de comunicación interventricular aislada. El 12.5 % (n = 8) tenían antecedente de cerclaje pulmonar y 22 (34.3 %) pacientes tenían síndrome de Down. Las CIV perimembranosas y la subaórtica se presentaron en 70 % de los casos. Los factores asociados a una mayor morbilidad y estancia prolongada en terapia intensiva fueron: lactato al final de la circulación extracorpórea (CEC) mayor a 3mmol/L (p = 0.02) y el síndrome de Down (p = 0.02) quienes presentaron una mayor frecuencia de complicaciones postoperatorias (p = 0.02). Conclusiones: se encontró una asociación estadísticamente significativa: cuanto mayor era la clase funcional preoperatoria, mayor era su estancia intrahospitalaria, con mayor número de días en terapia intensiva y mayor tiempo de ventilación mecánica asistida.

  18. Permanent chronotropic impairment after closure of atrial or ventricular septal defect

    DEFF Research Database (Denmark)

    Heiberg, Johan; Nyboe, Camilla; Hjortdal, Vibeke E

    2017-01-01

    on the chronotropic response to exercise. METHODS: We performed a search protocol based on the 'Preferred Reporting Items for Systematic Reviews and Meta- Analyses' (PRISMA) guidelines. A data collection form was specifically developed and data were extracted from the included studies by a primary reviewer...

  19. Discharge planning for children with ventricular septal defect and pulmonary arterial hypertension in China

    Directory of Open Access Journals (Sweden)

    Xiaoxiao Wu

    2015-06-01

    Conclusions: Discharge planning improves the maternal discharge readiness, maternal caring knowledge and maternal caring behaviors. However, this planning did not reduce the readmission rate of children with CHD-PAH.

  20. left ventricular inflow obstruction by giant atrial septal aneurysm in a ...

    African Journals Online (AJOL)

    ANEURYSM IN A NEONATE WITH HYPOPLASTIC RIGHT HEART. SYNDROME: CASE ... hypoplastic cardiac malformations of the right and left heart. In the adult .... then, Management and Staff Newborn Unit Kijabe. Mission Hospital.

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

    Energy Technology Data Exchange (ETDEWEB)

    Mishiro, Yuichiro; Oki, Takashi [Tokushima Univ. (Japan). School of Medicine; Iuchi, Arata [and others

    1999-06-01

    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 ({Delta}T) 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 {Delta}T was significantly shorter for all 4 walls, particularly the anterior and ventricular septal regions. The standard deviation for the {Delta}T, calculated from the {Delta}T 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)

  2. Transcatheter Closure of Atrial Septal Defect with Amplatzer Device in Children and Adolescents: Short and Midterm Results; an Iranian Experience

    Directory of Open Access Journals (Sweden)

    Saiyed-Habibollah Hosseini

    2011-06-01

    Full Text Available Objective:Transcatheter closure of secundum atrial septal defect (ASD with Amplatzer device is an alternative procedure to surgical repair, with some limitations. The aim of this study was to assess the initial and mid-term results of the treatment of ASD with Amplatzer septal occluder in children and adolescents. Methods:From May 2003 to January 2008 sixty three consecutive children and adolescent patients underwent transcatheter closure of ASD at a mean±SD age of 8.5±4.8 years (range 2.2 to 18 years. All procedures were performed under local anesthesia and moderate sedation or general anesthesia with transthoracic echocardiography and fluoroscopic guidance. Stretch diameter of ASD was determined by balloon sizing catheter. Device selection was based on and matched to the standard diameter of the septal defect. Follow up at 24 hours, 1 month, 6 months, 12 months and yearly thereafter included physical examination, electrocardiography and transthoracic echocardiography. Findings:The mean ASD diameter, measured with transthoracic echocardiography and balloon catheter were 19.5± 5.5 mm and 20.9± 6.2 mm, respectively. The mean follow up period was 32.4±18.8 months. Deployment of the device was successful in 57 (90.5% and failed in 6 (9.5% patients. The major complication included dislodgement of device in 1 patient and device embolization to right ventricular inlet (surgically removed in 1 patient. The minor complication included transient atrial tachycardia in 10 patients, paroxysmal supraventricular tachycardia in 2 patients during procedure, successfully terminated with medication. At 24 hour, 1 month, 6 month and 1 year follow up, total occlusion rates were 73.6 %, 91%, 94.7%, and 94.7%, respectively. Conclusion:Transcatheter occlusion of ASD with Amplatzer device is an effective and safe procedure with minimal complication rate and short hospital stay, as well as excellent short and intermediate outcome in children and adolescents.

  3. Muscle diseases: the muscular dystrophies.

    Science.gov (United States)

    McNally, Elizabeth M; Pytel, Peter

    2007-01-01

    Dystrophic muscle disease can occur at any age. Early- or childhood-onset muscular dystrophies may be associated with profound loss of muscle function, affecting ambulation, posture, and cardiac and respiratory function. Late-onset muscular dystrophies or myopathies may be mild and associated with slight weakness and an inability to increase muscle mass. The phenotype of muscular dystrophy is an endpoint that arises from a diverse set of genetic pathways. Genes associated with muscular dystrophies encode proteins of the plasma membrane and extracellular matrix, and the sarcomere and Z band, as well as nuclear membrane components. Because muscle has such distinctive structural and regenerative properties, many of the genes implicated in these disorders target pathways unique to muscle or more highly expressed in muscle. This chapter reviews the basic structural properties of muscle and genetic mechanisms that lead to myopathy and muscular dystrophies that affect all age groups.

  4. Evaluation of global and regional left ventricular systolic function in patients with frequent isolated premature ventricular complexes from the right ventricular outflow tract

    Institute of Scientific and Technical Information of China (English)

    YAO Jing; XU Jing; YONG Yong-hong; CAO Ke-jiang; CHEN Shao-liang; XU Di

    2012-01-01

    Background Frequent premature ventricular complexes from the right ventricular outflow tract (RVOT-PVCs) are associated with left ventricular dysfunction.This study adopted two-dimensional speckle tracking imaging to evaluate global and regional left ventricular myocardial function in patients with frequent RVOT-PVCs.Methods This study included 30 patients with frequent RVOT-PVCs and 30 healthy subjects.Aortic systolic velocity-time integral (AoVTI) and myocardium strain in circumferential (CS),radial (RS) and longitudinal (LS) directions were evaluated by conventional echocardiography and speckle tracking imaging.All values of patients with RVOT-PVCs were recorded during sinus (PVC-S) and PVC beats (PVC-V).Results Significant differences were demonstrated in global CS,RS and LS between the control subjects and the PVC-V (CS:(17.46±2.48)% vs.(11.52±3.28)%,RS:(48.26±10.20)% vs.(20.92±9.78)%,LS:(19.89±2.62)% vs.(11.79±3.66)%,P <0.01),and in segmental RS and LS of nearly all the left ventricular segments.Statistical differences in segmental CS between the PVC-V and the control subjects were only observed in anterior,anteroseptal and septal segments (only seen in anteroseptal and septal segments at apex).Furthermore,V/S AoVTI (AoVTI during the PVC beat divided by AoVTI during the sinus beat,then multiplied by 100%) correlated with coupling interval (r=0.67,P <0.001) and global strain (CS:r=0.48,P=0.007; RS:r=0.65,P <0.001; LS:r=0.65,P <0.001).Conclusions Frequent RVOT-PVCs can induce global and regional left ventricular systolic dysfunction.The reduction of hemodynamic parameters relates to the coupling interval and the global systolic function.

  5. 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.

  6. Time and age dependent decrease of NT-proBNP after septal myectomy in hypertrophic obstructive cardiomyopathy.

    Science.gov (United States)

    Cui, Hao; Wu, Xi; Wang, Shuiyun; Tang, Bing; Zhu, Changsheng; Chen, Haibo; Zhou, Xiaohui; Wu, Rong; Yu, Qinjun; Huang, Xiaohong

    2017-08-28

    NT-proBNP level is a predictive factor in hypertrophic cardiomyopathy patients, in which left ventricular outflow tract obstruction contributes to an increased NT-proBNP level. However, studies regarding the influence of septal myectomy on NT-proBNP level in hypertrophic obstructive cardiomyopathy are lacking. One hundred and eighty-two patients who underwent septal myectomy in Fuwai Hospital from May 2011 to March 2016 and were included in the present study. Preoperative and follow-up NT-proBNP was retrospectively collected. Correlation analysis was performed to determine the factors affecting preoperative NT-proBNP and postoperative decrease. The cohort had a median age of 46.2 [IQR: 36.5-53.1] years, and 106 (58.2%) patients were male. NT-proBNP decreased to 816.5 [IQR: 400.3-1661.8] pg/mL from preoperative 1732.4 [IQR: 819-3296.5] pg/mL (pdecrease was positively correlated with age (pdecrease after myectomy. Myectomy significantly reduced NT-proBNP level in hypertrophic obstructive cardiomyopathy patients, in which a time-dependent manner of decrease indicated myocardial remodeling of the heart after myectomy.

  7. Echocardiographic evaluation of patent foramen ovale and atrial septal defect.

    Science.gov (United States)

    Hari, Pawan; Pai, Ramdas G; Varadarajan, Padmini

    2015-01-01

    Patent foramen ovale (PFO) is a common variant present in up to 25% of the population. Atrial septal defect (ASD) is a direct communication between the 2 atrial chambers, of which the ostium secundum variety is the most common. This manuscript is an in depth review of the complex atrial septation, the diagnosis of PFO and ASD and its clinical and therapeutic implications.

  8. Familial Atrial Septal Defect and Sudden Cardiac Death

    DEFF Research Database (Denmark)

    Ellesøe, Sabrina Gade; Johansen, Morten Munk; Bjerre, Jesper Vandborg;

    2016-01-01

    OBJECTIVE: Atrial septal defect (ASD) is the second most common congenital heart defect (CHD) and is observed in families as an autosomal dominant trait as well as in nonfamilial CHD. Mutations in the NKX2-5 gene, located on chromosome 5, are associated with ASD, often combined with conduction...

  9. [Duchenne muscular dystrophy pathophysiology].

    Science.gov (United States)

    Péréon, Y; Mercier, S; Magot, A

    2015-12-01

    Dystrophin is a large cytoskeletal protein located at the plasma membrane in both muscle and non-muscle tissues, which mediates interactions between the cytoskeleton, cell membrane, and extracellular matrix. Dystrophin is a key component of multiprotein complexes (dystrophin- associated glycoprotein complex, or DGC). It is also involved in many intracellular cascades affecting membrane proteins such as calcium channels, or various signalisation pathways. In Duchenne Muscular Dystrophy, both dystrophin and DGC proteins are missing. This induces excessive membrane fragility and permeability, dysregulation of calcium homeostasis, oxidative damage, which in turn favour muscle cell necrosis. The latter is initially followed by regeneration. With age, the regenerative capacity of the muscles appears to be exhausted and muscle fibres are gradually replaced by connective and adipose tissue. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  10. Long-Term Outcome of Alcohol Septal Ablation for Obstructive Hypertrophic Cardiomyopathy in the Young and the Elderly.

    Science.gov (United States)

    Liebregts, Max; Steggerda, Robbert C; Vriesendorp, Pieter A; van Velzen, Hannah; Schinkel, Arend F L; Willems, Rik; van Cleemput, Johan; van den Berg, Maarten P; Michels, Michelle; ten Berg, Jurriën M

    2016-03-14

    The aim of this study was to compare outcomes of alcohol septal ablation (ASA) in young and elderly patients with obstructive hypertrophic cardiomyopathy (HCM). The American College of Cardiology Foundation/American Heart Association guidelines reserve ASA for elderly patients and patients with serious comorbidities. Information on long-term age-specific outcomes after ASA is scarce. This cohort study included 217 HCM patients (age 54 ± 12 years) who underwent ASA because of symptomatic left ventricular outflow tract obstruction. Patients were divided into young (age ≤55 years) and elderly (age >55 years) groups and matched by age in a 1:1 fashion to nonobstructive HCM patients. Atrioventricular block following ASA was more common in elderly patients (43% vs. 21%; p = 0.001), resulting in pacemaker implantation in 13% and 5%, respectively (p = 0.06). Residual left ventricular outflow tract gradient, post-procedural New York Heart Association functional class, and necessity for additional septal reduction therapy was comparable between age groups. During a follow-up of 7.6 ± 4.6 years, 54 patients died. The 5- and 10-year survival following ASA was 95% and 90% in patients age ≤55 years and 93% and 82% in patients age >55 years, which was comparable to their control groups. The annual adverse arrhythmic event rate following ASA was 0.7%/year in young patients and 1.4%/year in elderly patients, which was comparable to their control groups. ASA is similarly effective for reduction of symptoms in young and elderly patients; however, younger patients have a lower risk of procedure-related atrioventricular conduction disturbances. The long-term mortality rate and risk of adverse arrhythmic events following ASA are low, both in young and elderly patients, and are comparable to age-matched nonobstructive HCM patients. Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  11. Extra-anatomic right ventricle to pulmonary artery conduit: the apical-aortic equivalent for complex right ventricular outflow tract reconstruction.

    Science.gov (United States)

    Mori, Makoto; Siddharthan, Ragavan; Kogon, Brian

    2015-03-01

    We describe the case of a 22-year-old with tetralogy of Fallot, who underwent complete repair with ventricular septal defect closure and right ventricle to pulmonary artery conduit placement. She has undergone numerous subsequent conduit changes, each complicated by early stenosis and failure. Unfortunately, serial conduit changes can become increasingly challenging. While extra-anatomic conduits have been described for complex left ventricular outflow tract obstruction, they have not been described for right-sided obstruction. Herein, we present a patient who underwent successful placement of an extra-anatomic valved right ventricle to pulmonary artery conduit in the setting of complex right ventricular outflow tract obstruction.

  12. Cardiac Resynchronization for Corrected Transposition of the Great Arteries with Systemic Right Ventricle Failure after Tricuspid Valve Replacement and Ventricle Septal Defect Closure

    Directory of Open Access Journals (Sweden)

    Kosuke Fujii, MD

    2010-01-01

    Full Text Available A 32-year-old man developed systemic right ventricular (RV heart failure after ventricular septal defect (VSD closure and tricuspid valve replacement for corrected transposition of the great arteries with VSD and Ebstein anomaly. He subsequently experienced RV failure with wide QRS and atrial fibrillation (AF. Because corrective surgery for this condition seemed over risky, we decided to perform cardiac resynchronization therapy with implantation of an implantable cardioverter defibrillator (CRT-D. After CRT-D device implantation, the patient showed improved performance status in terms of New York Heart Association functional class, B-type brain natriuretic peptide levels, RV ejection fraction and cardiac electrical rhythm. CRT-D implantation is a useful approach for systemic RV failure with wide QRS duration showing right bundle branch block and AF.

  13. Absence of left ventricular hypertrophy in elite college basketball players.

    Science.gov (United States)

    Wolfe, L A; Martin, R P; Seip, R L

    1985-09-01

    Left ventricular dimensions of 11 successful male college basketball players engaged in pre-season conditioning (mean age, 20.3 years) and 13 tall healthy male controls (mean age, 21.6 years) were studied by echocardiography. Left ventricular internal dimension (LVIDd, mm), posterior wall thickness (PWT, mm), septal thickness (ST, mm), and calculated left ventricular mass (LV mass, g) in the athletes were within or only slightly in excess of echocardiographic normal limits and mean values were not significantly different from the control group. LVIDd (mm/m2 body surface area) was significantly lower in the athletes. However, five guard-type players displayed significantly greater mean values for PWT and LV mass compared to six taller forwards/centers with linear body builds. It was concluded that left ventricular hypertrophy is not a common characteristic of college basketball players. It was hypothesized that cardiac dimensions of young men may vary independently of gross body size in relation to somatotype or other anthropometric variables.

  14. Genetic heterogeneity of left-ventricular noncompaction cardiomyopathy.

    Science.gov (United States)

    Moric-Janiszewska, Ewa; Markiewicz-Łoskot, Grazyna

    2008-05-01

    Isolated noncompaction of the ventricular myocardium (INVM) sometimes referred to as spongy myocardium is a rare, congenital and also acquired cardiomyopathy. It appears to divide the presentation into neonatal, childhood and adult forms of which spongy myocardium and systolic dysfunction is the commonality. The disorder is characterized by a left ventricular hypertrophy with deep trabeculations, and with diminished systolic function, with or without associated left ventricular dilation. In half or more of the cases, the right ventricle is also affected. The sporadic type, however, in some patients, may be due to chromosomal abnormalities and the occurrence of familial incidence. Isolated noncompaction of the left ventricular myocardium in the majority of adult patients is an autosomal dominant disorder. The familial and X-linked disorders have been described by various authors. We here describe the genetic background of this disorder: some of the most mutated genes that are responsible for the disease are (G4.5 (tafazzin gene): alpha-dystrobrevin gene (DTNA); FKBP-12 gene; lamin A/C gene; Cypher/ZASP (LIM, LDB3) gene); and some genotype-phenotype correlations (Becker muscular dystrophy, Emery-Dreifuss muscular dystrophy or Barth syndrome) based on the literature review.

  15. Left ventricular mechanics in repaired tetralogy of Fallot with and without pulmonary valve replacement: analysis by three-dimensional speckle tracking echocardiography.

    Directory of Open Access Journals (Sweden)

    Shu-Na Li

    Full Text Available BACKGROUND: Altered septal curvature and left ventricular (LV geometry secondary to right ventricular (RV dilation render two-dimensional assessment of LV mechanics difficult in repaired tetralogy of Fallot (TOF patients. The novel three-dimensional (3D speckle tracking echocardiography enables comprehensive evaluation of true 3D LV mechanics. METHODS AND RESULTS: Seventy-six patients aged 23.6 ± 8.3 years, 55 with isolated repair (group I and 21 with subsequent pulmonary valve replacement (group II, and 34 healthy controls were studied. Three-dimensional volume datasets were acquired for assessment of LV global and regional 3D strain, systolic dyssynchrony index (SDI, twist, twist gradient (twist/LV length, and ejection fraction. A global performance index was calculated as (global 3D strain•twist gradient/SDI. The septal curvature and LV eccentricity were determined from the mid-ventricular short-axis. Compared with controls, group I and II patients had significantly reduced LV global 3D strain, LV twist, twist gradient, septal curvature, and global performance index, and greater LV systolic and diastolic eccentricity and SDI (all p<0.05. All but the four apical LV segments in patients had reduced regional 3D strain compared with controls (all p<0.05. Septal curvature correlated with LV global 3D strain (r=0.41, p<0.001, average septal strain (r=0.38, p<0.001, twist (r=0.32, p<0.001, twist gradient (r=0.33, p<0.001, and global performance index (r=0.43, p<0.001. CONCLUSIONS: Adverse 3D LV mechanics as characterized by impaired global and regional 3D systolic strain, mechanical dyssynchrony, and reduced twist is related to reduced septal curvature in repaired TOF patients with and without pulmonary valve replacement.

  16. Surgical treatment of post-infarction left ventricular pseudoaneurysm: Case series highlighting various surgical strategies.

    Science.gov (United States)

    Prifti, Edvin; Bonacchi, Massimo; Baboci, Arben; Giunti, Gabriele; Veshti, Altin; Demiraj, Aurel; Zeka, Merita; Rruci, Edlira; Bejko, Ervin

    2017-04-01

    The left ventricular pseudoaneurysm (LVP) is rare, the surgical experience is limited and its surgical treatment remains still a challenge with an elevated mortality. Herein, it is presented a retrospective analysis of our experience with acquired post infarct LVP over a10-year period. Between January 2006 through August 2016, a total of 13 patients underwent operation for post infarct pseudoaneurysm of the left ventricle. There were 10 men and 3 women and the mean age was 61 ± 7.6 years. 4 patients presented acute LVP. Two patients had preoperative intraortic balloon pump implantation. Various surgical techniques were used to obliterate the pseudoaneurysm such as direct pledgeted sutures buttressed by polytetrafluoroethylene felt, a Gore-Tex or Dacron patch, transatrial closure of LVP neck in submitral pseudoaneurysm, or linear closure in cases presenting associated postinfarct ventricular septal defect. Concomitant coronary artery bypasses were performed for significant stenoses in 12 patients, ventricular septal defect closure in 4 patients, mitral valve replacement in 3 and aortic valve replacement in 1 patient. Operative mortality was 30.8% (4 patients). Three of them were acute LVP. Three patients required the continuous hemodyalisis and 8 patients required intra-aortic balloon pump. At follow-up two deaths occurred at 1 and 3 years after surgery. In conclusion, this study revealed that surgical repair of post infarct left ventricular pseudoaneurysm was associated with an acceptable surgical mortality rate, that cardiac rupture did not occur in surgically treated patients.

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

    Directory of Open Access Journals (Sweden)

    Køber Lars V

    2009-11-01

    Full Text Available Abstract Introduction Tricuspid Annular Plane Systolic Excursion (TAPSE has independent prognostic value in heart failure patients but may be influenced by left ventricular (LV ejection fraction. The present study assessed the association of TAPSE and clinical factors, global and regional LV function in 634 patients admitted for symptomatic heart failure. Methods & Results TAPSE were correlated with global and regional measures of longitudinal LV function, segmental wall motion scores and measures of diastolic LV function as measured from transthoracic echocardiography. LV ejection fraction, wall motion index scores, atrio-ventricular annular plane systolic excursion of the mitral annulus were significantly related to TAPSE. Septal and posterior mitral annular plane systolic excursion (β = 0.56, p 2 = 0.28, p interaction = NS. Conclusion TAPSE is reduced with left ventricular dysfunction 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.

  18. Quantitative Evaluation of Left Ventricular Wall Motion in Patient with Coronary Artery Bypass Grafting Using Magnetic Resonance Tagging Technique

    Science.gov (United States)

    Inaba, Tadashi; Nakano, Takahiro; Tsutsumi, Masakazu; Kawasaki, Shingo; Kinosada, Yasutomi; Tokuda, Masataka

    Left ventricular wall motions during systole were investigated from a mechanical perspective by using a magnetic resonance tagging technique. Subjects were 7 patients with coronary artery bypass grafting (CABG). First, by analyzing strain in the left ventricular wall, cardiac contractility was evaluated in the patients with CABG. Next, by calculating displacement in the myocardial wall, paradoxical movements following CABG were quantitatively evaluated. Strain analysis showed local decreases in circumferential strain in 4 of 7 subjects. The results of displacement analysis clarified that following CABG, the degree of radial displacement was small in the septal wall and large in the lateral wall, and circumferential displacement towards the septal wall occurred in the anterior and posterior walls. Since this behavior was seen in both reduced and normal cardiac contractility groups, paradoxical movements in the present patients were not caused by reduced cardiac contractility, but rather by rigid-body motion of the entire heart.

  19. Anaesthesia management of a patient with hypertrophic obstructive cardiomyopathy undergoing Morrow′s septal myectomy

    Directory of Open Access Journals (Sweden)

    Naresh Kumar Agarwal

    2007-01-01

    Full Text Available Hypertrophic obstructive cardiomyopathy (HOCM is a rare disorder. There is paucity of literature on anaesthetic management of this disorder. Aim of this case report is to highlight the anaesthetic problems encountered during management of such patients. A thirty-five year old male was admitted with atypical chest pain for last one year. X-ray chest revealed cardiomegaly (CT ratio 0.6. Electrocardiographic findings were left axis deviation with left ventricular hypertrophy. On echocardiography, there was moderate mitral regurgitation (MR, systolic anterior motion (SAM of anterior mitral leaflet and prominent systolic narrowing of left ventricle cavity. Transoesophageal echocardiography (TOE also showed an anomalous muscle bundle stretching into LV causing obstruction. Preload was kept high. Systemic vascular resistance (SVR was maintained, avoiding use of vasodilators and inotropes. Morrow′s septal myectomy was done. Anomalous muscle bundle was excised. On postoperative TOE, there was no MR and no obstruction. Optimal anaesthetic management in such patients involves maintaining adequate preload, systemic vascular resistance and minimal outflow obstruction. Other considerations are to maintain haemodynamic stability, sinus rhythm and afterload. Transoesophageal echocardiography is an extremely useful monitoring device in such patients.

  20. Transcatheter Closure of Atrial Septal Defects Improves Cardiac Remodeling and Function of Adult Patients with Permanent Atrial Fibrillation

    Institute of Scientific and Technical Information of China (English)

    Liang Chen; Yuan Bai; Fei-Yu Wang; Zhi-Gang Zhang; Xing-Hua Shan; Tao Chen; Xian-Xian Zhao

    2015-01-01

    Background:Permanent atrial fibrillation (AF) is the most common form of dysrhythmia associated with atrial septal defects (ASDs) in patients older than 40 years.However,little is known about cardiac remodeling after transcatheter closure in patients with permanent AF.This study was designed to compare cardiac events and remodeling effects after transcatheter closure in such patients.Methods:Clinical data of 289 adult patients older than 40 years who underwent ASD closure at our center were analyzed retrospectively.Of them,63 patients with permanent AF were assigned to the case group,and the other 226 patients without permanent AF were assigned to the control group.Cardiac events and changes in left and right cardiac cavity dimensions before the procedure and 6 months after the procedure were compared between the two groups.Results:Patients in the case group were significantly older than those in the control group.The right ventricular (RV) volume and right atrial (RA) volume were decreased significantly in both the groups during a median follow-up period of 6 months after closure (P < 0.001).The left atrial dimensions,left ventricular end-systolic dimensions,left ventricular end-diastolic dimensions and left ventricular ejection fraction showed no significant change before and after the procedure in both the groups.Changes of the RV volume and RA volume in the case group were significantly smaller than those in the control group (P =0.005 and P < 0.001).The New York Heart Association cardiac function was improved in both the groups during the 6 months follow-up period.Conclusions:The transcatheter closure of ASD can improve the cardiac remodeling and cardiac function in patients with or without AF.

  1. Transcatheter Closure of Atrial Septal Defects Improves Cardiac Remodeling and Function of Adult Patients with Permanent Atrial Fibrillation

    Directory of Open Access Journals (Sweden)

    Liang Chen

    2015-01-01

    Full Text Available Background: Permanent atrial fibrillation (AF is the most common form of dysrhythmia associated with atrial septal defects (ASDs in patients older than 40 years. However, little is known about cardiac remodeling after transcatheter closure in patients with permanent AF. This study was designed to compare cardiac events and remodeling effects after transcatheter closure in such patients. Methods: Clinical data of 289 adult patients older than 40 years who underwent ASD closure at our center were analyzed retrospectively. Of them, 63 patients with permanent AF were assigned to the case group, and the other 226 patients without permanent AF were assigned to the control group. Cardiac events and changes in left and right cardiac cavity dimensions before the procedure and 6 months after the procedure were compared between the two groups. Results: Patients in the case group were significantly older than those in the control group. The right ventricular (RV volume and right atrial (RA volume were decreased significantly in both the groups during a median follow-up period of 6 months after closure (P < 0.001. The left atrial dimensions, left ventricular end-systolic dimensions, left ventricular end-diastolic dimensions and left ventricular ejection fraction showed no significant change before and after the procedure in both the groups. Changes of the RV volume and RA volume in the case group were significantly smaller than those in the control group (P = 0.005 and P < 0.001. The New York Heart Association cardiac function was improved in both the groups during the 6 months follow-up period. Conclusions: The transcatheter closure of ASD can improve the cardiac remodeling and cardiac function in patients with or without AF.

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

    Energy Technology Data Exchange (ETDEWEB)

    Erol, Cengiz, E-mail: drcengizerol@yahoo.com [Selcuk University, Selcuklu Faculty of Medicine, Department of Radiology, Alaeddin Keykubat Central Campus, Selcuklu, 42250 Konya (Turkey); Koplay, Mustafa, E-mail: koplaymustafa@hotmail.com [Selcuk University, Selcuklu Faculty of Medicine, Department of Radiology, Alaeddin Keykubat Central Campus, Selcuklu, 42250 Konya (Turkey); Olcay, Ayhan, E-mail: drayhanolcay@gmail.com [29 May Hospital, Department of Cardiology, Vatan Street, Fatih, 34150 Istanbul (Turkey); Kivrak, Ali Sami, E-mail: alisamikivrak@hotmail.com [Selcuk University, Selcuklu Faculty of Medicine, Department of Radiology, Alaeddin Keykubat Central Campus, Selcuklu, 42250 Konya (Turkey); Ozbek, Seda, E-mail: dsadr@hotmail.com [Selcuk University, Selcuklu Faculty of Medicine, Department of Radiology, Alaeddin Keykubat Central Campus, Selcuklu, 42250 Konya (Turkey); Seker, Mehmet, E-mail: hikmet.irfan@hotmail.com [29 May Hospital, Department of Radiology, Vatan Street, Fatih, 34150 Istanbul (Turkey); Paksoy, Yahya, E-mail: yahyapaksoy@yahoo.com [Selcuk University, Selcuklu Faculty of Medicine, Department of Radiology, Alaeddin Keykubat Central Campus, Selcuklu, 42250 Konya (Turkey)

    2012-11-15

    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.

  3. Modifying muscular dystrophy through TGFβ

    OpenAIRE

    Ceco, Ermelinda; McNally, Elizabeth M.

    2013-01-01

    Muscular dystrophy arises from ongoing muscle degeneration and insufficient regeneration. This imbalance leads to loss of muscle with replacement by scar or fibrosis resulting in muscle weakness and, eventually, loss of muscle function. Human muscular dystrophy is characterized by a wide range of disease severity, even when the same genetic mutation is present. This variability implies that other factors, both genetic and environmental, modify the disease outcome. There has been an ongoing ef...

  4. Dobutamine stress echocardiography for assessing the role of dynamic intraventricular obstruction in left ventricular ballooning syndrome

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    Repetto Alessandra

    2010-04-01

    Full Text Available Abstract Background Dynamic intraventricular obstruction has been observed in patients with left ventricular ballooning syndrome (LVBS and has been hypothesized as a possible mechanism of the syndrome. The aim of this study was to assess the prevalence and significance of dynamic intraventricular obstruction in patients with LVBS. Methods and Results Dobutamine stress echocardiography was carried out in 22 patients with LVBS (82% apical, all women, aged 68 ± 9 years. At baseline 1 patient had a > 30 mmHg LV gradient; during stress a LV gradient > 30 mm Hg developed in 6/21 patients (28% and was caused by systolic anterior motion of the mitral valve in the 3 patients with severe gradient (mean 116 ± 29 mmHg, who developed mitral regurgitation and impaired apical wall motion and by obstruction at mid-ventricular level in the other 3 with a moderate gradient (mean 46 ± 16 mmHg. Compared with patients without obstruction those with obstruction had a greater mean septal thickness (11.6 ± .6 vs 9.8. ± 3, p Conclusion Spontaneous or dobutamine-induced dynamic LV obstruction is documented in 32% of patients with LVBS, is correlated with the presence of septal hypertrophy and may play a role in the development of LVBS in this subset of patients. In those without septal hypertrophy a dynamic obstruction is rarely induced with dobutamine and is unlikely to be a major pathogenetic factor of the syndrome.

  5. Growth Trajectory in Children with Trisomy 21 with and without Atrioventricular Septal Defect.

    Science.gov (United States)

    Mackman, Chad A; Loomba, Rohit S; Slicker, Julie; Bartz, Peter J

    2016-07-01

    Trisomy 21 is associated with poor weight gain and atrioventricular septal defects. The impact of atrioventricular septal defects on weight gain in the setting of Trisomy 21 has not previously been described in the recent era. This study aimed to determine if such an association is present. Patients with Trisomy 21 with and without atrioventricular septal defects were identified. Clinical, surgical, and postoperative data were collected were for these patients and then compared between patients. Specifically, weight for age z-scores were compared at various time points in a univariate and multivariate fashion. Effect of timing of surgery in those with an atrioventricular septal defect was also studied. A total of 86 patients were identified, 42 with an atrioventricular septal defect. There was a difference in weight for age z-scores between patients with and without atrioventricular septal defects only at 2 months (P value .038) and 6 months (P value .003) of age. This persisted after multivariate regression which demonstrated atrioventricular septal defects as an independent risk factor. There was no difference noted in weight at 2 years of age in patients undergoing atrioventricular septal defect repair before and 150 days of life. There was a statistically significant, but not clinically relevant, difference in weight between the patients with Trisomy 21 with and without atrioventricular septal defects in our cohort. Those with atrioventricular septal defects required more nutritional intervention, such as gastrostomy tube placement. Timing of repair did not alter outcomes at midterm follow-up. © 2016 Wiley Periodicals, Inc.

  6. Reduced sodium current in the lateral ventricular wall induces inferolateral J-waves

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    Veronique Marlinde Frederica Meijborg

    2016-08-01

    Full Text Available Background: 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. Methods: 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.Results: 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.Conclusion: Reduced sodium current in the left lateral ventricular myocardium can cause inferolateral J-waves on the ECG.

  7. Ventricular Assist Device Support

    Science.gov (United States)

    Fasseas, Panayotis; Kutalek, Steven P.; Samuels, Fania L.; Holmes, Elena C.; Samuels, Louis E.

    2002-01-01

    We describe herein the cases of 2 patients who had ventricular arrhythmias. In one, a short-term biventricular assist device, the ABIOMED BVS 5000, was placed because the patient had sustained ventricular tachycardia and could not be weaned from cardiopulmonary bypass. Excellent hemodynamic support was maintained for several days while the antiarrhythmic therapy was maximized. Sinus rhythm was restored, and the patient was successfully weaned from the ventricular assist device. However, the substrate for the arrhythmia persisted, and a recurrence, 1 week later, resulted in the patient's death. In the 2nd patient, the use of an implantable left ventricular assist device was successful in temporarily alleviating the ventricular tachycardia associated with ischemic cardiomyopathy. However, after 2 days of device assistance, the patient experienced a recurrence of the tachycardia, which degenerated into ventricular fibrillation with a marked deterioration in the patient's hemodynamics. The arrhythmia persisted despite multiple attempts at external cardioversion, and internal cardioversion and placement of an automatic implantable cardioverter-defibrillator were necessary. This treatment, along with repeated boluses of amiodarone, led to successful suppression of the arrhythmias, and the patient eventually underwent transplantation. The mechanical hemodynamic support of the circulation by ventricular assist devices was effective in supporting these 2 patients who had sustained ventricular arrhythmias. (Tex Heart Inst J 2002;29:33–6) PMID:11995847

  8. Ventricular tachycardia in acromegaly.

    Science.gov (United States)

    Arias, Miguel A; Pachón, Marta; Rodríguez-Padial, Luis

    2011-02-01

    Cases of sudden cardiac death have been reported in patients with acromegaly. Malignant ventricular arrhythmias may play an important role in this fatal complication, but the exact mechanisms are not well understood. We report on an acromegalic patient presenting with documented recurrent syncopal ventricular tachycardia.

  9. Computational modeling of muscular thin films for cardiac repair

    Science.gov (United States)

    Böl, Markus; Reese, Stefanie; Parker, Kevin Kit; Kuhl, Ellen

    2009-03-01

    Motivated by recent success in growing biohybrid material from engineered tissues on synthetic polymer films, we derive a computational simulation tool for muscular thin films in cardiac repair. In this model, the polydimethylsiloxane base layer is simulated in terms of microscopically motivated tetrahedral elements. Their behavior is characterized through a volumetric contribution and a chain contribution that explicitly accounts for the polymeric microstructure of networks of long chain molecules. Neonatal rat ventricular cardiomyocytes cultured on these polymeric films are modeled with actively contracting truss elements located on top of the sheet. The force stretch response of these trusses is motivated by the cardiomyocyte force generated during active contraction as suggested by the filament sliding theory. In contrast to existing phenomenological models, all material parameters of this novel model have a clear biophyisical interpretation. The predictive features of the model will be demonstrated through the simulation of muscular thin films. First, the set of parameters will be fitted for one particular experiment documented in the literature. This parameter set is then used to validate the model for various different experiments. Last, we give an outlook of how the proposed simulation tool could be used to virtually predict the response of multi-layered muscular thin films. These three-dimensional constructs show a tremendous regenerative potential in repair of damaged cardiac tissue. The ability to understand, tune and optimize their structural response is thus of great interest in cardiovascular tissue engineering.

  10. 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.

  11. Septo-Hippocampo-Septal Loop and Memory Formation

    Directory of Open Access Journals (Sweden)

    Fatemeh Khakpai

    2012-12-01

    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.

  12. [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.

  13. Single-beat noninvasive imaging of ventricular endocardial and epicardial activation in patients undergoing CRT.

    Directory of Open Access Journals (Sweden)

    Thomas Berger

    Full Text Available BACKGROUND: Little is known about the effect of cardiac resynchronization therapy (CRT on endo- and epicardial ventricular activation. Noninvasive imaging of cardiac electrophysiology (NICE is a novel imaging tool for visualization of both epi- and endocardial ventricular electrical activation. METHODOLOGY/PRINCIPAL FINDINGS: NICE was performed in ten patients with congestive heart failure (CHF undergoing CRT and in ten patients without structural heart disease (control group. NICE is a fusion of data from high-resolution ECG mapping with a model of the patient's individual cardiothoracic anatomy created from magnetic resonance imaging. Beat-to-beat endocardial and epicardial ventricular activation sequences were computed during native rhythm as well as during ventricular pacing using a bidomain theory-based heart model to solve the related inverse problem. During right ventricular (RV pacing control patients showed a deterioration of the ventricular activation sequence similar to the intrinsic activation pattern of CHF patients. Left ventricular propagation velocities were significantly decreased in CHF patients as compared to the control group (1.6±0.4 versus 2.1±0.5 m/sec; p<0.05. CHF patients showed right-to-left septal activation with the latest activation epicardially in the lateral wall of the left ventricle. Biventricular pacing resulted in a resynchronization of the ventricular activation sequence and in a marked decrease of total LV activation duration as compared to intrinsic conduction and RV pacing (129±16 versus 157±28 and 173±25 ms; both p<0.05. CONCLUSIONS/SIGNIFICANCE: Endocardial and epicardial ventricular activation can be visualized noninvasively by NICE. Identification of individual ventricular activation properties may help identify responders to CRT and to further improve response to CRT by facilitating a patient-specific lead placement and device programming.

  14. Single-Beat Noninvasive Imaging of Ventricular Endocardial and Epicardial Activation in Patients Undergoing CRT

    Science.gov (United States)

    Berger, Thomas; Pfeifer, Bernhard; Hanser, Friedrich F.; Hintringer, Florian; Fischer, Gerald; Netzer, Michael; Trieb, Thomas; Stuehlinger, Markus; Dichtl, Wolfgang; Baumgartner, Christian; Pachinger, Otmar; Seger, Michael

    2011-01-01

    Background Little is known about the effect of cardiac resynchronization therapy (CRT) on endo- and epicardial ventricular activation. Noninvasive imaging of cardiac electrophysiology (NICE) is a novel imaging tool for visualization of both epi- and endocardial ventricular electrical activation. Methodology/Principal Findings NICE was performed in ten patients with congestive heart failure (CHF) undergoing CRT and in ten patients without structural heart disease (control group). NICE is a fusion of data from high-resolution ECG mapping with a model of the patient's individual cardiothoracic anatomy created from magnetic resonance imaging. Beat-to-beat endocardial and epicardial ventricular activation sequences were computed during native rhythm as well as during ventricular pacing using a bidomain theory-based heart model to solve the related inverse problem. During right ventricular (RV) pacing control patients showed a deterioration of the ventricular activation sequence similar to the intrinsic activation pattern of CHF patients. Left ventricular propagation velocities were significantly decreased in CHF patients as compared to the control group (1.6±0.4 versus 2.1±0.5 m/sec; p<0.05). CHF patients showed right-to-left septal activation with the latest activation epicardially in the lateral wall of the left ventricle. Biventricular pacing resulted in a resynchronization of the ventricular activation sequence and in a marked decrease of total LV activation duration as compared to intrinsic conduction and RV pacing (129±16 versus 157±28 and 173±25 ms; both p<0.05). Conclusions/Significance Endocardial and epicardial ventricular activation can be visualized noninvasively by NICE. Identification of individual ventricular activation properties may help identify responders to CRT and to further improve response to CRT by facilitating a patient-specific lead placement and device programming. PMID:21298045

  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. 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.

  17. Comparação de parâmetros eletrofisiológicos das estimulações cardíacas endocárdicas septal e apical Comparison of electrophysiological parameters of septal and apical endocardial cardiac stimulation

    Directory of Open Access Journals (Sweden)

    Juan Carlos Pachón Mateos

    2012-06-01

    Full Text Available FUNDAMENTO: A estimulação endocárdica convencional do ventrículo direito (VD ocasiona alargamento do QRS e dessincronização do miocárdio, comprometendo a função ventricular. Com a necessidade de estimulação menos deletéria, a estimulação septal do VD tem sido mais utilizada. Eventualmente têm sido relatados limiares mais altos e ondas R menores na estimulação septal. OBJETIVO: Comparar os parâmetros das estimulações apical e septal, intrapaciente, para verificar se existem diferenças que possam interferir na escolha do ponto de estimulação. MÉTODOS: Estudo prospectivo controlado. Foram incluídos 25 pacientes, com 67,2 ± 9 anos, 10 (40% mulheres, com indicações de marca-passo por bradiarritmias. Etiologias foram degenerativa em nove (36%, coronariopatia em oito (32%, doença de Chagas em sete (28%, e valvopatia em um (4% pacientes. Foram utilizados eletrodos de fixação ativa e avaliados os limiares de comando, impedância e onda R uni e bipolares no implante e após seis meses. RESULTADOS: A média aguda dos limiares de comando, ondas R e impedâncias unipolares/bipolares septais x apicais foram, respectivamente, 0,73x0,74V e 0,73x0,78V; 10x9,9 mV e 12,3x12,4 mV; 579x621 Ω e 611x629 Ω. Comparações entre parâmetros septais e apicais com teste t-pareado bicaudal demonstraram um P > 0,1. Após seis meses, a média dos limiares de comando, ondas R e Impedâncias unipolares/ bipolares septais x apicais foram, respectivamente, 0,5 x 0,72 V e 0,71 x 0,87 V; 11,4x9,5 mV e 12x11,2 mV; 423x426Ω e 578x550Ω, com P > 0,05, exceto comparando-se limiar de estimulação unipolar septal com apical unipolar p de 0,02. CONCLUSÃO: Utilizando comparações intrapaciente, não existem diferenças expressivas entre parâmetros eletrofisiológicos de estimulação septal e apical sendo que não há restrições para a escolha da estimulação septal em ventrículo direito.BACKGROUND: The conventional right ventricle (RV endocardial

  18. Bed Rest Muscular Atrophy

    Science.gov (United States)

    Greenleaf, John E.

    2000-01-01

    A major debilitating response from prolonged bed rest (BR) is muscle atrophy, defined as a "decrease in size of a part of tissue after full development has been attained: a wasting away of tissue as from disuse, old age, injury or disease". Part of the complicated mechanism for the dizziness, increased body instability, and exaggerated gait in patients who arise immediately after BR may be a result of not only foot pain, but also of muscular atrophy and associated reduction in lower limb strength. Also, there seems to be a close association between muscle atrophy and bone atrophy. A discussion of many facets of the total BR homeostatic syndrome has been published. The old adage that use determines form which promotes function of bone (Wolff's law) also applies to those people exposed to prolonged BR (without exercise training) in whom muscle atrophy is a consistent finding. An extreme case involved a 16-year-old boy who was ordered to bed by his mother in 1932: after 50 years in bed he had "a lily-white frame with limbs as thin as the legs of a ladder-back chair". These findings emphasize the close relationship between muscle atrophy and bone atrophy. In addition to loss of muscle mass during deconditioning, there is a significant loss of muscle strength and a decrease in protein synthesis. Because the decreases in force (strength) are proportionately greater than those in fiber size or muscle cross-sectional area, other contributory factors must be involved; muscle fiber dehydration may be important.

  19. Bed Rest Muscular Atrophy

    Science.gov (United States)

    Greenleaf, John E.

    2000-01-01

    A major debilitating response from prolonged bed rest (BR) is muscle atrophy, defined as a "decrease in size of a part of tissue after full development has been attained: a wasting away of tissue as from disuse, old age, injury or disease". Part of the complicated mechanism for the dizziness, increased body instability, and exaggerated gait in patients who arise immediately after BR may be a result of not only foot pain, but also of muscular atrophy and associated reduction in lower limb strength. Also, there seems to be a close association between muscle atrophy and bone atrophy. A discussion of many facets of the total BR homeostatic syndrome has been published. The old adage that use determines form which promotes function of bone (Wolff's law) also applies to those people exposed to prolonged BR (without exercise training) in whom muscle atrophy is a consistent finding. An extreme case involved a 16-year-old boy who was ordered to bed by his mother in 1932: after 50 years in bed he had "a lily-white frame with limbs as thin as the legs of a ladder-back chair". These findings emphasize the close relationship between muscle atrophy and bone atrophy. In addition to loss of muscle mass during deconditioning, there is a significant loss of muscle strength and a decrease in protein synthesis. Because the decreases in force (strength) are proportionately greater than those in fiber size or muscle cross-sectional area, other contributory factors must be involved; muscle fiber dehydration may be important.

  20. An unusual encounter of a "cobra" in the heart: rare appearance of an Amplatzer Septal Occluder.

    Science.gov (United States)

    Yip, W C; Chan, K Y

    2001-04-01

    This article presents the unusual appearance of a "cobra" due to "acute bending" of the proximal part and partial opening of the distal part of the left atrial disk of an Amplatzer Septal Occluder during the process of transcatheter closure of a secundum atrial septal defect in a 6-year-old boy. The possible reasons and method to overcome this technical problem, which resulted in successful occlusion of the atrial septal defect, are discussed.

  1. The first clinical experience with the new GORE® septal occluder (GSO)

    DEFF Research Database (Denmark)

    Søndergaard, Lars; Loh, Poay Huan; Franzen, Olaf

    2013-01-01

    Aims: A new GORE® septal occluder (GSO) was granted CE mark in Europe in June 2011 for the treatment of patent foramen ovale and atrial septal defect. Major changes have been made to the device and delivery system compared to the HELEX® device. The new delivery system has simplified the implantat......Aims: A new GORE® septal occluder (GSO) was granted CE mark in Europe in June 2011 for the treatment of patent foramen ovale and atrial septal defect. Major changes have been made to the device and delivery system compared to the HELEX® device. The new delivery system has simplified...

  2. Ventricular function following coronary artery bypass grafting: comparison between Gated SPECT and cardiac magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Mesquita, Claudio Tinoco [Hospital Pro-Cardiaco, Rio de Janeiro, RJ (Brazil). Servico de Medicina Nuclear; Instituto Nacional de Cardiologia, Rio de Janeiro, RJ (Brazil); Pessoa, Maria Carolina Pinheiro [Pro-Echo Hospital Samaritano, Rio de Janeiro, RJ (Brazil); Vasconcelos, Paulo Pontes [Centro de Diagnostico por Imagens (CDPI), Rio de Janeiro, RJ (Brazil); Oliveira Junior, Amarino Carvalho [Hospital Pro-Cardiaco, Rio de Janeiro, RJ (Brazil). Servico de Radiologia; Dohmann, Hans Fernando Rocha [Hospital Pro-Cardiaco, Rio de Janeiro, RJ (Brazil). Servico de Radiologia; Instituto Nacional de Cardiologia, Rio de Janeiro, RJ (Brazil); Reis, Adair Gomes dos [Nuclear Diagnosticos, SP (Brazil); Fonseca, Lea Mirian Barbosa da [Pro-Echo Hospital Samaritano, Rio de Janeiro, RJ (Brazil); Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil)

    2009-04-15

    Background: The assessment of left ventricular function may be impaired by the abnormal interventricular septal motion frequently found after coronary artery bypass grafting (CABG). Studies on the validation of gated SPECT as a tool for the assessment of left ventricular function in this patient group are scarce. Objective: We investigated the agreement and correlation between left ventricular ejection fraction (LVEF), end-diastolic volume (EDV), and end-systolic volume (ESV) as obtained using electrocardiogram-gated myocardial perfusion scintigraphy (gated SPECT) and cardiac magnetic resonance imaging in 20 patients undergoing coronary artery bypass grafting. Methods: Correlation was measured using Spearman's correlation coefficient ({rho}). Agreement was assessed using Bland-Altman analysis. Results: A good correlation was found between gated SPECT and cardiac magnetic resonance imaging in patients after CABG with regard to left ventricular ejection fraction ({rho} = 0.85; p =0.0001), moderate correlation for end-diastolic volume ({rho} = 0.51; p = 0.02), and non-significant correlation for end-diastolic volume ({rho} = 0.13; p = 0.5). Agreement ranges for LVEF, ESV and EDV were: -20% to 12%; -38 to 54 ml and; -96 to 100 ml, respectively. Conclusion: A reliable correlation was found for left ventricular ejection fraction as obtained by gated SPECT and magnetic resonance imaging in patients undergoing CABG. For ventricular volumes, however, the correlation is not adequate. (author)

  3. Interobserver and Intraobserver Agreement on Qualitative Assessments of Right Ventricular Dysfunction With Echocardiography in Patients With Pulmonary Embolism.

    Science.gov (United States)

    Weekes, Anthony J; Oh, Laura; Thacker, Gregory; Johnson, Angela K; Runyon, Michael; Rose, Geoffrey; Johnson, Thomas; Templin, Megan; Norton, H James

    2016-10-01

    To evaluate observer agreement using qualitative goal-directed echocardiographic criteria for right ventricular (RV) dysfunction prognostication in submassive pulmonary embolism (PE). Two emergency physicians and 2 cardiologists independently reviewed 31 packets of goal-directed echocardiographic video clips consisting of at least 3 windows obtained by emergency physicians from normotensive patients with PE. Nine packets were repeated to assess for intraobserver agreement. Right ventricular dysfunction criteria on goal-directed echocardiography were as follows: RV enlargement was present, with a right-to-left ventricular basal diameter ratio of 1.0 or higher and blunting of the apex of the RV in 2 or more different windows; RV systolic dysfunction was present if the tricuspid annulus moved toward the apex 10 mm or less and there was RV free wall hypokinesis; and septal deviation was present with any flattening or deviation of the ventricular septum toward the left ventricle. Among the 4 participants, there was 83.9% agreement on the presence or absence of RV enlargement (κ = 0.84), 74.2% agreement on the presence or absence of RV systolic dysfunction (κ = 0.69), and 71.0% agreement on the presence or absence of septal deviation (κ = 0.59). Intraobserver agreement was 100% for each RV dysfunction variable for each observer (κ = 1.0). Agreement was substantial for both severe RV enlargement and RV systolic dysfunction and moderate for septal deviation. Right ventricular dysfunction assessment with qualitative goal-directed echocardiographic criteria is reproducible for PE risk stratification.

  4. Assessment of Left Ventricular Radial Deformation by Speckle Tracking Imaging

    Institute of Scientific and Technical Information of China (English)

    Min PAN; Hao LUO; Ashraf Muhammad; Schultheis Judy; Xiaokui LI; David J. Sahn

    2009-01-01

    The left ventricular radial strain in the inner and outer layers was evaluated by using two-dimensional speckle tracking imaging (2DS). Twenty-five piglets were studied. The short axis views were acquired. Peak systolic radial strain was measured from 6 circumferential points related to 6 standard segments in the inner and outer layers respectively using 2DS methods. The peak positive first derivative (dp/dt) of left ventricular pressure was compared to the radial strain from 2DS. The inner band showed higher peak radial strain values as compared to the outer band at all of the segments (P<0.0001), but the differences had significance just in anteroseptal, posterior, inferior and septal seg-ments (P<0.05). Good correlation could be found between radial strain of inner and outer layers and peak dp/dt (P<0.001). These preliminary results showed that the degree of local deformation or wall thickening of the ventricular wall in its inner layer was more obvious than its outer layer. It is suggested that the 2DS technique is useful and sensitive for better understanding the regional and global myocar-dial motion and its relationship to the complex architecture of myocardium.

  5. Right ventricular dysfunction affects survival after surgical left ventricular restoration.

    Science.gov (United States)

    Couperus, Lotte E; Delgado, Victoria; Palmen, Meindert; van Vessem, Marieke E; Braun, Jerry; Fiocco, Marta; Tops, Laurens F; Verwey, Harriëtte F; Klautz, Robert J M; Schalij, Martin J; Beeres, Saskia L M A

    2017-04-01

    Several clinical and left ventricular parameters have been associated with prognosis after surgical left ventricular restoration in patients with ischemic heart failure. The aim of this study was to determine the prognostic value of right ventricular function. A total of 139 patients with ischemic heart failure (62 ± 10 years; 79% were male; left ventricular ejection fraction 27% ± 7%) underwent surgical left ventricular restoration. Biventricular function was assessed with echocardiography before surgery. The independent association between all-cause mortality and right ventricular fractional area change, tricuspid annular plane systolic excursion, and right ventricular longitudinal peak systolic strain was assessed. The additive effect of multiple impaired right ventricular parameters on mortality also was assessed. Baseline right ventricular fractional area change was 42% ± 9%, tricuspid annular plane systolic excursion was 18 ± 3 mm, and right ventricular longitudinal peak systolic strain was -24% ± 7%. Within 30 days after surgery, 15 patients died. Right ventricular fractional area change (hazard ratio, 0.93; 95% confidence interval, 0.88-0.98; P right ventricular longitudinal peak systolic strain (hazard ratio, 1.15; 95% confidence interval, 1.05-1.26; P Right ventricular function was impaired in 21%, 20%, and 27% of patients on the basis of right ventricular fractional area change, tricuspid annular plane systolic excursion, and right ventricular longitudinal peak systolic strain, respectively. Any echocardiographic parameter of right ventricular dysfunction was present in 39% of patients. The coexistence of several impaired right ventricular parameters per patient was independently associated with increased 30-day mortality (hazard ratio, 2.83; 95% confidence interval, 1.64-4.87, P right ventricular systolic dysfunction is independently associated with increased mortality in patients with ischemic heart failure undergoing surgical left

  6. Left Ventricular Hypertrophy

    Science.gov (United States)

    ... the chamber itself also increases. The enlarged heart muscle loses elasticity and eventually may fail to pump with as much force as needed. Left ventricular hypertrophy is more common in people who have uncontrolled ...

  7. Imagen corporal y dismorfia muscular

    OpenAIRE

    Roldan Pintor, Marta

    2015-01-01

    La presente investigación pretende demostrar la correlación existente entre tener una percepción negativa y distorsionada del propio cuerpo y la posibilidad de desarrollar una dismorfia muscular. Asociado a los cambios culturales, al cambio del ideal de belleza y reforzado por el papel de la publicidad y su influencia en los consumidores, la dismorfia muscular es un problema cada vez más frecuente. Se pretende explicar el recorrido a lo largo de la historia de estos c...

  8. Muscular atrophy in diabetic neuropathy

    DEFF Research Database (Denmark)

    Andersen, H; Gadeberg, P C; Brock, B

    1997-01-01

    Diabetic patients with polyneuropathy develop motor dysfunction. To establish whether motor dysfunction is associated with muscular atrophy the ankle dorsal and plantar flexors of the non-dominant leg were evaluated with magnetic resonance imaging in 8 patients with symptomatic neuropathy, in 8 non...... confirmed that the atrophy predominated distally. We conclude that muscular atrophy underlies motor weakness at the ankle in diabetic patients with polyneuropathy and that the atrophy is most pronounced in distal muscles of the lower leg indicating that a length dependent neuropathic process explains...

  9. [Right ventricular involvement in hypertrophic cardiomyopathy. A case report and brief review of the literature].

    Science.gov (United States)

    Comella, Alessandro; Magnacca, Massimo; Gistri, Roberto; Lombardi, Massimo; Neglia, Danilo; Poddighe, Rosa; Pesola, Antonio

    2004-02-01

    A clinical case of non-obstructive hypertrophic cardiomyopathy with involvement of the right ventricle is reported. The patient was a 42-year-old male with symptoms suggesting an effort angina of recent onset. The diagnosis was established by echocardiography, which showed asymmetric hypertrophy of the interventricular septum (20 mm), hypertrophy of the right ventricular free wall, and severe hypertrophy of the septal papillary muscle of the tricuspid valve. The patient underwent a complete diagnostic evaluation, including exercise stress test, Holter monitoring, magnetic resonance, myocardial tomoscintigraphy and complete hemodynamic assessment. Medical treatment with atenolol 50 mg day was started; at 1-year follow-up the patient's clinical conditions are good, with decrease of anginal episodes. The literature review elicits the paucity of information about this condition, despite a frequent involvement of both ventricles in hypertrophic obstructive cardiomyopathy. The case reported shows two atypical aspects: a) the involvement of the right ventricle in non-obstructive hypertrophic cardiomyopathy is anecdotal; b) this pattern of hypertrophy (right ventricular free wall/septal papillary muscle) has never been previously reported. Right ventricular involvement in patients with hypertrophic cardiomyopathy must be carefully investigated, because it may be more frequent than conventionally deemed.

  10. Serum cystatin C concentration as an independent marker for hypertensive left ventricular hypertrophy

    Institute of Scientific and Technical Information of China (English)

    Xin Li; Hang Zhu; Peng Li; Qian Xin; Jie Liu; Wei Zhang; You-Hong Xing; Hao Xue

    2013-01-01

    Background Serum cystatin C levels can be used to predict morbidity and mortality in patients with cardiovascular disease. However, the clinical relevance of serum cystatin C levels in patients with hypertensive left ventricular hypertrophy (LVH) has rarely been investigated. We designed the present study to investigate whether serum cystatin C levels are associated with cardiac structural and functional alterations in hypertensive patients. Methods We enrolled 823 hypertensive patients and classified them into two groups:those with LVH (n=287) and those without LVH (n=536). All patients underwent echocardiography and serum cystatin C testing. We analyzed the relationship be-tween serum cystatin C levels and LVH. Results Serum cystatin C levels were higher in hypertensive patients with LVH than in those without LVH (P<0.05). Using linear correlation analysis, we found a positive correlation between serum cystatin C levels and interven-tricular septal thickness (r=0.247, P<0.01), posterior wall thickness (r=0.216, P<0.01), and left ventricular weight index (r=0.347, P<0.01). When analyzed by multiple linear regression, the positive correlations remained between serum cystatin C and interventricular septal thickness (β=0.167, P<0.05), posterior wall thickness (β=0.187, P<0.05), and left ventricular weight index (β=0.245, P<0.01). Con-clusion Serum cystatin C concentration is an independent marker for hypertensive LVH.

  11. FIBROSIS MUSCULAR ESQUELETICA: PAPEL DE LOS FIBROBLASTOS EN LA DISTROFIA MUSCULAR

    OpenAIRE

    MEZZANO ROBINSON, VALERIA

    2007-01-01

    La distrofia muscular de Duchenne (DMD) es la distrofia muscular más frecuente en niños. Se caracteriza por degeneración muscular progresiva que lleva a atrofia muscular, invalidez y muerte alrededor de la 2da década de vida. El ratón mdx ha sido ampliame 97p.

  12. FIBROSIS MUSCULAR ESQUELETICA: PAPEL DE LOS FIBROBLASTOS EN LA DISTROFIA MUSCULAR

    OpenAIRE

    MEZZANO ROBINSON, VALERIA

    2007-01-01

    La distrofia muscular de Duchenne (DMD) es la distrofia muscular más frecuente en niños. Se caracteriza por degeneración muscular progresiva que lleva a atrofia muscular, invalidez y muerte alrededor de la 2da década de vida. El ratón mdx ha sido ampliame 97p.

  13. Radiofrequency ablation of idiopathic ventricular tachycardia

    Energy Technology Data Exchange (ETDEWEB)

    Vohra, J.; Shah, A.; Hua, W.; Gerloff, J.; Riters, A. [Royal Melbourne Hospital, Melbourne, VIC (Australia)

    1996-04-01

    Radiofrequency ablation (RFA) has been shown to be very effective in the treatment of supraventricular tachycardias and has replaced surgical ablation. Only a few reports of RFA for idiopathic ventricular tachycardia (VT) have appeared in the literature during the last two years. This paper presents our experience with RFA for idiopathic VT in 19 patients. In all patients the diagnostic study and therapeutic RFA were combined in a single procedure. Pacemapping were used to guide the site of RFA in patients with VT arising from the RV. Local activation time (LAT), Purkinje potentials (PP) and pacemapping were used to guide RFA in those patients with LV septal tachycardias. Idiopathic VT frequently arises from the RVOT and inferobasal portion of the LV septum. These tachycardias can be diagnosed on clinical and ECG grounds. RFA for idiopathic VT arising from these areas has a high success rate and this mode of treatment should be considered as a nonpharmacological curative treatment for symptomatic patients. 13 refs., 3 tabs., 4 figs.

  14. 180. Técnica de exclusión septal

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    J.M. Vignau Cano

    2012-04-01

    Conclusiones: la disfunción ventricular grave de origen isquémico es una patología cuyo abordaje incluye la revascularización miocárdica, la corrección de la insuficiencia mitral y la restauración de la geometría ventricular con la técnica más adecuada.

  15. Morphology of tricuspid valve in pulmonary atresia with intact ventricular septum.

    Science.gov (United States)

    Choi, Y H; Seo, J W; Choi, J Y; Yun, Y S; Kim, S H; Lee, H J

    1998-01-01

    Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital cardiac anomaly that has been classified into two types: one is a more frequent type having dysplasia of tricuspid valve (TV) with a small annulus, underdeveloped right ventricle (RV) with a hypoplastic cavity and a hypertrophic wall; the other type has severe dysplasia of TV and dilatation of RV, right atrium (RA), and right atrioventricular junction with thinning of the RV wall. We performed a morphologic study on 11 autopsied hearts with PAIVS, giving particular emphasis to the variation of morphology of the TV. We could classify these hearts into 3 groups according to the degree of right ventricular development. In the first group of 7 cases (type I), the RVs were underdeveloped. Thick leaflets, restricted valve apparatus with short chordae, and small annuli were characteristics of the TV. In the second group of 3 cases (type II), the RVs showed marked enlargement of the cavity and thinning of the wall. The TV showed redundant, dysplastic, sail-like anterior leaflets, and the downward displacement of septal leaflet and/or posterior leaflet, which are the findings frequently observed in Ebstein's malformation. The RVs were dilated and with partially unguarded tricuspid orifice. The septal leaflet of the TV was dysplastic and, in two cases, the septal leaflet showed chordal structure at the upper surface facing the RA, which is a peculiar finding that has not been described in the literature. The remaining case was a heart with a moderately developed RV (type III). The TV showed mildly dysplastic appearance and we classify this as a separate type, because we could expect the best surgical results in this type. This type had optimal size of RV and the mildest degree of dysplasia of TV. In PAIVS, the morphology of TV correlates well with the type of the right ventricular development.

  16. Risk prediction of ventricular arrhythmias and myocardial function in Lamin A/C mutation positive subjects.

    Science.gov (United States)

    Hasselberg, Nina E; Edvardsen, Thor; Petri, Helle; Berge, Knut E; Leren, Trond P; Bundgaard, Henning; Haugaa, Kristina H

    2014-04-01

    Mutations in the Lamin A/C gene may cause atrioventricular block, supraventricular arrhythmias, ventricular arrhythmias (VA), and dilated cardiomyopathy. We aimed to explore the predictors and the mechanisms of VA in Lamin A/C mutation-positive subjects. We included 41 Lamin A/C mutation-positive subjects. PR-interval and occurrence of VA were recorded. Left ventricular (LV) myocardial function was assessed as ejection fraction and speckle tracking longitudinal strain by echocardiography. Magnetic resonance imaging was performed to assess fibrosis in a selection of subjects. Ventricular arrhythmias were documented in 21 patients (51%). Prolonged PR-interval was the best predictor of VA (P function by strain was reduced in the interventricular septum compared with the rest of the LV segments (-16.7% vs. -18.7%, P = 0.001) and correlated to PR-interval (R = 0.41, P = 0.03). Myocardial fibrosis was found exclusively in the interventricular septum and only in patients with VA (P = 0.007). PR-interval was longer in patients with septal fibrosis compared with those without (320 ± 66 vs. 177 ± 40 ms, P PR-interval was the best predictor of VA in Lamin A/C mutation-positive subjects. Electrical, mechanical, and structural cardiac properties were related in these subjects. Myocardial function was most reduced in the interventricular septum and correlated to prolonged PR-interval. Myocardial septal fibrosis was associated with prolonged PR-interval and VA. Localized fibrosis in the interventricular septum may be the mechanism behind reduced septal function, atrioventricular block and VA in Lamin A/C mutation-positive subjects.

  17. Adrenergic stress reveals septal hypertrophy and proteasome impairment in heterozygous Mybpc3-targeted knock-in mice.

    Science.gov (United States)

    Schlossarek, Saskia; Schuermann, Friederike; Geertz, Birgit; Mearini, Giulia; Eschenhagen, Thomas; Carrier, Lucie

    2012-05-01

    Hypertrophic cardiomyopathy (HCM) is characterized by asymmetric septal hypertrophy and is often caused by mutations in MYBPC3 gene encoding cardiac myosin-binding protein C. In contrast to humans, who are already affected at the heterozygous state, mouse models develop the phenotype mainly at the homozygous state. Evidence from cell culture work suggested that altered proteasome function contributes to the pathogenesis of HCM. Here we tested in two heterozygous Mybpc3-targeted mouse models whether adrenergic stress unmasks a specific cardiac phenotype and proteasome dysfunction. The first model carries a human Mybpc3 mutation (Het-KI), the second is a heterozygous Mybpc3 knock-out (Het-KO). Both models were compared to wild-type (WT) mice. Mice were treated with a combination of isoprenaline and phenylephrine (ISO/PE) or NaCl for 1 week. Whereas ISO/PE induced left ventricular hypertrophy (LVH) with increased posterior wall thickness to a similar extent in all groups, it increased septum thickness only in Het-KI and Het-KO. ISO/PE did not affect the proteasomal chymotrypsin-like activity or β5-subunit protein level in Het-KO or wild-type mice (WT). In contrast, both parameters were markedly lower in Het-KI and negatively correlated with the degree of LVH in Het-KI only. In conclusion, adrenergic stress revealed septal hypertrophy in both heterozygous mouse models of HCM, but proteasome dysfunction only in Het-KI mice, which carry a mutant allele and closely mimic human HCM. This supports the hypothesis that proteasome impairment contributes to the pathophysiology of HCM.

  18. Effectiveness of caudal septal extension graft application in endonasal septoplasty

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    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

  19. From the Heart: Interatrial Septal Aneurysm Identified on Bedside Ultrasound

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    Michael Butterfield

    2014-09-01

    Full Text Available A 61 year-old man presented to the Emergency Department for one day of nonspecific chest pain. Bedside echocardiogram performed by the emergency physician revealed normal systolic cardiac function but also showed a large ( > 10mm bicornuate interatrial septal aneurysm (IASA projecting into the right atrium (Figure 1, Video 1. There was no evidence of intraatrial thrombus. A formal echocardiogram performed later that day confirmed the diagnosis and also detected a patent foramen ovale (PFO with a left-to-right shunt that reversed with Valsalva maneuver. [West J Emerg Med. 2014;15(6:719–720

  20. [Non-puncture treatment of sinusitis in nasal septal defects].

    Science.gov (United States)

    Shilenkova, V V; Markov, G I; Shilenkov, A A

    1994-01-01

    The [symbol: see text] sinus-catheter was used to treat purulent sinusitis with septal perforation or defective septum of the sinuses. The procedure described implies simultaneous introduction of two sinus-catheters the functioning channels of which unite into one output by means of T-joint. The method has been successfully tried in the treatment of exudative and aggravated chronic purulent hemisinusitis (6 cases). The authors hold that communication between nasal sinuses is not a contraindication for sinus-catheter application. This fact widely extends potentialities of its use.

  1. Superstitious bar pressing in hippocampal and septal rats.

    Science.gov (United States)

    Devenport, L D

    1979-08-17

    Unlike normal animals or those with sham lesions, rats with hippocampal and septal lesions behaved in an operant chamber as if a dependency existed between pellet delivery and their behavior, despite the fact that reinforcement was based on time, not behavior, and was therefore free. This superstitious behavior did not result from a general inability to inhibit responding, as responding rapidly ceased when the pellets were discontinued. These findings suggest that the hippocampus integrates information regarding response-reinforcer relations, which in the normal rat permits superfluous operant behavior to be eliminated.

  2. Wasting mechanisms in muscular dystrophy.

    Science.gov (United States)

    Shin, Jonghyun; Tajrishi, Marjan M; Ogura, Yuji; Kumar, Ashok

    2013-10-01

    Muscular dystrophy is a group of more than 30 different clinical genetic disorders that are characterized by progressive skeletal muscle wasting and degeneration. Primary deficiency of specific extracellular matrix, sarcoplasmic, cytoskeletal, or nuclear membrane protein results in several secondary changes such as sarcolemmal instability, calcium influx, fiber necrosis, oxidative stress, inflammatory response, breakdown of extracellular matrix, and eventually fibrosis which leads to loss of ambulance and cardiac and respiratory failure. A number of molecular processes have now been identified which hasten disease progression in human patients and animal models of muscular dystrophy. Accumulating evidence further suggests that aberrant activation of several signaling pathways aggravate pathological cascades in dystrophic muscle. Although replacement of defective gene with wild-type is paramount to cure, management of secondary pathological changes has enormous potential to improving the quality of life and extending lifespan of muscular dystrophy patients. In this article, we have reviewed major cellular and molecular mechanisms leading to muscle wasting in muscular dystrophy. This article is part of a Directed Issue entitled: Molecular basis of muscle wasting.

  3. Glucocorticoids for Duchenne Muscular Dystrophy

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    J Gordon Millichap

    2013-07-01

    Full Text Available Investigators at the Dubowitz Neuromuscular Centre, Great Ormond Street Hospital, and other centers in the UK, conducted a prospective longitudinal study across 17 neuromuscular centers in the UK of 360 boys aged 3-15 years with Duchenne muscular dystrophy who were treated with daily or intermittent (10 days on/10 days off prednisolone for a mean duration of 4 years.

  4. Inherited myopathies and muscular dystrophies

    NARCIS (Netherlands)

    Cardamone, Michael; Darras, Basil T.; Ryan, Monique M.

    The inherited myopathies and muscular dystrophies are a diverse group of muscle diseases presenting with common complaints and physical signs: weakness, motor delay, and respiratory and bulbar dysfunction. The myopathies are caused by genetic defects in the contractile apparatus of muscle, and

  5. Porcine models of muscular dystrophy

    Science.gov (United States)

    Duchenne muscular dystrophy is a progressive, fatal, X-linked disease caused by a failure to accumulate the cytoskeletal protein, dystrophin. This disease is modeled by a variety of animal models including several fish models, mice, rats, and dogs. While these models have contributed substantially t...

  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. Percutaneous closure of atrial septal defect with situs solitus and dextrocardia.

    Science.gov (United States)

    Galal, Mohammed Omar; Khan, Muhammad Arif; El-Segaier, Milad

    2015-02-01

    Percutaneous closure of secundum atrial septal defect associated with situs solitus and dextrocardia has not been reported previously. We describe the technical difficulties encountered during transcatheter closure of a secundum atrial septal defect in a 19-month-old girl with situs solitus and dextrocardia.

  8. 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.

  9. Diagnosis of asymptomatic atrial septal aneurysms using two-dimensional color Doppler and contrast transthoracic echocardiography

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    Objective To evaluate the dimensions of atrial septal aneurysm (ASA), the presence and characteristics of interatrial shunt, the movement of the wall of the aneurysm, and correlation between these findings and sign and/or symptoms suggesting embolism in Manisa, a district of a western Anatolian city of Turkey. Methods Two thousand five hundred cases were examined by routine transthoracic echocardiography (TTE) in both pediatric and adult cardiology outpatient clinics. ASA was detected in 20 cases and evaluated by two-dimensional color Doppler echocardiography (CDE). The length of the base, the maximum radius and the maximum displacement of ASA were measured. The shunt between the atria was examined by CDE. In cases where a shunt could not be found, galactose and palmitic acid was injected. Standard 12-lead electrocardiogram (ECG) and exercise stress test were also performed. Results No clinical signs or symptoms were found, suggesting a systemic or cerebral embolism. The maximum displacement of ASA was between 2 and 5 mm. All of the aneurysms were localized in the right atrium, and the walls of the aneurysm did not move beyond the base of the left atrium during the maximum displacement. Interatrial shunt was detected in 14 of 20 patients (70%) by CDE and in the remaining six cases by contrast TTE. Frequent ventricular ectopic beats were observed in one patient. Conclusions During routine TTE we observed 0.8% asymptomatic ASA in our population. The use of a contrast agent was found to be a valuable additional method in patients with ASA when the shunt could not be detected by CDE. The risk for embolism is not high when the maximum displacement of the wall of ASA was 5 mm or less and no bulge into the left atrium was observed. Based on our experience with this method, TTE is easy to perform, well-tolerated and acceptable.

  10. Surgical Closer of Atrial Septal Defect in Adults after 40 Years Old

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    Salehi Rezvanieh

    2014-01-01

    Full Text Available Objective: Atrial septal defect (ASD is the most common congenital heart disease in adults. The aim of this study was to determine the value of surgical closure of ASD in patients over 40 years of age. Materials and Methods: Retrospective analysis of 96 patients with the mean age of 47.58 ± 6.59, who had undergone surgical repair of ASD, was carried out. Pre and postoperative clinical status, New York Heart Association (NYHA functional class and systolic pulmonary artery pressure before and after surgical repair, complications of post operation were assessed and analyzed. The follow-up period was between 1 month and 16 years. Results: Before the operations, 62 patients (89.8% were placed in NYHA functional classes of II and III. However, after the surgeries most of the patients could be placed in the functional classes I and II. The mean of pulmonary artery pressure before the surgeries was about 46.68 ± 14.18 and dropped to 32 ± 11.89 mm Hg after the operations (P < 0.0001. Atrial fibrillation rhythm was present in 18 cases preoperatively which was reduced to 3 patients at the discharge time (16.6%. The mean right ventricular (RV sizes were reduced from 4.1 to 2.5 cm after the surgical repairs. In 35 cases (36.64%, we had no tricuspid valve regurgitation after surgery for RV systolic presser estimation. Conclusion: Surgical closure of ASD in patients over 40 years of age could improve their clinical status, and lead to a reduced pulmonary systolic as well as smaller RV sizes.

  11. Bailout transcatheter closure of patent foramen ovale for refractory hypoxaemia after left ventricular assist device implantation.

    Science.gov (United States)

    Fischer, Quentin; Kirsch, Matthias; Brochet, Eric; Juliard, Jean-Michel

    2015-08-01

    We describe the interdisciplinary management of a 59-year old man with ischaemic cardiomyopathy on a HeartMate II left ventricular assist device (LVAD) and temporary right extracorporeal membrane oxygenation (ECMO) as a bridge-to-heart transplantation. He suffered refractory hypoxaemia due to massive right-to-left shunting by a patent foramen ovale (PFO), diagnosed after weaning off of temporary right ECMO. Percutaneous closure of the PFO was successfully achieved with an Amplatzer septal occluder device, which allowed the patient's extubation and departure from hospital. The patient received heart transplantation 7 weeks after LVAD implantation and was discharged from the intensive care unit 2 weeks after transplantation.

  12. 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).

  13. Short- and Mid-term Results of Atrial Septal Defect and Patent Foramen Ovale Occlusion with Starway Septal Occluder Device

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    J Kojuri

    2011-09-01

    Full Text Available Background: With a prevalence of almost 7% of all congenital heart diseases, atrial septal defect (ASD is a common condition. Patent foramen ovale (PFO is also a congenital heart disease which is frequently sustained into adulthood. Objectives: To study the feasibility of closure of ASD and PFU by Starway septal occluder device and the incidence of its inherent complications and procedural failure in 62 patients referred to our center. Methods: Starway septal occluder device was used for closure of ASD and PFO in 62 patients. After left and right heart catheterization, transesophageal echocardiography-guided closure was done for the patients with immediate recording of the results. Patients were followed for 6 months by transesophageal echocardiography for observing short- and mid-term complications. Results: The 62 studied patients were categorized into 2 groups. Group 1 included 31 patients (64% females with ASD (mean±SD age: 26.7±7.6 years. Group 2 consisted of 31 patients (35.6% females with PFO (mean±SD age: 53.5±12.4 years. Size of the right ventricle (RV annulus was significantly (P=0.005 decreased after the intervention in the ASD group. Overall 5 (8% patients developed post-intervention complications (transient ischemic attack, leg edema, and residual shunt and procedural failure—4 (13% in ASD group and 1 (3% in PFO group. None of the patients developed device-related thrombosis, significant arrhythmia, aortic regurgitation and pericardial effusion after intervention. Conclusion: Starway occluder device is effective and safe with very low short- and mid-term complication rates.

  14. Vulnerability to ventricular fibrillation

    Science.gov (United States)

    Janse, Michiel J.

    1998-03-01

    One of the factors that favors the development of ventricular fibrillation is an increase in the dispersion of refractoriness. Experiments will be described in which an increase in dispersion in the recovery of excitability was determined during brief episodes of enhanced sympathetic nerve activity, known to increase the risk of fibrillation. Whereas in the normal heart ventricular fibrillation can be induced by a strong electrical shock, a premature stimulus of moderate intensity only induces fibrillation in the presence of regional ischemia, which greatly increases the dispersion of refractoriness. One factor that is of importance for the transition of reentrant ventricular tachycardia to ventricular fibrillation during acute regional ischemia is the subendocardial Purkinje system. After selective destruction of the Purkinje network by lugol, reentrant tachycardias still develop in the ischemic region, but they do not degenerate into fibrillation. Finally, attempts were made to determine the minimal mass of thin ventricular myocardium required to sustain fibrillation induced by burst pacing. This was done by freezing of subendocardial and midmural layers. The rim of surviving epicardial muscle had to be larger than 20 g. Extracellular electrograms during fibrillation in both the intact and the "frozen" left ventricle were indistinguishable, but activation patterns were markedly different. In the intact ventricle epicardial activation was compatible with multiple wavelet reentry, in the "frozen" heart a single, or at most two wandering reentrant waves were seen.

  15. Redefining the Septal L-Strut to Prevent Collapse

    Science.gov (United States)

    Ha, Dong-Heon; Kim, Sung Won; Cho, Dong-Woo

    2016-01-01

    During septorhinoplasty, septal cartilage is frequently resected for various purposes but the L-strut is preserved. Numerous materials are inserted into the nasal dorsum during dorsal augmenation rhinoplasty without considering nasal structural safety. This study used a finite element method (FEM) to redefine the septal L-strut, to prevent collapse as pressure moved from the rhinion to the supratip breakpoint on the nasal dorsum and as the contact percentage between the caudal L-strut and the maxillary crest changed. We designed a 1-cm-wide L-strut model based on computed tomography data. At least 45% of the width of the L-strut in the inferior portion of the caudal strut must be preserved during septoplasty to stabilize the septum. In augmentation rhinoplasty, the caudal L-strut must either be preserved perfectly or reinforced to prevent collapse or distortion of the L-strut. The dorsal augmentation material must be fixed in an augmentation pocket to prevent movement of graft material toward the supratip breakpoint, which can disrupt the L-strut. We conducted a numerical analysis using a FEM to predict tissue/organ behavior and to help clinicians understand the reasons for target tissue/organ collapse and deformation. PMID:27073993

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

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    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. Relationship Between Fragmented QRS Complexes and Cardiac Status in Duchenne Muscular Dystrophy: Multimodal Validation Using Echocardiography, Magnetic Resonance Imaging, and Holter Monitoring.

    Science.gov (United States)

    Cho, Min-Jung; Lee, Ji-Won; Lee, JeSang; Shin, Yong Bum; Lee, Hyoung Doo

    2017-06-01

    The presence of fragmented QRS is a known marker of heterogeneous ventricular activation around the myocardial scar area. We validated whether fragmented QRS shows any association with ventricular dysfunction, fibrosis, or ventricular arrhythmias in patients with Duchenne muscular dystrophy (DMD). Thirty-seven patients with DMD were evaluated using electrocardiography (ECG), echocardiography, cardiac magnetic resonance, and 24-h Holter monitoring. Associations between fragmented QRS and ventricular dysfunction, fibrosis, or ventricular arrhythmia were investigated. Fragmented QRS complexes were present in 31 of 37 (83.7%) patients, and they were associated with a significantly lower left ventricular ejection fraction along with an increased left ventricular Tei index as evaluated by echocardiography, and more frequent ventricular arrhythmia as indicated using 24-hour Holter monitoring compared with patients without fragmented QRS. The number of leads with fragmented QRS correlated negatively with left ventricular ejection fraction both using echocardiography (r = -0.616) and CMR (r = -0.516). Further, the number of leads with fragmented QRS showed a significant correlation with several other echocardiographic measurements (mitral Em and Sm, and left ventricular Tei index), and 2-dimensional speckle-tracking echocardiography derived global left ventricular longitudinal strain. The frequency of ventricular arrhythmia observed using Holter monitoring showed a significant positive correlation with the frequency of fragmented QRS on ECG (r = 0.674). There was a positive trend of correlation between fragmented QRS and the amount of myocardial fibrosis as assessed by late gadolinium enhancement using CMR, but the statistical significance of the relationship was low (r = 0.433, p = 0.056). Fragmentation of QRS complexes is associated with degrees of left ventricular dysfunction, fibrosis, and ventricular arrhythmias in patients with DMD.

  18. Left ventricular apical diseases.

    Science.gov (United States)

    Cisneros, Silvia; Duarte, Ricardo; Fernandez-Perez, Gabriel C; Castellon, Daniel; Calatayud, Julia; Lecumberri, Iñigo; Larrazabal, Eneritz; Ruiz, Berta Irene

    2011-08-01

    There are many disorders that may involve the left ventricular (LV) apex; however, they are sometimes difficult to differentiate. In this setting cardiac imaging methods can provide the clue to obtaining the diagnosis. The purpose of this review is to illustrate the spectrum of diseases that most frequently affect the apex of the LV including Tako-Tsubo cardiomyopathy, LV aneurysms and pseudoaneurysms, apical diverticula, apical ventricular remodelling, apical hypertrophic cardiomyopathy, LV non-compaction, arrhythmogenic right ventricular dysplasia with LV involvement and LV false tendons, with an emphasis on the diagnostic criteria and imaging features. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1007/s13244-011-0091-6) contains supplementary material, which is available to authorized users.

  19. Genetics progress on atrioventricular septal defect%先天性房室间隔缺损的遗传学进展

    Institute of Scientific and Technical Information of China (English)

    李晓维

    2011-01-01

    先天性房室间隔缺损(atrioventricular sepial defect,AVSD)是一种常见的心血管畸形,由于存在房室间隔(房间隔下部、室间隔上部)以及中央心内膜垫组织的缺损,造成左右心腔之间的异常交通.虽然AVSD的胚胎学、病理生理学以及诊断和治疗目前都已基本阐明,但其确切的发病机制仍无突破性进展.随着分子生物学技术的广泛应用以及分子遗传学研究的深入开展,AVSD在遗传学的研究中取得一系列新进展,一些基因被证实与AVSD的发生发展存在一定的相关性.%Atrioventricular septal defect (AVSD) is a common cardiovascular malformation because of atrioventricular septal (lower atrial septum, ventricular septal upper) and the endocardial cushion defect,resulting in abnormal chambers of the heart. At the present, although the embryology, pathophysiology,diagnosis and treatment of the AVSD are clarified, but its precise pathogenesis has still no breakthrough progress.With the wide application of molecular biology and the depth research of molecular genetics, a series of new progress about AVSD has been made in the genetic study, and some genes are confirmed to be related to the occurrence and development of AVSD. The aim of this article is to review and discuss genetic mechanisms and related genes of AVSD, and to further identify the major genes causing AVSD.

  20. Phase 3 Study of Ataluren in Patients With Nonsense Mutation Duchenne Muscular Dystrophy

    Science.gov (United States)

    2017-05-30

    Muscular Dystrophy, Duchenne; Muscular Dystrophies; Muscular Disorders, Atrophic; Muscular Diseases; Musculoskeletal Diseases; Neuromuscular Diseases; Nervous System Diseases; Genetic Diseases, X-Linked; Genetic Diseases, Inborn

  1. [Dystroglycan linkage and muscular dystrophy].

    Science.gov (United States)

    Shimizu, Teruo

    2002-11-01

    Dystroglycan is a key complex between basal lamina laminin, extracellularly and membrano-cytoskeleton, intracellularly. The damage of this linkage is turned out to cause muscular dystrophies. Dystroglycan knockout is lethal. Dystroglycan-associated intracellular proteins such as dystrophin, dystrobrevin, sarcoglycans, plectin and caveolin-3 are responsible for causing severe (Duchenne type) and moderate forms (Becker, LGMDs). Laminin, dystroglycan-binding extracellular protein, is deficient in the most severe form of congenital muscular dystrophy with normal intelligence and eye. Recently, a remarkable progress is made in most severe forms of congenital muscular dystrophy with anomalies of brain and eye such as Fukuyama type (Japan) and muscle-eye-brain disease (Finland). The gene product for Fukuyama type, fukutin, belongs to a family of glycosylation enzymes in bacteria and yeast. Since alpha-dystroglycan contains 14-15 o-glycans, ser/thr-mannose 2-1 GlcNAc 4-1 Gal 3-2 Sial in the middle third mucin-domain and the sial-o-glycan is essential for laminin-binding, and since alpha-dystroglycan is defective in Fukuyama type sarcolemma with anti both sugar moiety- and peptide-antidodies, defective fukutin causes incomplete o-glycosylation of alpha-dystroglycan. In '02, it is clarified that a glycosylation enzyme, POMGnT1 which modifies GlcNAc onto ser/thr-mannose, is defective in 6 MEB patients. The loss of the enzyme activity is turned out to lose alpha-dystroglycan from sarcolemma of MEB. These data strongly suggests that o-glycosylation defect of alpha-dystroglycan causes the most severe congenital muscular dystrophy such as Fukuyama type, MEB and Walker Warburg syndrome.

  2. Oclusão percutânea das comunicações interventriculares: experiência inicial Interventricular septal defects percutaneous occlusion: initial experiment

    Directory of Open Access Journals (Sweden)

    Francisco José Araújo Chamié de Queiróz

    2005-09-01

    Full Text Available OBJETIVO: Avaliar a experiência inicial com o fechamento percutâneo das comunicações interventriculares (CIV, em especial, das CIV perimembranosas (CIVPM com a nova prótese de Amplatzer. MÉTODOS: Foram submetidos ao procedimento onze pacientes, seis com CIV perimembranosas (CIVPM e cinco com CIV musculares (CIVM. Dois apresentavam infecções repetidas do trato respiratório e tinham baixo ganho ponderal. Um apresentou história prévia de endocardite infecciosa. Os demais eram assintomáticos e foram selecionados pela ecocardiografia transtorácica (ETT. RESULTADOS: No grupo das CIVM (n=5, um paciente tinha uma CIV apical, dois tinham defeitos médio-septais e dois pacientes apresentavam defeitos múltiplos. Neste grupo, foi utilizada uma prótese de comunicação interatrial (CIA (Amplatzer Septal Occluder® e cinco próteses para CIVM (Amplatzer VSD-MUSC Occluder®, sendo que uma mesma paciente recebeu dois dispositivos. Todos os implantes foram bem sucedidos e transcorreram sem complicações. No grupo das CIVPM (n=6, dois tinham aneurismas de septo membranoso. Em 5, identificamos dois pertuitos com angiografia seletiva. Utilizamos próteses de Amplatzer para canal arterial (PCA (Amplatzer Duct Occluder® em 1 paciente e próteses específicas para CIVPM nos demais (Amplatzer VSD-MEMB Occluder®. Um oclusor perimembranoso migrou imediatamente depois de liberado, sendo retirado da aorta descendente. Outro paciente apresentou bloqueio atrioventricular total (BAVT, que reverteu após corticoterapia. CONCLUSÃO: O procedimento é tecnicamente complexo e deve ser executado apenas em centros especializados. Apesar disso, mostrou-se seguro e eficaz nos casos selecionados, e pode ser oferecido como alternativa ao tratamento cirúrgico tradicional.OBJECTIVE: To assess the initial experiment with percutaneous closure of interventricular septal defects (IVSD, especially perimembranous IVSD (PMIVSD with the new Amplatzer prosthesis. METHODS

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

    Directory of Open Access Journals (Sweden)

    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.

  4. Association between arterial stiffness and left ventricular diastolic function in relation to gender and age

    Science.gov (United States)

    Kim, Hack-Lyoung; Lim, Woo-Hyun; Seo, Jae-Bin; Chung, Woo-Young; Kim, Sang-Hyun; Kim, Myung-A.; Zo, Joo-Hee

    2017-01-01

    Abstract Left ventricular (LV) diastolic dysfunction and subsequent overt heart failure are more prevalent in elderly women. Close interaction between arterial stiffness and LV morphology/function has been reported. The aim of this study was to investigate whether there is an age- and gender-dependent relationship between arterial stiffness and LV diastolic function. A total of 819 subjects (58.6 ± 13.3 years, 50.2% men) without structural heart disease (LV ejection fraction ≥50%) were retrospectively analyzed. All participants underwent transthoracic echocardiography and brachial-ankle pulse wave velocity (baPWV) measurement on the same day. The association of baPWV with septal e′ velocity and average E/e′ was assessed. In the total study subjects, baPWV was negatively correlated with septal e′ velocity (r = 0.383, P  0.05 for each). In conclusion, baPWV was independently associated with septal e′ velocity and E/e′ in elderly women but not in younger women or men. The results of this study provide additional evidence that increased arterial stiffness plays an important role in the development of heart failure with preserved ejection fraction as well as LV diastolic dysfunction in elderly women. PMID:28072727

  5. [Fractures in spinal muscular atrophy].

    Science.gov (United States)

    Febrer, Anna; Vigo, Meritxell; Rodríguez, Natalia; Medina, Julita; Colomer, Jaume; Nascimento, Andrés

    2013-09-01

    Objetivo. Determinar la frecuencia de fracturas en pacientes con atrofia muscular espinal, mecanismo de produccion, edad de aparicion y repercusion funcional. Pacientes y metodos. Se estudian 65 pacientes con atrofia muscular espinal. Se recogen las fracturas diagnosticadas mediante radiografia y se analizan los siguientes parametros: tipo de atrofia muscular espinal, marcha, edad en el momento de la fractura, mecanismo de produccion, localizacion, tratamiento aplicado y repercusion funcional. Resultados. Presentaron fracturas 13 pacientes (20%), con un total de 20 (cuatro presentaron dos o mas fracturas). La edad media fue de 6,35 años. La localizacion fue en su mayoria en el femur y el mecanismo de produccion, en 12 casos por caidas y en 8 por traumatismo menor. No detectamos ninguna fractura vertebral. Todas se trataron de manera conservadora. El unico paciente ambulante que presento una fractura dejo de caminar despues de la inmovilizacion. Conclusiones. La existencia de fracturas en estos pacientes interfiere en su calidad de vida y en el nivel funcional. Es importante la prevencion de las mismas en el manejo del paciente y vigilando la correcta postura en la silla de ruedas con sistemas de sujecion Deberian emprenderse mas estudios sobre la perdida de densidad mineral osea en estos pacientes y su posible relacion con las fracturas.

  6. Diffuse interstitial fibrosis assessed by cardiac magnetic resonance is associated with dispersion of ventricular repolarization in patients with hypertrophic cardiomyopathy.

    Science.gov (United States)

    Hurtado-de-Mendoza, David; Corona-Villalobos, Celia P; Pozios, Iraklis; Gonzales, Jorge; Soleimanifard, Yalda; Sivalokanathan, Sanjay; Montoya-Cerrillo, Diego; Vakrou, Styliani; Kamel, Ihab; Mormontoy-Laurel, Wilfredo; Dolores-Cerna, Ketty; Suarez, Jacsel; Perez-Melo, Sergio; Bluemke, David A; Abraham, Theodore P; Zimmerman, Stefan L; Abraham, M Roselle

    2017-06-01

    Hypertrophic cardiomyopathy (HCM) is characterized by myocyte hypertrophy, disarray, fibrosis, and increased risk for ventricular arrhythmias. Increased QT dispersion has been reported in patients with HCM, but the underlying mechanisms have not been completely elucidated. In this study, we examined the relationship between diffuse interstitial fibrosis, replacement fibrosis, QTc dispersion and ventricular arrhythmias in patients with HCM. We hypothesized that fibrosis would slow impulse propagation and increase dispersion of ventricular repolarization, resulting in increased QTc dispersion on surface electrocardiogram (ECG) and ventricular arrhythmias. ECG and cardiac magnetic resonance (CMR) image analyses were performed retrospectively in 112 patients with a clinical diagnosis of HCM. Replacement fibrosis was assessed by measuring late gadolinium (Gd) enhancement (LGE), using a semi-automated threshold technique. Diffuse interstitial fibrosis was assessed by measuring T1 relaxation times after Gd administration, using the Look-Locker sequence. QTc dispersion was measured digitally in the septal/anterior (V1-V4), inferior (II, III, and aVF), and lateral (I, aVL, V5, and V6) lead groups on surface ECG. All patients had evidence of asymmetric septal hypertrophy. LGE was evident in 70 (63%) patients; the median T1 relaxation time was 411±38 ms. An inverse correlation was observed between T1 relaxation time and QTc dispersion in leads V1-V4 (pdispersion in leads V1-V4 (odds ratio, 1.011 [1.004-1.0178, p=0.003). We found no correlation between presence and percentage of LGE and QTc dispersion. Diffuse interstitial fibrosis is associated with increased dispersion of ventricular repolarization in leads, reflecting electrical activity in the hypertrophied septum. Interstitial fibrosis combined with ion channel/gap junction remodeling in the septum could lead to inhomogeneity of ventricular refractoriness, resulting in increased QTc dispersion in leads V1-V4.

  7. [Trans-septal endoscopic approach of pituitary tumors].

    Science.gov (United States)

    Muñoz del Castillo, F; Jurado Ramos, A; De la Riva Aguilar, A; López Villarejo, P

    2003-10-01

    The advances in endoscopic instruments have eased the approach to the sellar region through the nasal cavity. We carry out an analysis of the surgical results on 20 patients that underwent surgery for sellar tumours through a transeptal-transphenoidal approach in the last 2 years in our hospital. The average was 45.6 years old, and 75% were females. 30% of cases were pituitary adenomas and another 30% acromegaly, 25% Cushing's disease and 10% prolactinomas. No complications were encountered during surgery being the most common postoperative complications, diabetes insipida in two cases (10%) and CSF leak in one case. At present 2 patients are having hormonal treatment for panhypopituitarism. No patients developed a septal perforation, nasal deformity, epistaxis, meningitis, lip numbness or oronasal fistula. The rest did have good results noith no recurrence and hormonal values back to normal.

  8. [Indications for implantable loop recorders in patients with channelopathies and ventricular tachycardias].

    Science.gov (United States)

    Köbe, Julia; Wasmer, Kristina; Reinke, Florian; Eckardt, Lars

    2016-12-01

    Implantable loop recorders (ILR) do not play a pivotal role in the current guidelines on ventricular arrhythmias except in identifying rhythm-symptom correlations if ventricular arrhythmias are assumed. Before a decision for a pure diagnostic implantable device is made, a thorough arrhythmic risk assessment is of major importance due to the potential lethal outcome of ventricular arrhythmias. Nevertheless, some clinical circumstances exist where long-term monitoring by an ILR may add significant information in electrical heart diseases, in patients with ventricular arrhythmias, or structural heart diseases and a potential risk of ventricular arrhythmias. As medical therapy (β-blocker therapy) plays an important role in long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardias (cpVT), the ILR can be used to control therapy in patients at risk. In electrical diseases without pharmacologic therapeutic options (e. g., Brugada syndrome), the ILR may be used in low-risk patients with atypical syncope as benign faints may occur without association to the underlying disease. Evidence on cardiomyopathies with preserved left ventricular function and nonsustained VT or premature ventricular complexes is scarce. The ILR may also add long-term information on the individual risk in these circumstances. In very rare diseases like infiltrative disease or muscular dystrophies, the ILR may also provide evidence on risk stratification. In summary, ILR in electrical heart diseases and in patients with ventricular tachycardia remains a very individual decision taking into account various clinical, electrocardiographic, and genetic parameters. The following review aims at highlighting possible indications and clinical scenarios for ILR in ventricular tachycardias and electrical heart diseases with-probably debatable-case presentations.

  9. Does asymptomatic septal agenesis exist? A review of 34 cases

    Energy Technology Data Exchange (ETDEWEB)

    Belhocine, Ouardia; Andre, Christine; Kalifa, Gabriel; Adamsbaum, Catherine [St Vincent de Paul Hospital, Radiology Department, Paris (France)

    2005-04-01

    Primary septal agenesis (PSA) is a rare brain malformation that can be isolated or part of developmental brain abnormalities (holoprosencephaly, septo-optic dysplasia or cortical malformation). Such associated malformation can be subtle, leading to difficulties in the prenatal management of PSA. Moreover, the neurological prognosis of isolated PSA remains debatable. The aims of the study were to specify the patterns and frequency of brain malformations associated with septal agenesis (SA), to identify the clinical prognosis, and to discuss the aetiology of PSA with the new insights provided by molecular genetics. The study consisted of a 14-year retrospective review of brain MRI in 34 patients having PSA (mean age, 5 years). Chiasm and optic nerves were not evaluated. Post-hydrocephalus SA or incomplete data were excluded. The clinical data were correlated to the MRI patterns. The study disclosed 82.5% associated lesions with MRI (28/34): 11 neuronal migration disorders, 9 holoprosencephalies (HP), 7 pituitary stalk interruptions, 1 corpus callosum partial agenesis; 17.5% (6/34) of cases were apparently isolated PAS. Clinically, the patients had motor dysfunction in 68% (23/34), mental retardation in 65% (22/34), blindness in 24% (8/34), endocrinological defects in 21% (7/34) and epilepsy in 18% (6/34) of cases. Nine percent of patients (3/34) were neurologically normal (including one with scoliosis and two infants younger than 2 years at the last follow-up). Patients with bilateral cortical anomalies and HP (even if mild) had the worst neurological prognosis. A severe motor impairment was present without evidence of hemispheric anomaly in 12% of patients (4/34). Interestingly, the frontal lobes were involved in 90% of cortical anomalies and HP, supporting the malformative aetiology of PSA. PSA rarely appears isolated and severe psychomotor impairment may occur in apparently isolated forms. These unfavourable results should be highlighted and need to be confirmed

  10. Late endocarditis of Amplatzer atrial septal occluder device in a child

    Institute of Scientific and Technical Information of China (English)

    Neerod; K; Jha; Laszlo; Kiraly; John; SK; Murala; Csaba; Tamas; Haitham; Talo; Hazem; El; Badaoui; Magdi; Tofeig; Malaika; Mendonca; Sameer; Sajwani; Mary; A; Thomas; Sura; Ahmed; Al; Doory; Mohammad; D; Khan

    2015-01-01

    Bacterial endocarditis following atrial septal defect closure using Amplatzer device in a child is extremely rare. We report a 10-year-old girl who developed late bacterial endocarditis, 6 years after placement of an Amplatzer atrial septal occluder device. Successful explantation of the device and repair of the resultant septal defect was carried out using a homograft patch. The rare occurrence of this entity prompted us to highlight the importance of a closed long-term follow up, review the management and explore preventive strategies for similar patients who have multiple co-morbidities and a cardiac device. A high index of suspicion is warranted particularly in pediatric patients.

  11. 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.

  12. Improved Left Ventricular Structure and Function After Successful Kidney Transplantation

    Directory of Open Access Journals (Sweden)

    Bernd Hewing

    2016-10-01

    Full Text Available Background/Aims: Cardiac changes observed in chronic kidney disease patients are of multifactorial origin including chronic uremia, hemodynamics or inflammation. Restoration of renal function by kidney transplantation (KTX may reverse cardiac changes. Novel echocardiographic methods such as speckle tracking echocardiography (STE allow early and sensitive detection of subtle changes of cardiac parameters. We evaluated changes of cardiac structure and function after KTX by advanced echocardiographic modalities. Methods: Thirty-one KTX recipients (female n=11 were evaluated by medical examination, laboratory testing and echocardiography before and after KTX (median follow-up 19 months. Left ventricular (LV and right ventricular (RV diameters and function were assessed by echocardiographic standard parameters. Longitudinal 2D strain of the LV (GLPS and left atrium (LA was determined by 2D STE. Results: After KTX, median serum creatinine level was 1.3 mg/dl (IQR, 1.2-1.5. Systolic blood pressure decreased significantly after KTX. Echocardiography showed a significant reduction in LV end-diastolic septal and posterior wall thickness and LV mass index after KTX, which was accompanied by an improvement of GLPS. There were no relevant changes in parameters of LA (reservoir, conduit or contractile function, LV diastolic or RV function after KTX. Conclusion: LV hypertrophy reversed after successful KTX and was accompanied by an improvement in longitudinal LV function as assessed by STE. Diastolic function and STE-derived LA function parameters did not change significantly after KTX.

  13. Quantitative assessment of regional right ventricular function with color kinesis.

    Science.gov (United States)

    Vignon, P; Weinert, L; Mor-Avi, V; Spencer, K T; Bednarz, J; Lang, R M

    1999-06-01

    We used color kinesis, a recent echocardiographic technique that provides regional information on the magnitude and timing of endocardial wall motion, to quantitatively assess regional right ventricular (RV) systolic and diastolic properties in 76 subjects who were divided into five groups, as follows: normal (n = 20), heart failure (n = 15), pressure/volume overload (n = 14), pressure overload (n = 12), and RV hypertrophy (n = 15). Quantitative segmental analysis of color kinesis images was used to obtain regional fractional area change (RFAC), which was displayed in the form of stacked histograms to determine patterns of endocardial wall motion. Time curves of integrated RFAC were used to objectively identify asynchrony of diastolic endocardial motion. When compared with normal subjects, patients with pressure overload or heart failure exhibited significantly decreased endocardial motion along the RV free wall. In the presence of mixed pressure/volume overload, the markedly increased ventricular septal motion compensated for decreased RV free wall motion. Diastolic endocardial wall motion was delayed in 17 of 72 segments (24%) in patients with RV pressure overload, and in 31 of 90 segments (34%) in patients with RV hypertrophy. Asynchrony of diastolic endocardial wall motion was greater in the latter group than in normal subjects (16% versus 10%: p kinesis images allows quantitative assessment of regional RV systolic and diastolic properties.

  14. Lesiones musculares en el deporte. Muscular injuries in sport.

    Directory of Open Access Journals (Sweden)

    Jiménez Díaz, José Fernando

    2006-04-01

    Full Text Available ResumenDurante la práctica de la actividad física hay una gran incidencia de lesiones musculares, si bien se han llevado a cabo pocos estudios clínicos sobre el tratamiento y la resolución de las mismas. Desde el punto de vista etiopatogénico, hay que señalar que la incidencia de lesión es mayor en aquellos músculos poliarticulares en condiciones de acumulación de fatiga y con condiciones ambientales desfavorables. La clasificación de las lesiones musculares permite distinguir entre aquellas que no afectan a la fascia produciéndose un sangrado dentro del mismo (intramuscular o bien si la fascia también se rompe, el sangrado se sitúa entre los diferentes músculos (intermuscular. El tratamiento de estas lesiones se realizará combinando reposo, compresión, aplicación de frío y elevación del área lesionada así como el desarrollo de un adecuado programa de readaptación funcional que permita al jugador incorporarse lo antes posible a la dinámica del equipo. En la actualidad se está llevando a cabo opciones terapéuticas con factores de crecimiento, terapia génica y células madre, si bien todavía no están lo suficientemente desarrolladas.AbstractDuring the practice of the physical activity there is a great effect of muscular injuries, though few clinical studies have been carried out on the treatment and the resolution of the same ones. Inside the reasons it is necessary to indicate that the effect of injury is major in those muscles you will polyarticulate in situation of fatigue and with environmental unfavorable conditions.The classification of the muscular injuries allows to distinguish between those that do not affect the fascia producing the bled intramuscular or if the fascia also breaks, the bled one places between the different muscles (intermuscular.The treatment will be realized combining rest, compression, application of cold and elevation of these injuries as well as the development of a program of functional

  15. Evaluation of left ventricular structures in normotensive and hypertensive subjects by two-dimensional echocardiography: Anthropometric correlates in hypertension

    Directory of Open Access Journals (Sweden)

    Mr. Agbo Julius Amaechi

    2008-07-01

    Full Text Available This study was conducted with the objective to establish a nomogram for some left ventricular structures and their alterations in hypertension. Correlations between left ventricular structures and anthropometric variables in hypertension were also established. A sample of 320 normotensive and 80 hypertensive subjects were studied. Echocardiograhic end diastolic diameter, posterior wall thickness and septal wall thickness were obtained. Subject height, weight, age and blood pressures were obtained. Blood pressures were measured in sitting position. The values of left ventricular mass (LVM, left ventricular mass index (LVMI and left relative wall thickness (RWT were computed. Parametric tests were conducted. Tests were two tailed with P < 0.05 indicating statistical significance. Normal values of left ventricular structures were established; LVM: 63.72g – 336.18g, LVMI: 38.16g/m – 222.64g/m, and RWT: 0.25 – 0.52. Significant differences (P < 0.05 were established in LVM, LVMI and RWT between normotensive and hypertensive subjects. Positive and significant correlations were noted between these variables and systolic blood pressure in hypertensive subjects. A simple linear regression of RWT on Body surface area gives RWT = - 0.058 BSA + 0.475 in normotensive subjects. Normal values of left ventricular structures and a linear regression model have been established which could be used in the assessment of morbidity in hypertension.

  16. Alcohol septal ablation and hypertrophic cardiomyopathy%酒精室间隔消融术与肥厚型心肌病

    Institute of Scientific and Technical Information of China (English)

    Carey Kimmelstiel; Barath Krishnamurthy; Andrew Weintraub; Navin Kapur; 王新国

    2009-01-01

    Patients with hypertrophic cardiomyopathy who experience refractory symptoms due to left ventricular outflow tract obstruction are often referred for definitive therapy consisting of either surgical myectomy or alcohol septal ablation ( ASA). There currently exists clinical equipoise regarding which therapy is the most efficacious in this challenging patient population. ASA utilizes common interventional techniques usually employed to treat atherosclerotic coronary artery disease to inject small aliquots of ethanol into a branch of the appropriate septal vessel to cause necrosis of the obstructing basal septal tissue. Myocardial contrast echocardiography is used to facilitate location of the most appropriate septal branch with success determined by an acute reduction in the resting and/or provoked gradient. Recent comparative data have suggested similar rates of long and short-term mortality in when comparing patients undergoing ASA and surgical myectomy, with ASA patients experiencing a higher rate of requirement for permanent pacemakers. In addition, patients treated by both techniques appear to have similar gradient reductions and improvement in symptomatic status. Comparisons of these two methods of treatment are limited by the non-randomized nature of the studies, retrospective data collection and the allocation of higher-risk patients to ASA treatment. Concern for the wide-spread adoption of ASA to drug-resistant HCM patients is warranted due to the potential for arrhythmogenesis is a patient population already at risk for life-threatening arrhythmias. There have been case reports of such arrhythmias, however, clinical series to date have not suggested an enhanced risk of sudden cardiac death in patients treated with ASA. Definitive answers concerning which patient subsets with drug-refractory hypertrophic cardiomyopathy would benefit from the two competing therapies can only be answered by a randomized clinical trial. However, for a variety of clinical and

  17. Transcatheter closure of atrial septal defect protects from pulmonary edema: septal occluder device gradually reduces LR shunt.

    Science.gov (United States)

    Murakami, Tsutomu; Nakazawa, Gaku; Horinouchi, Hitomi; Torii, Sho; Ijichi, Takeshi; Ohno, Yohei; Amino, Mari; Shinozaki, Norihiko; Ogata, Nobuhiko; Yoshimachi, Fuminobu; Yoshioka, Koichiro; Ikari, Yuji

    2017-01-01

    A 56-year-old woman was diagnosed as atrial septal defect (ASD) with pulmonary hypertension; pulmonary blood flow/systemic blood flow (Qp/Qs) of 2.3, pulmonary artery pressure (PAP) of 71/23(39) mmHg and diastolic dysfunction of left ventricle. PAP was improved after medical therapy; therefore, transcatheter ASD closure was performed. Seven days later, left-sided heart failure occurred, however, the improvement of Qp/Qs (1.7) and PAP of 51/21(32) was confirmed. Diuretic therapy was introduced which led to further decrease of PAP 40/12(25) and Qp/Qs (1.1). Because of gradual decrease of Qp/Qs, this patient appeared to be protected from acute pulmonary edema.

  18. Preventive echocardiographic examination in athletes and workers – Quadricuspid aortic valve and atrial septal aneurysm in a young basketball player

    Directory of Open Access Journals (Sweden)

    Karina Wierzbowska-Drabik

    2015-02-01

    Full Text Available Ensuring safety of young athletes and employees who perform hard physical work within the scope of their professional duties, with a special focus on prevention of a sudden cardiac death at sports fields or during hard physical work is one of the most important tasks, which demands joint effort of cardiologists and sport physicians or occupational physicians, who qualify patients for a job or a sport discipline. Apart from hypertrophic and arhythmogenic right ventricular cardiomyopathy, coronary anomalies and aortic dissection belong to the most frequent causes of dramatic complications during competitive exercise or work with an increased energy expenditure. Although a detailed medical history and a physical examination combined with 12-lead ECG assessment may significantly improve the safety in competitive sports, adding echocardiography examination gives a detailed and noninvasive insight into the heart morphology and function. Therefore, in our opinion, it should constitute a standard part of the evaluation of candidates for competitive sports. The practice indicates that beyond subjects with severe heart diseases and those classified as normal, there is a group of individuals with abnormalities which should be more closely monitored, but are not contraindications against professional sports or work with an increased energy expenditure. We describe the case of a young female with a diagnosis of rare congenital aortic valve disease, quadricuspid valve, with mild regurgitation and atrial septal aneurysm which was established during transthoracic echocardiography and confirmed and expanded during TEE examination.

  19. Genetics Home Reference: spinal and bulbar muscular atrophy

    Science.gov (United States)

    ... Kennedy spinal and bulbar muscular atrophy Kennedy's disease SBMA X-linked spinal and bulbar muscular atrophy Related ... Natural history of spinal and bulbar muscular atrophy (SBMA): a study of 223 Japanese patients. Brain. 2006 ...

  20. Asymmetric septal hypertrophy - a marker of hypertension in aortic stenosis (a SEAS substudy)

    DEFF Research Database (Denmark)

    Tuseth, Nora; Cramariuc, Dana; Rieck, Ashild E

    2010-01-01

    Some patients with aortic stenosis develop asymmetric septal hypertrophy (ASH) that may influence the surgical approach and is associated with higher perioperative morbidity. The aim of this analysis was to characterize further this subtype of aortic stenosis patients....

  1. Microvascular permeability changes might explain cardiac tamponade after alcohol septal ablation for hypertrophic cardiomyopathy.

    Science.gov (United States)

    Hsu, Jen-Te; Hsiao, Ju-Feng; Chang, Jung-Jung; Chung, Chang-Min; Chang, Shih-Tai; Pan, Kuo-Li

    2014-04-01

    Various sequelae of alcohol septal ablation for hypertrophic obstructive cardiomyopathy have been reported. Of note, some cases of cardiac tamponade after alcohol septal ablation cannot be well explained. We describe the case of a 78-year-old woman with hypertrophic obstructive cardiomyopathy in whom cardiac tamponade developed one hour after alcohol septal ablation, probably unrelated to mechanical trauma. At that time, we noted a substantial difference in the red blood cell-to-white blood cell ratio between the pericardial effusion (1,957.4) and the peripheral blood (728.3). In addition to presenting the patient's case, we speculate that a possible mechanism for acute tamponade--alcohol-induced changes in microvascular permeability--is a reasonable explanation for cases of alcohol septal ablation that are complicated by otherwise-unexplainable massive pericardial effusions.

  2. [Funtcional and morphological changes in nasal mucosa in patients presenting with septal deformation].

    Science.gov (United States)

    Piskunov, V S; Mezentseva, O Iu

    2011-01-01

    This study was designed to obtain morpho-functional characteristics of nasal mucosa associated with septal deformation. It has demonstrated disturbances in the mucociluary transport, compromised mucosal immunity, and morphological changes in the nasal cavity.

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

    Directory of Open Access Journals (Sweden)

    Kátia Marie Simões e Senna

    2015-08-01

    Full Text Available 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.

  4. Changes of Left Ventricular Geometry Shape and Left Ventricular Regional Function in Patients With Dilated Cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    Liang-yu WANG; Ming-xing XIE; Qing-bo LI; Ping CHEN; Zhi-xiong CAI; Zhi-dan ZHU

    2009-01-01

    Objectives To assess the left ventricle regional systolic and diastolic function, left ventricle geometry and left venti-tie sphericity indexes in patients with dilated cardiomyopathy (DCM) by quantitative tissue velocity imaging (QTVI). Methods Thirty normal subjects and 52 DCM patients underwent QTVI and colour Doppler flow imaging study in or-der to measure the left ventricular regional function along left ventricle apical long-axis view and the left ventricle geom-etry. Peak tissue velocities of left venticle regional muscular tissue during systole (Vs), systolic acceleration (a), ear-ly diastole(Ve) and left atrium contraction(Va) along left venticle apical long axis view were measured. The indexes of left ventdcular regional systolic and diastolic function were mearsured at the same time. The left ventricle geometry shape was reflected from the systolic and diastolic sphericity index (Sis and Sid), the left ventricular ejection fraction (LVEF) and D wave/A wave (PVd/Pva) of pulmonary veins flowing spectrum reflected the global left ventricular systolic and diastolic function. The Vs, Ve, Va, a, PVd/Pva ratio, LVEF, Sis, Sid and their correlations between normal subjects and patients with DCM were compared and analyzed. Results Vs, Ve, Va, a, PVd/Pva, Sis and Sid in patients with DCM were lower than those in normal persons. There were significant relations between Sis and a (r=0.6142, P<0.05), Ve/Va and Sid (r=0.6271, P<0.05). Conclusions QTVI offer a newer method which has a higher sensitivity and accuracy in evaluating the left venticle regional systolic and diastolic function in DCM patients. There was significant relation between regional cardiac function and left venticle sphericity.

  5. 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.

  6. Ventricular Assist Device Support: for Management of Sustained Ventricular Arrhythmias

    OpenAIRE

    2002-01-01

    We describe herein the cases of 2 patients who had ventricular arrhythmias. In one, a short-term biventricular assist device, the ABIOMED BVS 5000, was placed because the patient had sustained ventricular tachycardia and could not be weaned from cardiopulmonary bypass. Excellent hemodynamic support was maintained for several days while the antiarrhythmic therapy was maximized. Sinus rhythm was restored, and the patient was successfully weaned from the ventricular assist device. However, the s...

  7. Fatal septicemia in a patient with cerebral lymphoma and an Amplatzer septal occluder: a case report

    Directory of Open Access Journals (Sweden)

    Stöllberger Claudia

    2011-11-01

    Full Text Available Abstract Introduction The Amplatzer septal occluder is frequently used for percutaneous closure of an atrial septal defect. Complications include thrombosis and embolism, dislocation, cardiac perforation, and, rarely, infection. We report the case of a patient who had survived an occluder-related thromboembolism two years previously. Case presentation A 72-year-old Caucasian woman had received a septal occluder because of an atrial septal defect seven years ago. Two years ago, she underwent chemotherapy of a non-Hodgkin lymphoma, developed atrial fibrillation, and experienced a left-sided occluder thrombosis with stroke and peripheral embolism. Now, she presented with cerebral lymphoma, received glucocorticoids, and subsequently developed skin lesions. Swabs from the lesions and blood cultures were positive for methicillin-resistant Staphylococcus aureus and Pseudomonas aeruginosa. Endocarditis, however, was considered only two months later and echocardiography suggested aortic valve endocarditis. Despite antibiotic therapy, she died three days later because of septicemia, and no post-mortem investigation was carried out. It remains uncertain whether the septal occluder was endothelialized or infected and whether explantation might have changed the outcome. Conclusions If infections occur in patients with a septal occluder, endocarditis should be considered and echocardiography should be performed early. To prevent a fatal outcome, explantation of the septal occluder should be considered, especially in patients with problems that suggest delayed endothelialization. Post-mortem investigations, including bacteriologic studies, should be carried out in patients with a septal occluder in order to assess the focal and global long-term effects of these devices.

  8. Asymmetric septal hypertrophy - a marker of hypertension in aortic stenosis (a SEAS substudy)

    DEFF Research Database (Denmark)

    Tuseth, Nora; Cramariuc, Dana; Rieck, Ashild E

    2010-01-01

    Some patients with aortic stenosis develop asymmetric septal hypertrophy (ASH) that may influence the surgical approach and is associated with higher perioperative morbidity. The aim of this analysis was to characterize further this subtype of aortic stenosis patients.......Some patients with aortic stenosis develop asymmetric septal hypertrophy (ASH) that may influence the surgical approach and is associated with higher perioperative morbidity. The aim of this analysis was to characterize further this subtype of aortic stenosis patients....

  9. Total endoscopic robotic atrial septal defect repair in a patient with dextrocardia and situs inversus totalis.

    Science.gov (United States)

    Iino, Kenji; Watanabe, Go; Ishikawa, Norihiko; Tomita, Shigeyuki

    2012-04-01

    Situs inversus with mirror-image of the heart is a rare condition. The present report describes a case of a patient with dextrocardia with situs inversus who had atrial septal defect with multiple holes in the fossa ovalis. The patient underwent total endoscopic atrial septal defect repair using the da Vinci surgical system. This procedure was achieved safely with good clinical and excellent cosmetic results.

  10. Incomplete endothelialisation of an Amplatzer Septal Occluder device followed by meningitis and late acute bacterial endocarditis.

    Science.gov (United States)

    Białkowski, Jacek; Pawlak, Szymon; Banaszak, Paweł

    2016-04-01

    A 19-year-old woman with atrial septal defect treated percutaneously with an Amplatzer Septal Occluder 24 months earlier, who presented with a history of bacterial meningitis, was admitted with a diagnosis of endocarditis. After 6 weeks of treatment with antibiotics, the incompletely endothelialised occluder was surgically removed. The present report illustrates the need for long-term follow-up of patients who have received nitinol wire mesh occluders.

  11. Device and method for treatment of openings in vascular and septal walls

    Energy Technology Data Exchange (ETDEWEB)

    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.

  12. Cardiac pathology in spinal muscular atrophy: a systematic review.

    Science.gov (United States)

    Wijngaarde, C A; Blank, A C; Stam, M; Wadman, R I; van den Berg, L H; van der Pol, W L

    2017-04-11

    Hereditary proximal spinal muscular atrophy (SMA) is a severe neuromuscular disease of childhood caused by homozygous loss of function of the survival motor neuron (SMN) 1 gene. The presence of a second, nearly identical SMN gene (SMN2) in the human genome ensures production of residual levels of the ubiquitously expressed SMN protein. Alpha-motor neurons in the ventral horns of the spinal cord are most vulnerable to reduced SMN concentrations but the development or function of other tissues may also be affected, and cardiovascular abnormalities have frequently been reported both in patients and SMA mouse models. We systematically reviewed reported cardiac pathology in relation to SMN deficiency. To investigate the relevance of the possible association in more detail, we used clinical classification systems to characterize structural cardiac defects and arrhythmias. Seventy-two studies with a total of 264 SMA patients with reported cardiac pathology were identified, along with 14 publications on SMA mouse models with abnormalities of the heart. Structural cardiac pathology, mainly septal defects and abnormalities of the cardiac outflow tract, was reported predominantly in the most severely affected patients (i.e. SMA type 1). Cardiac rhythm disorders were most frequently reported in patients with milder SMA types (e.g. SMA type 3). All included studies lacked control groups and a standardized approach for cardiac evaluation. The convergence to specific abnormalities of cardiac structure and function may indicate vulnerability of specific cell types or developmental processes relevant for cardiogenesis. Future studies would benefit from a controlled and standardized approach for cardiac evaluation in patients with SMA.

  13. Collagen immunostains can distinguish capsular fibrous tissue from septal fibrosis and may help stage liver fibrosis.

    Science.gov (United States)

    Chen, Wei; Rock, Jonathan B; Yearsley, Martha M; Hanje, A James; Frankel, Wendy L

    2014-01-01

    Core-needle biopsy remains essential for diagnosis of cirrhosis; however, evaluation of fibrosis in such biopsies is often challenging due to the fragmented nature of cirrhotic liver specimens. It is also common to see portions of liver capsules present in the biopsy which adds to the diagnostic challenge. The distinction between capsular/subcapsular fibrous tissue and septal fibrosis is critical to avoid potential overstaging of liver fibrosis. We compared the differential immunostaining in liver capsular and septal areas for collagens III, IV, V, VI, vitronectin, laminin, Orcein, and Trichrome in 15 whole sections of explanted cirrhotic livers and 5 simulated liver biopsies. Collagens III, IV, V, VI, Trichrome, and Orcein show distinct staining patterns in capsular fibrous tissue and septal fibrosis. Collagen IV shows strong diffuse septal staining and consistently weak to negative capsular staining. Collagens III and VI stain similar to IV for septal fibrosis, whereas collagen V, Trichrome, and Orcein show strong staining in both areas. Collagen IV, possibly with III or VI in addition to the routine Trichrome and hematoxylin and eosin stain, is useful in differentiating capsular fibrous tissue from septal fibrosis on challenging and fragmented liver biopsies.

  14. Atrial Fibrillation in Hypertrophic Cardiomyopathy: Is the Extent of Septal Hypertrophy Important?

    Science.gov (United States)

    Park, Kyoung-Min; Im, Sung Il; Kim, Eun Kyoung; Lee, Sang-Chol; Park, Seung-Jung; Kim, June Soo; On, Young Keun

    2016-01-01

    Hypertrophic cardiomyopathy (HCM) is a cardiac disease associated with a high incidence of atrial fibrillation (AF). Recent studies have suggested that interventricular septum thickness may influence the risk stratification of patients with AF. We evaluated the effects of septal hypertrophy on morbidity and mortality in patients with HCM. Patients were followed for a median of 6.1 years and were divided into two groups according to the extent of septal hypertrophy. A total of 1,360 HCM patients were enrolled: 482 (33%) apical or apicoseptal, 415 (28%) asymmetric septal, 388 (27%) basal septal, 38 (2.6%) concentric, and 37 (2.5%) diffuse and mixed type. Ninety-two all-cause deaths and 21 cardiac deaths occurred. The total event rates were significantly higher for patients with HCM with more extensive septal hypertrophy (group A) compared to those with HCM ± focal septal hypertrophy (group B), regardless of type (phypertrophy (phypertrophy [odds ratio (OR) 5.44 (2.29-12.92), phypertrophy is an independent predictor of progression to AF in patients with HCM.

  15. Nutrition Considerations in Duchenne Muscular Dystrophy.

    Science.gov (United States)

    Davis, Jillian; Samuels, Emily; Mullins, Lucille

    2015-08-01

    Duchenne muscular dystrophy (DMD) is a serious degenerative muscular disease affecting males. Diagnosis usually occurs in childhood and is confirmed through genetic testing and/or muscle biopsy. Accompanying the disease are several nutrition-related concerns: growth, body composition, energy and protein requirements, constipation, swallowing difficulties, bone health, and complementary medicine. This review article addresses the nutrition aspects of DMD.

  16. [Congenital muscular dystrophies in children].

    Science.gov (United States)

    Scavone-Mauro, Cristina; Barros, Graciela

    2013-09-06

    From the clinical and genetic point of view, congenital muscular dystrophies (CMD) are a heterogenic group of diseases within neuromuscular pathologies. The best known forms are: merosin deficiency CMD, collagen VI deficiency CMD, LMNA-related CMD, selenoprotein-related CMD (SEPN1) and alpha-dystroglycan-related CMD. They present with a broad spectrum of clinical phenotypes. Most of them are transmitted by recessive autosomal inheritance. The initial manifestations very often begin in infancy or in the neonatal period. There are clinical suspicions of the existence of hypotonia and paresis, and they are characterised by a dystrophic pattern in the muscular biopsy (muscle replaced by fibroadipose tissue, with necrosis and cell regeneration). Advances in the understanding of the molecular pathogenesis of CMD have made it possible to make further progress in the classification of the different subtypes. The aim of this review is to comment on the advances made in recent years as regards the classification of CMD in terms of genetics, the proteins involved and their clinical presentation.

  17. Association between angiotensin-converting enzyme gene polymorphisms and regression of left ventricular hypertrophy in patients treated with angiotensin-converting enzyme inhibitors.

    Science.gov (United States)

    Kohno, M; Yokokawa, K; Minami, M; Kano, H; Yasunari, K; Hanehira, T; Yoshikawa, J

    1999-05-01

    An insertion/deletion (ID) polymorphism of the angiotensin-converting enzyme (ACE) gene is associated with left ventricular hypertrophy. The present study examined polymorphisms of the ACE gene in patients with essential hypertension and left ventricular hypertrophy who were participants in a long-term trial of therapy with an ACE inhibitor. ACE inhibitor therapy was administered for >2 years to 54 patients with hypertension who had moderate or severe left ventricular hypertrophy. Cardiac dimensions were monitored by echocardiography before the initiation of therapy and after 1 and 2 years of treatment. Serum ACE activity and plasma concentrations of brain natriuretic peptide, a marker for left ventricular hypertrophy, were also monitored. Eighteen patients had the II genotype for the angiotensin-converting enzyme gene, 19 had the ID genotype, and 17 had the DD genotype. Baseline (mean +/- SD) serum ACE activity was significantly greater (P <0.05) in the DD (18 +/- 7 IU/L) group than in the II (7 +/- 4 IU/L) or ID (12 +/- 6 IU/L) groups. ACE inhibitor therapy was effective in controlling blood pressure, and it reduced posterior and septal wall thickness, left ventricular mass index, and plasma brain natriuretic peptide concentration in all three groups. Despite similar blood pressure reductions, after 2 years, mean (+/- SD) regression in posterior wall thickness was significantly less (P <0.05) in the DD group (-9% +/- 5%) than in the ID (-21% +/- 7%) and II (-21% +/- 9%) groups. Similar results were seen for the reductions in brain natriuretic peptide levels. The magnitudes of regression of septal wall thickness and left ventricular mass index during therapy were less in the DD group than the II group (P <0.05). Hypertensive patients with the DD genotype are less likely to have regression of left ventricular hypertrophy when treated with ACE inhibitors than are patients with other ACE genotypes.

  18. Pocket proteins critically regulate cell cycle exit of the trabecular myocardium and the ventricular conduction system

    Directory of Open Access Journals (Sweden)

    David S. Park

    2013-07-01

    During development, the ventricular conduction system (VCS arises from the trabecular or spongy myocardium. VCS and trabecular myocytes proliferate at a significantly slower rate than compact zone myocardial cells, establishing a transmural cell cycle gradient. The molecular determinants of VCS/trabecular myocyte cell cycle arrest are not known. Given the importance of pocket proteins (Rb, p107 and p130 in mediating G0/G1 arrest in many cell types, we examined the role of this gene family in regulating cell cycle exit of the trabecular myocardium and ventricular conduction system. Using a combinatorial knockout strategy, we found that graded loss of pocket proteins results in a spectrum of heart and lung defects. p107/p130 double knockout (dKO hearts manifest dysregulated proliferation within the compact myocardium and trabecular bases, while the remaining trabecular region cell cycle exits normally. Consequently, dKO hearts exhibit defective cardiac compaction, septal hyperplasia and biventricular outflow tract obstruction, while the VCS appears relatively normal. Loss of all three pocket proteins (3KO is necessary to completely disrupt the transmural cell cycle gradient. 3KO hearts exhibit massive overgrowth of the trabecular myocardium and ventricular conduction system, which leads to fetal heart failure and death. Hearts carrying a single pocket protein allele are able to maintain the transmural cell cycle gradient. These results demonstrate the exquisite sensitivity of trabecular and conduction myocytes to pocket protein function during ventricular chamber development.

  19. 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.

  20. [Septal alcohol ablation in patients with hypertrophic cardiomyopathy].

    Science.gov (United States)

    López-Aburto, Gustavo; Palacios-Rodríguez, Juan Manuel; Cantú-Ramírez, Samuel; Galván-García, Eduardo; Tolosa-Dzul, Gonzalo; Morán-Benavente, Armando; Ontiveros-Martínez, Raúl

    2013-01-01

    Objetivo: conocer características demográficas, clínicas y hemodinámicas de los pacientes con ablación con alcohol para tratar la miocardiopatía hipertrófica septal obstructiva (MHSO). Métodos: estudio observacional, longitudinal, descriptivo de 21 pacientes con MHSO resistente a tratamiento o con gradiente = 30 mm Hg en reposo o = 60 mm Hg provocado y con movimiento sistólico anterior o insuficiencia mitral > grado II. Resultados: edad de 50 ± 16 años; hombres 38.1 % y mujeres 61.9 %. Los síntomas fueron angor 42.9 %, disnea 85.7 % y síncope 23.8 %. La clase funcional NYHA preablación fue grados III y IV en 61.9 %; al año todos tenían grados I y II. Preablación y al año, el espesor del septo interventricular fue de 22.7 ± 4.9 y 20.7 ± 3.1 mm. Preablación, después y al año, la fracción de eyección fue de 65.5 ± 7, 62.2 ± 6.5 y 68.7 ± 6.2 %. Preablación, después y al año, el gradiente del tracto de salida del ventrículo izquierdo fue de 106.9 ± 29.9, 44.6 ± 24.3 y 22.0 ± 5.7 mm Hg. Preablación, la insuficiencia mitral fue grados III y IV en 33.3 y 47.6 % y al año fue grados 0 en 52.4 %, I en 28.6 % y II en 19 %. No hubo defunciones intrahospitalarias. Conclusiones: la ablación septal con alcohol en pacientes con MHSO fue exitosa.

  1. 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).

  2. Association of interatrial septal abnormalities with cardiac impulse conduction disorders in adult patients: experience from a tertiary center in Kosovo.

    Directory of Open Access Journals (Sweden)

    Zaim Gashi

    2011-06-01

    Full Text Available Interatrial septal disorders, which include: atrial septal defect, patent foramen ovale and atrial septal aneurysm, are frequent congenital anomalies found in adult patients. Early detection of these anomalies is important to prevent their hemodynamic and/or thromboembolic consequences. The aims of this study were: to assess the association between impulse conduction disorders and anomalies of interatrial septum; to determine the prevalence of different types of interatrial septum abnormalities; to assess anatomic, hemodynamic, and clinical consequences of interatrial septal pathologies. Fifty-three adult patients with impulse conduction disorders and patients without ECG changes but with signs of interatrial septal abnormalities, who were referred to our center for echocardiography, were included in a prospective transesophageal echocardiography study. Intera trial septal anomalies were detected in around 85% of the examined patients.

  3. Usefulness of early diastolic mitral annular velocity to predict plasma levels of brain natriuretic peptide and transient heart failure development after device closure of atrial septal defect.

    Science.gov (United States)

    Masutani, Satoshi; Taketazu, Mio; Mihara, Chihiro; Mimura, Yuko; Ishido, Hirotaka; Matsunaga, Tamotsu; Kobayashi, Toshiki; Senzaki, Hideaki

    2009-12-15

    Device closure of atrial septal defect (ASD) is sometimes followed by elevation of plasma brain natriuretic peptide (BNP), a marker of heart failure, and progression to heart failure. This study tested the hypothesis that the underlying diastolic dysfunction, assessed on tissue Doppler images (TDI) before device closure, can predict BNP level after ASD closure. The study subjects were 39 consecutive patients (age 27.5 +/- 16.3 years, range 5 to 63) who underwent device closure for ASD. Echocardiographic evaluation using TDI and 2-dimensional and pulse wave Doppler were performed, together with plasma BNP measurement 1 day before and 2 days after ASD closure. Before ASD closure, an age-dependent decrease was noted in left ventricular relaxation, assessed by early diastolic mitral annular velocity. ASD closure resulted in a decrease in early diastolic mitral annular velocity (from 14.7 to 12.3 cm/s, p linear regression identified early diastolic mitral annular velocity before ASD closure and age as independent predictors of BNP levels after ASD closure (p annular velocity developed exertional dyspnea after the procedure. In conclusion, our results indicate that TDI measurements could be useful to detect underlying diastolic dysfunction that can potentially cause heart failure after ASD closure and emphasize the importance of ASD closure at a young age before impairment of left ventricular relaxation.

  4. Electrophysiologic abnormalities of children with ostium secundum atrial septal defect.

    Science.gov (United States)

    Ruschhaupt, D G; Khoury, L; Thilenius, O G; Replogle, R L; Arcilla, R A

    1984-06-01

    Sinus node (SN) and atrioventricular node (AVN) function were evaluated in 49 patients with secundum type atrial septal defect (ASD). Automaticity and conduction system function were assessed by intracardiac recording of the AH and HV intervals at rest, corrected SN recovery time, sinoatrial conduction time, AVN refractory period and the ability of the AVN to conduct rapidly paced atrial beats to the ventricles. Electrophysiologic abnormalities were found in 41% of the 34 patients who were studied before surgery. However, no preoperative abnormalities were present in children younger than 2.5 years. If only children older than 2.5 years were analyzed, the incidence of conduction abnormalities was similar for the patients studied before operation (62%) and those studied after operation (71%). The size and ejection fractions of the right and left ventricles, the magnitude of shunt flow and the size of the ASD did not differ between the patients with and those without electrophysiologic abnormalities. AVN dysfunction was present in 40% of the patients who were studied after surgical repair. While this frequency was more than twice the preoperative incidence of AVN dysfunction, it was not statistically significant. The data suggest that patient age is the major factor that influences the presence of conduction system dysfunction in patients with ASD.

  5. Asymmetrical septal hypertrophy in newborn infants of diabetic mothers.

    Science.gov (United States)

    Vela-Huerta, M M; Vargas-Origel, A; Olvera-López, A

    2000-01-01

    The objective of this paper is to determine the frequency and outcome of asymmetrical septal hypertrophy (ASH) in large-for-gestational-age infants (LGA) born to diabetic (DM) and nondiabetic mothers (NDM), and to establish the relationship between ASH and maternal diabetes control. A comparative study was design to assess ASH in infants born to DM and NDM. The study was conducted in the Departments of Neonatology and Pediatric Cardiology of the "Hospital de Gineco-Pediatria 48", Instituto Mexicano del Seguro Social from January to December 1997. Eighty-five full-term infants of DM (group A) and 85 LGA infants of NDM (group B) were included. As a control group (group C), we studied 85 healthy, full-term infants. In all cases a Doppler echocardiogram was obtained in the first 48 h after birth, and for the ASH infants, at 2 and 4 months. Chest X ray, electrocardiogram, and laboratory tests were performed as complementary studies. ASH was present in 38.8% of LGA infants of DM and in 7.1% of NDM. The difference was significant (p < 0.01). Interventricular septum (IVS) and IVS/ posterior wall of left ventricle ratio were significantly different between groups A and B with C. There was no correlation between Hb A1 level and the presence of ASH in group A. ASH is a common finding in infants of DM. We could not find a relationship between the degree of metabolic control during pregnancy and the incidence and severity of ASH.

  6. Current perspectives in percutaneous atrial septal defect closure devices

    Directory of Open Access Journals (Sweden)

    Bissessor N

    2015-07-01

    Full Text Available N Bissessor1–4 1Department of Cardiology, The Epworth Hospital, Melbourne, VIC, Australia; 2Division of Interventional Cardiology, The Alfred Hospital, Melbourne, VIC, Australia; 3Department of Clinical Science, Charles Sturt University Albury Campus, NSW, Australia; 4Heart Foundation, Griffith University, QLD, Australia Abstract: In the last decade, percutaneous atrial septal defect (ASD closure has become the treatment of choice in most clinical presentations of ASD. Percutaneous ASD closure has established procedural safety through operator experience and improved device structure and deliverability. There have also been advances in diagnostic capabilities. Devices have evolved from large bulky meshes to repositionable, minimal residual mesh content that easily endothelializes and conforms well to surrounding structures. Biodegradable technology has been introduced and will be closely watched as a future option. The evolution of ASD closure device usage in the last four decades incorporates development that minimizes a wide range of serious side effects that have been reported over the years. Complications reported in the literature include thrombus formation, air embolization, device embolization, erosions, residual shunts, and nickel hypersensitivity. Modern devices have intermediate to long term data with outcomes that have been favorable. Devices are available in multiple sizes with improved delivery mechanisms to recapture, reposition, and safely close simple and complex ASDs amenable to percutaneous closure. In this review, commonly used devices and deployment procedures are discussed together with a look at devices that show promise for the future. Keywords: ASD, congenital, Amplatzer, Gore Helex, Biostar, Figulla

  7. Recurrence of atrial septal defect in three generations

    Directory of Open Access Journals (Sweden)

    Celso Ferreira

    1999-08-01

    Full Text Available Beginning with a patient presenting with an atrial septal defect (ASD of the secundum type, the genealogy was identified in four affected individuals who belonged to three successive generations of the same family. The defects were visually confirmed in all individuals and were found to be anatomically similar. No other congenital malformations were present in these individuals. The genealogy was identified in 1972, when ASD recurred in two generations, and it was concluded that the mechanism of transmission was autosomal recessive. The fifth individual, identified 21 years later, and having an anomaly identical to that of the others, was the child of a couple who had no consaguinity and whose mother was a member of the previously studied genealogy. Considering the absence of phenotype in the parents and the rarity of the ASD gene in the general population, the occurrence of the uniparental disomy for this family nucleus, and the same autosomal recessive mechanism of transmission by this affected individual is possible. This study reports the familial occurrence of ASD by genetic mechanisms of transmission, emphasizing the necessity for genetic-clinical studies in members of the familial nucleus in order to detect new carriers, who usually are asymptomatic, thereby allowing for early and adequate treatment of individuals who may be affected.

  8. Ventricular assist device support for management of sustained ventricular arrhythmias.

    Science.gov (United States)

    Fasseas, Panayotis; Kutalek, Steven P; Samuels, Fania L; Holmes, Elena C; Samuels, Louis E

    2002-01-01

    We describe herein the cases of 2 patients who had ventricular arrhythmias. In one, a short-term biventricular assist device, the ABIOMED BVS 5000, was placed because the patient had sustained ventricular tachycardia and could not be weaned from cardiopulmonary bypass. Excellent hemodynamic support was maintained for several days while the antiarrhythmic therapy was maximized. Sinus rhythm was restored, and the patient was successfully weaned from the ventricular assist device. However, the substrate for the arrhythmia persisted, and a recurrence, 1 week later, resulted in the patient's death. In the 2nd patient, the use of an implantable left ventricular assist device was successful in temporarily alleviating the ventricular tachycardia associated with ischemic cardiomyopathy. However, after 2 days of device assistance, the patient experienced a recurrence of the tachycardia, which degenerated into ventricular fibrillation with a marked deterioration in the patient's hemodynamics. The arrhythmia persisted despite multiple attempts at external cardioversion, and internal cardioversion and placement of an automatic implantable cardioverter-defibrillator were necessary. This treatment, along with repeated boluses of amiodarone, led to successful suppression of the arrhythmias, and the patient eventually underwent transplantation. The mechanical hemodynamic support of the circulation by ventricular assist devices was effective in supporting these 2 patients who had sustained ventricular arrhythmias.

  9. Association of interatrial septal abnormalities with cardiac impulse conduction disorders in adult patients: experience from a tertiary center in Kosovo

    Science.gov (United States)

    Bakalli, Aurora; Pllana, Ejup; Koçinaj, Dardan; Bekteshi, Tefik; Dragusha, Gani; Gashi, Masar; Musliu, Nebih; Gashi, Zaim

    2011-01-01

    Interatrial septal disorders, which include: atrial septal defect, patent foramen ovale and atrial septal aneurysm, are frequent congenital anomalies found in adult patients. Early detection of these anomalies is important to prevent their hemodynamic and/or thromboembolic consequences. The aims of this study were: to assess the association between impulse conduction disorders and anomalies of interatrial septum; to determine the prevalence of different types of interatrial septum abnormalities; to assess anatomic, hemodynamic, and clinical consequences of interatrial septal pathologies. Fifty-three adult patients with impulse conduction disorders and patients without ECG changes but with signs of interatrial septal abnormalities, who were referred to our center for echocardiography, were included in a prospective transesophageal echocardiography study. Interatrial septal anomalies were detected in around 85% of the examined patients. Patent foramen ovale was encountered in 32% of the patients, and in combination with atrial septal aneurysm in an additional 11.3% of cases. Atrial septal aneurysm and atrial septal defect were diagnosed with equal frequency in 20.7% of our study population. Impulse conduction disorders were significantly more suggestive of interatrial septal anomalies than clinical signs and symptoms observed in our patients (84.91% vs 30.19%, P=0.002). Right bundle branch block was the most frequent impulse conduction disorder, found in 41 (77.36%) cases. We conclude that interatrial septal anomalies are highly associated with impulse conduction disorders, particularly with right bundle branch block. Impulse conduction disorders are more indicative of interatrial septal abnormalities in earlier stages than can be understood from the patient’s clinical condition. PMID:21977304

  10. Postpartal right ventricular thrombosis.

    Science.gov (United States)

    Velicki, Lazar; Milosavljević, Aleksandar; Majin, Marijan; Vujin, Bojan; Kovacević, Pavle

    2008-11-01

    The discovery of an intracardial mass in patients presents a serious diagnostic dilemma. The differential diagnosis of this condition may seem abundant, but myxomas and intracardial thrombosis are the most frequent diagnoses. A connection between pregnancy and the presence of thrombosis has been documented frequently. Normal pregnancy leads to changes of the coagulative and fibrinolytic status toward a hypercoagulable condition which has its own physiological justification (the risk of blood loss decreases during labor). The case of a patient suffering from postpartal right ventricular thrombosis, which was successfully resolved by surgery as described in this contribution, demonstrates the value of a multidisciplinary approach.

  11. ATRIAL SEPTAL DEFECT IN A 50 YEARS OLD MALE PRESENTING AS OBLIQUE SEPTAL CANAL THAT APPEARED TO BE REGULATED BY FIVE LUMINAL BANDS

    Directory of Open Access Journals (Sweden)

    Ainory Peter Gesase

    2014-09-01

    Full Text Available To date seven different types of atrial septal defects (ASD have been described and they include septum primum, septum secundum, superior vena cava, inferior vena cava, coronary sinus and patent foramen ovale types of ASD. One feature in common among these ASD’s is that they all present with a hole that may allow communication between the left and right atria. The current observation reports what appears to be a new type of ASD that is characterized by the presence of an oblique septal canal that opens into the right and left atria. The right atrial opening was D-shaped and measured about 0.9 cm wide and the left atrial opening was crescent-shaped and measuring about 0.5 cm wide. In addition to this the left atrial opening was associated with five luminal bands; the last three bands lies on the roof of the oblique septal canal. The action of pulling the first luminal band resulted into closure of the left atrial opening an indication that the bands prevented blood coming from the lungs from entering into the right atrium. The presence of oblique septal canal and luminal bands has not been reported in relation to the ASD’s. Continued documentation of such anomalies remains clinically important particularly in African settings where unexplained illnesses are easily attributed to endemic diseases.

  12. Rapid Two-stage Versus One-stage Surgical Repair of Interrupted Aortic Arch with Ventricular Septal Defect in Neonates

    Directory of Open Access Journals (Sweden)

    Meng-Lin Lee

    2008-11-01

    Conclusion: The outcome of rapid two-stage repair is comparable to that of one-stage repair. Rapid two-stage repair has the advantages of significantly shorter cardiopulmonary bypass duration and AXC time, and avoids deep hypothermic circulatory arrest. LVOTO remains an unresolved issue, and postoperative aortic arch restenosis can be dilated effectively by percutaneous balloon angioplasty.

  13. Right Ventricular Pacing and Sensing Function in High Posterior Septal and Apical Lead Placement in Cardiac Resynchronization Therapy

    Directory of Open Access Journals (Sweden)

    H.M. Kristiansen, MD

    2012-01-01

    Conclusions: The RV-HS lead position demonstrated stable and acceptable long-term pacing and sensing function, with rates of complications comparable to conventional RV-A lead position in CRT. The RV-HS lead position is feasible in CRT-P.

  14. The “excluding” suture technique for surgical closure of ventricular septal defects: A retrospective study comparing the standard technique

    Directory of Open Access Journals (Sweden)

    Roy Varghese

    2016-01-01

    Conclusion: Surgical closure of VSDs can be accomplished by placing sutures along the margins or away with comparable results. The incidence of CHB, however, seems to be less when the “excluding” technique is employed.

  15. Statistical insights into major human muscular diseases.

    Science.gov (United States)

    Gupta, Shakti; Kim, Sung-Min; Wang, Yu; Dinasarapu, Ashok Reddy; Subramaniam, Shankar

    2014-07-15

    Muscular diseases lead to muscle fiber degeneration, impairment of mobility, and in some cases premature death. Many of these muscular diseases are largely idiopathic. The goal of this study was to identify biomarkers based on their functional role and possible mechanisms of pathogenesis, specific to individual muscular disease. We analyzed the muscle transcriptome from five major muscular diseases: acute quadriplegic myopathy (AQM), amyotrophic lateral sclerosis (ALS), mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS), dermatomyositis (DM) and polymyositis (PM) using pairwise statistical comparison to identify uniquely regulated genes in each muscular disease. The genome-wide information encoded in the transcriptome provided biomarkers and functional insights into dysregulation in each muscular disease. The analysis showed that the dysregulation of genes in forward membrane pathway, responsible for transmitting action potential from neural excitation, is unique to AQM, while the dysregulation of myofibril genes, determinant of the mechanical properties of muscle, is unique to ALS, dysregulation of ER protein processing, responsible for correct protein folding, is unique to DM, and upregulation of immune response genes is unique to PM. We have identified biomarkers specific to each muscular disease which can be used for diagnostic purposes.

  16. Assessment of left ventricular function by tissue Doppler imaging in patients with ankylosing spondylitis

    Directory of Open Access Journals (Sweden)

    Osman Kuloglu

    2012-05-01

    Full Text Available Ankylosing spondylitis (ASpis a chronic, inflammatory and systemic disease affecting pericardium, myocardium and the conduction system of the heart. In this study, we aimed to analyse left ventricular systolic and diastolic functions using tissue Doppler imaging (TDI. 30 patients with ASp and 30 healthy volunteers having the similar demographic characteristics were included. Left ventricular systolic and diastolic functions were assessed by using two dimensional (2D echocardiography, M-mode, pulsed-wave (PW and tissue Doppler echocardiography. The peak systolic velocity (Sm, early diastolic myocardial peak velocity (m, late diastolic myocardial peak velocity (Am, isovolumic acceleration (IVA, myocardial precontraction time (PCTm, myocardial contraction time (CTm, myocardial relaxation time (RTm, and myocardial performance index (MPI were measured at septal and lateral mitral annulus. In conventional echocardiography, end-diastolic interventricular septum and posterior wall diameters were higher in patients with ASp than the control group. The ratio of E/A was significantly lower and deceleration time was significantly prolonged in patients with ASp, but mitral E and A velocities, isovolumic relaxation time and MPI were similar in patient and control group (P>0.05. Left ventricular lateral and septal wall tissue Doppler echocardiography showed that Em, Em/Am ratio and CTm were significantly lower, IVRTm was longer and MPI was higher in patients with ASp. No significant differences were detected between the groups for IVA, Sm, Am, PCTm, PCTm/CTm ratio (P>0.05. We have demonstrated that in patients with ASp, diastolic functions were impaired but systolic functions were preserved by using TDI.

  17. Porcine models of muscular dystrophy.

    Science.gov (United States)

    Selsby, Joshua T; Ross, Jason W; Nonneman, Dan; Hollinger, Katrin

    2015-01-01

    Duchenne muscular dystrophy is a progressive, fatal, X-linked disease caused by a failure to accumulate the cytoskeletal protein dystrophin. This disease has been studied using a variety of animal models including fish, mice, rats, and dogs. While these models have contributed substantially to our mechanistic understanding of the disease and disease progression, limitations inherent to each model have slowed the clinical advancement of therapies, which necessitates the development of novel large-animal models. Several porcine dystrophin-deficient models have been identified, although disease severity may be so severe as to limit their potential contributions to the field. We have recently identified and completed the initial characterization of a natural porcine model of dystrophin insufficiency. Muscles from these animals display characteristic focal necrosis concomitant with decreased abundance and localization of dystrophin-glycoprotein complex components. These pigs recapitulate many of the cardinal features of muscular dystrophy, have elevated serum creatine kinase activity, and preliminarily appear to display altered locomotion. They also suffer from sudden death preceded by EKG abnormalities. Pig dystrophinopathy models could allow refinement of dosing strategies in human-sized animals in preparation for clinical trials. From an animal handling perspective, these pigs can generally be treated normally, with the understanding that acute stress can lead to sudden death. In summary, the ability to create genetically modified pig models and the serendipitous discovery of genetic disease in the swine industry has resulted in the emergence of new animal tools to facilitate the critical objective of improving the quality and length of life for boys afflicted with such a devastating disease.

  18. Transcatheter stenting of the right ventricular outflow tract augments pulmonary arterial growth in symptomatic infants with right ventricular outflow tract obstruction and hypercyanotic spells.

    Science.gov (United States)

    McGovern, Eimear; Morgan, Conall T; Oslizlok, Paul; Kenny, Damien; Walsh, Kevin P; McMahon, Colin J

    2016-10-01

    We retrospectively reviewed all the children with right ventricular outflow tract obstruction, hypoplastic pulmonary annulus, and pulmonary arteries who underwent stenting of the right ventricular outflow tract for hypercyanotic spells at our institution between January, 2008 and December, 2013; nine patients who underwent cardiac catheterisation at a median age of 39 days (range 12-60 days) and weight of 3.6 kg (range 2.6-4.3 kg) were identified. The median number of stents placed was one stent (range 1-4). The median oxygen saturation increased from 60% to 96%. The median right pulmonary artery size increased from 3.3 to 5.5 mm (-2.68 to -0.92 Z-score), and the median left pulmonary artery size increased from 3.4 to 5.5 mm (-1.93 to 0 Z-scores). Among all, one patient developed transient pulmonary haemorrhage, and one patient had pericardial tamponade requiring drainage. Complete repair of tetralogy of Fallot +/- atrioventricular septal defect or double-outlet right ventricle was achieved in all nine patients. Transcatheter stent alleviation of the right ventricular outflow tract obstruction resolves hypercyanotic spells and allows reasonable growth of the pulmonary arteries to facilitate successful surgical repair. This represents a viable alternative to placement of a systemic-to-pulmonary artery shunt, particularly in small neonates.

  19. Late complete atrioventricular block after closure of an atrial septal defect with a gore septal occluder (GSO™).

    Science.gov (United States)

    Dittrich, Sven; Sigler, Matthias; Priessmann, Helga

    2016-04-01

    Temporary intermittent complete heart block (CHB) occurred the day after interventional closure of an ASD with a 30 mm Gore Septal Occluder (GSO™) in a 2 years and 11-month-old female. CHB disappeared without further treatment and stable sinus rhythm recovered within 3 days. Only short episodes of 2nd degree AV-block (Wenckebach periodicity) at rare intervals were documented in Holter-monitors the following 2 months. Eleven months after device implantation the patient suffered from long lasting episodes of CHB. Surgical removal of the device resulted in incomplete recovery of AV-conduction. Histopathological work-up of the explanted GSO showed complete endothelialization of the device and regular scar formation. One year after surgery, the child had sinus rhythm during daytime but needed VVI-pacing while sleeping. Young age, inferior localization of the defect, and use of a large device have been individual risk factors for CHB in this patient. Clinical course and histologic findings indicate that mechanical compression was the only cause for CHB. The cumulative number of reports of CHB after use of different ASD-devices supports the recommendation to postpone the intervention in asymptomatic patients to preschool-age. Early removal of a pushing device may increase the chance of complete recovery from CHB. © 2015 The Authors. Catheterization and Cardiovascular Interventions Published by Wiley Periodicals, Inc.

  20. Right ventricular metastasis of leiomyosarcoma

    Directory of Open Access Journals (Sweden)

    Stagmo Martin

    2009-05-01

    Full Text Available Abstract Metastatic presentation of leiomyosarcoma in the heart is very rare. We present transthoracic echocardiography and combined PET/CT images of a case with a large right ventricular metastasis of leiomyosarcoma. The patient was placed on cytostatic drugs for palliative purposes, but passed away one month later because of an untreatable ventricular tackycardia.

  1. Mitochondria and left ventricular hypertrophy

    Institute of Scientific and Technical Information of China (English)

    Haiyan Zhu; Shiwen Wang

    2008-01-01

    @@ Introduction Left ventricular hypertrophy (LVH) is one of the vicious organ damages of essential hypertension.It contributes a lot to high mortality of essential hypertension due to sudden cardiac death,ventricular arrhythmia and heart failure.Many factors involve in the pathogenesis of hypertension-induced LVH including inherited variants as well as environmental factors.

  2. Divertículo ventricular congênito associado à taquicardia ventricular Congenital ventricular diverticulum associated with ventricular tachycardia

    Directory of Open Access Journals (Sweden)

    Ranieli Pitol

    2005-02-01

    Full Text Available Divertículos ventriculares congênitos são raros. Clinicamente, podem ser assintomáticos ou causa de embolização sistêmica, insuficiência cardíaca, insuficiência valvar, ruptura ventricular, arritmia ventricular ou morte súbita. Apresentamos caso de uma mulher de 56 anos com taquicardia ventricular sustentada, na qual, durante a investigação, foi diagnosticada a presença de um divertículo na posição ínfero-basal do ventrículo esquerdo. Comentam-se as características clínicas e o tratamento desta doença infreqüente.Congenital ventricular diverticula are rare. Clinically, they may be asymptomatic or cause systemic embolization, heart failure, valvular regurgitation, ventricular rupture, ventricular arrhythmia, or sudden death. We report the case of a 56-year-old woman with sustained ventricular tachycardia, who, during investigation, was diagnosed with a diverticulum in the inferobasal portion of the left ventricle. The clinical characteristics and treatment of this rare disease are discussed.

  3. Optical and thermal properties of nasal septal cartilage.

    Science.gov (United States)

    Youn, J I; Telenkov, S A; Kim, E; Bhavaraju, N C; Wong, B J; Valvano, J W; Milner, T E

    2000-01-01

    The aim of the study was to measure the spectral dependence of optical absorption and reduced scattering coefficients and thermal conductivity and diffusivity of porcine nasal septal cartilage. Values of optical and thermal properties determined in this study may aid in determining laser dosimetry and allow selection of an optical source wavelength for noninvasive diagnostics for laser-assisted reshaping of cartilage. The diffuse reflectance and transmittance of ex vivo porcine nasal septal cartilage were measured in the 400- to 1,400-nm spectral range by using a spectrophotometer. The reflectance and transmittance data were analyzed by using an inverse adding-doubling algorithm to obtain the absorption (mu(a)) and reduced scattering (mu(a)') coefficients. A multichannel thermal probe controller system and infrared imaging radiometer methods were applied to measure the thermal properties of cartilage. The multichannel thermal probe controller system was used as an invasive technique to measure thermal conductivity and diffusivity of cartilage at three temperatures (27, 37, 50 degrees C). An infrared imaging radiometer was used as a noninvasive method to measure the thermal diffusivity of cartilage by using a CO(2) laser source (lambda = 10.6 microm) and an infrared focal plane array (IR-FPA) camera. The optical absorption peaks at 980 nm and 1,180 nm in cartilage were observed and corresponded to known absorption bands of water. The determined reduced scattering coefficient gradually decreased at longer wavelengths. The thermal conductivity values of cartilage measured by using an invasive probe at 27, 37, and 50 degrees C were 4.78, 5.18, and 5.76 mW/cm degrees C, respectively. The corresponding thermal diffusivity values were 1.28, 1.31, and 1.40x 10(-3) cm(2)/sec. Because no statistically significant difference in thermal diffusivity values with increasing temperature is found, the average thermal diffusivity is 1.32 x 10(-3) cm(2)/sec. The numerical estimate

  4. Left ventricular long axis tissue Doppler systolic velocity is independently related to heart rate and body size.

    Science.gov (United States)

    Peverill, Roger E; Chou, Bon; Donelan, Lesley

    2017-01-01

    The physiological factors which affect left ventricular (LV) long-axis function are not fully defined. We investigated the relationships of resting heart rate and body size with the peak velocities and amplitudes of LV systolic and early diastolic long axis motion, and also with long-axis contraction duration. Two groups of adults free of cardiac disease underwent pulsed-wave tissue Doppler imaging at the septal and lateral mitral annular borders. Group 1 (n = 77) were healthy subjects heart rate, height or body surface area (BSA) for either LV wall in either group, but SDur was inversely correlated with heart rate for both walls and both groups, and after adjustment for heart rate, males in both groups had a shorter septal SDur. Septal and lateral s` were independently and positively correlated with SExc, heart rate and height in both groups, independent of sex and age. There were no correlations of heart rate, height or BSA with either e` or EDExc for either wall in either group. Heart rate and height independently modify the relationship between s` and SExc, but neither are related to EDExc or e`. These findings suggest that s` and SExc cannot be used interchangeably for the assessment of LV long-axis contraction.

  5. Genome-Wide Association Study of Down Syndrome-Associated Atrioventricular Septal Defects.

    Science.gov (United States)

    Ramachandran, Dhanya; Zeng, Zhen; Locke, Adam E; Mulle, Jennifer G; Bean, Lora J H; Rosser, Tracie C; Dooley, Kenneth J; Cua, Clifford L; Capone, George T; Reeves, Roger H; Maslen, Cheryl L; Cutler, David J; Feingold, Eleanor; Sherman, Stephanie L; Zwick, Michael E

    2015-07-20

    The goal of this study was to identify the contribution of common genetic variants to Down syndrome-associated atrioventricular septal defect, a severe heart abnormality. Compared with the euploid population, infants with Down syndrome, or trisomy 21, have a 2000-fold increased risk of presenting with atrioventricular septal defects. The cause of this increased risk remains elusive. Here we present data from the largest heart study conducted to date on a trisomic background by using a carefully characterized collection of individuals from extreme ends of the phenotypic spectrum. We performed a genome-wide association study using logistic regression analysis on 452 individuals with Down syndrome, consisting of 210 cases with complete atrioventricular septal defects and 242 controls with structurally normal hearts. No individual variant achieved genome-wide significance. We identified four disomic regions (1p36.3, 5p15.31, 8q22.3, and 17q22) and two trisomic regions on chromosome 21 (around PDXK and KCNJ6 genes) that merit further investigation in large replication studies. Our data show that a few common genetic variants of large effect size (odds ratio >2.0) do not account for the elevated risk of Down syndrome-associated atrioventricular septal defects. Instead, multiple variants of low-to-moderate effect sizes may contribute to this elevated risk, highlighting the complex genetic architecture of atrioventricular septal defects even in the highly susceptible Down syndrome population.

  6. Sinus Venosus Atrial Septal Defect Complicated by Eisenmenger Syndrome and the Role of Vasodilator Therapy

    Science.gov (United States)

    Anuwatworn, Amornpol; Bendaly, Edgard; Prescott-Focht, Julia A.; Clark, Richard; Jonsson, Orvar

    2016-01-01

    Sinus venosus atrial septal defect is a rare congenital, interatrial communication defect at the junction of the right atrium and the vena cava. It accounts for 5–10% of cases of all atrial septal defects. Due to the rare prevalence and anatomical complexity, diagnosing sinus venous atrial septal defects poses clinical challenges which may delay diagnosis and treatment. Advanced cardiac imaging studies are useful tools to diagnose this clinical entity and to delineate the anatomy and any associated communications. Surgical correction of the anomaly is the primary treatment. We discuss a 43-year-old Hispanic female patient who presented with dyspnea and hypoxia following a laparoscopic myomectomy. She had been diagnosed with peripartum cardiomyopathy nine years ago at another hospital. Transesophageal echocardiography and computed tomographic angiography of the chest confirmed a diagnosis of sinus venosus atrial septal defect. She was also found to have pulmonary arterial hypertension and Eisenmenger syndrome. During a hemodynamic study, she responded to vasodilator and she was treated with Ambrisentan and Tadalafil. After six months, her symptoms improved and her pulmonary arterial hypertension decreased. We also observed progressive reversal of the right-to-left shunt. This case illustrates the potential benefit of vasodilator therapy in reversing Eisenmenger physiology, which may lead to surgical repair of the atrial septal defect as the primary treatment. PMID:27974976

  7. Sinus Venosus Atrial Septal Defect Complicated by Eisenmenger Syndrome and the Role of Vasodilator Therapy

    Directory of Open Access Journals (Sweden)

    Amornpol Anuwatworn

    2016-01-01

    Full Text Available Sinus venosus atrial septal defect is a rare congenital, interatrial communication defect at the junction of the right atrium and the vena cava. It accounts for 5–10% of cases of all atrial septal defects. Due to the rare prevalence and anatomical complexity, diagnosing sinus venous atrial septal defects poses clinical challenges which may delay diagnosis and treatment. Advanced cardiac imaging studies are useful tools to diagnose this clinical entity and to delineate the anatomy and any associated communications. Surgical correction of the anomaly is the primary treatment. We discuss a 43-year-old Hispanic female patient who presented with dyspnea and hypoxia following a laparoscopic myomectomy. She had been diagnosed with peripartum cardiomyopathy nine years ago at another hospital. Transesophageal echocardiography and computed tomographic angiography of the chest confirmed a diagnosis of sinus venosus atrial septal defect. She was also found to have pulmonary arterial hypertension and Eisenmenger syndrome. During a hemodynamic study, she responded to vasodilator and she was treated with Ambrisentan and Tadalafil. After six months, her symptoms improved and her pulmonary arterial hypertension decreased. We also observed progressive reversal of the right-to-left shunt. This case illustrates the potential benefit of vasodilator therapy in reversing Eisenmenger physiology, which may lead to surgical repair of the atrial septal defect as the primary treatment.

  8. [Unusual muscular involvement in ankylosing spondylitis].

    Science.gov (United States)

    Wattiaux, M J; Rondier, J; Bletry, O; Godeau, P; Cayla, J

    1985-03-01

    Muscle involvement in ankylosing spondylitis has been little studied. The authors report two cases with marked muscular atrophy and functional impotence, which had directed the diagnosis towards a myopathy over a period of several years in the first case, and a suspected primary muscular disease associated with ankylosing spondylitis in the second. Muscle biopsies eliminated the diagnosis of myopathy in both cases, with rapid functional recovery with proper treatment. Following a review of the literature, two hypotheses can be considered to explain the muscular involvement in ankylosing spondylitis: one mechanism which appears well-established is a radiculitis with involvement of the paravertebral muscles: other authors suggest that there is nonspecific, generalized muscular involvement in this disorder.

  9. [Muscular strength development by electromagnetic stimulation].

    Science.gov (United States)

    Gorodnichev, R M; Beliaev, A G; Pivovarova, E A; Shliakhtov, V N

    2014-01-01

    A new tool for muscular strength development by electromagnetic stimulation (MS) of muscular during voluntary contraction has been described. 18 healthy subjects (men) took part in the research. They were devided into two groups--control (CG) and experimental (EG). Subjects of CG and EG have equal muscular strength parameters. M. gastrocnemius of subjects in EG was exposed to MS (1.8 T, 5 Hz) during training exercises (plantar foot flection). The subjects of CG did not receive MS. The torque of plantar foot flection of EG subjects increased significantly (24%) during 10 days training. The torque of plantar foot flection of CG subjects did not change significantly. We hypothesize increasing of muscular strength of EG subjects was result of high-threshold motor units activation under MS.

  10. Types of SMA (Spinal Muscular Atrophy)

    Science.gov (United States)

    ... genes other than the SMN1 gene. Spinal Muscular Atrophy Respiratory Distress (SMARD) SMARD is a very rare ... and 50. It causes muscle weakness and wasting (atrophy) throughout the body, which is most noticeable in ...

  11. Duchenne muscular dystrophy - a molecular service

    African Journals Online (AJOL)

    Duchenne muscular dystrophy using molecular technology was instituted at the ..... utilising non-fat dry milk for analysis of proteins and nucleic acids transferred ... acid to high specific activity in vitro by nick translation with DNA polymerase.

  12. Targeting latent TGFβ release in muscular dystrophy.

    Science.gov (United States)

    Ceco, Ermelinda; Bogdanovich, Sasha; Gardner, Brandon; Miller, Tamari; DeJesus, Adam; Earley, Judy U; Hadhazy, Michele; Smith, Lucas R; Barton, Elisabeth R; Molkentin, Jeffery D; McNally, Elizabeth M

    2014-10-22

    Latent transforming growth factor-β (TGFβ) binding proteins (LTBPs) bind to inactive TGFβ in the extracellular matrix. In mice, muscular dystrophy symptoms are intensified by a genetic polymorphism that changes the hinge region of LTBP, leading to increased proteolytic susceptibility and TGFβ release. We have found that the hinge region of human LTBP4 was also readily proteolysed and that proteolysis could be blocked by an antibody to the hinge region. Transgenic mice were generated to carry a bacterial artificial chromosome encoding the human LTBP4 gene. These transgenic mice displayed larger myofibers, increased damage after muscle injury, and enhanced TGFβ signaling. In the mdx mouse model of Duchenne muscular dystrophy, the human LTBP4 transgene exacerbated muscular dystrophy symptoms and resulted in weaker muscles with an increased inflammatory infiltrate and greater LTBP4 cleavage in vivo. Blocking LTBP4 cleavage may be a therapeutic strategy to reduce TGFβ release and activity and decrease inflammation and muscle damage in muscular dystrophy.

  13. Reality television and the muscular male ideal.

    Science.gov (United States)

    Dallesasse, Starla L; Kluck, Annette S

    2013-06-01

    Although researchers have examined the negative effects of viewing reality television (RTV) on women's body image, this research has not been extended to men. Exploring the extent to which RTV depicts men who embody the muscular ideal may enhance our understanding of the potential influence of this media genre. We explored the extent to which RTV depicted men who embodied the muscular ideal using a quantitative content analysis. Based on binomial tests, the primary male cast members of programs airing on networks popular among young adult men during the Fall 2009 broadcast season were more muscular, with lower levels of body fat, than average U.S. men. The chest-to-waist and shoulder-to-waist ratios of these cast members did not differ as a function of program type (i.e., reality drama, endurance, and romance). Young men who view RTV programs included in the present study would be exposed to an unrepresentative muscular ideal. Published by Elsevier Ltd.

  14. Ventilatory support in facioscapulohumeral muscular dystrophy.

    NARCIS (Netherlands)

    Wohlgemuth, M.; Kooi, E.L. van der; Kesteren, R.G. van; Maarel, S.M. van der; Padberg, G.W.A.M.

    2004-01-01

    Respiratory insufficiency due to respiratory muscle weakness is a common complication of many neuromuscular diseases. The prevalence of respiratory failure in facioscapulohumeral muscular dystrophy (FSHD) is unknown. The authors identified 10 FSHD patients on nocturnal ventilatory support at home,

  15. Brain MRI Findings in Congenital Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2006-03-01

    Full Text Available Brain magnetic resonance imaging (MRI findings in 13 patients with congenital muscular dystrophy (MDCIC and Fukutin-related protein (FKRP gene mutations were retrospectively reviewed in a study at Hammersmith Hospital, London, UK, and European centers.

  16. Anoctamin 5 muscular dystrophy in Denmark

    DEFF Research Database (Denmark)

    Witting, Nanna; Duno, Morten; Petri, Helle

    2013-01-01

    Since the initial description in 2010 of anoctamin 5 deficiency as a cause of muscular dystrophy, a handful of papers have described this disease in cases of mixed populations. We report the first large regional study and present data on new aspects of prevalence, muscular and cardiac phenotypic...... characteristics, and muscle protein expression. All patients in our neuromuscular unit with genetically unclassified, recessive limb girdle muscular dystrophy (LGMD2), Miyoshi-type distal myopathy (MMD) or persistent asymptomatic hyperCK-emia (PACK) were assessed for mutations in the ANO5 gene. Genetically...... confirmed patients were evaluated with muscular and cardiopulmonary examination. Among 40 unclassified patients (28 LGMD2, 5 MMD, 7 PACK), 20 were homozygous or compound heterozygous for ANO5 mutations, (13 LGMD2, 5 MMD, 2 PACK). Prevalence of ANO5 deficiency in Denmark was estimated at 1:100.000 and ANO5...

  17. Symbolic dynamics of ventricular tachycardia and ventricular fibrillation

    Science.gov (United States)

    Wang, Jun; Chen, Jie

    2010-05-01

    In this paper, the symbolic dynamics analysis was used to analyze the complexity of normal heartbeat signal (NSR), Ventricular tachycardia (VT) and ventricular fibrillation (VF) signals. By calculating the information entropy value of symbolic sequences, the complexities were quantified. Based on different information entropy values, NSR, VT and VF signals were distinguished with satisfactory results. The study showed that a sudden drop of symbolic sequence’s entropy value indicated that the patients most likely entered the episode of ventricular tachycardia and this was a crucial episode for the clinical treatment of patients. It had important clinical significance for the automatic diagnosis.

  18. Duchenne muscular dystrophy: the management of scoliosis

    Science.gov (United States)

    Gardner, Adrian C.; Roper, Helen P.; Chikermane, Ashish A.; Tatman, Andrew J.

    2016-01-01

    This study summaries the current management of scoliosis in patients with Duchenne Muscular Dystrophy. A literature review of Medline was performed and the collected articles critically appraised. This literature is discussed to give an overview of the current management of scoliosis within Duchenne Muscular Dystrophy. Importantly, improvements in respiratory care, the use of steroids and improving surgical techniques have allowed patients to maintain quality of life and improved life expectancy in this patient group.

  19. The superhealing MRL background improves muscular dystrophy

    OpenAIRE

    2012-01-01

    Abstract Background Mice from the MRL or “superhealing” strain have enhanced repair after acute injury to the skin, cornea, and heart. We now tested an admixture of the MRL genome and found that it altered the course of muscle pathology and cardiac function in a chronic disease model of skeletal and cardiac muscle. Mice lacking γ-sarcoglycan (Sgcg), a dystrophin-associated protein, develop muscular dystrophy and cardiomyopathy similar to their human counterparts with limb girdle muscular dyst...

  20. Minimizing ventricular pacing by a novel atrioventricular (AV) delay hysteresis algorithm in patients with intact or compromised intrinsic AV conduction and different atrial and ventricular lead locations.

    Science.gov (United States)

    Pakarinen, Sami; Toivonen, Lauri

    2013-09-01

    To investigate if an advanced AV search hysteresis (AVSH) algorithm, Ventricular Intrinsic Preference (VIP(™)), reduces the incidence of ventricular pacing (VP) in sinus node dysfunction (SND) with both intact and compromised AV conduction and with intermittent AV block regardless of the lead positions in the right atria and the ventricle. Patients were classified as having intact AV (AVi) conduction if the PR interval was ≤ 210 ms on ECG and 1:1 AV conduction during atrial pacing up to 120 bpm with PR interval ≤ 350 ms. Otherwise the AV conduction was classified as compromised (AVc). Both AVi and AVc patients were randomized to VIP ON or OFF. VIP performed an intrinsic AV conduction search every 30 s for three consecutive atrial cycles with the extension of the sensed and paced AV (SAV/PAV) delays from basic values of 150/200 ms to 300/350 ms. Extended AV intervals were allowed for three cycles when VP occurred before returning to basic AV delays. The primary end-point was %VP at 12 months. Among 389 patients, 30.1% had intact and 69.9% had compromised AV conduction. The mean %VP at 12 months was 9.6% by VIP compared to 51.8% with standard AV settings in patients with AVi (P < 0.0001) and 28.0% versus 78.9% (P < 0.0001) with AVc. With VIP, excessive %VP among most used lead positions was not seen. Conversely, when VIP was off %VP was low only in patients who had leads in the RA septal-RV septal position (23.0%). VIP feature reduces VP both in patients with SND and with intermittent heart block regardless of the lead positions in the right atria and the ventricle.