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Sample records for atrial septal defect

  1. Atrial Septal Defect (For Parents)

    Science.gov (United States)

    ... location, and severity of the defect. Very small ASDs might not need any treatment. In other cases, the cardiologist may recommend follow-up visits for observation. Usually, though, if an ASD hasn't closed on its own by the ...

  2. Atrial septal defects in Florida panthers.

    Science.gov (United States)

    Cunningham, M W; Dunbar, M R; Buergelt, C D; Homer, B L; Roelke-Parker, M E; Taylor, S K; King, R; Citino, S B; Glass, C

    1999-07-01

    Ostium secundum atrial septal defects (ASDs) were observed in six (3 M, 3 F) of 33 (20 M, 13 F) (18%) Florida panthers (Puma concolor coryi) necropsied by veterinary pathologists between 1985 and 1998. A seventh ASD was found in a female panther necropsied in the field and is included in the pathological description but not the prevalence of ASDs in Florida panthers. One panther (FP205) with severe ASD also had tricuspid valve dysplasia (TVD). Atrial septal defects and/or TVD are believed to have caused or contributed to the deaths of three (9%) Florida panthers in this study. Mean diameter +/- SD of ASDs was 9.0 +/- 4.7 mm (range 3 to 15 mm). Gross pathological changes attributed to ASDs/TVD in severely affected panthers (ASD > or = 10 mm) (n = 4) included mild right ventricular dilatation (n = 3) and hypertrophy (n = 2), mild to severe right atrial dilatation (n = 2), and acute pulmonary edema (n = 3). Panthers with mild ASDs (ASD < or = 5 mm) (n = 3) had no other detectable gross pathological changes associated with the ASDs. Histological examination of lungs of three panthers with severe ASDs revealed mild to moderate dilatation with fibrosis and smooth muscle atrophy of the tunica media of medium to large caliber arteries (n = 2), interstitial and/or pleural fibrosis (n = 2), perivascular fibrosis (n = 1), and acute to chronic edema (n = 3). Twenty-six necropsied panthers were examined one or more times while living; medical records were retrospectively evaluated. Antemortem radiographic, electrocardiographic, and echocardiographic examinations were performed on two panthers with severe ASDs (FP20 and FP205). Thoracic radiographic abnormalities in both included right heart enlargement, and in FP205 (severe ASD and TVD), mild pulmonary overperfusion. Electrocardiographic examination of FP205 revealed a right ventricular hypertrophy pattern, while FP205 had a normal electrocardiogram. Echocardiographic examination of FP20 revealed marked right atrial dilatation

  3. Atrial septal defects: Pattern, clinical profile, surgical techniques and ...

    African Journals Online (AJOL)

    Background: Atrial septal defect (ASD) is a congenital heart defect that leads to shunting of blood between left and right atria. It may be asymptomatic and sometimes may present with heart failure. Surgical repair is definitive, but currently non-surgical procedure is used to close the defect. Materials and Methods: It is a ...

  4. 50. Successful percutanous closure of spiral atrial septal defect

    Directory of Open Access Journals (Sweden)

    Mashail Abdulaziz Alobaidan

    2015-10-01

    An unusual morphology of atrial septal defect has been described where there is an apparently “double atrial septum” (Roberson, 2006. The terminology around this lesion has been attributed to be the wide separation of the primary atrial septum (primum septum from the secondary septum (septum secundum and the “spiral” spatial arrangement of the margins of the atrial septal defect (ASD has led to the term spiral ASD to describe this arrangement. This has been described to be associated with a high risk of device embolization or technical failure in the placement of an occluder device. We report the echocardiographic findings and outcome of a patient with this form of ASD in whom percutaneous occlusion was successful of which is considered up to date to be the first successful closure of this type of ASD.

  5. Three-dimensional transesophageal echocardiography of the atrial septal defects

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    Romero-Cárdenas Ángel

    2008-07-01

    Full Text Available Abstract Transesophageal echocardiography has advantages over transthoracic technique in defining morphology of atrial structures. Even though real time three-dimensional echocardiographic imaging is a reality, the off-line reconstruction technique usually allows to obtain higher spatial resolution images. The purpose of this study was to explore the accuracy of off-line three-dimensional transesophageal echocardiography in a spectrum of atrial septal defects by comparing them with representative anatomic specimens.

  6. Three-dimensional transesophageal echocardiography of the atrial septal defects

    Science.gov (United States)

    Roldán, Francisco-Javier; Vargas-Barrón, Jesús; Vázquez-Antona, Clara; Castellanos, Luis Muñoz; Erdmenger-Orellana, Julio; Romero-Cárdenas, Ángel; Martínez-Ríos, Marco-Antonio

    2008-01-01

    Transesophageal echocardiography has advantages over transthoracic technique in defining morphology of atrial structures. Even though real time three-dimensional echocardiographic imaging is a reality, the off-line reconstruction technique usually allows to obtain higher spatial resolution images. The purpose of this study was to explore the accuracy of off-line three-dimensional transesophageal echocardiography in a spectrum of atrial septal defects by comparing them with representative anatomic specimens. PMID:18638394

  7. Pulmonary edema following transcatheter closure of atrial septal defect

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    Keerthi Chigurupati

    2015-01-01

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

  8. Three-dimensional echocardiographic assessment of atrial septal defects

    Science.gov (United States)

    German, Charles; Nanda, Navin C.

    2015-01-01

    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. PMID:25566714

  9. Three-dimensional echocardiographic assessment of atrial septal defects

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    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.

  10. Recent advances in managing septal defects: atrial septal defects [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    P Syamasundar Rao

    2017-11-01

    Full Text Available The purpose of this review is to discuss the management of atrial septal defects (ASD, paying particular attention to the most recent developments. There are four types of ASDs: ostium secundum, ostium primum, sinus venosus, and coronary sinus defects. The fifth type, patent foramen ovale—which is present in 25 to 30% of normal individuals and considered a normal variant, although it may be the seat of paradoxical embolism, particularly in adults—is not addressed in this review. The indication for closure of the ASDs, by and large, is the presence of right ventricular volume overload. In asymptomatic patients, the closure is usually performed at four to five years of age. While there was some earlier controversy regarding ASD closure in adult patients, currently it is recommended that the ASD be closed at the time of presentation. Each of the four defects is briefly described followed by presentation of management, whether by surgical or percutaneous approach, as the case may be. Of the four types of ASDs, only the ostium secundum defect is amenable to percutaneous occlusion. For ostium secundum defects, transcatheter closure has been shown to be as effective as surgical closure but with the added benefits of decreased hospital stay, avoidance of a sternotomy, lower cost, and more rapid recovery. There are several FDA-approved devices in use today for percutaneous closure, including the Amplatzer® Septal Occluder (ASO, Amplatzer® Cribriform device, and Gore HELEX® device. The ASO is most commonly used for ostium secundum ASDs, the Gore HELEX® is useful for small to medium-sized defects, and the cribriform device is utilized for fenestrated ASDs. The remaining types of ASDs usually require surgical correction. All of the available treatment modes are safe and effective and prevent the development of further cardiac complications.

  11. Tetralogy of Fallot and atrial septal defect in a white Bengal Tiger cub (Panthera tigris tigris).

    Science.gov (United States)

    Pazzi, Paolo; Lim, Chee K; Steyl, Johan

    2014-03-04

    A 3-week-old female white Bengal Tiger cub (Panthera tigris tigris) presented with acute onset tachypnoea, cyanosis and hypothermia. The cub was severely hypoxaemic with a mixed acid-base disturbance. Echocardiography revealed severe pulmonic stenosis, right ventricular hypertrophy, high membranous ventricular septal defect and an overriding aorta. Additionally, an atrial septal defect was found on necropsy, resulting in the final diagnosis of Tetralogy of Fallot with an atrial septal defect (a subclass of Pentalogy of Fallot). This report is the first to encompass arterial blood gas analysis, thoracic radiographs, echocardiography and necropsy findings in a white Bengal Tiger cub diagnosed with Tetralogy of Fallot with an atrial septal defect.

  12. Tetralogy of Fallot and atrial septal defect in a white Bengal Tiger cub (Panthera tigris tigris)

    OpenAIRE

    Pazzi, Paolo; Lim, Chee K; Steyl, Johan

    2014-01-01

    A 3-week-old female white Bengal Tiger cub (Panthera tigris tigris) presented with acute onset tachypnoea, cyanosis and hypothermia. The cub was severely hypoxaemic with a mixed acid–base disturbance. Echocardiography revealed severe pulmonic stenosis, right ventricular hypertrophy, high membranous ventricular septal defect and an overriding aorta. Additionally, an atrial septal defect was found on necropsy, resulting in the final diagnosis of Tetralogy of Fallot with an atrial septal defect ...

  13. 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. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  14. Clinically silent atrial septal defects with evidence for cerebral embolization.

    Science.gov (United States)

    Harvey, J R; Teague, S M; Anderson, J L; Voyles, W F; Thadani, U

    1986-11-01

    The cause of stroke in young patients frequently cannot be established. Eleven consecutive patients, age 50 and younger, had clinical evidence of cerebral embolization. Results of physical, radiographic, electrocardiographic, and two-dimensional echocardiographic examinations were normal in all patients. During normal respiration, eight of the patients had right-to-left shunts at the atrial level shown by microcavitation contrast two-dimensional echocardiography. Six of the eight patients with positive contrast studies had cardiac catheterization. Five of six patients had an atrial septal defect, normal right and left heart pressures, and small right-to-left shunts during a Valsalva strain. Four patients had surgical closure of the defect, which ranged in size from 5 to 10 mm. The remaining patients received anticoagulants. Interatrial communications appear to be common in young patients with stroke, suggesting paradoxical embolization as a possible mechanism. Contrast two-dimensional echocardiography should be done in such patients because it is the only noninvasive technique that reliably finds these defects.

  15. Klippel-Feil syndrome associated with atrial septal defect.

    Science.gov (United States)

    Bejiqi, Ramush; Retkoceri, Ragip; Bejiqi, Hana; Zeka, Naim; Maloku, Arlinda; Berisha, Majlinda

    2013-01-01

    Three major features result from this abnormality: a short neck, a limited range of motion in the neck, and a low hairline at the back of the head. Most affected people have one or two of these characteristic features. Less than half of all individuals with Klippel-Feil syndrome have all three classic features of this condition. The etiology of Klippel-Feil syndrome and its associated conditions is unknown. The syndrome can present with a variety of other clinical syndromes, including fetal alcohol syndrome, Goldenhar syndrome, anomalies of the extremities etc. Associated anomalies occur in the auditory system, neural axis, cardiovascular system, and the musculoskeletal system. Cardiovascular anomalies, mainly septal defects, were found in 7 patients in Hensinger's series, with 4 of these individuals requiring corrective surgery. In our case we have had registered a nonrestrictive atrial septal defect and corrective surgical intervention at age 18 months in the Santa Rosa Children's Hospital (USA) has been done successfully. Careful examinations of specialist exclude anomalies in other organs and systems. Radiographs and MRI of the thoracic and lumbosacral spine are obtained and other anomalies have been excluded.

  16. [A case of tricuspid pouch associated with ventricular septal defect and functional left ventricular-right atrial communication].

    Science.gov (United States)

    Watanabe, H; Maeda, K; Minowa, T; Shimasaki, T; Shimanuki, T; Kasuya, S; Sakashita, I; Takano, S; Kato, K

    1992-03-01

    We reported a 60-year-old woman with tricuspid pouch associated with ventricular septal defect and subsequent left ventricular-right atrial communication. Preoperative angiographic findings suggested the presence of membranous septal aneurysm, ventricular and atrial septal defects, and tricuspid insufficiency. However, at operation, besides perimembranous inlet type ventricular septal defect, a pouch, 1.5 cm in diameter was found in the adjacent part to the septal leaflet within the anterior one of the tricuspid valve. The ventricular septal defect was closed with a patch. From these findings, it is speculated that the tricuspid pouch was formed by the effect of jet stream through the ventricular septal defect.

  17. First successful totally endoscopic atrial septal defect closure with robotic assistance

    Directory of Open Access Journals (Sweden)

    А. Н. Архипов

    2015-10-01

    Full Text Available Median sternotomy has long been a conventional surgical approach in patients with atrial septal defects. However, with improvements in surgical techniques and introduction of new technologies, minimally invasive procedures are gaining more and more attention of surgeons managing congenital heart diseases. In this paper we introduce a case of successful totally thoracoscopic atrial septal defect closure performed by using the da Vinci surgical system in a 25-year old female.

  18. 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

    2012-01-01

    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. PMID:22200951

  19. Long-term mortality in patients with atrial septal defect

    DEFF Research Database (Denmark)

    Nyboe, Camilla; Karunanithi, Zarmiga; Nielsen-Kudsk, Jens Erik

    2017-01-01

    Aims: In this nationwide cohort of atrial septal defect (ASD) patients, the largest to date, we report the longest follow-up time with and without closure in childhood and adulthood compared with a general population cohort. Methods and results: Using population-based registries, we included Danish...... of ASD patients with that of a birth year and sex matched general population cohort. The median follow-up from ASD diagnosis was 18.1 years (range 1-53 years). Patients with ASD had a higher mortality [adjusted hazard ratio (HR): 1.7; 95% confidence interval (CI): 1.5-1.9] compared with the general...... population cohort. The adjusted HR 30 days after closure was 1.4 (95% CI: 1.2-1.7), and it was 2.4 (95% CI: 2.0-2.9) for patients without closure. Conclusion: Overall, ASD patients had a higher long-term mortality than a general population cohort matched on birth year and gender. Our data indicate a lower...

  20. Robotic-assisted endoscopic atrial septal defect closure: analysis of 115 cases in a single center.

    Science.gov (United States)

    Yang, Ming; Gao, Changqing; Xiao, Cangsong

    2012-06-01

    To summarize the experience with the application of robotic technique in totally endoscopic atrial septal defect closure in a single center. Between January 2007 and September 2011, 115 patients with the diagnosis of secundum type atrial septal defects underwent robotic atrial septal defect repair with the assistance of da Vinci surgical system. The patients had a median age of 35 years and a median defect diameter of 28 mm. Cardiopulmonary bypass was established via peripheral cannulation. Via three 8-mm ports and one 15-mm port in the right chest, the surgeon manipulated the microinstruments to complete the defect closure with or without tricuspid valve plasty. Echocardiography was performed intraoperatively, before discharge and at 30 days after the operation. Atrial septal defect closure was completed on arrested heart in 44 patients and on beating heart in 61 patients. No deaths or conversions to alternate techniques occurred in these cases. No residual shunt was detected by intraoperative or postoperative echocardiography. The mean operating time and cardiopulmonary bypass time on bearing heart group were significantly shorter than those on arrested heart group. The median ventilation time, intensive care unit stay, drainage volume, or length of hospital stay showed no significant differences between the two groups. Secundum type atrial septal defect closure can be successfully performed with the assistance of the robotic system with good surgical results.

  1. Sinus Venosus Atrial Septal Defect Complicated by Eisenmenger Syndrome and the Role of Vasodilator Therapy

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    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.

  2. Iatrogenic intra-atrial macro-reenterant tachycardia following transcatheter closure of atrial septal defect treated by radiofrequency ablation

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    Ibrahim Marai

    2011-01-01

    Full Text Available Percutaneous closure of an atrial septal defect (ASD has been established as a safe and effective alternative to surgical management. We describe a case of a 41-year-old patient in whom an Amplatzer septal occluder device was used to close a moderately large ASD and who subsequently developed incessant intra-atrial macro-reenterant tachycardia. The tachycardia was terminated by radiofrequency ablation guided by electroanatomical mapping.

  3. Successful closure of atrial septal defect by retrograde transarterial approach after unsuccessful transfemoral venous approach

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    Sadık Volkan Emren

    2016-06-01

    Full Text Available Although many of the ostium secundum atrial septal defects can be successfully closed by percutaneous transvenous approach, surgical method as well is preferred in some cases due to vascular reasons or due to the anatomical characteristics of the defect. Detecting ostium secundum atrial septal defect in a 26-year-old female patient, who presented with shortness of breath, percutaneous closure procedure was planned. During percutaneous closure, it was observed that guidewire and catheter persistently went towards patent foramen ovale because of quite flaccid structure of interatrial septum. For this reason, occluder could not be placed in the defect area. Before using the option of surgery for the patient, guidewire and simmons 1 diagnostic catheter were pushed forward towards to the left ventricle and then left atrium by transarterial approach through the femoral artery. Guidewire and the catheter were easily passed through atrial septal defect without passing through patent foramen ovale benefiting from the left atrial pressure. Occluder was retrogradely carried via transarterial route and successfully implanted in the defect area. Transarterial approach may be an alternative before surgery in selected cases in which percutaneous closure of atrial septal defect has failed.

  4. Anatomy of a wrong diagnosis: false Sinus Venosus Atrial Septal Defect

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    Montresor Graziano

    2003-11-01

    Full Text Available Abstract In contrast with transthoracic echocardiography, transesophageal echocardiography provides a sure way to make the diagnosis of sinus venosus atrial septal defect; on the other hand this abnormality is more complex than that seen with the secundum atrial septal defect, and inexperienced operators may fail to recognize properly the defect. In front of a high reported sensitivity using transesophageal echocardiography, specificity is difficult to assess, due to possible underreporting of diagnostic errors. We describe a false positive diagnosis of sinus venosus atrial septal defect, in the setting of enlarged right chambers of the heart because of pressure overload. Modified anatomy of the heart, together with the presence of a prominent linear structure(probably Eustachian Valve and an incomplete examination in this case made image interpretation very prone to misinterpretation. In this anatomical setting transesophageal longitudinal "bicaval" view may be sub-optimal for examining the atrial septum, potentially showing false images that need to be known for correct image interpretation. Nonetheless, a scan plane taken more accurately at the superior level would have demonstrated/excluded the pathognomonic feature of sinus venosus atrial septal defect in the high atrial septum, between the fatty limbus and the inferior aspect of the right pulmonary artery; moreover TEE allows morphological information about the posterior structures of the heart that need to be investigated in detail for a complete diagnosis.

  5. [A case of subpulmonary membranous stenosis associated with atrial septal defect].

    Science.gov (United States)

    Ono, Y; Momokawa, T; Shuto, K; Munakata, M; Suzuki, S; Koie, H

    1993-09-01

    The patient was a 5 years old male who had had the cyanosis and congestive heart failure from his neonatal period. Dopamine, digitalis and diuretics disappeared his symptoms and he had been followed up as the out-patient. Preoperative cardiac catheterization revealed atrial septal defect and moderately pulmonary stenosis with two-staged systolic pressure gradient in a right ventricular cavity. Right ventriculogram showed subpulmonary crescent-shaped, linear filling defect. Ventricular septal defect was not detected. He was underwent open heart surgery and subpulmonary membranous stenosis was found out. Pressure gradient across the right ventricular outflow tract was diminished by the resection of the membranous structure. Atrial septal defect without lower margin was closed directly. Postoperative course was uneventful. Right ventricular apical systolic pressure was decreased to the degree of 27 mmHg postoperatively.

  6. Nickel hypersensitivity following closure of atrial septal defect: A case report and review of the literature.

    Science.gov (United States)

    Dickison, Philippa; Harris, Victoria; Smith, Saxon D

    2018-01-29

    We present an unusual case where symptoms of headache and chest pain persisted for 3 years following the implantation of a septal occluder device for an atrial septal defect despite endothelialisation of the device. The patient was found to have nickel hypersensitivity on patch testing. Following the removal of the device the patient had complete resolution of headaches and chest pain up to 10 months post-explantation. © 2018 The Australasian College of Dermatologists.

  7. The role of cardiac MRI in the diagnosis and management of sinus venosus atrial septal defect

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    Madhusudan Ganigara

    2014-01-01

    Full Text Available Sinus venosus atrial septal defects (SV-ASDs are inter-atrial communications caused by a deficiency of the common wall between the superior or inferior vena cava and the right-sided pulmonary veins. The diagnosis can be challenging, especially in adults with delayed presentation. We present images that illustrate an example of the role of cardiac magnetic resonance imaging (CMRI in the diagnosis and follow-up of a patient with SV-ASD.

  8. Surgical Outcomes of Congenital Atrial Septal Defect Using da VinciTM Surgical Robot System.

    Science.gov (United States)

    Kim, Ji Eon; Jung, Sung-Ho; Kim, Gwan Sic; Kim, Joon Bum; Choo, Suk Jung; Chung, Cheol Hyun; Lee, Jae Won

    2013-04-01

    Minimally invasive cardiac surgery has emerged as an alternative to conventional open surgery. This report reviews our experience with atrial septal defect using the da VinciTM surgical robot system. This retrospective study included 50 consecutive patients who underwent atrial septal defect repair using the da VinciTM surgical robot system between October 2007 and May 2011. Among these, 13 patients (26%) were approached through a totally endoscopic approach and the others by mini-thoracotomy. Nineteen patients had concomitant procedures including tricuspid annuloplasty (n=10), mitral valvuloplasty (n=9), and maze procedure (n=4). The mean follow-up duration was 16.9±10.4 months. No remnant interatrial shunt was detected by intraoperative or postoperative echocardiography. The atrial septal defects were mainly repaired by Gore-Tex patch closure (80%). There was no operative mortality or serious surgical complications. The aortic cross clamping time and cardiopulmonary bypass time were 74.1±32.2 and 157.6±49.7 minutes, respectively. The postoperative hospital stay was 5.5±3.3 days. The atrial septal defect repair with concomitant procedures like mitral valve repair or tricuspid valve repair using the da VinciTM system is a feasible method. In addition, in selected patients, complete port access can be helpful for better cosmetic results and less musculoskeletal injury.

  9. Percutaneous closure of congenital aortocaval fistula with a coexisting secundum atrial septal defect

    DEFF Research Database (Denmark)

    Loh, Poay Huan; Jensen, Tim; Søndergaard, Lars

    2012-01-01

    Congenital aortocaval fistula is a very rare anomaly. Clinically, it resembles conditions that cause left-to-right shunt of blood. We report a case of such anomaly in combination with a secundum atrial septal defect in a 13-month-old girl who presented with failure to thrive and exertional respir...

  10. Concordant familial segregation of atrial septal defect and Axenfeld-Rieger anomaly in father and son.

    Science.gov (United States)

    Calcagni, Giulio; Digilio, Maria Cristina; Capolino, Rossella; Dallapiccola, Bruno; Marino, Bruno

    2006-10-01

    The association of congenital heart defect and ocular malformations is involved in several genetic syndromes, metabolic diseases and environmental entities. We report here on father and son, both presenting with the combination of atrial septal defect and congenital ocular anomalies in Axenfeld-Rieger anomaly. The son had anterior iridotrabecular dysgenesis and posterior embryotoxon bilaterally, corneal leucoma and marked iridial vascularization at right. The father had bilateral anterior iridotrabecular dysgenesis, posterior embryotoxon and nystagmus, and corneal leucoma at left. No additional malformations were noted in these patients. The Axenfeld-Rieger syndrome seems to be a spectrum of developmental disorders. The present report confirms the existence of a specific Axenfeld-Rieger phenotype associated with congenital heart defect. Atrial septal defect is the anatomic type of congenital heart defect linked to this condition.

  11. Prolonged postoperative desaturation in a child with Down syndrome and atrial septal defect

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    Renu Sinha

    2011-01-01

    Full Text Available We report prolonged desaturation in a child with Down syndrome (DS and atrial septal defect due to undiagnosed interstitial lung disease. An 18-month-old child with DS was scheduled for bilateral lens aspiration for cataract. The child had atrial septal defect and hypothyroidism. He also had delayed milestones and hypotonia with episodes of recurrent respiratory tract infection necessitating repeated hospitalization. Preoperative evaluation was unremarkable. General anaesthesia and controlled ventilation using proseal laryngeal mask airway was instituted. He had uneventful intraoperative period. In the postoperative period, the child had desaturation 1 hour after surgery on discontinuation of oxygen supplementation by face mask, which improved with oxygen therapy. Supplemental oxygen via face mask was continued and weaned off over several days. On further evaluation, the child was diagnosed as having interstitial lung disease. He improved and discharged from the hospital 15 days after the surgery with room air saturation of 90%.

  12. Device closure in adults with atrial septal defect in Shiraz, a single center registry

    OpenAIRE

    Ostovan, Mohammad Ali; Kojuri, Javad; Dehghani, Pooyan; Razazi, Vida; Moarref, Alireza

    2016-01-01

    Introduction: Successful closure of atrial septal defect (ASD) improves patients? functional class and exercise capacity. In this study we evaluate the safety and feasibility of percutaneous device closure of ASDs. Methods: Two hundred fifty six patients with significant ASD according to our criteria were enrolled. The patients were treated using nitinol wire mesh transcatheter devices. Complications were followed for a median of 2.5 years. Results: Success rate was 98.4% with 3 unsuccessful ...

  13. 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

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    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.

  14. Evaluation of morphological characteristics of septal rims affecting successful transcatheter atrial septal defect closure in children and adults.

    Science.gov (United States)

    Oflaz, Mehmet Burhan; Pac, Feyza Aysenur; Kibar, Ayse Esin; Balli, Sevket; Ece, Ibrahim

    2013-01-01

    Determining other echocardiographic predictors along with the measured atrial septal defect (ASD) size and evaluating the closure together with these predictors would increase the chance of success for transcatheter closure of ASD. To evaluate echocardiographic parameters affecting defect closure in children and adult patients with secundum ASD. In all patients, size of ASD, total length of atrial septum (TS), superior-posterior, inferior-posterior, superior-anterior and inferior-anterior rims surrounding the defect were measured by transesophageal echocardiography (TEE), and several measurement ratios were derived on the basis of TEE parameters. A total 216 patients with secundum ASD were included in this study. The device was successfully implanted in 65 children and 65 adults. Both in pediatric and adult cases, the ratio of successful closure was found to be significantly higher when the ratio of defect size to TS was ≤ 0.35, the ratio of superior-anterior (SA) rim to the defect size was > 0.75 and the ratio of inferior-posterior (IP) rim to the defect size was > 1.0. It was found that having more than one of these predictors in a single case increased the chance of closure success significantly (p rim to defect size > 0.75 and a ratio of IP rim to defect size > 1.0 were found to be echocardiographic predictors that could be used in successful transcatheter ASD closure both in children and adults.

  15. Deficient Surrounding Rims in Patients Undergoing Transcatheter Atrial Septal Defect Closure.

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    Kijima, Yasufumi; Akagi, Teiji; Takaya, Yoichi; Taniguchi, Manabu; Nakagawa, Koji; Kusano, Kengo; Sano, Shunji; Ito, Hiroshi

    2016-08-01

    The influence of deficient rims surrounding atrial septal defects (ASDs) in patients undergoing transcatheter closure has yet to be clarified. The aim of this study was to assess the influence of a deficient surrounding rim on the procedural success and clinical outcome of transcatheter ASD closure using an Amplatzer septal occluder. A total of 474 patients (mean age, 46 ± 22 years) with ostium secundum ASDs measuring ≤40 mm in diameter who had undergone attempted transcatheter closure using Amplatzer septal occluders from September 2007 to August 2013 were assessed. A comprehensive transesophageal echocardiographic examination was done to assess the morphologic characteristics of the defects in all patients. Subjects were classified into three groups by the extent and location of rim deficiency (rims (sufficient group, n = 101), patients with single deficient rims, (single group, n = 338), and patients with multiple rim deficiencies (multiple group, n = 35). There was a significant difference in the maximal defect diameter among the sufficient, single, and multiple groups (15 ± 6, 18 ± 6, and 29 ± 7 mm, respectively, P rim deficiencies as determined by transesophageal echocardiography, successful transcatheter ASD closure using Amplatzer septal occluders is more difficult to accomplish. However, if closure is successful, rim deficiencies rarely affect intermediate-term outcomes. Copyright © 2016 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

  16. Assessment of atrial septal defects in adults comparing cardiovascular magnetic resonance with transoesophageal echocardiography

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    Brown Michael A

    2010-07-01

    Full Text Available Abstract Background Many adult patients with secundum-type atrial septal defects (ASDs are able to have these defects fixed percutaneously. Traditionally, this has involved an assessment of ASD size, geometry and atrial septal margins by transoesophageal echocardiography (TOE prior to percutaneous closure. This is a semi-invasive technique, and all of the information obtained could potentially be obtained by non-invasive cardiovascular magnetic resonance (CMR. We compared the assessment of ASDs in consecutive patients being considered for percutaneous ASD closure using CMR and TOE. Methods Consecutive patients with ASDs diagnosed on transthoracic echocardiography (TTE were invited to undergo both CMR and TOE. Assessment of atrial septal margins, maximal and minimal defect dimensions was performed with both techniques. Analyses between CMR and TOE were made using simple linear regression and Bland Altman Analyses. Results Total CMR scan time was 20 minutes, and comparable to the TOE examination time. A total of 20 patients (M:F = 5:15, mean age 42.8 years ± 15.7 were included in the analyses. There was an excellent agreement between CMR and TOE for estimation of maximum defect size (R = 0.87. The anterior inferior, anterior superior and posterior inferior margins could be assessed in all patients with CMR. The posterior superior margin could not be assessed in only one patient. Furthermore, in 1 patient in whom TOE was unable to be performed, CMR was used to successfully direct percutaneous ASD closure. Conclusions CMR agrees with TOE assessment of ASDs in the work-up for percutaneous closure. Potentially CMR could be used instead of TOE for this purpose.

  17. Multicenter midterm follow-up results using the gore septal occluder for atrial septal defect closure in pediatric patients.

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    Grohmann, Jochen; Wildberg, Christian; Zartner, Peter; Abu-Tair, Tariq; Tarusinov, Gleb; Kitzmüller, Erwin; Schmoor, Claudia; Stiller, Brigitte; Kampmann, Christoph

    2017-06-01

    To assess the safety and efficacy of the Gore Septal Occluder (GSO) used for device-closure of significant secundum-type atrial septal defects (ASD II) focusing on pediatric patients. The GSO is a patch-like double disc device. Due to its design, it is assumed to be safe, even when implanted in ASDs with deficient retro-aortic rims. Multicenter retrospective analysis of consecutive children and adolescents with a GSO in situ for at least 12 months according to a 1- to 4-year midterm follow-up. Hundred and seventy three pediatric patients were enrolled. At implantation, median age was 6 years (range 0.7-17.9), median body weight and length were 21 kg (6.4-95) and 119 cm (65-193). Median follow-up period was 20 months (range 12-51). ASD anatomy was comprised of single defects in 131 patients (76%), multi-fenestrated defects in 42 (24%), and deficient retro-aortic rims in 33 (19%). Follow-up confirmed an overall closure-rate of 95.4%. Small residual shunts were reported in eight patients (4.6%) without need for any re-intervention. Complications were classified as minor events both during the initial procedure (9 patients, 5.2%) and on follow-up (another 9 patients), including transient AV block II in three patients (1.8%) and four snare-retrievals (2.4%) during the initial procedure. Periprocedural and midterm follow-up data have shown the GSO to be effective and safe for ASD device closure in children and adolescents. GSO may be considered the first-choice device in deficient retro-aortic rims and multi-fenestrated defects, when covering most of the atrial septum is necessary. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  18. Is Three-Dimensional Echocardiography Useful in Evaluation of Atrial Septal Defects?

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    Charles German

    2015-06-01

    Full Text Available Of all birth defects, Congenital Heart Disease (CHD remains the most prevalent. These malformations are largely multifactorial with both environmental and genetic components, but known chromosomal abnormalities and mutations of single genes account for less than 10% of all cardiac defects (1. They affect approximately 6 to 13 newborns per 1000 live births (2 and are made up of 5 major Atrial Septal Defects (ASDs, including primum and secundum type defects, sinus venosus and coronary sinus defects, and Patent Foramen Ovale (PFO. However, there is debate within the medical community regarding inclusion of PFOs and coronary sinus defects within the realm of CHD. PFOs do not have absent septal tissue, and coronary sinus defects, or unroofed coronary sinus, represent an abnormal communication between the superior portion of the coronary sinus and the neighboring left atrium. Regardless, these anomalies can go undetected at birth, particularly if asymptomatic. Though some defects may be innocuous at birth, the continued shunting of blood between the atria can lead to pulmonary hypertension, heart failure, and even death. Thus, finding an accurate and reliable means of diagnosis via echocardiography is necessary in establishing the optimal treatment and ultimately improving patient mortality.

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

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    S Das

    2016-01-01

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

  20. Sinus venosus atrial septal defect: a rare cause of misplacement of pacemaker leads

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    Bodian M

    2013-07-01

    Full Text Available Malick Bodian,1 Fatou Aw,1 Mouhamadou Bamba Ndiaye,1 Adama Kane,1 Modou Jobe,1 Alioune Tabane,1 Alassane Mbaye,2 Simon Antoine Sarr,1 Maboury Diao,1 Moustapha Sarr,1 Serigne Abdou Bâ1 1Department of Cardiology, Aristide Le Dantec Teaching Hospital, 2Grand Yoff General Hospital, Dakar, Senegal Abstract: Routine implantation of pacemakers and implantable cardioverter defibrillators is not commonly associated with complications. However, in some cases we see misplacement of pacemaker leads which is most often related to the presence of underlying cardiac anomalies. We report the case of misplacement of a pacemaker lead into the left ventricle of a 56-year-old patient paced in VVI/R mode and with a tined type pacemaker lead because of a symptomatic complete atrioventricular block. Electrocardiogram showed a pacemaker-generated rhythm with a right bundle branch block pattern. Chest X-ray showed the pacemaker lead located relatively high in relation to the diaphragm. Echocardiography visualized the pacemaker lead in the left heart chambers (atrium and ventricle, hence confirming its aberrant course. Further, the defect causing its passage to the left heart chambers was a sinus venosus atrial septal defect. The patient reported no complication related to the misplacement of the lead. After a brief period of oral anticoagulation, the lead was inserted into the right ventricle by percutaneous technique. Keywords: pacemaker, lead misplacement, sinus venosus atrial septal defect

  1. Current concept of transcatheter closure of atrial septal defect in adults.

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    Akagi, Teiji

    2015-01-01

    After the introduction of catheter intervention for atrial septal defect (ASD) in the pediatric population, therapeutic advantages of this less invasive procedure were focused on adult through geriatric populations. The most valuable clinical benefits of this procedure are the significant improvement of symptoms and daily activities, which result from the closure of left to right shunt without thoracotomy and cardiopulmonary bypass surgery. These benefits contribute to increase the number of adult patients of this condition who have hesitated over surgical closure. In terms of technical point of view for catheter closure of ASD, the difficulties still exist in some morphological features of defect, or hemodynamic features in the adult population. Morphological features of difficult ASD closure are (1) large (≥30 mm) ASD, (2) wide rim deficiency, and (3) multiple defects. Hemodynamic features of difficult ASD are (1) severe pulmonary hypertension, (2) ventricular dysfunction, and (3) restrictive left ventricular compliance (diastolic dysfunction) after ASD closure. To complete the catheter ASD closure under these difficult conditions, various procedural techniques have been introduced. These are new imaging modalities such as real-time three-dimensional imaging, new technical modifications, and new concepts for hemodynamic evaluation. Especially, real-time three-dimensional transesophageal echocardiography can provide the high quality imaging for anatomical evaluation including maximum defect size, surrounding rim morphology, and the relationship between device and septal rim. In adult patients, optimal management for their comorbidities is an important issue, which includes cardiac function, atrial arrhythmias, respiratory function, and renal function. Management of atrial arrhythmias is a key issue for the long-term outcome in adult patients. Because the interventional procedures are not complication-free techniques, the establishment of a surgical back

  2. Value of the Ratio of Occluder Versus Atrial Septal Length for Predicting Arrhythmia Occurrence after Transcatheter Closure in Children with Ostium Secundum Atrial Septal Defect

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    Mei Jin

    2015-01-01

    Full Text Available Background: Transcatheter occlusion has been applied to treat ostium secundum atrial septal defect (OS ASD since 1997. During the clinical practice, several postoperative complications including arrhythmia have been reported. This study aimed to evaluate the value of the ratio of atrial septal occluder (ASO versus atrial septal length (ASL for predicting arrhythmia occurrence after transcatheter closure in children with OS ASD. Methods: Six hundred and fifty-one children diagnosed with OS ASD underwent occlusion procedures after completing routine examinations. The onsets and types of arrhythmia both during and after the occlusion procedures were monitored. Treatments were given based on the individual types of arrhythmia. The binary logistic regression analysis and receiver-operating characteristic (ROC curve were used in the analysis of value of the ratio of ASO/ASL for predicting postoperative arrhythmia occurrence. Results: Transcather occlusions were conducted in 651 children, among whom 7 children had different types and degrees of arrhythmia, with an incidence of 1.1%. The types of arrhythmia included sinus bradycardia, atrial premature beats, bundle branch block, and different degrees of atrioventricular block. Normal electrocardiograph findings were resumed in these 7 patients following active therapies such as corticoids, nutrition, and surgeries. The binary logistic regression and ROC analysis suggested that the ratio of ASO/ASL exhibited an intermediate predictive value for predicting arrhythmia occurrence after occlusion procedures. A cut-off value of 0.576 in the ratio provided a sensitivity of 87.5% and a specificity of 76.2% with an area under the ROC curve of 0.791 (95% confidence intervals, 0.655-0.926; P < 0.05 in predicting arrhythmia occurrence after the closure procedures. Conclusions: The ratio of ASO/ASL might be a useful index for predicting arrhythmia occurrence after closure procedures in children with OS ASD.

  3. 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.

  4. Single Centre Experience for Percutaneous Closure of Secundum Atrial Septal Defect

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    Ramazan Aydemir

    2011-12-01

    Full Text Available Introduction: Atrial septal defect (ASD is the most common congenital heart disease in adults and constitutes 5-10% of all congenital heart disease. Primary surgical closure has been the standard approach for many years with high success rate. Transcatheter closure of ASD in selected patients has became the contemprorary practice that has the advantages of short hospital stay and relative ease of prosedure. In this article, we reported the results of percutaneous closure of ASD in our center.
Methods: Between 2009-2011, thirty-two patients (mean age 36±18 years and 24 females who had secundum type ASD which detected by transthoracic echocardiography (TTE and transesophageal echocardiography (TEE were included in this study. Twenty-nine (90,6% patients were closed percutaneously. Tree patients were referred for surgery because of failure of percutaneous closure. Amplatzer Septal Occluder was used in 25 (86,2% patients and Occlutech device was used in other patients. All procedures were performed under local anesthesia. All of the patients were examined with TTE before the procedure and, at the 1st, 3rd, 6th and 12th month follow-up visits.
Results: Pre-operative mean pulmonary artery pressure was 33,3±7,5 mmHg and mean pulmonary to systemic flow (Qp/Qs ratio was 2,9±1,1. Mean ASD diameter measured by TEE was 20,1±6,6 mm and mean streched diameter measured by balloon catheter was 22,1±5,9 mm. Mean device size 24±6 mm (range 12-36 mm. In the follow-up period no major complication was observed, but mild to moderate residual shunt flow was detected in two patients (% 6,8.
Conclusion: Percutaneous ASD closure has been performed with high success and low complication rates in patients with secundum atrial septal defect in our center.

  5. Off-pump atrial septal defect closure using the universal cardiac introducer®: creation of models of atrial septal defects in the pig access and surgical technique.

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    Guiraudon, Gerard M; Jones, Douglas L; Bainbridge, Daniel; Moore, John T; Wedlake, Chris; Linte, Cristian; Wiles, Andrew; Peters, Terry M

    2009-01-01

    : Optimal atrial septal defect (ASD) closure should combine off-pump techniques with the effectiveness and versatility of open-heart techniques. We report our experience with off-pump ASD closure using the Universal Cardiac Introducer (UCI) in a porcine model. The goal was to create an ASD over the fossa ovale (FO) and position a patch over the ASD under ultrasound (US) imaging and augmented virtual reality guidance. : An US probe (tracked with a magnetic tracking system) was positioned into the esophagus (transesophageal echocardiographic probe) for real-time image-guidance. The right atrium (RA) of six pigs was exposed via a right lateral thoracotomy or medial sternotomy. The UCI was attached to the RA wall. A punching tool was introduced via the UCI, navigated and positioned, under US guidance, to create an ASD into the FO. A patch with its holder and a stapling device were introduced into the RA via the UCI. The patch was positioned on the ASD. Occlusion of the ASD was determined using US and Doppler imaging. : The FO membrane was excised successfully in all animals. US image-guidance provided excellent visualization. The patch was positioned in all cases with complete occlusion of the ASD. The stapling device proved too bulky, impeding circumferential positioning. : Using the UCI, ASD closure was safe and feasible. US imaging, combined with virtual and augmented reality provided accurate navigating and positioning. This study also provided valuable information on the future design of anchoring devices for intracardiac procedures.

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

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    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.)

  7. Tulip deformity with Cera atrial septal defect devices: a report of 3 cases.

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    Kohli, Vikas

    2015-02-01

    Device closure of secundum atrial septal defect (ASD) is the treatment of choice when anatomy is favourable. Amplatzer device has remained the gold standard for closure of ASD. Cobra deformity is a well-reported problem with devices. Recently, Tulip deformity has been reported in a single case. We report a series of cases where we noted Tulip deformity along with inability to retract the device in the sheath in Cera Lifetech devices. This resulted in prolongation of procedure, excessive fluoroscopic exposure and additional interventional procedures not usually anticipated in ASD device closure. We believe that the problem is due to the stiffness of the device resulting in its inability to be retracted into the sheath. We also report a unique way of retrieving the device.

  8. Transcatheter closure of atrial septal defect in a patient with Noonan syndrome after corrective surgery

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    Mangovski Ljupčo

    2015-01-01

    Full Text Available Introduction. Transcatheter atrial septal defect (ASD closure is considered to be a gold standard for patients with the suitable anatomy as compared to cardiac surgery. Reocurrence of ASD after surgical closure is a very rare late complication which can be successfully managed with transcatheter procedure. Case report. We reported a female patient with Noonan syndrome who presented with hemodinamically significant ASD 37 years after the corrective cardiac surgery. Due to numerous comorbidities which included severe kyphoscoliosis, pectus excavatum and multiple surgeries we decided to perform transcatheter closure of ASD. The procedure itself was very challenging due to the patient’s short stature and heart’s orientation in the chest, but was performed successfully. The subsequent follow-up was uneventful and the patient reported improvement in the symptoms. Conclusion. Transcatheter closure of ASD in a patient with Noonan syndrome with the history of surgically corrected ASD can be performed successfully, despite challenging chest anatomy.

  9. Limited access atrial septal defect closure and the evolution of minimally invasive surgery.

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    Izzat, M B; Yim, A P; El-Zufari, M H

    1998-04-01

    While minimizing the "invasiveness" in general surgery has been equated with minimizing "access", what constitutes minimally invasive intra-cardiac surgery remains controversial. Many surgeons doubt the benefits of minimizing access when the need for cardiopulmonary bypass cannot be waived. Recognizing that median sternotomy itself does entail significant morbidity, we investigated the value of alternative approaches to median sternotomy using atrial septal defect closure as our investigative model. We believe that some, but not all minimal access approaches are associated with reduced postoperative morbidity and enhanced recovery. Our current strategy is to use a mini-sternotomy approach in adult patients, whereas conventional median sternotomy remains our standard approach in the pediatric population. Considerable clinical experiences coupled with documented clinical benefits are fundamental before a certain approach is adopted in routine practice.

  10. Comparison of transcatheter closure of secundum atrial septal defect using the Amplatzer septal occluder associated with deficient versus sufficient rims.

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    Du, Zhong-Dong; Koenig, Peter; Cao, Q-Ling; Waight, David; Heitschmidt, Mary; Hijazi, Ziyad M

    2002-10-15

    To evaluate the feasibility of transcatheter closure of secundum atrial septal defects (ASDs) associated with deficient rims (rim of 0 to 4 mm (n = 20), an inferior rim of 2 mm (n = 2), or a posterior rim of 4 mm (n = 1) as assessed by transesophageal echocardiography (TEE) or intracardiac echocardiography (ICE). Forty-eight patients with sufficient rims (>5 mm) who underwent closure served as controls. There were no differences between the 2 groups in ASD stretched diameter and device size (p >0.05). Of 23 patients with deficient rims, 17 (74%) had immediate complete closure compared with 44 of 48 patients (92%) with sufficient rims (p rims vs 94% for patients with sufficient rims at 24 hours and 100% vs 93% at 6 months, respectively). The fluoroscopic time and procedure time were longer in patients with deficient rims (13 +/- 7 and 72 +/- 26 minutes, respectively) compared with those with sufficient rims (10 +/- 4 and 61 +/- 22 minutes, respectively). No major complications were encountered either during or after the closure procedure in both groups. Thus, transcatheter closure of ASDs associated with small anterior, inferior, or posterior rims is feasible using an ASO. Long-term follow-up data are still needed to assess long-term safety and efficacy.

  11. Transesophageal Echocardiographic Characteristics of Secundum-Type Atrial-Septal Defect in Adult Patients

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    Arezou Zoroufian

    2009-12-01

    Full Text Available Background: Given the dearth of data in the existing literature on the size and morphologic variability of secundumtype atrial-septal defect (ASD-II in adult patients, we aimed to address this issue in a series of consecutive adult patients evaluated by transesophageal echocardiography (TEE.Methods: A total of 50 patients (68.0% female with isolated ASD-II underwent TEE for the evaluation of the defect. The morphological characteristics of the defect were evaluated, and the largest defect size was measured. The ASD rim wasdivided into 6 sectors: the superior-anterior, superior-posterior, superior, inferior-anterior, inferior-posterior, and inferior.The minimal length of the defect rims was determined.Results: Mean age at the time of evaluation was 33.62±14.48 years. Mean defect diameter in the all the study patients was 20.80±8.17 mm. Thirteen morphological variations were detected. Deficiency of one rim was detected in 14 (28% patients,two in 16 (32%, three in 2 (4%, and four in 2 (4%. Deficiency of the superior anterior rim was found in 24% of the patients as the most frequent morphology. There was a significant correlation between the defect size and number of deficient rims (γ=0.558, P value<0.001. Forty-eight (96% patients emerged for defect closure: 22 (46.2% suitable for percutaneousclosure and 26 (53.8% for surgical closure. Two patients with small defects were recommended for medical treatment and follow-up.Conclusion: ASD-II is larger and more morphologically variable in adults than in children. Based on the findings of the present and previous studies and given the advantages of percutaneous treatment, it is advisable to make a decision on ASD-II closure as soon as possible before it outgrows the transcatheter closure suitability criteria.

  12. Altered Pulmonary Venous Flow Pattern in Young Adults with Atrial Septal Defect

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    Mozhgan Parsaee

    2016-08-01

    Full Text Available Introduction: Atrial septal defect (ASD is a common congenital heart disease and causes left-to-right shunting and significant right ventricular (RV volume overload. The goal of this study was to evaluate the influence of these hemodynamic changes on pulmonary venous flow pattern in young adults.Methods: Complete echocardiographic examination was performed in a group of 40 young adults (aged < 40 years who had secundum type ASD and was compared to 40 age-matched individuals in control group who had no cardiac abnormality. Systolic and diastolic flow velocities in pulmonary veins (PV, superior vena cava (SVC, inferior vena cava (IVC and RV functional parameters were recorded and evaluated.Results: As opposed to healthy young individuals who showed distinct S and D waves with diastolic predominance in pulmonary vein Doppler, in patients with ASD a continuous flow with increased systolic peak that began in systole and continued to the late diastole was observed. The RV systolic function increased compared to the control group.Conclusions: In patients with ASD, the pattern of pulmonary veins flow transforms into a single continuous antegrade wave with systolic dominance due to persistent shunting of left atrial blood in to right heart chambers as well as increased RV pump function on pulmonary vein (by means of ASD, SVC and IVC, and could be used as a screening method for the presence of secundum type ASDs in young adults.

  13. Conscious sedation using dexmedetomidine for percutaneous transcatheter closure of atrial septal defects: A single center experience

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    Pushkar Mahendra Desai

    2016-01-01

    Full Text Available Objective: The aim of this study is to determine safety and feasibility of conscious sedation using dexmedetomidine for transcatheter atrial septal defect (ASD device closure. Material and Methods: A retrospective institutional review of transcatheter ASD device closure without endotracheal intubation over 18 months. The protocol included topical oropharyngeal anesthesia using lignocaine followed by dexmedetomidine bolus 1 μg/kg intravenously over 10 min and maintenance dose 0.2-0.7 μg/kg/h. Ramsay sedation score 2-3 was maintained. Patients were analyzed regarding demographic profile, device size, procedure time, anesthesia time, recovery time, hospital stay, and any hemodynamic or procedural complications. Results: A total of 43 patients with mean age 31.56 ± 13.74 years (range: 12-56 years were analyzed. Mean anesthesia duration was 71.75 + 21.08 min. Mean recovery time was 7.6 ± 3.01 min. 16 females and one male patient required additional propofol with a mean dose of 30.8 ± 10.49 mg. No hemodynamic instability was noted. No patient required general anesthesia with endotracheal intubation. The procedure was successful in 93.02% of patients. Four patients developed atrial fibrillation. All patients were satisfied. Conclusion: Conscious sedation using dexmedetomidine is a safe and effective anesthetic technique for percutaneous ASD closure.

  14. Transcathether closure of a right pulmonary artery-to-left atrial fistula using Amplatzer muscular ventricular septal defect occluder.

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    Ece, Ibrahim; Uner, Abdurrahman; Cuce, Ferhat; Balli, Sevket

    2014-10-01

    A right pulmonary artery-to-left atrial fistula is a very rare cyanotic congenital heart defect and is characterized by cyanosis and normal auscultation of the heart. Interventional closure of the fistula using occluder devices and coils has been rarely reported. We report the successful closure of a RPA-to-left atrial fistula using an Amplatzer muscular ventricular septal defect occluder in a child with cyanosis. The two-dimensional echocardiogram with bubble contrast study demonstrated the communication between right pulmonary artery and left atrium. Computerized tomography confirmed the diagnosis and delineated the anatomy.

  15. Totally robotic atrial septal defect closure: 7-year single-institution experience and follow-up.

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    Xiao, Cangsong; Gao, Changqing; Yang, Ming; Wang, Gang; Wu, Yang; Wang, Jiali; Wang, Rong; Yao, Minghui

    2014-12-01

    Robotic technology has been applied to atrial septal defect (ASD) repair for more than 10 years, but the number of cases reported is limited and results of long-term follow-up are not clear. This study reports on a large group of patients who underwent totally robotic ASD repair on an arrested or beating heart at a single institution with a 7-year follow-up. From 2007 to 2013, 160 patients (median age, 36 years; range, 11-66 years) at our centre underwent selective repair of secundum-type ASD using the da Vinci robotic system. The first 54 cases were performed on an arrested heart (arrested-heart group, n = 54) and the remainder on a beating heart (beating-heart group, n = 106). The mean diameter of defects was 2.9 cm (range, 1.1-4.1 cm). Cardiopulmonary bypass was achieved via cannulation of the femoral vessels and the right internal jugular vein. Blood cardioplegic arrest was induced using a transthoracic Chitwood clamp in the arrested-heart group. With the assistance of a robotic surgical system, atrial septal defect repairs were performed with or without tricuspid valvuloplasty via three 8-mm ports, a camera port and a working port in the right chest. Transoesophageal echocardiography was used to evaluate surgical results and follow-up. Complete ASD closure was verified by intraoperative transoesophageal echocardiography in all patients. None of the procedures was converted to an alternate technique and there were no major complications. There were significant learning curves for cross-clamp time, operative duration and cardiopulmonary bypass time. The beating-heart group had significantly shorter operative and cardiopulmonary bypass durations than the arrested-heart group (P = 0.000). The two groups had similar durations of mechanical ventilation and intensive care unit and hospital stays, and similar drainage volumes. During the 39 ± 21 months of follow-up, no patient required reoperation because of a residual shunt or tricuspid valve regurgitation. ASD can

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

    Science.gov (United States)

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

    2015-08-01

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

  17. Robotically assisted totally endoscopic atrial septal defect repair: insights from operative times, learning curves, and clinical outcome.

    Science.gov (United States)

    Bonaros, Nikolaos; Schachner, Thomas; Oehlinger, Armin; Ruetzler, Elisabeth; Kolbitsch, Christian; Dichtl, Wolfgang; Mueller, Silvana; Laufer, Guenther; Bonatti, Johannes

    2006-08-01

    Remote access perfusion and robotics have enabled totally endoscopic closure of atrial septal defect and patent foramen ovale. The aim of this study was to address learning curve issues of totally endoscopic atrial septal defect repair on the basis of a single-center experience and to investigate whether long cardiopulmonary bypass and aortic occlusion times influence intraoperative and postoperative outcomes. Seventeen patients (median age, 35 years; range, 16 to 55 years) underwent totally endoscopic atrial septal defect repair using remote access perfusion and robotic technology (da Vinci telemanipulation system). Learning curves were assessed by means of regression analysis with logarithmic curve fit. The effect of operative variables on clinical outcome was analyzed by linear regression using the Spearman's rho coefficient. No operative mortality or serious surgical complications were observed. No residual shunt was detected at intraoperative or postoperative echocardiography. Significant learning curves were noted for total operative time: y(min) = 406 - 49 ln(x) (r2 = 0.725; p = 0.002); cardiopulmonary bypass time: y(min) = 225 - 42 ln(x) (r2 = 0.699; p = 0.003); and aortic occlusion time: y(min) = 117 - 25 ln(x) (r2 = 0.517; p = 0.04), x = number of procedures. Median ventilation time, intensive care unit stay, and hospital length of stay were 7 hours (range, 2 to 19 hours), 26 hours (range, 15 to 120 hours), and 8 days (range, 5 to 14 days), respectively. No correlation was detected between cardiopulmonary bypass time and intubation time (r2 = 0.283; p = 0.326), intensive care unit stay (r2 = -0.138; p = 0.639), or total length of stay (r2 = 0.013; p = 0.962). Totally endoscopic atrial septal defect repair can be performed safely, and learning curves for operative times are steep. Longer cardiopulmonary bypass times had no negative impact on intraoperative and postoperative outcome.

  18. Atypical atrial septal defects in children: noninvasive evaluation by cardiac MRI

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    Beerbaum, Philipp; Parish, Victoria; Bell, Aaron [Guy' s and St. Thomas' Hospital, Division of Imaging Sciences, King' s College London, London (United Kingdom); Gieseke, Juergen [Philips Medical Systems, Best (Netherlands); Koerperich, Hermann; Sarikouch, Samir [Ruhr-University of Bochum, Department of Congenital Heart Disease and Institute for Magnetic Resonance Imaging, Heart and Diabetes Centre North Rhine-Westfalia, Bad Oeynhausen (Germany)

    2008-11-15

    Atypical left-to-right shunts at the level of the atrium in children such as sinus venosus atrial septal defects (ASDs) and partial anomalous pulmonary venous return (PAPVR) may be difficult to assess by transthoracic or transoesophageal echocardiography. Free-breathing cardiac MRI may be a powerful alternative. To assess the value of free-breathing cardiac MRI in the delineation of atypical ASDs in children. A total of 82 children (mean age 5.9 years, range 1.1-15.7 years) with suspected ASD and inconclusive transthoracic echocardiography underwent cardiac MRI under free-breathing, mostly sedated conditions. Phase-contrast MRI was used for defect visualization and shunt quantification, and multiphase inflow MR angiography for delineation of pulmonary/systemic venous connections. Of the 82 patients, 34 (41%) were diagnosed with atypical shunt lesions at the level of the atrium and 48 (59%) with simple secundum ASDs. No false-negative or false-positive findings were reported by MRI compared to cardiac catheterization and intraoperative findings. Superior sinus venosus ASD with partial anomalous PAPVR was present in 10 of the 82 children (12.2%), whereas 2 (2.4%) had a large posterior-inferior defect, 5 (6.1%) had isolated PAPVR, and 17 (20.7%) had multiple ASDs and/or associated vascular anomalies. Q{sub p}/Q{sub s} by phase-contrast MRI agreed well with oximetry values (mean difference 3%, limits of agreement {+-}21-25%; Bland/Altman analysis). Free-breathing cardiac MRI under sedation allows reliable identification of atypical left-to-right shunt defects at the level of the atrium in children in whom transcatheter ASD closure is unsuitable, including delineation of pulmonary or systemic venous anomalies and shunt quantification. (orig.)

  19. Consanguinity and isolated atrial septal defect in North East of Iran.

    Science.gov (United States)

    Moghaddam, Hasan Mottaghi; Esfehani, Reza Jafarzadeh; Panah, Nader Yazdan; Esfehani, Ali Jafarzadeh

    2014-01-01

    The rate of consanguineous marriage is high in Middle Eastern countries such as Iran. The relationship between consanguineous marriage and congenital heart disease is discussed in some studies, but there is not much data for relationship between atrial septal defect (ASD) and consanguineous marriage. The aim of this study was to evaluate the relationship between consanguineous marriage and ASD echocardiographic characteristics. This was a cross-sectional study approved by Mashhad University of Medical Sciences ethics committee and took place in Mashhad, Iran, for a period of 3 years from August 2008 till September 2011. In this cross-sectional study, 113 ASD patients participated and they were categorized into 3 groups on the basis of family relationship between their parents: first group-"no relationship," second group- "third degree relationship," and third group- "far relationship." Among the 54 male and 59 female ASD patients, the most prevalent type of ASD was ASD secundum (85.0%) followed by sinus venosus (8.8%). A total of 56% patients were present in the first group and 15% and 29% in the second group and the third group, respectively." The relationship between consanguinity and type of ASD (P consanguinity (P=.003) was also observed. Considering the fact that there is a high prevalence of ASD and consanguineous marriage in Iran and bearing in mind the results of the present study, we recommend educating couples about the outcomes of consanguineous marriage in pre-marriage counseling.

  20. Pulmonary function in children after surgical and percutaneous closure of atrial septal defect.

    Science.gov (United States)

    Zaqout, Mahmoud; De Baets, Frans; Schelstraete, Petra; Suys, Bert; Panzer, Joseph; Francois, Katrien; Bove, Thierry; Coomans, Ilse; De Wolf, Daniel

    2010-11-01

    This study aimed to study differences in lung function after surgical and percutaneous atrial septal defect (ASD) closure. Several studies have demonstrated abnormalities of pulmonary function in adults and children with ASD. These abnormalities persist even a few years after correction. This study compared pulmonary function between patients who underwent ASD closure by surgery and those who had closure by device. This is the ideal pediatric population for studying changes in lung function caused by cardiopulmonary bypass or sternotomy. The 46 patients in this study were treated by percutaneous closure (group 1) or surgical closure (group 2) of ASD and then scheduled for pulmonary function testing an average of 5.8 years after ASD closure. The mean values of functional residual capacity, total lung capacity, and residual volume did not differ between the two groups. The surgical group showed a significant decrease in expiratory reserve volume (p function. Longitudinal lung function follow-up assessment after cardiac surgery is warranted to detect and measure restrictive abnormalities in this type of congenital heart disease and others.

  1. Lung biopsy diagnosis of operative indication in secundum atrial septal defect with severe pulmonary vascular disease.

    Science.gov (United States)

    Yamaki, Shigeo; Kumate, Munetaka; Yonesaka, Susumu; Maeda, Katsuhide; Endo, Masato; Tabayashi, Koichi

    2004-10-01

    Surgical indication was determined by lung biopsy in 91 patients with secundum atrial septal defect (ASD) and severe pulmonary hypertension > 70 mm Hg of pulmonary arterial peak pressure and/or pulmonary vascular resistance of > 8 U/m(2). Pulmonary vascular disease (PVD) in ASD was classified into four types: (1) Musculoelastosis consisting of longitudinal muscle bundles and elastic fibers; surgery is indicated no matter how severely the peripheral small pulmonary arteries are occluded. Surgery was performed in all of the 20 patients, and the postoperative course was uneventful. (2) Plexogenic pulmonary arteriopathy: surgery is indicated for a PVD index < or = 2.3. Surgery was performed in 25 of the 32 patients. The remaining seven patients for whom surgery was not indicated are under follow-up observation. No deaths have occurred among the 32 patients. (3) Thromboembolism of small pulmonary arteries: Surgery is indicated for all such cases. Surgery was indicated in all of the five patients. (4) Mixed type of plexogenic pulmonary arteriopathy and musculoelastosis: Surgery is indicated if the collateral is not observed. Surgery was performed in 15 of the 25 patients. The remaining 10 patients for whom surgery was not indicated are under follow-up observation. Nine of these 91 patients associated with primary pulmonary hypertension were eliminated from this study. No deaths due to PVD occurred among the 82 patients who underwent lung biopsy diagnosis. Lung biopsy diagnosis is concluded to be very effective.

  2. Heart rate variability in patients with atrial septal defect and healthy children.

    Science.gov (United States)

    Bakari, Süleyman; Koca, Bülent; Oztunç, Funda; Abuhandan, Mahmut

    2013-06-01

    Heart rate variability (HRV) measures are altered in various cardiac and non-cardiac situations in children. The autonomic nervous system is assumed to have a role in the pathophysiology of atrial septal defect (ASD). In this study, we evaluated the autonomic system by measuring HRV in children with ASD. Twenty-eight patients with ASD and 32 healthy children (mean ages: 6.6±2.1 years and 6.4±2.2 years, respectively) were enrolled in the study. Twenty-four-hour ambulatory electrocardiographic recordings were obtained and the seven time-domain (SDNN, SDANN, rMSSD, SD, SDNN index, PNN50, and mean RR) and four frequency-domain (VLF, LF, HF, and LF/HF ratio) indices of HRV were analyzed. A significant decrease in calculated HRV variables was observed in children with ASD as compared to controls. The HRV alteration was found in both time-domain and frequency-domain parameters. Our results indicate that HRV is decreased in children with ASD, which implies parasympathetic withdrawal and sympathetic predominance. Copyright © 2013 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

  3. Totally thoracoscopic surgery for the treatment of atrial septal defect without of the robotic Da Vinci surgical system.

    Science.gov (United States)

    Liu, Gaoli; Qiao, Yanli; Ma, Liming; Ni, Liangchun; Zeng, Shanguang; Li, Qingchen

    2013-05-01

    More and more surgeons and patients focus on the minimally invasive surgical techniques in the 21st century. Totally thoracoscopic operation provides another minimal invasive surgical option for patients with ASD (atrial septal defect). In this study, we reported our experience of 61 patients with atrial septal defect who underwent totally thoracoscopic operation and discussed the feasibility and safety of the new technique. From January 2010 to October 2012, 61 patients with atrial septal defect underwent totally thoracoscopic closure but not traditional median sternotomy surgery. We divided the 61 patients into two groups based on the operation sequence. The data of group A (the first 30 cases) and group B (the last 31 cases). The mean age of the patients was 35.1 ± 12.8 years (range, 6.3 to 63.5 years), and mean weight was 52.7 ± 11.9 kg (range, 30.5 to 80 kg). Mean size of the atrial septal defect was 16.8 ± 11.3 mm (range, 13 to 39 mm) based on the description of the echocardiography. All patients underwent totally thoracoscopy successfully, 36 patients with pericardium patch and 25 patients were sutured directly. 7 patients underwent concomitant tricuspid valvuloplasty with Key technique. No death, reoperation or complete atrioventricular block occurred. The mean time of cardiopulmonary bypass was 68.5 ± 19.1 min (range, 31.0 to 153.0 min), the mean time of aortic cross-clamp was 27.2 ± 11.3 min (range, 0.0 to 80.0 min) and the mean time of operation was 149.8 ± 35.7 min (range, 63.0 to 300.0 min). Postoperative mechanical ventilation averaged 4.9 ± 2.5 hours (range, 3.5 to 12.6 hours), and the duration of intensive care unit stay 20.0 ± 4.8 hours (range, 15.5 to 25 hours). The mean volume of blood drainage was 158 ± 38 ml (range, 51 to 800 ml). No death, residual shunt, lung atelectasis or moderate tricuspid regurgitation was found at 3-month follow-up. The totally thoracoscopic operation is feasible and safe for

  4. Efficacy of a novel IGS system in atrial septal defect repair

    Science.gov (United States)

    Mefleh, Fuad N.; Baker, G. Hamilton; Kwartowitz, David M.

    2013-03-01

    Congenital heart disease occurs in 107.6 out of 10,000 live births, with Atrial Septal Defects (ASD) accounting for 10% of these conditions. Historically, ASDs were treated with open heart surgery using cardiopulmonary bypass, allowing a patch to be sewn over the defect. In 1976, King et al. demonstrated use of a transcatheter occlusion procedure, thus reducing the invasiveness of ASD repair. Localization during these catheter based procedures traditionally has relied on bi-plane fluoroscopy; more recently trans-esophageal echocardiography (TEE) and intra-cardiac echocardiography (ICE) have been used to navigate these procedures. Although there is a high success rate using the transcatheter occlusion procedure, fluoroscopy poses radiation dose risk to both patient and clinician. The impact of this dose to the patients is important as many of those undergoing this procedure are children, who have an increased risk associated with radiation exposure. Their longer life expectancy than adults provides a larger window of opportunity for expressing the damaging effects of ionizing radiation. In addition, epidemiologic studies of exposed populations have demonstrated that children are considerably more sensitive to the carcinogenic effects radiation. Image-guided surgery (IGS) uses pre-operative and intra-operative images to guide surgery or an interventional procedure. Central to every IGS system is a software application capable of processing and displaying patient images, registration between multiple coordinate systems, and interfacing with a tool tracking system. We have developed a novel image-guided surgery framework called Kit for Navigation by Image Focused Exploration (KNIFE). In this work we assess the efficacy of this image-guided navigation system for ASD repair using a series of mock clinical experiments designed to simulate ASD repair device deployment.

  5. Echocardiographic Reference Values for Right Atrial Size in Children with and without Atrial Septal Defects or Pulmonary Hypertension.

    Science.gov (United States)

    Koestenberger, Martin; Burmas, Ante; Ravekes, William; Avian, Alexander; Gamillscheg, Andreas; Grangl, Gernot; Grillitsch, Marlene; Hansmann, Georg

    2016-04-01

    Right atrial (RA) size may become a very useful, easily obtainable, echocardiographic variable in patients with congenital heart disease (CHD) with right-heart dysfunction; however, according studies in children are lacking. We investigated growth-related changes of RA dimensions in healthy children. Moreover, we determined the predictive value of RA variables in both children with secundum atrial septal defect (ASD) and children with pulmonary hypertension (PH) secondary to CHD (PH-CHD). This is a prospective study in 516 healthy children, in 80 children with a secundum ASD (>7 mm superior-inferior dimension), and in 42 children with PH-CHD. We determined three RA variables, i.e., end-systolic major-axis length, end-systolic minor-axis length, and end-systolic area, stratified by age, body weight, length, and surface area. RA end-systolic length and area z scores were increased in children with ASD and PH-CHD when compared to those variables in the healthy control population. Using the Youden Index to determine the best cutoff scores in sex- and age-specific RA dimensions, we observed a sensitivity and specificity up to 94 and 91 %, respectively, in ASD children and 98 and 94 %, respectively, in PH-CHD children. We provide normal values (z scores -2 to +2) for RA size and area in a representative, large pediatric cohort. Enlarged RA variables with scores >+2 were predictive of secundum ASD and PH-CHD. Two-dimensional determination of RA size can identify enlarged RAs in the setting of high volume load (ASD) or pressure load (PH-CHD).

  6. TOGETHER WiTH ATRiAL SEPTAL DEFECT, BiCUSPiD AORTA, PECTUS EXCAVATUM AND MENTAL RETARDATiON : A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Mustafa Necati Dagli

    2015-03-01

    Full Text Available In this case, patient with mental retardation accepted in our clinic complain with shortness of breath. Then physical examination inspection evident in the pectus excavatum and made Echocardiography Atrial septal defect (ASD and bicuspid aortic consistent with the view were identified.Congenital abnormalities in patients with the aim of further evaluation of transesophageal echocardiography (TEE planned. In TEE, 26 mm secundum atrial septal defect, bicuspid aortic valve was detected. Percutaneous transcatheter atrial septal defect closure with septal occluder device. Pectus excavatum, such as mental retardation or even cardiac abnormalities do not complain about aspects of the research must be done. Cardiac abnormalities regardless of the direction of the association needs to be done to show that we have a rigorous screening. [J Contemp Med 2015; 5(1.000: 48-50

  7. Transcatheter closure of a small atrial septal defect with an Amplatzer™ patent foramen ovale occluder in a working dog with cyanosis and exercise intolerance at high altitude.

    Science.gov (United States)

    Shelden, A; Wesselowski, S; Gordon, S G; Saunders, A B

    2017-12-01

    A 6.5-year-old male Border Collie presented for transcatheter closure of an atrial septal defect due to exercise intolerance and cyanosis while working and training at altitude. A small, left-to-right shunting secundum atrial septal defect was confirmed with no evidence of significant right-sided volume overload. Pulmonary hypertension with subsequent right-to-left interatrial shunting occurring during exercise at high altitude was suspected and prompted the closure of the defect due to the dog's continued athletic requirements. The anatomy of the defect prompted use of a patent foramen ovale occluder rather than an atrial septal defect occluder, which was deployed using a combination of fluoroscopic and transesophageal echocardiographic guidance. The owner did not report continued exercise intolerance or cyanosis and the dog's lifestyle and residence at altitude was unchanged. Copyright © 2017 Elsevier B.V. All rights reserved.

  8. No-touch aorta robot-assisted atrial septal defect repair via two ports.

    Science.gov (United States)

    Ishikawa, Norihiko; Watanabe, Go; Tarui, Tatsuya

    2018-01-02

    Atrial septal defect (ASD) repairs have been successfully performed on arrested hearts with robotic assistance. The present study assessed the feasibility, safety, and efficacy of totally endoscopic cardiac surgery using a no-touch aorta technique for ASD via only 2 ports, and we named this procedure two-port robotic cardiac surgery (TROCS). Between May 2014 and June 2016, 8 consecutive patients underwent TROCS for ASD using the da Vinci surgical system (Intuitive Surgical Inc.) at our institute. All of the procedures were performed via only 2 port incisions in the right chest. One was the camera port, and the other was the port for the robotic instruments. Both robotic instruments were inserted through this port and crossed while being prevented from colliding with each other. The surgeon console was set to the reverse of default settings so that both masters would control the inverse instrument. TROCS for ASD was carried out under ventricular fibrillation induced by combinations of an electrical fibrillator, injection of potassium, and hypothermia without aortic cross-clamping. All cases were successfully repaired. The mean operation, cardiopulmonary bypass and ventricular fibrillation times were 129.6 ± 29.0 min, 66.9 ± 24.5 min and 9.6 ± 5.9 min, respectively, and the estimated blood loss volume was 28.1 ± 58.6 ml. No patients required blood transfusion during their hospital stay, and their cosmetic results were excellent. TROCS for ASD using no-touch aorta technique was achieved safely with good clinical results and excellent cosmetic results.

  9. Constrictive pericarditis in a patient with sinus venosus atrial septal defect and anomalous right upper pulmonary venous return

    Directory of Open Access Journals (Sweden)

    Uppu Santosh

    2009-01-01

    Full Text Available This is a report of a 49-year-old male, who presented with typical signs and symptoms of constrictive pericarditis. He was diagnosed with sinus venosus atrial septal defect (ASD and anomalous right upper pulmonary venous return during his adolescence, which was elected not to be repaired. During the attempted repair of the ASD it was noted there was a thick fibrous material covering the heart, which had progressed over time leading to frank constrictive pericarditis. His ASD spontaneously closed over time. There have been less than 10 cases reported with constrictive pericarditis of nonsurgical etiology in a patient with ASD, and none with sinus venosus ASD.

  10. [Left ventricular-right atrial communication with a residual ventricular septal defect: a case report of successful VSD patch closure with tricuspid valvuloplasty using folded patch].

    Science.gov (United States)

    Kanazawa, M; Katoh, T; Suzuki, K; Fujimura, Y; Tsuboi, H; Esato, K

    1992-12-01

    A 18-year-old woman with combination of left ventricular right atrial communication (LV-RA communication) and residual ventricular septal defect (VSD) was presented. She underwent the radical operation of atrial septal defect, VSD and infundibular pulmonary stenosis at the age 11 years. Surgery was performed under the diagnosis of LV-RA communication and residual VSD. Two small defect were existed at the septal leaflet of the tricuspid valve, which communicated to left ventricle. The part of patch being used as closure of VSD at the previous operation, including perforated part of the septal leaflet was resected. A folded patch was used to reinforce the residual VSD and valvuloplasty was made using the remaining intact tricuspid tissues. Post-operative clinical course was uneventful. Shunt flow from LV to RA was not demonstrated in the postoperative left ventricular angiocardiography.

  11. [The role of MRI for the evaluation of atrial septal defects before and after percutaneous occlusion with the amplatzer septal occluder(R)].

    Science.gov (United States)

    Weber, C; Dill, T; Mommert, I; Hofmann, T; Adam, G

    2002-11-01

    Evaluation of morphologic and functional MRI of atrial septal defects (ASD) before and after percutaneous occlusion with the Amplatzer Septal Occluder (AOC). Comparison of MRI with transesophageal echocardiography (TEE), balloon measurement (IVBM) and cardiac catheterization with shunt quantification (CCSQ). Twenty patients with ASD were examined before and three months after AOC implantation. ECG-triggered, breath-hold T1-weighted Turbo Spin Echo Segmented FLASH 2D and dynamic turbo-FLASH-GRE sequences after application of 0.2 mmol gadolinium DTPA per kg body weight were obtained in a 1.5 T MRI system. Defect size, and distance to coronal sinus (CS) and right upper pulmonary vein (RUPV) were determined for pre-interventional planning, and the AOC size was measured quantitatively for post-interventional follow-up. The shunts were evaluated qualitatively (occurrence of jets), semiquantitatively (jet length, turbulence square product) and quantitatively (flow measurement in the thoracic aorta and in the left and right pulmonary arteries). The average size of the ASD measured by MRI was 17.6 mm (11 - 24.8 mm) in the axial view, 15.9 mm (10.8 - 28.9 mm) in the sagittal view and 16.4 mm (12.1 - 24.8 mm) in the short axis view. In comparison, the average defect size was 15 mm (8 - 24 mm) by TEE and 20 mm (13 - 27 mm) by IVBM. The average distance to the RUPV was 17 mm (9.6 - 21.9 mm) and to the CS 11.2 mm (5 - 17 mm). The AOC was visualized with only minimal artifacts. Qualitative analysis of the MRI findings revealed an occurrence of jets in 17/20 patients. Semiquantitative analysis documented a high correlation for jet length and square product of the turbulence to defect size r = 0.81 resp. r = 0.82. Mean QP/QS-ratio measured by MR-volumetry was 1.6 +/- 0.29 and by MR-flow 1.6 +/- 0.26. The corresponding measurements were 1.7 +/- 0.3 for TEE and 1.5 +/- 0.5 for CCSQ. In comparison to TEE, the correlation coefficient was r = 0.96 for MR-volumetry and r = 0.85 for MR

  12. [Maze procedure in a case of dextrocardia with atrial septal defect and persistent left superior vena cava].

    Science.gov (United States)

    Muraoka, Arata; Kawada, Masaaki; Misawa, Yoshio

    2014-08-01

    A 52-year-old man was diagnosed with dextrocardia at the age of 1 year and was asymptomatic until 1 year before admission. He was transferred to our hospital for management of atrial fibrillation. A transthoracic echocardiogram showed dextrocardia with atrial septal defect;moderate tricuspid valve regurgitation; and a large, persistent left superior vena cava. A cardiac catheterization study revealed that pulmonary flow/systemic flow (Qp/Qs) was 3.6 and that pulmonary vascular resistance was 2.5 Wood U·m². Intracardiac repair with tricuspid annuloplasty and a maze procedure was scheduled. When establishing cardiopulmonary bypass, venous drainage was initially obtained from the inferior vena cava and the left superior vena cava, and the small superior vena cava was then directly cannulated after opening the right atrium. The patient's postoperative course was uneventful, and serial electrocardiograms have demonstrated maintenance of normal sinus rhythm for 3.5 years after the operation.

  13. Transcatheter closure of secundum atrial septal defect in adults: report of our first experience in a developing country

    Directory of Open Access Journals (Sweden)

    Animasahun BA

    2014-04-01

    Full Text Available B Adeola Animasahun,1 Yele Aluko,2 Adeyemi Johnson,3 Kofo Ogunyankin,3 Sunita Maheshwari41Department of Paediatrics and Child Health, Lagos State University College of Medicine, Lagos, Nigeria; 2Mid Carolina Cardiology, Charlotte, NC, USA; 3First Cardiology Consultants, Lagos, Nigeria; 4Narayana Hrudayalaya Institute of Cardiac Sciences, Bangalore, IndiaBackground: Since the first report on device closure of atrial septal defect (ASD in 1976, the procedure has gained wide acceptance and has been used worldwide, including parts of Africa. The advantages when compared to surgical closure include a shorter procedure time and hospital stay and no scarring. This mode of treatment was not available in Nigeria prior to the cases reported here, requiring patients to travel abroad for the procedure.Methods: A cardiac catheterization laboratory became available in Lagos, Nigeria in 2009, and in December 2010 the laboratory collaborated with Lagos State University College of Medicine and Lagos State University Teaching Hospital to perform the device closure on two women (aged 34 and 62 years with secundum ASD. To the best of our knowledge, these are the first times the procedure has been performed in Nigeria.Results: Both patients made successful recovery and are stable.Conclusion: Transcatheter closure of ASD is now safe and available in Nigeria.Keywords: atrial septal defect, transcatheter closure, Nigeria

  14. Late complete atrioventricular block after closure of an atrial septal defect with a gore septal occluder (GSO™)

    Science.gov (United States)

    Sigler, Matthias; Priessmann, Helga

    2015-01-01

    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 Wiley Periodicals, Inc. PMID:26354228

  15. Atrial flutter in a patient with atrial septal defect and anomalous venous drainage: unusual approach for ablation.

    Science.gov (United States)

    Roca-Luque, Ivo; Rivas, Nuria; Dos, Laura; Francisco, Jaume; Pérez-Rodon, Jordi; Pijuan, Antònia; Garcia-Dorado, David; Moya, Àngel

    2017-07-01

    Atrial flutter ablation in CHD (Congenital Heart Disease) patients is a challenging procedure because of the possibility of multiple circuits. Electroanatomical mapping and pacing maneuvers are crucial to determine critical isthmus. Moreover, vascular abnormalities and residual cardiac defects need to be known before the ablation to decide the better strategy for ablation.

  16. [Feasibility of device closure for multiple atrial septal defects using 3D printing and ultrasound-guided intervention technique].

    Science.gov (United States)

    Qiu, X; Lü, B; Xu, N; Yan, C W; Ouyang, W B; Liu, Y; Zhang, F W; Yue, Z Q; Pang, K J; Pan, X B

    2017-04-25

    Objective: To investigate the feasibility of trans-catheter closure of multiple atrial septal defects (ASD) monitored by trans-thoracic echocardiography (TTE) under the guidance of 3D printing heart model. Methods: Between April and August 2016, a total of 21 patients (8 male and 13 female) with multiple ASD in Fuwai Hospital of Chinese Academy of Medical Sciences underwent CT scan and 3-dimensional echocardiography for heart disease model produced by 3D printing technique. The best occlusion program was determined through the simulation test on the model. Percutaneous device closure of multiple ASD was performed follow the predetermined program guided by TTE. Clinical follow-up including electrocardiogram and TTE was arranged at 1 month after the procedure. Results: The trans-catheter procedure was successful in all 21 patients using a single atrial septal occluder. Mild residual shunt was found in 5 patient in the immediate postoperative period, 3 of them were disappeared during postoperative follow-up. There was no death, vascular damage, arrhythmia, device migration, thromboembolism, valvular dysfunction during the follow-up period. Conclusion: The use of 3D printing heart model provides a useful reference for transcatheter device closure of multiple ASD achieving through ultrasound-guided intervention technique, which appears to be safe and feasible with good outcomes of short-term follow-up.

  17. Surgery for complications of trans-catheter closure of atrial septal defects : a multi-institutional study from the European Congenital Heart Surgeons Association

    NARCIS (Netherlands)

    Sarris, George E.; Kirvassilis, George; Zavaropoulos, Prodromos; Belli, Emre; Berggren, Hakan; Carrel, Thierry; Comas, Juan V.; Corno, Antonio F.; Daenen, Willem; Di Carlo, Duccio; Ebels, Tjark; Fragata, Jose; Hamilton, Leslie; Hraska, Viktor; Jacobs, Jeffrey; Lazarov, Stojan; Mavroudis, Constantine; Metras, Dominique; Rubay, Jean; Schreiber, Christian; Stellin, Giovanni

    Objective: This study aims to analyse the collective experience of participating European Congenital Heart Surgeons Association centres in the surgical management of complications resulting from trans-catheter closure of atrial septal defects (ASDs). Methods: The records of all (n = 56) patients,

  18. Acquired Left Atrial-to-Right Ventricular Shunt with Mitral Valve Incompetence: A Rare Sequela after Repair of Atrioventricular Septal Defect

    OpenAIRE

    Mohapatra, Srikant; Minhas, Harpreet Singh; Virmani, Sanjula; Mishra, Bana Bihari; Mukherjee, Kaushik; Banerjee, Amit

    2009-01-01

    Acquired left ventricular-to-right atrial communication is encountered periodically. This condition is chiefly attributable to surgical mishaps, trauma, endocarditis, or endomyocardial biopsy. In a few instances, a Gerbode-like defect develops after the repair of an atrioventricular septal defect. Our search of the worldwide medical literature revealed just 1 report of a “mirror” occurrence of a Gerbode-like defect: a shunt between the left atrium and the right ventricle.

  19. Imaging atrial septal defects by real-time three-dimensional transesophageal echocardiography: step-by-step approach.

    Science.gov (United States)

    Saric, Muhamed; Perk, Gila; Purgess, Jan R; Kronzon, Itzhak

    2010-11-01

    There are currently no standardized three-dimensional (3D) transesophageal echocardiographic (TEE) views of the interatrial septum and atrial septal defects (ASDs). Without a standardized approach, it is difficult to ascertain the important anatomic relationships (such as the location of the aortic rim of an ASD), to perform relevant measurements (such as the size of an ASD or the size of its rims), or to guide the deployment of catheters and devices during atrial septal closure. Using a 3D TEE matrix-array transducer, 706 TEE studies were performed over a 14-month period. The purpose of the study was to develop a standardized protocol for anatomically correct orientation of 3D TEE images of the interatrial septum and ASDs. Among 706 TEE studies, there were 23 patients with ASDs, representing 3.3% of the study population. Eighteen patients had secundum ASDs, two had primum ASDs, and three had sinus venosus ASDs of the superior vena cava type. A protocol for properly orienting 3D TEE images of the interatrial septum and ASDs was developed. When the images are acquired at an angle of 0°, the septum is properly oriented by the tilt-up-then-left maneuver. The initial 3D TEE image in first tilted up to reveal the right atrial side of the septum. Then the image is tilted 180° around its vertical axis to reveal the left atrial side of the septum; the aortic rim is on the left, the superior vena cava on the top, and the right-sided pulmonary vein ostia on the right side of the screen. For acquisitions at a higher angle, the rotate-left-in-z-axis maneuver is used. The image is first tilted up to reveal the right atrial side of the septum, as in the tilt-up-then-left maneuver. The image is then rotated counterclockwise in the z axis until the superior vena cave is at 12 o'clock. Finally, the image is tilted 180° around its vertical axis to reveal the left atrial side of the septum. The use of standardized tilt-up-then-left and rotate-left-in-z-axis maneuvers enhances the

  20. A novel one-shot circular stapler closure for atrial septal defect in a beating-heart porcine model.

    Science.gov (United States)

    Tarui, Tatsuya; Tomita, Shigeyuki; Ishikawa, Norihiko; Ohtake, Hiroshi; Watanabe, Go

    2015-02-01

    In surgical atrial septal defect (ASD) closure, there are no techniques or devices that can close the ASD accurately in a short time under a beating heart. We have developed a simple and automatic ASD closure technique using a circular stapler. This study assessed the feasibility and efficacy of a new circular stapler closure for ASD. Under a continuous beating heart, hand-sewn patch plasty ASD closure was performed in 6 pigs (group A) and circular stapler ASD closure was performed in 6 pigs (group B). The time to close the ASD and the effectiveness of the closure were compared. Closure was significantly faster in group B (10.5 ± 1.0 seconds) than in group A (664 ± 10 seconds; p heart porcine model. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  1. Robotic atrial septal defect closure and tricuspid annuloplasty in a case of situs inversus totalis with dextrocardia.

    Science.gov (United States)

    Onan, Burak; Aydin, Unal; Kahraman, Zeynep; Bakir, Ihsan

    2017-03-01

    Situs inversus totalis (SIT) with dextrocardia is an uncommon congenital positional anomaly, which is characterized by a symmetrical 'mirror-image' orientation of all organs in relation to the midline. Although sternotomy and thoracotomy is traditionally used in patients with SIT with dextrocardia, a totally endoscopic robotic surgery is an alternative surgical approach to intracardiac anomalies. Placement of robotic ports, transthoracic aortic clamp, cardioplegia delivery, and peripheral vascular cannulation is made from the left side of the chest, as a mirror orientation of the right-sided robotic cardiac procedures. Here, we present a patient who underwent concomitant robotic atrial septal defect closure and tricuspid annuloplasty with posterior plication using the da Vinci surgical system.

  2. Transcatheter closure of large atrial septal defects with deficient aortic or posterior rims using the "Greek maneuver". A multicenter study.

    Science.gov (United States)

    Thanopoulos, Basil D; Dardas, Petros; Ninios, Vlasis; Eleftherakis, Nicholaos; Karanasios, Evangelos

    2013-10-09

    We report a modification ("Greek maneuver") of the standard atrial septal defect (ASD) closure technique using the Amplatzer septal occluder (ASO) to facilitate closure of large ASDs with deficient aortic or posterior rims. 185 patients (median 10.8, range 3 to 52 years) with large ASDs (mean diameter 26±7 mm, range 20-40 mm) with a deficient aortic (134 patients) or posterior (51 patients) rim underwent catheter closure with the ASO using the "Greek maneuver" under transesophageal guidance. The Greek maneuver is applied when protrusion of the aortic edge of the deployed left disk of the device in to the right atrium is detected by echo. To circumvent this left disk is recaptured and the whole delivery system is pushed inward and leftward into the left atrium where the left disk and the 2/3 of right disk are simultaneously released. This maneuver forces the left disk to become parallel to the septum preventing the protrusion of the device into the right atrium. The ASO was successfully implanted and was associated with complete closure in 175/185 (95%) of the patients. There were no early or late complications related to the procedure during a follow-up period ranging from 6 months to 7 years. The "Greek maneuver" is a simple quite useful trick that facilitates closure of large ASDs associated with or without deficient aortic or posterior rims. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  3. Transthoracic echocardiography is a safe alternative for assessment and guidance of transcatheter closure of secundum atrial septal defect in children.

    Science.gov (United States)

    Baruteau, Alban-Elouen; Hascoët, Sébastien; Fraisse, Alain

    2017-05-01

    2D-transesophageal echocardiography (TEE) is routinely performed to guide percutaneous ASD closure in children. We aimed to assess whether two-dimensional (2D)-transthoracic echocardiography (TTE) is a safe alternative for assessment and guidance of atrial septal defect (ASD) closure in unselected children. We performed a retrospective single-center study including 389 consecutive children aged less than 15-year-old who underwent percutaneous ASD closure under 2D-TEE (1998-2005, n=133) or 2D-TTE (2005-2014, n=256). A balloon calibration was performed in all cases for the Amplatz Septal Occluder choice. ASDs were larger and rims deficiencies were more frequent in the TTE-guided group. The procedure was successful in 376 patients [96.7%; 95% confidence interval (CI), 94.4-98.2%]. The success rate tended to be higher in the TTE- versus TEE-guided group (98.0% versus 94.0%, P=0.069). Device migration occurred in 4 patients (1.0%; 95% CI: 0.3-1.6%), all after TEE-guided procedure (P=0.013). Early major adverse events were observed in 5 patients (1.3%; 95% CI: 0.4-3.0%), all in the TEE group (P=0.004). Fluroroscopic time and irradiation dose were not different among the 2 groups (P=0.450 and P=0.130 respectively). After a median follow-up of 7 years (range, 1-16 years), no adverse events was reported. One (0.3%, 95% CI: 0-1.4%) 12-year-old patient developed atrial fibrillation 5 years after the procedure. Pregnancies were uneventful in 72 cases. When a balloon sizing is performed, 2D-TTE imaging is as efficient as 2D-TEE to guide percutaneous ASD closure in children. The procedure can safely be done in spontaneously breathing children under TTE guidance alone in experienced centers.

  4. Intermediate and long-term followup of percutaneous device closure of fossa ovalis atrial septal defect by the Amplatzer septal occluder in a cohort of 529 patients

    Directory of Open Access Journals (Sweden)

    Tomar Munesh

    2011-01-01

    Full Text Available Objectives : The aim of present study is to analyze the intermediate and long-term follow up results of percutaneous closure of fossa ovalis atrial septal defect (ASD with Amplatzer septal occluder (ASO in a large cohort of patients including children and adults. Methods : Between May 1998 and July 2008, 529 patients (age group 2-77 years, median 28 years underwent successful device closure with an ASO at single tertiary referral cardiac center in India.. This was out of an attempted 543 cases. The procedure was carried out in catheterization laboratory under transesophageal echocardiographic and fluoroscopy guidance. The mean size of ASD was 20 mm (7-40 mm while size of septal occluder was 10-40 mm (mean 24 mm. Two devices were deployed in four patients. Three patients developed transitory pulmonary edema in immediate postprocedure period requiring ICU care for 48 hrs. All patients were advised for Aspirin (3-5 mg/kg, maximum 150 mg once daily for 6 months. In patients with device 30 mm or larger, Clopidogril ( 75 mg once daily was given for 3 months in addition to Aspirin. Clinical evaluation, echocardiogram were done on 3 months, 6 months and then at 1, 3, 5, 7 and 10 years of follow up. Transesophageal echocardiography (TEE was performed in case of any doubt on clinical evaluation or on transthoracic echocardiography (n=10. Results : Followup data is available for 496 patients (93.7%. Followup period is from 12 months to 120 months (median 56 months. On followup, device was in position in all patients, no residual shunt and no evidence of thrombosis. Interventricular septal motion normalized on day of procedure in 89% patients, in 6% over 3 months while flat septal motion persisted in 5% (n=25, all in age group > 40 years of cases, though right ventricular dilatation persisted in 10% (n=50, age more than 40 years of patients. Symptom-free survival was 96.7 % (480/496 in patients who came for followup. Only one 68 year old patient with

  5. Cardiac remodeling and effects on exercise capacity after interventional closure of atrial septal defects in different adult age groups.

    Science.gov (United States)

    Jategaonkar, Smita; Scholtz, Werner; Schmidt, Henning; Fassbender, Dieter; Horstkotte, Dieter

    2010-03-01

    Interventional closure of atrial septal defects (ASD) has become a standard procedure in pediatric and adult patients. We report immediate and mid-term results in different adult age groups. A retrospective analysis of 332 patients undergoing percutaneous ASD closure between 1998 and 2008 was performed. Beside echocardiographic and hemodynamic measurements, the NYHA functional class was assessed before and after ASD closure. The peak oxygen uptake (VO(2peak)) was available in a subgroup of 154 patients. The different age groups did not differ significantly in shunt volume or defect diameter, but a significant increase could be detected in mean pulmonary arterial pressure, mean left atrial pressure and pulmonary vascular resistance (PVR9 with increasing age). Right ventricular enlargement was present in all age groups, but the degree of enlargement increased with age. Transcatheter ASD closure was successful in 99.4%, major adverse events occurred in four patients (1.2%). At 3 months from closure, 123 patients reported an improvement, 7 of worsening and 163 of no change in their functional capacity. A significant increase in VO(2peak) could be registered in all subgroups after ASD closure. When classified by shunt volume no change could be detected in VO(2peak) in the patients with a Q(p):Q(s) 2 had a highly significant VO(2peak) increase. Patients benefit from interventional closure of hemodynamically significant ASD regardless of their age. However, the defect should be repaired as early as possible to prevent hemodynamic complications, such as the development of pulmonary hypertension and cardiac arrhythmias.

  6. Syndrome of extreme insulin resistance (Rabson-Mendenhall phenotype) with atrial septal defect: clinical presentation and treatment outcomes.

    Science.gov (United States)

    Dutta, Deep; Maisnam, Indira; Ghosh, Sujoy; Mukhopadhyay, Satinath; Chowdhury, Subhankar

    2013-01-01

    Syndrome of extreme insulin resistance (SEIR) is a rare spectrum disorder with a primary defect in insulin receptor signalling, noted primarily in children, and is often difficult to diagnose due to the clinical heterogeneity.SEIR was diagnosed in an adolescent girl with facial dysmorphism,exuberant scalp and body hair, severe acanthosis, lipoatrophy, dental abnormalities, and short stature (Rabson-Mendenhall phenotype). She had elevated fasting (422.95 pmol/L) and post-glucose insulin levels(>2083 pmol/L). Total body fat was decreased (11%; dual-energy X-ray absorptiometry). Basal growth hormone (GH) was increased (7.9 μg/L)with normal insuline-like growth factor 1 (37.6 nmol/L) suggestive of GH resistance. She had fatty liver and polycystic ovaries. Echocardiography revealed ostium secundum type atrial septal defect (ASD). Blood glucose normalized with pioglitazone (30 mg/day). Delayed development, severe insulin resistance, mild hyperglycemia, absence of ketosis, and remarkable response of hyperinsulinemia and hyperglycemia to pioglitazone which persisted even after 1 year of diagnosis are some of the notable features of this patient. This is perhaps the first report of occurrence of congenital heart disease (ASD) in a patient of SEIR (Rabson-Mendenhall phenotype). This report highlights the clinical features of SEIR and the role of insulin sensitizers like pioglitazone in the management of such patients.

  7. Atrial septal defect in adults: echocardiography and cardiopulmonary exercise capacity associated with hemodynamics before and after surgical closure.

    Science.gov (United States)

    Suchon, Elzbieta; Tracz, Wieslawa; Podolec, Piotr; Sadowski, Jerzy

    2005-10-01

    The study aimed to evaluate pre and postoperative echocardiographic data and exercise capacity in relation to age and hemodynamics in adults with atrial septal defect (ASD). Fifty-two subjects with ASD (mean age: 38.6+/-15 years) were enrolled. Echocardiography and cardiopulmonary exercise test were performed before and a year after surgery. Pre and postoperative data were analyzed for the entire group and then compared in terms of age: or=40 years, right ventricular systolic pressure (RVSP): 30 mmHg and pulmonary to systemic flow ratio (Qp/Qs): or=2.5. After surgery right ventricle dimension decreased in all patients, although it remained significantly larger in patients over 40 years. There was a negative correlation between peak oxygen uptake and preoperative RVSP (r=-0.69, PExercise capacity improved irrespective of the age at surgery, preoperative RVSP and Qp/Qs, although it failed to normalize in patients with RVSP >30 mmHg. Adults with ASD benefit from surgical closure irrespective of the actual age at surgery. Patient's age at surgery and pulmonary hypertension crucially impact the results of surgical intervention. Early defect correction is therefore highly recommendable, specifically with a view to preventing the hemodynamic consequences of ASD.

  8. 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.

    Science.gov (United States)

    Strandberg-Larsen, Katrine; Skov-Ettrup, Lise Skrubbeltrang; Grønbaek, Morten; Andersen, Anne-Marie Nybo; Olsen, Jørn; Tolstrup, Janne

    2011-07-01

    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 (VSD) or of an atrial septal defect (ASD). 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 of alcohol. Few (if any) women with an excessive/abusive intake of alcohol were enrolled into the Danish National Birth Cohort. 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 their first 3½-years of life; they included 198 infants with a VSD and 145 with an ASD. Neither the number of episodes of binge drinking nor binge drinking during three different developmental periods was associated with VSD or ASD. Women drinking ½-1½, 2, and 3+ drinks of alcohol per week had adjusted prevalence ratios of delivering an infant with a VSD of 1.22 (95% CI = 0.90-1.66); 1.38 (95% CI = 0.83-2.28); and 1.10 (95% CI = 0.54-2.23), respectively. The test for trend was 0.29. Prenatal exposure to low-to-moderate levels of alcohol on a weekly basis or occasional binge drinking during the early part of pregnancy was not statistical significantly associated with the prevalence of isolated VSD and ASD in offspring. Copyright © 2011 Wiley-Liss, Inc.

  9. Feasibility and safety of transthoracic echocardiography-guided transcatheter closure of atrial septal defects with deficient superior-anterior rims.

    Science.gov (United States)

    Li, Gui-Shuang; Li, Hai-De; Yang, Jie; Zhang, Wen-Quan; Hou, Zong-Shen; Li, Qing-Chen; Zhang, Yun

    2012-01-01

    Although previous studies showed that transthoracic echocardiography (TTE) can be used to guide transcatheter closure of atrial septal defect (ASD), whether TTE can be used to guide transcatheter closure of secundum ASD with a deficient superior-anterior rim is unknown and this critical issue was addressed in the present study. A total of 280 patients with secundum ASD who underwent transcatheter ASD closure were recruited and divided into groups A and B depending on ASD superior-anterior rim>4 mm (n = 118) or ≤4 mm (n = 162). TTE was used to guide Amplatzer-type septal occluder (ASO) positioning and assess residual shunt. Procedure success was defined as no, trivial and small residual shunt immediately after the procedure as assessed by color Doppler flow imaging. Group A and group B did not differ in complication rate (8.55% vs.7.55%), procedure success rate (98.3% vs. 95.0%) or complete closure rate immediately after the procedure (89.7% vs. 89.3%) or at 6-month follow-up (98.3% vs. 96.8%). The mean procedure and fluoroscopy time in group B were much longer than those in group A. In conclusion, the absence of a sufficient superior-anterior rim in patients undergoing percutaneous closure of secundum-type ASDs using fluoroscopic and TTE guidance is associated with slightly greater device malposition and migration as well as increased procedural and fluoroscopic times, but the overall complication rate did not differ with TTE guidance when compared to historical controls that used TEE guidance.

  10. Bronchial compression in an infant with isolated secundum atrial septal defect associated with severe pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Sung-Hee Park

    2012-08-01

    Full Text Available Symptomatic pulmonary arterial hypertension (PAH in patients with isolated atrial septal defect (ASD is rare during infancy. We report a case of isolated ASD with severe PAH in an infant who developed airway obstruction as cardiomegaly progressed. The patient presented with recurrent severe respiratory insufficiency and failure to thrive before the repair of the ASD. Echocardiography confirmed volume overload on the right side of heart and severe PAH (tricuspid regurgitation [TR] with a peak pressure gradient of 55 to 60 mmHg. The chest radiographs demonstrated severe collapse of both lung fields, and a computed tomography scan showed narrowing of the main bronchus because of an intrinsic cause, as well as a dilated pulmonary artery compressing the main bronchus on the left and the intermediate bronchus on the right. ASD patch closure was performed when the infant was 8 months old. After the repair of the ASD, echocardiography showed improvement of PAH (TR with a peak pressure gradient of 22 to 26 mmHg, and the patient has not developed recurrent respiratory infections while showing successful catch-up growth. In infants with symptomatic isolated ASD, especially in those with respiratory insufficiency associated with severe PAH, extrinsic airway compression should be considered. Correcting any congenital heart diseases in these patients may improve their symptoms.

  11. Feasibility, Safety and Long-Term Follow-Up of Transcatheter Closure of Secundum Atrial Septal Defects with Deficient Rims.

    Science.gov (United States)

    Cao, Chunhui; Wang, Zhonghua; Huang, Jun; Fan, Lingxia; Li, Ren; Wang, Shushui; Li, Yufen; Zhang, Zhiwei

    2016-01-01

    The aim of this work was to evaluate the feasibility and safety of transcatheter closure procedures for the treatment of atrial septal defects (ASDs) with insufficient rims. A total of 507 secondary ASDs were divided into two groups based on whether they had deficient rims or not (152 vs. 355 cases, respectively). Any complications, including residual shunt, heart arrhythmia, occluder translocation, etc., were followed up for 1-3 years. There were no differences in gender, weight, exposure time, ECG states, pulmonary pressure, the intervention success rate, occurrence of residual shunt, the operation time and occurrence of residual shunt during follow-up between the two groups (p > 0.05). However, the occurrence of rhythm disorders was significantly different between the two groups; ASDs with deficient rims were at an elevated risk (p rims group at 24 h postoperation, but no differences in arrhythmia incidence at any of the other follow-up time points (1, 3, 6, 12 and 36 months; p > 0.05). Patients with deficient rims experience a high success rate of ASD intervention and low rate of complications when the procedures are performed by experienced operators. © 2016 S. Karger AG, Basel.

  12. Right Ventricular Outflow Tract Velocity Time Integral Determination in 570 Healthy Children and in 52 Pediatric Atrial Septal Defect Patients.

    Science.gov (United States)

    Koestenberger, Martin; Nage, Bert; Ravekes, William; Avian, Alexander; Burmas, Ante; Grangl, Gernot; Cvirn, Gerhard; Gamillscheg, Andreas

    2015-08-01

    Determination of the right ventricular outflow tract velocity time integral (RVOT VTI) is an important part of the noninvasive investigation of pulmonary blood flow in adults; however, age-related pediatric reference data are lacking. We examined growth-related changes of RVOT VTI values in children and the predictive value of RVOT VTI values in identifying enhanced pulmonary blood flow in children with secundum type atrial septal defect (ASD). A prospective study was conducted in a group of 570 healthy children and 52 children with a moderate-sized to large ASD. We determined the effects of age, body length (BL), body weight (BW), and body surface area (BSA) on RVOT VTI values. The predictive value of normal values stratified for age, BW, BL, and BSA was tested in our 52 ASD children. RVOT VTI values ranged from mean 9.7 ± 1.2 cm in neonates to 23.3 ± 2.7 cm in children with 18 years of age and showed a positive correlation with age, BL, BSA, and BW. In our population, RVOT VTI z-scores showed a high specificity for detecting ASD patients (>97 %) with sensitivity up to 71 %. We provide normal ranges and calculated z-scores of pediatric RVOT VTI values. Normal RVOT VTI z-scores might be additional predictors in identifying increased pulmonary blood flow in patients with ASD.

  13. Cardiac magnetic resonance imaging and a rare case of an atrial myxoma causing an atrial septal defect

    Directory of Open Access Journals (Sweden)

    Matthew Grant, MD

    2017-12-01

    Full Text Available A 40 year-old athletic woman presented with worsening dyspnea on exertion over the preceding several months. Chest radiograph showed borderline cardiomegaly and subsequent echocardiography demonstrated a 5.0-cm left atrial mass as well as left-to-right interatrial shunting through a patent foramen ovale. Cardiac magnetic resonance imaging was performed, which demonstrated signal characteristics consistent with an atrial myxoma. The patient then underwent urgent surgical treatment with good technical and clinical outcome. Histologic examination confirmed an atrial myxoma. Cardiac magnetic resonance imaging was valuable in characterizing the nature of the atrial mass and patent foramen ovale, helping guide the surgical approach.

  14. Cardiac alpha-myosin (MYH6 is the predominant sarcomeric disease gene for familial atrial septal defects.

    Directory of Open Access Journals (Sweden)

    Maximilian G Posch

    Full Text Available Secundum-type atrial septal defects (ASDII account for approximately 10% of all congenital heart defects (CHD and are associated with a familial risk. Mutations in transcription factors represent a genetic source for ASDII. Yet, little is known about the role of mutations in sarcomeric genes in ASDII etiology. To assess the role of sarcomeric genes in patients with inherited ASDII, we analyzed 13 sarcomeric genes (MYH7, MYBPC3, TNNT2, TCAP, TNNI3, MYH6, TPM1, MYL2, CSRP3, ACTC1, MYL3, TNNC1, and TTN kinase region in 31 patients with familial ASDII using array-based resequencing. Genotyping of family relatives and control subjects as well as structural and homology analyses were used to evaluate the pathogenic impact of novel non-synonymous gene variants. Three novel missense mutations were found in the MYH6 gene encoding alpha-myosin heavy chain (R17H, C539R, and K543R. These mutations co-segregated with CHD in the families and were absent in 370 control alleles. Interestingly, all three MYH6 mutations are located in a highly conserved region of the alpha-myosin motor domain, which is involved in myosin-actin interaction. In addition, the cardiomyopathy related MYH6-A1004S and the MYBPC3-A833T mutations were also found in one and two unrelated subjects with ASDII, respectively. No mutations were found in the 11 other sarcomeric genes analyzed. The study indicates that sarcomeric gene mutations may represent a so far underestimated genetic source for familial recurrence of ASDII. In particular, perturbations in the MYH6 head domain seem to play a major role in the genetic origin of familial ASDII.

  15. Intracardiac echo-guided radiofrequency catheter ablation of atrial fibrillation in patients with atrial septal defect or patent foramen ovale repair: a feasibility, safety, and efficacy study.

    Science.gov (United States)

    Lakkireddy, Dhanunjaya; Rangisetty, Umamahesh; Prasad, Subramanya; Verma, Atul; Biria, Mazda; Berenbom, Loren; Pimentel, Rhea; Emert, Martin; Rosamond, Thomas; Fahmy, Tamer; Patel, Dimpi; Di Biase, Luigi; Schweikert, Robert; Burkhardt, David; Natale, Andrea

    2008-11-01

    Intracardiac Echo-Guided Radiofrequency Catheter. Patients with atrial septal defect (ASD) are at higher risk for atrial fibrillation (AF) even after repair. Transseptal access in these patients is perceived to be difficult. We describe the feasibility, safety, and efficacy of pulmonary vein antral isolation (PVAI) in these patients. We prospectively compared post-ASD/patent foramen ovale (PFO) repair patients (group I, n = 45) with age-gender-AF type matched controls (group II, n = 45). All the patients underwent PVAI through a double transseptal puncture with a roving circular mapping catheter technique guided by intracardiac echocardiography (ICE). The short-term (3 months) and long-term (12 month) failure rates were assessed. In group I, 23 (51%) had percutaneous closure devices and 22 (49%) had a surgical closure. There was no significant difference between group I and II in the baseline characteristics. Intracardiac echo-guided double transseptal access was obtained in 98% of patients in group I and in 100% of patients in group II. PVAI was performed in all patients, with right atrial flutter ablation in 7 patients in group I and in 4 patients in group II. Over a mean follow-up of 15 +/- 4 months, group I had higher short-term (18% vs 13%, P = 0.77) and long-term recurrence (24% vs 18%, P = 0.6) than group II. There was no significant difference in the perioperative complications between the two groups. Echocardiography at 3 months showed interatrial communication in 2 patients in group I and 1 patient in group II, which resolved at 12 months. Percutaneous AF ablation using double transseptal access is feasible, safe, and efficacious in patients with ASD and PFO repairs.

  16. Genome-wide association study of multiple congenital heart disease phenotypes identifies a susceptibility locus for atrial septal defect at chromosome 4p16

    Science.gov (United States)

    Cordell, Heather J.; Bentham, Jamie; Topf, Ana; Zelenika, Diana; Heath, Simon; Mamasoula, Chrysovalanto; Cosgrove, Catherine; Blue, Gillian; Granados-Riveron, Javier; Setchfield, Kerry; Thornborough, Chris; Breckpot, Jeroen; Soemedi, Rachel; Martin, Ruairidh; Rahman, Thahira J.; Hall, Darroch; van Engelen, Klaartje; Moorman, Antoon F.M.; Zwinderman, Aelko H; Barnett, Phil; Koopmann, Tamara T.; Adriaens, Michiel E.; Varro, Andras; George, Alfred L.; dos Remedios, Christobal; Bishopric, Nanette H.; Bezzina, Connie R.; O’Sullivan, John; Gewillig, Marc; Bu’Lock, Frances A.; Winlaw, David; Bhattacharya, Shoumo; Devriendt, Koen; Brook, J. David; Mulder, Barbara J.M.; Mital, Seema; Postma, Alex V.; Lathrop, G. Mark; Farrall, Martin; Goodship, Judith A.; Keavney, Bernard D.

    2013-01-01

    We carried out a genome-wide association study (GWAS) of congenital heart disease (CHD). Our discovery cohort comprised 1,995 CHD cases and 5,159 controls, and included patients from each of the three major clinical CHD categories (septal, obstructive and cyanotic defects). When all CHD phenotypes were considered together, no regions achieved genome-wide significant association. However, a region on chromosome 4p16, adjacent to the MSX1 and STX18 genes, was associated (P=9.5×10−7) with the risk of ostium secundum atrial septal defect (ASD) in the discovery cohort (N=340 cases), and this was replicated in a further 417 ASD cases and 2520 controls (replication P=5.0×10−5; OR in replication cohort 1.40 [95% CI 1.19-1.65]; combined P=2.6×10−10). Genotype accounted for ~9% of the population attributable risk of ASD. PMID:23708191

  17. Robotic-assisted closure of atrial septal defect under real-time three-dimensional echo guide: in vitro study.

    Science.gov (United States)

    Suematsu, Yoshihiro; Kiaii, Bob; Bainbridge, Daniel T; del Nido, Pedro J; Novick, Richard J

    2007-10-01

    Several advances in robotic technology and imaging systems have enabled the broad application of minimally invasive techniques in cardiac surgery. We have previously demonstrated that real-time three-dimensional echocardiography (RT3DE) provided adequate imaging and anatomic detail to act as a sole guide for surgical task performance. In this study, we examined the feasibility of robotic-assisted RT3DE-guided repair of atrial septal defect (ASD) in an in vitro study. Exp. I: An RT3DE system with x4 matrix transducer (Sonos 7500, Philips Medical Systems, Andover, MA) was compared to two-dimensional echo (2DE) in the performance of common surgical tasks with the da Vinci Robotic Surgical System (Intuitive Surgical, Sunnyvale, CA). Completion times and deviation of suture from an echogenic target (mm) were measured. Exp. II: Porcine ASDs (n=10) were created and closed with robotic-assisted direct suturing in a water bath. During all experiments the operator was blinded to the target and operated only under ultrasonic guidance. Compared to 2DE guidance, completion times improved by 70% (probotic system was significantly smaller (2DE: 4+/-2mm, 3DE: 0.2+/-0.3mm, p=0.0002) in RT3DE-guided tasks. RT3DE provided satisfactory images and sufficient anatomical detail for suturing. All surgical tasks were successfully performed with accuracy. These initial experiments demonstrate the feasibility of robotic-assisted direct closure of ASD under RT3DE guidance. An endoscopic port access approach may be possible with refinements in telemanipulator technology and further development of the transesophageal echo transducer.

  18. Clinical comparison of robotic minimally invasive surgery and transcatheter interventional occlusion for adult secundum atrial septal defect

    Directory of Open Access Journals (Sweden)

    Cheng WANG

    2016-06-01

    Full Text Available Objective  To assess the safety and efficiency of robotic minimally invasive surgery and transcatheter interventional occlusion for treatment of adult secundum atrial septal defect (ASD by comparing the early and recent postoperative follow-up results of the two minimally invasive surgery. Methods  Thirty adult patients with secundum ASD, who admitted to the General Hospital of PLA from Jan. 2008 to Dec. 2014 and received treatment of da Vinci Surgical System, were recruited as TEASD-R group, meanwhile, another 30 adult patients who received transcatheter interventional occlusion were recruited under the strict 1:1 criterion as TIASD-O group. The early postoperative complications, in-hospital conditions, recent postoperative follow-up results and the quality of life 30d and 6 months after operation were compared and retrospectively analyzed between the two groups. Results  The success rates of surgery were 100% in the both groups, no early and recent postoperative complications (residual shunt, pericardial effusion, cerebral infarction, peripheral vascular embolism, new arrhythmia, etc. were found in TEASD-R group. While some of corresponding complications existed in TISAD-O group, and the differences were of statistical significance (P<0.05 between the two groups in the incidence of postoperative new arrhythmia, tricuspid incompetence and pulmonary hypertension, as well as in the early size of right atrium and in-hospital time. SF-36 quality of life questionnaire showed that the difference of somatic pain 30d after operation was of statistical significance (P<0.05 between the two groups, but the difference disappeared 6 months after operation. Conclusion  Robotic minimally invasive surgery for adult secundum ASD is feasible, safe and efficacious since no postoperative complications occurred such as tricuspid incompetence and pulmonary hypertension, but the longer operative and inhosptial time are the shortages of the operation. DOI: 10

  19. Late infectious endocarditis of surgical patch closure of atrial septal defects diagnosed by 18F-fluorodeoxyglucose gated cardiac computed tomography (18F-FDG-PET/CT): a case report.

    Science.gov (United States)

    Honnorat, Estelle; Seng, Piseth; Riberi, Alberto; Habib, Gilbert; Stein, Andreas

    2016-08-24

    In contrast to percutaneous atrial septal occluder device, surgical patch closure of atrial defects was known to be no infective endocarditis risk. We herein report the first case of late endocarditis of surgical patch closure of atrial septal defects occurred at 47-year after surgery. On September 2014, a 56-year-old immunocompetent French Caucasian man was admitted into the Emergency Department for 3-week history of headache, acute decrease of psychomotor performance and fever at 40 °C. The diagnosis has been evoked during his admission for the management of a brain abscess and confirmed using 18F-fluorodeoxyglucose gated cardiac computed tomography (18F-FDG-PET/CT). Bacterial cultures of surgical deep samples of brain abscess were positive for Streptococcus intermedius and Aggregatibacter aphrophilus as identified by the matrix-assisted laser desorption/ionization-time of flight (MALDI-TOF) mass spectrometry and confirmed with 16S rRNA gene sequencing. The patient was treated by antibiotics for 8 weeks and surgical patch closure removal. In summary, late endocarditis on surgical patch and on percutaneous atrial septal occluder device of atrial septal defects is rare. Cardiac imaging by the 18F-fluorodeoxyglucose gated cardiac computed tomography (18F-FDG-PET/CT) could improve the diagnosis and care endocarditis on surgical patch closure of atrial septal defects while transthoracic and transesophageal echocardiography remained difficult to interpret.

  20. Ventricular septal defect.

    Science.gov (United States)

    Giboney, G S

    1983-05-01

    This article has discussed the ventricular septal defect, its occurrence, physiology, and therapy, and nursing concerns. The VSD, a communication allowing left-to-right shunting of blood at the ventricular level, is the most common congenital heart defect. Surgical correction is often required for large defects before the age of 12 months, and primary correction is now considered standard procedure. Small defects usually close spontaneously, and moderate defects are closely monitored for signs indicating the need for surgical intervention. Nursing care begins with child and family assessment and evaluation of the strengths and weaknesses of the family system. The child's developmental level is a major consideration in formulating interventions for his benefit. Play therapy is a useful vehicle in relating to the child in a nonthreatening manner preoperatively and in allowing the child to work through his hospitalization postoperatively. Maintaining the physical integrity of a child just out of the operating room is a challenge. Continuing support of the family system is a significant aspect of nursing's responsibility toward child and family. Discharge planning and intervention strive to prepare the family for the transition from hospital to home both physically and emotionally.

  1. Atrial septal defect

    Science.gov (United States)

    ... years old) ECG Heart MRI Transesophageal echocardiography (TEE) Treatment ASD may not need treatment if there are few or no symptoms, or ... often not cause problems, or will close without treatment. Larger ASDs (8 to 10 mm), often do not close ...

  2. Closure of a high ventricular septal defect after transcatheter aortic valve implantation with an atrial septal occluder-hybrid treatment for a rare complication.

    Science.gov (United States)

    Hamm, Karsten; Reents, Wilko; Kerber, Sebastian; Diegeler, Anno

    2017-03-01

    A patient with porcelain aorta underwent transcatheter aortic valve implantation with a self-expandable prosthesis for severe aortic stenosis. After postdilatation trace paravalvular regurgitation was accepted. 10 weeks later the patient returned with complete heart block and underwent pacemaker implantation. A new heart murmur prompted further investigation. A ventricular septal defect from the left ventricular outflow tract into the right ventricle was detected. It was successfully closed under direct surgical visualization and total cardiopulmonary bypass in an aortic no touch approach. Closure was accomplished with a percutaneous Amplatzer-PFO-occluder. Functional result was excellent. © The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  3. Surgical treatment of atrial septal aneurysm.

    Science.gov (United States)

    Wos, S; Bachowski, R; Domaradzki, W; Jasinski, M; Matuszewski, M; Ceglarek, W; Deja, M

    1996-12-01

    The atrial septal aneurysm (ASA) is a morphologic abnormality known to cause peripheral and pulmonary embolism. 28-52% patients with ASA have embolic events. However ASA -- with no other concomittant cardiac patology has rarely been reported as the indication for open heart surgery. In this work, five cases of patients operated for ASA are presented. Embolic complications with cerebral symptoms were presented in three cases. The diagnosis of ASA was established with the use of transesophageal echocardiography. The surgical correction of the defect was performed in extracorporeal circulation. The aneurysmal part of interatrial septum was excised and replaced with a pericardial patch. The postoperative course was uneventful. The patients have no new embolic events during the follow up period of one year.

  4. An inferior sinus venosus interatrial communication associated with a secundum atrial septal defect, clinically presenting in an adult patient: autopsy report

    Directory of Open Access Journals (Sweden)

    Silvana Maria Lovisolo

    2011-12-01

    Full Text Available Atrial septal defects (ASD are the most common congenital cardiac lesionin adults, representing up to 40% of acyanotic shunt lesions in patients olderthan 40 years. Secundum ASD comprises defects in the area of the ovalfossa, and may be associated with other types of interatrial communications.We present a case of a 25 year-old female patient who was asymptomaticuntil the age 22 when she started presenting exertion dyspnea and lowerlimbs edema, during her third pregnancy. The patient was admitted in theemergency department because of a febrile respiratory distress syndromedying in less than 36 hours after her arrival. The autopsy examination revealedpneumonia and a secundum ASD, associated with a rare form of interatrialcommunication, the inferior sinus venosus defect, which is characterized byan anomalous connection of the right pulmonary veins to the inferior caval veinat its opening in the right atrial cavity, while retaining partially their connectionto the left atrium. A dysplastic and thickened mitral valve was also present.Signs of pulmonary hypertension confirm the Eisenmenger syndrome in thisadult patient.

  5. 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...... their first 3½-years of life; they included 198 infants with a VSD and 145 with an ASD. Neither the number of episodes of binge drinking nor binge drinking during three different developmental periods was associated with VSD or ASD. Women drinking ½-1½, 2, and 3+ drinks of alcohol per week had adjusted...

  6. Oclusión transitoria de comunicación interauricular en el síndrome de Lutembacher Temporary occlusion of atrial septal defect in the Lutembacher syndrome

    Directory of Open Access Journals (Sweden)

    Alejandro E. Contreras

    2011-08-01

    Full Text Available Se describe una paciente de 82 años de edad, con síntomas de insuficiencia cardíaca avanzada e hipertensión arterial pulmonar. Un ecocardiograma transtorácico mostró una comunicación interauricular tipo ostium secundum y estenosis valvular mitral concomitante (síndrome de Lutembacher. La valoración ecocardiográfica de la enfermedad mitral se vio dificultada por la presencia del defecto interauricular. Se realizó test de oclusión percutánea transitoria de la comunicación interauricular, observándose la aparición de estenosis valvular mitral grave. El tamaño del defecto interauricular modificó las manifestaciones clínicas y el test de oclusión transitoria ayudó a decidir la conducta terapéutica.We report the case of an 82 year-old woman with symptoms of advanced heart failure and pulmonary arterial hypertension. An echocardiogram showed an ostium secundum type atrial septal defect and concomitant mitral valve stenosis (Lutembacher syndrome. Echocardiographic assessment of mitral pathology was hampered by the interatrial septal defect. Transient percutaneous occlusion test of the atrial septal defect was performed and severe mitral valve stenosis was detected. Atrial septal defect size modified the clinical manifestations and the transient occlusion test helped to decide the therapeutic strategy.

  7. Ventricular Septal Defect (VSD)

    Science.gov (United States)

    ... before getting pregnant. If you have a family history of heart defects or other genetic disorders, consider talking with a genetic counselor before getting pregnant. By Mayo Clinic Staff . Mayo Clinic Footer Legal Conditions and Terms ...

  8. The ostium primum or partial atrioventricular septal defect

    OpenAIRE

    Radermecker, Marc; Fontaine, Raphael; Limet, Raymond

    2007-01-01

    Often assimilated to simple inter-atrial communication, the ostium primum, or partial atrio-ventricular septal defect, is an entity that is characterized by a different embryological mechanism and requires some specific surgical expertise. Basically, knowledge of the morphology of the common atrioventricular valve with 5 components, the topography of the A-V node and His bundle, and the ventricular consequences of the absence of atrio-ventricular septal structures must be taken into account. ...

  9. The floating harbor syndrome with cardiac septal defect.

    Science.gov (United States)

    Lazebnik, N; McPherson, E; Rittmeyer, L J; Mulvihill, J J

    1996-12-18

    The Floating Harbor syndrome of short stature, very delayed bone age, expressive language delay, and characteristic facial changes has not been associated with cardiac anomalies, except for one patient with pulmonic stenosis. We report on a 10-year-old boy with the syndrome and tetralogy of Fallot with atrial septal defect.

  10. Ventricular septal defect in infants and children with increased pulmonary vascular resistance and pulmonary hypertension--surgical management: leaving an atrial level communication.

    Science.gov (United States)

    Khan, Inam Ullah; Ahmed, Iftikhar; Mufti, Waqar A; Rashid, Azhar; Khan, Asif Ali; Ahmed, Syed Afzal; Imran, Muhammad

    2006-01-01

    To evaluate the surgical and medical efficacy of the patients operated for Ventricular Septal Defect (VSD) with Pulmonary Hypertension and Pulmonary Vascular Resistance (PVR). Infants and children with elevated PVR and Pulmonary Hypertension are associated with significant mortality and morbidity after surgical closure. Circulatory assist devices and sophisticated medicines may not be available to help in the management of infants and children with elevated Pulmonary artery pressure and resistance. We left Patent Foramen Ovale (PFO) or made atrial communication to decrease the morbidity and mortality associated with the closure of large VSD in this risky group. Sixteen infants and children were operated with median age of 12 months, operated by the same surgeon (IU), from January' 2004 to December' 2005. They were with large VSD of elevated PVR (3.9+0.3) and underwent VSD closure leaving PFO or artificial ASD (5mm). Surgical approach was through right atrium. Post operatively, all the patients were electively ventilated for 36 hours. They were given intravenous dilators (Glyceral Trinitrate + Phentolamine) and oral Sildenafil up to 1 mg /Kg, six hourly. Five cases went into acute pulmonary hypertensive crisis postoperatively, and were rescued by Prostacycline Nebulization. Sixteen patients had VSD as the primary lesion that underwent operation. The overall early mortality was 6.25% (1/16). There have been no late deaths. Closure of large VSD with elevated PVR can be performed, leaving PFO or artificial ASD, with acceptable mortality and morbidity.

  11. Right Ventricular Outflow Tract (RVOT) Changes in Children with an Atrial Septal Defect: Focus on RVOT Velocity Time Integral, RVOT Diameter, and RVOT Systolic Excursion.

    Science.gov (United States)

    Koestenberger, Martin; Ravekes, William; Avian, Alexander; Grangl, Gernot; Burmas, Ante; Raith, Wolfgang; Cvirn, Gerhard; Grillitsch, Marlene; Gamillscheg, Andreas

    2016-09-01

    Aim of the study was to determine the influence of right heart volume overload in children with atrial septal defect (ASD) on right ventricular outflow tract (RVOT) variables. A prospective study was conducted in 115 children (age range: 2 days-18.1 years) with a moderate to large ASD. We determined effects of age, body length (BL), body weight (BW), and body surface area (BSA) on the variables RVOT diameter, RVOT velocity time integral (VTI), and RVOT systolic excursion (SE), and tested the predictive value of published normal values for age, BW, BL, and BSA in our ASD patients. In our pediatric ASD patients, the age-specific RVOT diameter (z-score: +2.2, 95% CI: 2.0-2.4, P 2.0. The age-specific RVOT VTI z-score (z-score: +3.6, 95% CI: 3.2-3.9, P 2.0. The age-specific RVOT SE z-score was not increased but slightly lower compared to normal values (z-score: -0.5, 95% CI: -0.7 to -0.3, P 2.0 while 12% of the patients had a z-score children with moderate to large ASD. © 2016, Wiley Periodicals, Inc.

  12. Fabrication and characterization of chitosan nanoparticles and collagen-loaded polyurethane nanocomposite membrane coated with heparin for atrial septal defect (ASD) closure.

    Science.gov (United States)

    Kaiser, Eva; Jaganathan, Saravana Kumar; Supriyanto, Eko; Ayyar, Manikandan

    2017-07-01

    Atrial septal defect (ASD) constitutes 30-40% of all congenital heart diseases in adults. The most common complications in the treatment of ASD are embolization of the device and thrombosis formation. In this research, an occluding patch was developed for ASD treatment using a well-known textile technology called electrospinning. For the first time, a cardiovascular occluding patch was fabricated using medical grade polyurethane (PU) loaded with bioactive agents namely chitosan nanoparticles (Cn) and collagen (Co) which is then coated with heparin (Hp). Fourier transform infrared spectrum showed characteristic vibrations of several active constituents and changes in the absorbance due to the inclusion of active ingredients in the patch. The contact angle analysis demonstrated no significant decrease in contact angle compared to the control and the composite patches. The structure of the electrospun nanocomposite (PUCnCoHp) was examined through scanning electron microscopy. A decrease in nanofiber diameter between control PU and PUCnCoHp nanocomposite was observed. Water uptake was found to be decreased for the PUCnCoHp nanocomposite against the control. The hemocompatibility properties of the PUCnCoHp ASD occluding patch was inferred through in vitro hemocompatibility tests like activated partial thromboplastin time (APTT), prothrombin time (PT) and hemolysis assay. It was found that the PT and APTT time was significantly prolonged for the fabricated PUCnCoHp ASD occluding patch compared to the control. Likewise, the hemolysis percentage was also decreased for the PUCnCoHp ASD patch against the control. In conclusion, the developed PUCnCoHp patch demonstrates potential properties to be used for ASD occlusion.

  13. Evaluation of left-to-right shunts in adults with atrial septal defect using first-pass radionuclide cardiography

    DEFF Research Database (Denmark)

    Kelbaek, H; Aldershvile, J; Svendsen, Jesper Hastrup

    1992-01-01

    outputs of the right and left ventricle was 0.04 l.min-1, the limits of agreement -0.80 to 0.88 l.min-1 and the 95% confidence interval for the bias -0.14 to 0.22 l.min-1. Right-sided cardiac catheterization was performed to assess the severity of the arterio-venous shunt by oximetry in 18 adult patients......Non-invasive determination of left-to-right shunts at the atrial level was performed by a new procedure using first-pass radionuclide measurement of cardiac output of the right and left ventricle. In 23 patients with coronary artery disease without shunt the mean difference between the cardiac...

  14. 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

  15. [A rare ventricular septal defect: a case report].

    Science.gov (United States)

    Notarangelo, Maria Francesca; Bontardelli, Federico; Taliani, Umberto; Agostinelli, Andrea; Vignali, Luigi; Ardissino, Diego

    2013-04-01

    Left ventricular-right atrial communications, known collectively as the Gerbode defect, are rare types of ventricular septal defects. Acquired forms of this defect have been described as a complication of cardiac surgery, bacterial endocarditis, chest trauma, or myocardial infarction. Diagnosis of this rare defect is challenging, but can be confirmed with echocardiography or cardiac magnetic resonance imaging. Until 6 years ago, these communications were corrected only surgically, often with relatively high mortality. However, few case reports of transcatheter closures of the defects have recently been reported with excellent results. We describe a 69-year-old patient with left ventricular-right atrial communication secondary to mitral valve surgery. The diagnosis was made by transesophageal and real-time three-dimensional echocardiography. The defect was closed percutaneously using an Amplatzer device. At follow-up, there was no residual flow and the patient improved clinically.

  16. Atrial Septal Defect (For Kids)

    Science.gov (United States)

    ... People, Places & Things That Help Feelings Q&A Movies & More Quizzes Kids' Dictionary of Medical Words En ... hole is cardiac catheterization. This method uses a thin, flexible tube called a catheter (say: KATH-uh- ...

  17. Sinus Venosus Atrial Septal Defect

    Science.gov (United States)

    2010-04-01

    the atria. The presence of PAPVR compounds the problem with additional left-to-right shunt pathways. While the exact embryology is controversial...Cardiology 2001; 87:305-09. 4. Sadley TW. Langman’s Medical Embryology , 7th ed. Williams and Wilkins 1995: 191-201. 5. Al Zaghal AM et. al

  18. Atrial Septal Defect (For Teens)

    Science.gov (United States)

    ... ASDs happen during a baby's development in the mother's womb. Before birth, the heart develops from a ... around the mouth or on the lips and tongue loss of appetite weight loss feeling lethargic a ...

  19. Effect of Clopidogrel and Aspirin vs Aspirin Alone on Migraine Headaches After Transcatheter Atrial Septal Defect Closure: The CANOA Randomized Clinical Trial.

    Science.gov (United States)

    Rodés-Cabau, Josep; Horlick, Eric; Ibrahim, Reda; Cheema, Asim N; Labinaz, Marino; Nadeem, Najaf; Osten, Mark; Côté, Mélanie; Marsal, Josep Ramon; Rivest, Donald; Marrero, Alier; Houde, Christine

    2015-11-24

    The occurrence of new-onset migraine attacks is a complication of transcatheter atrial septal defect (ASD) closure. It has been suggested that clopidogrel may reduce migraine attacks after ASD closure. To assess the efficacy of clopidogrel, used in addition to taking aspirin, for the prevention of migraine attacks following ASD closure. Randomized, double-blind clinical trial performed in 6 university hospitals in Canada. Participants were 171 patients with an indication for ASD closure and no history of migraine. Patients were randomized (1:1) to receive dual antiplatelet therapy (aspirin + clopidogrel [the clopidogrel group], n = 84) vs single antiplatelet therapy (aspirin + placebo [the placebo group], n = 87) for 3 months following transcatheter ASD closure. The first patient was enrolled in December 2008, and the last follow-up was completed in February 2015. The primary efficacy outcome was the monthly number of migraine days within the 3 months following ASD closure in the entire study population. The incidence and severity of new-onset migraine attacks, as evaluated by the Migraine Disability Assessment questionnaire, were prespecified secondary end points. A zero-inflated Poisson regression model was used for data analysis. The mean (SD) age of the participants was 49 (15) years and 62% (106) were women. Patients in the clopidogrel group had a reduced mean (SD) number of monthly migraine days within the 3 months following the procedure (0.4 [95% CI, 0.07 to 0.69] days) vs the placebo group (1.4 [95% CI, 0.54 to 2.26] days; difference, -1.02 days [95% CI, -1.94 to -0.10 days]; incident risk ratio [IRR], 0.61 [95% CI, 0.41 to 0.91]; P = .04) and a lower incidence of migraine attacks following ASD closure (9.5% for the clopidogrel group vs 21.8% for the placebo group; difference, -12.3% [95% CI, -23% to -1.6%]; odds ratio [OR], 0.38 [95% CI, 0.15 to 0.89]; P = .03). Among patients with migraines, those in the clopidogrel group had less

  20. Cerebral infarction and ventricular septal defect.

    Science.gov (United States)

    Shuiab, A

    1989-07-01

    With the availability of contrast echocardiography, patent foramen ovale is frequently detected in patients with stroke, especially in those with no clear etiology and/or the young patient with stroke. Before this report, an association of stroke with ventricular septal defect had not been reported. In this communication, we describe a 38-year-old patient who developed an occipital lobe infarction and who, on investigation, was found to have a ventricular septal defect. Other investigations, which included four-vessel cerebral angiography, collagen disease workup, and coagulation profile, were all normal. We believe this case further extends the spectrum of cerebral ischemic events that may occur with intracardiac shunts.

  1. Atrial septal aneurysm associated with additional cardiovascular comorbidities in two middle age female patients with ECG signs of right bundle branch block: two case reports.

    Science.gov (United States)

    Bakalli, Aurora; Kamberi, Lulzim; Pllana, Ejup; Gashi, Afrim

    2008-07-19

    Atrial septal aneurysm (ASA) is often associated with other atrial septal abnormalities, particularly with atrial septal defect type ostium secundum or patent foramen ovale. ECG signs of incomplete or complete right bundle branch block are known to be associated with atrial septal defects, however such correlation with other atrial septal abnormalities is not documented. We report here two cases of middle age female patients that presented with dyspnea on physical effort, right bundle branch block (RBBB) on ECG and ASA combined with other cardiac disorders. Transesophageal echocardiography revealed additional information to the ones obtained by surface echocardiography, in both cases. ASA associated with RBBB on ECG may serve as a hint for the presence of additional cardiac abnormalities, thus rousing the demand for a detailed cardiac investigation.

  2. Persistent iatrogenic atrial septal defect after a single-puncture, double-transseptal approach for pulmonary vein isolation using a remote robotic navigation system: results from a prospective study.

    Science.gov (United States)

    Rillig, Andreas; Meyerfeldt, Udo; Kunze, Markus; Birkemeyer, Ralf; Miljak, Tomislav; Jäckle, Sebastian; Hajredini, Bajram; Treusch, Fabian; Jung, Werner

    2010-03-01

    Persistent iatrogenic atrial septal defect (iASD) after transseptal puncture for pulmonary vein isolation (PVI) has been described recently as a complication of PVI. No data exists evaluating systematically the incidence and clinical implications of iASDs after PVI using a remote robotic navigation system (RNS) with sheaths with a distinct larger outer diameter. In this prospective study, 40 patients with either paroxysmal (n = 22, 55%) or persistent symptomatic atrial fibrillation were treated with circumferential PVI using an RNS. In all patients, a single-puncture, double-transseptal approach was used to access the left atrium. Transoesophageal echocardiography was performed before and the day after PVI as well as after a 3 and 6 months follow-up (FU). The day after ablation an iASD was detected in 38 of 40 (95%) patients with a mean diameter of 3.45 +/- 1.5 mm. At 6-month FU, the iASDs were closed in 30 of 39 (78.9%) patients. During the 6-month FU period, no patient died or suffered from cerebral or cardiac embolism. After a single-puncture, double-transseptal approach for PVI using the RNS, iASDs show a high spontaneous closure rate of 78.9% after a 6-month FU period. Persistent iASDs following PVI with the RNS are not associated with an increased rate of paradoxical embolism or with relevant shunting.

  3. left ventricular inflow obstruction by giant atrial septal aneurysm

    African Journals Online (AJOL)

    ANEURYSM IN A NEONATE WITH HYPOPLASTIC RIGHT HEART. SYNDROME: CASE REPORT. C. YUKO-JOWI and C. A. OKELLO. SUMMARY. 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.

  4. Left Ventricular Inflow Obstruction by Giant Atrial Septal Aneurysm in ...

    African Journals Online (AJOL)

    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 ...

  5. MULTIPLE VENTRICULAR SEPTAL DEFECTS: A NEW STRATEGY

    Directory of Open Access Journals (Sweden)

    Antonio Francesco Corno

    2013-07-01

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

  6. Circulatory adjustments after birth: effects on ventricular septal defect

    Science.gov (United States)

    Rudolph, Abraham M.

    1971-01-01

    The haemodynamic effects and clinical manifestations of congenital heart lesions may be greatly influenced by developmental changes in the circulation. The sequential changes associated with abnormal communications between the systemic and pulmonary circulations are well typified by the effect on ventricular septal defect. The possible influences of ventricular septal defect on the foetal circulation are first presented, and the interrelationship between ventricular septal defect and postnatal adjustments of the circulation are discussed. PMID:5572641

  7. Anatomical-embryological correlates in atrioventricular septal defect.

    OpenAIRE

    Allwork, S P

    1982-01-01

    Recent embryological studies have supported the consideration that the ventricular septum is multifocal in origin. These data have also provided excellent correlation of the morphology of malformed hearts with their embryology. In particular, atrioventricular septal defect correlates accurately with these observations on ventricular septation. Many of the names given to atrioventricular septal defect (for example ostium primum, persistent atrioventricular canal, endocardial cushion defect) in...

  8. 59. Early and late results of routine leaflet augmentation for complete atrio-ventricular septal defect repair

    OpenAIRE

    A. Arifi; Najm, H; Khan, A.; Ahmad, M; Khan, M A; M. Elanany

    2016-01-01

    Complete AVSD (CAVSD) is characterized by the presence of a common atrio-ventricular (AV) orifice, an inter-atrial communication, and a ventricular septal defect (VSD). Results of surgical correction of atrio-ventricular septal defects (AVSDs) have improved over the last decades; however, the need for reoperation for left atrio-ventricular valve regurgitation, after primary AVSD repair remains a major concern. The aim of our study is to assess the outcome of the routine leaflet augmentation t...

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

    Science.gov (United States)

    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.

  10. Transaortic closure of residual intramural ventricular septal defect.

    Science.gov (United States)

    Belli, E; Houyel, L; Serraf, A; Lacour-Gayet, F; Petit, J; Planché, C

    2000-05-01

    Residual intramural ventricular septal defect is an unusual cause of left-to-right shunt after biventricular repair of conotruncal anomalies. It results from the insertion of the patch within the trabeculated right ventricular free wall related to the ventriculoinfundibular fold creating a communication through the intertrabeculated spaces to the right ventricular cavity. This complication often leads to unsuccessful reoperations unless the exact mechanism of the shunt has been identified. Five patients presented with residual intramural ventricular septal defects. Three had double outlet right ventricle, one pulmonary atresia with ventricular septal defect, and one tetralogy of Fallot. One patient was unsuccessfully reoperated on for closure of the residual ventricular septal defect through the right ventricular approach. The surgical treatment, which consisted of patch closure of the residual intramural ventricular septal defect through aortotomy, was successful in 3 patients. In the 2 remaining patients the hemodynamically insignificant residual intramural ventricular septal defect remained untouched. No mortality or morbidity occurred. Residual intramural ventricular septal defect should be suspected in presence of a residual ventricular septal defect after biventricular repair of conotruncal anomalies. It is not accessible through either atriotomy or right ventriculotomy. The transaortic approach allows an easy treatment of this rare complication.

  11. Experiência inicial no fechamento percutâneo da comunicação interatrial com a prótese de Amplatzer Initial experience in percutaneous occlusion of atrial septal defects with the Amplatzer device

    Directory of Open Access Journals (Sweden)

    Valmir F. Fontes

    1998-03-01

    Full Text Available OBJETIVO: Analisar a experiência inicial no fechamento percutâneo da comunicação interatrial ostium secundum (CIA OS com a prótese de Amplatzer. MÉTODOS: Sete pacientes foram submetidos ao procedimento através da via venosa anterógrada, orientados pela ecocardiografia transesofágica (ETE e sob anestesia geral. Uma criança era portadora de 2 CIA e de canal arterial (CA. As CIA medidas pelo ETE variaram de 8,7 a 20mm. Um ecocardiograma transtorácico foi realizado na manhã seguinte do procedimento. RESULTADOS: Oito próteses foram implantadas nos 7 pacientes com sucesso. Em um paciente, o CA foi ocluído na mesma sessão com mola de Gianturco, tendo surgido taquicardia supraventricular durante a oclusão de uma das CIA, controlada com adenosina. Todos receberam alta hospitalar na manhã seguinte, com oclusão total dos defeitos. CONCLUSÃO: O procedimento mostrou-se seguro, eficaz e versátil, podendo ser considerado como uma alternativa terapêutica inicial em pacientes selecionados com CIA OS.PURPOSE: To evaluate our initial experience with percutaneous closure of secundum type atrial septal defects (ASD with the Amplatzer septal occluder. METHODS: Seven patients underwent occlusion by anterograde approach, under general anesthesia and transesophageal echocardiography (TEE guidance. One child had 2 ASD and a patent ductus arteriosus (PDA. The ASD size ranged from 8,7 to 20mm as measured by TEE. A transthoracic echocardiogram was performed in the morning after the procedure. RESULTS: Eight devices were successfully implanted in 7 patients and the PDA was occluded with a Gianturco coil at the same session. In this patient, there was an episode of supraventricular tachycardia during the occlusion of one ASD which was reverted with adenosin. All patients were discharged the day after, with complete occlusion of all defects. CONCLUSION: The procedure is safe, effective and versatile. It can be applied as an initial alternative to the

  12. Ventricular septal defect following blunt chest trauma

    Directory of Open Access Journals (Sweden)

    Lisa Ryan

    2012-01-01

    Full Text Available We present a 32-year-old male with ventricular septal defect (VSD following blunt chest trauma. Traumatic VSD is a rare but potentially life-threatening injury, the severity, course and presentation of which are variable. While the diagnosis of myocardial injury may be challenging, cardiac troponins are useful as a screening and diagnostic test. The proposed pathophysiological mechanisms in the development of traumatic VSD are early mechanical rupture and delayed inflammatory rupture. We conducted a literature review to investigate the pathogenesis, distribution of patterns of presentation, and the associated prognoses in patients with VSD following blunt chest trauma. We found that traumatic VSDs diagnosed within 48 hours were more likely to be severe, require emergency surgery and were associated with a higher mortality. Children with traumatic VSDs had an increased mortality risk. Smaller lesions may be managed conservatively but should be followed up to detect late complications. In both groups elective repair was associated with a good outcome.

  13. Five-year clinical and echocardiographic evaluation of the Das AngelWings atrial septal occluder.

    Science.gov (United States)

    Kay, Joseph D; O'Laughlin, Martin P; Ito, Kristin; Wang, Andrew; Bashore, Thomas M; Harrison, J Kevin

    2004-02-01

    The late outcome of patients treated with atrial septal occluder devices remains incompletely defined. The purpose of this study was to assess the late outcome (range 4-7 years postprocedure) of patients in whom the Das AngelWings septal occluder device was implanted in the atrial septum. We report the clinical and echocardiographic outcome, at an average of 5 years following the procedure, of patients treated with the Das AngelWings device used to close either a secundum atrial septal defect (ASD) or a patent foramen ovale (PFO). Thirty-two patients underwent successful percutaneous closure of an atrial septal closure, patent foramen ovale, or fenestration in the lateral tunnel of their Fontan with the Das AngelWings device between June 1995 and March 1998 at Duke University Medical Center. Two of the 32 patients were lost to follow-up. The remaining 30 patients were divided into 3 groups based on indication for device implantation. Group 1 consisted of 14 patients with a secundum ASD and predominantly left-to-right atrial shunting. Group 2 consisted of 8 patients who had a PFO and who suffered a thromboembolic event. Group 3 (compassionate use) consisted of 10 patients with multiple comorbid medical problems with predominantly right-to-left shunting at the atrial level causing hypoxemia. Eight of the patients in group 3 were severely ill at the time of device implantation. The 2 remaining patients in group 3 underwent AngelWings implantation for closure of right-to-left shunting through a Fontan fenestration. Mean follow-up was 59 months. There was no device embolization. No patient in the ASD or PFO/stroke group had a clinical complication. By radiographic examination, 2 of 27 patients had evidence of fracture of the nitinol framework at 2-year follow-up. Residual shunting was present in 44% at 24 hours, 20% at 1 year, and 18.8% at 2 years by use of Doppler color flow imaging and/or microcavitation echocardiographic studies. Mild mitral regurgitation caused by

  14. The influence of deficient retro-aortic rim on technical success and early adverse events following device closure of secundum atrial septal defects: An Analysis of the IMPACT Registry®.

    Science.gov (United States)

    O'Byrne, Michael L; Gillespie, Matthew J; Kennedy, Kevin F; Dori, Yoav; Rome, Jonathan J; Glatz, Andrew C

    2017-01-01

    Concern regarding aortic erosion has focused attention on the retro-aortic rim in patients undergoing device closure of atrial septal defects (ASD), but its effect on early outcomes is not well studied. A multicenter retrospective cohort study of patients undergoing device occlusion of ASD between 1/2011-10/2014 was performed, using data from the IMproving Pediatric and Adult Congenital Treatment Registry. Subjects were divided between those with retro-aortic rim technical failure and major early adverse events. Case times were measured as surrogates of technical complexity. The effect of deficient retro-aortic rim on primary outcomes was assessed using hierarchical logistic regression, adjusting for other suspected covariates and assessing whether they represent independent risk factors RESULTS: 1,564 subjects (from 77 centers) were included, with deficient retro-aortic rim present in 40%. Technical failure occurred in 91 subjects (5.8%) and a major early adverse event in 64 subjects (4.1%). Adjusting for known covariates, the presence of a deficient retro-aortic rim was not significantly associated with technical failure (OR: 1.3, 95% CI: 0.9-2.1) or major early adverse event (OR: 0.7, 95% CI: 0.4-1. 2). Total case (P = 0.01) and fluoroscopy time (P = 0.02) were greater in subjects with deficient rim, but sheath time was not significantly different (P = 0.07). Additional covariates independently associated with these outcomes were identified. Deficient retro-aortic rim was highly prevalent but not associated with increased risk of technical failure or early adverse events. Studies with longer follow-up are necessary to assess other outcomes, including device erosion. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  15. Right to Left Ventricular Diameter Ratio ≥0.42 is the Warning Flag for Suspecting Atrial Septal Defect in Preschool Children: Age- and Body Surface Area-Related Reference Values Determined by M-Mode Echocardiography.

    Science.gov (United States)

    Hashimoto, Ikuo; Watanabe, Kazuhiro; Ichida, Fukiko

    2016-04-01

    It is not always easy to observe and screen atrial septal defects (ASD) using echocardiography. In addition, there are no established echocardiographic reference indices for screening patients with ASDs. We retrospectively reviewed our database and recruited 151 isolated ASD patients and 2769 healthy subjects. In total, 307 echocardiographic studies were performed for ASD patients. Surgical repairs were done in 75 of the ASD patients. The ratio of right to left ventricular end-diastolic dimensions (RVD/LVD), which was determined by M-mode echocardiography, was used as an index of RV dilatation. After obtaining age- and body surface area (BSA)-related RVD/LVD nomograms in healthy subjects, we calculated the z-scores of RVD/LVD for all subjects and obtained the optimal cut-off values to differentiate patients with ASD from healthy subjects. The optimal cut-off values were high in neonates and gradually decreased with an increase in the age and BSA, but were almost constant in children aged >4 years or whose BSA was >0.65 m(2). The cut-off values of RVD/LVD for suspected ASD were ≥0.42 in children aged >4 years or those whose BSA was >0.65 m(2). Those for an ASD operation were ≥0.46 in those whose BSA > 0.65 m(2). The RVD/LVD determined by M-mode echocardiography is a useful index to evaluate RV dilatation in patients with ASDs. The RVD/LVD ≥ 0.42 is the warning flag for suspecting ASD in preschool children and that ≥0.46 may be a clinical important sign to determine ASD operation.

  16. Relationships of the tricuspid valve to the membranous ventricular septum in Down's syndrome without endocardial cushion defect: study of 28 specimens, 14 with a ventricular septal defect.

    Science.gov (United States)

    Rosenquist, G C; Sweeney, L J; McAllister, H A

    1975-10-01

    The commissure between the anterior and medial leaflets of the tricuspid valve is commonly absent in Down's syndrome without endocardial cushion defect (19 of 28 specimens). As a result, aneurysm of the membranous ventricular septum may develop (eight of 14 specimens with ventricular septal defect limited to the membranous ventricular septum) and the potential for left ventricle-to-right atrial communication is increased.

  17. Cryoballoon ablation for paroxysmal atrial fibrillation in the presence of an Amplatzer Septal Occluder device

    Directory of Open Access Journals (Sweden)

    Jubran A. Rind

    2016-09-01

    Full Text Available Cryoballoon ablation of the pulmonary veins (CAPV has been demonstrated to be non-inferior to radiofrequency (RF ablation for paroxysmal atrial fibrillation (AFib. As CAPV requires a larger transseptal sheath than RF ablation, it can be challenging in the presence of an Amplatzer™ Septal Occluder (ASO device. Real-time three-dimensional transesophageal echocardiography (RT3DTEE provides enhanced visualization of various complex cardiac defects and has revolutionized interventional procedures by guiding catheter positioning. We describe successful RT3DTEE guided transseptal puncture for CAPV of paroxysmal AFib in the presence of an ASO in a 53-year-old male.

  18. Outcome of transcatheter closure of muscular ventricular septal defects with the Amplatzer ventricular septal defect occluder.

    Science.gov (United States)

    Thanopoulos, B D; Rigby, M L

    2005-04-01

    To present further experience and intermediate term outcome in 30 patients with single muscular ventricular septal defects (MVSDs) who underwent transcatheter closure with the Amplatzer ventricular septal defect occluder (AVSDO). Thirty patients, aged 4 months to 16 years, with MVSDs underwent transcatheter closure with the AVSDO. The device consists of two low profile disks made of Nitinol wire mesh with a 7 mm connecting waist. The prosthesis size (waist diameter) was selected to be equal to the balloon "stretched" diameter of the defect. A 7-9 French sheath was used to deliver the AVSDO. Fluoroscopy and transoesophageal echocardiography guided the procedure. The stretched diameter of the defects ranged from 6-14 mm. The communication was completely occluded in 28 of 30 patients (93% closure rate). One patient (a 4 month old infant) with sustained complete left bundle branch block after the procedure went on to develop complete heart block one year later. No other complications were observed during a mean follow up of 2.2 years (range 0.25-4.5 years). The AVSDO is an efficient prosthesis that can be safely used in the majority of patients with a single MVSD. Further studies are required to establish long term results in a larger patient population.

  19. Spontaneous closure of muscular ventricular septal defects

    Directory of Open Access Journals (Sweden)

    Pejčić Ljiljana

    2017-01-01

    Full Text Available Introduction/Objective. Ventricular septal defect (VSD is the most frequently diagnosed congenital heart anomaly. The prognosis is usually good as it has spontaneous closure evolution, especially small muscular VSDs. The aim of this study was to determine the natural history of isolated muscular VSDs including the frequency of spontaneous closure in relation to location in the muscular septum and the age at the time of closure. Methods. The study included 96 children (52 girls and 44 boys with isolated muscular VSD diagnosed during the first month of life. We analyzed the tendency of spontaneous closure of these defects for the duration of childhood during a follow-up period of 16 years. Two-dimensional color Doppler echocardiography was performed to detect muscular VSD as a primary cardiac lesion. There was significant prevalence of small apical versus trabecular defects and their outcomes were evaluated. Results. Our study evaluated 91 children, 49 (53.8% girls and 42 (46.2% boys who did not undergo surgery. Apically located VSD was diagnosed in 68 (74.7%, while trabecular defects were found in 23 (25.3% children. Spontaneous closure occurred in 56 out of 91 cases (61.5%. The time of spontaneous closure was most commonly recorded during the first six months after birth (46.4%. The overall rate of spontaneous closure was 81.3% by the end of the first year. Apically located ventricular defects underwent spontaneous closure in the majority of patients, in comparison to trabecular ventricular defects (χ2 = 12.581; p < 0.001. Kaplan–Meier analysis demonstrated a significant difference in the average time required for spontaneous closure between the analyzed patient groups (log-rank = 9.64, p = 0.002. Conclusion. The frequency of spontaneous closure of muscular VSDs, especially apically located, is very high in the first six months, especially within the first year of life. It is advisable to detect them early on using color flow imaging and to

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

    Science.gov (United States)

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

    2016-09-01

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

  1. Ventricular septal defect due to blunt chest trauma | Olivier | South ...

    African Journals Online (AJOL)

    Blunt chest trauma may cause cardiac trauma, this possibility often being overlooked. Various anatomical structures may be affected. A case of ventr'icular septal defect due to blunt chest trauma is described and the relevant literature is reviewed.

  2. Anatomic biventricular repair in right isomerism with noncommitted ventricular septal defect.

    Science.gov (United States)

    Katewa, Ashish; Marwah, Ashutosh; Singh, Vishal; Ramaswamy, Arun; Sharma, Rajesh

    2012-07-01

    Biventricular repair in right atrial isomerism is rarely feasible due to associated anomalies of venous connection, ventricular imbalance, nonroutabilty of the interventricular communication, a common atrioventricular junction, and inadequate pulmonary arterial branches. These patients are also often not ideal for univentricular repair due to some of the above associations. We describe a novel surgical technique that was utilized in such a patient for biventricular repair of a child with right atrial isomerism with total anomalous pulmonary venous connection, regurgitant common atrioventricular valve, hypoplastic left ventricle, nonroutable ventricular septal defect, and pulmonary stenosis.

  3. Atrial septal defect as a cause of chronic cough and recurrent infections in a 4-year-old boy

    Directory of Open Access Journals (Sweden)

    Piotr Fuss

    2017-09-01

    Full Text Available Recurrent infections of the respiratory system among children are the most common reason for ambulatory treatment and one of the main causes of hospitalisation. Out of many factors that can be potentially responsible for the recurrent infections in children it is necessary to consider the ones that are connected with the immaturity of the immune system of a child and ones that can disturb the proper functioning of this system. It seems that the most important observations are those pointing at links between recurrent infections of the respiratory tract and the allergic process. The present article describes a case of a boy with recurrent respiratory infections, who was diagnosed towards immune system malfunction and allergies. An echocardiographic examination revealed a major defect of the interatrial septum. This  heart condition was treated with cardiac surgery as a result of which infections occur less frequently and do not require the hospitalisation of the patient.

  4. Association between the European GWAS-identified susceptibility locus at chromosome 4p16 and the risk of atrial septal defect: a case-control study in Southwest China and a meta-analysis.

    Directory of Open Access Journals (Sweden)

    Li Zhao

    Full Text Available Atrial septal defect (ASD is the third most frequent type of congenital heart anomaly, featuring shunting of blood between the two atria. Gene-environment interaction remains to be an acknowledged cause for ASD occurrence. A recent European genome-wide association study (GWAS of congenital heart disease (CHD identified 3 susceptibility SNPs at chromosome 4p16 associated with ASD: rs870142, rs16835979 and rs6824295. A Chinese-GWAS of CHD conducted in the corresponding period did not reveal the 3 susceptibility SNPs, but reported 2 different risk SNPs: rs2474937 and rs1531070. Therefore, we aimed to investigate the associations between the 3 European GWAS-identified susceptibility SNPs and ASD risk in the Han population in southwest China. Additionally, to increase the robustness of our current analysis, we conducted a meta-analysis combining published studies and our current case-control study. We performed association, linkage disequilibrium, and haplotype analysis among the 3 SNPs in 190 ASD cases and 225 age-, sex-, and ethnicity-matched healthy controls. Genotype and allele frequencies among the 3 SNPs showed statistically significant differences between the cases and controls. Our study found that individuals carrying the allele T of rs870142, the allele A of rs16835979, and the allele T of rs6824295 had a respective 50.1% (odds ratio (OR = 1.501, 95% confidence interval (CI = 1.122-2.009, PFDR-BH = 0.018, 48.5% (OR = 1.485, 95%CI = 1.109-1.987, PFDR-BH = 0.012, and 38.6% (OR = 1.386, 95%CI = 1.042-1.844, PFDR-BH = 0.025 increased risk to develop ASD than wild-type allele carriers in our study cohort. In the haplotype analysis, we identified a disease-risk haplotype (TAT (OR = 1.540, 95%CI = 1.030-2.380, PFDR-BH = 0.016. Our meta-analysis also showed that the investigated SNP was associated with ASD risk (combined OR (95%CI = 1.35 (1.24-1.46, P < 0.00001. Our study provides compelling evidence to motivate better understanding of the etiology

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

    Directory of Open Access Journals (Sweden)

    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?

  6. Unidirectional ventricular septal valved patch for repair of late presenting ventricular septal defect with aortopulmonary window

    Directory of Open Access Journals (Sweden)

    Neeti Makhija

    2016-01-01

    Full Text Available Management of long standing left to right shunt lesion resulting in elevated pulmonary vascular resistance (PVR is challenging. Limited surgical options are further complicated by an unpredictable postoperative period. Unidirectional valve patch (UVP closure has shown to be useful in cases of the large ventricular septal defect (VSD who present late. We report a case of large aortopulmonary window coexisting with a large VSD with severe pulmonary artery hypertension and significantly elevated PVR that was managed surgically by closure of the window by sandwich technique and closure of the septal defect with a UVP. This report emphasizes the importance of UVP in the management of such patients.

  7. Unidirectional ventricular septal valved patch for repair of late presenting ventricular septal defect with aortopulmonary window

    Science.gov (United States)

    Makhija, Neeti; Narula, Jitin; Keshri, Vikas Kumar; Gupta, Saurabh Kumar; Talwar, Sachin

    2016-01-01

    Management of long standing left to right shunt lesion resulting in elevated pulmonary vascular resistance (PVR) is challenging. Limited surgical options are further complicated by an unpredictable postoperative period. Unidirectional valve patch (UVP) closure has shown to be useful in cases of the large ventricular septal defect (VSD) who present late. We report a case of large aortopulmonary window coexisting with a large VSD with severe pulmonary artery hypertension and significantly elevated PVR that was managed surgically by closure of the window by sandwich technique and closure of the septal defect with a UVP. This report emphasizes the importance of UVP in the management of such patients. PMID:27011704

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

    African Journals Online (AJOL)

    Introduction. Ventricular septal defect (VSD) is a common congenital heart disease (CHD). Spontaneous closure of the VSD may occur, depending on the type and size of defects. This study was conducted to determine the natural history of VSD in a group of Nigerian children. Subjects and methods. Sixty-one children ...

  9. Natural history of ventricular septal defects in Nigerian children

    African Journals Online (AJOL)

    Introduction. Ventricular septal defect (VSD) is a common congenital heart disease (CHD). Spontaneous closure of the VSD may occur, depending on the type and size of defects. This study was conducted to determine the natural history of VSD in a group of Nigerian children. Subjects and methods. Sixty-one children ...

  10. 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

  11. [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.

  12. Successful extensive enlargement of a non-committed ventricular septal defect in double outlet right ventricle.

    Science.gov (United States)

    Ishibashi, Nobuyuki; Aoki, Mitsuru; Fujiwara, Tadashi

    2005-08-01

    We performed an arterial switch operation in a patient with double outlet right ventricle with non-committed ventricular septal defect, and abnormal insertion of the tension apparatus of the tricuspid valve which produced moderate tricuspid regurgitation. This required extensive enlargement of the ventricular septal defect between the attachments of the cords of the tricuspid valve so as to create the interventricular rerouting that made possible the arterial switch operation. Postoperatively, we produced a straight, unobstructed, left ventricular outflow tract, improved the extent of tricuspid regurgitation, and achieved low right atrial pressures. Enlargement of the interventricular communication can set the scene for biventricular repair in this particular subset of patients with both arterial trunks arising from the morphologically right ventricle.

  13. An Unusual Mechanism of Closure of Muscular Ventricular Septal Defects

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    Soham Dasgupta

    2017-01-01

    Full Text Available Ventricular septal defects (VSDs are the most common congenital heart defects. Most of the small or moderate size (<6 mm muscular VSDs close spontaneously within the first two years of life. The usual mechanism of spontaneous closure involves muscular tissue encroachment with superimposed fibrosis or primary fibrous tissue formation around the margins of the defect. We describe an unusual mechanism of spontaneous closure of a muscular VSD.

  14. Angiographic differentiation of type of ventricular septal defects

    Energy Technology Data Exchange (ETDEWEB)

    Cheon, Mal Soon; Park, Hee Young; Kim, Yang Sook [Marynoll Hospital, Busan (Korea, Republic of)

    1989-06-15

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

  15. Visualization of atrial septal aneurysm and patent foramen ovale by three-dimensional transesophageal echocardiography.

    Science.gov (United States)

    Najib, Mohammad Q; Ganji, Jhansi L; Chaliki, Hari P

    2016-12-01

    : Transesophageal echocardiography is frequently performed for further evaluation of sources of embolism and better evaluation of atrial septum in patients with cerebral ishemic events. Although two-dimensional transesophageal echocardiography can depict atrial septal aneurysm and patent foramen ovale, the full extent of the patent foramen ovale cannot be easily discerned in some cases. We describe a patient with transient cerebral ischemia where three-dimensional echocardiography provided incremental value when compared to two-dimensional transesophageal echocardiography in the assessment of atrial septal aneurysm and patent foramen ovale.

  16. Aspects of surgery for congenital ventricular septal defect

    NARCIS (Netherlands)

    G. Bol-Raap (Goris)

    2007-01-01

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

  17. Eventos catastróficos associados ao tratamento da comunicação interatrial tipo ostium secundum: razões para não se subestimar este tipo de cardiopatia congênita Catastrophic events associated to the surgical treatment of ostium secundum atrial septal defects: reasons for not underestimating this type of congenital cardiopathy

    Directory of Open Access Journals (Sweden)

    Paulo Roberto B. Evora

    2004-12-01

    Full Text Available OBJETIVO: O presente trabalho clínico foi motivado pela frustrante experiência de quatro pacientes operados para o tratamento cirúrgico da comunicação interatrial tipo ostium secundum (CIA-II, que vieram a falecer em condições extremamente dramáticas. MÉTODO: Estudo retrospectivo embasado em dados de prontuários. As pesquisas bibliográficas incluíram: tromboembolismo paradoxal (cerebral, pulmonar ou mesentérico, malformações vasculares do sistema nervoso central e conexões anômalas das veias cavas. Estas pesquisas da literatura foram embasadas em possíveis eventos, inesperados e catastróficos, que levaram quatro pacientes ao óbito. RESULTADOS: Os quatros pacientes, todos do sexo feminino, foram submetidos a atriosseptorrafia com tempo de parada cardíaca isquêmica inferior a 20 minutos, em circulação extracorpórea. As causas de óbito foram: isquemia intestinal não-oclusiva, ruptura de aneurisma cerebral de artéria comunicante anterior, cor pulmonale com hipertensão arterial e tromboembolismo e um provável tromboembolismo cerebral em uma criança que precisou ser reoperada pela drenagem da veia cava inferior em átrio esquerdo. CONCLUSÃO: A lição final deste trabalho é: "Não subestime a comunicação interatrial em cirurgia cardíaca!".OBJECTIVE: The present article was motivated by the frustrating experiences with four patients who underwent surgical treatment of ostium secundum atrial septal defect (ASD-II and who died in extremely dramatic circumstances. METHOD: This is a retrospective study based on clinical data. The bibliographical researche included: paradoxical thromboembolism (cerebral, lung or mesenteric, central nervous system vascular malformations and anomalous vena cava connections. This research was based on possible events, unexpected and catastrophic, that could have directly caused the patients' deaths. RESULTS: All patients were female, the operations were performed under cardiopulmonary

  18. Catheter ablation of atrial tachycardia after interatrial defect repair with patch apposition.

    Science.gov (United States)

    Nguyen, B L; Garante, C M; Tersigni, F; Sergiacomi, R; Petrassi, M; Di Matteo, A; Tufano, F; Alessandri, N

    2012-02-01

    A 54-year-old woman with history of septal atrial mixoma surgically treated and drug-refractory supraventricular tachyarrhythmia underwent catheter ablation of macro-reentry areas near the pericardial patch placed to repair an interatrial defect. The use of ablative therapy has been successful to cure this arrhythmia.

  19. Cirurgia valvar mitral e da comunicação interatrial: abordagem minimamente invasiva ou por esternotomia Mitral valve and atrial septal defect surgery: minimally invasive or sternotomy approach

    Directory of Open Access Journals (Sweden)

    Josué V. Castro Neto

    2012-08-01

    atrial septal defect (ASD. METHODS: Forty patients underwent surgery for correction of MV disease or ASD. Patients were divided into group A (GA (n=20, access by right minithoracotomy and video-assistance; and group B (GB (n=20, access by full MS. Aortic cross-clamp and cardiopulmonary bypass time, intensive care unit (ICU time, hospital stay and morbidity were compared in this prospective study . RESULTS: Fifteen patients were submitted to MV procedures and five to ASD corrections in each group. There were 9 mitral replacements (7 bioprostetic and 2 mechanical and 6 repairs in GA, and 10 (all bioprostetic and five in GB. The mean aortic cross-clamp and cardiopulmonary bypass time, in minutes, were 65.1 ± 29.3 in GA and 50.2 ± 21.4 in GB (p=0.074; and 91.8±35 in GA and 63.7±27.3 in GB ( p=0.008. The mean ICU time, in hours, were 51.7 ± 16.3 in GA and 55.8±17.5 in GB (p=0.45. The in hospital stay, in days, were 5.2 ± 1 in GA and 6.4±1.5 in GB (p=0.009. CONCLUSION: MI access for correction of the MV disease and ASD implicated in a longer cardiopulmonary bypass time for finalization of the main procedure, nevertheless it didn´t affect patient's recuperation. MI treated patients were discharged earlier than sternotomy treated patients.

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

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    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.

  1. Transcatheter device closure of a traumatic ventricular septal defect

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    Mohamed Kasem

    2014-01-01

    Full Text Available A traumatic ventricular septal defect (VSD resulting from blunt chest injury is a very rare event in children. The clinical symptoms and timing of presentation are variable, so diagnosis and management of traumatic VSD may be challenging. Decision to close the traumatic VSD is usually based on a combination of severity of heart failure symptoms, hemodynamics, and defect size. We present a case of a 7-year-old boy who was run over by a truck and presented with head and liver injury initially. He was subsequently found to have a traumatic VSD. The VSD was closed percutaneously.

  2. Right-to-left shunting through the unidirectional valved patch after closure of ventricular septal defect

    National Research Council Canada - National Science Library

    Sachin Talwar; Poonam Kapoor; Jitin Narula; Vikas Keshri; Shiv Choudhary; Balram AIran

    2017-01-01

    Postoperative transesophageal echocardiography images of a patient undergoing unidirectional valved patch closure of ventricular septal defect in the setting of severe pulmonary hypertension are presented...

  3. Acquired ventricular septal defect due to infective endocarditis

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    Randi E Durden

    2018-01-01

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

  4. 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 (p<0.001). Arrhythmias occurred in 502 patients, with a significantly higher incidence in group A than in group B (p<0.001). Among patients with arrhythmias, the incidence of AF was significantly higher in group A than group B (p<0.001). In univariate Cox analysis, a greater extent of septal hypertrophy (p<0.001), E/E´ ratio (p = 0.011), and mitral regurgitation grade (p = 0.003) were significantly associated with developing AF. In multivariate Cox analyses, a greater extent of septal hypertrophy [odds ratio (OR) 5.44 (2.29-12.92), p<0.001] in patients with HCM was significantly associated with developing AF. In conclusion, a greater extent of septal hypertrophy is an independent predictor of progression to AF in patients with HCM.

  5. 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.

  6. Right ventricular remodeling in restrictive ventricular septal defect

    Science.gov (United States)

    Monreal, Gretel; Youtz, Dane J.; Phillips, Alistair B.; Eyman, Mahala E.; Gorr, Matthew W.; Velten, Christina; Lucchesi, Pamela A.; Wold, Loren E.; Gerhardt, Mark A.

    2014-01-01

    Restrictive ventricular septal defect (rVSD) presents with little/no hemodynamic aberrations despite a patent septal defect. Clinically, these patients are observed with the hope that the defect will functionally close over time without the need for surgical repair and development of heart failure. Without evidence supporting a definitive therapeutic strategy, rVSD patients may have increased risk of a poor outcome. We tested the hypothesis that rVSD results in subclinical RV diastolic dysfunction and molecular remodeling. Five pigs underwent surgical rVSD creation. Echocardiography, hemodynamics, myocyte contractility experiments, and proteomics/Western blot were performed 6-weeks post-rVSD and in controls. *p < 0.05. LV and RV hemodynamics in rVSD were comparable to controls. The tricuspid valve early/late diastolic inflow velocity ratio (TV E/A ratio) decreased from 1.6 ± 0.05 in controls to 1.0 ± 0.08* in rVSD, indicating RV diastolic dysfunction. rVSD RV myocytes showed abnormalities in contraction (departure velocity (Vd) − 51%*, Vd time +55%*) and relaxation (return velocity (Vr) −50%*, Vr time +62%*). Mitochondrial proteins (fatty acid, TCA cycle) increased 2-fold*, indicating heightened RV work. Desmin protein upregulated 285%* in rVSD RV myocardium, suggesting cytoskeletal remodeling. rVSD causes RV diastolic dysfunction, myocyte functional impairment, and mitochondrial/cytoskeletal protein upregulation in our model. Desmin upregulation may hinder sarcomeric organization/relaxation, representing a key subclinical early marker for future RV dysfunction. TV E/A measurements are a non-invasive modality to assess rVSD patients for diastolic dysfunction. Translational research applications may lead to fundamental changes in the clinical management of rVSD by providing evidence for early repair of the defect. PMID:20637777

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

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    Saji Philip

    2017-09-01

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

  8. The surgical anatomy of double-outlet right ventricle with concordant atrioventricular connection and noncommitted ventricular septal defect.

    Science.gov (United States)

    Stellin, G; Ho, S Y; Anderson, R H; Zuberbuhler, J R; Siewers, R D

    1991-12-01

    In describing hearts with double-outlet right ventricle, we have had problems with how best to use the term noncommitted as applied to the ventricular septal defect. We reviewed, therefore, 63 hearts with double-outlet right ventricle in the setting of usual atrial arrangement and a concordant atrioventricular connection. From these, 18 hearts with potentially noncommitted defects were identified and studied in detail. The defect was unequivocally remote from the ventricular outflow tracts in 16 of these hearts, being perimembranous with excavation to open into the inlet of the right ventricle in 12, two of these also having straddling of an atrioventricular valve. One heart had a muscular defect situated in the inlet part of the muscular septum, whereas the defect was the ventricular component of an atrioventricular septal defect in the other three. In the remaining two hearts the defect was anatomically juxtaposed to a subarterial outlet. The pathway to the outflow tract, however, was obstructed by leaflets of a straddling valve. Our study shows, therefore, the need to distinguish between anatomic "commitment" of the defect from the problems in terms of commitment that may confront the surgeon in the operating room. Not only does the distance between the interventricular communication and one of the subarterial outflow tract need to be assessed (the anatomic commitment), but also the presence and nature of any intervening extraneous tissues (the surgical commitment) requires assessment.

  9. Transcatheter Device Closure of Muscular Ventricular Septal Defect

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    Yun-Ching Fu

    2011-02-01

    Full Text Available Traditional treatment of muscular ventricular septal defect (VSD is surgical closure, but it carries considerable morbidity and mortality. The Amplatzer muscular VSD occluder (AGA Medical Corporation, Plymouth, MN, USA was approved by the United States Food and Drug Administration in September 2007 and by the Taiwan Department of Health in September 2009. It is a self-expandable double-disc device made from a nitinol wire mesh. Dacron polyester patches are sewn into each disc and the connecting waist to increase the thrombogenicity of the device. Many reports have shown that transcatheter device closure of muscular VSDs is effective and safe. It has no scar, less pain, shorter hospital stay, and less cost compared to the traditional open heart surgery.

  10. Surgical treatment of partial atrioventricular septal defect: functional analysis of the mitral valve in the postoperative period

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    Josué Viana Castro Neto

    2002-11-01

    Full Text Available OBJECTIVE: To study mitral valve function in the postoperative period after correction of the partial form of atrioventricular septal defect. METHODS: Fifty patients underwent surgical correction of the partial form of atrioventricular septal defect. Their mean age was 11.8 years and 62% of the patients were males. Preoperative echocardiography showed moderate and severe mitral insufficiency in 44% of the patients. The mitral valve cleft was sutured in 45 (90% patients (group II - GII. Echocardiographies were performed in the early postoperative period, and 6 and 12 months after hospital discharge. RESULTS: The patients who had some type of arrhythmia in the postoperative period had ostium primum atrial septal defect of a larger size (2.74 x 2.08 cm. All 5 patients in group I (GI, who did not undergo closure of the cleft, had a competent mitral valve or mild mitral insufficiency in the preoperative period. One of these patients began to have moderate mitral insufficiency in the postoperative period. On the other hand, in GII, 88.8% and 82.2% of the patients had competent mitral valve or mild mitral insufficiency in the early and late postoperative periods, respectively. CONCLUSION: The mitral valve cleft was repaired in 90% of cases. Echocardiography revealed competent mitral valve or mild mitral insufficiency in 88.8% and 82.2% of GII patients in the early and late postoperative periods, respectively.

  11. An association between left axis deviation and an aneurysmal defect in children with a perimembranous ventricular septal defect.

    OpenAIRE

    Farrú-Albohaire, O; Arcil, G; Hernández, I

    1990-01-01

    Conspicuous left axis deviation was found in two thirds (27 patients) of 44 children with a perimembranous ventricular septal defect, echocardiographic signs of apposition of the septal tricuspid valve leaflet, and an aneurysm of the membranous septum. In 10 patients earlier electrocardiograms did not show left axis deviation; this feature appeared when the aneurysm of the membranous septum was first seen on the echocardiogram. None of the 44 controls with perimembranous ventricular septal de...

  12. The comparative relationships between locations of the papillary muscles and electrophysiologic QRS axis in patients with atrioventricular septal defect and common as opposed to separate orifices in the valve guarding the common atrioventricular junction.

    Science.gov (United States)

    Low, Liying; Idriss, Salim F; Anderson, Robert H; Maynard, Charles; Wagner, Galen; Hakacova, Nina

    2017-03-01

    Knowledge regarding factors that influence deviation of the QRS axis is important when seeking to differentiate between physiological and pathological changes. We hypothesised that, in contrast to those patients with an atrioventricular septal defect and common atrioventricular junction permitting only atrial shunting, those associated with ventricular shunting would show no relationship between the positions of the papillary muscles and the degree of the leftward deviation of the QRS axis. We compared the positions of endocardial origin of the papillary muscles, and the frontal plane QRS axis, in patients with atrioventricular septal defects and common atrioventricular junction permitting exclusively atrial as opposed to atrial and ventricular shunting. We analysed 18 patients with atrial and ventricular shunting and 23 patients with exclusively atrial shunting. The correlation coefficient between the ratio of distances of the papillary muscles from the mid-septum and the amount of leftward deviation in the frontal plane QRS axis was 0.1 (p=0.4) in those with ventricular shunting and 0.26 (p=0.01) in those with exclusively atrial shunting. In contrast to patients with the so-called primum form of atrioventricular septal defect, in whom the locations of the papillary muscles correlate with the degree of QRS axis, such relationships are lacking in patients with defects permitting both atrial and ventricular shunting. It may be, therefore, that the presence of ventricular shunting and/or their younger age causes pressure overload, which negates the leftward QRS forces caused by the abnormally positioned papillary muscles.

  13. MORTAL PULPOTOMY ON CHILDREN WITH VENTRICULAR SEPTAL DEFECT

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    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.

  14. 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.

  15. Gerbode ventricular septal defect diagnosed at cardiac MR imaging: case report.

    Science.gov (United States)

    Cheema, Omar M; Patel, Ankit A; Chang, Su Min; Shah, Dipan J

    2009-07-01

    This report describes the ability of cardiac magnetic resonance (MR) imaging to depict an unusual cardiac defect. A type of ventricular septal defect called the Gerbode defect, which results in a communication between the left ventricle and the right atrium, is presented. To the authors' knowledge, this is the first time cardiac MR imaging has been utilized to characterize this defect. (c) RSNA, 2009.

  16. Sequential management of post-myocardial infarction ventricular septal defects.

    Science.gov (United States)

    Trivedi, Kalyani R; Aldebert, Philippe; Riberi, Alberto; Mancini, Julien; Levy, Gilles; Macia, Jean-Christophe; Quilicci, Jacques; Habib, Gilbert; Fraisse, Alain

    2015-05-01

    Ventricular septal defect (VSD) after acute myocardial infarction is a catastrophic event. We describe our multicentre experience of a defect closure strategy that combined surgery and transcatheter closure. Data were obtained by retrospective chart review. Twenty patients (mean age, 67 years) from three centres were studied. Median time from myocardial infarction to VSD was 6 (range, 3-9) days. Acute cardiogenic shock occurred in 12 (60%) patients. Median defect diameter by echocardiography was 18 (range, 12-28) mm. Median time to first surgical or percutaneous closure was 18 (range, 4-96) days. Twenty-seven procedures were performed in the 20 patients. Surgical closure was undertaken in 14 patients and contraindicated in eight, six of whom underwent percutaneous closure; the other two, after reconsideration, proceeded to surgical closure. No procedural complications occurred with percutaneous closure. Percutaneous closure patients were older than surgical patients (75 vs. 64 years; P=0.01) and had a higher mean logistic EuroSCORE (87% vs. 67%; P=0.02). Rates of residual shunt and mortality did not differ between surgical and percutaneous patients (P=0.12 and 0.3, respectively). Those who underwent early VSD closure (<21 days after myocardial infarction) had higher rates of residual shunt (P=0.09) and mortality (P=0.01), irrespective of closure strategy. The mortality rate was also higher after early percutaneous closure (P=0.001), but not after early surgery. Finally, predicted mortality (logistic EuroSCORE) was higher than hospital mortality (≤30 days) in our patient population (75% vs. 30%; P=0.01). Vigorous pursuit of closure of post-myocardial infarction VSD with a sequential surgical and/or percutaneous approach is recommended for improved outcomes. Copyright © 2015. Published by Elsevier Masson SAS.

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

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    Sriram Sabade

    2010-01-01

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

  18. Echocardiographic diagnosis of left ventricular-right atrial communication (Gerbode-type defect) in an adult with chronic renal failure: a case report.

    Science.gov (United States)

    Eroglu, Serpil; Sade, Elif; Bozbas, Huseyin; Pirat, Bahar; Yildirir, Aylin; Muderrisoglu, Haldun

    2008-03-01

    Left ventricular-right atrial communication, known as a Gerbode-type defect, is a rare form of ventricular septal defect. It is usually congenital, but rarely acquired. Clinical presentation is associated with the volume of the shunt. Transthoracic echocardiography is the most useful diagnostic method. We present a 63-year-old man with chronic renal failure and left ventricular-right atrial shunt.

  19. Concomitant ablation for atrial fibrillation during septal myectomy in patients with hypertrophic obstructive cardiomyopathy.

    Science.gov (United States)

    Bogachev-Prokophiev, Alexander V; Afanasyev, Alexander V; Zheleznev, Sergei I; Pivkin, Alexei N; Fomenko, Michael S; Sharifulin, Ravil M; Karaskov, Alexander M

    2017-09-01

    The appearance of atrial fibrillation is associated with significant clinical deterioration in patients with obstructive hypertrophic cardiomyopathy; therefore, maintenance of sinus rhythm is desirable. Guidelines and most articles have reported the results of catheter ablation and pharmacologic atrial fibrillation treatment; nevertheless, data regarding concomitant procedures during septal myectomy are limited. The aim of this study was to assess the outcomes of concomitant atrial fibrillation treatment in patients with obstructive hypertrophic cardiomyopathy. Between 2010 and 2013 in our clinic, 187 patients with obstructive hypertrophic cardiomyopathy underwent extended myectomy. In 45 cases, concomitant Cox-Maze IV procedure was performed; however, obstructive hypertrophic cardiomyopathy was the primary indication for surgery. Atrial fibrillation was paroxysmal in 26 patients (58%) and nonparoxysmal in 19 patients (42%). The mean age of patients was 52.8 ± 14.2 years (range, 22-74 years). Mean peak gradient was 90.7 ± 24.2 mm Hg, and interventricular septum thickness was 26.1 ± 4.3 mm. Mean atrial fibrillation duration was 17.3 ± 8.5 months. There were no early deaths. No procedure-related complications occurred with regard to ablation procedure. Complete atrioventricular block was achieved in 2 patients (4.0%). Mean crossclamping time was 61 ± 36 minutes. Peak left ventricular outflow tract gradient was 12.6 ± 5.5 mm Hg based on transesophageal echocardiography. The Maze IV procedure was used for ablation in all patients (radiofrequency ablation with bipolar clamp + cryolesion for mitral and tricuspid lines). Because of the atrial wall thickness (5-6 mm), applications were performed 8 to 10 times on each line. There were no cases of pacemaker implantation due to sinus node dysfunction. All patients were discharged in stable sinus rhythm. Mean follow-up was 23.7 ± 1.3 months. The rate of atrial fibrillation freedom was 100% (45

  20. Genome-Wide Association Study of Down Syndrome-Associated Atrioventricular Septal Defects

    National Research Council Canada - National Science Library

    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

    2015-01-01

    .... 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...

  1. Coronary artery to pulmonary artery communications in pulmonary atresia with ventricular septal defect

    National Research Council Canada - National Science Library

    Sridhar, Anuradha; Subramanyan, Raghavan; Cherian, Kotturathu Mammen

    2013-01-01

    ...% of patients with pulmonary atresia and ventricular septal defect (PA-VSD). A diligent look for these abnormal communications is important to prevent perioperative complications and achieve a complete repair...

  2. Alergia al néquel manifestada como edema pulmonar no cardiogénico en paciente pos-cierre de comunicación interauricular con dispositivo tipo Amplatzer Nickel allergy manifested as noncardiogenic pulmonary edema in a patient post-closure of atrial septal defect with Amplatzer device

    Directory of Open Access Journals (Sweden)

    Luis A Gutiérrez

    2012-10-01

    Full Text Available El cierre percutáneo es la modalidad predilecta para el tratamiento de los defectos septales tipo ostium secundum cuando la anatomía es favorable, y reporta una tasa de éxito excelente así como también un bajo porcentaje de complicaciones. Se presenta el caso de un cierre exitoso de defecto septal tipo ostium secundum con dispositivo tipo Amplatzer en un paciente con antecedente de alergia a metales no detectada previamente, quien presentó edema pulmonar no cardiogénico, fiebre y pericarditis secundarios al níquel del dispositivo, pero tuvo mejoría y evolución satisfactoria con tratamiento médico.The percutaneous closure of ostium secundum septal defects is the preferred treatment modality when the anatomy is appropriate, as it shows high success and low complication rates. We present a case of a succesful percutaneous closure of an ostium secundum septal defect with an Amplatzer septal occluder device in a patient with an undetected metal allergy which led her to non cardiogenic pulmonary edema, fever and pericarditis related to the nickel contained in the device, with improvement and satisfactory evolution after medical treatment.

  3. [Intraoperative transesophageal echocardiography in robotic perimembranous ventricular septal defect repair].

    Science.gov (United States)

    Wang, Yao; Gao, Chang-Qing; Wang, Gang; Shen, Yan-Song; Wang, Jia-Li; Xiao, Cang-Song; Yang, Ming

    2013-11-01

    To explore the role of intraoperative transesophageal echocardiography (TEE) in robotic perimembranous ventricular septal defect (VSD) repair. A retrospective analysis was conducted with intraoperative TEE data of 18 consecutive patients who underwent robotic perimembranous VSD repair from January 2009 to August 2012. (1) Before cardiopulmonary bypass (CPB), TEE was performed to document the anatomic types, numbers, and the size of VSD. The procedures were predetermined by the surgeon according to TEE information. (2) During the establishment of peripheral CPB, TEE was used to guide the placement of cannulae in inferior vena cava (IVC), superior vena cava (SVC), and ascending aorta (AAO). (3) After weaning from CPB, TEE was conducted to evaluate the effect of the procedure. (1) Accuracy of TEE was 100% for diagnosing the anatomic types of VSD. All the surgical procedures were performed based on the predetermined information. (2) Under TEE guidance, all the cannulae in the SVC, IVC and AAO were located in correct positions. (3) In all patients, TEE confirmed successful VSD repair. TEE is a useful tool in the assessment of robotic perimembranous VSD repair.

  4. Cilia gene mutations cause atrioventricular septal defects by multiple mechanisms.

    Science.gov (United States)

    Burnicka-Turek, Ozanna; Steimle, Jeffrey D; Huang, Wenhui; Felker, Lindsay; Kamp, Anna; Kweon, Junghun; Peterson, Michael; Reeves, Roger H; Maslen, Cheryl L; Gruber, Peter J; Yang, Xinan H; Shendure, Jay; Moskowitz, Ivan P

    2016-07-15

    Atrioventricular septal defects (AVSDs) are a common severe form of congenital heart disease (CHD). In this study we identified deleterious non-synonymous mutations in two cilia genes, Dnah11 and Mks1, in independent N-ethyl-N-nitrosourea-induced mouse mutant lines with heritable recessive AVSDs by whole-exome sequencing. Cilia are required for left/right body axis determination and second heart field (SHF) Hedgehog (Hh) signaling, and we find that cilia mutations affect these requirements differentially. Dnah11avc4 did not disrupt SHF Hh signaling and caused AVSDs only concurrently with heterotaxy, a left/right axis abnormality. In contrast, Mks1avc6 disrupted SHF Hh signaling and caused AVSDs without heterotaxy. We performed unbiased whole-genome SHF transcriptional profiling and found that cilia motility genes were not expressed in the SHF whereas cilia structural and signaling genes were highly expressed. SHF cilia gene expression predicted the phenotypic concordance between AVSDs and heterotaxy in mice and humans with cilia gene mutations. A two-step model of cilia action accurately predicted the AVSD/heterotaxyu phenotypic expression pattern caused by cilia gene mutations. We speculate that cilia gene mutations contribute to both syndromic and non-syndromic AVSDs in humans and provide a model that predicts the phenotypic consequences of specific cilia gene mutations. © The Author 2016. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  5. Contribution of copy-number variation to Down syndrome-associated atrioventricular septal defects.

    Science.gov (United States)

    Ramachandran, Dhanya; Mulle, Jennifer G; Locke, Adam E; Bean, Lora J H; Rosser, Tracie C; Bose, Promita; Dooley, Kenneth J; Cua, Clifford L; Capone, George T; Reeves, Roger H; Maslen, Cheryl L; Cutler, David J; Sherman, Stephanie L; Zwick, Michael E

    2015-07-01

    The goal of this study was to identify the contribution of large copy-number variants to Down syndrome-associated atrioventricular septal defects, the risk for which in the trisomic population is 2,000-fold more as compared with that of the general disomic population. Genome-wide copy-number variant analysis was performed on 452 individuals with Down syndrome (210 cases with complete atrioventricular septal defects; 242 controls with structurally normal hearts) using Affymetrix SNP 6.0 arrays, making this the largest heart study conducted to date on a trisomic background. Large, common copy-number variants with substantial effect sizes (OR > 2.0) do not account for the increased risk observed in Down syndrome-associated atrioventricular septal defects. By contrast, cases had a greater burden of large, rare deletions (P Down syndrome-associated atrioventricular septal defects, whereas large, common copy-number variants do not appear to increase the risk of Down syndrome-associated atrioventricular septal defects. The genetic architecture of atrioventricular septal defects is complex and multifactorial in nature.

  6. 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.

  7. A modified infarct exclusion technique for repair of anteroapical postinfarction ventricular septal defect.

    Science.gov (United States)

    Bayezid, Omer; Turkay, Cengiz; Golbasi, Ilihan

    2005-01-01

    Ventricular septal defects complicate approximately 1% to 2% of cases of acute myocardial infarction. Such postinfarction defects require urgent surgical treatment because, on medical treatment alone, 60% to 70% of patients die within the first 2 weeks. Despite the development of various surgical techniques for repair of postinfarction ventricular septal defect, the condition carries a high risk of recurrence and subsequent death. We describe a modification of the infarct exclusion technique in which the septal portion of the patch is reinforced by the right ventricular free wall. This modification appears to prevent leaks to the right ventricle through the ventricular septal defect, from anywhere around the patch. We applied this modified technique to 4 patients with anteroapical postinfarction ventricular septal defect. There was 1 early death, due to mesenteric artery occlusion secondary to embolus. No residual shunt was found during the postoperative period. We believe that our modification to the infarct exclusion technique might reduce both operative mortality and recurrence, by supporting friable endocardial tissue with right ventricular wall. We suggest that it be considered for use in patients with anteroapical ventricular septal defect and no severe right ventricular dysfunction.

  8. Transcatheter closure of perimembranous ventricular septal defects in infants and children using the Amplatzer perimembranous ventricular septal defect occluder.

    Science.gov (United States)

    Thanopoulos, Basil Vasilios D; Rigby, Michael L; Karanasios, Evangelos; Stefanadis, Christodoulos; Blom, Nico; Ottenkamp, Jaap; Zarayelyan, Armine

    2007-04-01

    There are very few published reports of the transcatheter closure of perimembranous ventricular septal defects (PMVSDs) using the Amplatzer PMVSD occluder with encouraging initial results. This report presents initial and 1-year results from 54 patients with PMVSDs who underwent transcatheter closure at 5 different institutions with the Amplatzer PMVSD occluder. Sixty-five patients with PMVSDs were enrolled at 5 European centers. Eleven of the 65 patients did not fulfill the patient selection criteria at the initial echocardiographic evaluation or at cardiac catheterization. As a result, a total of 54 patients underwent attempted transcatheter closure using the Amplatzer PMVSD occluder. The median age of the patients was 5.1+/-3.6 years (range 0.3 to 13), and the median weight 18.5+/-10.3 kg (range 5 to 45). Devices were permanently implanted in 49 of 54 patients. Complete occlusion of the communication at 1-year follow-up was observed in 46 of 49 patients (94%). Main early procedural complications included (1) device embolization (2 patients), (2) severe bradycardia with hemodynamic compromise (2 patients), and (3) Mobitz II (2:1) heart block (1 patient). Late procedural complications included complete heart block (1 patient). No other complications were observed during follow-up. In conclusion, the Amplatzer PMVSD occluder is promising device that can be used for transcatheter closure in selected patients with PMVSDs. Further studies and long-term follow-up are required before this technique enters routine clinical practice.

  9. Intramural Ventricular Septal Defect Is a Distinct Clinical Entity Associated With Postoperative Morbidity in Children After Repair of Conotruncal Anomalies

    National Research Council Canada - National Science Library

    Patel, Jyoti K; Glatz, Andrew C; Ghosh, Reena M; Jones, Shannon M; Natarajan, Shobha; Ravishankar, Chitra; Mascio, Christopher E; Spray, Thomas L; Cohen, Meryl S

    2015-01-01

    BACKGROUND—Intramural ventricular septal defects (VSDs) are interventricular communications through right ventricular free wall trabeculations that can occur after repair of conotruncal anomalies...

  10. Dispersion of P wave duration and P wave vector in patients with atrial septal aneurysm.

    Science.gov (United States)

    Janion, Marianna; Kurzawski, Jacek; Sielski, Janusz; Ciuraszkiewicz, Katarzyna; Sadowski, Marcin; Radomska, Edyta

    2007-07-01

    Atrial septal aneurysm (ASA) may be involved in the genesis of atrial arrhythmias as a consequence of disturbances in the propagation of depolarization, which may be easily assessed by P wave dispersion measurement. The aim of this study is to assess the dispersion of P wave duration and P wave vector in patients with ASA and to determine the effect of associated interatrial shunt on the magnitude of P wave dispersion. The study population consisted of 23 healthy volunteers and 88 patients with ASA base more than 15 mm and protrusion more than 7.5 mm. The size of aneurysms and atria was determined by echocardiography and P wave dispersion was measured on the surface ECG. In ASA patients, dispersion of P wave duration was significantly increased when compared with healthy controls (7.8 +/- 12.1 vs. 3.7 +/- 3.5 ms; P wave vector was also significantly increased (8.5 +/- 10.1 degrees vs. 4.6 +/- 3.6 degrees ; P wave duration and P wave vector. Variation in P wave duration was significantly correlated with the dispersion of P wave vector and age of these patients. Dispersion of P wave vector was significantly decreased in ASA patients with interatrial shunt. P wave dispersion in ASA patients may predispose to the development of atrial arrhythmias.

  11. 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. Copyright © 2015 Ramachandran et al.

  12. Percutaneous closure of large VSD using a home-made fenestrated atrial septal occluder in 18-year-old with pulmonary hypertension.

    Science.gov (United States)

    Kamali, Hacer; Saritas, Turkay; Erdem, Abdullah; Akdeniz, Celal; Tuzcu, Volkan

    2014-06-09

    Hemodynamically significant muscular ventricular septal defects in children after the infantile period are a rare occurrence and ideal for transcatheter closure. In cases of severe concomitant pulmonary hypertension, it may be necessary to fenestrate the device. In this report, we present an 18-year old patient with a large mid-trabecular ventricular septal defect and severe pulmonary hypertension that underwent percutaneous closure of the defect with a home-made fenestrated atrial septal occluder. An 18-year-old female patient referred to us with complaints of dyspnea (NYHA score of 2-3). Physical examination revealed an apical rumble and a harsh second heart sound. Echocardiographic examination revealed a large mid-trabecular ventricular septal defect with bidirectional shunt and the widest diameter measuring 22 mm on 2D echocardiography. Left and right heart cavities were enlarged. Before and after the vasoreactivity test performed during cardiac catheterization, average aortic pressure was 65 → 86 mmHg, average pulmonary artery pressure: 58 → 73 mmHg, Qp/Qs: 1.6 → 3.2, PVR: 4.6 → 4.3 Wood/U/m2 and PVR/SVR: 0.5 → 0.2. On left-ventricular angiocardiogram, the largest end-diastolic defect diameter was 21 mm. The closure procedure was performed with transthoracic echocardiographic guidance, using a 24 mm Cera septal occluder and a 14 F sheath dilator to make a 4.5-5 mm opening. Measured immediately after the procedure and during cardiac catheterization one month later, average aortic pressure was 75 → 75 mmHg, average pulmonary artery pressure: 66 → 30 mmHg, Qp/Qs 1.5 → 1.4, PVR: 4.4 → 2.9 Wood/U/m2 and PVR/SVR: 0.4 → 0.2. Transthoracic echocardiographic examination performed 24 hours after the procedure showed a max 35-40 mmHg gradient between the left and right ventricles through the fenestration. After the procedure, we observed sporadic early ventricular systoles and a nodal rhythm disorder

  13. Risk Factors for Delayed Extubation after Ventricular Septal Defect Closure: a Prospective Observational Study

    Directory of Open Access Journals (Sweden)

    Divyakant Parmar

    Full Text Available Objective: The objective of our study was to determine the feasibility of early extubation and to identify the risk factors for delayed extubation in pediatric patients operated for ventricular septal defect closure. Methods: A prospective, observational study was carried out at our Institute. This study involved consecutive 135 patients undergoing ventricular septal defect closure. Patients were extubated if feasible within six hours after surgery. Based on duration of extubation, patients were divided two groups: Group 1= extubation time ≤ 6 hours, Group 2= extubation time >6 hours. Results: A total of 99 patients were in Group 1 and 36 patients in Group 2. Duration of ventilation was 4.4±0.9 hours in Group 1 and 25.9±24.9 hours in Group 2 (P<0.001. Univariate analysis showed that young age, low weight, low partial pressure of oxygen, trisomy 21, multiple ventricular septal defect, high vasoactive inotropic score, transient heart block and low cardiac output syndrome were associated with delayed extubation. However, regression analysis revealed that only trisomy 21 (OR: 0.248; 95%CI: 0.176-0.701; P=0.001, low cardiac output syndrome (OR: 0.291; 95%CI: 0.267-0.979; P=0.001, multiple ventricular septal defect (OR: 0.243; 95%CI: 0.147-0.606; P=0.002 and vasoactive inotropic score (OR: 0.174 95%CI: 0.002-0.062; P=0.039 are strongest predictors for delayed extubation. Conclusion: Trisomy 21, low cardiac output syndrome, multiple ventricular septal defect and high vasoactive inotropic score are significant risk factors for delay in extubation. Age, weight, pulmonary artery hypertension, size of ventricular septal defect, aortic cross-clamp and cardiopulmonary bypass time did not affect early extubation.

  14. Familial Atrial Septal Defect and Sudden Cardiac Death

    DEFF Research Database (Denmark)

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

    2016-01-01

    disturbances, cardiomyopathies, complex CHD, and sudden cardiac death as well. Here, we show that NKX2-5 mutations primarily occur in ASD patients with conduction disturbances and heritable ASD. Furthermore, these families are at increased risk of sudden cardiac death. RESULTS: We screened 39 probands.......1 × 10(-9) ). The majority of patients (74%) had ASD with conduction disturbance. Nineteen patients (15%) of 120 with familial ASD and conduction disturbance died from sudden cardiac death of which nine (8%) were confirmed mutation carriers, and 10 were possible carriers. CONCLUSIONS: NKX2-5 mutations...... mainly occur in familial CHD, the signature phenotype is ASD with conduction disturbances and mutation carriers are at increased risk of sudden cardiac death. We suggest that familial ASD patients should be screened for NKX2-5 mutations and, if they are mutation carriers, implantation of an implantable...

  15. Assessment of muscular ventricular septal defect closure by transcatheter or surgical approach: a three-dimensional echocardiographic study.

    Science.gov (United States)

    Acar, P; Abdel-Massih, T; Douste-Blazy, M-Y; Dulac, Y; Bonhoeffer, P; Sidi, D

    2002-09-01

    Previous classification of muscular ventricular septal defects (VSDs) visualized on two-dimensional echocardiography relied on artificial divisions of the septum. New visualization of the ventricular septum integrating the third dimension would facilitate communication between cardiologists and surgeons. The objectives of this study were (1) to assess in patients with muscular ventricular septal defects the accuracy of left ventricular three-dimensional echocardiographic reconstructions in demonstrating the position, the size and the tissue rims of the defects; (2) to compare findings by three-dimensional echocardiography with those obtained by surgical and transcatheter approaches. Twenty-six patients, aged from one month to 40 years, with muscular ventricular septal defects underwent three-dimensional echocardiographic study. From the left ventricular three-dimensional echocardiographic reconstructions, the localization, the maximal diameter and the tissue rim of the defect were analysed and compared with surgical or transcatheter findings. Optimal three-dimensional echocardiographic reconstructions were obtained in 22 patients. Nineteen had a single muscular ventricular septal defect and three had multiple muscular ventricular septal defects. The muscular ventricular septal defect localizations were the inlet septum in three, the outlet septum in three, the mid-muscular septum in 14 and the apex in eighth. In 10 patients who underwent surgical closure, the correlation between three-dimensional echocardiography and surgery for muscular ventricular septal defect maximal diameter was y=0 x 95 x +0.13 (r=0.98; Pventricular septal defect localization were complete. In five patients who underwent transcatheter closure, the mean difference between three-dimensional echocardiographic maximal diameter and stretched diameter was 1 x 8+/-0 x 5 mm. The three-dimensional echocardiographic left ventricular views provide a new and easily communicated visualization of various

  16. RESIDUAL DEFECTS AFTER SURGICAL REPAIR OF VENTRICULAR SEPTAL DEFECTS IN CHILDREN:

    Directory of Open Access Journals (Sweden)

    K Sayadpour-Zanjani

    2008-12-01

    Full Text Available "nResidual ventricular septal defects (VSD are major complications after cardiac surgery. We studied the incidence of this complication, risk factors for its occurrence and short-term follow-up in 179 pediatric patients that underwent surgical closure of VSD from April 2003 until May 2004. All data were gathered retrospectively except measurements of shunt ratio. Studied risk factors included age, sex, weight, height, ejection fraction, VSD size, presence of pulmonary stenosis (PS, responsible surgeon, use of patch material for closing VSD, mean degree of hypothermia, cardiopulmonary bypass and aortic cross-clamp times, hemorrhage, documented infection, and surgical approach for defect closure. The incidence of all residual VSDs was 56% and significant ones (i.e. with Qp/Qs > 1.5 22%. The only statistically significant risk factors were higher age, weigh and height of the patients. There was notable but statistically insignificant differences in residual shunt incidence among the patients of different surgeons and with the use of different patch materials. During the median follow-up period of 9.5 months, 35% of the residual defects were closed spontaneously. Six patients underwent catheterization, three of which were candidates of residual VSD closure. As residual VSD is a hemodynamically and psychologically important complication, we recommend VSD closure at lower age and the use of intraoperative epicardial or transesophageal echocardiography to minimize its occurrence.

  17. Exome sequencing identifies rare variants in multiple genes in atrioventricular septal defect

    NARCIS (Netherlands)

    D'Alessandro, Lisa C. A.; Al Turki, Saeed; Manickaraj, Ashok Kumar; Manase, Dorin; Mulder, Barbara J. M.; Bergin, Lynn; Rosenberg, Herschel C.; Mondal, Tapas; Gordon, Elaine; Lougheed, Jane; Smythe, John; Devriendt, Koen; Bhattacharya, Shoumo; Watkins, Hugh; Bentham, Jamie; Bowdin, Sarah; Hurles, Matthew E.; Mital, Seema

    2016-01-01

    The genetic etiology of atrioventricular septal defect (AVSD) is unknown in 40% cases. Conventional sequencing and arrays have identified the etiology in only a minority of nonsyndromic individuals with AVSD. Whole-exome sequencing was performed in 81 unrelated probands with AVSD to identify

  18. Development of the cardiac conduction system in atrioventricular septal defect in human trisomy 21

    NARCIS (Netherlands)

    Blom, Nico A.; Ottenkamp, Jaap; Deruiter, Marco C.; Wenink, Arnold C. G.; Gittenberger-de Groot, Adriana C.

    2005-01-01

    In patients with atrioventricular septal defect (AVSD), the occurrence of nonsurgical AV block has been reported. We have looked for an explanation in the development of the AV conduction system. Human embryos with AVSD and trisomy 21 and normal embryos were examined (age 5-16 wk gestation).

  19. Atrioventricular septal defect : advanced imaging from early development to long-term follow-up

    NARCIS (Netherlands)

    Calkoen, Emmeline E.

    2016-01-01

    The aim of this thesis is to review the current knowledge on atrioventricular septal defect (AVSD) (Part 1), to study the pathogenesis of AVSD (Part 2) and finally to analyze cardiac outcome long-term after AVSD correction (Part 3). Studies are performed with novel imaging techniques. In part 2 it

  20. Extracorporeal membrane oxygenation in a child with traumatic ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Jennifer Y. Lam

    2018-01-01

    Full Text Available Traumatic ventricular septal defect is an uncommon event following blunt thoracic trauma. Within the pediatric trauma literature, extracorporeal membrane oxygenation is most commonly used for secondary acute respiratory distress syndrome. We present the first account of rescue extracorporeal membrane oxygenation to allow for safe transport and access to definitive operative repair in the setting of blunt cardiac injury.

  1. Exercise capacity and participation of children with a ventricular septal defect.

    NARCIS (Netherlands)

    Binkhorst, M.; Belt, T van de; Hoog, M. de; Dijk, Arie van; Schokking, M.; Hopman, M.

    2008-01-01

    Existing data on exercise performance in children with a ventricular septal defect (VSD) are scarce and inconclusive. We aimed to elucidate whether and why exercise capacity and physical activity level are decreased in children with VSD. Children 9 to 17 years of age with a surgically (operated, n =

  2. Repair of Posterior Infarct Ventricular Septal Defect in a Patient with Dextrocardia and Situs Inversus.

    Science.gov (United States)

    Nesta, Marialisa; Mazza, Andrea; Perri, Gianluigi; Bruno, Piergiorgio; Massetti, Massimo

    2016-03-01

    We report a patient with situs inversus who developed a large posterior interventricular septum pseudoaneurysm with a septal defect following a myocardial infarction. The ventricular septum was approached through the left ventricle and the entrance of the pseudoaneurysm was repaired with a strip of equine pericardium. © 2016 Wiley Periodicals, Inc.

  3. [Aneurysm of the membranous ventricular septum with ventricular septal defect, mitral and tricuspid insufficiency].

    Science.gov (United States)

    Tanaka, K; Yasunaga, H; Egashira, A; Kumate, M; Kawara, T; Kosuga, K

    1998-10-01

    A seventy-year-old man was admitted at our hospital because of dyspnea. Echocardiogram and left ventriculogram showed an aneurysm formation of the membranous ventricular septum and small left-to-right shunt through ventricular septum defect and also severe mitral and tricuspid insufficiency. Operation was performed after medical therapy for congestive heart failure. During operation, mitral leaflets showed no organic lesions nor prolapse, but the annulus was dilated. The cause of mitral insufficiency, we thought, might be congenital, and the annulus dilatation was caused of mitral insufficiency, we thought, might be congenital, and the annulus dilatation was caused to produce tricuspid insufficiency secondary. The ventricular septal communication became small (diameter; 5 mm) and was associated with aneurysm formation of the remaining portion of the membranous septum. And the aneurysm, protruding to the septal leaflet of tricuspid valves, enhanced tricuspid insufficiency. It was reported by many authors that the aneurysm formation was related to spontaneous closure of ventricular septal defect. Patients with small ventricular septal defect, without any symptoms, must be followed intensively, or they might get cardiac complications, such as arrhythmia, right ventricular outflow obstruction, tricuspid insufficiency, and so on.

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

    African Journals Online (AJOL)

    dimensional echocardiographic examination revealed a mid‑muscular VSD. The connection between the defect and the trauma was not initially appreciated. Facilities for required urgent open‑heart surgery were not available. Cardiac failure ...

  5. Percutaneous closure of perimembranous ventricular septal defect with an Amplatzer Duct Occluder in a dextrocardia patient.

    Science.gov (United States)

    Ergene, Oktay; Nazli, Cem; Kocabas, Ugur; Duygu, Hamza; Akyildiz, Zehra Ilke; Hijazi, Ziyad M

    2011-07-15

    In this article we report an unusual case of dextrocardia patient with perimembranous ventricular septal defect (VSD) whose defect is closed by percutaneous method with Amplatzer Duct Occluder-II device. To our best knowledge, this was the first time this device has been used to close a membranous defect in a patient with dextrocardia. Our case demonstrates the feasibility of percutaneous VSD closure in challenging patients by using appropiate techniques and devices for particular patients. Copyright © 2009 Elsevier Ireland Ltd. All rights reserved.

  6. Can Computer Tomography Predict Compromise of Cardiac Structures After Percutaneous Closure of Interatrial Septal Defects?

    Science.gov (United States)

    Wagdi, Philipp

    2011-12-01

    Erosion of a cardiac structure after device closure of an interatrial septal communication (IASC-C), although rare, is a major and severe adverse event which may be underreported. On the other hand, unexplained episodes of transient chest pain occur more often and may be quite distressing. We sought to define the parameters relating the devices to the adjacent cardiac structures and to determine whether computer tomography (CT) could predict erosion of atrial or aortic wall or precordial pain symptoms occurring in the first months after device implantation. Retrospective observational study of 20 patients who underwent CT for de novo chest pain occurring after IASC-C or as a diagnostic test for suspected or proven coronary artery disease (CAD). Clinical follow up was for 20.5 ± 17.6 (6-84) months. CT was done 18 ± 10 (2-28) weeks after IASC-C. Indentation of the aortic root was found in 11 (55%) patients, the left atrial wall in 13 (65%) and the right atrial wall in eight (40%) of patients. Contact without indentation was found in nine (45%), 6 (30%) and 11 (55%) of patients respectively. Device indenting of the left and right atrial, as well as the aortic wall, occured in the majority of the patients examined after IASC-C. This finding may explain bouts of chest pain after the intervention in some patients, but does not predict clinically relevant erosion of a cardiac structure.

  7. Hemolytic anemia after atrioventricular septal defect repair without synthetic material.

    Science.gov (United States)

    Tsang, J C; Shum-Tim, D; Tchervenkov, C I; Jutras, L; Sinclair, B

    1999-11-01

    We report a rare case of severe hemolytic anemia following repair of a congenital heart defect without the use of prosthetic material. A review of the literature, diagnosis, and management are described. Although this is an unusual complication following congenital heart surgery, a high index of suspicion must be maintained and a possible mechanical cause should be sought and corrected.

  8. Pregnancy in peritoneal dialysis and an infant with a ventricular septal defect

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    Abdullah Alhwiesh

    2015-01-01

    Full Text Available Fertility is markedly reduced in dialysis patients. Estimates of the frequency of conception in dialysis patients range from 1.4% per year in Saudi Arabia to 0.5% in the United States. The reasons for the rarity of pregnancy in dialysis patients are not well understood. In addition, there is a marked increase in the risk of pre-eclampsia, hydramnios, hypertension crisis, early uterine contractions and pre-term delivery. Herein, we report a 38-year-old Saudi woman with chronic renal failure who completed the full term of pregnancy uneventfully on peritoneal dialysis. Using a biocompatible dialysate solution, adequate metabolic and blood pressure control were achieved during pregnancy. The delivered infant was small for gestational age and was born with a ventricular-septal defect. To the best of our knowledge, this is the first case report in the literature of ventricular-septal defect in an infant born to a mother on peritoneal dialysis.

  9. Post-Myocardial Infarction Ventricular Septal Defect Six Months following Coronary Artery Bypass Grafting.

    Science.gov (United States)

    Fiedler, Amy G; Sundt Iii, Thoralf M; Tolis, George

    2017-08-25

    Mechanical complications following acute myocardial infarction are associated with high mortality. We present the first reported case of a new post myocardial infarction ventricular septal defect (VSD) within six months of coronary artery bypass grafting. The patient underwent successful surgical correction of the VSD with the assistance of mechanical circulatory support (MCS). This case highlights the importance of mechanical circulatory support in the management of cardiogenic shock associated with rare complications of myocardial infarction, even after surgical revascularization.

  10. Ventricular septal defect and double-chambered right ventricle in an alpaca.

    Science.gov (United States)

    Poser, Helen; Dalla Pria, Angela; De Benedictis, Giulia M; Stelletta, Calogero; Berlanda, Michele; Guglielmini, Carlo

    2015-03-01

    A 20-month-old male alpaca was referred for evaluation of a cardiac murmur evident since birth. Echocardiography identified a ventricular septal defect (VSD) and a fibro-muscular band causing a stenosis of the right ventricular outflow tract. Right ventricular catheterization and selective angiography confirmed the diagnosis of VSD and double-chambered right ventricle with bidirectional shunting. Copyright © 2014 Elsevier B.V. All rights reserved.

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

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    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.

  12. Left atrial accessory appendages, diverticula, and left-sided septal pouch in multi-slice computed tomography. Association with atrial fibrillation and cerebrovascular accidents.

    Science.gov (United States)

    Hołda, Mateusz K; Koziej, Mateusz; Wszołek, Karolina; Pawlik, Wiesław; Krawczyk-Ożóg, Agata; Sorysz, Danuta; Łoboda, Piotr; Kuźma, Katarzyna; Kuniewicz, Marcin; Lelakowski, Jacek; Dudek, Dariusz; Klimek-Piotrowska, Wiesława

    2017-10-01

    The aim of this study is to provide a morphometric description of the left-sided septal pouch (LSSP), left atrial accessory appendages, and diverticula using cardiac multi-slice computed tomography (MSCT) and to compare results between patient subgroups. Two hundred and ninety four patients (42.9% females) with a mean of 69.4±13.1years of age were investigated using MSCT. The presence of the LSSP, left atrial accessory appendages, and diverticula was evaluated. Multiple logistic regression analysis was performed to check whether the presence of additional left atrial structures is associated with increased risk of atrial fibrillation and cerebrovascular accidents. At least one additional left atrial structure was present in 51.7% of patients. A single LSSP, left atrial diverticulum, and accessory appendage were present in 35.7%, 16.0%, and 4.1% of patients, respectively. After adjusting for other risk factors via multiple logistic regression, patients with LSSP are more likely to have atrial fibrillation (OR=2.00, 95% CI=1.14-3.48, p=0.01). The presence of a LSSP was found to be associated with an increased risk of transient ischemic attack using multiple logistic regression analysis after adjustment for other risk factors (OR=3.88, 95% CI=1.10-13.69, p=0.03). In conclusion LSSPs, accessory appendages, and diverticula are highly prevalent anatomic structures within the left atrium, which could be easily identified by MSCT. The presence of LSSP is associated with increased risk for atrial fibrillation and transient ischemic attack. Copyright © 2017 Elsevier B.V. All rights reserved.

  13. Familial ebstein anomaly, left ventricular hypertrabeculation, and ventricular septal defect associated with a MYH7 mutation.

    Science.gov (United States)

    Bettinelli, Audra L; Mulder, Theodorus J; Funke, Birgit H; Lafferty, Katherine A; Longo, Sherri A; Niyazov, Dmitriy M

    2013-12-01

    Ebstein anomaly is a rare congenital heart defect that most often occurs sporadically within a kindred. Familial cases, although reported, are uncommon. At this time, the genetic etiology of Ebstein anomaly is not fully elucidated. Here, we describe clinical and molecular investigations of a rare case of familial Ebstein anomaly in association with a likely pathogenic mutation of the MYH7 gene. The severity of presentation varies, and Ebstein anomaly can be observed in association with such other heart defects as ventricular septal defect and left ventricular (LV) hypertrabeculation, as seen in our family of study. In our family of study, the 31-year-old father and four of his children have been diagnosed with Ebstein anomaly. Genetic testing revealed that the father was heterozygous for the Glu1220del variant detected in exon 27 of the MYH7 gene. The MYH7 gene encodes the β-myosin heavy chain and is expressed in cardiac muscle. DNA sequencing of three of his affected children confirmed that they carried the same variant while the fourth affected child was not available for testing. This is the first report of familial Ebstein anomaly associated with the Glu1220del mutation of the MYH7 gene. The mutation segregates with disease in a family with autosomal dominant transmission of congenital heart defects including Ebstein anomaly and other associated cardiovascular defects including LV hypertrabeculation and ventricular septal defect. © 2013 Wiley Periodicals, Inc.

  14. Robotically assisted perventricular closure of perimembranous ventricular septal defects: preliminary results in Yucatan pigs.

    Science.gov (United States)

    Amin, Zahid; Woo, Russell; Danford, David A; Froemming, Stacey E; Reddy, Vadiyala M; Lof, John; Overman, David

    2006-02-01

    Robotic systems allow surgeons to perform minimally invasive cardiac surgery in adults. Experience in the pediatric population, however, is limited. Perventricular closure of muscular ventricular septal defects has been reported in humans but requires a median sternotomy. The objective of this study was to assess the feasibility of robotically assisted closure of perimembranous ventricular septal defects by using the perventricular approach. The procedure was attempted in 7 pigs with naturally occurring perimembranous ventricular septal defects. Echocardiography was performed to confirm the presence and assess the size of the defect. A 3-armed da Vinci system consisting of two 8-mm instrument ports and a 12-mm endoscopy port was used. A pericardiotomy was performed, and the right ventricular free wall was visualized. A spinal needle was advanced into the right ventricular cavity. By using echocardiographic guidance, a glide wire was advanced through the angiocatheter and manipulated through the defect into the left ventricle or the ascending aorta. A delivery sheath was advanced over the wire. An appropriately sized Amplatzer device was deployed through the sheath. The procedure was successful in 5 pigs. One device was removed because it was smaller than the defect and an appropriately sized device was not available. The placement failed in the second pig in the series. Four pigs were followed up for 1 to 4 months. Angiograms performed before the pigs were killed documented complete occlusion in 3 and mild-to-moderate shunt in 1. Robotically assisted perventricular closure with the Amplatzer Membranous VSD Occluder is feasible. This approach avoids the associated morbidities of cardiopulmonary bypass and median sternotomy. Further investigation and refinements are needed, however, before application of this approach in humans.

  15. The left atrial septal pouch as a risk factor for stroke: A systematic review.

    Science.gov (United States)

    Strachinaru, Mihai; Castro-Rodriguez, Jose; Verbeet, Thierry; Gazagnes, Marie-Dominique

    2017-04-01

    The left atrial septal pouch (LASP) is formed by incomplete fusion of the septum primum and septum secundum, leaving a cavity open towards the left atrium, but without interatrial shunting. There is no recommendation concerning strategy in the presence of a LASP, especially in the setting of stroke. The aim of this review was to determine whether the LASP could be incriminated as the aetiology of a stroke. We included all pertinent publications on the subject, and calculated hazard ratios for ischaemic stroke and cryptogenic stroke. There were only five case-control studies concerning the LASP, involving 516 stroke patients and 779 controls. Overall LASP prevalence was 21%, with a slightly higher prevalence in the cryptogenic stroke group (26%), but this difference was not statistically significant (P=0.27). In a random-effects meta-analysis, there was no difference between controls and patients with ischaemic stroke (hazard ratio 1.20, 95% confidence interval 0.96-1.53; P=0.14). Cryptogenic stroke appeared more frequently in patients with LASP (hazard ratio 1.53, 95% confidence interval 1.07-2.24; P=0.02), but this was driven by only one severely underpowered study. The published case reports demonstrated that thrombus formation inside the pouch can occur in the presence of major predisposing factors. The LASP can be a site for thrombus formation, leading to embolic events, but its presence does not correlate with an increased incidence of stroke. Associated factors should be taken into consideration in the setting of stroke. Further studies are necessary to validate a possible relationship with cryptogenic stroke. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  16. Digital rectal examination in the evaluation of rectovaginal septal defects.

    Science.gov (United States)

    Rachaneni, Suneetha; Atan, Ixora Kamisan; Shek, Ka Lai; Dietz, Hans Peter

    2017-02-17

    The objective was to evaluate the diagnostic potential of digital rectal examination in the identification of a true rectocele. This is a retrospective observational study utilising 187 archived data sets of women presenting with lower urinary tract symptoms and/or pelvic organ prolapse between August 2012 and November 2013. Evaluation included a standardised interview, ICS-POPQ, rectal examination and 4D translabial ultrasound. The main outcome measure was the diagnosis of rectocele by digital rectal palpation on Valsalva manoeuvre. This diagnosis correlated with the sonographic diagnosis of rectocele to determine agreement between digital examination and ultrasound findings. Complete data sets were available for 180 participants. On imaging, the mean position of the rectal ampulla was 11.07 (-36.3 to 44.3) mm below the symphysis pubis; 42.8% (77) had a rectocele of a depth of ≥10 mm. On palpation, a rectocele was detected in 60 women (33%). Agreement between palpation and imaging was observed in 77%; the kappa was 0.52 (CI 0.39-0.65). On receiver operator characteristic analysis, the area under the curve was 0.854 for the relationship between rectocele pocket depth and the detection of rectocele on palpation. Moderate agreement was found between digital rectal examination for rectocele and translabial ultrasound findings of a "true rectocele". Digital rectal examination may be used to identify these defects in clinical practice. Extending the clinical examination of prolapse to include rectal examination to palpate defects in the rectovaginal septum may reduce the need for defecatory proctograms for the assessment of obstructive defecation and may help triage patients in the management of posterior compartment prolapse.

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

    Directory of Open Access Journals (Sweden)

    K.A. Kalashnikova

    2016-05-01

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

  18. [Surgical correction of symptomatic ventricular septal defects in patients less than 6 months of age].

    Science.gov (United States)

    Cabrera Duro, A; Martínez Corrales, P; Llorente Urcullo, A; Aramburu Arriaga, N; Rodrigo Carbonero, D; Alcíbar Villa, J; Pastor Menchaca, E; Navarro Quintana, C

    1999-10-01

    Our objective was to evaluate the efficiency of a single surgical intervention in patients with symptomatic interventricular septal defects during the first six months of life. Between 1989 and 1997, 42 patients, 20 males and 22 females with an average age of 3.9 +/- 0.3 months and an average weight of 4 +/- 0.4 kg, were operated. Seven suffered from Down's syndrome. All of the patients became symptomatic during the first two months of life. The defect was localized by using Echo-Doppler in all of the cases. Thirty-six had perimembranous ventricular septal defects, 2 were muscular, 3 multiple and 1 was infundibular. The average defect size was 8 +/- 1.2 mm. A catheter was placed in 34 patient with the following results: Left to right shunt with 2.2 +/- 1.2, right ventricle systolic pressure of 57 +/- 20 mmHg (16 with systemic pulmonary pressure) and an average pulmonary pressure of 38 +/- 1.8 mmHg. The average pulmonary vascular resistance was 28 +/- 1.8 U/m2. Deep hypothermia (18 degrees C was applied during the surgery and the average cardiac arrest time was 31 +/- 4 minutes. None of the patients died during or after the surgical procedure. Patients required minimum ionotropic support during the first hours. The average time in the intensive care unit was 3.5 +/- 0.6 days, with an average hospitalization time of 11.2 +/- 2.1 days. Immediate complications included one hypertensive crisis, four junctional ectopic tachycardias, two atrio-ventricular blocks, 1 transient arrhythmia, two atelectasia-pneumonias, two patients with stridor and two sternal infections. During the follow-up period, two patients required a second intervention to repair the patch. We believe that one-time surgery is adequate to correct symptomatic ventricular septal defects.

  19. 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.

  20. Importance of Close Follow-Up in the Fetus with Premature Atrial Contractions Accompanied by Atrial Septal Aneurysm: A Case Report

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    Yilmaz Yozgat

    2013-01-01

    Full Text Available Rhythms that derive from parts of atria other than the sinus node are called premature atrial contractions (PACs. Vast majority of fetal PACs are idiopathic. Fetal PACs usually have a good prognosis and disappear spontaneously during pregnancy or after delivery. Development of fetal tachycardia or fetal bradycardia is rarely reported during follow-up of fetuses diagnosed with PACs. To the best of our knowledge, coexistence of tachycardia and bradycardia leading to hemodynamic impairment has not yet been reported. We present a fetus diagnosed with PACs and atrial septal aneurysm (ASA on the 23rd week of gestation proceeding to fetal bradycardia and fetal tachycardia and consequently hemodynamic impairment. We suggest closer follow-up of fetuses with PACs accompanied by ASA.

  1. Pulmonary atresia with dextroposition of the aorta and ventricular septal defect in three Arabian foals.

    Science.gov (United States)

    Vitums, A; Bayly, W M

    1982-03-01

    Three Arabian males foals were presented with cyanosis, heart murmur, and exercise intolerance, Results of clinical evaluation suggested a tentative diagnosis of ventricular septal defect in conjunction with malformations of the great arteries. Each foal had a poor prognosis and was killed at the owners' requests. At necropsy, the malformed hearts of the three foals were virtually identical. Each heart had a large defect in the upper interventricular septum. The aorta originated from the hypertrophied right ventricle and partially overrode the ventricular septal defect. The aortic ostium was guarded by three semilunar valvules. The position of the valvules and the ostia of the coronary arteries was abnormal. The pulmonary arteries arose from the persistent ductus arteriosus. The pulmonary trunk was reduced to a thin, fibrous cord extending from a short and narrow persistent ductus arteriosus to the level of the semilunar valve, where it fused with the wall of the aorta. No communication of the atretic pulmonary trunk with the cavity of the right ventricle was seen.

  2. Challenges in treatment of postinfarction ventricular septal defect and heart failure

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    Mangovski Ljupčo

    2015-01-01

    Full Text Available Introduction. Acquired ventricular septal defect (VSD is uncommon, but serious mechanical complication of acute myocardial infarction with poor outcome and high mortality rate in surgically or medically treated patients. Case report. We report a 58-year-old male patient admitted to our hospital six days following acute inferior myocardial infarction complicated by ventricular septal rupture with signs of heart failure. Coronary angiography revealed 3-vessel disease, with proximally occluded dominant right coronary artery. Transthoracic echo exam revealed aneurysm of a very thin inferior septum and the basal portion of the inferior left ventricular wall, with septal wall rupture. One of the VSD dimensions was 15 mm and left- to right shunt was calculated 2 : 1. Since the patient was at too high risk for surgical closure, transcatheter closure of VSD was chosen as a better option. Under short intravenous sedation, 24 mm Amplatzer device was implanted percutaneously with transesophageal echo guidance. The post-procedural result revealed a small residual shunt, but it was followed by significant improvement of the patient’s clinical status. A 24h Holter ECG monitoring did not show cardiac rhythm or conduction disturbances. Coronary angiography was repeated ten days following the procedure, after hemodynamic stabilization of the patient, with direct stenting of the circumflex artery and the intermediate artery. Ostial left descending artery lesion was left for further functional significance assessment. Conclusion: Percutaneous closure with a septal occluder device can be definitive primary treatment for anatomically suitable patients or it can serve as a bridge to surgical treatment.

  3. Right bundle branch block as a marker for interatrial septal abnormalities.

    Science.gov (United States)

    Bakalli, Aurora; Koçinaj, Dardan; Georgievska-Ismail, Ljubica; Bekteshi, Tefik; Pllana, Ejup; Sejdiu, Basri

    2012-02-01

    Interatrial septal anomalies, which include atrial septal defect, patent foramen ovale, and atrial septal aneurysm, are common disorders among adult patients. Early detection of interatrial septal anomalies is important in order to prevent haemodynamic consequences and/or thromboembolic events. Electrocardiogram offers some clues that should serve as hints for detection of interatrial abnormalities. The aim of our study was to analyse the interatrial septum by transoesophageal echocardiography in patients with electrocardiogram signs of right bundle branch block and in those without right bundle branch block. In a prospective study, 87 adult patients were included, that is, 41 with electrocardiogram signs of right bundle branch block forming the first group and 46 without right bundle branch block forming the second group. Interatrial septal anomalies were present in 80.5% of the patients with right bundle branch block, with patent foramen ovale (39.02%) being the most prevalent disorder, followed by atrial septal aneurysm (21.9%) and atrial septal defect (19.5%). Interatrial septal abnormalities were significantly more frequent in the first group compared with the second group (80.5% versus 6.5%, p value less than 0.001). Independently, patent foramen ovale was significantly more prevalent in patients with right bundle branch block (39.02% versus 4.3%, p value less than 0.001), as were atrial septal aneurysm (21.9% versus 2.2%, p value equal 0.01) and atrial septal defect (19.5% versus 0%, p value equal 0.004). Right bundle branch block should serve as a valuable indicator to motivate a detailed search for interatrial septal abnormalities.

  4. 59. Early and late results of routine leaflet augmentation for complete atrio-ventricular septal defect repair

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    A. Arifi

    2016-07-01

    Full Text Available Complete AVSD (CAVSD is characterized by the presence of a common atrio-ventricular (AV orifice, an inter-atrial communication, and a ventricular septal defect (VSD. Results of surgical correction of atrio-ventricular septal defects (AVSDs have improved over the last decades; however, the need for reoperation for left atrio-ventricular valve regurgitation, after primary AVSD repair remains a major concern. The aim of our study is to assess the outcome of the routine leaflet augmentation technique in CAVSD repair. A retrospective database and chart review analysis of all patients who underwent AV canal repair at king Abdul-Aziz Cardiac Center during period from 1999 to September 2014 was conducted. Demographic data, associated anomalies, operative data, ICU and hospital course were reviewed. Early outcomes were reviewed for postoperative complications (Chylothorax, complete AV block, Arrhythmias, early mortality and late outcomes were reviewed for Left AV valve regurgitation requiring for re-intervention and late mortality. Two hundred and sixty patients underwent leaflet augmentation technique to repair complete AVSD, between January 1999 and September 2014. The mean age was (131.5 months, and mean weight (6.06 kg. A variety of concomitant procedures were performed at the time of repair of the CAVSD, including a total of 49 patients (18.8% who underwent PDA ligation. Repair of TV (Right AV valve was performed in 11 patients (4.2%, 9 patients (3.46% required RVOTO resection, in 5 patients (1.92%, PA plasty was done and 2 patients (0.76% required ECMO after CAVSD repair. Regarding reoperations, a total of 17 patients (of 260 required reoperation after initial CAVSD repair. The most common indication for reoperation was left AV valve regurgitation in 16 patients (6% in the follow up period up to 15 years. One patient (0.38% required diaphragmatic plication. The overall mortality was 3 patients (1.1%. Leaflet augmentation for the repair of the

  5. Surgical Outcomes of a Modified Infarct Exclusion Technique for Post-Infarction Ventricular Septal Defects

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    In Sook Kim

    2015-12-01

    Full Text Available Background: Postinfarction ventricular septal defects (pVSDs are a serious complication of acute myocardial infarctions. The aim of this study was to analyze the clinical outcomes of the surgical treatment of pVSDs. Methods: The medical records of 23 patients who underwent operations (infarct exclusion in 21 patients and patch closure in two patients to treat acute pVSDs from 2001 to 2011 were analyzed. Intra-aortic balloon counterpulsation was performed in 19 patients (82.6%, one of whom required extracorporeal membrane support due to cardiogenic shock. The mean follow-up duration was 26.2±18.6 months. Results: The in-hospital mortality rate was 4.3% (1/23. Residual shunts were found in seven patients and three patients required reoperation. One patient needed reoperation due to the transformation of an intracardiac hematoma into an abscess. No patients required reoperation due to recurrence of a ventricular septal defect during the follow-up period. The cumulative survival rate was 95.5% at one year, 82.0% at five years, and 65.6% at seven years. Conclusion: The use of a multiple- patch technique with sealants appears to be a reliable method of reducing early mortality and the risk of significant residual shunting in patients with pVSDs.

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

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    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.

  7. Extended vertical transatrial septal approach for the removal of left atrial myxoma

    NARCIS (Netherlands)

    Zeebregts, CJAM; Schepens, MAAM; Knaepen, PJ

    Objective: Optimal exposure greatly facilitates left atrial myxomectomy and is mandatory for safe and efficacious tumour removal. The purpose of this study was to evaluate one institutions experience, with an alternative to the classical approach, for the removal of left atrial myxoma. Methods: In

  8. Pulmonary blood supply by a branch from the distal ascending aorta in pulmonary atresia with ventricular septal defect: differential diagnosis of fifth aortic arch.

    Science.gov (United States)

    Yoo, S J; Moes, C A; Burrows, P E; Molossi, S; Freedom, R M

    1993-10-01

    A patient with pulmonary atresia and a ventricular septal defect is described in whom an arterial branch from the distal ascending aorta supplied segments of both lungs. The branch is considered to represent a persistent fifth aortic arch. The possible morphogenesis and differential diagnosis of a communication between the ascending aorta and the pulmonary artery in pulmonary atresia with ventricular septal defect are discussed.

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

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

    2010-10-15

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

  10. The surgical anatomy of ventricular septal defect part IV: double outlet ventricle.

    Science.gov (United States)

    Anderson, R H; Ho, S Y; Wilcox, B R

    1996-01-01

    In this fourth part of our series of articles concerned with the surgical anatomy of ventricular septal defects (VSDs), we have analyzed the arrangements when both arterial trunks arise from the same ventricle. The essence of these anomalies is that the interventricular communication is an integral part of the circulation. Unless the surgeon constructs an alternative route of exit, closure of this defect would isolate one of the ventricles. The usual surgical approach, therefore, is to patch the hole between the ventricles into one or other of the subarterial outflow tracts. This means that all the components of the ventricular outflow tracts are of potential surgical importance. In hearts with double outlet right ventricle, the VSD can be categorized as being subaortic, subpulmonary, doubly committed, or non-committed. It is also important to determine whether its anatomical borders, as seen from the right ventricle, are in part fibrous or exclusively muscular, so as to establish the location of the atrioventricular conduction axis. It is possible, according to the nature of these borders, to place the defects into one of three groups, perimenbranous, muscular, or doubly committed and juxta-arterial. The size of the defect is another important surgical consideration. Double outlet left ventricle is a significantly more rare malformation, but the rules for determining the disposition of the conducting tissues are the same.

  11. Simulation of Eisenmenger syndrome with ventricular septal defect using equivalent electronic system.

    Science.gov (United States)

    Korurek, Mehmet; Yildiz, Mustafa; Yüksel, Ayhan; Şahin, Alparslan

    2012-06-01

    In this study, we aim to investigate the simulation of the cardiovascular system using an electronic circuit model under normal and pathological conditions, especially the Eisenmenger syndrome. The Eisenmenger syndrome includes a congenital communication between the systemic and pulmonary circulation, with resultant pulmonary arterial hypertension and right-to-left reversal of flow through the defect. When pulmonary vascular resistance exceeds systemic vascular resistance, it results in hypoxaemia and cyanosis. The Westkessel model including Resistor-Inductance-Capacitance pi-segments was chosen in order to simulate both systemic and pulmonary circulation. The left and right heart are represented by trapezoidal shape stiffness for better simulation results. The Eisenmenger syndrome is simulated using a resistance (septal resistance) connected between the left ventricle and right ventricle points of the model. Matlab® is used for the model implementation. In this model, although there is a remarkable increase in the pulmonary artery pressure and right ventricle pressure, left ventricle pressure, aortic pressure, aortic flow, and pulmonary compliance decrease in the Eisenmenger syndrome. In addition, left-to-right septal flow reversed in these diseases. Our model is effective and available for simulating normal cardiac conditions and cardiovascular diseases, especially the Eisenmenger syndrome.

  12. 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.

  13. Factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of repair of complete atrioventricular septal defect

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    Marcelo Felipe Kozak

    2015-09-01

    Full Text Available AbstractIntroduction:Left atrioventricular valve regurgitation is the most concerning residual lesion after surgical correction of atrioventricular septal defects.Objective:To determine factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of surgical repair of complete atrioventricular septal defect.Methods:We assessed the results of 53 consecutive patients 3 years-old and younger presenting with complete atrioventricular septal defect that were operated on at our practice between 2002 and 2010. The following variables were considered: age, weight, absence of Down syndrome, grade of preoperative atrioventricular valve regurgitation, abnormalities on the left atrioventricular valve and the use of annuloplasty. Median age was 6.7 months; median weight was 5.3 Kg; 86.8% had Down syndrome. At the time of preoperative evaluation, there were 26 cases with moderate or severe left atrioventricular valve regurgitation (49.1%. Abnormalities on the left atrioventricular valve were found in 11.3%; annuloplasty was performed in 34% of the patients.Results:At the time of postoperative evaluation, there were 21 cases with moderate or severe left atrioventricular valve regurgitation (39.6%. After performing a multivariate analysis, the only significant factor associated with moderate or severe left atrioventricular valve regurgitation was the absence of Down syndrome (P=0.03.Conclusion:Absence of Down syndrome was associated with moderate or severe postoperative left atrioventricular valve regurgitation after surgical repair of complete atrioventricular septal defect at our practice.

  14. 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

  15. Near miss sudden cardiac death on a young patient with repaired atrioventricular septal defect.

    Science.gov (United States)

    Papadopoulou, Sofia A; Dimopoulos, Konstantinos; Gatzoulis, Michael A

    2008-11-28

    Patients with congenital heart disease often face the prospect of long-term haemodynamic or arrhythmic complications for which lifelong follow-up in specialist adult congenital heart disease (ACHD) centres is required. We describe the case of a 25-year-old man with repaired atrioventricular septal defect who was referred to our centre after a ventricular fibrillation arrest. Serial echocardiograms in previous years had shown progressive severe left ventricular outflow obstruction, but the patient had not been operated on as he was deemed asymptomatic and reluctant to consider surgery. Management and criteria for further intervention in ACHD patients often differ from those of patients with acquired heart disease and reliance on symptoms alone is not good practice and may prove catastrophic.

  16. 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.

  17. Ventricular septal defect and bidirectional shunting? Things are not what they seem.

    Science.gov (United States)

    Bonaque González, Juan Carlos; Navarro, Francisco; Valencia, Félix; Aguado, María J

    2013-01-01

    This report describes the case of a 19-year-old woman with a diagnosis of muscular ventricular septal defect. Bidirectional shunting was observed during a transthorathic echocardiography evaluation which also suggested normal pulmonary arterial pressure. Moreover, anomalous and hypertrophic right ventricular muscular bands were observed. After having ruled out other possibilities, the plausible explanation is one, which is not described in the literature. The findings may be explained as a sequestrated portion of the cavity of the right ventricle that remains isolated from the rest of the right ventricle (RV) by anomalous and hypertrophic right ventricular muscular bands, with communication only between the left ventricle and the sequestrated part of the RV. This is an unusual variant of two-chambered RV simulating two-chambered left ventricle.

  18. 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 implantation procedure and the retrievability of the device after deployment if needed. The design of the GSO has improved the device apposition ability and tissue response whilst keeping its atraumatic design, low septal profile with minimal septal distortion and long-term biocompatibility. The first three...... of these patients had patent foramen ovale and one had secundum atrial septal defect. In all the cases, the GSO devices were successfully deployed in the first attempt without any complication. Only one patient had a minor residual shunt detected immediately after the device deployment. All the patients were re...

  19. The left atrioventricular valve in partial atrioventricular septal defect: management strategy and surgical outcome.

    Science.gov (United States)

    Al-Hay, Amira A A; Lincoln, Christopher R; Shore, Darryl F; Shinebourne, Elliot A

    2004-10-01

    To test the hypothesis that in patients with a partial atrioventricular septal defect (PAVSD) and a competent left atrioventricular valve (LAVV), sutures should be placed across the line of apposition of the superior and inferior bridging leaflets, septal commissure (SC), to prevent the development of regurgitation. Outcome of surgery and risk factors for the need for LAVV reoperation of patients with mild or no LAVV regurgitation (LAVVR) were evaluated. Controversy over management of the LAVV in PAVSD. One hundred and forty seven children with PAVSD underwent surgical repair at the Royal Brompton Hospital between January 1983 and December 1999. Of this group, 21 (16.7%) had LAVVR of sufficient severity to require surgical intervention and were therefore excluded from analysis. The median age and weight at repair of those with mild or no LAVVR was 4.1 years and 15.4 kg. One hundred and eight had normal chromosomes, 13 Down syndrome and five other syndromes. The interatrial communication was closed using a pericardial patch in 62.7% and with synthetic material in the remainder. Intraoperative testing of LAVV competence was undertaken using saline injection into the left ventricle. In 80.9%, sutures were placed across the line of apposition of the left sided superior and inferior bridging leaflets partially to close the SC (sometimes incorrectly named the mitral valve cleft). The overall hospital mortality was 3.2% (95% confidence interval (CI) 1, 8.4%), which did not differ statistically in the last 20 years. No specific risk factors for early death were identified. Eleven patients (8.7%, 95% CI 4.7, 15.4%) required reoperation, 10 for LAVV repair and 1 resection of subaortic stenosis. Univariate analysis of risk factors for LAVV reoperation were low weight, relatively small size LAVV, the presence of a small preoperative interventricular interchordal communication and duration of ventilation. Ten (9.8%) of 102 patients in whom SC was sutured required LAVV

  20. 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.

  1. Preoperative cardiac computed tomography for demonstration of congenital cardiac septal defect in adults

    Energy Technology Data Exchange (ETDEWEB)

    Eom, Hye-Joung; Yang, Dong Hyun; Kang, Joon-Won; Lim, Tae-Hwan [University of Ulsan College of Medicine, Department of Radiology and Research Institute of Radiology, Cardiac Imaging Center, Asan Medical Center, Seoul (Korea, Republic of); Kim, Dae-Hee; Song, Jong-Min; Kang, Duk-Hyun; Song, Jae-Kwan [University of Ulsan College of Medicine, Department of Cardiology and Heart Institute, Cardiac Imaging Center, Asan Medical Center, Seoul (Korea, Republic of); Kim, Joon Bum; Jung, Sung-Ho; Choo, Suk Jung; Chung, Cheol Hyun; Lee, Jae Won [University of Ulsan College of Medicine, Department of Cardiothoracic surgery, Cardiac Imaging Center, Asan Medical Center, Seoul (Korea, Republic of)

    2015-06-01

    We aimed to evaluate the role of preoperative cardiac computed tomography (CT) for adults with congenital cardiac septal defect (CSD). Sixty-five consecutive patients who underwent preoperative CT and surgery for CSD were included. The diagnostic accuracy of CT and the concordance rate of the subtype classification of CSD were evaluated using surgical findings as the reference standard. Sixty-five patients without CSD who underwent cardiac valve surgery were used as a control group. An incremental value of CT over echocardiography was described retrospectively. Sensitivity and specificity of CT for diagnosis of CSD were 95 % and 100 %, respectively. The concordance rate of subtype classification was 91 % in CT and 92 % in echocardiography. The maximum size of the defect measured by CT correlated well with surgical measurement (r = 0.82), and the limit of agreement was -0.9 ± 7.42 mm. In comparison with echocardiography, CT was able to detect combined abnormalities in three cases, and exclusively provided correct subtype classification or clarified suspected abnormal findings found on echocardiography in seven cases. Cardiac CT can accurately demonstrates CSD in preoperative adult patients. CT may have an incremental role in preoperative planning, particularly in those with more complex anatomy. (orig.)

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

    Science.gov (United States)

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

    2014-10-01

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

  3. Novel and Functional DNA Sequence Variants within the GATA6 Gene Promoter in Ventricular Septal Defects

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    Chunyu Li

    2014-07-01

    Full Text Available Congenital heart disease (CHD is the most common birth defect in humans. Genetic causes and underlying molecular mechanisms for isolated CHD remain largely unknown. Studies have demonstrated that GATA transcription factor 6 (GATA6 plays an essential role in the heart development. Mutations in GATA6 gene have been associated with diverse types of CHD. As GATA6 functions in a dosage-dependent manner, we speculated that changed GATA6 levels, resulting from DNA sequence variants (DSVs within the gene regulatory regions, may mediate the CHD development. In the present study, GATA6 gene promoter was genetically and functionally analyzed in large groups of patients with ventricular septal defect (VSD (n = 359 and ethnic-matched healthy controls (n = 365. In total, 11 DSVs, including four SNPs, were identified in VSD patients and controls. Two novel and heterozygous DSVs, g.22169190A>T and g.22169311C>G, were identified in two VSD patients, but in none of controls. In cultured cardiomyocytes, the activities of the GATA6 gene promoter were significantly reduced by the DSVs g.22169190A>T and g.22169311C>G. Therefore, our findings suggested that the DSVs within the GATA6 gene promoter identified in VSD patients may change GATA6 levels, contributing to the VSD development as a risk factor.

  4. 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.

  5. A Rare Case of Double-Chambered Right Ventricle Associated with Ventricular Septal Defect and congenital Absence of the Pulmonary Valve

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    Georges Khoueiry

    2014-01-01

    Full Text Available Double-chambered right ventricle (DCRV is a rare congenital heart disorder involving 2 different right ventricle (RV pressure compartments that is often associated with ventricular septal defect (VSD. Usually, the obstruction is caused by an anomalous muscle bundle crossing the RV from the interventricular septum to the RV free wall. We are reporting a case of double-chambered right ventricle associated with ventricular septal defect and congenital absence of the pulmonary valve, a rare form of congenital infundibular pulmonary stenosis. In addition to ventricular septal defect, our patient had congenital absence of the pulmonary valve, which is very unusual and has never been reported to our knowledge.

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

    Science.gov (United States)

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

    2014-09-01

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

  7. Atrial Fibrillation after Robotic Cardiac Surgery

    OpenAIRE

    LEONARDO CANALE; STEPHANIE MICK; RAVI NAIR; TOMISLAV MIHALJEVIC; JOHANNES BONATTI

    2014-01-01

    Atrial fibrillation is a common arrhythmia after conventional open heart surgery. A minimally invasive robotic approach has the potential to lower its occurrence. We sought to review the literature on the incidence of post operative atrial fibrillation in robotic heart surgery and compare it to the incidence in conventional cardiac surgery. The types of operation investigated were: coronary artery bypass surgery, mitral valve repair, atrial septal defect closure and myxoma excision. Operation...

  8. 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

  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.

  10. Compound heterozygous or homozygous truncating MYBPC3 mutations cause lethal cardiomyopathy with features of noncompaction and septal defects

    Science.gov (United States)

    Wessels, Marja W; Herkert, Johanna C; Frohn-Mulder, Ingrid M; Dalinghaus, Michiel; van den Wijngaard, Arthur; de Krijger, Ronald R; Michels, Michelle; de Coo, Irenaeus FM; Hoedemaekers, Yvonne M; Dooijes, Dennis

    2015-01-01

    Familial hypertrophic cardiomyopathy (HCM) is usually caused by autosomal dominant pathogenic mutations in genes encoding sarcomeric or sarcomere-associated cardiac muscle proteins. The disease mainly affects adults, although young children with severe HCM have also been reported. We describe four unrelated neonates with lethal cardiomyopathy, and performed molecular studies to identify the genetic defect. We also present a literature overview of reported patients with compound heterozygous or homozygous pathogenic MYBPC3 mutations and describe their clinical characteristics. All four children presented with feeding difficulties, failure to thrive, and dyspnea. They died from cardiac failure before age 13 weeks. Features of left ventricular noncompaction were diagnosed in three patients. In the fourth, hypertrabeculation was not a clear feature, but could not be excluded. All of them had septal defects. Two patients were compound heterozygotes for the pathogenic c.2373dup p.(Trp792fs) and c.2827C>T p.(Arg943*) mutations, and two were homozygous for the c.2373dup and c.2827C>T mutations. All patients with biallelic truncating pathogenic mutations in MYBPC3 reported so far (n=21) were diagnosed with severe cardiomyopathy and/or died within the first few months of life. In 62% (13/21), septal defects or a patent ductus arteriosus accompanied cardiomyopathy. In contrast to heterozygous pathogenic mutations, homozygous or compound heterozygous truncating pathogenic MYBPC3 mutations cause severe neonatal cardiomyopathy with features of left ventricular noncompaction and septal defects in approximately 60% of patients. PMID:25335496

  11. Robot-Assisted Partial Atrioventricular Canal Defect Repair and Cryo-Maze Procedure.

    Science.gov (United States)

    Mandal, Kaushik; Srivastava, Aseem R; Nifong, L Wiley; Chitwood, W Randolph

    2016-02-01

    Atrial septal defect is one of the most common congenital heart anomalies in adults. Patients with partial atrioventricular canal defects, previously known as ostium primum atrial septal defect, usually present at an early age, and only a few reach adulthood without surgical correction. Herein, we describe a young woman who presented with an ostium primum defect and severe symptomatic mitral and tricuspid regurgitation with paroxysmal atrial fibrillation. A complex repair was successfully done through a left atrial approach using robot-assistance. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  12. 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.

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

    NARCIS (Netherlands)

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

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Maitrey D Gajjar

    2015-01-01

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

  15. Factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of repair of incomplete atrioventricular septal defect

    Directory of Open Access Journals (Sweden)

    Marcelo Felipe Kozak

    2015-04-01

    Full Text Available AbstractIntroduction:Left atrioventricular valve regurgitation is the most concerning residual lesion after surgical correction of atrioventricular septal defect.Objective:To determine factors associated with moderate or greater left atrioventricular valve regurgitation within 30 days of surgical repair of incomplete atrioventricular septal defect.Methods:We assessed the results of 51 consecutive patients 14 years-old and younger presenting with incomplete atrioventricular septal defect that were operated on at our practice between 2002 and 2010. The following variables were considered: age, weight, absence of Down syndrome, grade of preoperative left atrioventricular valve regurgitation, abnormalities on the left atrioventricular valve and the use of annuloplasty. The median age was 4.1 years; the median weight was 13.4 Kg; 37.2% had Down syndrome. At the time of preoperative evaluation, there were 23 cases with moderate or greater left atrioventricular valve regurgitation (45.1%. Abnormalities on the left atrioventricular valve were found in 17.6%; annuloplasty was performed in 21.6%.Results:At the time of postoperative evaluation, there were 12 cases with moderate or greater left atrioventricular valve regurgitation (23.5%. The variation between pre- and postoperative grades of left atrioventricular valve regurgitation of patients with atrioventricular valve malformation did not reach significance (P=0.26, unlike patients without such abnormalities (P=0.016. During univariate analysis, only absence of Down syndrome was statistically significant (P=0.02. However, after a multivariate analysis, none of the factors reached significance.Conclusion:None of the factors studied was determinant of a moderate or greater left atrioventricular valve regurgitation within the first 30 days of repair of incomplete atrioventricular septal defect in the sample. Patients without abnormalities on the left atrioventricular valve benefit more of the operation.

  16. Coronary to pulmonary fistula as the primary source of pulmonary blood supply in pulmonary atresia with ventricular septal defect

    OpenAIRE

    Isman Firdaus; Cholid T. Tjahjono; Ganesja H. Harimurti; Poppy S. Roebiono

    2004-01-01

    A communication between the coronary and pulmonary arteries, so called coronary to pulmonary fistula, is a rare source of pulmonary supply in pulmonary atresia (PA) with ventricular septal defect (VSD). A 4 year old girl referred to National Cardiovascular Center Harapan Kita, Jakarta with symptoms and signs of increased pulmonary blood flow since infancy and was confirmed by the chest x-rays. Heart examination revealed normal first heart sound with single loud second heart sound and an eject...

  17. Radiofrequency-assisted "reconstruction" of the right ventricular outflow tract in muscular pulmonary atresia with ventricular septal defect.

    OpenAIRE

    Hausdorf, G; Schulze-Neick, I.; Lange, P. E.

    1993-01-01

    A case of pulmonary atresia with ventricular septal defect is reported in which a communication was established between the right ventricle and the hypoplastic pulmonary artery by intervention, despite muscular atresia of the right ventricular outflow tract. The atresia was perforated with a special designed radiofrequency catheter (Osypka). After the creation of a canal within the muscular atresia, balloon dilatation (diameters 2, 3.5, and 7.2 mm) was performed. Arterial oxygen saturation in...

  18. 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, ploss were significantly correlated with BNP (r=0.75, 0.69 and 0.49, ploss 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.

  19. 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%.

  20. Contribution of Copy Number Variation to Down Syndrome-associated Atrioventricular Septal Defects

    Science.gov (United States)

    Ramachandran, Dhanya; Mulle, Jennifer G.; Locke, Adam E.; Bean, Lora J.H.; Rosser, Tracie C.; Bose, Promita; Dooley, Kenneth J.; Cua, Clifford L.; Capone, George T.; Reeves, Roger H.; Maslen, Cheryl L.; Cutler, David J.; Sherman, Stephanie L.; Zwick, Michael E.

    2014-01-01

    Purpose The goal of this study was to identify the contribution of large copy number variants (CNV) to Down syndrome (DS) associated atrioventricular septal defects (AVSD), whose risk in the trisomic population is 2000-fold more compared to general disomic population. Methods Genome-wide CNV analysis was performed on 452 individuals with DS (210 cases with complete AVSD; 242 controls with structurally normal hearts) using Affymetrix SNP 6.0 arrays, making this the largest heart study conducted to date on a trisomic background. Results Large common CNVs with substantial effect sizes (OR>2.0) do not account for the increased risk observed in DS-associated AVSD. In contrast, cases had a greater burden of large rare deletions (p<0.01) and intersected more genes (p<0.007) when compared to controls. We also observed a suggestive enrichment of deletions intersecting ciliome genes in cases compared to controls. Conclusion Our data provide strong evidence that large rare deletions increase the risk of DS-associated AVSD, while large common CNVs do not appear to increase the risk of DS-associated AVSD. The genetic architecture of AVSD is complex and multifactorial in nature. PMID:25341113

  1. 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.

  2. Infective endocarditis following patch closure of ventricular septal defect: a cross-sectional Doppler echocardiographic study.

    Science.gov (United States)

    Shrivastava, S; Radhakrishnan, S

    1989-10-01

    Cross-sectional and Doppler echocardiographic characteristics of infective endocarditis are described in six cases following patch closure of a ventricular septal defect. The patients presented to us with fever one to five months after surgery. Five of them also had congestive cardiac failure. Cross-sectional echocardiography showed large masses over the patch in all cases. Dehiscence of the lower end of the patch was identified in three of them, and, in two cases, the right sinus of Valsalva had ruptured into the right ventricle. Doppler detected turbulent flow in the right ventricle in five cases, and a continuous signal indicating an aorto-right ventricular communication in two cases. A signal indicative of aortic regurgitation was also found in the latter two cases. Staphylococcus aureus was cultured from the blood in three cases and Aspergillus was identified at autopsy in one. The echocardiographic findings were confirmed in three cases (one during surgery and two at autopsy). Dehiscence of the patch and large masses were associated with a poor prognosis.

  3. A HAND2 Loss-of-Function Mutation Causes Familial Ventricular Septal Defect and Pulmonary Stenosis

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    Yu-Min Sun

    2016-04-01

    Full Text Available Congenital heart disease (CHD is the most common developmental abnormality, and is the leading noninfectious cause of mortality in neonates. Increasing evidence demonstrates that genetic defects play an important role in the pathogenesis of CHD. However, CHD exhibits substantial heterogeneity, and the genetic determinants for CHD remain unknown in the overwhelming majority of cases. In the current study, the coding exons and flanking introns of the HAND2 gene, which encodes a basic helix-loop-helix transcription factor essential for normal cardiovascular development, were sequenced in 192 unrelated patients with CHD, and a novel heterozygous mutation, p.S65I, was identified in a patient with congenital ventricular septal defect (VSD. Genetic analysis of the index patient’s pedigree revealed that the mutation was present in all seven affected family members available, but absent in the 13 unaffected family members examined. Besides, in addition to VSD, five of the proband’s close relatives also had pulmonary stenosis (PS, and the proband’s son also had double outlet right ventricle (DORV. The missense mutation, which altered an evolutionarily conserved amino acid, was absent in 300 unrelated, ethnically matched healthy individuals. Biological analyses using a dual-luciferase reporter assay system showed that the mutant HAND2 was associated with significantly diminished transcriptional activity. Furthermore, the mutation abolished the synergistic activation between HAND2 and GATA4, as well as NKX2.5—two other cardiac core transcriptional factors that have been causally linked to CHD. These findings indicate that HAND2 loss-of-function mutation contributes to human CHD, perhaps via its interaction with GATA4 and NKX2.5.

  4. 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.

  5. Aneurysm of the membranous septum in adult patients with perimembranous ventricular septal defect.

    Science.gov (United States)

    Yilmaz, A T; Ozal, E; Arslan, M; Tatar, H; Oztürk, O Y

    1997-02-01

    The aneurysm of the membranous septum (AMS) has often been considered as benign in the minds of many previous investigators. We have analyzed the complications with AMS in adult patients. Fifty-one cases (20%) of AMS in 254 adult patients with perimembranous ventricular septal defect (VSD) are described. The diagnosis of AMS was based on angiographic criteria. Thirty-nine (76.5%) of the 51 patients with AMS were aged between 20 and 29 years. All patients but one with AMS had a pulmonary-to-systemic flow (Qp/Qs) of less than 2.3 (range 1-2.1, mean 1.4). In a patient who had a ruptured aneurysm, the Qp/Qs was 2.7. There were six main complications affected by AMS and/or VSD; aortic valve prolapse in 24 patients (47%), aortic regurgitation in 15 (29.4%), tricuspid insufficiency in nine (17.6%), right ventricular outflow tract obstruction in two (4%), and rupture of the aneurysm in one patient (2%). Seven patients (13.7%) had prior bacterial endocarditis. All patients underwent surgery. Aneurysm and VSD were closed by direct suture in nine and with a patch in 42 patients. Aortic valve repair was performed in 13 patients in whom regurgitation was mild to moderate, and replacement was required in two patients with severe aortic regurgitation. There were no early or late deaths. Residual communication and recurrence of the aneurysm was noted three and seven years postoperatively in two patients where VSD had been closed by direct suture. According to present data, aneurysm formation functionally reduces the VSD size, but it has the potential consequence of promoting tricuspid insufficiency, aortic valve prolapse, right ventricular outflow tract obstruction, rupture and bacterial endocarditis. Therefore, we recommend that AMS should be resected completely and the defect produced closed with a patch in order to prevent further enlargement and consequent complications even if there are no cardiac symptoms.

  6. Repair of double outlet right ventricle with doubly-committed ventricular septal defect.

    Science.gov (United States)

    Uemura, H; Yagihara, T; Kadohama, T; Kawahira, Y; Yoshikawa, Y

    2001-07-01

    To investigate our surgical results of intraventricular rerouting in patients having double outlet right ventricle with doubly-committed ventricular septal defect. We undertook repair in 8 patients with this particular feature. Of these, 2 patients had pulmonary stenosis, and another had interruption of the aortic arch. The subarterial defect was unequivocally related to both the aortic and the-pulmonary orifices in all, albeit slightly deviated towards the aortic orifice in one, and towards the pulmonary orifice in another. Intraventricular rerouting was carried out via incisions to the right atrium and the pulmonary trunk. To ensure reconstruction of an unobstructed pulmonary pathway, a limited right ventriculotomy was made in 5. All patients survived the procedure, and are currently doing well, with follow-up of 25 to 194 months, with a mean of 117+/-68 months. Catheterization carried out 16+/-6 months after repair demonstrated excellent ventricular parameters. Mean pulmonary arterial pressure was 16+/-7 mmHg, being higher than 20 mmHg in 2 patients. No significant obstruction was found between the right ventricle and the pulmonary arteries. A pressure gradient across the left ventricular outflow tract became significant in one patient in whom a small outlet septum was present, and a heart-shaped baffle had been used for intraventricular rerouting. Reoperation was eventually needed in this patient for treatment of the obstruction, which proved to be progressive. Precise recognition of the morphologic features is of paramount importance when choosing the optimal options for biventricular repair in patients with double outlet right ventricle and doubly-committed interventricular communication.

  7. Association of interatrial septal abnormalities with cardiac impulse conduction disorders in adult patients: experience from a tertiary center in Kosovo.

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    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.

  8. Atrioventricular Septal Defect with Common Atrioventricular Junction Guarded by a Common Valve Consisting of Left Atrioventricular Trifoliate Valve

    Science.gov (United States)

    Krasniqi, Xhevdet; Gashi, Masar; Berisha, Blerim; Pllana, Ejup; Bakalli, Aurora; Abazi, Flora; Koçinaj, Dardan

    2013-01-01

    Introduction: Atrioventricular septal defect with common atrioventricular junction is a rare adult congenital cardiac syndrome. This occurrence with prolonged survival is exceptionally rare. Case report: We present the case of a patient who presented with this defect with common atrioventricular junction who survived to the age of 32. We describe a 32-year-old man with atrioventricular septal defect with common atrioventricular junction guarded by a common valve. His history, clinical course, and anatomic findings are discussed along with the factors which may have contributed to his longevity, which is unique in the medical literature. His management reflected the state of medical knowledge at the time when he presented, and although alternate approaches may have been utilized if the patient presented today. We discuss the findings, frequency, classifi cation, and management of congenital defects. Development of embryonic structure is altered by interaction between genetics and environmental factors toward a rare associated of congenital cardiac defects-complex congenital heart disease. Conclusion: This case demonstrates that patients with very complex congenital cardiac disease may survive to adulthood, presenting challenges in both medical and surgical treatment. PMID:24554809

  9. Atrioventricular septal defect with common atrioventricular junction guarded by a common valve consisting of left atrioventricular trifoliate valve.

    Science.gov (United States)

    Krasniqi, Xhevdet; Gashi, Masar; Berisha, Blerim; Pllana, Ejup; Bakalli, Aurora; Abazi, Flora; Koçinaj, Dardan

    2013-12-01

    Atrioventricular septal defect with common atrioventricular junction is a rare adult congenital cardiac syndrome. This occurrence with prolonged survival is exceptionally rare. We present the case of a patient who presented with this defect with common atrioventricular junction who survived to the age of 32. We describe a 32-year-old man with atrioventricular septal defect with common atrioventricular junction guarded by a common valve. His history, clinical course, and anatomic findings are discussed along with the factors which may have contributed to his longevity, which is unique in the medical literature. His management reflected the state of medical knowledge at the time when he presented, and although alternate approaches may have been utilized if the patient presented today. We discuss the findings, frequency, classifi cation, and management of congenital defects. Development of embryonic structure is altered by interaction between genetics and environmental factors toward a rare associated of congenital cardiac defects-complex congenital heart disease. This case demonstrates that patients with very complex congenital cardiac disease may survive to adulthood, presenting challenges in both medical and surgical treatment.

  10. 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. 

  11. Primary biventricular repair of atrioventricular septal defects: an analysis of reoperations.

    Science.gov (United States)

    Vohra, Hunaid A; Chia, Alicia X F; Yuen, Ho Ming; Vettukattil, Joseph J; Veldtman, Gruschen; Gnanapragasam, James; Roman, Kevin; Salmon, Anthony P; Haw, Marcus P

    2010-09-01

    The purpose of this study was to analyze the factors affecting reoperation after primary biventricular atrioventricular septal defect (AVSD) repair. Between April 1997 and April 2007, 93 consecutive patients underwent surgery for biventricular correction of AVSD with a median age of 5.8 months (range, 9 days to 68.9 years). Fifty-three patients had complete AVSD, 6 patients had an intermediate type, and 29 patients had partial AVSD; 4 patients had a complete AVSD with associated tetralogy of Fallot, and 1 patient had a complete AVSD with double-outlet right ventricle. There was no in-hospital mortality. There were 2 late deaths (2.2%). Forty-three reoperations were performed in 23 patients (24.7%), of which 18 were for repair of significant left atrioventricular valve regurgitation and 8 were mitral valve replacements. Seven patients (7.5%) required insertion of a permanent pacemaker. The overall 5-year freedom from reoperation after AVSD repair was 73.6% +/- 4.8%. In the multivariate analysis for complete AVSDs, Down syndrome (p = 0.01) and the presence of right ventricular dominance (p = 0.03) were independent predictors of reoperation. At last follow-up, 76 patients (83.5%) were in New York Heart Association class I, and 68 patients (74.7%) were not taking any heart failure medications. Echocardiographic examination showed absent to mild left atrioventricular valve regurgitation in 76.5%; moderate, in 19.8%; and severe, in 3.7% of patients. Down syndrome and right ventricular dominance are independent predictors of reoperation after complete AVSD repair. Biventricular repair of isolated AVSD with a small left ventricle can be successfully accomplished with no mortality. 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  12. 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

  13. Analysis of MTHFR and MTRR Gene Polymorphisms in Iranian Ventricular Septal Defect Subjects

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    Ali Etemad

    2013-01-01

    Full Text Available Ventricular septal defect (VSD is one of the most common types of congenital heart defects (CHD. There are vivid multifactorial causes for VSD in which both genetic and environmental risk factors are consequential in the development of CHD. Methionine synthase reductase (MTRR and methylenetetrahydrofolate reductase (MTHFR are two of the key regulatory enzymes involved in the metabolic pathway of homocysteine. Genes involved in homocysteine/folate metabolism may play an important role in CHDs. In this study; we determined the association of A66G and C524T polymorphisms of the MTRR gene and C677T polymorphism of the MTHFR gene in Iranian VSD subjects. A total of 123 children with VSDs and 125 healthy children were included in this study. Genomic DNA was extracted from the buccal cells of all the subjects. The restriction fragment length polymorphism polymerase chain reaction (PCR-RFLP method was carried out to amplify the A66G and C524T polymorphism of MTRR and C677T polymorphism of MTHFR genes digested with Hinf1, Xho1 and Nde1 enzymes, respectively. The genotype frequencies of CC, CT and TT of MTRR gene among the studied cases were 43.1%, 40.7% and 16.3%, respectively, compared to 52.8%, 43.2% and 4.0%, respectively among the controls. For the MTRR A66G gene polymorphism, the genotypes frequencies of AA, AG and GG among the cases were 33.3%, 43.9% and 22.8%, respectively, while the frequencies were 49.6%, 42.4% and 8.0%, respectively, among control subjects. The frequencies for CC and CT genotypes of the MTHFR gene were 51.2% and 48.8%, respectively, in VSD patients compared to 56.8% and 43.2% respectively, in control subjects. Apart from MTHFR C677T polymorphism, significant differences were noticed (p < 0.05 in C524T and A66G polymorphisms of the MTRR gene between cases and control subjects.

  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.

  15. Combined double chambered right ventricle, tricuspid valve dysplasia, ventricular septal defect, and subaortic stenosis in a dog.

    Science.gov (United States)

    Scurtu, Iuliu; Tabaran, Flaviu; Mircean, Mircea; Giurgiu, Gavril; Nagy, Andras; Catoi, Cornel; Ohad, Dan G

    2017-11-29

    Double chambered right ventricle (DCRV) is a congenital heart anomaly where the right ventricle is divided into two chambers. We describe, for the first time, an unusual combination of DCRV combined with some other congenital heart defects. A 1.2-year-old Golden Retriever was presented with lethargy, exercise intolerance and ascites. Physical examination revealed an irregularly irregular pulse and a grade V/VI, systolic, right cranial murmur. Electrocardiography revealed widened and splintered QRS complexes with a right bundle-branch block pattern. Radiography demonstrated right-sided cardiomegaly. Two-dimensional echocardiography identified a DCRV with tricuspid valve dysplasia. The patient died despite abdominocentesis and 4 days of oral pharmacotherapy, and necropsy revealed an anomalous fibromuscular structure that divided the right ventricle into two compartments. Another finding was tricuspid valve dysplasia with hypoplasia of the posterior and septal leaflets. The anterior leaflet was prominent, being part of the anomalous structure that divided the right ventricle. Necropsy also identified a perimembranous ventricular septal defect and mild subaortic stenosis. Histopathological examination of the fibromuscular band that separated the right ventricle identified longitudinally oriented layers of dense fibrous connective tissue and myocardial cells arranged in a plexiform pattern. The muscular component was well represented at the ventral area of the fibromuscular band, and was absent in the central zone. Superficially, the endocardium presented areas of nodular hyperplasia covering mainly the fibrous part of the abnormal structure. The nodules were sharply demarcated and were composed by loosely arranged connective tissue with myxoid appearance, covered by discrete hyperplastic endocardium. Concomitant cardiac malformations involving DCRV, tricuspid valve dysplasia, perimembranous ventricular septal defect and mild subaortic stenosis have not been previously

  16. 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...... on the right ventricle. The boy's clinical condition improved markedly, so he was discharged two weeks after the procedure. The ultimate therapeutic option for medically refractory PAH is LTX or heart-lung transplantation, but because of the short life span after LTX, time was bought by postponing the time...

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

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    Francesco Secchi

    2013-01-01

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

  18. Traumatic ventricular septal defect and flail tricuspid valve: successful management by an extracorporeal membrane oxygenator-supported hybrid approach.

    Science.gov (United States)

    Ling, Frederick S; Massey, H Todd

    2014-03-01

    We report the successful management of a critically ill patient with a traumatic ventricular septal defect (VSD) and flail tricuspid valve sustained in a motorcycle accident. Multiple orthopedic injuries prevented emergency cardiac surgery. The patient was stabilized by venous arterial extracorporeal membrane oxygenator support which allowed initial orthopedic repair. Repair of his cardiac injuries was then accomplished using a hybrid approach of percutaneous VSD closure using an Amplatzer post myocardial infarction VSD occluder which was also coil embolized followed by surgical tricuspid valve replacement. Copyright © 2013 Wiley Periodicals, Inc.

  19. Repair of postinfarct ventricular septal defect and total myocardial revascularization in a case of dextrocardia with situs inversus.

    Science.gov (United States)

    Kuthe, Sachin A; Mohite, Prashant N; Sarangi, Siddharth; Mathews, Sarin; Thingnam, Shyam K; Reddy, Sreenivas

    2011-01-01

    We report a case of an elderly man who suffered an acute myocardial infarction (MI) with the complication of a post-MI ventricular septal defect (VSD). Situs inversus with dextrocardia was diagnosed during the course of hospitalization. Total myocardial revascularization was achieved using saphenous vein conduits. The VSD was approached through the right ventricle and repaired with a polytetrafluoroethylene patch. Although several cases of coronary artery bypass grafting (CABG) in the presence of dextrocardia have been reported in the literature, this is the first case of repair of a post-MI VSD along with CABG.

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

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    Dong Li

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

  1. Extracorporeal membrane oxygenation system as a bridge to reparative surgery in ventricular septal defect complicating acute inferoposterior myocardial infarction

    Science.gov (United States)

    Pascual, Isaac; Avanzas, Pablo; Hernandez-Vaquero, Daniel; Alvarez, Ruben; Díaz, Rocio; Díaz, Beatriz; Martín, María; Carro, Amelia; Muñiz, Guillermo; Silva, Jacobo; Moris, Cesar

    2017-01-01

    Post-infarction ventricular septal defect (VSD) is a rare but potentially lethal complication of acute myocardial infarction. Medical management is usually futile, so definitive surgery remains the treatment of choice but the risk surgery is very high and the optimal timing for surgery is still under debate. A 55-year-old man with no previous medical history attended the emergency-room for 12 h evolution of oppressive chest pain and strong anginal pain 7 days ago. On physical examination, blood pressure was 96/70 mmHg, pansystolic murmur over left sternal border without pulmonary crackles. An electrocardiogram revealed sinus rhythm 110 bpm, elevation ST and Q in inferior-posterior leads. Transthoracic echocardiogram showed inferoposterior akinesia, posterior-basal septal rupture (2 cm × 2 cm) with left-right shunt. Suspecting VSD in inferior-posterior acute myocardial infarction evolved, we performed emergency coronarography with 3-vessels disease and complete subacute occlusion of the mid segment of the right coronary artery. Left ventriculography demonstrated shunting of contrast from the left ventricule to the right ventricule. He was rejected for heart transplantation because of his age. Considering the high surgical risk to early surgery and his hemodynamic and clinical stability, delayed surgical treatment is decided, and 4 days after admission the patient suffered hemodynamic instability so venoarterial extracorporeal membrane oxygenation system (ECMO) is implanted as a bridge to reparative surgery. The 9th day after admission double bypass, interventricular defect repair with pericardial two-patch exclusion technique, and ECMO decannulation were performed. The patient’s postoperative course was free of complications and was discharged 10 days post VSD repair surgery. Follow-up 3-month later revealed the patient to be in good functional status and good image outcome with intact interventricular septal patch without shunt. ECMO as a bridge to reparative

  2. Miniinvasive hybrid closure of multiple muscular ventricular septal defects in a premature infant with novel use of Amplatzer Duct Occluder II – a case report

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    Ireneusz Haponiuk

    2011-03-01

    Full Text Available Muscular ventricular septal defects (mVSD appearing together with other septal defects are frequently regarded as “concomitant” pathologies, that nevertheless should be considered while the patient is referred for intervention. We followed a conception of mVSDs’ miniinvasive treatment with a hybrid approach based on perventricular implantation of occluding devices. In this paper we report a hybrid procedure performed in a premature infant referred for surgical correction of a large perimembranous VSD with a simultaneous perventricular approach for concomitant muscular ventricular septal defect. The device of choice, because of the patient’s small size and weight, was the Amplatzer Duct Occluder II. Colour Doppler showed complete closure of all VSDs 8 months after surgery with no complications related to the procedure.

  3. Genetic polymorphisms of the TYMS gene are not associated with congenital cardiac septal defects in a Han Chinese population.

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    Jian-Yuan Zhao

    Full Text Available BACKGROUND: Clinical research indicates that periconceptional administration of folic acid can reduce the occurrence of congenital cardiac septal defects (CCSDs. The vital roles of folate exhibits in three ways: the unique methyl donor for DNA expression regulation, the de novo biosynthesis of purine and pyrimidine for DNA construction, and the serum homocysteine removal. Thymidylate synthase (TYMS is the solo catalysis enzyme for the de novo synthesis of dTMP, which is the essential precursor of DNA biosynthesis and repair process. To examine the role of TYMS in Congenital Cardiac Septal Defects (CCSDs risk, we investigated whether genetic polymorphisms in the TYMS gene associated with the CCSDs in a Han Chinese population. METHOD: Polymorphisms in the noncoding region of TYMS were identified via direct sequencing in 32 unrelated individuals composed of half CCSDs and half control subjects. Nine SNPs and two insertion/deletion polymorphisms were genotyped from two independent case-control studies involving a total of 529 CCSDs patients and 876 healthy control participants. The associations were examined by both single polymorphism and haplotype tests using logistic regression. RESULT: We found that TYMS polymorphisms were not related to the altered CCSDs risk, and even to the changed risk of VSDs subgroup, when tested in both studied groups separately or in combination. In the haplotype analysis, there were no haplotypes significantly associated with risks for CCSDs either. CONCLUSION: Our results show no association between common genetic polymorphisms of the regulatory region of the TYMS gene and CCSDs in the Han Chinese population.

  4. Cardiac resynchronization therapy for heart failure induced by left bundle branch block after transcatheter closure of ventricular septal defect

    Science.gov (United States)

    Du, Rong-Zeng; Qian, Jun; Wu, Jun; Liang, Yi; Chen, Guang-Hua; Sun, Tao; Zhou, Ye; Zhao, Yang; Yan, Jin-Chuan

    2014-01-01

    A 54-year-old female patient with congenital heart disease had a persistent complete left bundle branch block three months after closure by an Amplatzer ventricular septal defect occluder. Nine months later, the patient suffered from chest distress, palpitation, and sweating at daily activities, and her 6-min walk distance decreased significantly (155 m). Her echocardiography showed increased left ventricular end-diastolic diameter with left ventricular ejection fraction of 37%. Her symptoms reduced significantly one week after received cardiac resynchronization therapy. She had no symptoms at daily activities, and her echo showed left ventricular ejection fraction of 46% and 53%. Moreover, left ventricular end-diastolic diameter decreased 6 and 10 months after cardiac resynchronization therapy, and 6-min walk distance remarkably increased. This case demonstrated that persistent complete left bundle branch block for nine months after transcatheter closure with ventricular septal defect Amplatzer occluder could lead to left ventricular enlargement and a significant decrease in left ventricular systolic function. Cardiac resynchronization therapy decreased left ventricular end-diastolic diameter and increased left ventricular ejection fraction, thereby improving the patient's heart functions. PMID:25593586

  5. Coronary to pulmonary fistula as the primary source of pulmonary blood supply in pulmonary atresia with ventricular septal defect

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    Isman Firdaus

    2004-12-01

    Full Text Available A communication between the coronary and pulmonary arteries, so called coronary to pulmonary fistula, is a rare source of pulmonary supply in pulmonary atresia (PA with ventricular septal defect (VSD. A 4 year old girl referred to National Cardiovascular Center Harapan Kita, Jakarta with symptoms and signs of increased pulmonary blood flow since infancy and was confirmed by the chest x-rays. Heart examination revealed normal first heart sound with single loud second heart sound and an ejection systolic murmur at the pulmonary area. ECG demonstrated sinus rhythm with normal axis and biventricular hypertrophy. Echocardiography was performed and truncus arteriosus (TA type I was suspected with perimembranus VSD, overriding of the aorta, and dilated main pulmonary artery. But on cardiac catheterization studies, a non obstructive fistula was found between the left coronary and main pulmonary artery coexisted with PA and VSD. A successful surgery was performed subsequently and confirmed the above diagnosis. Although there were episodes of pulmonary hypertension crisis during early post operative course, she was then discharge from the hospital in a good condition. Since irreversible pulmonary vascular disease may develop in a non restrictive coronary to pulmonary fistula, early recognition of this anomaly is very important for better surgical result. (Med J Indones 2004; 13: 237-40Keywords: coronary to pulmonary fistula, pulmonary atresia, ventricular septal defect

  6. A rare type of Gerbode defect.

    Science.gov (United States)

    Panduranga, Prashanth; Mukhaini, Mohammed

    2011-07-01

    A Gerbode defect is a left ventricle to right atrial communication. The type I defect (direct, acquired) results in a direct shunt through the atrioventricular part of membranous septum, while a type II (indirect, congenital) defect results in an indirect shunt through a perimembranous ventricular septal defect (VSD) and a defect in the septal tricuspid valve leaflet. We report a rare type of Gerbode defect wherein a small perimembranous VSD is completely covered by an elongated sail-like anterior tricuspid leaflet forming an aneurysm and directing the shunt into right atrium. © 2011, Wiley Periodicals, Inc.

  7. An unusual variation of left ventricular-right atrial communication.

    Science.gov (United States)

    Komai, H; Naito, Y; Fujiwara, K; Takagaki, Y; Nishimura, Y; Kawasaki, S

    1996-01-01

    We herein present a 5-month-old boy with an infravalvular-type left ventricular-right atrial communication without an abnormal tricuspid valve leaflet. The thick chorda of the anterior tricuspid leaflet was inserted into the upper edge of the ventricular septal defect which was placed just under the commissure. The jet from the septal defect pushed this thick chorda and opened the commissure, which thus caused the abnormal pathway from the left ventricle to the right atrium. This mechanism of left ventricular-right atrial communication has not yet been reported elsewhere.

  8. Second natural history study of congenital heart defects. Quality of life of patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect.

    Science.gov (United States)

    Gersony, W M; Hayes, C J; Driscoll, D J; Keane, J F; Kidd, L; O'Fallon, W M; Pieroni, D R; Wolfe, R R; Weidman, W H

    1993-02-01

    Quality of life of patients with congenital heart defects is an important aspect of the assessment of outcome of medical and surgical treatment. All participants in the Second Natural History Study of Congenital Heart Defects who completed the study questionnaire were included in this analysis. The questionnaire completed by the patients included inquiries relative to medical history, marital and family life, self-perception of well-being, insurability, and employability. The following general observations were apparent. The study cohort reported a self-perception of health status that was similar to that of the general population. Second, 35-40% of patients had not had a cardiac evaluation within the previous 10 years. Third, 16% of the patients had no health insurance, and 35.7% of the patients had no life insurance. For the patients with pulmonary stenosis and ventricular septal defect, the percent who were married was less than that of the corresponding national age- and sex-specific population. For patients with aortic stenosis, the percent married was greater than that of the general population for some age groups. The proportion of divorced or separated individuals was similar to that of the general population. For all three defects, the level of educational attainment exceeded the national average. Last, unemployment rates were similar to national averages except for women with aortic stenosis, who had a significantly higher unemployment rate than did age- and sex-matched controls. Patients initially identified with aortic stenosis, pulmonary stenosis, or ventricular septal defect in 1959-1973 have a quality of life in the mid-1980s similar to that of the general US population.

  9. Preincision Initiation of Dexmedetomidine Maximally Reduces the Risk of Junctional Ectopic Tachycardia in Children Undergoing Ventricular Septal Defect Repairs.

    Science.gov (United States)

    Gautam, Nischal K; Turiy, Yuliya; Srinivasan, Chandra

    2017-04-10

    To evaluate whether initiation of dexmedetomidine (DEX) infusion before surgical incision and cardiopulmonary bypass (CPB) versus initiation after CPB had an impact on the incidence of junctional ectopic tachycardia (JET). Retrospective cohort study. Single tertiary-care cardiac center. Children undergoing cardiopulmonary bypass for repair of congenital heart disease involving ventricular septal defects between January 2010 and February 2013. None. One hundred thirty-four patients undergoing ventricular septal defect closure were included in the final analysis. Of the 99 patients (74%) exposed to DEX, intraoperative initiation was performed in 73 (pre-CPB, n = 39 patients [29%]; intraoperative post-CPB initiation, n = 34 patients [25%]), and postoperative initiation was performed on arrival to the intensive care unit (ICU) in 26 patients (19%). In 71 of the 73 patients, infusions that were initiated intraoperatively were continued in the postoperative period for up to the first 12 hours. Postoperative JET was observed in 22 of the 134 patients (15%). Of the 99 patients exposed to DEX in the perioperative period, JET was observed in 8 patients (11%). Of the 35 patients not exposed to any DEX, JET was observed in 12 patients (34%). Analysis was performed using DEX exposure and timing as predictor variables. Multivariable analysis modeled with DEX exposure as a predictor variable showed that when initiated preincision and continued through the postoperative period, DEX was associated with significant reduction in postoperative JET (odds ratio [OR] 0.09, 95% confidence interval [CI] 0.02-0.37, p = 0.002). Exposure to DEX in the postoperative period alone did not result in suppression of JET (OR 0.5, 95% CI 0.11-2.17, p = 0.366). When modeled by using timing of DEX initiation as the predictive variable, preincision initiation of DEX infusion resulted in significantly greater suppression of JET (OR 0.04, 95% CI 0.002-0.28, p = 0.006) compared with initiation

  10. Closure of patent foramen ovale defects using GORE® CARDIOFORM septal occluder

    DEFF Research Database (Denmark)

    Hardt, Stefan E; Eicken, Andreas; Berger, Felix

    2017-01-01

    (2.6%) were diagnosed new onset paroxysmal atrial fibrillation, which were successfully treated. No thrombembolic events occurred. Closure was successful in 94.2% of subjects at discharge evaluation and 96.9% at 6 months follow-up. CONCLUSION: This prospective, multicenter study adds to previous...

  11. 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.

  12. Clinical Characteristics of Adult Uncorrected Secundum Atrial Septal Defect, A Pilot Study

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    Lucia Krisdinarti

    2016-12-01

    Defek septum atrium (DSA merupakan penyakit jantung bawaan yang paling sering dijumpai pada usia dewasa karena tanda dan gejalanya yang samar. Hipertensi paru (HP merupakan komplikasi DSA yang mendorong pasien mendatangi rumah sakit karena munculnya gejala yang berat. Hampir semua pasien DSA dewasa mengalami HP yang menyebabkan angka kesakitan dan kematian yang bermakna. Tujuan penelitian ini adalah untuk mengetahui karakteristik klinis pasien dengan DSA dan HP. Disain penelitian adalah potong lintang. Subjek dicuplik secara berurutan dari klinik rawat jalan dan rawat inap. Data demografi, medis dan pencitraan dikumpulkan dan dicatat dalam suatu case report form. Penelitian deskriptif dilakukan untuk menilai karakteristik subjek. Sebanyak tujuh puluh enam subjek dicuplik. Mayoritas subjek adalah wanita (77,6 % dalam usia muda, produktif dan subur (63,2 %. Gejala paling sering adalah sesak saat aktivitas, mudah lelah, dan berdebar. Sebagian besar pasien masuk dalam kelas fungsional WHO II (70,2 %. Rerata saturasi oksigen 96,4 %. Berdasar hasil ekhokardiografi, sebanyak 77,6 subjek telah mengalami HP. Rerata diameter terpanjang defek adalah 2,7 centimeter. Arah aliran darah melewati defek sebagian besar kiri ke kanan (77,6 %. Fungsi ventrikel kanan dan kiri dalam batas normal. Kateterisasi jantung kanan menunjukkan rerata tekanan atrium kiri sebesar 11,5 mmHg yang menandakan HP prekapiler/arteri. Rerata tekanan arteri pulmonalis sebesar 42.0 mmHg. Nilai indeks tahanan arteri pulmonalis sebagian besar kurang dari 4 Wood unit/m2 (63,7 % yang menunjukkan kemungkinan bisa dilakukan penutupan defek. Sedangkan 24,6 % subjek kontraindikasi untuk penutupan. Hipertensi arteri pulmonalis (HAP didiagnosis pada 77,6 % subjek, sedangkan 13,2 % subjek mengalami HAP borderline. Sebagai kesimpulan, sebagian besar DSA dewasa telah mengalami HAP, sebagian besar wanita muda yang berusia produktif, sebagian besar mengunjungi rumah sakit karena gejala HP, arah aliran sebagian besar dari kiri ke kanan dan sebagian besar telah mengalami penurunan kapasitas fungsional.

  13. Complete atrioventricular block after percutaneous device closure of perimembranous ventricular septal defect: A single-center experience on 1046 cases.

    Science.gov (United States)

    Bai, Yuan; Xu, Xu-Dong; Li, Chang-Yong; Zhu, Jia-Qi; Wu, Hong; Chen, Shao-Ping; Chen, Feng; You, Xiao-Hua; Zhao, Xian-Xian; Qin, Yong-Wen

    2015-10-01

    Complete atrioventricular block (cAVB) has been deemed a rare complication after transcatheter closure for ventricular septal defect (VSD). However, this serious event appears to be underrecognized and is worth being investigated further. To determine the incidence and predisposing factors of cAVB associated with closure of VSD using a modified double-disk occluder (MDO). From December 21, 2001 to December 31, 2014, 1046 patients with perimembranous ventricular septal defect underwent percutaneous closure using the MDO. Electrocardiography was evaluated before the procedure, within 1 week after the procedure, and then at 1, 3, 6, and 12 months and every year thereafter. Other baseline and procedural parameters were also evaluated and a comparison between patients requiring pacemakers and those not suffering from cAVB was done. cAVB occurred in 17 patients (1.63%) after the procedure. Of the 17 patients, 8 underwent permanent pacemaker (PPM) implantation. The cAVB occurred within 30 days after the procedure in 14 patients and after 1 year in 3 patients. In comparison patients aged ≤18 years, patients aged >18 years were more prone to cAVB (P = .025). Logistic regression revealed no significant parameter to predict later requirement for PPM. The incidence of cAVB after transcatheter closure of VSD was acceptable, as part of the cAVB population recovered after administration of corticosteroid and application of a temporary pacemaker. Late cAVB (>1 year) appears to make it more difficult to restore normal conduction block. Because of the recurrence of cAVB, life-long follow-up with periodic electrocardiography examination may be mandatory. Copyright © 2015. Published by Elsevier Inc.

  14. Time Course of Atrial Fibrillation in Patients With Congenital Heart Defects

    NARCIS (Netherlands)

    Teuwen, C.P.; Ramdjan, T.T.; Gotte, M.; Brundel, B.J.; Evertz, R.; Vriend, J.W.; Molhoek, S.G.; Dorman, H.G.; Opstal, J.M. van; Konings, T.C.; Voort, P. van der; Delacretaz, E.; Houck, C.; Yaksh, A.; Jansz, L.J.; Witsenburg, M.; Roos-Hesselink, J.W.; Triedman, J.K.; Bogers, A.J.; Groot, N.M. de

    2015-01-01

    BACKGROUND: The incidence of atrial fibrillation (AF) is rising in the aging patients with congenital heart defects (CHD). However, studies reporting on AF in patients with CHD are scarce. The aim of this multicenter study was to examine in a large cohort of patients with a variety of CHD: (1) the

  15. Time Course of Atrial Fibrillation in Patients With Congenital Heart Defects

    NARCIS (Netherlands)

    Teuwen, Christophe P.; Ramdjan, Tanwier T. T. K.; Gotte, Marco; Brundel, Bianca J. J. M.; Evertz, Reinder; Vriend, Joris W. J.; Molhoek, Sander G.; Dorman, Henderikus G. R.; van Opstal, Jurren M.; Konings, Thelma C.; van der Voort, Pepijn; Delacretaz, Etienne; Houck, Charlotte; Yaksh, Ameeta; Jansz, Luca. J.; Witsenburg, Maarten; Roos-Hesselink, Jolien W.; Triedman, John K.; Bogers, Ad J. J. C.; de Groot, Natasja M. S.

    2015-01-01

    Background The incidence of atrial fibrillation (AF) is rising in the aging patients with congenital heart defects (CHD). However, studies reporting on AF in patients with CHD are scarce. The aim of this multicenter study was to examine in a large cohort of patients with a variety of CHD: (1) the

  16. Anesthetic management of a child with complete atrioventricular septal defect and single ventricle posted for noncardiac surgery

    Science.gov (United States)

    Mir, Aabid Hussain; Ali, Zulfiqar; Dar, Bashir Ahmad; Naqash, Imtiaz A.; Bashir, Samreena

    2016-01-01

    Congenital heart defects are associated with various physiological disturbances. They pose anesthetic challenges for both cardiac and noncardiac surgeries. Atrioventricular septal defects are due to a developmental failure in the separation of atria and the ventricles into separate chambers and failure in the separation of mitral and tricuspid valves. We present a case of a child (1½ years), weighing 10 kg, diagnosed as congenital hydrocephalus who was planned for ventriculoperitoneal shunt. Child was having an oxygen saturation of 76% on room air. Anesthesia was induced with morphine and propofol. After tracheal intubation, saturation improved to 93%. Anesthesia was maintained with a combination of oxygen and nitrous oxide along with isoflurane. Measures were taken to maintain normovolemia and avoid hypotension, hypoxia, tachycardia, cardiac dysrhythmias and acidosis. The patient remained hemodynamically stable, maintaining arterial blood gasses within normal limits. The overall intraoperative course remained uneventful. At the end of the procedure, patient was reversed with neostigmine 60 mcg/kg and glycopyrrolate 10 mcg/kg. Extubation was done after the child was alert and opening eyes and was shifted to intensive care on oxygen inhalation for further monitoring. PMID:27746571

  17. Automatic classification framework for ventricular septal defects: a pilot study on high-throughput mouse embryo cardiac phenotyping.

    Science.gov (United States)

    Xie, Zhongliu; Liang, Xi; Guo, Liucheng; Kitamoto, Asanobu; Tamura, Masaru; Shiroishi, Toshihiko; Gillies, Duncan

    2015-10-01

    Intensive international efforts are underway toward phenotyping the entire mouse genome by modifying all its [Formula: see text] genes one-by-one for comparative studies. A workload of this scale has triggered numerous studies harnessing image informatics for the identification of morphological defects. However, existing work in this line primarily rests on abnormality detection via structural volumetrics between wild-type and gene-modified mice, which generally fails when the pathology involves no severe volume changes, such as ventricular septal defects (VSDs) in the heart. Furthermore, in embryo cardiac phenotyping, the lack of relevant work in embryonic heart segmentation, the limited availability of public atlases, and the general requirement of manual labor for the actual phenotype classification after abnormality detection, along with other limitations, have collectively restricted existing practices from meeting the high-throughput demands. This study proposes, to the best of our knowledge, the first fully automatic VSD classification framework in mouse embryo imaging. Our approach leverages a combination of atlas-based segmentation and snake evolution techniques to derive the segmentation of heart ventricles, where VSD classification is achieved by checking whether the left and right ventricles border or overlap with each other. A pilot study has validated our approach at a proof-of-concept level and achieved a classification accuracy of 100% through a series of empirical experiments on a database of 15 images.

  18. Gerbode Defect—A Rare Defect of Atrioventricular Septum and Tricuspid Valve

    Science.gov (United States)

    Gangurde, Pranil; Mahajan, Ajay

    2015-01-01

    Left ventricular to right atrial communications (the Gerbode defect) are rare types of ventricular septal defect and present as direct or an indirect type. We hereby, report two cases, one direct and another indirect type. Cardiopulmonary bypass surgery was done and a successful suture closure of ventricularseptal defect using pericardial patch was performed. PMID:26500939

  19. Pulmonary atresia and ventricular septal defect with collaterals to right lung associated with anomalous left pulmonary artery from the ascending aorta

    Energy Technology Data Exchange (ETDEWEB)

    Khositseth, Anant [Mahidol University, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Bangkok (Thailand); Siripornpitak, Suvipaporn; Pornkul, Ratanaporn [Mahidol University, Department of Radiology, Ramathibodi Hospital, Bangkok (Thailand)

    2010-12-15

    We present a 10-month-old boy with cyanosis. This is a rare case of pulmonary atresia, ventricular septal defect (VSD), major aorto-pulmonary collateral arteries (MAPCAs) to the right lung with absent native right pulmonary artery (RPA) in association with anomalous left pulmonary artery (LPA) from the ascending aorta (AAo). Echocardiography was unable to identify all of the cardiovascular abnormalities. Multidetector CT demonstrated all of these abnormalities and is the investigation of choice instead of cardiac catheterization. (orig.)

  20. 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.

  1. Important ECG diagnosis-aiding indices of ventricular septal defect children with or without congestive heart failure.

    Science.gov (United States)

    Guo, M; Huang, M N; Bai, Z; Hsieh, K S

    2001-04-15

    In this paper we perform a statistical study of the conventional RR intervals and two newly defined PR' and RT intervals of ECG data. A quadratic classification rule is applied to extract several important ECG diagnosis-aiding indices among normal children and children with ventricular septal defect (VSD) with or without congestive heart failure (CHF). The results show that certain statistics computed from PR', RR and RT intervals are important diagnosis-aiding indices. Best classification vectors are searched for pairwise classification. Two methods, minimum distance criterion and a two-stage classification procedure, are considered for three-way classification. Furthermore, logistic regression models based on transformations of these important diagnosis-aiding indices are proposed. The receiver operating characteristic curves of the proposed models show better performance than those of linear and quadratic logistic models. In order to proceed with this study, a computer algorithm to automatically detect the three intervals is developed and the related ECG data are collected and analysed. The algorithm is also enhanced with an outlier detection procedure for the automatic measurements of the PR' and RT intervals. Copyright 2001 John Wiley & Sons, Ltd.

  2. Radiofrequency-assisted "reconstruction" of the right ventricular outflow tract in muscular pulmonary atresia with ventricular septal defect.

    Science.gov (United States)

    Hausdorf, G; Schulze-Neick, I; Lange, P E

    1993-04-01

    A case of pulmonary atresia with ventricular septal defect is reported in which a communication was established between the right ventricle and the hypoplastic pulmonary artery by intervention, despite muscular atresia of the right ventricular outflow tract. The atresia was perforated with a special designed radiofrequency catheter (Osypka). After the creation of a canal within the muscular atresia, balloon dilatation (diameters 2, 3.5, and 7.2 mm) was performed. Arterial oxygen saturation increased from 64% to 78%. Lateral deviation of the radiofrequency catheter resulting in a lateral perforation of the atretic muscular infundibulum was well tolerated without later sequelae. Early restenosis within two weeks necessitated the implantation of a stent within the "recanalised" atresia resulting in an increased anterograde flow to the pulmonary artery. This case shows that "recanalisation" of muscular atresia of the pulmonary artery by radiofrequency is a promising technique. Additionally, stent implantation into the infundibulum to prevent restenosis is a first step to interventional right ventricular outflow tract reconstruction.

  3. Double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly) and other complex congenital cardiac malformations in an American Quarter Horse foal.

    Science.gov (United States)

    Kohnken, Rebecca; Schober, Karsten; Godman, Jennifer; Gardner, Alison; Jenkins, Tiffany; Schroeder, Eric; Baker, Peter; Dunbar, Laura

    2018-02-01

    A 4-week-old American Quarter Horse colt presented with a recent history of diarrhea and decreased activity level. On initial physical examination, the animal was bright and alert and major findings were limited to a loud systolic heart murmur radiating widely over both sides of the thorax. While in the hospital, the clinical condition of the foal warranted further imaging to determine the cause and extent of cardiac disease. A variety of congenital cardiac malformations were identified during echocardiographic examination and autopsy, including a double outlet right ventricle and a subpulmonary interventricular septal defect (Taussig-Bing anomaly), ventricular inversion with atrioventricular discordance, tricuspid valve atresia, a septum primum interatrial septal defect, mitral valve dysplasia with a cleft in the septal mitral valve cusp, aortic, and subaortic stenosis, tubular hypoplasia of the ascending aorta and the aortic arch, a patent ductus arteriosus, an aberrant circumflex coronary artery, and aberrant left and right subclavian arteries. Echocardiographic and postmortem findings of the cardiac defects in this foal are presented and discussed. Copyright © 2017 Elsevier B.V. All rights reserved.

  4. 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. 

  5. 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

  6. Ablação por cateter do flutter atrial. Caracterização eletrofisiológica da interrupção da condução pelos istmos posterior e septal Catheter ablation of atrial flutter. Electrophysiological characterization of posterior and septal isthmus block

    Directory of Open Access Journals (Sweden)

    José Marcos Moreira

    1998-07-01

    Full Text Available OBJETIVO: Avaliar os tipos de bloqueio obtidos nos istmos posterior (entre o anel tricuspídeo e veia cava inferior e septal (entre o anel tricuspídeo e óstio do seio coronário, após ablação do flutter atrial (FLA. MÉTODOS: Foram submetidos à ablação por radiofreqüência (RF 14 pacientes com FLA tipo I (9 homens em 16 procedimentos. A ativação atrial ao redor do anel tricuspídeo foi avaliada em ritmo sinusal utilizando-se cateter "Halo" com 10 pares de eletrodos (H1-2 a H19-20, durante estimulação do seio coronário proximal (SCP e região póstero-lateral do átrio direito (H1-2, antes e após ablações lineares. De acordo com a frente de programação do impulso definiu-se: ausência de bloqueio (condução bidirecional, bloqueio incompleto (condução bidirecional com retardo num dos sentidos e bloqueio completo (ausência de condução pelo istmo. O intervalo desta ativação (deltaSCP/H1-2 foi analisado. RESULTADOS: Bloqueio completo foi obtido em 7 procedimentos (44% e incompleto em 4 (25%. O deltaSCP/H1-2 foi de 74 ± 26ms no primeiro grupo e de 30,5 ± 7,5ms no segundo (pPURPOSE: Evaluate the different types of conduction blocks obtained between inferior vena cava-tricuspid annulus (posterior isthmus and between tricuspid annulus-coronary sinus ostium (septal isthmus after radiofrequency (RF catheter ablation of atrial flutter (AFL METHODS: In 16 procedures, 14 patients (pts, 9 male, with type I AFL underwent RF ablation. Atrial activation around tricuspid annulus was performed with a 10-bipole "Halo" catheter (H1-2; H19-20. In sinus rhythm, isthmus conduction was evaluated during proximal coronary sinus (PCS and low lateral right atrium (H1-2 pacing, before and after linear ablation. According to the wave front of impulse propagation we assessed absence of block (bidirectional conduction; incomplete block (bidirectional conduction with delay in one front of impulse propagation and complete block (absence of conduction

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

    Science.gov (United States)

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

    2015-11-01

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

  8. Does surgically induced right bundle branch block really effect ventricular function in children after ventricular septal defect closure?

    Science.gov (United States)

    Karadeniz, Cem; Atalay, Semra; Demir, Fikri; Tutar, Ercan; Ciftci, Omer; Ucar, Tayfun; Uysalel, Adnan; Eyileten, Zeynep

    2015-03-01

    In this prospective study, we aimed to assess left and right ventricular function in terms of the presence of right bundle branch block (RBBB) in the cases with repaired ventricular septal defect (VSD). Fifty-three patients who had VSD surgery at least 1-year preceding admission and 52 healthy controls were enrolled into the study. All the participants underwent electrocardiographic and echocardiographic examination. The cases with RBBB were determined. The conventional and tissue Doppler echocardiographic measurements of the patients with and without RBBB were compared with each other and healthy controls. Twenty-eight of VSD repair groups were male and 25 were female. Control group consisted of 30 males and 22 females. The mean age of the study and control groups was 7.5 ± 5.0 and 6.9 ± 4.3 years, respectively. RBBB was detected in 20 of 53 (37.7 %) operated patients. The only significant difference between the cases with and without RBBB was decreased right ventricular fractional area change (%) in the former group (33 ± 7 vs. 39 ± 5 p < 0.05). When compared to controls, operated group had statistically lower [corrected] tricuspid annular plane systolic excursion (p < 0.05), lower systolic, early diastolic, and late diastolic myocardial velocities, higher left and right ventricular myocardial performance indices, irrespective of the presence of RBBB. The ratios of mitral or tricuspid inflow to left or right ventricular myocardial in early diastolic velocities measured from lateral annular levels were increased in operated group (all p values <0.05). In conclusion, RBBB in the cases with surgical VSD repair might be associated with right ventricular dysfunction. Biventricular systolic and diastolic dysfunction may develop following VSD repair irrespective of the presence of RBBB. Tissue Doppler-derived myocardial performance indices are useful in detection of those subclinical dysfunctions.

  9. De Novo and Rare Variants at Multiple Loci Support the Oligogenic Origins of Atrioventricular Septal Heart Defects.

    Directory of Open Access Journals (Sweden)

    James R Priest

    2016-04-01

    Full Text Available Congenital heart disease (CHD has a complex genetic etiology, and recent studies suggest that high penetrance de novo mutations may account for only a small fraction of disease. In a multi-institutional cohort surveyed by exome sequencing, combining analysis of 987 individuals (discovery cohort of 59 affected trios and 59 control trios, and a replication cohort of 100 affected singletons and 533 unaffected singletons we observe variation at novel and known loci related to a specific cardiac malformation the atrioventricular septal defect (AVSD. In a primary analysis, by combining developmental coexpression networks with inheritance modeling, we identify a de novo mutation in the DNA binding domain of NR1D2 (p.R175W. We show that p.R175W changes the transcriptional activity of Nr1d2 using an in vitro transactivation model in HUVEC cells. Finally, we demonstrate previously unrecognized cardiovascular malformations in the Nr1d2tm1-Dgen knockout mouse. In secondary analyses we map genetic variation to protein-interaction networks suggesting a role for two collagen genes in AVSD, which we corroborate by burden testing in a second replication cohort of 100 AVSDs and 533 controls (p = 8.37e-08. Finally, we apply a rare-disease inheritance model to identify variation in genes previously associated with CHD (ZFPM2, NSD1, NOTCH1, VCAN, and MYH6, cardiac malformations in mouse models (ADAM17, CHRD, IFT140, PTPRJ, RYR1 and ATE1, and hypomorphic alleles of genes causing syndromic CHD (EHMT1, SRCAP, BBS2, NOTCH2, and KMT2D in 14 of 59 trios, greatly exceeding variation in control trios without CHD (p = 9.60e-06. In total, 32% of trios carried at least one putatively disease-associated variant across 19 loci,suggesting that inherited and de novo variation across a heterogeneous group of loci may contribute to disease risk.

  10. Intramural Ventricular Septal Defect is a Distinct Clinical Entity Associated with Postoperative Morbidity in Children after Repair of Conotruncal Anomalies

    Science.gov (United States)

    Patel, Jyoti K; Glatz, Andrew C; Ghosh, Reena M; Jones, Shannon M; Natarajan, Shobha; Ravishankar, Chitra; Mascio, Christopher E; Spray, Thomas L; Cohen, Meryl S

    2015-01-01

    Background Intramural ventricular septal defects (VSDs) are interventricular communications through right ventricular free wall trabeculations that can occur after repair of conotruncal anomalies. We assessed the prevalence of residual intramural VSDs and their effect on postoperative course. Methods and Results Children who underwent biventricular repair of a conotruncal anomaly from 1/1/06 to 6/30/13 and had a post-operative transthoracic echocardiogram were included. Images were reviewed for residual intramural or non-intramural VSDs. The primary outcome was a composite of mortality, extracorporeal membrane oxygenation (ECMO) use, and need for subsequent catheter or surgical VSD closure. The secondary outcome was post-operative hospital length of stay (PLOS). A residual VSD was present in 256 of the 442 subjects (58%), of which 231 (90%) were <2mm in size. Forty-nine (11%) had intramural VSDs and 207 (47%) had non-intramural VSDs. Patients with intramural VSDs were more likely to reach the primary composite outcome compared to those with non-intramural VSDs or no residual VSD (14/49 [29%] vs 15/207 [7%] vs 6/186 [3%], p<0.0001). In addition, those with intramural VSDs had longer PLOS compared to those with non-intramural VSDs or no residual VSD (20 days [IQR 11-42] vs 7 days [5-14] vs 6 [4-11], p=0.0001). These associations remained significant after adjusting for known risk factors for poor outcomes, including residual VSD size and operative complexity. Conclusions Among residual VSDs after repair of conotruncal anomalies, intramural VSDs are uniquely associated with postoperative morbidity, mortality, and longer PLOS. It is important to recognize intramural VSDs in the postoperative period. PMID:26246174

  11. Early Correction of Common Atrioventricular Septal Defects: A Single-Center 20-Year Experience.

    Science.gov (United States)

    Vida, Vladimiro L; Tessari, Chiara; Castaldi, Biagio; Padalino, Massimo A; Milanesi, Ornella; Gregori, Dario; Stellin, Giovanni

    2016-12-01

    Over the past 20 years our policy has been to electively repair common atrioventricular canal defects (CAVCD) in patients between 8 and 12 weeks of age. We sought to evaluate the results of our past 20-year experience. From January 1992 to April 2014, 159 consecutive patients underwent CAVCD repair (133 patients had complete CAVCD and 26 patients had a transitional form of CAVCD). Surgical repair was accomplished with a double-patch (n = 137 [86%]) or a modified single patch (n = 22 [14%]) technique. Median age at operation was 96 days (interquartile range [IQR], 73-128 days); 90 patients were younger than 3 months of age. There were 3 operative (1.9%) and 12 late (7.7%) deaths. Median follow-up time after repair was 8.2 years (IQR, 3.6-15 years). Twenty patients (13%) required reoperation-16 (10%) for left atrioventricular valve (LAVV) regurgitation. Reoperation on the LAVV was more frequent in patients with a dysplastic LAVV preoperatively (p = 0.01; odds ratio [OR], 4.2; 95% confidence interval [CI], 1.33-13.5) and in patients who underwent closure for an absent/incomplete cleft at the time of repair (p = 0.01; OR, 5.4; 95% CI, 1.4-21). Late LAVV performance (regurgitation greater than or equal to moderate or the need for reoperation), including late deaths and patients who underwent reoperation, was significantly worse in patients older than 3 months at repair (10 of 83 patients [12%] versus 20 of 73 patients [27%]; hazard ratio [HR], 2.71; 95% CI, 1.19-6.19) and in patients with LAVV dysplasia (19 of 68 patients [28%] versus 11 of 88 patients [12%]; HR, 3; 95% CI, 1.53-8.51). Individualized early repair of CAVCD is safe and beneficial, with good early and long-term results. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  12. Right atrial myxoma as a possible cause of hemorrhagic stroke and ...

    African Journals Online (AJOL)

    A clinical diagnosis of hypertensive hemorrhagic cerebrovascular accident was made. The patient died suddenly a few hours after presentation. Post‑mortem examination revealed a small intracerebral hemorrhage in the left superior temporal lobe as well as a large right atrial myxoma, a ventricular septal defect in the ...

  13. ATRIAL SEPTAI. ANEURYSM' IN AN 80-YEAR-OLD WOMAN: A ...

    African Journals Online (AJOL)

    types according to the motion of the aneurysm. 4 We report the ... hypertensive heart disease was made. Figure 1: Parasternal ... which was based mainly on clinical diagnosis. 6 A report from Abidjan stated that atrial septal defect was the commonest congenital heart disease. 7 Other reports from. Nigeria, South Africa and ...

  14. [Traumatic left ventricle-right atrial communication. A case report].

    Science.gov (United States)

    Benyass, A; Belmadani, K; Lakhal, Z; Moustaghfir, A; Hda, A; Boukili, M A; Ohayon, V; Maazouzi, W; Archane, M I

    1999-11-01

    The authors report the case of an acquired left ventricle-right atrial communication after open chest trauma. The initial clinical presentation was a haemothorax and haemopericardium responding well to emergency surgical drainage. Secondarily, a systolic murmur suggesting a ventricular septal defect and signs of right heart failure were observed. Colour Doppler echocardiography led to the diagnosis of a left ventricle-right atrial communication associated with tricuspid regurgitation with dilatation of the right heart chambers and pulmonary hypertension. At surgery, a defect in the membranous interventricular septum was confirmed with rupture of the septal tricuspid leaflet causing tricuspid regurgitation. The surgeon closed the defect with a patch and performed a De Vega tricuspid valvuloplasty. The postoperative outcome was uneventful.

  15. A case of an elderly man who required repeated repair of a ventricular septal defect and tricuspid rupture after blunt chest trauma.

    Science.gov (United States)

    Matsuyama, Shigefumi; Imazuru, Tomohiro; Nakagawa, Kaori; Ikeda, Tsukasa; Harada, Tadanori; Ota, Hiroo; Ozawa, Naomi; Iida, Mitsuru; Shimokawa, Tomoki

    2017-11-25

    Several cases of traumatic ventricular septal defect (VSD) have been reported. However, traumatic VSD complicated by tricuspid rupture is rare. We report a case of traumatic VSD with tricuspid rupture who required repeated repair of both conditions. A 69-year-old man was transferred to our hospital for emergent surgical repair of traumatic VSD and tricuspid rupture. Although emergent repair was performed, a new left-to-right shunt and moderate tricuspid regurgitation appeared during his postoperative course. A reoperation was performed 4 months after the first operation. The borders of the defect were very fibrotic and strong compared with those in the first operation. Surgical treatment of traumatic VSD should be postponed in hemodynamically stable patients. When emergent repair is performed, careful follow-up is necessary to diagnose new VSD.

  16. Incremental Value of Live/Real Time Three-Dimensional over Two-Dimensional Transesophageal Echocardiography in the Assessment of Atrial Septal Pouch.

    Science.gov (United States)

    Elsayed, Mahmoud; Hsiung, Ming C; Meggo-Quiroz, L David; Elguindy, Mostafa; Uygur, Begum; Tandon, Rohit; Guvenc, Tolga; Keser, Nurgul; Vural, Mustafa G; Bulur, Serkan; Chahwala, Jugal R; Abtahi, Firoozeh; Nanda, Navin C

    2015-12-01

    An atrial septal pouch (ASP) results from partial fusion of the septum primum and the septum secundum, and depending on the site of fusion, the pouch can be left-sided (LASP) or right-sided (RASP). LASPs have been described in association with thrombi found in patients admitted with acute strokes, raising awareness of its potential cardioembolic role, especially in those with no other clearly identifiable embolic source. We retrospectively studied 39 patients in whom the presence of an ASP had been identified by three-dimensional transesophageal echocardiography (3DTEE) and who had a two-dimensional transesophageal echocardiogram (2DTEE) performed during the same clinical encounter. The incremental value provided by 3DTEE over 2DTEE included the detection of six ASPs not found by 2DTEE; the detection of two ASPs in the same subject (in four patients) not identified by 2DTEE; larger ASP measurements of length and height in over 80% of the cases; and measurement of the ASP width (elevational axis) for the calculation of the area of the ASP opening, because of its unique capability to view the pouch en face. In addition, the volume of ASP and of the echogenic masses contained in the ASP (four of 39 patients) could be calculated by 3DTEE, which is a superior parameter of size characterization when compared to individual dimensions. One of these patients who presented with ischemic stroke diagnosed by magnetic resonance imaging had a large (>2 cm) mass in a LASP, with echolucencies similar to those seen in thrombi and associated with clot lysis and resolution. This mass completely disappeared on anticoagulant therapy lending credence that it was most likely a thrombus. There was no history of stroke or any other type of embolic event in the other three patients with masses in ASP. In conclusion, this retrospective study highlights the incremental value of 3DTEE over 2DTEE in the comprehensive assessment and characterization of ASPs, which can aid in the clarification of

  17. A comparison of ventricular function during high right ventricular septal and apical pacing after his-bundle ablation for refractory atrial fibrillation.

    Science.gov (United States)

    Mera, F; DeLurgio, D B; Patterson, R E; Merlino, J D; Wade, M E; León, A R

    1999-08-01

    This study compares LV performance during high right ventricular septal (RVS) and apical (RVA) pacing in patients with LV dysfunction who underwent His-bundle ablation for chronic AF. We inserted a passive fixation pacing electrode into the RVA and an active fixation electrode in the RVS. A dual chamber, rate responsive pulse generator stimulated the RVA through the ventricular port and the RVS via the atrial port. Patients were randomized to initial RVA (VVIR) or RVS (AAIR) pacing for 2 months. The pacing site was reversed during the next 2 months. At the 2 and 4 month follow-up visit, each patient underwent a transthoracic echocardiographical study and a rest/exercise first pass radionuclide ventriculogram. We studied nine men and three women (mean age of 68 +/- 7 years) with congestive heart failure functional Class (NYHA Classification): I (3 patients), II (7 patients), and III (2 patients). The QRS duration was shorter during RVS stimulation (158 +/- 10 vs 170 +/- 11 ms, P < 0.001). Chronic capture threshold and lead impedance did not significantly differ. LV fractional shortening improved during RVS pacing (0.31 +/- 0.05 vs 0.26 +/- 0.07, P < 0.01). RVS activation increased the resting first pass LV ejection fraction (0.51 +/- 0.14 vs 0.43 +/- 0.10, P < 0.01). No significant difference was observed during RVS and RVA pacing in the exercise time (5.6 +/- 3.2 vs 5.4 +/- 3.1, P = 0.6) or the exercise first pass LV ejection fraction (0.58 +/- 0.15 vs 0.55 +/- 0.16, P = 0.2). The relative changes in QRS duration and LV ejection fraction at both pacing sites showed a significant correlation (P < 0.01). We conclude that RVS pacing produces shorter QRS duration and better chronic LV function than RVA pacing in patients with mild to moderate LV dysfunction and chronic AF after His-bundle ablation.

  18. Percutaneous closure of congenital Gerbode defect using Nit-Occlud? L? VSD coil

    OpenAIRE

    Phan, Quang T; Kim, Sang-Wook; Nguyen, Hieu L

    2017-01-01

    We present a case report about percutaneous closure of a congenital Gerbode defect using Nit-Occlud? L? VSD coil. The patient was referred to our hospital with a diagnosis of ventricular septal defect (VSD) and severe pulmonary arterial hypertension. But transthoracic echocardiography revealed a communication between the left ventricle (LV) and the right atrial (RA), called Gerbode defect. Catheterization confirmed the shunt from the LV to the RA. We successfully closed the defect with a VSD ...

  19. Time Course of Atrial Fibrillation in Patients With Congenital Heart Defects.

    Science.gov (United States)

    Teuwen, Christophe P; Ramdjan, Tanwier T T K; Götte, Marco; Brundel, Bianca J J M; Evertz, Reinder; Vriend, Joris W J; Molhoek, Sander G; Dorman, Henderikus G R; van Opstal, Jurren M; Konings, Thelma C; van der Voort, Pepijn; Delacretaz, Etienne; Houck, Charlotte; Yaksh, Ameeta; Jansz, Luca J; Witsenburg, Maarten; Roos-Hesselink, Jolien W; Triedman, John K; Bogers, Ad J J C; de Groot, Natasja M S

    2015-10-01

    The incidence of atrial fibrillation (AF) is rising in the aging patients with congenital heart defects (CHD). However, studies reporting on AF in patients with CHD are scarce. The aim of this multicenter study was to examine in a large cohort of patients with a variety of CHD: (1) the age of onset and initial treatment of AF, coexistence of atrial tachyarrhythmia and (2) progression of paroxysmal to (long-standing) persistent/permanent AF during long-term follow-up. Patients (n=199) with 15 different CHD and documented AF episodes were studied. AF developed at 49±17 years. Regular atrial tachycardia (AT) coexisting with AF occurred in 65 (33%) patients; 65% initially presented with regular AT. At the end of a follow-up period of 5 (0-24) years, the ECG showed AF in 81 patients (41%). In a subgroup of 114 patients, deterioration from paroxysm of AF to (long-standing) persistent/permanent AF was observed in 29 patients (26%) after only 3 (0-18) years of the first AF episode. Cerebrovascular accidents/transient ischemic attacks occurred in 26 patients (13%), although a substantial number (n=16) occurred before the first documented AF episode. Age at development of AF in patients with CHD is relatively young compared with the patients without CHD. Coexistence of episodes of AF and regular AT occurred in a considerable number of patients; most of them initially presented with regular AT. The fast and frequent progression from paroxysmal to (long-standing) persistent or permanent AF episodes justifies close follow-up and early, aggressive therapy of both AT and AF. © 2015 American Heart Association, Inc.

  20. Análise dos fatores de risco na correção cirúrgica do defeito septal atrioventricular de forma total Risk factors analysis in the surgical repair of complete atrioventricular septal defect

    Directory of Open Access Journals (Sweden)

    Eduardo Keller Saadi

    1993-06-01

    Full Text Available Pacientes com defeito septal atrioventricular de forma total (DSAVT freqüentemente apresentam insuficiência cardíaca intratável e hipertensão arterial pulmonar nos primeiros meses de vida, e apenas uma minoria sobrevive sem tratamento cirúrgico precoce. Por essa razão, indica-se a correção definitiva para alterar favoravelmente a história natural da doença. Entretanto, vários fatores são responsáveis pela alta mortalidade cirúrgica. O presente trabalho estuda a experiência na correção cirúrgica do DSAVT com o objetivo de identificar alguns fatores de risco estatisticamente significativos para a ocorrência de morte operatória. Analisaram-se, retrospectivamente, 52 pacientes submetidos, entre janeiro de 1974 e dezembro de 1990, a cirurgia definitiva para correção de DSAVT no Royal Brompton and National Heart and Lung Institute, sendo estudadas as seguintes variáveis: idade, peso, sexo, ano da operação, presença de síndrome de Down, grau de regurgitação da valva AV, bandagem prévia do tronco pulmonar, presença de anomalias associadas, pressão sistólica pulmonar, duplo orifício mitral, classificação do defeito segundo Rastelli, emprego de parada circulatória e técnica de correção (1 x 2 retalhos. Todos os fatores foram avaliados isoladamente, mediante a análise univariada. Para determinar quais os fatores que, independentemente da ação de outros, contribuíram significativamente para maior mortalidade cirúrgica, foi utilizada a análise multivariada com regressão logística. A análise multivariada demonstrou que o baixo peso na época da operação e a técnica de correção com um retalho aumentam significativamente a mortalidade cirúrgica.Patients with complete atrioventricular septal defects (CAVSD frequently present with severe heart failure which cannot be controllable medically and pulmonary hypertension in infancy. Just a small number survives without early surgical treatment. For this reason

  1. Transcatheter closure of left ventricle to right atrial communication using cera duct occluder.

    Science.gov (United States)

    Ganesan, Gnanavelu; Paul, G Justin; Mahadevan, Vaikom S

    Left ventricle-right atrial communication could be congenital (Gerbode defect) or acquired as a complication of surgery or infective endocarditis and leads to volume overloading of pulmonary circulation. Two types, direct and indirect types are known depending on the involvement of septal tricuspid leaflet. Transcatheter closure of this defect is feasible and appears an attractive alternative to surgical management. Various devices like Amplatzer duct occluder I, II, Muscular ventricular septal defect device etc. have been used to close this defect. We report two patients, a preteen boy with direct left ventricle-right atrial communication as post operative complication and an adult female with indirect communication who underwent transcatheter closure with Cera duct occluder (Lifetech Scientific (Shenzhen), China). Copyright © 2017. Published by Elsevier B.V.

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

    Directory of Open Access Journals (Sweden)

    Eiki Tayama

    2012-01-01

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

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

    Science.gov (United States)

    Tayama, Eiki; Fujita, Satoshi; Ueda, Tomohiro; Imasaka, Ken-Ich; Enomoto, Naofumi; Onitsuka, Hirofumi; Tomita, Yukihiro

    2012-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Pouya Nezafati

    2015-01-01

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

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

    Science.gov (United States)

    Nezafati, Pouya; Nezafati, Mohammad Hassan; Hoseinikhah, Hamid

    2015-01-01

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

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

    Science.gov (United States)

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

    2010-02-15

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

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

    Science.gov (United States)

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

    2010-01-01

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

  8. Robotic-assisted closure of atrial septal defect under real-time three-dimensional echo guide: in vitro study

    National Research Council Canada - National Science Library

    Suematsu, Yoshihiro; Kiaii, Bob; Bainbridge, Daniel T; del Nido, Pedro J; Novick, Richard J

    2007-01-01

    ... in adults and neurodevelopmental dysfunction in children [4,5] . Recently, real-time three-dimensional echocardiography (RT3DE) has been introduced to visualize the heart noninvasively without ECG or respiratory gating. This also has great potential for expanded application not only for diagnostic purposes but also for image-guided interventio...

  9. Use of dopamine infusion improved oxygenation in a patient of Ebstein′s anomaly with atrial septal defect

    Directory of Open Access Journals (Sweden)

    Mukul C Kapoor

    2013-01-01

    Full Text Available We present the successful perioperative management of an adult patient with Ebstein′s anomaly for abdominal rectopexy surgery. The patient developed mild hypotension and a fall in peripheral oxygen saturation (SpO 2 after administration of a graded epidural block. Correction of the fall in the blood pressure; however, did not improve the SpO 2 . The patient was administered an intravenous infusion of dopamine to improve the cardiac output and this led to improvement in the SpO 2 .

  10. The predictors of dislodgement and outcomes of transcatheter closure of complex atrial septal defects in adolescents and adults

    Directory of Open Access Journals (Sweden)

    Wei-Chieh Lee

    2015-11-01

    Conclusion: Eroded and floppy IAS or aneurysm formation post ASO implantation and peri-procedure arrhythmia could predict ASO dislodgement in complex ASD closure. Transcatheter closure of ASDs under TEE guidance is feasible in complex cases.

  11. Proximal isovelocity surface area (PISA) as a noninvasive method for the estimation of the shunt quantification in perimembranous ventricular septal defects.

    Science.gov (United States)

    Eren, M; Dagdeviren, B; Bolca, O; Polat, M; Gürlertop, Y; Norgaz, T; Tezel, T

    2001-02-01

    This study was designed to assess the reliability of the proximal isovelocity surface area (PISA) method for the estimation of shunt quantification in perimembranous ventricular septal defects (PVSD). The study group was composed of 30 patients (age 11 +/- 7 years, 13 female) with PVSD. The shunt flow (Qp-Qs) and the ratio of the pulmonary flow to the systemic flow (Qp/Qs) were calculated by spectral Doppler and catheterization. The Qp-Qs, the defect area (DA), and the shunt volume (SV) were obtained by the PISA method. The PISA method estimated the DA (cm(2)/m(2)), the SV (cm(3)/m(2)), and the Qp-Qs (L/min/m(2)) to be equal to (2 x pi x R(2) x NL)/(V(max) x Body surface area), DA x TVI(shunt), and to SV x Heart rate, respectively (R is the distance of the maximal PISA from the first aliasing line to the left ventricular side of the defect, NL is the nyquist limit, and V(max) and TVI(shunt) are the peak velocity and time-velocity integral of transdefect Doppler tracing obtained by continuous-wave Doppler). The PISA method (3.4 +/- 1.5 L/min/m(2)) underestimated the Qp-Qs according to spectral Doppler (r = 0.96, P PISA findings (Qp-Qs, DA, SV) and the catheterization Qp/Qs (r = 0.86, 0.84, and 0.86; P PISA findings in identifying large defects were high (0.90, 0.93, and 0.90 for cut-off values of Qp-Qs = 3.67 L/min/m(2), DA = 0.44 cm(2)/m(2), and SV = 43 cm(3)/m(2), respectively). As a result, the PISA method can be a simple and reliable alternative to the spectral Doppler method in the identification of large shunts in PVSD.

  12. An endocardial pathway involving Tbx5, Gata4, and Nos3 required for atrial septum formation.

    Science.gov (United States)

    Nadeau, Mathieu; Georges, Romain O; Laforest, Brigitte; Yamak, Abir; Lefebvre, Chantal; Beauregard, Janie; Paradis, Pierre; Bruneau, Benoit G; Andelfinger, Gregor; Nemer, Mona

    2010-11-09

    In humans, septal defects are among the most prevalent congenital heart diseases, but their cellular and molecular origins are not fully understood. We report that transcription factor Tbx5 is present in a subpopulation of endocardial cells and that its deletion therein results in fully penetrant, dose-dependent atrial septal defects in mice. Increased apoptosis of endocardial cells lacking Tbx5, as well as neighboring TBX5-positive myocardial cells of the atrial septum through activation of endocardial NOS (Nos3), is the underlying mechanism of disease. Compound Tbx5 and Nos3 haploinsufficiency in mice worsens the cardiac phenotype. The data identify a pathway for endocardial cell survival and unravel a cell-autonomous role for Tbx5 therein. The finding that Nos3, a gene regulated by many congenital heart disease risk factors including stress and diabetes, interacts genetically with Tbx5 provides a molecular framework to understand gene-environment interaction in the setting of human birth defects.

  13. High Prevalence of Associated Birth Defects in Congenital Hypothyroidism

    Directory of Open Access Journals (Sweden)

    Alok Sachan

    2010-01-01

    Full Text Available Aim. To identify dysmorphic features and cardiac, skeletal, and urogenital anomalies in patients with congenital hypothyroidism. Patients and Methods. Seventeen children with congenital primary hypothyroidism were recruited. Cause for congenital hypothyroidism was established using ultrasound of thyroid and T99mc radionuclide thyroid scintigraphy. Malformations were identified by clinical examination, echocardiography, X-ray of lumbar spine, and ultrasonography of abdomen. Results. Ten (59% patients (6 males and 4 females had congenital malformations. Two had more than one congenital malformation (both spina bifida and ostium secundum atrial septal defect. Five (29% had cardiac malformations, of whom three had only osteum secundum atrial septal defect (ASD, one had only patent ductus arteriosus (PDA, and one patient had both ASD and PDA. Seven patients (41% had neural tube defects in the form of spina bifida occulta. Conclusion. Our study indicates the need for routine echocardiography in all patients with congenital hypothyroidism.

  14. Surgical technique of double switch procedure: Senning with arterial switch operation for congenitally corrected transposition of the great arteries with ventricular septal defect.

    Science.gov (United States)

    Ilin, Alexey S; Teplov, Pavel V; Sakovich, Valeriy A; Ohye, Richard G

    2016-01-01

    We present a case of 12-month-old boy with congenitally corrected transposition of great arteries with L-looped ventricles and L-transposition of great arteries and ventricular septal defect. When admitted to the hospital, the patient had the appearance of congestive heart failure due to moderate to severe tricuspid valve regurgitation and right ventricle dysfunction. The pulmonary artery (PA) banding was required first because of low systolic pressure in the morphological left ventricle less than 70% confirmed by catheterization. Three months later, the patient appeared to be a good candidate for anatomical repair and a double switch procedure-Senning with arterial switch-was performed. The early postoperative period was relatively smooth and uneventful. Tricuspid valve insufficiency was resolved immediately after surgery. Mild systolic dysfunction of the left ventricle with mild mitral insufficiency was confirmed by the 2D strain method of echocardiography on the second day of the postoperative period and it improved over the next 21 days. Thirty days later after the procedure, the patient underwent catheterization of his superior vena cava tunnel because of the slightly increased blood flow velocity diagnosed by echocardiography. In 3 months after the surgery, the boy was asymptomatic and was doing well. The patient's functional status was I according to the NYHA classification. © The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  15. Transcatheter closure of perimembranous ventricular septal defect with the Amplatzer(®) membranous VSD occluder 2: initial world experience and one-year follow-up.

    Science.gov (United States)

    Tzikas, Apostolos; Ibrahim, Reda; Velasco-Sanchez, Daniel; Freixa, Xavier; Alburquenque, Marcela; Khairy, Paul; Bass, John L; Ramirez, Juan; Aguirre, Daniel; Miro, Joaquim

    2014-03-01

    To describe the initial world experience and mid-term follow-up of perimembranous ventricular septal defect (pmVSD) closure with a newly designed occluder. Transcatheter closure of pmVSDs has been associated with a substantial risk of complete heart block, prompting many centers to abandon this intervention. A prospective multicenter cohort study was conducted on patients with pmVSD undergoing catheter closure using the Amplatzer(®) Membranous VSD Occluder 2 in the initial 4 pilot centers. Nineteen patients, median age 6 years (range 1.4-62 years), were enrolled and followed for 14 ± 3 months (range 8-20 months). The median weight was 26 kg (range 9.3-96 kg) and the mean Qp/Qs ratio was 1.8 ± 0.7. The defect on left ventricular side measured 9.9 ± 3.5 mm and the orifice on right ventricular side was 8.1 ± 2.8 mm by echocardiography. Mean device size was 9.4 ± 2.4 mm (range 5-14 mm). An eccentric device was employed in 9 patients (47%) and a concentric device in 10 (53%). Overall, 18 patients (95%) had successful device implants. Procedural time was 122 ± 39 min. There were no procedural complications. Mild residual shunting was initially observed in 14 (78%) patients. At last follow-up, mild residual shunting persisted in only 3 (17%) patients. There was no significant increase in aortic or tricuspid regurgitation. No patient had any degree of AV block, although one developed a transient left anterior fascicular block. Holter evaluation, obtained in all patients, was unremarkable in all. This early cohort experience using a novel adapted transcatheter closure device for pmVSD suggests that the procedure is feasible, safe, and effective. Copyright © 2013 Wiley Periodicals, Inc.

  16. Septal rhinoscleroma

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    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.

  17. 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.

  18. Effects of surgical en bloc rotation of the arterial trunk on the conduction system in children with transposition of the great arteries, ventricular septal defect and pulmonary stenosis.

    Science.gov (United States)

    Prandstetter, Christoph; Tulzer, Andreas; Mair, Rudolf; Sames-Dolzer, Eva; Tulzer, Gerald

    2016-03-01

    The standard surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis is the Rastelli operation. Recently, en bloc rotation of the arterial trunk, by cutting out the aortic and the pulmonary root in one block and by rotating it 180°, has been introduced as a new option for anatomical repair. To evaluate the effects of this surgical method on the conduction system, pre-operative, post-operative, and follow-up electrocardiograms as well as patient charts were reviewed retrospectively. A total of 16 consecutive patients with transposition of the great arteries and left outflow tract obstruction were treated with en bloc rotation. During the post-operative period, there were two patients with complete atrio-ventricular block, one with junctional ectopic tachycardia, one with ventricular tachycardia, and one with supraventricular tachycardia. None of the patients had a typical right bundle branch block pattern before surgery; however, this pattern was detectable after surgery in eight out of 16 patients (50%), which persisted during the follow-up. All patients without typical right bundle branch block pattern showed a median QRS duration of 65 ms (54-112 ms) before surgery, 62 ms (54-122 ms) after surgery, and 84 ms (66-128 ms) at the last follow-up visit. This compares well with a similar Rastelli cohort, where a right bundle branch block prevalence of 77% was reported. Out of 16 patients, 12 showed non-specific ST changes and negative T-waves, which persisted during follow-up with an unknown significance for the future. Our data suggest that en bloc rotation of the arterial trunk seems not to have more negative effects on the conduction system than the Rastelli operation.

  19. Promoting Pulmonary Arterial Growth via Right Ventricle-to-Pulmonary Artery Connection in Children With Pulmonary Atresia, Ventricular Septal Defect, and Hypoplastic Pulmonary Arteries.

    Science.gov (United States)

    Rabinowitz, Edon J; Epstein, Shilpi; Kohn, Nina; Meyer, David B

    2017-09-01

    Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation. We retrospectively reviewed data for all patients (N = 10) with initial echocardiographic diagnosis of PA-VSD and hypoplastic pulmonary arteries operated in our center from October 2008 to August 2016. Pulmonary arterial vessel size measured on preoperative and postoperative angiography was used to calculate Nakata index. Seven patients had PA-VSD, three had virtual PA-VSD, and seven had MAPCAs. All underwent creation of RV-PA connection at a median age of 7.5 days and weight 3.6 kg. Eight patients had RV-PA conduits, two had a transannular patches, and seven had major pulmonary artery reconstruction simultaneously. There were no deaths or serious morbidity; one conduit required revision prior to complete repair. Complete repair with ventricular septation and RV pressure less than half systemic was achieved in all patients at a median age of 239 days. Nakata index in neonatal period was 54 mm2/m2 (range 15-144 mm2/m2) and at time of septation 184 mm2/m2 (range 56-510 mm2/m2; P = .004). Growth rates of right and left branch pulmonary arteries were similar. The 10 patients underwent 28 catheterizations with 13 interventions in 8 patients prior to full repair. Early palliative RV-PA connection promotes pulmonary arterial growth and facilitates eventual full repair with VSD closure with low RV pressure and operative risk.

  20. Electrophysiological Mechanisms of Atrial Flutter

    Directory of Open Access Journals (Sweden)

    Ching- Tai Tai

    2006-04-01

    Full Text Available Atrial flutter (AFL is a common arrhythmia in clinical practice. Several experimental models such as tricuspid regurgitation model, tricuspid ring model, sterile pericarditis model and atrial crush injury model have provided important information about reentrant circuit and can test the effect of antiarrhythmic drugs. Human atrial flutter has typical and atypical forms. Typical atrial flutter rotates around tricuspid annulus and uses the crista terminalis and sometimes sinus venosa as the boundary. The IVC-tricuspid isthmus is a slow conduction zone and the target of radiofrequency ablation. Atypical atrial flutter may arise from the right or left atrium. Right atrial flutter includes upper loop reentry, free wall reentry and figure of eight reentry. Left atrial flutter includes mitral annular atrial flutter, pulmonary vein-related atrial flutter and left septal atrial flutter. Radiofrequency ablation of the isthmus between the boundaries can eliminate these arrhythmias.

  1. Congenital heart defects in Kabuki syndrome.

    Science.gov (United States)

    Yuan, Shi-Min

    2013-01-01

    Kabuki syndrome (KS) is an entity of multiple congenital malformations with mental retardation with undetermined etiology. Congenital heart defects are one of the clinical manifestations of KS with insufficient elucidations. Literature of congenital heart defects associated with KS was comprehensively retrieved, collected and reviewed. The clinical features of the congenital heart defects in the patients with KS were summarized. Congenital heart defects were one of the clinical manifestations of KS with 90.6% of the patients being diagnosed prenatally or at an early age. Left-sided obstructions/aortic dilation and septal defects were the fi rst two types of anomalies, accounting up to 46.1% and 32.9%, respectively. The most common congenital heart defects were coarctation of the aorta, and atrial and ventricular septal defects. Fifteen (19.7%) patients received surgical repair of congenital heart defects at a mean age of 0.8 ± 1.3 years. Congenital heart defects are one of the clinical manifestations of KS with 90.6% of the patients being diagnosed prenatally or at an early age. About 20% of the patients warranted surgical repair of the heart defects. Patients with KS require close follow-up in terms of their etiology, clinical presentations and long-term prognosis.

  2. Incidence and risk factors of post-operative arrhythmias and sudden cardiac death after atrioventricular septal defect (AVSD) correction: Up to 47years of follow-up.

    Science.gov (United States)

    Kharbanda, Rohit K; Blom, Nico A; Hazekamp, Mark G; Yildiz, Pinar; Mulder, Barbara J M; Wolterbeek, Ron; Weijerman, Michel E; Schalij, Martin J; Jongbloed, Monique R M; Roest, Arno A W

    2018-02-01

    Atrioventricular septal defect (AVSD) has an incidence of 4-5.3 per 10.000 live births and is associated with Down syndrome (DS). Data on arrhythmias and sudden cardiac death (SCD) after AVSD correction is scarce. To analyse the incidence of post-operative arrhythmias and SCD after AVSD correction and explore risk factors. This is a retrospective multicenter study including patients after biventricular AVSD correction. Univariate and multivariate analyses were performed to explore risk factors. A total of 415 patients were included with a mean follow-up duration of 9years (range; operative SVTs were documented in 33 patients (8%) and late post-operative SVTs in 15 patients (3.6%). Non-syndromic AVSD (p=0.022, HR=2.64; 95% CI=1.15-6.04) and cAVSD (p=0.005, HR=3.7; 95% CI=1.39-7.51) were independent risk factors for early post-operative SVTs and significant more late post-operative SVTs occurred in non-syndromic patients (p=0.016, HR=6.38; 95% CI=1.42-28.71) and in pAVSD (p=0.045, HR=3.703; 95% CI=1.03-13.32). Fifteen patients (3.6%) received a pacemaker. Non-syndromic AVSD (p=0.008, HR=15.82; 95% CI=2.04-122.47), pAVSD (p=0.017, HR=6.26; 95% CI=1.39-28.28) and re-operation (p=0.007, HR=4.911; 95% CI=1.54-15.64) were independent risk factors for postoperative pacemaker implantation. Late life-threatening ventricular arrhythmias and SCD occurred in 0.5% and 1.7% respectively. There is good long-term survival after AVSD correction and incidence of SCD is low. Non-syndromic AVSD and cAVSD are independent risk factors for early post-operative SVTs. Non-syndromic AVSD patients have significant more early 3rd degree AVB and late post-operative SVTs. Non-syndromic patients with partial AVSD who have undergone reoperation have a significant higher risk of pacemaker implantation. Copyright © 2017 Elsevier B.V. All rights reserved.

  3. Facts about Ventricular Septal Defect

    Science.gov (United States)

    ... a healthy weight gain, a special high-calorie formula might be prescribed. Some babies become extremely tired ... file Microsoft PowerPoint file Microsoft Word file Microsoft Excel file Audio/Video file Apple Quicktime file RealPlayer ...

  4. [Informed consent in congenital defect heart surgery].

    Science.gov (United States)

    Oka, Norihiko; Miyaji, Kagami

    2014-09-01

    In pediatric cardiac surgery, informed consent is often obtained from the patient's parents. The parents are commonly under great stress due to being in a situation where they have to make the important decision of whether their child will undergo cardiac surgery. Cardiac surgery does not necessarily determine the patient's prognosis if he or she has other organ anomalies or systemic diseases. In such cases, communication among the cardiac surgeon, pediatrician, and family becomes even more crucial. In pediatric cardiac surgery, it is important to obtain informed consent with adequate explanations, an understanding of the parents' stressful situation, and knowledge of the patient's background. In this article, we introduce two examples of obtaining informed consent for two common pediatric cardiac operations, atrial septal defect closure and ventricular septal defect closure.

  5. Single Ventricle Defects

    Science.gov (United States)

    ... of this information Congenital Heart Defects • Home • About Congenital Heart Defects Introduction Healthy Heart Function Common Types of Heart Defects - Aortic Valve Stenosis (AVS) - Atrial ...

  6. A case with double-chambered right ventricle and left ventricular-right atrial communication (Gerbode defect): potential pitfall for Eisenmenger syndrome.

    Science.gov (United States)

    Gur, Demet Ozkaramanli; Gur, Ozcan; Goksuluk, Huseyin; Oral, Dervis

    2012-09-01

    The ventricular septal defect (VSD) can rarely be associated with other malformations such as double-chambered right ventricle (DCRV) in which hypertrophied muscle bundles divide the right ventricle into two chambers causing progressive obstruction (Mao et al., Asia Pac J Thorac Cardiovasc Surg 5:14-17, 1996). Most VSDs close spontaneously by apposition of the tricuspid leaflets, but the process is rarely disrupted, resulting in communication between left ventricle and right atrium called Gerbode defect [Cho et al., J Cardiovasc Ultrasound 19(3):148-151, 2011]. Hence, the Gerbode defect involves potential misinterpretation of its high-velocity shunt as pulmonary hypertension. Here we present a case with DCRV and Gerbode defect initially misdiagnosed to have Eisenmenger syndrome.

  7. Balloon atrial septostomy under echocardiographic guide: case series

    Directory of Open Access Journals (Sweden)

    SM Meraji

    2012-12-01

    Full Text Available Background: Balloon atrial septostomy is an emergent procedure in pediatric cardiology. Nowadays, most patients in need of the procedure have acceptable outcomes after surgical repair. Thus, it is important to perform this procedure as safe as possible. By performing early arterial switch operation and prostaglandin infusion, the rate of balloon atrial septostomy has markedly decreased. However, not all centers performing early arterial switch repairs have abandoned atrial septostomy, even in patients who respond favorably to prostaglandin infusion.Case presentation: In total, eight 1- to 15-day old term neonates admitted in Shahid Rajaee Heart Center in Tehran, Iran from October 2009 to February 2011, with congenital heart diseases were scheduled for balloon atrial septostomy. In six cases the procedure was done exclusively under echocardiographic guidance and in two cases with the help of fluoroscopy. Success was defined as the creation of an atrial septal defect with a diameter equal to or more than 5 mm and ample mobility of its margins.Results: Male sex was predominant (87% and the mean age of the neonates was six days. The diagnosis in all cases was simple transposition of great arteries. The procedure was successful in all patients with any cardiovascular complication.Conclusion: Balloon atrial septostomy is an emergent procedure that can be done safely and effectively under echocardiographic guidance. According to the feasibility of this technique it could be performed fast, safe and effective at bedside, avoiding patient transportation to hemodynamic laboratory or referral center.

  8. Case report: airway and concurrent hemodynamic management in a neonate with oculo-auriculo-vertebral (Goldenhar) syndrome, severe cervical scoliosis, interrupted aortic arch, multiple ventricular septal defects, and an unstable cervical spine.

    Science.gov (United States)

    Char, Danton S; Gipp, Melanie; Boltz, M Gail; Williams, Glyn D

    2012-09-01

    We report the challenging case of a 1-week-old, term, 2.4 kg neonate with Goldenhar syndrome (including microcephaly, left microtia, left facial palsy, dextro-scoliosis of the cervical spine, and cervico-thoracic levoscoliosis), multiple ventricular septal defects, a type B interrupted aortic arch, a large patent ductus arteriosis, and radiographic and clinical signs concerning for an unstable cervical spine. Our anesthesia team was consulted for perioperative management of this patient during her surgical repair. This case report describes the use of the Air-Q size 1 laryngeal airway (LA) to assist fiberoptic intubation in an ASA 4 neonate with cardiac disease, an anticipated difficult airway with the addition of an unstable cervical spine, as well as the anesthetic techniques used to maintain hemodynamic stability while the airway was secured. © 2012 Blackwell Publishing Ltd.

  9. Lower rate of selected congenital heart defects with better maternal diet quality: a population-based study.

    Science.gov (United States)

    Botto, Lorenzo D; Krikov, Sergey; Carmichael, Suzan L; Munger, Ronald G; Shaw, Gary M; Feldkamp, Marcia L

    2016-01-01

    To evaluate whether better diet quality in mothers is associated with lower risk for major non-syndromic congenital heart defects in their children. Multicentre population-based case-control study, the National Birth Defects Prevention Study. Ten sites in the USA. Mothers of babies with major non-syndromic congenital heart defects (n=9885) and mothers with unaffected babies (n=9468) with estimated date of delivery from 1997 to 2009. Adjusted ORs for specific major congenital heart defects by quartiles of maternal diet quality in the year before pregnancy, assessed by the Diet Quality Index for pregnancy (DQI-P) and the Mediterranean Diet Score. Quartile 1 (Q1) reflecting the worst diet quality and Q4 the best diet quality. Better diet quality was associated with reduced risk for some conotruncal and atrial septal heart defects. For DQI-P, estimated risks reductions (Q4 vs Q1) for conotruncal defects were 37% for tetralogy of Fallot (OR 0.63, 95% CI 0.49 to 0.80) and 24% overall (OR 0.76, 95% CI 0.64 to 0.91); and for septal defects, 23% for atrial septal defects (OR 0.77, 95% CI 0.63 to 0.94) and 14% overall (OR 0.86, 95% CI 0.75 to 1.00). Risk reductions were weaker or minimal for most other major congenital heart defects. Better diet quality is associated with a reduced occurrence of some conotruncal and septal heart defects. This finding suggests that a reduction in certain cardiac malformations may be an additional benefit of improved maternal diet quality, reinforcing current preconception care recommendations. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  10. Prevalence of congenital heart defects associated with Down syndrome in Korea.

    Science.gov (United States)

    Kim, Min-A; Lee, You Sun; Yee, Nan Hee; Choi, Jeong Soo; Choi, Jung Yun; Seo, Kyung

    2014-11-01

    Congenital heart defect (CHD) is common in infants with Down syndrome (DS), which is the principle cause of mortality. However, there is no data available for the frequency and types of CHD in infants with DS in Korea. We investigated the frequency of CHD in infants with DS in Korea. After the survey on birth defects was conducted throughout the country, the prevalence of CHD in DS in 2005-2006 was calculated. This study was conducted based on the medical insurance claims database of the National Health Insurance Corporation. The number of total births in Korea was 888,263 in 2005-2006; of them, 25,975 cases of birth defects were identified. The prevalence of DS was 4.4 per 10,000 total births, accounting for 1.5% of all birth defects. Of the 394 infants with DS, 224 (56.9%) had a CHD. Atrial septal defect was the most common defect accounting for 30.5% of DS followed by ventricular septal defect (19.3%), patent duct arteriosus (17.5%), and atrioventricular septal defect (9.4%). Our study will be helpful to demonstrate the current status of DS and to identify the distribution of CHD in infants with DS in Korea.

  11. The vectorcardiogram as an aid to diagnosis in left ventricular-right atrial communication.

    Science.gov (United States)

    Shakibi, J G; Aryanpur, I; Paydar, M; Yazdanyar, A; Siassi, B

    1977-01-01

    Frank vectorcardiograms (VCGs) in four patients with left ventricular-right atrial (LV-RA) communication were analyzed and compared with published values of VCGs of normal individuals and those from a group of patients with membranous ventricular septal defect. It was observed that the QRS-loops in the frontal and sagittal planes of the patients with LV-RA communication are shifted more superiorly than usual. Thus, almost 50% of the QRS-loop area was superior to the X- and Z-coordinates in the frontal and sagittal planes, whereas in the controls less than 10% of the frontal and sagittal plane QRS-loops were superior to the horizontal axes. The QRS-loops of patients with LV-RA communication thus seem to be halfway between normal and endocardial cushion defect loops. An unusual degree of superior orientation of the QRS-loop in a patient with clinical findings of a ventricular septal defect should arouse suspicion of a LV-RA communication. Also in patients with an isolated ventricular septal defect but with an exaggerated superior orientation of the QRS-loop in the frontal and sagittal planes, the interatrial septum should be examined at the time of operation to exclude the possibility of an associated LV-RA communication.

  12. How Slow Can We Go? 4 Frames Per Second (fps) Versus 7.5 fps Fluoroscopy for Atrial Septal Defects (ASDs) Device Closure.

    Science.gov (United States)

    Hiremath, Gurumurthy; Meadows, Jeffery; Moore, Phillip

    2015-06-01

    Radiation exposure remains a significant concern for ASD device closure. In an effort to reduce radiation exposure, the default fluoroscopy frame rate in our Siemens biplane pediatric catheterization laboratory was reduced to 4 fps in November 2013 from an earlier 7.5 fps fluoro rate. This study aims to evaluate the components contributing to total radiation exposure and compare the procedural success and radiation exposure during ASD device closure using 4 versus 7.5 fps fluoroscopy rates. Twenty ASD device closures performed using 4 fps fluoro rate were weight-matched to 20 ASD closure procedures using 7.5 fps fluoro rate. Baseline characteristics, procedure times and case times were similar in the two groups. Device closure was successful in all but one case in the 4 fps group. The dose area product (DAP), normalized DAP to body weight, total radiation time and fluoro time were lower in the 4 fps group but not statistically different than the 7.5 fps. The number of cine images and cine times were identical in both groups. Fluoroscopy and cineangiography contributed equally to radiation exposure. Fluoroscopy at 4 fps can be safe and effective for ASD device closure in children and adults. There was no increase in procedure time, cine time, fluoro time or complications at this slow fluoro rate. There was a trend toward decreased radiation exposure as measured by indexed DAP although not statistically significant in this small study. Further study with multiple operators using 4 fps fluoroscopy for simple interventional procedures is recommended.

  13. Variations in height of jugular "a" wave in relation to heart rate in normal subjects and in patients with atrial septal defect.

    OpenAIRE

    Thiron, J M; Cribier, A.; Cazor, J L; Letac, B

    1980-01-01

    Analysis of jugular tracings in seven normal subjects in sinus rhythm whose heart rate varied spontaneously from one moment to another during expiratory apnoea, showed that a pronounced variation occurred in the size of the "a" waves in relation to that of the "v" waves and in accordance with the corresponding RR interval. In the 53 measurements which were carried out, the "a/v" ratio had a mean value of 0.9 for a heart rate above 87, 1.4 for a heart rate between 87 and 68, and 1.1 for a hear...

  14. Detection of inter-atrial conduction defects with unfiltered signal-averaged P-wave ECG in patients with lone atrial fibrillation.

    Science.gov (United States)

    Platonov, P G; Carlson, J; Ingemansson, M P; Roijer, A; Hansson, A; Chireikin, L V; Olsson, S B

    2000-01-01

    To demonstrate a possible inter-atrial conduction delay in patients with lone paroxysmal atrial fibrillation (PAF) using 'unfiltered' signal-averaged P-wave ECG (PSAECG) and compare these results with those obtained with conventional filter settings. Twenty one patients with lone PAF and 20 healthy volunteers (control group) were enrolled in the study. An orthogonal lead surface ECG was high-pass filtered at 0.8 Hz, averaged with template matching, and combined into a spatial magnitude ('unfiltered' technique). Results were compared with conventionally filtered (40-300 Hz) PSAECG. The filtered technique revealed no differences in P-wave duration between the two groups (121 +/- 12 vs 128 +/- 15 ms, control and PAF groups respectively, ns). Double-peaked P-wave spatial magnitudes (interpeak distance >30 ms) were revealed in 11 of 21 PAF patients but only in two of 18 controls (PUnfiltered' PSAECG revealed significant differences in orthogonal P-wave morphology in patients with lone PAF, indicating the possibility of an inter-atrial conduction delay, while conventional P-wave duration analysis failed to discriminate between the two groups.

  15. Atrial Ectopics Precipitating Atrial Fibrillation

    Directory of Open Access Journals (Sweden)

    Johnson Francis

    2015-04-01

    Full Text Available Holter monitor tracing showing blocked atrial ectopics and atrial ectopic precipitating atrial fibrillation is being demonstrated. Initially it was coarse atrial fibrillation, which rapidly degenerated into fine atrial fibrillation.

  16. Significant survival advantage of high pulmonary vein index and the presence of native pulmonary artery in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: results from preoperative computed tomography angiography.

    Science.gov (United States)

    Jia, Qianjun; Cen, Jianzheng; Zhuang, Jian; Zhong, Xiaomei; Liu, Xiaoqing; Li, Jiahua; Liang, Changhong; Huang, Meiping

    2017-08-01

    The prognosis of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA-VSD-MAPCAs) after surgery shows substantial clinical heterogeneity and predictors for outcomes are lacking. This study aimed to assess the predictive value of preoperative cardiac computed tomography angiography (CTA) for survival in patients with PA-VSD-MAPCAs. We retrospectively analysed PA-VSD-MAPCA patients with preoperative CTA who underwent both right ventricular outflow tract reconstruction and MAPCA unifocalization ( n  = 24) or pulmonary artery rehabilitation ( n  = 28). The end-point was overall survival. Prognostic values of CTA were assessed using Cox univariate and multivariate analyses. The significant threshold of independent parameters was calculated using receiver-operating characteristic (ROC) curves. During a median follow-up of 1145 days, a total of 13 deaths were observed. Multivariate analysis identified a high pulmonary vein index (PVI) [hazard ratio (HR) = 0.03; 95% confidence interval (CI): 0.03, 0.28; P  advantage in PA-VSD-MAPCA patients. A PVI ≥438 mm 2 /m 2 may be a reliable positive prognosticator that could improve the decision-making strategy for PA-VSD-MAPCA patients.

  17. Types and distribution of congenital heart defects associated with trisomy 21 in Singapore.

    Science.gov (United States)

    Tan, Monica; Xu, Cunzhi; Sim, Sarah K R; Seow, Adeline L H; Tan, Teng Hong; Quek, Swee Chye

    2013-03-01

    Atrioventricular septal defect (AVSD) is widely accepted as the most common type of congenital heart defect in trisomy 21. Most of these studies, however, were conducted in Caucasian communities. The few Asian studies that had been conducted on this subject yielded different results. In the largest study of its kind in Asia, we described the distribution of types of congenital heart defects associated with trisomy 21 in Singapore. Five hundred and eighty-eight patients with trisomy 21 born in 1996-2010, and confirmed by karyotyping, were included in the study. The diagnosis of congenital heart defects were made on echocardiography. Variables extracted for analysis were demographics (race and gender) and the types of congenital heart defects. Except for complex cyanotic heart defects, haemodynamically significant lesions were accounted for separately in cases where more than one type of congenital heart defect coexisted in a patient. Ventricular septal defect (VSD) (39.2%) was the most common congenital heart defect associated with trisomy 21 in our study, followed by patent ductus arteriosus (34.3%), secundum atrial septal defect (23.4%) and AVSD (15.6%). This study validates previous smaller Asian studies identifying VSD as the most common cardiac lesion associated with trisomy 21. A high proportion (25.0%) of trisomy 21 patients with tetralogy of Fallot also had AVSDs. Coarctation of the aorta was uncommon. VSD was the most common congenital heart defect seen in trisomy 21 in our study. A high proportion (25.0%) of trisomy 21 patients with tetralogy of Fallot also had AVSDs. © 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  18. [An adult case of left ventricular-right atrial communication with a false aneurysm of membranous septum].

    Science.gov (United States)

    Ashida, Y; Okada, M; Taniguchi, I; Yamaga, T

    1993-12-01

    A case of left ventricular-right atrial (LV-RA) communication associated with a false aneurysm of membranous septum is presented. The patient was a 51-year-old woman. Using a left ventriculography this case was diagnosed preoperatively as LV-RA communication. We found that the right atrium was directly communicated with the left ventricle through a fibrous membranous aneurysm adhering to the septal leaflet of the tricuspid valve. It was suggested that a false aneurysm, made by jet lesion from the left ventricle, perforated to the right atrium. So the current case was considered an acquired infravaluvular type LV-RA communication formed in the course of the natural closure of the membranous ventricular septal defect.

  19. Endocardial cushion defect

    Science.gov (United States)

    ... Philadelphia, PA: Elsevier; 2016:chap 426. Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Atrioventricular septal defect. In: Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK, eds. Kirklin/Barratt- ...

  20. Air Pollution and the Risk of Cardiac Defects: A Population-Based Case-Control Study.

    Science.gov (United States)

    Hwang, Bing-Fang; Lee, Yungling Leo; Jaakkola, Jouni J K

    2015-11-01

    Previous epidemiologic studies have assessed the role of the exposure to ambient air pollution in the development of cardiac birth defects, but they have provided somewhat inconsistent results. To assess the associations between exposure to ambient air pollutants and the risk of cardiac defects, a population-based case-control study was conducted using 1087 cases of cardiac defects and a random sample of 10,870 controls from 1,533,748 Taiwanese newborns in 2001 to 2007.Logistic regression was performed to calculate odds ratios for 10 ppb increases in O3 and 10 μg/m increases in PM10. In addition, we compared the risk of cardiac defects in 4 categories-high exposure (>75th percentile); medium exposure (75th to 50th percentile); low exposure (percentile); reference (percentile) based on the distribution of each pollutant. The risks of ventricular septal defects (VSD), atrial septal defects (ASD), and patent ductus arteriosus (PDA) were associated with 10 ppb increases in O3 exposure during the first 3 gestational months among term and preterm babies. In comparison between high PM10 exposure and reference category, there were statistically significant elevations in the effect estimates of ASD for all and terms births. In addition, there was a negative or weak association between SO2, NO2, CO, and cardiac defects.The study proved that exposure to outdoor air O3 and PM10 during the first trimester of gestation may increase the risk of VSD, ASD, and PDA.

  1. Percutaneous closure of congenital Gerbode defect using Nit-Occlud(®) Lê VSD coil.

    Science.gov (United States)

    Phan, Quang T; Kim, Sang-Wook; Nguyen, Hieu L

    2017-07-26

    We present a case report about percutaneous closure of a congenital Gerbode defect using Nit-Occlud(®) Lê VSD coil. The patient was referred to our hospital with a diagnosis of ventricular septal defect (VSD) and severe pulmonary arterial hypertension. But transthoracic echocardiography revealed a communication between the left ventricle (LV) and the right atrial (RA), called Gerbode defect. Catheterization confirmed the shunt from the LV to the RA. We successfully closed the defect with a VSD coil. After uneventful 6 mo follow-up, the patient was out of dyspnea, the symptom urged him to have medical attention. This case report is to discuss the diagnosis and percutaneous treatment approach for this rare congenital heart disease.

  2. Congenital heart defects in molecularly proven Kabuki syndrome patients.

    Science.gov (United States)

    Digilio, Maria Cristina; Gnazzo, Maria; Lepri, Francesca; Dentici, Maria Lisa; Pisaneschi, Elisa; Baban, Anwar; Passarelli, Chiara; Capolino, Rossella; Angioni, Adriano; Novelli, Antonio; Marino, Bruno; Dallapiccola, Bruno

    2017-11-01

    The prevalence of congenital heart defects (CHD) in Kabuki syndrome ranges from 28% to 80%. Between January 2012 and December 2015, 28 patients had a molecularly proven diagnosis of Kabuki syndrome. Pathogenic variants in KMT2D (MLL2) were detected in 27 patients, and in KDM6A gene in one. CHD was diagnosed in 19/27 (70%) patients with KMT2D (MLL2) variant, while the single patient with KDM6A change had a normal heart. The anatomic types among patients with CHD included aortic coarctation (4/19 = 21%) alone or associated with an additional CHD, bicuspid aortic valve (4/19 = 21%) alone or associated with an additional CHD, perimembranous subaortic ventricular septal defect (3/19 = 16%), atrial septal defect ostium secundum type (3/19 = 16%), conotruncal heart defects (3/19 = 16%). Additional CHDs diagnosed in single patients included aortic dilatation with mitral anomaly and hypoplastic left heart syndrome. We also reviewed CHDs in patients with a molecular diagnosis of Kabuki syndrome reported in the literature. In conclusion, a CHD is detected in 70% of patients with KMT2D (MLL2) pathogenic variants, most commonly left-sided obstructive lesions, including multiple left-sided obstructions similar to those observed in the spectrum of the Shone complex, and septal defects. Clinical management of Kabuki syndrome should include echocardiogram at the time of diagnosis, with particular attention to left-sided obstructive lesions and mitral anomalies, and annual monitoring for aortic arch dilatation. © 2017 Wiley Periodicals, Inc.

  3. Chromosome 5q33 deletions associated with congenital heart defects.

    Science.gov (United States)

    Starkovich, Molly; Lalani, Seema R; Mercer, Catherine L; Scott, Daryl A

    2016-12-01

    Congenital heart defects (CHD) are present in over 1% of all newborns and are the leading cause of birth-defect-related deaths in the United States. We describe two male subjects with CHD, one with an atrial septal defect, a ventricular septal defect, and pulmonary artery stenosis; and the other with tetralogy of Fallot and a right aortic arch, who carry partially overlapping, de novo deletions of chromosome 5q33. The maximum region of overlap between these deletions encompasses HAND1 and SAP30L, two genes that have previously been shown to play a role in cardiac development. HAND1 encodes a basic helix-loop-helix transcription factor. Cardiac-specific ablation of Hand1 in mice causes septal, valvular, and outflow tract defects. SAP30L, its paralog SAP30, and other SAP proteins form part of a multi-subunit complex involved in transcriptional regulation via histone deacetylation. Morpholino knockdown of sap30L in zebrafish, which do not have a distinct sap30 gene, leads to cardiac hypoplasia and cardiac insufficiency. We subsequently identified two other individuals with chromosomal deletions involving HAND1 and SAP30L in whom cardiac-related medical problems were not described. These observations suggest that haploinsufficiency of HAND1 and/or SAP30L may contribute to the development of CHD, although the contribution of other genes on chromosome 5q33 cannot be excluded. Our findings also suggest that the penetrance of CHD associated with 5q33 deletions is incomplete and may be influenced by other genetic, environmental or stochastic factors. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  4. Simplified progressive approach for the ablation of scar related atrial macroreentrant tachycardias.

    Science.gov (United States)

    Nava, Santiago; Iturralde-Torres, Pedro; Márquez, Manlio F; Gómez-Flores, Jorge; Cline, Bernardo; Colin-Lizalde, Luis; Victoria, Diana; Hermosillo, Antonio G

    2013-01-01

    Radiofrequency ablation of scar related right atrial flutter is challenging. Long procedures, prolonged fluoroscopic times and high percentages of recurrences are of concern. We present a simple and progressive approach based on a single electroanatomic map of the right atrium. Twenty-two consecutive patients with atrial flutter and history of cardiac surgery were included. An electrophysiologic study was performed to define localization (left or right) and cavo-tricuspid isthmus participation using entrainment mapping. After a critical isthmus was localized, ablation was performed with an external irrigated tip catheter with a power limit of 30 W. Potential ablation sites were confirmed by entrainment. The predominant cardiopathy was atrial septal defect. All arrhythmias were localized in the right atrium; mean cycle length of the clinical flutter was 274 ± 31 ms. Only 40% had cavo-tricuspid isthmus participation. None of the patients with successful ablation had recurrences after 13 ± 9.4 months of follow-up. A progressive approach with only one activation/voltage CARTO(®) map of the atrium and ablation of all potential circuits is a highly effective method for ablating scar related macroreentrant atrial arrhythmias. Copyright © 2012 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

  5. Association of certain chronic maternal diseases with the risk of specific congenital heart defects: a population-based study.

    Science.gov (United States)

    Vereczkey, A; Gerencsér, B; Czeizel, A E; Szabó, I

    2014-11-01

    Previous epidemiological studies have evaluated cases with all congenital heart defects (CHDs), rather than analysing different types of CHD. The objective of this study was to evaluate the possible association of certain chronic maternal diseases with the risk of different types of CHD, because the role of possible environmental factors in the origin of CHDs is unclear in the vast majority of patients. Different types of CHD, diagnosed after lethal outcome (autopsy report) or after surgical intervention (catheter or correction), were evaluated in order to estimate the possible role of chronic maternal diseases in their origin. This analysis was based on the rates of medically recorded chronic maternal diseases in 3562 live-born cases with CHDs, 38,151 population controls without any birth defects, and 16,602 malformed controls with other isolated congenital abnormalities, using the data set of the population-based Hungarian Case-Control Surveillance of Congenital Abnormalities (1980-1996). Maternal epilepsy treated with carbamazepine and migraine were found to be associated with higher risk of ventricular septal defect; panic disorders were associated with higher risk of hypoplastic left heart; type I diabetes mellitus was associated with higher risk of coarctation of the aorta; chronic hypertension was associated with higher risk of ventricular septal defect, common atrioventricular canal and common truncus; and paroxysmal supraventricular tachycardia was associated with higher risk of atrial septal defect secundum, common atrioventricular canal and ventricular septal defect. In conclusion, certain chronic maternal diseases were found to be associated with higher risk of specific CHDs. Appropriate treatment of these diseases may help to prevent these CHDs. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  6. Dietary Glycemic Index and the Risk of Birth Defects

    Science.gov (United States)

    Parker, Samantha E.; Werler, Martha M.; Shaw, Gary M.; Anderka, Marlene; Yazdy, Mahsa M.

    2012-01-01

    Prepregnancy diabetes and obesity have been identified as independent risk factors for several birth defects, providing support for a mechanism that involves hyperglycemia and hyperinsulinemia in the development of malformations. Data from the National Birth Defects Prevention Study from 1997 to 2007 were used to investigate the association between the maternal dietary glycemic index (DGI) and the risk of birth defects among nondiabetic women. DGI was categorized by using spline regression models and quartile distributions. Adjusted odds ratios and 95% confidence intervals were calculated. The joint effect of DGI and obesity was also examined. Among the 53 birth defects analyzed, high DGI, categorized by spline regression, was significantly associated with encephalocele (adjusted odds ratio (aOR) = 2.68), diaphragmatic hernia (aOR = 2.58), small intestinal atresia/stenosis (aOR = 2.97) including duodenal atresia/stenosis (aOR = 2.48), and atrial septal defect (aOR = 1.37). Using quartiles to categorize DGI, the authors identified associations with cleft lip with cleft palate (aOR = 1.23) and anorectal atresia/stenosis (aOR = 1.40). The joint effect of high DGI and obesity provided evidence of a synergistic effect on the risk of selected birth defects. High DGI is associated with an increased risk of a number of birth defects under study. Obesity coupled with high DGI appears to increase the risk further for some birth defects. PMID:23171874

  7. [Winging effect in interatrial defect after transseptal mitral valvuloplasty: an anatomical study].

    Science.gov (United States)

    Martuscelli, E; Avella, A; Berni, A; Melis, M; Soda, G; Ferraironi, A; Nigri, A; Reale, A

    1990-04-01

    In the balloon catheters the redundancy of the deflated balloon produces 2 or 3 sort of thin wings. The presence of wings can reduce the catheter "pushability" or, in certain conditions, can determine tissue lesion (winging effect). Simulating a transeptal valvuloplasty of the mitral valve by technique of the 2 balloon in human fresh heart, we studied the winging effect over the interatrial septum in 12 hearts; in the first 6 the atrial septum was dilated with a 6 mm balloon (Group A), in the second 6 the atrial septum was dilated with a 10 mm balloon (Group B). The procedure was completed introducing consecutively 2 bigger balloons (15 + 20 mm). We suppose that the winging effect of the 2 bigger balloons (15 + 20 mm) could counter balance the theoretical advantage of a small balloon (6 mm). The atrial septal defect (ASD) after septal dilatation was 5.0 +/- 0.59 mm x 1.56 +/- 0.25 mm (long axis x short axis) in Group A and 6.53 +/- 0.35 x 2.16 +/- 0.39 mm in Group B (p less than 0.01). The final ASD (after introducing the 2 bigger balloons) was 7.04 +/- 1.06 x 2.36 +/- 0.57 mm in Group A and 7.03 +/- 0.18 x 2.16 +/- 0.32 mm in Group B (NS). Our data show that the winging effect can determine biological negative effects.

  8. Correção simultânea de defeito congênito intracardíaco e pectus excavatum Simultaneous repair of congenital heart defect and pectus excavatum

    Directory of Open Access Journals (Sweden)

    João Roberto Breda

    2007-09-01

    Full Text Available Relatamos tratamento simultâneo de pectus excavatum e defeito congênito intracardíaco representado por comunicação interatrial ostium secundum. Paciente do sexo masculino, 8 anos de idade, com diagnóstico clínico e ecocardiográfico de comunicação interatrial, associada à deformidade da parede torácica tipo pectus excavatum. Foi encaminhado para operação com correção simultânea do defeito congênito intracardíaco associado ao reparo do pectus. O tratamento operatório simultâneo do pectus excavatum e defeitos congênitos intracardíacos torna difícil o acesso ao coração. Foi feita a correção simultânea dessas alterações, com satisfatório resultado, sobretudo estético, para o paciente.The author describes the simultaneous treatment of pectus excavatum and congenital intracardiac defect (atrial septal defect represented by the interatrial foramen secundum. An 8-year-old boy, with clinical and echocardiography diagnosis of atrial septal defect associated with pectus excavatum was referred to a simultaneous surgical treatment of both abnormalities. The simultaneous surgical treatment of both pectus excavatum and congenital intracardiac defects make it difficult to access the heart. In this case, the simultaneous surgical treatment of atrial septal defect and pectus excavatum was a valuable alternative to surgical repair of both abnormalities, mainly due to its cosmetic outcome.

  9. Septal myocardial perfusion imaging with thallium-201 in the diagnosis of proximal left anterior descending coronary artery disease

    Energy Technology Data Exchange (ETDEWEB)

    Pichard, A.D.; Wiener, I.; Martinez, E.; Horowitz, S.; Patterson, R.; Meller, J.; Goldsmith, S.J.; Gorlin, R.; Herman, M.V.

    1981-07-01

    The use of myocardial perfusion imaging (MPI) to identify obstructive coronary disease of the left anterior descending coronary artery proximal to the first septal perforator (prox LAD) was studied in 60 patients. Perfusion of the septum and anteroapical areas with thallium-201 injected during exercise was compared to results of coronary arteriography. Septal MPI defect was found in 92.3% of patients with obstruction of the proximal LAD, 27.7% of patients with obstruction of LAD distal to first septal perforator, 0% in patients with obstructions involving right or circumflex arteries, and in 10.5% of patients without coronary disease. Anteroapical MPI defects were found with similar frequency in the three groups with obstructive coronary disease. Septal MPI defect had a sensitivity of 92.3% and specificity of 85.4% in the diagnosis of proximal LAD disease. Normal septal perfusion with thallium-201 virtually excluded proximal LAD disease.

  10. Maternal smoking during pregnancy and the risk of congenital heart defects in offspring: a systematic review and metaanalysis.

    Science.gov (United States)

    Lee, Laura J; Lupo, Philip J

    2013-02-01

    Although a previous metaanalysis indicated that maternal smoking during pregnancy increased the risk of congenital heart defects (CHD) in offspring, the effect of smoking on individual CHD subtypes was not determined. Because CHDs are anatomically, clinically, epidemiologically, and developmentally heterogeneous, the authors conducted a systematic review and metaanalysis of the association between maternal smoking during pregnancy and the risk of CHDs, including CHD subtypes among offspring. Two types of summary relative risk (RR) estimates (any smoking vs no smoking and increasing categories of smoking, i.e., light, medium, and heavy) were calculated for CHDs as a group and for a number of CHD subtypes using both fixed- and random-effects models. Random effects estimates were reported if there was evidence of heterogeneity among the studies. Consistent with the previous metaanalysis, the authors observed a positive association between maternal smoking during pregnancy and the risk of CHDs as a group (RR, 1.11; 95 % confidence interval [CI], 1.02-1.21; number of cases [n] = 18,282). Additionally, women who smoked during pregnancy were more likely to have a child with 12 (71 %) of 17 CHD subtypes analyzed compared with women who did not smoke. The highest risk was for septal defects as a group (RR, 1.44; 95 % CI, 1.16-1.79; n = 2977). The evidence of dose response was observed for septal defects as a group, atrial septal defects, and atrioventricular septal defects. This systematic review and metaanalysis suggests that maternal smoking is modestly associated with an increased risk of CHDs and some CHD subtypes.

  11. Multifocal Atrial Tachycardia in 2 Children

    Directory of Open Access Journals (Sweden)

    Ming-Yun Hsieh

    2006-09-01

    Full Text Available The incidence of multifocal atrial tachycardia (MAT is very low and accounts for less than 1% of supraventricular tachycardia in infants and children. In this report, the clinical characteristics, medical treatment and outcome of MAT in 2 children are described. The first patient presented with tachycardia and respiratory failure since the day after birth. First, he received amiodarone, propranolol, and digoxin and then amiodarone alone. The heart rhythm converted to sinus rhythm 2 weeks after hospitalization. Although nonsustained MAT was occasionally observed when the infant suffered from pulmonary infection, the frequency of recurrent MAT decreased as the infant grew up. The second patient was a 5-year-old girl. She had congenital heart disease with double outlets of right ventricle (DORV, patent ductus arte-riosus, coarctation of aorta, and ventricular and atrial septal defects. She underwent total correction at the age of 4 years. MAT was noted 3 months after the operation with the presentation of congestive heart failure. The heart rate slowed down and returned to normal sinus rhythm within several hours after amiodarone use. The symptoms and signs of congestive heart failure also disappeared. The patient took amiodarone regularly, and no tachycardia was detected during the follow-up period. MAT is considered to be a relatively benign arrhythmia with likely good outcome if there is no severe underlying illness. It can be well controlled under appropriate drugs, and a long period of follow-up is suggested. If pharmacologic intervention is required, we suggest that amiodarone may be an excellent choice.

  12. Ablation of Atrial Fibrillation in Patients with Congenital Heart Disease.

    Science.gov (United States)

    Refaat, Marwan M; Ballout, Jad; Mansour, Moussa

    2017-12-01

    With improved surgical techniques and medical management for patients with congenital heart diseases, more patients are living longer and well into adulthood. This improved survival comes with a price of increased morbidity, mainly secondary to increased risk of tachyarrhythmias. One of the major arrhythmias commonly encountered in this subset of cardiac patients is AF. Similar to the general population, the risk of AF increases with advancing age, and is mainly secondary to the abnormal anatomy, abnormal pressure and volume parameters in the hearts of these patients and to the increased scarring and inflammation seen in the left atrium following multiple surgical procedures. Catheter ablation for AF has been shown to be a very effective treatment modality in patients with refractory AF. However, data and guidelines regarding catheter ablation in patients with congenital heart disease are not well established. This review will shed light on the procedural techniques, success rates and complications of AF catheter ablation in patients with different types of CHD, including atrial septal defects, tetralogy of Fallot, persistent left superior vena cava, heterotaxy syndrome and atrial isomerism, and Ebstein anomaly.

  13. Fluconazole use and birth defects in the National Birth Defects Prevention Study.

    Science.gov (United States)

    Howley, Meredith M; Carter, Tonia C; Browne, Marilyn L; Romitti, Paul A; Cunniff, Christopher M; Druschel, Charlotte M

    2016-05-01

    Low-dose fluconazole is used commonly to treat vulvovaginal candidiasis, a condition occurring frequently during pregnancy. Conflicting information exists on the association between low-dose fluconazole use among pregnant women and the risk of major birth defects. We used data from the National Birth Defects Prevention Study to examine this association. The National Birth Defects Prevention Study is a multisite, population-based, case-control study that includes pregnancies with estimated delivery dates from 1997 to 2011. Information on fluconazole use in early pregnancy was collected by self-report from 31,645 mothers of birth defect cases and 11,612 mothers of unaffected controls. Adjusted odds ratios and 95% confidence intervals were estimated for birth defects with 5 or more exposed cases; crude odds ratios and exact 95% confidence intervals were estimated for birth defects with 3-4 exposed cases. Of the 43,257 mothers analyzed, 44 case mothers and 6 control mothers reported using fluconazole. Six exposed infants had cleft lip with cleft palate, 4 had an atrial septal defect, and each of the following defects had 3 exposed cases: hypospadias, tetralogy of Fallot, d-transposition of the great arteries, and pulmonary valve stenosis. Fluconazole use was associated with cleft lip with cleft palate (odds ratio = 5.53; confidence interval = 1.68-18.24) and d-transposition of the great arteries (odds ratio = 7.56; confidence interval = 1.22-35.45). The associations between fluconazole and both cleft lip with cleft palate and d-transposition of the great arteries are consistent with earlier published case reports but not recent epidemiologic studies. Despite the larger sample size of the National Birth Defects Prevention Study, fluconazole use was rare. Further investigation is needed in large studies, with particular emphasis on oral clefts and conotruncal heart defects. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Maternal autoimmune disease and birth defects in the National Birth Defects Prevention Study.

    Science.gov (United States)

    Howley, Meredith M; Browne, Marilyn L; Van Zutphen, Alissa R; Richardson, Sandra D; Blossom, Sarah J; Broussard, Cheryl S; Carmichael, Suzan L; Druschel, Charlotte M

    2016-11-01

    Little is known about the association between maternal autoimmune disease or its treatment and the risk of birth defects. We examined these associations using data from the National Birth Defects Prevention Study, a multi-site, population-based, case-control study. Analyses included 25,116 case and 9897 unaffected control infants with estimated delivery dates between 1997 and 2009. Information on autoimmune disease, medication use, and other pregnancy exposures was collected by means of telephone interview. Adjusted odds ratios (ORs) and 95% confidence intervals (CIs) were estimated for birth defects with five or more exposed cases; crude ORs and exact 95% CIs were estimated for birth defects with three to four exposed cases. Autoimmune disease was reported by 373 mothers (279 case and 94 control mothers). The majority of birth defects evaluated were not associated with autoimmune disease; however, a statistically significant association between maternal autoimmune disease and encephalocele was observed (OR, 4.64; 95% CI, 1.95-11.04). Eighty-two mothers with autoimmune disease used an immune modifying/suppressing medication during pregnancy; this was associated with encephalocele (OR, 7.26; 95% CI, 1.37-24.61) and atrial septal defects (OR, 3.01; 95% CI, 1.16-7.80). Our findings suggest maternal autoimmune disease and treatment are not associated with the majority of birth defects, but may be associated with some defects, particularly encephalocele. Given the low prevalence of individual autoimmune diseases and the rare use of specific medications, we were unable to examine associations of specific autoimmune diseases and medications with birth defects. Other studies are needed to confirm these findings. Birth Defects Research (Part A) 106:950-962, 2016. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  15. Syndromes and Disorders Associated with Omphalocele (III: Single Gene Disorders, Neural Tube Defects, Diaphragmatic Defects and Others

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    Chih-Ping Chen

    2007-06-01

    Full Text Available Omphalocele can be associated with single gene disorders, neural tube defects, diaphragmatic defects, fetal valproate syndrome, and syndromes of unknown etiology. This article provides a comprehensive review of omphalocele-related disorders: otopalatodigital syndrome type II; Melnick–Needles syndrome; Rieger syndrome; neural tube defects; Meckel syndrome; Shprintzen–Goldberg omphalocele syndrome; lethal omphalocele-cleft palate syndrome; cerebro-costo-mandibular syndrome; fetal valproate syndrome; Marshall–Smith syndrome; fibrochondrogenesis; hydrolethalus syndrome; Fryns syndrome; omphalocele, diaphragmatic defects, radial anomalies and various internal malformations; diaphragmatic defects, limb deficiencies and ossification defects of skull; Donnai–Barrow syndrome; CHARGE syndrome; Goltz syndrome; Carpenter syndrome; Toriello–Carey syndrome; familial omphalocele; Cornelia de Lange syndrome; C syndrome; Elejalde syndrome; Malpuech syndrome; cervical ribs, Sprengel anomaly, anal atresia and urethral obstruction; hydrocephalus with associated malformations; Kennerknecht syndrome; lymphedema, atrial septal defect and facial changes; and craniosynostosis- mental retardation syndrome of Lin and Gettig. Perinatal identification of omphalocele should alert one to the possibility of omphalocele-related disorders and familial inheritance and prompt a thorough genetic counseling for these disorders.

  16. Safety of pulmonary vein isolation and left atrial complex fractionated atrial electrograms ablation for atrial fibrillation with phased radiofrequency energy and multi-electrode catheters

    NARCIS (Netherlands)

    Mulder, A.A.W.; Balt, J.C.; Wijffels, M.C.; Wever, E.F.; Boersma, L.V.

    2012-01-01

    AIMS: Recently, a multi-electrode catheter system using phased radiofrequency (RF) energy was developed specifically for atrial fibrillation (AF) ablation: the pulmonary vein ablation catheter (PVAC), the multi-array septal catheter (MASC), and the multi-array ablation catheter (MAAC). Initial

  17. Amplatzer duct occluder II for closure of congenital Gerbode defects.

    Science.gov (United States)

    Vijayalakshmi, I B; Natraj Setty, H S; Chitra, Narasimhan; Manjunath, Cholenahally N

    2015-11-15

    Congenital left ventricle to right atrial communications (Gerbode defects) are extremely rare (0.08%) type of ventricular septal defects. They were traditionally closed by surgery in the past. There are few case reports and small series of acquired and congenital Gerbode defects, closed with various types of devices. Aim of our study is to assess the feasibility, efficacy, and complications of transcatheter closure of congenital Gerbode defects with Amplatzer duct occluder II (ADO II). Twelve consecutive cases of Gerbode defects, age ranging from 10 months to 16 years (mean 6.7 years), weight ranging from 6.5 kg to 34 kg (mean 19.3 kg), were diagnosed on transthoracic echocardiography. Transcatheter closure of Gerbode defects was done successfully through retrograde approach with ADO II. No aortic or tricuspid regurgitation or residual shunt occurred in any of the patients. One patient developed transient complete heart block needing temporary pacing. The soft low profile, easily trackable ADO II appears to be ideal for closure of Gerbode defects, as the central cylinder fits in the defect and the soft retention discs on either side, without polyester material, do not impinge on either aortic, mitral, or tricuspid valve. We report the successful transcatheter closure of twelve cases of congenital Gerbode defects with ADO II. Transcatheter closure of congenital Gerbode defects with ADO II is safe, effective, and an attractive alternative to surgical closure. ADO II appears to be tailor made for Gerbode defects, as the success rate is very high and complication rate is very low. © 2015 Wiley Periodicals, Inc.

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

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    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.

  19. Ethnical Variations in the Incidence of Congenital Heart Defects in Gorgan, Northern Iran: A Single-Center Study

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    Bagher Nikyar

    2015-10-01

    Full Text Available Background: Congenital heart disease (CHD is the most common congenital anomaly in newborns. This study was performed to determine the live birth incidence of CHD by ethnicity and sex in Gorgan, Northern Iran.Methods: In this longitudinal, hospital-based study, 18162 live births in Dezyani Hospital in Gorgan, North of Iran, were screened for CHD, from 2007 through 2009. Clinical examination, echocardiography, color Doppler, and cardio catheterization were used as diagnostic tools. Sex, ethnicity, and type of CHD for each case were recorded in a pre-designed questionnaire.Results: The incidence rates of CHD in the native Fars, Sistani, and Turkmen subjects were 5.73 (95%CI: 4.53-7.15,12.27 (95%CI: 8.74-16.73, and 15.93 (95%CI: 10.00-24.02 per 1000 live births, respectively. The Turkmen to native Fars and Sistani to native Fars relative risk for congenital CHD malformations was 2.77 (95%CI: 1.73-4.44; p value < 0.001 and 1.29 (95%CI: 0.77-2.18; p value < 0.323, respectively. While atrial septal defect was the most common lesion in the native Fars subjects (2.14 per 1000 [95%CI: 1.42-3.06] and in the Sistani subjects (2.84 per 1000 [95%CI: 1.29-5.36], in the Turkmen subjects, ventricular septal defect (4.36 per 1000 [95%CI: 1.59-9.43], followed by atrial septal defect, was the most frequent lesion.Conclusion: This study showed that the incidence and pattern of CHD among live births in Gorgan, North of Iran, varied according to ethnicity. The risk of CHD was higher in the Turkmen and Sistani groups than in the Fars population

  20. Electrophysiological Mechanisms of Atrial Flutter

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    Ching-Tai Tai

    2009-02-01

    Full Text Available Atrial flutter (AFL is a common arrhythmia in clinical practice. Several experimental models, such as tricuspid regurgitation model, tricuspid ring model, sterile pericarditis model and atrial crush injury model, have provided important information about reentrant circuit and can test the effects of antiarrhythmic drugs. Human AFL has typical and atypical forms. Typical AFL rotates around the tricuspid annulus and uses the crista terminalis and sometimes sinus venosa as the boundary. The tricuspid isthmus is a slow conduction zone and the target of radiofrequency ablation. Atypical AFL may arise from the right or left atrium. Right AFL includes upper loop reentry, free wall reentry and figure-of-8 reentry. Left AFL includes mitral annular AFL, pulmonary vein-related AFL and left septal AFL. Radiofrequency ablation of the isthmus between the boundaries can eliminate these arrhythmias.

  1. Consanguineous marriage and congenital heart defects: a case-control study in the neonatal period.

    Science.gov (United States)

    Yunis, Khalid; Khalid, Yunis; Mumtaz, Ghina; Ghina, Mumtaz; Bitar, Fadi; Fadi, Bitar; Chamseddine, Fadi; Fadi, Chamseddine; Kassar, May; May, Kassar; Rashkidi, Joseph; Joseph, Rashkidi; Makhoul, Ghaith; Makhoul, Ghaith; Tamim, Hala; Hala, Tamim

    2006-07-15

    The independent effect of consanguinity on the prevalence of congenital heart defects (CHDs), all and specific types, was investigated in newborns admitted to nine hospitals located in Beirut, Lebanon and members of the National Collaborative Perinatal Neonatal Network (NCPNN). Cases were 173 newborns admitted to the Neonatal Intensive Care Units (NICU) of participating hospitals during the 3-year period from January 1, 2000 to December 31, 2002 and diagnosed during their hospital stay as having one or more CHD. Cases with chromosomal abnormalities were excluded. Cases with more than one CHD were assigned one principal malformation. Controls consisted of a random sample of 865 newborns without a CHD admitted to the NICU during the same period. After controlling for confounders, first cousin consanguinity remained significantly associated with an increased risk of CHD: infants born to first cousin marriages had a 1.8 times higher risk of having a CHD diagnosed at birth compared to those born to unrelated parents (95% CI: 1.1-3.1). In particular, first-cousin marriage was a significant risk factor for ventricular septal defect (VSD), atrial septal defect (ASD), hypoplastic left heart (HLH), and single ventricle (SV). No association was found with d-transposition of the great arteries, coarctation, pulmonary atresia (PA), atrioventricular septal defect (AVSD), and tetralogy of Fallot (TOF). The results of this study suggest a familial factor in the multifactorial etiology of CHDs. Additional epidemiologic and family-based genetic studies are needed to understand the complex cause of CHDs. Copyright 2006 Wiley-Liss, Inc.

  2. Atrial fibrillation

    African Journals Online (AJOL)

    ABEOLUGBENGAS

    Objective: Atrial fibrillation is the commonest chronic arrhythmia and the etiology is widely varied. The aim of this study was to determine the etiology, clinical characteristics and treatment offered to adult patients with atrial fibrillation managed in a referral hospital in Port Harcourt, southern Nigeria. Methods:A retrospective ...

  3. Molecular signatures of cardiac defects in Down syndrome lymphoblastoid cell lines suggest altered ciliome and Hedgehog pathways.

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    Clémentine Ripoll

    Full Text Available Forty percent of people with Down syndrome exhibit heart defects, most often an atrioventricular septal defect (AVSD and less frequently a ventricular septal defect (VSD or atrial septal defect (ASD. Lymphoblastoid cell lines (LCLs were established from lymphocytes of individuals with trisomy 21, the chromosomal abnormality causing Down syndrome. Gene expression profiles generated from DNA microarrays of LCLs from individuals without heart defects (CHD(-; n = 22 were compared with those of LCLs from patients with cardiac malformations (CHD(+; n = 21. After quantile normalization, principal component analysis revealed that AVSD carriers could be distinguished from a combined group of ASD or VSD (ASD+VSD carriers. From 9,758 expressed genes, we identified 889 and 1,016 genes differentially expressed between CHD(- and AVSD and CHD(- and ASD+VSD, respectively, with only 119 genes in common. A specific chromosomal enrichment was found in each group of affected genes. Among the differentially expressed genes, more than 65% are expressed in human or mouse fetal heart tissues (GEO dataset. Additional LCLs from new groups of AVSD and ASD+VSD patients were analyzed by quantitative PCR; observed expression ratios were similar to microarray results. Analysis of GO categories revealed enrichment of genes from pathways regulating clathrin-mediated endocytosis in patients with AVSD and of genes involved in semaphorin-plexin-driven cardiogenesis and the formation of cytoplasmic microtubules in patients with ASD-VSD. A pathway-oriented search revealed enrichment in the ciliome for both groups and a specific enrichment in Hedgehog and Jak-stat pathways among ASD+VSD patients. These genes or related pathways are therefore potentially involved in normal cardiogenesis as well as in cardiac malformations observed in individuals with trisomy 21.

  4. Molecular Signatures of Cardiac Defects in Down Syndrome Lymphoblastoid Cell Lines Suggest Altered Ciliome and Hedgehog Pathways

    Science.gov (United States)

    Ripoll, Clémentine; Rivals, Isabelle; Ait Yahya-Graison, Emilie; Dauphinot, Luce; Paly, Evelyne; Mircher, Clothilde; Ravel, Aimé; Grattau, Yann; Bléhaut, Henri; Mégarbane, André; Dembour, Guy; de Fréminville, Bénédicte; Touraine, Renaud; Créau, Nicole; Potier, Marie Claude; Delabar, Jean Maurice

    2012-01-01

    Forty percent of people with Down syndrome exhibit heart defects, most often an atrioventricular septal defect (AVSD) and less frequently a ventricular septal defect (VSD) or atrial septal defect (ASD). Lymphoblastoid cell lines (LCLs) were established from lymphocytes of individuals with trisomy 21, the chromosomal abnormality causing Down syndrome. Gene expression profiles generated from DNA microarrays of LCLs from individuals without heart defects (CHD−; n = 22) were compared with those of LCLs from patients with cardiac malformations (CHD+; n = 21). After quantile normalization, principal component analysis revealed that AVSD carriers could be distinguished from a combined group of ASD or VSD (ASD+VSD) carriers. From 9,758 expressed genes, we identified 889 and 1,016 genes differentially expressed between CHD− and AVSD and CHD− and ASD+VSD, respectively, with only 119 genes in common. A specific chromosomal enrichment was found in each group of affected genes. Among the differentially expressed genes, more than 65% are expressed in human or mouse fetal heart tissues (GEO dataset). Additional LCLs from new groups of AVSD and ASD+VSD patients were analyzed by quantitative PCR; observed expression ratios were similar to microarray results. Analysis of GO categories revealed enrichment of genes from pathways regulating clathrin-mediated endocytosis in patients with AVSD and of genes involved in semaphorin-plexin-driven cardiogenesis and the formation of cytoplasmic microtubules in patients with ASD-VSD. A pathway-oriented search revealed enrichment in the ciliome for both groups and a specific enrichment in Hedgehog and Jak-stat pathways among ASD+VSD patients. These genes or related pathways are therefore potentially involved in normal cardiogenesis as well as in cardiac malformations observed in individuals with trisomy 21. PMID:22912673

  5. Endoscopic closure of septal perforations.

    Science.gov (United States)

    Alobid, Isam

    2017-05-26

    The management of septal perforations is a challenge for the surgeon. A wide variety of surgical techniques have been described, with different approaches. There is no scientific evidence to support a particular approach. The objective of this review is to present a practical guide on the technique of choice for each case of septal perforation. Inspection of the nasal mucosa, the size of the perforation, the location and especially the osteo-cartilaginous support, are the pillars of a successful surgery. For the sliding or rotating flaps of the mucosa of the septum it is essential to know in advance if the elevation of the mucopericondrio or mucoperiosteo of the septum is possible, otherwise the use of these flaps would not be indicated. The flaps of the lateral wall or nasal floor are the alternative. The pericranial flap may be indicated in total or near total perforations. The remnant of the nasal septum and status of osteo-cartilaginous support are the determining factors in the management of septal perforations. Each case should be evaluated individually and the approach chosen according to the size and location of the perforation, mucosal quality, personal history, previous surgery and the experience of the surgeon. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

  6. Vertical zonality of septal nectaries of Monocots

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    Аnastasiya Odintsova

    2013-04-01

    Full Text Available Considering the septal nectary as a system of exogenous cavities inside the ovary and taking account of possibilities of various ways of the formation of nectary walls we propose to apply the concept of vertical zonality to the analysis of the septal nectary structure. The comparative analysis of the gynoecium with septal nectaries must include data about the nectary vertical zones and its location in the structural zones of the gynoecium.

  7. 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.

  8. Anterograde conduction to the His bundle during right ventricular overdrive pacing distinguishes septal pathway atrioventricular reentry from atypical atrioventricular nodal reentrant tachycardia.

    Science.gov (United States)

    Nagashima, Koichi; Kumar, Saurabh; Stevenson, William G; Epstein, Laurence M; John, Roy M; Tedrow, Usha B; Koplan, Bruce A; Michaud, Gregory F

    2015-04-01

    Distinguishing orthodromic atrioventricular reciprocating tachycardia (ORT) using a retrograde septal accessory pathway (AP) from atypical atrioventricular nodal reentrant tachycardia (AVNRT) may be challenging. Specifically, excluding the presence and participation of an AP may require multiple diagnostic maneuvers. The purpose of this study was to assess the relative value of commonly used right ventricular (RV) pacing maneuvers, including identification of anterograde His-bundle activation with entrainment, to differentiate ORT using a retrograde septal AP from atypical AVNRT. From March 2009 to June 2014, 56 patients (28 female; age 43.9 ± 17.4 years) who underwent electrophysiologic study and ablation for supraventricular tachycardia (26 ORT using septal AP and 30 atypical AVNRT) that exhibited a concentric atrial activation pattern and a septal ventriculoatrial interval >70 ms were analyzed. Overdrive pacing maneuvers or ventricular extrastimuli failed on at least 1 occasion to correctly identify a septal AP. Overall, 16 ORT patients and 26 AVNRT patients had successful RV entrainment, and 12 (75%) ORT patients showed anterograde His capture (11 patients) and/or anterograde septal ventricular capture (3 patients). None of the patients with atypical AVNRT showed anterograde conduction to the His bundle with entrainment. RV pacing maneuvers are useful to exclude an AP in patients with AVNRT having concentric atrial activation sequence and a septal ventriculoatrial interval >70 ms; however, none are consistently diagnostic. When observed in this patient population, anterograde His-bundle or septal ventricular capture during RV entrainment was diagnostic for ORT using a septal AP. Copyright © 2015 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  9. Interventions on atrial septum under cardiopulmonary bypass and without aortic crossclamping

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    Ye. V. Krivoshchekov

    2015-10-01

    Full Text Available The study centered on the possibility of surgery on the atrial septum (AS without aortic clamping and cardiac arrest. We compared two groups of patients who underwent intervention on AS as the major or concomitant stage of surgical treatment. Nineteen patients were assigned to the main group; they were operated under CPB without aortic clamping and cardioplegia. The control group consisted of 20 patients, whose AS was surgically treated by using a standard technique with aortic occlusion. The groups were comparable by age and disease. The patients of both groups withstood the surgical interventions satisfactorily, with no mortality. The mean CBP time in the patients operated without aortic clamping was shorter than that in the control group patients (operated with aortic clamping by about 18-20 minutes. The major surgical stage time did not differ between the groups. The technique of atrial septal defect closure or its dissection on the beating heart without cardioplegia is safe, comfortable for a surgeon and allows saving time required for the recovery period. Besides, it does not cause myocardial lesion associated with cardioplegia.

  10. Study of prevalence & risk factors of congenital heart defect (Review Article

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    ali dehghani

    2017-09-01

    Full Text Available Abstract Background: Congenital heart defects are known as the state that comes from birth and influences on structure and function of baby's heart, The different types of defects can range from mild (e.g., a small hole between the heart chambers to hard (like a flaw or weakness in a part of the heart. Method: This article is a review article in which the articles published in Farsi and English that the bases valid as Medline, Google Scholar, Pubmed, Springer, SID index has been used, as well as for the study of keywords associated with the use of MESH keywords in identifying and no time limit listed in the databases were searched. Result The prevalence of congenital heart defect, in general, less than one per cent in newborn. The ventricular wall abnormalities defect (VSD, atrial septal defect of (ASD, patent ductus arteriosus (PDA and tetralogy of Fallot (TOF Top among the most types of congenital heart anomalies. Factors such as the age of the parents at conception, maternal risk of diabetes, influenza and febrile illness during pregnancy, drug use during pregnancy and taking a multivitamin before and during the Pregnancy were influenced of newborns with congenital heart defects. Conclusion: According to studies need to be conducted in the presence of multiple risk factors for these disorders, seem to have a detailed plan to Study of More about the factors that affect the risk of developing these disorders, as well as interventions to reduce risk factors identified particularly during pregnancy.

  11. Influence of thrombolytic therapy on the patterns of ventricular septal rupture after acute myocardial infarction

    OpenAIRE

    Rhydwen, G; Charman, S; Schofield, P

    2002-01-01

    Background: Post-myocardial infarction ventricular septal defect (VSD) complicates ∼2% of myocardial infarctions. Thrombolytic therapy may accelerate the time from myocardial infarction to VSD formation. The effects of thrombolytic therapy in patients with a post-myocardial infarction VSD were investigated.

  12. Association Between Local Bipolar Voltage and Conduction Gap Along the Left Atrial Linear Ablation Lesion in Patients With Atrial Fibrillation.

    Science.gov (United States)

    Masuda, Masaharu; Fujita, Masashi; Iida, Osamu; Okamoto, Shin; Ishihara, Takayuki; Nanto, Kiyonori; Kanda, Takashi; Sunaga, Akihiro; Tsujimura, Takuya; Matsuda, Yasuhiro; Mano, Toshiaki

    2017-08-01

    A bipolar voltage reflects a thick musculature where formation of a transmural lesion may be hard to achieve. The purpose of this study was to explore the association between local bipolar voltage and conduction gap in patients with persistent atrial fibrillation (AF) who underwent atrial roof or septal linear ablation. This prospective observational study included 42 and 36 consecutive patients with persistent AF who underwent roof or septal linear ablations, respectively. After pulmonary vein isolation, left atrial linear ablations were performed, and conduction gap sites were identified and ablated after first-touch radiofrequency application. Conduction gap(s) after the first-touch roof and septal linear ablation were observed in 13 (32%) and 19 patients (53%), respectively. Roof and septal area voltages were higher in patients with conduction gap(s) than in those without (roof, 1.23 ± 0.77 vs 0.73 ± 0.42 mV, p = 0.010; septal, 0.96 ± 0.43 vs 0.54 ± 0.18 mV, p = 0.001). Trisected regional analyses revealed that the voltage was higher at the region with a conduction gap than at the region without. Complete conduction block across the roof and septal lines was not achieved in 3 (7%) and 6 patients (17%), respectively. Patients in whom a linear conduction block could not be achieved demonstrated higher ablation area voltage than those with a successful conduction block (roof, 1.91 ± 0.74 vs 0.81 ± 0.51 mV, p = 0.001; septal, 1.15 ± 0.56 vs 0.69 ± 0.31 mV, p = 0.006). In conclusion, a high regional bipolar voltage predicts failure to achieve conduction block after left atrial roof or septal linear ablation. In addition, the conduction gap was located at the preserved voltage area. Copyright © 2017 Elsevier Inc. All rights reserved.

  13. Congenital heart defects in children with oral clefts

    Directory of Open Access Journals (Sweden)

    Nahvi H.

    2007-09-01

    Full Text Available   Background: Oral clefts are among the most common congenital anomalies. Infants with oral clefts often have other associated congenital defects, especially congenital heart defects. The reported incidences and the types of associated malformations and congenital heart defects vary between different studies. The purpose of this study was to assess the incidence of associated congenital heart defects in children with oral clefts. Methods: All infants with cleft lip and palate referred to the Children's Medical Center and Bahramy; the teaching Hospitals of the Tehran University of Medical Sciences from 1991 to 2005 were prospectively enrolled in this study group. All patients were examined and noted by an academic cleft team contain; a pediatrician and a pediatric surgeon, and received cardiac consultation and echocardiography by a pediatric cardiologist. non cardiac associated anomalies, still born and patients without echocardiography were excluded from the study.  Data including age, gender, exposure to contagions and high risk elements ,consanguinity and familial history of oral cleft, type of oral cleft, results of cardiac consultation and echocardiography and associated cardiac anomalies were cumulated and analyzed by SSPS version 13.5Results: Among the 284 infants with oral clefts, 162 were male (57% and 122 were female (43%. Seventy-nine patients (27.8% had cleft lip, 84 (29.5% had cleft palate and 121 (42.6% had both cleft lip and palate. Of all the patients, 21.1% had congenital heart defects. the most common type Of these congenital heart defects(28.3%  was atrial septal defect.Conclusions: For patients with cleft lip and palate, we recommend preoperative cardiac consultation, careful examination and routine echocardiography for associated cardiac anomalies, as well as appropriate management and prophylactic antibiotic therapy for those with associated congenital heart anomaly.

  14. A randomized comparison of permanent septal versus apical right ventricular pacing: short-term results.

    Science.gov (United States)

    Victor, Frederic; Mabo, Philippe; Mansour, Hassan; Pavin, Dominique; Kabalu, Guillaume; de Place, Christian; Leclercq, Christophe; Daubert, J Claude

    2006-03-01

    This study compared chronic right ventricular (RV) pacing at the septum versus apex. Chronic RV apical pacing may be detrimental to ventricular function. This randomized, pilot study examined whether, compared with apical, permanent septal pacing preserves cardiac function. Ablation of the atrioventricular junction for permanent AF, followed by implantation of a DDDR pacemaker connected to two ventricular leads was performed in 28 patients. One lead screwed into the septum and another placed at the apex were connected to the atrial and ventricular port, respectively. Septum or apex was paced by programming AAIR or VVIR modes, respectively. Patients were randomly assigned, 4 months later, to pacing at one site for 3 months, and crossed over to the other for 3 months. New York Heart Association class, QRS width and axis, left ventricular ejection fraction (LVEF), exercise duration, and peak oxygen uptake were measured. Results in patients with LVEF > 45% and < or = 45% were compared. Septal pacing was associated with shorter QRS (145 +/- 4 msec vs 170 +/- 4 msec, P < 0.01) and normal axis (40 degrees +/- 10 degrees vs -71 +/- 4 degrees , P < 0.01). At 3 months, among patients with baseline LVEF < or = 45%, LVEF was 42 +/- 5% after septal pacing versus 37 +/- 4% after apical pacing (P < 0.001). In contrast to RV apical pacing, chronic RV septal pacing preserved LVEF in patients with baseline LVEF < or = 45%.

  15. [Hypertrophic cardiomyopathy with ventricular septal hypertrophy localized to the apical region of the left ventricle (apical ASH)].

    Science.gov (United States)

    Koga, Y; Takahashi, H; Ifuku, M; Itaya, M; Adachi, K; Toshima, H

    1984-08-01

    Clinical and morphologic features are described in a subgroup of 22 patients with hypertrophic cardiomyopathy, who showed ventricular septal hypertrophy localized to the apical region (apical asymmetric septal hypertrophy: apical ASH). All patients had ventricular septal thickness of 17 mm or less with an average of 13 +/- 3 mm in the M-mode echocardiograms. In contrast, the two-dimensional echocardiograms demonstrated septal hypertrophy localized to the apical region, with an average septal thickness of 20 +/- 3 mm at the mitral valve and papillary muscle levels. On the left ventriculogram, 82% of patients with apical ASH showed inward concavity of the right-inferior wall of the left ventricle, indicating hypertrophy of the ventricular septum. However, no patient showed spade-like appearance of the left ventricle and only two showed giant T wave inversion exceeding 10 mm. Echocardiographic examination performed in eight affected relatives revealed typical asymmetric septal hypertrophy (ASH) in four relatives, one of them showing a resting pressure gradient of 30 mmHg in the left ventricle. The remaining four relatives showed similar apical ASH. When compared with patients of typical ASH, patients with apical ASH demonstrated significantly greater cardiac size, left ventricular end-diastolic pressure, left atrial and left ventricular diameters and significantly lower percent fractional shortening. Atrial fibrillation, B bump of the mitral echogram and heart failure were more frequent in this subgroup of patients. Thus, apical ASH appeared to be a part of the morphologic spectrum of hypertrophic cardiomyopathy with ASH and to be a separate disease entity from apical hypertrophy previously described in Japan. Severely impaired diastolic performances associated with mild to moderately depressed systolic function of the left ventricle were the characteristic clinical features of the subgroup of patients with apical ASH.

  16. A Comprehensive In Silico Analysis on the Structural and Functional Impact of SNPs in the Congenital Heart Defects Associated with NKX2-5 Gene-A Molecular Dynamic Simulation Approach.

    Directory of Open Access Journals (Sweden)

    Firoz Abdul Samad

    Full Text Available Congenital heart defects (CHD presented as structural defects in the heart and blood vessels during birth contribute an important cause of childhood morbidity and mortality worldwide. Many Single nucletotide polymorphisms (SNPs in different genes have been associated with various types of congenital heart defects. NKX 2-5 gene is one among them, which encodes a homeobox-containing transcription factor that plays a crucial role during the initial phases of heart formation and development. Mutations in this gene could cause different types of congenital heart defects, including Atrial septal defect (ASD, Atrial ventricular block (AVB, Tetralogy of fallot and ventricular septal defect. This highlights the importance of studying the impact of different SNPs found within this gene that might cause structural and functional modification of its encoded protein. In this study, we retrieved SNPs from the database (dbSNP, followed by identification of potentially deleterious Non-synonymous single nucleotide polymorphisms (nsSNPs and prediction of their effect on proteins by computational screening using SIFT and Polyphen. Furthermore, we have carried out molecular dynamic simulation (MDS in order to uncover the SNPs that would cause the most structural damage to the protein altering its biological function. The most important SNP that was found using our approach was rs137852685 R161P, which was predicted to cause the most damage to the structural features of the protein. Mapping nsSNPs in genes such as NKX 2-5 would provide valuable information about individuals carrying these polymorphisms, where such variations could be used as diagnostic markers.

  17. A Comprehensive In Silico Analysis on the Structural and Functional Impact of SNPs in the Congenital Heart Defects Associated with NKX2-5 Gene-A Molecular Dynamic Simulation Approach.

    Science.gov (United States)

    Abdul Samad, Firoz; Suliman, Bandar A; Basha, Syed Hussain; Manivasagam, Thamilarasan; Essa, Musthafa Mohamed

    2016-01-01

    Congenital heart defects (CHD) presented as structural defects in the heart and blood vessels during birth contribute an important cause of childhood morbidity and mortality worldwide. Many Single nucletotide polymorphisms (SNPs) in different genes have been associated with various types of congenital heart defects. NKX 2-5 gene is one among them, which encodes a homeobox-containing transcription factor that plays a crucial role during the initial phases of heart formation and development. Mutations in this gene could cause different types of congenital heart defects, including Atrial septal defect (ASD), Atrial ventricular block (AVB), Tetralogy of fallot and ventricular septal defect. This highlights the importance of studying the impact of different SNPs found within this gene that might cause structural and functional modification of its encoded protein. In this study, we retrieved SNPs from the database (dbSNP), followed by identification of potentially deleterious Non-synonymous single nucleotide polymorphisms (nsSNPs) and prediction of their effect on proteins by computational screening using SIFT and Polyphen. Furthermore, we have carried out molecular dynamic simulation (MDS) in order to uncover the SNPs that would cause the most structural damage to the protein altering its biological function. The most important SNP that was found using our approach was rs137852685 R161P, which was predicted to cause the most damage to the structural features of the protein. Mapping nsSNPs in genes such as NKX 2-5 would provide valuable information about individuals carrying these polymorphisms, where such variations could be used as diagnostic markers.

  18. Gerbode defect following endocarditis and misinterpreted as severe pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Allajbeu Iris

    2010-09-01

    Full Text Available Abstract A Gerbode -type defect is a ventricular septal defect communicating directly between the left ventricle and right atrium. It is usually congenital, but rarely is acquired, as a complication of endocarditis. This can be anatomically possible because the normal tricuspid valve is more apically displaced than the mitral valve. However, identification of an actual communication is often extremely difficult, so a careful and meticulous echocardiogram should be done in order to prevent echocardiographic misinterpretation of this defect as pulmonary arterial hypertension. The large systolic pressure gradient between the left ventricle and the right atrium would expectedly result in a high velocity systolic Doppler flow signal in right atrium and it can be sometimes mistakably diagnosed as tricuspid regurgitant jet simulating pulmonary arterial hypertension. We present a rare case of young woman, with endocarditis who presented with severe pulmonary arterial hypertension. The preoperative diagnosis of left ventricle to right atrial communication (acquired Gerbode defect was suspected initially by echocardiogram and confirmed at the time of the surgery. A point of interest, apart from the diagnostic problem, was the explanation for its mechanism and presentation. The probability of a bacterial etiology of the defect is high in this case.

  19. Gerbode defect following endocarditis and misinterpreted as severe pulmonary arterial hypertension.

    Science.gov (United States)

    Xhabija, Nereida; Prifti, Edvin; Allajbeu, Iris; Sula, Fatmir

    2010-09-30

    A Gerbode-type defect is a ventricular septal defect communicating directly between the left ventricle and right atrium. It is usually congenital, but rarely is acquired, as a complication of endocarditis. This can be anatomically possible because the normal tricuspid valve is more apically displaced than the mitral valve. However, identification of an actual communication is often extremely difficult, so a careful and meticulous echocardiogram should be done in order to prevent echocardiographic misinterpretation of this defect as pulmonary arterial hypertension. The large systolic pressure gradient between the left ventricle and the right atrium would expectedly result in a high velocity systolic Doppler flow signal in right atrium and it can be sometimes mistakably diagnosed as tricuspid regurgitant jet simulating pulmonary arterial hypertension. We present a rare case of young woman, with endocarditis who presented with severe pulmonary arterial hypertension. The preoperative diagnosis of left ventricle to right atrial communication (acquired Gerbode defect) was suspected initially by echocardiogram and confirmed at the time of the surgery. A point of interest, apart from the diagnostic problem, was the explanation for its mechanism and presentation. The probability of a bacterial etiology of the defect is high in this case.

  20. Humeral septal aperture associated with supracondylar process: a case report and review of the literature.

    Science.gov (United States)

    Paraskevas, George K; Natsis, Konstantinos; Anastasopoulos, Nikolaos; Ioannidis, Orestis; Kitsoulis, Panagiotis

    2012-01-01

    The supracondylar process is usually a beak-like osseous prominence located at the anteromedial aspect of the distal portion of the humerus. It is usually asymptomatic but occasionally may compress underlying structures such as the median or ulnar nerve, the brachial artery or its branches. The term septal aperture defines an oval or round shaped bony defect of the septum that separates the olecranon from the coronoid fossa of the humerus. It is of significance for surgeons because it may alter the fracture pattern at the region and thus their management. We present a rare case of coexistence of supracondylar process and septal aperture in a macerated left humerus. The reported incidence of the supracondylar process alone varies from 0.28% to 2.78%, while that of the septal aperture from 6.9% to 60%. We have reviewed the literature and emphasized the radiological and surgical significance of the findings.

  1. Atrial Fibrillation

    Science.gov (United States)

    ... A-Z Clinical Trials Publications and Resources Health Education and Awareness The Science Science Home Blood Disorders ... heart's two upper chambers—called the atria (AY-tree-uh)—to fibrillate. The ... a difficult decision concerning surgery for patients with atrial fibrillation, the ...

  2. [Atrial defibrillator].

    Science.gov (United States)

    Jung, W; Lüderitz, B

    2000-01-01

    Atrial fibrillation (AF) is a frequent and costly health care problem representing the most common arrhythmia resulting in hospital admission. Total mortality and cardiovascular mortality are significantly increased in patients with AF compared to controls. In addition to symptoms of palpitations, patients with AF have an increased risk of stroke and may also develop decreased exercise tolerance and left ventricular dysfunction. All of these problems may be reversed with restoration and maintenance of sinus rhythm. External electrical cardioversion has been a remarkably effective and safe method for termination of this arrhythmia. Originally described by Lown et al. in 1963, it has been a well accepted mode of acute therapy. However, this technique requires general anesthesia or heavy sedation. Internal atrial defibrillation has been evaluated as an alternative approach to the external technique for over 2 decades. Recent studies have shown that low-energy internal atrial defibrillation using biphasic shocks is an effective and safe means in restoring sinus rhythm in patients with AF and should be considered especially in patients in whom external cardioversion attempts have failed. Implantable Atrial Defibrillator: Recently, a stand alone IAD, the Metrix System (models 3000 and 3020), has entered clinical investigation. Atrial defibrillation is accomplished by a shock delivered between electrodes in the right atrium and the coronary sinus. The right atrium lead has an active fixation in the right atrium. The coronary sinus lead has a natural spiral configuration for retention in the coronary sinus, and can be straightened with a stylet. Both leads are 7 French in diameter and the defibrillation coils are each 6 cm in length. The electrodes may be placed using separate leads, or very soon by using a single bipolar lead. A separate bipolar right ventricular lead is used for R wave synchronization and post shock pacing. The Metrix defibrillator can be used to induce

  3. Human fingernail as interpositional graft material in the treatment of nasal septal perforations.

    Science.gov (United States)

    Akçal, Arzu; Karsidag, Semra; Ozkaya, Ozay; Sirvan, Selami Serhat; Sevim, Kamuran Zeynep; Kabukcuoglu, Fevziye

    2014-04-01

    The etiology of nasal septal perforations involves iatrogenic, traumatic, inflammatory, infectious, neoplastic, and caustic causes. To ensure successful closure, an appropriate interpositional graft material should be selected, and this graft material should be covered with healthy tissue. The study included 18 New Zealand white rabbits weighing 2 to 2.5 kg. Nasal septal perforations were created in group 1. After the creation of defects in group 2, repair was performed with cartilage graft and bilateral mucoperichondrial advancement flaps. After septal nasal perforations in group 3, the defect was covered with fingernail and bilateral mucoperichondrial flaps. At week 12, the rabbits were sacrificed. The septum site that had been repaired with fingernail was intact. No nail exposition, wound site decomposition, or re-perforation was observed. No findings of a breach of the structural integrity of the fingernails or disintegration were encountered. Fingernails can be used as an interpositional graft material in place of cartilage in eligible cases for the repair of nasal septal perforations. Fingernails have several properties that enable their use in such cases, such as form preservation that is similar to cartilage, the lack of live cells, easy availability, and a lack of donor-site morbidity at removal. Copyright © 2014 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  4. The role of temporalis fascia for free mucosal graft survival in small nasal septal perforation repair.

    Science.gov (United States)

    Jeon, Eun-Ju; Choi, Jin; Lee, Joo-Hyung; Kim, Sung-Won; Nam, In-Chul; Park, Yong-Su; Jin, Sang-Gyun; Cheon, Byung-Jun

    2014-01-01

    Temporalis fascia has been used widely as a interposition graft for mucosal rotation flap in nasal septal perforation repair. However, the exact role of temporalis fascia in healing process has not yet been clarified. For the pedicle of rotation flap has been considered as a major vehicle for nutrition distribution, the role of temporalis fascia has been devaluated. In this study, we experienced small nasal septal perforation repairs using free mucosal graft not having pedicles but covered by temporalis fascia. Three patients with small nasal septal perforations not larger than 1 × 1 cm were included. In 2 patients, the perforations were repaired using free composite grafts from the inferior turbinate mucosa covered by continuous temporalis fascia not divided, and the surgical results were successful with complete healings. In 1 patient, however, the temporalis fascia was divided into 2 parts to better fit the shape of the perforation, and the graft failed to survive. These surgical results suggest that the temporalis fascia might have an important role in healing process of nasal septal defect and could be used as a beneficial options for small mucosal defect repair surgeries using free mucosal grafts.

  5. Increasing Prevalence of Atrial Fibrillation and Permanent Atrial Arrhythmias in Congenital Heart Disease.

    Science.gov (United States)

    Labombarda, Fabien; Hamilton, Robert; Shohoudi, Azadeh; Aboulhosn, Jamil; Broberg, Craig S; Chaix, Marie A; Cohen, Scott; Cook, Stephen; Dore, Annie; Fernandes, Susan M; Fournier, Anne; Kay, Joseph; Macle, Laurent; Mondésert, Blandine; Mongeon, François-Pierre; Opotowsky, Alexander R; Proietti, Anna; Rivard, Lena; Ting, Jennifer; Thibault, Bernard; Zaidi, Ali; Khairy, Paul

    2017-08-15

    Atrial arrhythmias are the most common complication encountered in the growing and aging population with congenital heart disease. This study sought to assess the types and patterns of atrial arrhythmias, associated factors, and age-related trends. A multicenter cohort study enrolled 482 patients with congenital heart disease and atrial arrhythmias, age 32.0 ± 18.0 years, 45.2% female, from 12 North American centers. Qualifying arrhythmias were classified by a blinded adjudicating committee. The most common presenting arrhythmia was intra-atrial re-entrant tachycardia (IART) (61.6%), followed by atrial fibrillation (28.8%), and focal atrial tachycardia (9.5%). The proportion of arrhythmias due to IART increased with congenital heart disease complexity from 47.2% to 62.1% to 67.0% in patients with simple, moderate, and complex defects, respectively (p = 0.0013). Atrial fibrillation increased with age to surpass IART as the most common arrhythmia in those ≥50 years of age (51.2% vs. 44.2%; p heart disease, with a predominantly paroxysmal pattern. However, atrial fibrillation increases in prevalence and atrial arrhythmias progressively become permanent as the population ages. Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  6. Left ventricular hypertrabeculation/noncompaction with epilepsy, other heart defects, minor facial anomalies and new copy number variants

    Directory of Open Access Journals (Sweden)

    Nagel Bert

    2012-07-01

    Full Text Available Abstract Background Left ventricular hypertrabeculation/noncompaction (LVHT is a cardiac abnormality of unknown etiology which has been described in children as well as in adults with and without chromosomal aberrations. LVHT has been reported in association with various cardiac and extracardiac abnormalities like epilepsy and facial dysmorphism. Case presentation A unique combination of LVHT, atrial septal defect, pulmonary valve stenosis, aortic stenosis, epilepsy and minor facial anomalies is presented in a 5.5 years old girl. Microarray-based genomic hybridization (array-CGH detected six previously not described copy number variants (CNVs inherited from a clinically unaffected father and minimally affected mother, thus, most likely, not clinically significant but rare benign variants. Conclusions Despite this complex phenotype de novo microdeletions or microduplications were not detected by array CGH. Further investigations, such as whole exome sequencing, could reveal point mutations and small indels as the possible cause.

  7. Genome-wide linkage analysis of congenital heart defects using MOD score analysis identifies two novel loci.

    Science.gov (United States)

    Flaquer, Antònia; Baumbach, Clemens; Piñero, Estefania; García Algas, Fernando; de la Fuente Sanchez, María Angeles; Rosell, Jordi; Toquero, Jorge; Alonso-Pulpon, Luis; Garcia-Pavia, Pablo; Strauch, Konstantin; Heine-Suñer, Damian

    2013-05-24

    Congenital heart defects (CHD) is the most common cause of death from a congenital structure abnormality in newborns and is often associated with fetal loss. There are many types of CHD. Human genetic studies have identified genes that are responsible for the inheritance of a particular type of CHD and for some types of CHD previously thought to be sporadic. However, occasionally different members of the same family might have anatomically distinct defects - for instance, one member with atrial septal defect, one with tetralogy of Fallot, and one with ventricular septal defect. Our objective is to identify susceptibility loci for CHD in families affected by distinct defects. The occurrence of these apparently discordant clinical phenotypes within one family might hint at a genetic framework common to most types of CHD. We performed a genome-wide linkage analysis using MOD score analysis in families with diverse CHD. Significant linkage was obtained in two regions, at chromosome 15 (15q26.3, P(empirical) = 0.0004) and at chromosome 18 (18q21.2, P(empirical) = 0.0005). In these two novel regions four candidate genes are located: SELS, SNRPA1, and PCSK6 on 15q26.3, and TCF4 on 18q21.2. The new loci reported here have not previously been described in connection with CHD. Although further studies in other cohorts are needed to confirm these findings, the results presented here together with recent insight into how the heart normally develops will improve the understanding of CHD.

  8. 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 Marie

    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...

  9. Perinatal outcomes and congenital heart defect prognosis in 53313 non-selected perinatal infants.

    Science.gov (United States)

    Xie, Donghua; Wang, Hua; Liu, Zhiyu; Fang, Junqun; Yang, Tubao; Zhou, Shujin; Wang, Aihua; Qin, Jiabi; Xiong, Lili

    2017-01-01

    To evaluate perinatal outcomes and congenital heart defect (CHD) prognosis in a non-selected population. The population-based surveillance data used in this assessment of CHDs were based on birth defect surveillance data collected from 2010-2012 in Liuyang City, China. Infants living with CHDs were followed up for 5 years to determine their prognosis. Prevalence, prenatal diagnosis, perinatal outcomes, and total and type-specific prognosis data were assessed using SPSS 18.0. In total, 190 CHD cases were identified among the 53313 included perinatal infants (PIs), indicating a CHD prevalence of 35.64 per 10000 PIs in this non-selected population. The five most frequently identified types of CHDs were ventricular septal defects (VSDs, 38.95%), atrial septal defects (ASDs, 15.79%), cardiomegaly (7.89%), tetralogy of Fallot (TOF, 5.79%), and atrioventricular septal defects (AVSDs, 5.26%). Of the 190 CHD cases, 110 (57.89%) were diagnosed prenatally, 30 (15.79%) were diagnosed with associated malformations, and 69 (36.32%) resulted in termination of pregnancy (TOP). Moreover, 15 (7.89%) PIs died within 7 days after delivery, and 42 (22.10%) died within 1 year. In contrast, 79 (41.58%) were still alive after 5 years. When TOP cases were included, the 5-year survival rate of PIs with prenatally detected CHDs was lower than that of PIs with postnatally detected CHDs (25.45% vs. 63.75%). The CHD subtype associated with the highest rate of infant (less than 1 year old) mortality was transposition of the great arteries (100%). The subtypes associated with higher 5-year survival rates were patent ductus arteriosus (80%), ASD (63.33%), VSD (52.70%) and AVSD (50%). The rates of prenatal CHD detection and TOP were high in this study population, and the 5-year survival rate of PIs with CHDs was low. The government should strengthen efforts to educate pediatricians regarding this issue and provide financial assistance to improve the prognosis of infants living with CHDs, especially

  10. Down's syndrome with Ventricular septal Defect (VsD)

    African Journals Online (AJOL)

    In Down's syndrome, 95% of all cases are caused by non disjunction: one cell has two 21st chromosomes instead of one, so the resulting fertilized egg has three. 21st chromosomes. Hence the scientific name, trisomy. 21. The cause of the non disjunction error isn't known, but there is definitely connection with maternal age.

  11. Transcatheter device closure of postmyocardial infarction ventricular septal defect

    Directory of Open Access Journals (Sweden)

    You-Lin Nie

    2017-01-01

    Conclusion: Our findings indicate that transcatheter device closure of PMIVSD is technically feasible, safe, and effective to reduce the shunt. The crucial prognostic factors were ascertained to be age ≥ 80 years, systolic blood pressure ≤ 90 mmHg, and procedure time ≥180 minutes.

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

    African Journals Online (AJOL)

    Date of Acceptance: 20‑Jul‑2014. Address for correspondence: Dr. OO Ogunkunle,. Department of ... a large VSD in the muscular portion measuring 2.57 cm. [Figure 1]. At the time, the connection between the ... also, the absence of clinical symptoms suggestive of a large VSD prior to the trauma in our patient, should have.

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

    African Journals Online (AJOL)

    2014-07-20

    Jul 20, 2014 ... He presented 11 days later with features of acute congestive cardiac failure. ... trauma to the chest. Any such patient should undergo careful echocardiographic evaluation. There is an ... complained of chest pains. At a private ...

  14. Straddling tricuspid valve without a ventricular septal defect.

    OpenAIRE

    Isomatsu, Y; Kurosawa, H.; Imai, Y

    1989-01-01

    A four year old girl with pulmonary atresia had a straddling tricuspid valve without an interventricular communication. The overriding tricuspid valve had two orifices, which connected with the right and the left ventricles. Valve tissue separated both orifices and was firmly connected to the crest of the ventricular septum, thus sealing off the expected interventricular communication. Surgical correction was performed and the outcome was satisfactory.

  15. Straddling tricuspid valve without a ventricular septal defect.

    Science.gov (United States)

    Isomatsu, Y; Kurosawa, H; Imai, Y

    1989-09-01

    A four year old girl with pulmonary atresia had a straddling tricuspid valve without an interventricular communication. The overriding tricuspid valve had two orifices, which connected with the right and the left ventricles. Valve tissue separated both orifices and was firmly connected to the crest of the ventricular septum, thus sealing off the expected interventricular communication. Surgical correction was performed and the outcome was satisfactory.

  16. 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.

  17. Gene network and familial analyses uncover a gene network involving Tbx5/Osr1/Pcsk6 interaction in the second heart field for atrial septation.

    Science.gov (United States)

    Zhang, Ke K; Xiang, Menglan; Zhou, Lun; Liu, Jielin; Curry, Nathan; Heine Suñer, Damian; Garcia-Pavia, Pablo; Zhang, Xiaohua; Wang, Qin; Xie, Linglin

    2016-03-15

    Atrial septal defects (ASDs) are a common human congenital heart disease (CHD) that can be induced by genetic abnormalities. Our previous studies have demonstrated a genetic interaction between Tbx5 and Osr1 in the second heart field (SHF) for atrial septation. We hypothesized that Osr1 and Tbx5 share a common signaling networking and downstream targets for atrial septation. To identify this molecular networks, we acquired the RNA-Seq transcriptome data from the posterior SHF of wild-type, Tbx5(+/) (-), Osr1(+/-), Osr1(-/-) and Tbx5(+/-)/Osr1(+/-) mutant embryos. Gene set analysis was used to identify the Kyoto Encyclopedia of Genes and Genomes pathways that were affected by the doses of Tbx5 and Osr1. A gene network module involving Tbx5 and Osr1 was identified using a non-parametric distance metric, distance correlation. A subset of 10 core genes and gene-gene interactions in the network module were validated by gene expression alterations in posterior second heart field (pSHF) of Tbx5 and Osr1 transgenic mouse embryos, a time-course gene expression change during P19CL6 cell differentiation. Pcsk6 was one of the network module genes that were linked to Tbx5. We validated the direct regulation of Tbx5 on Pcsk6 using immunohistochemical staining of pSHF, ChIP-quantitative polymerase chain reaction and luciferase reporter assay. Importantly, we identified Pcsk6 as a novel gene associated with ASD via a human genotyping study of an ASD family. In summary, our study implicated a gene network involving Tbx5, Osr1 and Pcsk6 interaction in SHF for atrial septation, providing a molecular framework for understanding the role of Tbx5 in CHD ontogeny. © The Author 2016. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  18. 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)....

  19. Maternal residential exposure to agricultural pesticides and birth defects in a 2003 to 2005 North Carolina birth cohort.

    Science.gov (United States)

    Rappazzo, Kristen M; Warren, Joshua L; Meyer, Robert E; Herring, Amy H; Sanders, Alison P; Brownstein, Naomi C; Luben, Thomas J

    2016-04-01

    Birth defects are responsible for a large proportion of disability and infant mortality. Exposure to a variety of pesticides have been linked to increased risk of birth defects. We conducted a case-control study to estimate the associations between a residence-based metric of agricultural pesticide exposure and birth defects. We linked singleton live birth records for 2003 to 2005 from the North Carolina (NC) State Center for Health Statistics to data from the NC Birth Defects Monitoring Program. Included women had residence at delivery inside NC and infants with gestational ages from 20 to 44 weeks (n = 304,906). Pesticide exposure was assigned using a previously constructed metric, estimating total chemical exposure (pounds of active ingredient) based on crops within 500 meters of maternal residence, specific dates of pregnancy, and chemical application dates based on the planting/harvesting dates of each crop. Logistic regression was used to estimate odds ratios (ORs) and 95% confidence intervals for four categories of exposure (90(th) percentiles) compared with unexposed. Models were adjusted for maternal race, age at delivery, education, marital status, and smoking status. We observed elevated ORs for congenital heart defects and certain structural defects affecting the gastrointestinal, genitourinary and musculoskeletal systems (e.g., OR [95% confidence interval] [highest exposure vs. unexposed] for tracheal esophageal fistula/esophageal atresia = 1.98 [0.69, 5.66], and OR for atrial septal defects: 1.70 [1.34, 2.14]). Our results provide some evidence of associations between residential exposure to agricultural pesticides and several birth defects phenotypes. Birth Defects Research (Part A) 106:240-249, 2016. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  20. 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.

  1. Sports participation in adults with congenital heart disease

    NARCIS (Netherlands)

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

    2015-01-01

    textabstractBackground: It is unclearwhether sports participation in adultswith repaired congenital heart disease is safe and has benefits. Methods: Congenital heart disease (ConHD) patients who underwent corrective surgery for Atrial Septal Defect, Ventricular Septal Defect, Pulmonary Stenosis,

  2. Trisomy 18

    Science.gov (United States)

    ... congenital heart disease , such as: Atrial septal defect (ASD) Patent ductus arteriosus (PDA) Ventricular septal defect (VSD) Tests may also show kidney problems, including: Horseshoe kidney Hydronephrosis Polycystic kidney Treatment There are no specific treatments for trisomy 18. ...

  3. Transcatheter occlusion of baffle leaks following atrial switch procedures for transposition of the great vessels (d-TGV).

    Science.gov (United States)

    Balzer, David T; Johnson, Mark; Sharkey, Angela M; Kort, Henry

    2004-02-01

    Baffle-related complications following atrial switch procedures for transposition are relatively common. Transcatheter treatment of baffle stenosis has an established role as a therapeutic modality. However, transcatheter device closure of atrial baffles leaks has rarely been reported. We report four patients who underwent device closure of baffle leaks using the Amplatzer septal occluder following atrial switch procedures in order to demonstrate the safety and utility of this method of treatment and to establish its role as a suitable alternative to surgical closure. Copyright 2004 Wiley-Liss, Inc.

  4. Experiencia multicéntrica colombiana durante tres años en el cierre percutáneo de la comunicación interventricular con diferentes dispositivos oclusores. Propuesta para la simplicación de la técnica en pacientes seleccionados Colombian multicentric experience during three years for percutaneous closure of interventricular septal defect with different occluder devices. Proposal for technique simplification in selected patients

    Directory of Open Access Journals (Sweden)

    Manuel R Téllez

    2010-09-01

    ,9% acusaron aparición o acentuación de episodios de cefalea vascular migrañosa. Hubo una complicación mayor dada por bloqueo atrioventricular completo y embolización del dispositivo. Conclusiones: el cierre endovascular de la comunicación interventricular es un procedimiento factible en nuestro medio, con el cual se alcanza un éxito cercano al 100%. El dispositivo para cierre de ductus de última generación puede implantarse con modificaciones en la técnica que aminora los tiempos de operatividad (hasta 60%, anestesia, utilización de medio de contraste y estancia hospitalaria. Por otra parte, su perfil hace posible el uso de sistemas de entregas 4 French que posibilitaría la inclusión de lactantes menores.Objectives: we describe the experience of the endovascular closure of interventricular communication performed for three years in different centers of interventional cardiology and highlight its result in a group of selected patients in whom such procedure was made by the use of a last generation device designed for the closure of ductus arteriosus. Methodology: between June 2006 and October 2009, a total of 34 patients underwent endovascular occlusion for interventricular septal defect. Indication of this procedure included volume overload, pressure and volume overload, chronic congestive heart failure, aortic valve prolapse with insufficiency and traumatic injury. Mean age was 12,9 years old and mean weight was 36 kg. A prevalence in male gender (53% was evidenced. Main blood flow calculation showed Qp / Qs of 1.71 to 1 and the pulmonary vascular resistance was 1.18 U Wood/m2. Materials and methods: PM VSD occluder device was implanted with the classic technique (62,5% while the Duct Occluder II (28,1% was used with the proposed simplified technique. General anesthesia was used in 98% cases; 60% were guided by fluoroscopy and transesophageal echocardiography and 40% with transthoracic echocardiography. Results: size of the defect varied from 4 to 12 mm. 91% of

  5. Atrial tachyarrhythmia in adult congenital heart disease

    Science.gov (United States)

    Karbassi, Arsha; Nair, Krishnakumar; Harris, Louise; Wald, Rachel M; Roche, S Lucy

    2017-01-01

    The adult congenital heart disease (ACHD) population continues to grow and most cardiologists, emergency room physicians and family doctors will intermittently come into contact with these patients. Oftentimes this may be in the setting of a presentation with atrial tachyarrhythmia; one of the commonest late complications of ACHD and problem with potentially serious implications. Providing appropriate initial care and ongoing management of atrial tachyarrhythmia in ACHD patients requires a degree of specialist knowledge and an awareness of certain key issues. In ACHD, atrial tachyarrhythmia is usually related to the abnormal anatomy of the underlying heart defect and often occurs as a result of surgical scar or a consequence of residual hemodynamic or electrical disturbances. Arrhythmias significantly increase mortality and morbidity in ACHD and are the most frequent reason for ACHD hospitalization. Intra-atrial reentrant tachycardia and atrial fibrillation are the most prevalent type of arrhythmia in this patient group. In hemodynamically unstable patients, urgent cardioversion is required. Acute management of the stable patient includes anticoagulation, rate control, and electrical or pharmacological cardioversion. In ACHD, rhythm control is the preferred management strategy and can often be achieved. However, in the long-term, medication side-effects can prove problematic. Electrophysiology studies and catheter ablation are important treatments modalities and in certain cases, surgical or percutaneous treatment of the underlying cardiac defect has a role. ACHD patients, especially those with complex CHD, are at increased risk of thromboembolic events and anticoagulation is usually required. Female ACHD patients of child bearing age may wish to pursue pregnancies. The risk of atrial arrhythmias is increased during pregnancy and management of atrial tachyarrhythmia during pregnancy needs specific consideration. PMID:28706585

  6. Minimally invasive septal myectomy for the treatment of hypertrophic obstructive cardiomyopathy and intrinsic mitral valve disease.

    Science.gov (United States)

    Gilmanov, Daniyar Sh; Bevilacqua, Stefano; Solinas, Marco; Ferrarini, Matteo; Kallushi, Enkel; Santarelli, Philippo; Farneti, Pier Andrea; Glauber, Mattia

    2015-01-01

    Transaortic left ventricular septal myectomy described by Morrow is a classical procedure for the treatment of systolic anterior motion of the mitral apparatus associated with hypertrophic obstructive cardiomyopathy (HOCM). We aimed to review our results of transmitral septal myectomy and mitral valve repair/replacement in patients with intrinsic mitral valve disease associated with HOCM, operated on through a minimally invasive approach. Between 2005 and 2014, 19 patients [7 men (37%); mean (SD) age, 69.4 (14.5) years] were treated with minimally invasive approach for degenerative mitral regurgitation and HOCM. Preoperative peak left ventricular outflow tract (LVOT) gradient was 66 (24) mm Hg. Severe mitral regurgitation was diagnosed in 16 cases (84%). New York Heart Association functional class III to IV heart failure was present in 13 patients (68%). Fifteen patients (79%) underwent mitral valve replacement, and four patients (21%) underwent mitral valve repair. Left ventricular outflow tract obstruction was corrected directly in all patients via the mitral valve with septal myectomy/myotomy, avoiding aortotomy in majority of the patients. No significant prolongation of extracorporeal circulation/aortic cross-clamping times was observed (P = 0.41 and P = 0.67, respectively) when compared with a similar population without HOCM. No iatrogenic ventricular septal defect developed in treated patients. No hospital mortality occurred. Resting LVOT gradient reduced at discharge to 13 (22) mm Hg (P = 0.025). Transmitral left ventricular septal myectomy in patients with degenerative mitral valve disease is quite a simple, feasible, and effective technique and does not require aortotomy in most cases. It can be performed with low early mortality and satisfactory resolution of LVOT obstruction in a minimally invasive setting.

  7. 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

  8. Hypertrophic Obstructive Cardiomyopathy: Surgical Myectomy and Septal Ablation.

    Science.gov (United States)

    Nishimura, Rick A; Seggewiss, Hubert; Schaff, Hartzell V

    2017-09-15

    Hypertrophic cardiomyopathy is a genetic disorder characterized by marked hypertrophy of the myocardium. It is frequently accompanied by dynamic left ventricular outflow tract obstruction and symptoms of dyspnea, angina, and syncope. The initial therapy for symptomatic patients with obstruction is medical therapy with β-blockers and calcium antagonists. However, there remain a subset of patients who have continued severe symptoms, which are unresponsive to medical therapy. These patients can be treated with septal reduction therapy, either surgical septal myectomy or alcohol septal ablation. When performed by experienced operators working in high-volume centers, septal myectomy is highly effective with a >90% relief of obstruction and improvement in symptoms. The perioperative mortality rate for isolated septal myectomy in most centers is <1%. Alcohol septal ablation is a less invasive treatment. In many patients, the hemodynamic and clinical results are comparable to that of septal myectomy. However, the results of alcohol septal ablation are dependent on the septal perforator artery supplying the area of the contact between the hypertrophied septum and the anterior leaflet of the mitral valve. There are some patients, particularly younger patients with severe hypertrophy, who do not uniformly experience complete relief of obstruction and symptoms. Both techniques of septal reduction therapy are highly operator dependent. The final decision as to which approach should be selected in any given patient is dependent up patient preference and the availability and experience of the operator and institution at which the patient is being treated. © 2017 American Heart Association, Inc.

  9. Defeito do anel fibroso mitral posterior com aneurisma de átrio esquerdo e insuficiência mitral: tratamento cirúrgico com sucesso Defect of the posterior mitral fibrous ring with left atrial aneurysm and mitral insufficiency: successful surgical treatment

    Directory of Open Access Journals (Sweden)

    Ronaldo Machado BUENO

    1999-10-01

    Full Text Available Aneurismas em átrio esquerdo são pouco comuns, podendo ocorrer na aurícula ou na parede do átrio esquerdo. Freqüentemente, são assintomáticos, podendo ocorrer arritmias, fenômenos tromboembólicos ou insuficiência cardíaca como complicação da sua evolução. Apresentamos paciente de 39 anos, do sexo feminino, com defeito do anel posterior da valva mitral levando a grande dilatação aneurismática da parede posterior do átrio esquerdo com insuficiência mitral. O diagnóstico foi feito pela radiografia de tórax (abaulamento de silhueta cardíaca esquerda e ecocardiograma (grande aneurisma do átrio esquerdo posteriormente à parede posterior do ventrículo esquerdo com insuficiência mitral. O estudo hemodinâmico sugeriu pseudo-aneurisma de ventrículo esquerdo. Submetida a tratamento cirúrgico com auxílio da circulação extracorpórea, realizou-se anuloplastia mitral e exclusão do aneurisma com reconstrução do assoalho do átrio esquerdo com retalho de pericárdio bovino. A paciente apresentou boa evolução pós-operatória, recebendo alta hospitalar no oitavo dia em boas condições clínicas.Atrial aneurysm is a rare condition and can be found on the atrial appendage or on the atrial wall. Most patients are asymptomatic, but arrhythmias, thromboembolism, and heart failure are common complications. We present a 39 years old female patient with posterior mitral ring defect causing a great aneurysm of the left posterior atrial wall with mitral insufficiency. The diagnosis was achieved by chest roentgenogram (marked prominence of the upper left heart border and echocardiography (great left atrial aneurysm behind the left posterior ventricular wall with mitral insufficiency. Left ventricular cineangiogram suggested the presence of a false aneurysm of the left ventricle. The patient subsequently underwent surgical treatment with cardiopulmonary bypass. Posterior mitral valve annuloplasty was performed with aneurysm exclusion

  10. Incidental discovery of an unusual right atrial membrane in an adult patient

    Directory of Open Access Journals (Sweden)

    Nasrin N Aldawoodi

    2012-01-01

    Full Text Available We describe presence of an unusual right atrial membrane in a 30-year old female with end stage renal disease, hypertension and peripheral vascular disease. The patient was scheduled for midline sternotomy and pericardiotomy and removal of a migrated vascular stent in the right pulmonary artery. An intraoperative transesophageal echocardiogram (TEE revealed an unusual membranous structure with fenestrations that stretched across the right atrium with attachments superiorly at the free wall and inferiorly at the inter-atrial septum. There was no evidence of flow obstruction across the tricuspid valve. Some of the considerations for the likely diagnosis of this structure were a prominent Eustachian valve, persistent Chiari network, aneurysmal inter-atrial septum, an inter-atrial septal cyst or Cor triatriatum dexter (CTD.

  11. Hemostasis of Left Atrial Appendage Bleed With Lariat Device

    Directory of Open Access Journals (Sweden)

    Amena Hussain, MD

    2014-09-01

    Full Text Available New devices designed for minimally invasive closure of the left atrial appendage (LAA may be a viable alternative for patients in whom anticoagulation is considered high risk. The Lariat (Sentreheart, Redwood City, CA, which is currently FDA-approved for percutaneous closure of tissue, requires both trans-septal puncture and epicardial access. However it requires no anticoagulation after the procedure. Here we describe a case of effusion and tamponade during a Lariat procedure with successful completion of the case and resolution of the effusion.

  12. Double-contrast, single-phase computed tomography angiography for ruling out left atrial appendage thrombus prior to atrial fibrillation ablation

    NARCIS (Netherlands)

    Teunissen, Cas; Habets, Jesse; Velthuis, BK; Cramer, Maarten J; Loh, KP

    Prior to atrial fibrillation (AF) ablation, computed tomography angiography (CTA) is increasingly used for left atrial appendage (LAA) thrombus detection. LAA filling defects on CTA may represent thrombus or incomplete contrast mixing with blood. A pre-bolus of contrast material with delay before

  13. National time trends in congenital heart defects, Denmark, 1977-2005.

    Science.gov (United States)

    Oyen, Nina; Poulsen, Gry; Boyd, Heather A; Wohlfahrt, Jan; Jensen, Peter K A; Melbye, Mads

    2009-03-01

    Time trends in congenital heart defects (CHD) by specific phenotype and with long follow-up time are rarely available for an entire population. We present trends in national CHD prevalences over the past 3 decades. We linked information from the National Patient Register, the Causes of Death Register, and the Danish Cytogenetic Central Register for all persons born in Denmark, 1977 to 2005, and registered in the Civil Registration System, yielding a cohort of 1,763,591 persons-18,207 with CHD. Individuals with CHDs were classified by phenotype (heterotaxia, conotruncal defect, atrioventricular septal defect, anomalous pulmonary venous return, left and right ventricular outflow tract obstructions, septal defects, complex defects, associations, patent ductus arteriosus, unspecified, and other specified) by combining International Classification of Diseases codes using a hierarchical approach. From 1977 to 2005, the overall CHD birth prevalence increased from 73 to 113 per 10,000 live births. Generally, prevalence increased for defects diagnosed in infancy, until 1996-1997, and then stabilized. For each 5-year interval, isolated septal defects and severe defects increased by 22% (95% CI, 20%-25%) and 5% (95% CI, 4%-7%), respectively. Among the severe defects, conotruncal defects and atrioventricular septal defect showed the largest prevalence increases. Women had a lower prevalence of severe defects during the 1980s. The CHD prevalence increase was unchanged when persons with extracardiac defects or chromosomal aberrations were excluded. CHD birth prevalence increased from the beginning of the 1980s but stabilized in the late 1990s.

  14. Deglutition induced atrial tachycardia and atrial fibrillation.

    Science.gov (United States)

    Kanjwal, Yousuf; Imran, Naser; Grubb, Blair

    2007-12-01

    Deglutition induced supraventricular tachycardia is an uncommon condition postulated to be a vagally mediated phenomenon due to mechanical stimulation. Patients usually present with mild symptoms or may have severe debilitating symptoms. Treatment with Class I agents, beta blockers, calcium channel blockers, amiodarone and radiofrquency catheter ablation has shown to be successful in the majority of reported cases. We report the case of a 46-year-old healthy woman presenting with palpitations on swallowing that was documented to be transient atrial tachycardia with aberrant ventricular conduction as well as transient atrial fibrillation. She was successfully treated with propafenone with no induction of swallowing-induced tachycardia after treatment. This is also the first case to show swallowing-induced atrial tachycardia and atrial fibrillation in the same patient.

  15. A Novel Alpha Cardiac Actin (ACTC1 Mutation Mapping to a Domain in Close Contact with Myosin Heavy Chain Leads to a Variety of Congenital Heart Defects, Arrhythmia and Possibly Midline Defects.

    Directory of Open Access Journals (Sweden)

    Céline Augière

    Full Text Available A Lebanese Maronite family presented with 13 relatives affected by various congenital heart defects (mainly atrial septal defects, conduction tissue anomalies and midline defects. No mutations were found in GATA4 and NKX2-5.A set of 399 poly(AC markers was used to perform a linkage analysis which peaked at a 2.98 lod score on the long arm of chromosome 15. The haplotype analysis delineated a 7.7 meganucleotides genomic interval which included the alpha-cardiac actin gene (ACTC1 among 36 other protein coding genes. A heterozygous missense mutation was found (c.251T>C, p.(Met84Thr in the ACTC1 gene which changed a methionine residue conserved up to yeast. This mutation was absent from 1000 genomes and exome variant server database but segregated perfectly in this family with the affection status. This mutation and 2 other ACTC1 mutations (p.(Glu101Lys and p.(Met125Val which result also in congenital heart defects are located in a region in close apposition to a myosin heavy chain head region by contrast to 3 other alpha-cardiac actin mutations (p.(Ala297Ser,p.(Asp313His and p.(Arg314His which result in diverse cardiomyopathies and are located in a totally different interaction surface.Alpha-cardiac actin mutations lead to congenital heart defects, cardiomyopathies and eventually midline defects. The consequence of an ACTC1 mutation may in part be dependent on the interaction surface between actin and myosin.

  16. 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

  17. Congenital heart defects in recurrent reciprocal 1q21.1 deletion and duplication syndromes: rare association with pulmonary valve stenosis.

    Science.gov (United States)

    Digilio, M Cristina; Bernardini, Laura; Consoli, Federica; Lepri, Francesca R; Giuffrida, M Grazia; Baban, Anwar; Surace, Cecilia; Ferese, Rosangela; Angioni, Adriano; Novelli, Antonio; Marino, Bruno; De Luca, Alessandro; Dallapiccola, Bruno

    2013-03-01

    Microdeletion 1q21.1 (del 1q21.1) and the reciprocal microduplication 1q21.1 (dup 1q21.1) are newly recognized genomic disorders, characterized by developmental delay, dysmorphic features and congenital malformations. Congenital heart defect (CHD) is a major feature of del 1q21.1, and has been occasionally reported in dup 1q21.1. We report here a family segregating del 1q21.1 in 3 members. Two of the affected family members had CHD, including the proband with syndromic atrial septal defect, pulmonary valve stenosis (PVS), and muscular ventricular septal defects, and the maternal uncle with non-syndromic PVS. This finding prompted investigation of the role of recurrent rearrangements of chromosome 1q21.1 in the pathogenesis of PVS. We gathered 38 patients with PVS (11 syndromic and 27 non-syndromic), and searched for genomic rearrangements of 1q21.1. A dup 1q21.1 was detected in a single sporadic non-syndromic patient. Review of the CHDs in published del 1q21.1 and dup 1q21.1 subjects showed a great heterogeneity in anatomic types. In conclusion, the present family illustrates recurrent CHD in del 1q21.1, expressing either as syndromic in one family member or as non-syndromic in the another one. The spectrum of CHDs associated with del 1q21.1 and dup 1q21.1 can occasionally include PVS. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  18. Rare variants in NR2F2 cause congenital heart defects in humans

    NARCIS (Netherlands)

    Al Turki, Saeed; Manickaraj, Ashok K.; Mercer, Catherine L.; Gerety, Sebastian S.; Hitz, Marc-Phillip; Lindsay, Sarah; D'Alessandro, Lisa C. A.; Swaminathan, G. Jawahar; Bentham, Jamie; Arndt, Anne-Karin; Low, Jacoba; Breckpot, Jeroen; Gewillig, Marc; Thienpont, Bernard; Abdul-Khaliq, Hashim; Harnack, Christine; Hoff, Kirstin; Kramer, Hans-Heiner; Schubert, Stephan; Siebert, Reiner; Toka, Okan; Cosgrove, Catherine; Watkins, Hugh; Lucassen, Anneke M.; O'Kelly, Ita M.; Salmon, Anthony P.; Bu'lock, Frances A.; Granados-Riveron, Javier; Setchfield, Kerry; Thornborough, Chris; Brook, J. David; Mulder, Barbara; Klaassen, Sabine; Bhattacharya, Shoumo; Devriendt, Koen; Fitzpatrick, David F.; Wilson, David I.; Mital, Seema; Hurles, Matthew E.

    2014-01-01

    Congenital heart defects (CHDs) are the most common birth defect worldwide and are a leading cause of neonatal mortality. Nonsyndromic atrioventricular septal defects (AVSDs) are an important subtype of CHDs for which the genetic architecture is poorly understood. We performed exome sequencing in 13

  19. Prevention of atrial fibrillation by inter-atrial septum pacing guided by electrophysiological testing, in patients with delayed interatrial conduction.

    Science.gov (United States)

    Manolis, A G; Katsivas, A G; Vassilopoulos, C; Koutsogeorgis, D; Louvros, N E

    2002-04-01

    Interatrial septum (IAS) pacing seems efficient in synchronizing atrial depolarization in patients (pts) with delayed inter-atrial conduction, but its clinical role in preventing atrial tachyarrhythmias is still debated. This study was conducted in order to evaluate the clinical efficacy of IAS pacing guided by pace mapping of the IAS, as an alternative treatment modality in pts with drug refractory paroxysmal atrial fibrillation (PAF). We evaluated 29 pts (13 male, 16 female, 60 +/- 11 years), with drug refractory PAF, normal sinus node function and prolonged inter-atrial conduction time (P wave 142 +/- 10 ms). Multipolar catheters were inserted and the electrograms from the high right atrium (HRA) and proximal, middle and distal coronary sinus (CS) were recorded. The IAS was paced from multiple sites. The site of IAS where the timing between HRA and distal CS was AAIR (75-140 bpm) mode, with random selection of the order and after discontinuation of antiarrhythmic treatment. During the fourth period, the same AAIR mode was assessed, but antiarrhythmic drugs were also administered. We compared the arrhythmia free interval among the four periods. The proportion of atrial paced beats in AAIR pacing mode plus antiarrhythmics was significantly higher compared with the drug-free period in AAIR mode (57 +/- 9% and 49 +/- 9% respectively, P=0017) and with AAT pacing mode (44 +/- 10%,(, PAAIR mode. These intervals did not differ significantly from the pre-implantation period (24.1 +/- 6.3 days). The arrhythmia free interval in AAIR pacing in combination with antiarrhythmic drug therapy was 38.7 +/- 8.1 days and this was significantly longer than the previous periods (P<0.05). Atrial septal pacing in combination with antiarrhythmic drug therapy reduced the incidence of PAF in pts with prolonged inter-atrial conduction times. Pace mapping of the IAS is an attractive technique to assess the shortest atrial activation time between HRA and distal CS. Whether placement of the

  20. Septal alcoholization in hypertrophic cardiomyopathy: about 11 cases

    African Journals Online (AJOL)

    Outcomes of septal alcoholization in hypertrophic obstructive cardiomyopathy are not enough studied in all centers. The purpose of this study was to determine the outcomes of septal alcoholization in hypertrophic obstructive cardiomyopathy in our hospital. A retrospective and prospective descriptive study focused on all ...

  1. Delay in diagnosis of right atrial myxoma

    Energy Technology Data Exchange (ETDEWEB)

    Northcote, R.J.; Sethia, B.; Ballantyne, D.

    1985-02-01

    Clinical, echocardiographic, and nuclear angiographic findings in a 51-year-old woman who presented with a history of dyspnea are discussed. Initial echocardiography revealed no abnormality. However, a subsequent radionuclide angiogram revealed a filling defect on the right side of the heart. This represented a right atrial myxoma. Radionuclide angiography can provide a useful noninvasive tool in the diagnosis of intracardiac tumors when echocardiography has not been helpful.

  2. Molecular Diffusion through Cyanobacterial Septal Junctions

    Directory of Open Access Journals (Sweden)

    Mercedes Nieves-Morión

    2017-01-01

    Full Text Available Heterocyst-forming cyanobacteria grow as filaments in which intercellular molecular exchange takes place. During the differentiation of N2-fixing heterocysts, regulators are transferred between cells. In the diazotrophic filament, vegetative cells that fix CO2 through oxygenic photosynthesis provide the heterocysts with reduced carbon and heterocysts provide the vegetative cells with fixed nitrogen. Intercellular molecular transfer has been traced with fluorescent markers, including calcein, 5-carboxyfluorescein, and the sucrose analogue esculin, which are observed to move down their concentration gradient. In this work, we used fluorescence recovery after photobleaching (FRAP assays in the model heterocyst-forming cyanobacterium Anabaena sp. strain PCC 7120 to measure the temperature dependence of intercellular transfer of fluorescent markers. We find that the transfer rate constants are directly proportional to the absolute temperature. This indicates that the “septal junctions” (formerly known as “microplasmodesmata” linking the cells in the filament allow molecular exchange by simple diffusion, without any activated intermediate state. This constitutes a novel mechanism for molecular transfer across the bacterial cytoplasmic membrane, in addition to previously characterized mechanisms for active transport and facilitated diffusion. Cyanobacterial septal junctions are functionally analogous to the gap junctions of metazoans.

  3. Chlamydial conjunctivitis presenting as pre septal cellulitis

    Directory of Open Access Journals (Sweden)

    Diaper Charles JM

    2007-03-01

    Full Text Available Abstract Chlamydia conjuctivitis results from infection by chlamydia trachomatis, the commonest treatable sexually transmitted infection in Europe. Its clinical manifestations involve the conjunctiva and the cornea. The inflammation under the upper eyelid may be sufficient to present as ptosis, however previously it has not been documented to cause a preseptal cellulitis. We present such a case. A 15-year-old girl was diagnosed with a left viral conjunctivitis. Five days later, she returned with marked oedema of the left upper and lower lids accompanied by erythema. The tarsal conjunctiva revealed follicles and large papillae and extra ocular movements revealed discomfort on elevation. A secondary diagnosis of bacterial pre septal cellulitis was made and the treatment was changed a broad spectrum oral antibiotic. On review at two days, the patient now complained of a large amount of purulent discharge in association with the marked pre septal swelling. As previous bacteriology and virology had been negative, the patient was re swabbed for chlamydia. This proved positive and her symptoms completely resolved following administration of Azithromycin. In this particular case recognition of the pathogen is important to alert the patient to the likelihood of unknown genital infestation. In all cases of positive culture, the patient should be counselled to attend a genitourinary clinic and to alert any sexual partners to the need to do likewise.

  4. Three Achilles’ heels of alcohol septal ablation

    Directory of Open Access Journals (Sweden)

    M. G. Kashtanov

    2017-11-01

    Full Text Available This manuscript looks at basic limitations of alcohol septal ablation in obstructive hypertrophic cardiomyopathy. They include high-grade atrioventricular blockages, residual obstructions of the left ventricular outflow tract and the so-called proarrhythmic effects of alcohol septal ablation procedure. All these weaknesses are reviewed in the context of incidence, etiology, and prevention.Received 25 February 2017. Accepted 10 April 2017.Funding: The study did not have sponsorship.Conflict of interest: The authors declare no conflict of interest.Author contributionsConception and study design: M.G. Kashtanov.Data collection and analysis: M.G. Kashtanov.Drafting the article: M.G. Kashtanov, E.M. Idov.Final approval of the version to be published: M.G. Kashtanov, S.D. Chernyshev, L.V. Kardapoltsev, S.V. Berdnikov, E.M. Idov.Full text of the article is in the online version of this paper at http://dx.doi.org/10.21688/1681-3472-2017-3-12-22

  5. Olfactory receptors in the mouse septal organ.

    Science.gov (United States)

    Kaluza, Jan F; Gussing, Fredrik; Bohm, Staffan; Breer, Heinz; Strotmann, Jörg

    2004-05-15

    In this study we have identified a repertoire of chemosensory receptors expressed in the septal organ (SO). The results suggest that septal organ neurons are specified to express receptor genes belonging to class II olfactory receptors that are also expressed in the main olfactory epithelium. We found no evidence for the expression of members from the vomeronasal receptor gene families. In the SO, no topography analogous to the receptor expression zones of the main olfactory epithelium was evident. The majority of identified receptors corresponds to genes with restricted expression in the medial and lateral zones of the main olfactory epithelium. This coincides with the expression of olfactory cell adhesion molecule (OCAM) throughout the SO, which is considered as a marker for the medial-lateral zones. In contrast, NADPH:quinone oxidoreductase 1 expression, a characteristic marker for the dorsal zone, was lacking in the SO. Most of the receptor types were found to be expressed in rather few SO neurons; as an exception, the receptor mOR244-3 was observed in a very high proportion of cells. Although a very high fraction of SO neurons expressed mOR244-3, we found no evidence for the coexpression of different receptors in individual cells. Copyright 2004 Wiley-Liss, Inc.

  6. Off-pump atrial septostomy with thoracoscopic scissors under transesophageal echocardiography guidance

    Directory of Open Access Journals (Sweden)

    Raj R Benedict

    2013-01-01

    Full Text Available Selected children with congenital heart defects undergoing palliative closed heart procedures require a cardiopulmonary bypass (CPB run only for the purpose of creating an inter-atrial communication. We report a simple technique of atrial septostomy using thoracoscopy scissors under transesophageal echocardiography guidance without the need for CPB.

  7. Prenatal Alcohol Exposure and Congenital Heart Defects: A Meta-Analysis.

    Science.gov (United States)

    Yang, Jiaomei; Qiu, Huizhen; Qu, Pengfei; Zhang, Ruo; Zeng, Lingxia; Yan, Hong

    2015-01-01

    There are still inconsistent conclusions about the association of prenatal alcohol drinking with congenital heart defects (CHDs). We conducted this meta-analysis to investigate the association between prenatal alcohol exposure and the risk of overall CHDs and the CHDs subtypes. Case-control and cohort studies published before March 2015 were searched through PubMed and Embase. Two authors independently extracted data and scored the study quality according to the Newcastle-0ttawa Scale. The pooled ORs and 95%CI were estimated using the random-effects model and heterogeneity was assessed by the Q test and I2 statistic. A total of 20 studies were finally included. The results provided no evidence of the association between prenatal alcohol exposure and the risk of overall CHDs (OR = 1.06, 95%CI = 0.93-1.22), ventricular septal defects (VSDs) (OR = 1.04, 95%CI = 0.86-1.25), or atrial septal defects (ASDs) (OR = 1.40, 95%CI = 0.88-2.23). However, prenatal alcohol drinking was marginally significantly associated with conotruncal defects (CTDs) (OR = 1.24, 95%CI = 0.97-1.59) and statistically significantly associated with d-Transposition of the Great Arteries (dTGA) (OR = 1.64, 95%CI = 1.17-2.30). Moreover, both prenatal heavy drinking and binge drinking have a strong association with overall CHDs (heavy drinking: OR = 3.76, 95%CI = 1.00-14.10; binge drinking: OR = 2.49, 95%CI = 1.04-5.97), and prenatal moderate drinking has a modest association with CTDs (OR = 1.35, 95%CI = 1.05-1.75) and dTGA (OR = 1.86, 95%CI = 1.09-3.20). In conclusion, the results suggested that prenatal alcohol exposure was not associated with overall CHDs or some subtypes, whereas marginally significant association was found for CTDs and statistically significant association was found for dTGA. Further prospective studies with large population and better designs are needed to explore the association of prenatal alcohol exposure with CHDs including the subtypes in specific groups.

  8. Atrial Fibrillation and Hyperthyroidism

    Directory of Open Access Journals (Sweden)

    Jayaprasad N

    2005-10-01

    Full Text Available Atrial fibrillation occurs in 10 – 15% of patients with hyperthyroidism. Low serum thyrotropin concentration is an independent risk factor for atrial fibrillation. Thyroid hormone contributes to arrythmogenic activity by altering the electrophysiological characteristics of atrial myocytes by shortening the action potential duration, enhancing automaticity and triggered activity in the pulmonary vein cardio myocytes. Hyperthyroidism results in excess mortality from increased incidence of circulatory diseases and dysrhythmias. Incidence of cerebral embolism is more in hyperthyroid patients with atrial fibrillation, especially in the elderly and anti-coagulation is indicated in them. Treatment of hyperthyroidism results in conversion to sinus rhythm in up to two-third of patients. Beta-blockers reduce left ventricular hypertrophy and atrial and ventricular arrhythmias in patients with hyperthyroidism. Treatment of sub clinical hyperthyroidism is controversial. Optimizing dose of thyroxine treatment in those with replacement therapy and beta-blockers is useful in exogenous subclinical hyperthyroidism.

  9. [Atrial fibrillation and stroke].

    Science.gov (United States)

    Aamodt, Anne Hege; Sandset, Per Morten; Atar, Dan; Tveit, Arnljot; Russell, David

    2013-08-06

    More than 70,000 Norwegians have atrial fibrillation, which is a major risk factor for ischemic stroke. A large proportion of ischemic strokes caused by atrial fibrillation could be prevented if patients receive optimal prophylactic treatment. This article describes the risk for ischemic stroke in patients with atrial fibrillation, and discusses who should receive prophylactic treatment and which therapy provides the best prevention. The article is based on recently published European, American and Canadian guidelines, a search in PubMed and the authors' own clinical experience. The new risk score CHA2DS2-VASc is better than the CHADS2 score for identifying patients with atrial fibrillation who have a truly low risk of ischemic stroke and are not in need of antithrombotic treatment. Oral anticoagulation therapy is recommended for patients with two or more risk factors for thromboembolism in addition to atrial fibrillation (CHA2DS2-VASc ≥ 2). Patients with atrial fibrillation and a single additional risk factor (CHA2DS2-VASc =1) an individual assessment should be made as to who should receive oral anticoagulants, and for patients with CHA2DS2-VASc = 0 antithrombotic treatment is not recommended. New oral anticoagulants are at least as effective as warfarin for preventing ischemic stroke in patients with nonvalvular atrial fibrillation, they carry a lower risk of cerebral haemorrhage, especially intracranial haemorrhage and are more practical in use. Platelet inhibitors have a minimal role in stroke prevention in patients with atrial fibrillation. Risks stratifying patients using the CHA2DS2-VASc score is a better method for assessing which patients with atrial fibrillation who should receive oral anticoagulation. The introduction of new oral anticoagulants will simplify preventive treatment and hopefully lead to a more efficient anticoagulation treatment in a larger number of patients with atrial fibrillation.

  10. Alcohol septal ablation to overcome shock.

    Science.gov (United States)

    Galle, Katalien; De Sutter, Johan; Cornelis, Kristoff

    2010-04-01

    A 69-year-old man, known with hypertrophic obstructive cardiomyopathy (HOCM), was referred to our hospital because of progressive hypoxaemia and sepsis after admission for respiratory infection. Once at the emergency department, cardiopulmonary resuscitation, intubation and mechanical ventilation were necessary. Despite vasopressors and colloids the patient remained haemodynamically unstable. Because of the conviction that the distributive shock, caused by sepsis, was worsened by an associated obstructive shock related to the HOCM, an alcohol septal ablation (ASA) was attempted in these acute circumstances. Immediately after the ASA the gradient over the left ventricular outflow tract disappeared and the mean arterial pressure and oxygenation increased. Despite his cardiovascular recuperation the patient died a couple of days later. Nevertheless we achieved an improvement of the haemodynamic situation of this patient with HOCM by performing an urgent ASA.

  11. What Next After Failed Septal Ventricular Tachycardia Ablation?

    Directory of Open Access Journals (Sweden)

    Laurent Roten, MD

    2012-07-01

    Full Text Available Ablation of ventricular tachycardia (VT by conventional radiofrequency ablation can be impossible if the ventricular wall at the targeted ablation site is very thick, as for example the ventricular septum. We present a case of a patient with incessant, non-sustained slow VT originating from the septal part of the lower outflow tracts. Radiofrequency catheter ablation from both ventricles as well as from the anterior cardiac vein were not successful. Both high power radiofrequency ablation and bipolar radiofrequency ablation neither were successfull. Finally, ethanol ablation of the first septal perforator successfully terminated arrhythmia. We discuss the possibilities to overcome failed conventional radiofrequency VT ablation of a septal focus.

  12. Effects of beta-blockade on atrial and atrioventricular nodal refractoriness, and atrial fibrillatory rate during atrial fibrillation in pigs

    NARCIS (Netherlands)

    van den Berg, MP; van de Ven, LLM; Witting, W; Crijns, JGM; Haaksma, J; Bel, KJ; de Langen, CDJ; Lie, KI

    1997-01-01

    Despite their widespread use in atrial fibrillation, the effects of beta-adrenoceptor blockers on atrial and atrioventricular (AV) nodal refractoriness, and atrial fibrillatory rate during atrial fibrillation have been incompletely characterised. In particular, it is unknown whether additional

  13. Changes in left atrial deformation in hypertrophic cardiomyopathy: Evaluation by vector velocity imaging

    Directory of Open Access Journals (Sweden)

    Hala Mahfouz Badran

    2012-12-01

    Full Text Available Objectives: Hypertrophic cardiomyopathy (HCM represents a generalized myopathic process affecting both ventricular and atrial myocardium. We assessed the global and regional left atrial (LA function and its relation to left ventricular (LV mechanics and clinical status in patients with HCM using Vector Velocity Imaging (VVI. Methods: VVI of the LA and LV was acquired from apical four- and two-chamber views of 108 HCM patients (age 40±19years, 56.5% men and 33 healthy subjects, all had normal LV systolic function. The LA subendocardium was traced to obtain atrial volumes, ejection fraction, velocities, and strain (ɛ/strain rate (SR measurements. Results: Left atrial reservoir (ɛsys,SRsys and conduit (early diastolic SRe function were significantly reduced in HCM compared to controls (P-1.8s-1 was 81% sensitive and 30% specific, SRa>-1.5s-1 was 73% sensitive and 40% specific. By multivariate analysis global LVɛsys and LV septal thickness are independent predictors for LAɛsys, while end systolic diameter is the only independent predictor for SRsys, P<.001. Conclusion: Left atrial reservoir and conduit function as measured by VVI were significantly impaired while contractile function was preserved among HCM patients. Left atrial deformation was greatly influenced by LV mechanics and correlated to severity of phenotype.

  14. 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...

  15. Aortic arch obstruction neonates with biventricular physiology: left-open compared to closed inter-atrial communication during primary repair--a retrospective study.

    Science.gov (United States)

    Rüffer, André; Bechtold, Caroline; Purbojo, Ariawan; Toka, Okan; Glöckler, Martin; Dittrich, Sven; Cesnjevar, Robert Anton

    2015-04-17

    Leaving an inter-atrial communication (IAC) open for left atrial decompression is often recommended in neonates with aortic arch obstruction undergoing primary repair. In this study, outcomes in these patients were compared to those with intact atrial septum after repair. Between 2000 and 2013, 53 consecutive neonates with severe aortic arch obstruction (hypoplasia: n = 45, interruption: n = 8) underwent primary repair from an anterior approach. Median age and weight were 8 days (range: 2-30) and 3.2 kg (range: 2.4-4.4), respectively. Cardiac morphology included a ventricular septal defect (VSD, large: n = 28, small: n = 7), malposition of great arteries (n = 10), and severe left ventricular outflow tract obstruction (LVOTO, n = 10). During corrective surgery IAC was closed (group-I, n = 37) or partially left-open (group-II, n = 16). Primary endpoints were hospital death, and re-intervention (surgery and/or balloon) due to aortic arch re-coarctation or recurrent LVOTO. Statistically significant variables by univariate analysis were incorporated in the corresponding multivariable regression model. Regarding morphological discrepancies more patients in group-II presented with LVOTO (p = 0.05), or the combination of arch hypoplasia, intact ventricular septum and normal ventriculo-arterial connection (p = 0.017). Hospital mortality was 8.1% in group-I and 37.5% in group-II (p = 0.016). Re-intervention was performed in 13 patients (group-I: n = 6 vs. group-II: n = 7) due to aortic arch re-coarctation (n = 12) and/or recurrent LVOTO (n = 3), and resulted in a Kaplan-Meier freedom from re-intervention of 87 ± 6% and 79 ± 8% in group-I, and 64 ± 14% and 64 ± 14% in group-II after 1 and 5 years, respectively (p = 0.016). Multivariate analysis revealed LVOTO as an independent risk factor for hospital death (p = 0.042), whereas both LVOTO and left-open IAC (p = 0.001 and 0.01) were

  16. Atrial mass: a myxoma?

    National Research Council Canada - National Science Library

    Chatzis, Andrew C; Kostopanagiotou, Kostas; Kousi, Theofili; Mitropoulos, Fotios

    2016-01-01

    A middle‐aged woman with a history of resected colorectal cancer and receiving chemotherapy presented with a right atrial mass and the provisional diagnosis of myxoma supported by echocardiography, computed...

  17. Atrial Fibrillation and Stroke

    Science.gov (United States)

    ... faulty heart valves, lung disease, and stimulant or alcohol abuse. Some people will have no identifiable cause for their AF. × Definition Atrial fibrillation (AF) describes the rapid, irregular beating ...

  18. Congenital heart defects and left ventricular non-compaction in males with loss-of-function variants in NONO.

    Science.gov (United States)

    Scott, Daryl A; Hernandez-Garcia, Andres; Azamian, Mahshid S; Jordan, Valerie K; Kim, Bum Jun; Starkovich, Molly; Zhang, Jinglan; Wong, Lee-Jun; Darilek, Sandra A; Breman, Amy M; Yang, Yaping; Lupski, James R; Jiwani, Amyn K; Das, Bibhuti; Lalani, Seema R; Iglesias, Alejandro D; Rosenfeld, Jill A; Xia, Fan

    2017-01-01

    The non-POU domain containing octamer-binding gene (NONO) is located on chromosome Xq13.1 and encodes a member of a small family of RNA-binding and DNA-binding proteins that perform a variety of tasks involved in RNA synthesis, transcriptional regulation and DNA repair. Loss-of-function variants in NONO have been described as a cause of intellectual disability in males but have not been described in association with congenital heart defects or cardiomyopathy. In this article, we seek to further define the phenotypic consequences of NONO depletion in human subjects. We searched a clinical database of over 6000 individuals referred for exome sequencing and over 60 000 individuals referred for CNV analysis. We identified two males with atrial and ventricular septal defects, left ventricular non-compaction (LVNC), developmental delay and intellectual disability, who harboured de novo, loss-of-function variants in NONO. We also identified a male infant with developmental delay, congenital brain anomalies and severe LVNC requiring cardiac transplantation, who inherited a single-gene deletion of NONO from his asymptomatic mother. We conclude that in addition to global developmental delay and intellectual disability, males with loss-of-function variants in NONO may also be predisposed to developing congenital heart defects and LVNC with the penetrance of these cardiac-related problems being influenced by genetic, epigenetic, environmental or stochastic factors. Brain imaging of males with NONO deficiency may reveal structural defects with abnormalities of the corpus callosum being the most common. Although dysmorphic features vary between affected individuals, relative macrocephaly is a common feature. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  19. Disease: H00939 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available organ malformation. Patients often have cardiac defects such as atrial septal defects, an increased superfic... Immunol 21:472-80 (2009) PMID:21219176 (description, gene) Klein C Genetic defects in severe congenital neu

  20. Conduction Velocity around the Tricuspid Valve Annulus during Typical Atrial Flutter by Electro-anatomic Mapping System

    Directory of Open Access Journals (Sweden)

    Akira Sawa, MD

    2006-01-01

    Full Text Available Objective: Conduction velocity around the tricuspid valve annulus (TA. during typical atrial flutter (AFL. has been shown to be slowest in the inferior vena cava-tricuspid valve (IVC-TV. isthmus when compared to the septal or free wall segments of the TA. We investigated the conduction velocity in IVC-TV isthmus, dividing into three areas. Methods: We evaluated conduction velocity around the TA during typical AFL in 10 patients, using an electro-anatomic mapping system (CARTO™. Conduction velocity was calculated at six areas around the TA including the septal wall, upper wall, lateral wall, and isthmus wall, which was further divided into three areas, lateral isthmus, mid isthmus, and septal isthmus. Results: Conduction velocity around the TA during typical AFL was slowest in the IVC-TV isthmus. Further, conduction velocities (m/sec. in the mid isthmus (0.44±0.17. and septal isthmus (0.45±0.22. were significantly slower (p < 0.05. than that in the upper wall (0.67±0.26. Conclusions: The relatively slower conduction in IVC-TV isthmus resulted from the relatively slower conduction in the area from mid to septal isthmus.

  1. lutembacher's syndrome: a rare combination of congenital and ...

    African Journals Online (AJOL)

    FOBUR

    ABSTRACT. Lutembacher's syndrome is defined as the rare combination of congenital atrial septal defect and acquired mitral stenosis. The haemodynamic effects of this syndrome are a result of the interplay between the relative effects of the atrial septal defect and mitral stenosis. Mitral stenosis augments the left to right ...

  2. lutembacher's syndrome: a rare combination of congenital and ...

    African Journals Online (AJOL)

    FOBUR

    Because the mitral stenosis was, in fact, rheumatic in aetiology, the syndrome was defined eventually as a combination of congenital atrial septal defect and acquired, almost always rheumatic, mitral stenosis. Keywords:Lutembacher's syndrome, congenital heart disease, valvular heart disease, atrial septal defect,.

  3. Lutembacher's syndrome: A rare combination of congenital and ...

    African Journals Online (AJOL)

    Lutembacher's syndrome is defined as the rare combination of congenital atrial septal defect and acquired mitral stenosis. The haemodynamic effects of this syndrome are a result of the interplay between the relative effects of the atrial septal defect and mitral stenosis. Mitral stenosis augments the left to right shunt through ...

  4. Left atrial appendage occlusion

    Directory of Open Access Journals (Sweden)

    Ahmad Mirdamadi

    2013-01-01

    Full Text Available Left atrial appendage (LAA occlusion is a treatment strategy to prevent blood clot formation in atrial appendage. Although, LAA occlusion usually was done by catheter-based techniques, especially percutaneous trans-luminal mitral commissurotomy (PTMC, it can be done during closed and open mitral valve commissurotomy (CMVC, OMVC and mitral valve replacement (MVR too. Nowadays, PTMC is performed as an optimal management of severe mitral stenosis (MS and many patients currently are treated by PTMC instead of previous surgical methods. One of the most important contraindications of PTMC is presence of clot in LAA. So, each patient who suffers of severe MS is evaluated by Trans-Esophageal Echocardiogram to rule out thrombus in LAA before PTMC. At open heart surgery, replacement of the mitral valve was performed for 49-year-old woman. Also, left atrial appendage occlusion was done during surgery. Immediately after surgery, echocardiography demonstrates an echo imitated the presence of a thrombus in left atrial appendage area, although there was not any evidence of thrombus in pre-pump TEE. We can conclude from this case report that when we suspect of thrombus of left atrial, we should obtain exact history of previous surgery of mitral valve to avoid misdiagnosis clotted LAA, instead of obliterated LAA. Consequently, it can prevent additional evaluations and treatments such as oral anticoagulation and exclusion or postponing surgeries including PTMC.

  5. A Giant Left Atrial Myxoma

    Directory of Open Access Journals (Sweden)

    Medhat F. Zaher

    2014-01-01

    Full Text Available Atrial myxomas are the most common primary cardiac tumors. Patients with left atrial myxomas generally present with mechanical obstruction of blood flow, systemic embolization, and constitutional symptoms. We present a case of an unusually large left atrial myxoma discovered incidentally in a patient with longstanding dyspnea being managed as bronchial asthma.

  6. Morphometric analysis of septal aperture of humerus

    Directory of Open Access Journals (Sweden)

    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.

  7. Improvement in diastolic intraventricular pressure gradients in patients with HOCM after ethanol septal reduction

    Science.gov (United States)

    Rovner, Aleksandr; Smith, Rebecca; Greenberg, Neil L.; Tuzcu, E. Murat; Smedira, Nicholas; Lever, Harry M.; Thomas, James D.; Garcia, Mario J.

    2003-01-01

    We sought to validate measurement of intraventricular pressure gradients (IVPG) and analyze their change in patients with hypertrophic obstructive cardiomyopathy (HOCM) after ethanol septal reduction (ESR). Quantitative analysis of color M-mode Doppler (CMM) images may be used to estimate diastolic IVPG noninvasively. Noninvasive IVPG measurement was validated in 10 patients undergoing surgical myectomy. Echocardiograms were then analyzed in 19 patients at baseline and after ESR. Pulsed Doppler data through the mitral valve and pulmonary venous flow were obtained. CMM was used to obtain the flow propagation velocity (Vp) and to calculate IVPG off-line. Left atrial pressure was estimated with the use of previously validated Doppler equations. Data were compared before and after ESR. CMM-derived IVPG correlated well with invasive measurements obtained before and after surgical myectomy [r = 0.8, P < 0.01, Delta(CMM - invasive IVPG) = 0.09 +/- 0.45 mmHg]. ESR resulted in a decrease of resting LVOT systolic gradient from 62 +/- 10 to 29 +/- 5 mmHg (P < 0.001). There was a significant increase in the Vp and IVPG (from 48 +/- 5to 74 +/- 7 cm/s and from 1.5 +/- 0.2 to 2.6 +/- 0.3 mmHg, respectively, P < 0.001 for both). Estimated left atrial pressure decreased from 16.2 +/- 1.1 to 11.5 +/- 0.9 mmHg (P < 0.001). The increase in IVPG correlated with the reduction in the LVOT gradient (r = 0.6, P < 0.01). Reduction of LVOT obstruction after ESR is associated with an improvement in diastolic suction force. Noninvasive measurements of IVPG may be used as an indicator of diastolic function improvement in HOCM.

  8. Blunt traumatic left atrial appendage rupture and cardiac herniation.

    Science.gov (United States)

    Nhan, Nguyen Huu; Anh, Pham Tho Tuan; Trung, Tran Minh; Pezzella, A Thomas

    2014-06-01

    A 42-year-old man sustained blunt thoracic trauma after a motor vehicle accident. He underwent an urgent operation. Operative findings included a large hematoma, a 4-cm tear in the left atrial appendage, and a long pleuropericardial rupture along the right phrenic nerve. We repaired the left atrial appendage without cardiopulmonary bypass, and closed the pericardial defect primarily. The patient recovered fully and was discharged on the 6th postoperative day. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  9. [Classification of congenital ventricular defects using echocardiography for transcatheter closure].

    Science.gov (United States)

    Lai, Yu-qiong; Huang, Xin-sheng

    2009-02-01

    To determine the anatomical variation and classification of ventricular septal defect (VSD) using echocardiography for percutaneous catheter closure in eligible cases. The isolated ventricular septal defect was diagnosed with echocardiography in 240 patients , and 167 patients screened by transthoracic echocardiography were suitable for percutaneous catheter closure, but only 62 with isolated perimembranous VSD voluntarily received the procedure. The procedure was successful in 58 patients, with a success rate of 93.5% with Amplatzer device. The diameter of VSD ranged from 2.4 to 13.9 (mean 5.3-/+2.0) mm with echocardiography, and the size of Amplatzer device ranged from 4-18 (mean 8.3-/+2.9) mm. Perimembranous ventricular septal defect was complicated by aneurysm formation in 22 patients. Residual trivial or mild shunt was seen in seven (12%) patients at 24 h and one (1.7%) patient at 3 months. Seven (12.1%) patients developed heart block, 3 (5.2%) had intermittence and transient complete heart block, and one had transient second degree atrioventricular block disappearing in 3 to 10 days, and 3 (5.2%) patients had complete right bundle branch block lasting for one month. None of the patients developed significant aortic regurgitation (P>0.05), although 22 showed a superior margin of the defect less than 3 mm from the aortic valve. The mean distance from the aortic valve was 3.7-/+2.7 (1.0 to 10.5) mm. No significant mitral and tricuspid regurgitation occurred in these patients. Four patients had unsuccessful procedures. Percutaneous closure with Amplatzer device can be carried out successfully in a majority of suitable defects screened using transthoracic echocardiography. Echocardiography can exactly demonstrate the anatomical variation and classification of ventricular septal defect in adults. Attention should be given to the misdiagnosis by echocardiography of a doubly committed defect as a perimembranous outflow defect. Heart block can be an important

  10. 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.

  11. Cavotricuspid isthmus mapping to assess bidirectional block during common atrial flutter radiofrequency ablation.

    Science.gov (United States)

    Chen, J; de Chillou, C; Basiouny, T; Sadoul, N; Filho, J D; Magnin-Poull, I; Messier, M; Aliot, E

    We sought to compare published methods to an alternative approach ascertaining cavotricuspid isthmus (CTI) block during atrial flutter ablation. In 39 consecutive patients who underwent an atrial flutter ablation procedure, a 24-pole mapping catheter was positioned so that 2 adjacent dipoles were bracketing the targeted CTI line of block (LOB), with proximal dipoles lateral to the LOB and distal dipoles in the coronary sinus. Two pacing sites were lateral (positions A and B) and 2 were septal (positions C and D) to the LOB, with locations A and D closest to the LOB. A resulting CTI block was accepted when 3 criteria were fulfilled: (1) complete reversal of the right atrial depolarization on the 24-pole catheter when pacing in the coronary sinus, (2) conduction delays from A to D greater than from B to D, and (3) conduction delays from D to A greater than from C to A. A successful CTI block was obtained in all patients. Before CTI block was obtained, a progressive CTI conduction delay was observed in 11 patients (28.2%). During the procedure, the 3 criteria defined above were either all present or all absent. This study establishes that reversal of the atrial depolarization sequence up to the LOB is a definitive and mandatory criteria of successful atrial flutter ablation.

  12. Major Intrahepatic Veno-Venous Fistula after Fontan Operation Treated by Transcatheter Implantation of Amplatzer Septal Occluder through Internal Jugular Vein

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    Alper Guzeltas

    Full Text Available Abstract Six months after undergoing a Fontan operation, a 7-year-old boy with right atrial isomerism and a single functional ventricle was admitted to our emergency department with cyanosis. Emergency cardiac catheterization revealed a large veno-venous fistula that began in a left hepatic vein, connected to the left accessory hepatic veins, and drained into the common atrium, resulting in desaturation. The fistula was occluded proximally with an Amplatzer septal occluder, with satisfying results; the patient's systemic arterial saturation decreased during his hospital stay. Three weeks after the first intervention, a second procedure was performed to retrieve the first device and to close the fistula distally. Multiple attempts with different types of gooseneck snares and a bioptome catheter failed to retrieve the first device, so a telescopic method was used to re-screw it. Using a Mullins long sheath and delivery sheath, the delivery cable was manipulated to fit into the slot of the end screw, and the cable was rotated gently in a clockwise direction to re-screw the device. Then, another Amplatzer septal occluder was placed at the distal end of the fistula. In conclusion, distal transcatheter occlusion of intrahepatic veno-venous fistulas might lead to better clinical outcomes in selected patients. Amplatzer septal occluder device can be retrieve without any complication within three weeks.

  13. Atrial natriuretic peptide in patients with heart failure and chronic atrial fibrillation : Role of duration of at atrial fibrillation

    NARCIS (Netherlands)

    Van Den Berg, MP; Crijns, HJGM; Van Veldhuisen, DJ; Van Gelder, IC; De Kam, PJ; Lie, KI

    The purpose of this study was to analyze the determinants of atrial natriuretic peptide level in patients with congestive heart failure and atrial fibrillation. In particular, the duration of atrial fibrillation was analyzed because atrial fibrillation per se might have a specific effect on atrial

  14. Assessment of atrial electromechanical delay and left atrial mechanical functions in patients with psoriasis vulgaris.

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    Aksan, Gökhan; Nar, Gökay; Soylu, Korhan; İnci, Sinan; Yuksel, Serkan; Ocal, Hande Serra; Yuksel, Esra Pancar; Gulel, Okan

    2015-04-01

    Increased frequency of atrial fibrillation (AF) has been demonstrated in psoriasis cases. Prolongation of the duration of atrial electromechanical delay (AEMD) is a well-known characteristic of the atrium, which is vulnerable to AF. In the current study, our aims are to investigate AEMD durations and mechanical functions of the left atrium (LA) in patients with psoriasis. A total of 90 patients, 45 with psoriasis vulgaris and 45 as the control group, were included in the study. Atrial electromechanical coupling (PA) and intra- and inter-atrial electromechanical delay (IA-AEMD) were measured with tissue Doppler echocardiography. P-wave dispersion (PWD) was calculated from the 12-lead electrocardiogram. The severity of the disease was evaluated by the Psoriasis Area and Severity Index. The durations of PA lateral and PA septal were significantly high in the psoriasis group when compared with the control group (47.7 ± 9.8 vs. 57.1 ± 8.4 msec, P < 0.001 and 38.6 ± 9.9 vs. 43.6 ± 8 msec, P = 0.016, respectively). The durations of IA-AEMD, intra-right electromechanical delay, and intra-left electromechanical delay in the psoriasis group were significantly prolonged compared with the control group (15.2 ± 4.1 vs. 21.7 ± 5.6 msec, P < 0.001; 6 ± 2.5 vs. 8.7 ± 2.7 msec, P < 0.001; and 9.1 ± 3.9 vs. 13.5 ± 5.2 msec, P < 0.001; respectively). PWD was significantly higher in patients with psoriasis vulgaris compared with controls (36.1 ± 7.9 vs. 40.2 ± 9.1 msec, P = 0.043). In the present study, we found prolongation in the durations of AEMD and PWD in the psoriasis group compared with the control group. These results might be an early predictor of AF and other arrhythmias. © 2014, Wiley Periodicals, Inc.

  15. Pharmacological Treatment for Atrial Fibrillation

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    Kaoru Sugi, MD PhD

    2005-01-01

    Full Text Available Pharmacological treatment for atrial fibrillation has a variety of purposes, such as pharmacological defibrillation, maintenance of sinus rhythm, heart rate control to prevent congestive heart failure and prevention of both cerebral infarction and atrial remodeling. Sodium channel blockers are superior to potassium channel blockers for atrial defibrillation, while both sodium and potassium channel blockers are effective in the maintenance of sinus rhythm. In general, digitalis or Ca antagonists are used to control heart rate during atrial fibrillation to prevent congestive heart failure, while amiodarone or bepridil also reduce heart rates during atrial fibrillation. Anticoagulant therapy with warfarin is recommended to prevent cerebral infarction and angiotensin converting enzyme antagonists or angiotensin II receptor blockers are also used to prevent atrial remodeling. One should select appropriate drugs for treatment of atrial fibrillation according to the patient's condition.

  16. Volume of the human septal forebrain region is a predictor of source memory accuracy.

    Science.gov (United States)

    Butler, Tracy; Blackmon, Karen; Zaborszky, Laszlo; Wang, Xiuyuan; DuBois, Jonathan; Carlson, Chad; Barr, William B; French, Jacqueline; Devinsky, Orrin; Kuzniecky, Ruben; Halgren, Eric; Thesen, Thomas

    2012-01-01

    Septal nuclei, components of basal forebrain, are strongly and reciprocally connected with hippocampus, and have been shown in animals to play a critical role in memory. In humans, the septal forebrain has received little attention. To examine the role of human septal forebrain in memory, we acquired high-resolution magnetic resonance imaging scans from 25 healthy subjects and calculated septal forebrain volume using recently developed probabilistic cytoarchitectonic maps. We indexed memory with the California Verbal Learning Test-II. Linear regression showed that bilateral septal forebrain volume was a significant positive predictor of recognition memory accuracy. More specifically, larger septal forebrain volume was associated with the ability to recall item source/context accuracy. Results indicate specific involvement of septal forebrain in human source memory, and recall the need for additional research into the role of septal nuclei in memory and other impairments associated with human diseases.

  17. Risk of congenital heart defects in the offspring of smoking mothers: a population-based study.

    Science.gov (United States)

    Sullivan, Patrick M; Dervan, Leslie A; Reiger, Sheridan; Buddhe, Sujatha; Schwartz, Stephen M

    2015-04-01

    To conduct a population-based study examining the occurrence of congenital heart defects (CHDs) in relation to maternal smoking during the first trimester of pregnancy. This retrospective case-control study used Washington State birth certificates from 1989 to 2011 and linked hospital discharge International Classification of Diseases, 9th revision, codes to identify singleton nonsyndromic CHD cases and determine maternal prenatal smoking status. We calculated ORs from multivariate logistic regression models to compare maternal first-trimester smoking status (any and daily number of cigarettes) among 14,128 cases, both overall and by phenotype, and 60,938 randomly selected controls frequency matched on birth year. Offspring of mothers reporting cigarette use in the first trimester of pregnancy were more likely to be born with a CHD (aOR 1.16 [1.08-1.24]) independent of demographic characteristics and other prenatal risk factors for CHDs. Maternal smoking was most strongly associated with pulmonary valve anomalies (aOR 1.48 [95% CI: 1.15-1.90]), pulmonary artery anomalies (aOR 1.71 [1.40-2.09]), and isolated atrial septal defects (aOR 1.22 [1.08-1.38]). The association between maternal smoking and CHDs was stronger with increasing number of daily cigarettes and among older (35+ years) mothers compared with younger mothers. We provide evidence that maternal smoking during pregnancy is a risk factor for select CHD phenotypes. Maternal smoking may account for 1.4% of all CHDs. New findings include a strong dose-dependence of the association and augmented risk in older mothers. Copyright © 2015 Elsevier Inc. All rights reserved.

  18. Assessment of left atrial mechanical functions in thyroid dysfunction.

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    Karabag, Turgut; Dogan, Sait M; Bayraktaroğlu, Taner; Sayin, Muhammet R; Buyukuysal, Cagatay; Akpinar, Ibrahim; Aydin, Mustafa

    2013-01-01

     Thyroid hormone deficiency can lead to the impairment of cardiac function.  The aim of the study was to determine the left atrial (LA) mechanical function in patients with subclinical hypothyroidism (SHT) and overt hypothyroidism (OHT) and investigate associations of LA mechanical function with diastolic function.   Twenty‑six patients with newly diagnosed SHT (mean age, 42.2 ±12.5 years), 21 patients with OHT (40.2 ±8.5 years) and 28 healthy volunteers (42.4 ±11.2 years) were enrolled in this study. Patients were evaluated by standard M‑mode echocardiographic measurements, mitral Doppler flow analysis, and tissue Doppler parameters at the lateral, septal, and right ventricular annuli. LA volumes were measured using the disc method, and the parameters of LA mechanical function were calculated.   The active emptying volume (AEV) and active emptying fraction (AEF) were significantly higher in the OHT and SHT groups compared with controls. The passive emptying volume and passive emptying fraction were lower in the OHT and SHT groups compared with controls, but the differences were not significant. The conduit volume and the E/A ratio were significantly lower in the OHT and SHT groups compared with controls. The lateral and septal E/Em were significantly higher in the OHT and SHT groups than in the control group, but the septal Em/Am was significantly lower. Diastolic function parameters showed significant associations with AEV and AEF.   LA mechanical function is impaired in patients with thyroid dysfunction. Our findings suggest that this impairment is secondary to that of the left ventricular diastolic function.

  19. Increased amount of atrial fibrosis in patients with atrial fibrillation secondary to mitral valve disease

    NARCIS (Netherlands)

    Geuzebroek, Guillaume S. C.; van Amersfoorth, Shirley C. M.; Hoogendijk, Mark G.; Kelder, Johannes C.; van Hemel, Norbert M.; de Bakker, Jacques M. T.; Coronel, Ruben

    2012-01-01

    Objective: Atrial fibrosis is related to atrial fibrillation but may differ in patients with mitral valve disease or lone atrial fibrillation. Therefore, we studied atrial fibrosis in patients with atrial fibrillation + mitral valve disease or with lone atrial fibrillation and compared it with

  20. Atrial myxoma presenting with orthostatic hypotension in an 84-year-old Hispanic man: a case report

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    Halstead Michael

    2009-12-01

    Full Text Available Abstract Introduction Left atrial myxomas remain the most common benign primary cardiac tumors, and these cardiac growths can masquerade as mitral stenosis, infective endocarditis and collagen vascular disease. Atrial myxomas are found in approximately 14-20% of the population and can lead to embolization, intercardiac obstructions, conduction disturbances and lethal valve obstructions. Case presentation An 84-year-old Hispanic man presented with complaints of dizziness upon standing, and with no prior history of heart murmurs, syncope, shortness of breath, or chest pain. Physical examination revealed evidence of orthostatic hypotension and a soft grade 1/6 systolic murmur at the left sternal border. A transthoracic echocardiogram revealed a large atrial myxoma occupying the majority of the left atrium, with the posterior border of the large atrial mass defined by eccentric mitral regurgitation identified during cardiac catheterization. Left atrial myxoma excision was performed, revealing a 7 × 6.5 × 4.5 cm atrial tumor attached to a 4 × 3 × 2 cm stalk of atrial septal tissue. Conclusion This patient didn't present with the common symptoms associated with an atrial myxoma, which may include chest pain, dyspnea, orthopnea, peripheral embolism or syncope. Two-dimensional echocardiography provides substantial advantages in detecting intracardiac tumors. We recommend a two-dimensional echocardiogram in the workup of orthostatic hypotension of unknown etiology after the common causes such as autonomic disorders, dehydration, and vasodilative dysfunctions have been ruled out. By illustrating this correlation between orthostasis and an atrial myxoma, we hope to facilitate earlier identification of these intracardiac growths.

  1. P-Wave Dispersion and Atrial Electromechanical Delay in Patients with Preeclampsia.

    Science.gov (United States)

    İnci, Sinan; Nar, Gökay; Aksan, Gökhan; Sipahioğlu, Haydar; Soylu, Korhan; Dogan, Ali

    2015-01-01

    To investigate the duration of atrial electromechanical delay (EMD) and left atrial mechanical function in patients with preeclampsia. This study included 26 pregnant women with preeclampsia and 24 age-matched pregnant women without preeclampsia (control group). Atrial electromechanical coupling (PA) and intra-atrial and interatrial EMD were measured using tissue Doppler echocardiography. P-wave dispersion (PWD) was measured via 12-lead electrocardiography. All data were analyzed using SPSS v.15.0 for Windows (SPSS, Inc., Chicago, Ill., USA). Differences in continuous variables between groups were examined using a nonparametric Mann-Whitney U test. Correlation analysis was performed using Spearman's coefficient of correlation. Categorical values were compared using a χ2 test. PA lateral and PA septal durations were significantly longer in the preeclampsia group than in the control group [74.6 ± 8.1 vs. 62.3 ± 5.3 ms (p < 0.001) and 59.7 ± 5.3 vs. 56.2 ± 4.9 ms (p = 0.005), respectively]. The duration of interatrial EMD and intra-atrial EMD in the preeclampsia group was significantly longer than in the control group [25.4 ± 4.6 vs. 13.2 ± 3.9 ms (p < 0.001) and 10.5 ± 1.9 vs. 7.1 ± 1.2 ms (p < 0.001), respectively]. PWD was significantly higher in patients with preeclampsia (43.1 ± 9.1 ms) than in the controls (37.6 ± 7.9 ms; p = 0.008). There was a significant correlation between PWD and interatrial EMD and intra-atrial EMD [r = 0.46 (p < 0.001) and r = 0.39 (p < 0.001), respectively]. The duration of atrial EMD and PWD was prolonged in patients with preeclampsia. © 2015 S. Karger AG, Basel.

  2. INTRACARDIAC ATRIAL DEFIBRILLATION

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    Dosdall, Derek J.; Ideker, Raymond E.

    2007-01-01

    Intravascular ventricular defibrillation and intravascular atrial defibrillation have many similarities, some of which are as follows. An important factor influencing the outcome of the shock is the potential gradient field created throughout the ventricles or the atria by the shock. A minimum potential gradient is required throughout the ventricles and probably the atria to defibrillate. The value of this minimum potential gradient is affected by several factors including the duration, tilt, and number of phases of the waveform. For shock strengths near the defibrillation threshold, earliest activation following failed shocks arises in a region in which the potential gradient is low. The defibrillation threshold energy can be decreased by adding a third and even a fourth defibrillation electrode in regions where the shock potential gradient is low for the shock field created by the first two defibrillation electrodes and giving two sequential shocks, each through a different set of electrodes. However, the addition of more electrodes and sequential shocks complicates both the device and its implantation. Since patients are conscious when the atrial defibrillation shock is given, they experience pain during the shock, which is one of the main drawbacks of intravascular atrial defibrillation. Unfortunately, the pain threshold for defibrillation shocks is so low that a shock of less than 1 Joule is uncomfortable and is not much less painful than shocks several times stronger. Therefore, even though electrode configurations exist that have lower atrial defibrillation threshold energy requirements than the atrial defibrillation threshold with standard defibrillation electrode configurations used in implantable cardioverter/defibrillators (ICDs) for ventricular defibrillation, they are not clinically practical because their shocks are almost as painful as with the standard ICD electrode configurations and they would cause the ICD to be more complicated and to take

  3. Lah is a transmembrane protein and requires Spa10 for stable positioning of Woronin bodies at the septal pore of Aspergillus fumigatus.

    Science.gov (United States)

    Leonhardt, Yannik; Kakoschke, Sara Carina; Wagener, Johannes; Ebel, Frank

    2017-03-10

    Woronin bodies are specialized, fungal-specific organelles that enable an immediate closure of septal pores after injury to protect hyphae from excessive cytoplasmic bleeding. In most Ascomycetes, Woronin bodies are tethered at the septal pore by so-called Lah proteins. Using the pathogenic mold Aspergillus fumigatus as a model organism, we show that the C-terminal 288 amino acids of Lah (LahC288) bind to the rim of the septal pore. LahC288 essentially consists of a membrane spanning region and a putative extracellular domain, which are both required for the targeting to the septum. In an A. fumigatus rho4 deletion mutant that has a severe defect in septum formation, LahC288 is recruited to spot-like structures in or at the lateral membrane. This suggests that LahC is recruited before Rho4 starts to govern the septation process. Accordingly, we found that in wild type hyphae Lah is bound before a cross-wall emerges and thus enables a tethering of Woronin bodies at the site of the newly formed septum. Finally, we identified Spa10, a member of a recently described family of septal pore-associated proteins, as a first protein that directly or indirectly interacts with LahC to allow a stable positioning of Woronin bodies at the mature septum.

  4. Folic acid in pregnant women associated with reduced prevalence of severe congenital heart defects in their children: a national population-based case-control study.

    Science.gov (United States)

    Czeizel, Andrew E; Vereczkey, Attila; Szabó, István

    2015-10-01

    Previous Hungarian intervention trials have shown an association between periconceptional folic-acid-containing multivitamin supplementation and significantly reduced risk of congenital heart defects (CHDs). These findings were confirmed in observational multivitamin studies in the USA, and studies in the Netherlands and China regarding folic acid. The objective of this observational population-based study was to estimate the possible preventive effect of folic acid supplementation for different CHDs during their critical period of development. Evaluation of medically recorded use of folic acid (calculated daily average 5.6mg) during the critical period of development of eight types of CHD (verified through autopsy reports or after catheter examination and/or surgical correction) in the population-based Hungarian Case-Control Surveillance of Congenital Abnormalities (HCCSCA), 1980-1996, containing 22,843 cases with congenital abnormalities and 38,151 population controls without any CHDs, including 5395 matched controls of 3567 live-born cases with various CHDs. A conditional logistic regression model was used to estimate the relative risk/protection [odds ratio (OR) with 95% confidence intervals (CI)] of folic acid in the mothers of cases with various types of CHD and their matched controls. There was a significant decrease in the prevalence of cases with ventricular septal defect (OR 0.57, 95% CI 0.45-0.73), tetralogy of Fallot (OR 0.53, 95% CI 0.17-0.94), d-transposition of great arteries (OR 0.47, 95% CI 0.26-0.86) and atrial septal defect secundum (OR 0.63, 95% CI 0.40-0.98) in infants born to mothers who had taken high doses of folic acid during the critical period of CHD development. The risk of development of certain types of CHD was significantly reduced in pregnant women who were supplemented with folic acid. Thus, CHDs should be included as a secondary assessment in neural-tube-defect preventive programs. Copyright © 2015 Elsevier Ireland Ltd. All rights

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

    Science.gov (United States)

    Karadavut, Yunus; Akyıldız, Ilker; Karadaş, Hatice; Dinç, Aykut Erdem; Tulacı, Gökçe; Tastan, Eren

    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. To evaluate the effectiveness of caudal septal extension graft (CSEG) application in patients who underwent endonasal septoplasty for a short and deviated nasal septum. 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. In the control group, preoperative and postoperative minimal cross-sectional areas (MCA1) were 0.44±0.10cm2 and 0.60±0.11cm2, respectively (pCirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  6. Unilateral atrial fibrillation - how common is atrial divorce?

    Science.gov (United States)

    Ker, J

    2017-06-01

    Atrial fibrillation is the most common pathologic supraventricular tachycardia. It has many causes, is an expensive disease, impairs quality of life and leads to an increased risk of death. Atrial dissociation is characterised by the presence of two independent sets of P-waves. This peculiar abnormality may give rise to the scenario where one atrium is in atrial fibrillation while the other is in sinus rhythm. This is the first published case of atrial dissociation where the phenomenon is demonstrated by transmitral and transtricuspid pulsed wave Doppler.

  7. HYPERTHYROIDISM AND ATRIAL FIBRILLATION

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    I. M. Marusenko

    2017-01-01

    Full Text Available Review on a problem of the development of atrial fibrillation in patients with thyrotoxicosis is presented. Thyrotoxicosis is one of the most frequent endocrine diseases, conceding only to a diabetes mellitus. The most frequent reasons of hyperthyroidism are Graves’ disease and functional thyroid autonomy. The authors give an analysis of data on the cardiac effects of thyrotoxicosis, features of heart remodeling under the influence of thyroid hyperfunction, prevalence of atrial fibrillation in thyrotoxicosis, depending on age, as well as the possibility of restoring sinus rhythm in the combination of these diseases. Particular attention is paid to the effect on the heart of subclinical thyrotoxicosis, which is defined as a dysfunction of the thyroid gland, characterized by low serum concentration of thyrotropin, normal values of free thyroxine and free triiodothyronine. Subclinical hyperthyroidism is also capable of causing heart remodeling and diastolic dysfunction.Prevalence of thyrotoxicosis in elderly people is higher in areas of iodine deficiency; it is relevant for our country due to the large territory of iodine deficiency. In elderly patients, the cardiac effects of thyrotoxicosis prevail in the clinical picture, that makes it difficult to diagnose endocrine disorders, and correction of thyrotoxicosis is critically important for the successful control of the heart rhythm. The article also discusses the problem of thyrotoxic cardiomyopathy, caused by the toxic effect of excess thyroid hormones: features of this heart disorder, factors affecting its formation, clinical significance and contribution to the development of rhythm disturbances. The greatest significance is the development of atrial fibrillation as a result of thyrotox-icosis in older patients who already have various cardiovascular diseases.Atrial fibrillation is the most frequent heart rhythm disorder in thyrotoxicosis. The main cause of arrhythmia in hyperthyroidism is the

  8. Cryoablation of septal accessory pathways in children: midterm results.

    Science.gov (United States)

    Karadeniz, Cem; Akdeniz, Celal; Turan, Ozlem; Tuzcu, Volkan

    2014-09-01

    Radiofrequency (RF) catheter ablation in the septal arrhythmia substrates has an increased risk of irreversible atrioventricular block. Despite its safety profile, several studies reported a lower acute success rate and a higher recurrence rate with cryoablation of septal accessory pathways (APs) when compared to RF ablation. The aim of this study was to assess the efficacy and safety of cryoablation of right septal APs using an electroanatomical mapping system guidance. A total of 43 consecutive patients (13.2 ± 5.5 years) underwent cryoablation for right septal APs. The EnSite system (St. Jude Medical, St. Paul, MN, USA) was used in all procedures. No fluoroscopy was used in 90% of patients (39/43). The mean fluoroscopy time in the remaining four patients was 3.7 ± 0.7 minutes. An electrophysiology catheter with 2-mm distal spacing was used to determine the precise AP location. The majority of the patients (20/43) had anteroseptal, and remaining of the patients had posteroseptal (15/43) and midseptal (8/43) APs. A 6-mm-tip catheter was used in 33 patients and 8-mm-tip catheter was used in nine patients (both catheters were used in one patient). The mean procedure duration and number of complete cryoablation lesions were 181.5 ± 60.6 minutes and 6.1 ± 3.1 minutes, respectively. Acute success was achieved in 40 of 43 patients (93%). No complications were noted. During a mean follow-up of 8.8 ± 4.8 months, five patients (12.5%) experienced recurrence. Our data suggest that cryoablation of septal APs can be performed safely with comparable efficacy to the reported RF ablation results using a limited fluoroscopy approach. ©2014 Wiley Periodicals, Inc.

  9. ECG of the month. Irregular rhythm in a 25-year-old man with three prior cardiac operations. Coarse atrial fibrillation with a rapid ventricular response, left anterior fascicular block, left ventricular hypertrophy with repolarization abnormality.

    Science.gov (United States)

    Glancy, D Luke; Ahmed, Jameel; Ayalloore, Siby G; LeLorier, Paul A; Diwan, Pranav M; Helmcke, Frederick R

    2013-01-01

    The patient underwent closure of an atrial septal defect at age 3, had a leaking "mitral" valve repaired at age 9, and at age 13 had a "mitral" valve replacement. He began taking warfarin sodium at that time and remained symptom-free until 10 days before his initial visit here when he presented to another hospital with dyspnea and palpitations. Treatment there consisted of lisinopril 10 mg qd, carvedilol 6.25 mg bid, aldactone 25 mg qd, furosemide 40 mg qd, digoxin 0.25 mg qd, and a continuation of warfarin sodium 7.5 mg qd. An echocardiogram showed a left ventricular ejection fraction of 20%. After diuresis, he was referred to our cardiology clinic. On his initial visit here, his heart rate was an irregular 120 beats/min, his blood pressure was 106/77 mmHg, and closing and opening snaps of a normally