Atrioventricular Cushion Defects Research Articles

Hypoplastic right heart syndrome

Congenital heart diseases (CHDs) are of two types-acyanotic and cyanotic. Acyanotic heart diseases include atrial septal defect ASD, ventricular septal defect VSD, patent ductus arteriosus PDA, coarctation of aorta and atrioventricular cushion defects. Cyanotic heart disease includes Fallot’s tetralogy, transposition of the great vessels, Total anomalous pulmonary venous return, Tricuspid atresia and single ventricle or hypoplastic left heart or hypoplastic right heart syndrome (HRHS). Hypoplastic right heart is a rarer condition than the hypoplastic left heart. Our patient had a cyanotic heart disease; presented to us with hypoplastic right heart, a very rare association consisting of pulmonary atresia combined with a hypoplastic right ventricle and well-developed pulmonary arteries. Early clinical diagnosis and surgical intervention saved this child.

An Unusual Atrial Septal Defect Producing Symptoms in a Young Lady

Atrial septal defect is the most common congenital heart disease among adult population. Secundum variety of atrial septal defect comprises most frequently encountered atrial septal anomaly which presents during late age. Primum atrial septal defect usually associated with atrio-ventricular cushion defect among patients with Down syndrome. Small number of patients may present with fenestrated atrial septal defect. We have presented case of a woman who is young and symptomatic due to atrial septal defect comprising both secundum and primum type.

Role of Semaphorin Signaling During Cardiovascular Development.

Semaphorins are a family of secreted and membrane‐bound molecules that play critical functions in diverse biological processes.[1][1], [2][2], [3][3], [4][4], [5][5], [6][6], [7][7], [8][8], [9][9] SEMA1A/Fasciclin IV is the founding member of the semaphorin family and was first reported in 1992

Echocardiographic Finding of Non-Obstructive Fibromuscular Band in Right Atrium

chambers by a fold of tissue, a membrane or a fibro muscular band., The upper part of the corresponding atrium receives venous blood, whereas the lower portion is in contact with the atrioventricular valve The membrane that separates the atrium into two parts may be a complete imperforated band or may have one or more holes for blood to pass (11-20). Cortriatriatum sinister is a congenital heart defect characterised by a fibro muscular membrane in left atrium. Where as in Cortriatrium dexter Right atrium has fibro muscular bandit is diagnosed in paediatric age group of patients.and adult cases are extremely rare.Symptoms will depend on the type and degree of obstruction like Tricuspid stenosis. The membrane may be complete or may contain one or more opening of varying size. It can be treated surgically and has no medical treatment. Other congenital conditions like tetralogy of Fallot, double outlet right ventricle, coarctation of the aorta, partial anomalous pulmonary venous connection, persistent left superior vena cava with unroofed coronary sinus, ventricular septal defect, atrioventricular cushion defects and common atrioventricular canal., Asplenia or polysplenia have been reported in adults. In adults, cortriatriatum is frequently an isolated finding (Figures 2 and 3) (21-30). Conclusion

Defecto de la tabicación atrioventricular con 2 válvulas asociado a ausencia del pericardio. Informe de un caso

We present a case of a patient with a cardiac malformation that represents a form of embryo-anatomical transition of an atrioventricular septal defect between a 2 valves form to a common valve form. This entity was associated with pericardium absence. Throughout several studies we have precisely established a diagnostic sequence by determining the adequate contribution of each method and we have been able to clear out the proper nomenclature of the atrioventricular cushion defect.

Occurrence and pattern of congenital heart diseases in a rural area of sub-Saharan Africa

The extent of congenital heart disease in Cameroon remains largely unknown. The aim of this study was to determine the occurrence and pattern of congenital heart diseases in the Cardiac Centre of St Elizabeth Catholic General Hospital, situated in a rural area of Cameroon. Between November 2002 and November 2008, a population of 2 123 patients with suspected cardiac pathologies were consulted at St Elizabeth Catholic General Hospital referral cardiac centre. Of these patients, 292 subjects were recruited for the study, based on detection of (1) precordial murmurs and/or cardiomegaly on chest X-ray examination, or (2) congenital heart diseases on transthoracic Doppler echocardiography examination. Congenital heart diseases and inorganic murmurs were found in 95.5 and 4.5% of the patients, respectively. Congenital heart diseases included tetralogy of Fallot (26.1%), isolated ventricular septal defect (38.8%), atrioventricular cushion defect (7.3%), isolated atrial septal defect (2.8%), arterial duct cases (12.4%), common arterial trunk (1.3%), isolated stenosis of the pulmonary artery (2.6%), coarctation of the aorta (1.1%), congenital mitral valve regurgitation (1.2%), atresia of the triscupid valve (1.6%), double-outlet right ventricle (2.1%), anomalous pulmonary venous return (1.5%) and left isomerism (1.2%). Our data show that there is a high occurrence of congenital heart disease in this hospital in a rural zone of sub-Saharan Africa and that isolated ventricular septal defect is the predominant pathology. Post-surgical follow up remains very challenging as many parents cannot afford their children's medical treatment or are generally not well educated.

The Ubiquitin Ligase Nedd4-1 Is Required for Heart Development and Is a Suppressor of Thrombospondin-1

Nedd4 (Nedd4-1) is a Hect domain E3 ubiquitin ligase that also contains a C2 domain and three WW domains. Despite numerous in vitro studies, its biological function in vivo is not well understood. Here we show that disruption of Nedd4-1 in mice (leaving Nedd4-2 intact) caused embryonic lethality at mid gestation, with pronounced heart defects (double-outlet right ventricle and atrioventricular cushion defects) and vasculature abnormalities. Quantitative mass spectrometry and immunoblot analyses of lysates from the wild type and knock-out mouse embryonic fibroblasts to identify Nedd4-1 in vivo targets revealed dramatically increased amounts of thrombospondin-1 (Tsp-1) in the knock-out mouse embryonic fibroblasts and embryos. Tsp-1 is an inhibitor of angiogenesis, and its elevated level was mediated primarily by enhanced transcription. Interestingly, the administration of aspirin (an inhibitor of Tsp-1) to the pregnant heterozygote mothers led to a reduction in Tsp-1 levels and a substantial rescue of the embryonic lethality. These results suggest that Nedd4-1 is a suppressor of Tsp1 and that increased levels of Tsp-1 in the Nedd4-1 knock-out mice may have contributed to the developmental defect observed in the embryos.

Anaesthetic Management of an Adult with a Modified Fontan Procedure in Noncardiac Surgery: A case report

Given improvements in the diagnosis and treatment of congenital heart disease, more patients with heart disease reach adolescence or adulthood and require noncardiac surgery. Since 1971, the Fontan operation has been widely used for the management of tricuspid atresia and a variety of univentricular heart malformations. Although the life expectancy of these patients has increased, few reports are available on their anaesthetic management when a noncardiac operation is indicated. We report on the anaesthetic management of a 40-years-old male patient scheduled for permanent pacemaker insertion who underwent a modified Fontan procedure at the age of 27 years for pulmonary stenosis, common atrioventricular cushion defect, and for corrected transposition of the great arteries

RXR alpha deficiency confers genetic susceptibility for aortic sac, conotruncal, atrioventricular cushion, and ventricular muscle defects in mice.

Retinoid-dependent pathways play a central role in regulating cardiac morphogenesis. Recently, we characterized gene-targeted RXR alpha -/- embryos, which display an atrial-like ventricular phenotype with the development of heart failure and lethality at embryonic day 14.5. To quantitate the frequency and complexity of cardiac morphogenic defects, we now use microdissection and scanning electron microscopy to examine 107 wild-type, heterozygous, and homozygous embryos at embryonic day 13.5, 14.5, and 15.5. RXR alpha -/- embryos display complex defects, including ventricular septal, atrioventricular cushion, and conotruncal ridge defects, with double outlet right ventricle, aorticopulmonary window, and persistent truncus arteriosus. In addition, heterozygous RXR alpha embryos display a predisposition for trabecular and papillary muscle defects, ventricular septal defects, conotruncal ridge defects, atrioventricular cushion defects, and pulmonic stenosis. Lastly, we show that the intermediate anatomic phenotype displayed by heterozygous embryos is mirrored in the molecular marker MLC-2a. The intermediate phenotype of RXR alpha heterozygous embryos documents a gene dosage effect for RXR alpha in maintaining normal cardiac morphogenesis. In addition, some defects in RXR alpha mutant mice are phenocopies of human congenital heart defects, thereby suggesting that a relative deficiency in RXR alpha or molecules downstream in its signaling pathway may represent congenital heart disease-susceptibility genes.

Etiology of right bundle branch block pattern following surgical repair of atrioventricular cushion defects: R. Antar and E. Krongrad. Chest 73:642–646 (May), 1978

Etiology of right bundle branch block pattern following surgical repair of atrioventricular cushion defects: R. Antar and E. Krongrad. Chest 73:642–646 (May), 1978

Etiology of Right Bundle Branch Block Pattern following Surgical Repair of Atrioventricular Cushion Defects

Electrocardiograms and surgical data from 12 patients with complete atrioventricular canal defects and 24 patients with ostium primum defects were evaluated before and after open heart surgery in order to document the incidence of postoperative right bundle branch block. The use and precise location of septal sutures utilized for repair were specifically noted in order to assess their possible role in the genesis of the postoperative right bundle branch block pattern. Septal sutures were placed in all patients with atrioventricular canal defects, and ten (83 percent) of these 12 patients developed right bundle branch block. Seven (29 percent) of the 24 patients with ostium primum defects developed a postoperative right bundle branch block, and in all seven patients, septal sutures were utilized. None of the patients with an ostium primum defect without septal sutures developed a right bundle branch block, and none of the patients in this series developed complete heart block.

Electrocardiographic and vectorcardiographic changes following surgery in persistent common atrioventricular canal

The electrocardiogram and vectorcardiogram are probably the most reliable tools in the diagnosis of atrioventricular cushion defects. Our observations require further evaluation. Evidence has been offered suggesting that the electrocardiogram and vectorcardiogram also may assist in the evaluation of the effectiveness of surgical repair. It appears that there is enough evidence to conclude that the electrocardiographic and vectorcardiographic patterns observed in atrioventricular cushion defects are due not only to a congenital aberration of the left bundle but also to cardiac enlargement.

The electrocardiogram in atrial septal defects and atrioventricular cushion defects

The electrocardiogram in atrial septal defects and atrioventricular cushion defects

The electrocardiogram of patients with atrioventricular cushion defects (defects of the atrioventricular canal) ∗

The electrocardiogram is the most important single item in alerting the physician to the diagnosis of congenital cardiac anomalies stemming from defective formation of the atrioventricular canal. It is characterized by left axis deviation with the early manifest frontal vector characteristically in the 0 ° to −60 ° zone. The tracings usually show evidence of enlargement of the right ventricle, particularly an rSR configuration in right precordial leads, the picture of volume overloading of the right ventricle. Different hemodynamic profiles (for instance, the amount of shunting at the atrial and ventricular levels, the degree of mitral insufficiency and the pulmonary hypertensive level) will be additively reflected in the basic electrocardiographic pattern. The typical electrocardiogram may be encountered occasionally in other types of congenital heart disease, particularly ventricular septal defects located posteriorly under the septal tricuspid leaflet. It is believed that the electrocardiographic pattern is related to a congenital aberration of the left bundle branch system.