Complete atrioventricular septal defect: anatomy, pathology and surgical timing

Complete atrioventricular septal defect is a congenital heart disease characterized by an ostium primum atrial septal defect, a common atrioventricular valve and a variable degree of posterior ventricular septum defect. Complete atrioventricular septal defect accounts for about 3% of congenital heart diseases and occurs in two out of every 10.000 live births. It is closely associated with Down syndrome and 40-45% of patients have Down syndrome. According to Rastelli's classification, three types of complete atrioventricular septal defect have been described (Rastelli type A, B, and C) based on the morphology of the superior bridging leaflet of the common atrioventricular valve. The atrial and ventricular defects in complete atrioventricular septal defect lead to a high pressure and volume load in right ventricle, resulting in the development of pulmonary hypertension and congestive heart failure in these patients. Congestive heart failure symptoms appear in early infancy The diagnosis and the evaluation of anatomical details are performed by echocardiographic examination. Over time, pulmonary hypertension becomes irreversible, thus precluding the surgical therapy. Medical treatment plays only a minor role and the goal of the medical treatment is to maintain the condition of the patients in a good level until surgery. The surgery should be performed before the development of pulmonary vascular disease and the first 3-6 months is generally preferred

Transcatheter closure of ventricular septal defect with two different devices

Transcatheter closure of a multi-hole perimembranous ventricular septal defect with an aneurysm is challenging. Specific ventricular septal defect closure devices have been developed, but some occluders are reportedly used in an off-label manner. This report describes a child who had a multi-hole perimembranous ventricular septal defect with an aneurysm and underwent successful transcatheter closure using two different ocduders: the Occlutech Duct Occluder (Occlutech, Helsingborg, Sweden) and the Amplatzer Duct Occluder II (St. Jude Medical, Saint Paul, Minnesota, United States of America). Transcatheter dosure of a multi-hole perimembranous ventricular septa! defect with an aneurysm using these two different devices can be performed safely by an experienced interventionist in selected patients

Incidentally detected small fistula flows in pulmonary artery by colour Doppler: echocardiographic findings and follow-up results

Abnormal small fistulous flows in the pulmonary artery were detected on routine transthoracic echocardiography in asymptomatic patients by colour Doppler echocardiography. The most likely diagnosis is small coronary artery-pulmonary artery fistulas. We evaluated the clinical, echocardiographic, and follow-up findings of 101 patients. The mean age at first echocardiographic evaluation was 4.3 +/- 4.2 years. In 79 (78.2%) of the patients, fistula flow in the pulmonary artery was diagnosed at the first presentation and the remaining 22 patients (21.8%) were diagnosed between the 2nd and 10th examination. The echocardiography indication was cardiac murmur in 42 (41.6%), routine cardiac control in 30 (29.7%), additional CHD in 14 (13.8%), non-specific chest pain in 11 (10.9%), suspicion of inflammatory heart disease in 2 (2%), and syncope in 2 (2%) patients. In 70 (69.3%) patients, fistulous flow was located in the anterior aspect of the main pulmonary artery, in 23 (22.8%) patients on the aortic side of the pulmonary artery and in 8 (7.9%) patients on the right pulmonary artery. Additional cardiac anomalies were ventricular septal defect in 8, patent ductus arteriosus in 6, atrial septal defect in 5, mitral valve prolapse in 4, coarctation of aorta in 4, bicuspid aortic valve in 3, and Kawasaki syndrome in 1 patient. Sixty-four patients (63.3%) were followed during a mean of 52.6 +/- 43.7 months. Spontaneous closure was detected in only three patients; the others remained almost unchanged during the follow-up. Since the fistulas are thin and hemodynamically insignificant, echocardiography is an appropriate method to monitor these patients without performing any invasive diagnostic procedures for the fistula source

Transcatheter correction of Scimitar syndrome: occlusion of abnormal pulmonary venous drainage and vascular supply in an infant

Treatment of Scimitar syndrome is usually surgical; however, if there is "dual drainage" - that is, one to the inferior caval vein and the other to the left atrium - it is possible to successfully treat this anomaly via a less-invasive transcatheter approach. We report a case of Scimitar syndrome in a 21-month-old, male infant successfully treated with transcatheter embolisation

Anomalous left coronary artery from pulmonary artery (ALCAPA) in asymptomatic 11-year-old case

Origin of the left coronary artery from the pulmonary trunk [Anomalous origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA)] is a rare congenital heart defect. ALCAPA is one of the common causes of myocardial ischemia or infarction in infants and children. In this report, we present an eleven-years-old patient presenting with murmur and accompanied by echocardiographic findings of dilated cardiomyopathy and angiocardiographic investigation that revealed left coronary artery originating from the pulmonary artery. (Turk Arch Ped 2012; 47: 298-301

Transcatheter correction of Scimitar syndrome: occlusion of abnormal pulmonary venous drainage and vascular supply in an infant

Treatment of Scimitar syndrome is usually surgical; however, if there is "dual drainage" - that is, one to the inferior caval vein and the other to the left atrium - it is possible to successfully treat this anomaly via a less-invasive transcatheter approach. We report a case of Scimitar syndrome in a 21-month-old, male infant successfully treated with transcatheter embolisation

Acquired origin of the left anterior descending coronary artery from the pulmonary artery: A complication of the arterial switch operation

The prevalence of coronary anomalies in the transposition of the great arteries is high. Transfer of the coronary arteries during arterial switch operation is the principle step and incomplete transport of the coronary arteries to the neoaortic root results into iatrogenic coronary problems. We present a case with the residual left anterior descending coronary artery originating from the pulmonary artery as a complication of the failure of transfer during the arterial switch operation

Computational Analysis of Wall Shear Stress Patterns on Calcified and Bicuspid Aortic Valves : Focus on Radial and Coaptation Patterns

Calcification and bicuspid valve formation are important aortic valve disorders that disturb the hemodynamics and the valve function. The detailed analysis of aortic valve hemodynamics would lead to a better understanding of the disease’s etiology. We computationally modeled the aortic valve using simplified three-dimensional geometry and inlet velocity conditions obtained via echocardiography. We examined various calcification severities and bicuspid valve formation. Fluid-structure interaction (FSI) analyses were adapted using ANSYS Workbench to incorporate both flow dynamics and leaflet deformation accurately. Simulation results were validated by comparing leaflet movements in B-mode echo recordings. Results indicate that the biomechanical environment is significantly changed for calcified and bicuspid valves. High flow jet velocities are observed in the calcified valves which results in high transvalvular pressure difference (TPG). Wall shear stresses (WSS) increased with the calcification on both fibrosa (aorta side) and ventricularis (left ventricle side) surfaces of the leaflet. The WSS distribution is regular on the ventricularis, as the WSS values proportionally increase from the base to the tip of the leaflet. However, WSS patterns are spatially complex on the fibrosa side. Low WSS levels and spatially complex WSS patterns on the fibrosa side are considered as promoting factors for further calcification and valvular diseasesQatar National Research Fun