OBJECTIVES This study summarized patterns of presentation and attrition in complex pulmonary atresia. BACKGROUND Assessment of the potential impact of surgical strategies for managing complex pulmonary atresia requires information about variability in age and physiology at presentation of the condition. METHODS We performed a retrospective review of age at presentation, referral source, pul...

Clinical and pathologic findings of an unusual case of cardiac malformation are presented. The main features were those of atresia of the right atrioventricular valve associated with two distinct atrioventricular orifices connecting the morphologically left atrium to the underlying morphologically left ventricle. Other distinguishing characteristics were ostium secundum atrial septal defect, no...

pulmonary circulation in the newborn with pulmonary atresia. Thorax 32: 129, 1977 40. Haworth SG, Sauer U, Buhlmeyer C, Reid L: Development of the pulmonary circulation in ventricular septal defect: a quantitative structural study. Am J Cardiol 40: 781, 1977 41. Davis GD, Fulton RE, Ritter DG, Mair DD, McGoon DC: Congenital pulmonary atresia with ventricular septal defect: angiographic and surg...

Pulmonary agenesis is an uncommon anomaly that has been reported in isolation and in association with other congenital defects. Such defects include oesophageal atresia, cardiac malformation, horseshoe kidney, and anal atresia. Over a period of three years we have seen five neonates or fetuses with unilateral agenesis of the lung. All the cases had three or more anomalies seen in the VACTERL se...

At birth an infant was found to have an unusual series of abnormalities with a coronary sinus type atrial septal defect complicating pulmonary atresia with an intact ventricular septum and Ebstein's anomaly. The functionally important anomalies were diagnosed by echocardiography and cardiac catheterisation. The coronary sinus defect and Ebstein's anomaly were detected only at necropsy. Although...

BACKGROUND In patients with complex pulmonary stenosis or atresia, a detailed delineation of all sources of pulmonary blood supply is necessary for planning surgical and transcatheter procedures and usually requires diagnostic cardiac catheterization. The goals of this study were to determine whether gadolinium-enhanced 3D magnetic resonance angiography (MRA) can provide a noninvasive alternati...

Tetralogy of Fallot (TOF) is one of the most common congenital heart malformations comprising a ventricular septal defect, right ventricular outflow tract obstruction, right ventricular hypertrophy, and overriding aorta. A rare variant includes pulmonary atresia and major aortopulmonary collateral arteries. Altered hemodynamics within the functional single-ventricle results in turbulent flow an...

OBJECTIVE To describe the morphology of the pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries with and without monosomy 22q11. DESIGN A retrospective analysis of all patients with this congenital heart defect who are being followed at the University Children's Hospital Erlangen. SETTING A tertiary referral centre f...