A histologic study was performed on 22 specimens of pulmonary atresia with ventricular septal defect to 1) ascertain the existence of the main pulmonary artery; 2) distinguish the ductus arteriosus from the systemic collateral arteries (SCA); 3) establish the nutritive or functional nature of collateral circulation; and 4) evaluate the morphology of the distal pulmonary bed. Three cases had abs...

Pulmonary artery agenesis is a rare congenital abnormality in which atresia was encountered in the short segment of the right or left pulmonary arteries. It can be isolated or associated with cardiac abnormalities such as tetralogy of Fallot, septal defects or pulmonary stenosis. The majority of cases are diagnosed in childhood whereas some cases yield no symptoms until adulthood. We evaluated ...

or atresia. Seventy-one per cent of the infant group and 90% of patients over 1 year of age were long-term survivors (up to 6 years). Tetralogy of Fallot was the most commonly encountered anomaly in all 141 patients (66%); transposition of the great vessels and pulmonary stenosis occurred in 18%; and tricuspid atresia with pulmonary stenosis in 10%. The presence of an excessively large shunt an...

Cross sectional echocardiographic studies were assessed prospectively in 58 infants in whom right ventricular outflow obstruction was subsequently shown angiographically. A subcostal cut was used to display simultaneously the short axis of the aortic root and the long axis of the right ventricular outflow tract. This facilitated the differentiation of the common right ventricular outflow tract ...

Congenital left main coronary artery (LMCA) atresia is a rare anomaly and surgical revascularization is recommended once the diagnosis is confirmed. In some cases, LMCA atresia will coexist with other cardiac anomalies, mostly in pediatric patients. A 32-year-old woman had congenital LMCA atresia associated with patent ductus arteriosus (PDA), aortic regurgitation (AR), and moderate pulmonary h...

Pulmonary atresia may occur with a normal or an abnormal aortic root; in either case the presence of a collateral circulation to the lungs is obligatory for survival. This may be via a patent ductus arteriosus, if the main branches of the pulmonary artery are developed, by enlarged bronchial arteries anastomosing with the pulmonary artery branches or supplying the lungs direct, or by means of s...

The case of a 54-year-old housewife, oldest published survivor with pulmonary atresia and a ventricular septal defect (pseudotruncus arteriosus), is reported. Her remarkably favorable course is likely related to the absence of progressive hemodynamic changes, with moderate pulmonary flow adequate for nearly normal arterial oxygenation yet without increasing pulmonary vascular resistance. This c...