Decreased left ventricular coronary artery density in pulmonary atresia and intact ventricular septum.

Background: The prognosis for pulmonary atresia and intact ventricular septum (PAIVS) has been poor. Our hypothesis is that intrinsic abnormal left ventricular (LV) intramyocardial circulation might be related to the poor outcomes of these patients.

Methods: Neonatal heart specimens were examined microscopically in four groups of 6 cases each.

A criss-cross heart with twisted atrioventricular connections, "perfect streaming," and double discordance.

At 24 weeks gestational age, a term female infant was diagnosed with complex congenital heart disease. The antenatal cardiac diagnosis was uncertain and included univentricular heart. Following delivery, the child remained well and was normally saturated.

Independent factors associated with mortality, reintervention, and achievement of complete repair in children with pulmonary atresia with ventricular septal defect.

Objectives: We described morphologic characteristics, particularly pulmonary anatomy, and determined the prevalence of definitive end states and their determinants in children with pulmonary atresia associated with ventricular septal defect (PAVSD).

Background: Pulmonary atresia associated with ventricular septal defect represents a broad morphologic spectrum that greatly influences management and outcomes.

Methods: From 1975 to 2004, 220 children with PAVSD presented to our institution.

Outcomes in 45 children with ductal origin of the distal pulmonary artery.

Background: There are no large series describing the morphologic spectrum and the clinical outcomes of children with ductal origin of the distal pulmonary artery (PA).

Methods: Medical records were reviewed for all children presenting between 1970 and 2001. Angiograms were reviewed at presentation and at last available follow-up.