Definition and Morphology Pulmonary atresia with intact ventricular septum (PAIVS) was first described by John Hunter in 1783. It is a rare congenital cardiac malformation with considerable morphologic heterogeneity, and until recently, relatively poor outcome. There is complete atresia of…
The aortic arch, branch pulmonary arteries, and the ductus arteriosus have a close spatial relationship to the airways and esophagus. Abnormalities of the position or course of these vascular structures can cause obstruction to the major airways or esophagus by…
The right ventricle (RV) can be hypoplastic in various settings. It can be small in the presence of deficient ventricular or atrioventricular septation, producing so-called left ventricular dominance. The chamber can also be small and incomplete in the setting of…
Introduction Although rare, with an incidence of 8 per 1000 births, congenital heart disease (CHD) has increased in prevalence due to the success of surgical and medical management in childhood. A significant proportion of patients with repaired CHD surviving to…