Anomalies

Ventricular Septal Defect, Eisenmenger Type

A large basilar septal defect, dextroposition of the aortic valve, and a hypoplastic or absent infundibular septum probably are due to hypoplasia or absence of the conal cushions.

Ventricular Septal Defect, Supracristal Type

The supracristal type of ventricular septal defect probably is due to either simple failure of truncal and conal septal fusion or septal malalignment, which prevents fusion.

Tetralogy of Fallot

The primary anomaly in tetralogy of Fallot probably is an anterior displacement to a varying degree of the conal septum, leading to unequal partitioning of the conus and reduction of the right ventricular infundibulum. A large basilar ventricular septal defect and dextroposition of the aortic valve result from failure of the displaced conal septum to participate in closure of the interventricular foramen. Pulmonary vascular hypoplasia probably is a secondary result of diminished forward blood flow. As was mentioned above, tetralogy of Fallot frequently is associated with 22q11 deletion, particularly in the setting of severe pulmonary atresia or when associated with extracardiac anomalies.

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