Isolated infundibuloarterial inversion (S,D,I): a newly recognized form of congenital heart disease.

A newly recognized form of congenital heart disease is presented that is characterized by viscero-atrial situs solitus (S), D-loop ventricles (D), and inverted normally related great arteries (I), the segmental combination being (S,D,I). This anomaly may be called isolated infundibuloarterial inversion because only the subsemilunar infundibulum and the great arteries are inverted, whereas the atrial and the ventricles are not. All three patients had atrioventricular concordance, ventriculoatrial concordance, dextrocardia, superoinferior ventricles, crisscross atrioventricular relations, underdevelopment of the right ventricle, a large ventricular septal defect, and an inverted tetralogy of Fallot type of malformation of the infundibulum and great arteries. The condition known as crisscross atrioventricular relations was found in these three patients to be a major ventricular malposition characterized by marked clockwise rotation of the ventricles, as seen from the front. Two of these three cases were diagnosed accurately and repaired successfully.