Gross distortion of atrioventricular and ventriculo-arterial relations associated with left juxtaposition of atrial appendages. Bizarre form of atrioventricular criss-cross.

Two pathological specimens are described in which a bizarre distortion of atrioventricular and ventriculoarterial relations existed. In both cases anterior and leftward displacement of the tricuspid valve coexisted with rightward displacement of the infundibular regions and semilunar valves. Abnormal expansion of the AV canal, possibly secondary to malrotation of the cardiac tube, is considered as a possible cause of the anomaly. One of the hearts showed severe subpulmonary stenosis produced by a valve-like structure which was well differentiated, with a complete annulus and chordae tendineae that inserted in a parachute-like fashion on the infundibular septum. The morphology of this structure was clearly different from that fo the fibrous tags described in other anomalies. Its origin is uncertain. These anomalies have important implications in relation to the nomenclature and classification of congenital heart malformations, and illustrate the value of recent modifications of the segmental approach.