The criss-cross and superoinferior ventricular heart: an angiocardiographic study

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Embryology

Background:

• Superoinferior and criss-cross atrioventricular (A-V) connections represent rare congenital heart anomalies.
• These conditions are hypothesized to arise from disruptions in ventricular looping during embryonic development.

Purpose of the Study:

• To describe the angiocardiographic features of superoinferior and criss-cross A-V connections.
• To clarify the nature of the "criss-cross" appearance and its underlying A-V connections.
• To explore the embryologic, morphologic, and clinical implications of these cardiac malformations.

Main Methods:

• Retrospective analysis of angiocardiographic data from 11 patients with superoinferior and criss-cross A-V connections.
• Review of previously reported cases to identify associated cardiac anomalies and clinical features.

Main Results:

• The "criss-cross" angiocardiographic appearance is often an illusion, with underlying A-V connections being concordant, discordant, or straddling.
• Associated anomalies frequently include transposition of the great arteries, small right ventricles, and pulmonary outflow tract obstruction.
• Ventricular rotational anomalies and underdeveloped right A-V valve inflow contribute to the perceived criss-cross pattern.

Conclusions:

• Distorted ventricular looping due to postseptation disturbances explains the observed A-V connections.
• These findings challenge existing segmental nomenclatures for congenital heart disease.
• Understanding these anomalies is crucial for embryologic, morphologic, and clinical management.