[Ebstein's "like" anomaly ventricular double inlet. A rare association]

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiac Anatomy

Background:

• The association of univentricular heart with double inlet and an Ebstein's-like anomaly of the common atrioventricular valve is exceptionally rare.
• Understanding such complex cardiac malformations is crucial for accurate diagnosis and management.

Purpose of the Study:

• To describe two unique cases of univentricular heart with double inlet and common atrioventricular valve anomaly.
• To illustrate the utility of the segmental sequential system in diagnosing complex congenital heart disease.

Main Methods:

• Detailed morphological description of two hearts with the specified anomalies.
• Application of the segmental sequential system to determine atrial situs, atrioventricular, and ventriculoarterial connections.

Main Results:

• Both cases presented with atrial situs solitus, univentricular heart, common atrioventricular valve, and double outlet ventricle with crossed great arteries.
• Significant variations in atrioventricular valve morphology and associated anomalies, including infundibular and pulmonary valve stenosis, were observed.

Conclusions:

• The described cases fit within the atrioventricular univentricular connection category using the segmental sequential system.
• These complex anomalies highlight the vast spectrum of congenital heart disease, enhancing pediatric cardiology knowledge.