Aortic levopositions without ventricular inversion

Area of Science:

• Cardiology
• Congenital Heart Disease
• Pediatric Cardiology

Background:

• Aortic levoposition, where the aorta is anterior and left of the pulmonary artery without ventricular inversion, is a rare congenital anomaly.
• These cases deviate from the typical 'loop rule' of cardiac development, occurring in approximately 0.9% of reviewed cases.

Purpose of the Study:

• To analyze the anatomical features of aortic levoposition without ventricular inversion.
• To investigate the spectrum of associated cardiac anomalies and their potential syndromic implications.
• To evaluate the surgical relevance and outcomes for this specific condition.

Main Methods:

• Multi-center case series analysis.
• Review of own cases and existing literature.
• Detailed anatomical and correlative anomaly assessment.

Main Results:

• A high incidence of associated anomalies was observed, including malrotations, atrial appendage juxtaposition, atrioventricular valve defects, ventricular septal defects, and pulmonary outflow tract obstructions.
• Absence of the coronary sinus was noted in some cases, independent of asplenia or specific vena cava drainage.
• The coronary artery pattern in anatomically corrected malposition with two ventricles resembled that seen in ventricular inversion.

Conclusions:

• Aortic levoposition without ventricular inversion may represent a distinct syndrome with significant diagnostic and surgical importance.
• Associated anomalies suggest a complex developmental etiology.
• Accurate preoperative evaluation and surgical intervention can improve outcomes for affected individuals.