Double outlet right atrium with three atrioventricular valves

Area of Science:

• Cardiology
• Congenital Heart Disease
• Cardiac Surgery

Background:

• Atrioventricular (AV) valves are crucial for heart function.
• AV septal defects encompass a range of malformations.
• Rare presentations of AV septal defects require detailed investigation.

Purpose of the Study:

• To describe the unique anatomy and function of a rare AV septal defect.
• To analyze the pathophysiology and hemodynamics of this specific condition.
• To discuss the surgical repair approach for this complex case.

Main Methods:

• Anatomical and functional assessment of AV valves in a patient with AV septal defect.
• Hemodynamic evaluation of cardiac function.
• Review of surgical repair techniques.

Main Results:

• A rare AV septal defect with three AV valves was identified.
• The patient presented with severe subaortic stenosis.
• Despite a large atrioventricular communication, no regurgitation or cyanosis was observed.

Conclusions:

• This case highlights a rare variant of AV septal defects with unusual valve morphology.
• Understanding the pathophysiology is key to managing such complex congenital heart anomalies.
• Surgical repair strategies must be tailored to the specific anatomical abnormalities.