Trans-aortic DORV repair.

Area of Science:

• Cardiovascular Surgery
• Congenital Heart Disease
• Pediatric Cardiology

Background:

• Double-outlet right ventricle (DORV) encompasses complex ventriculoarterial connections.
• Existing literature documents trans-aortic correction for related conditions like Tetralogy of Fallot and VSDs without outflow tract obstruction.
• A pure trans-aortic approach for DORV with VSD and right ventricular outflow tract obstruction has not been previously reported.

Observation:

• A 20-year-old male with diagnosed DORV, VSD, and pulmonary stenosis (PS) presented with cyanosis.
• The patient underwent a pure trans-aortic repair procedure.
• This marks the first reported instance of a complete trans-aortic DORV VSD PS repair.

Findings:

• The trans-aortic approach provides excellent visualization of cardiac defects.
• This surgical technique avoids right atriotomy, right ventriculotomy, and potential injury to coronary arteries and the tricuspid valve.
• Patch placement on the left ventricular outflow side may reduce the likelihood of residual shunts post-repair.

Implications:

• The trans-aortic approach is a viable option for selected patients requiring intracardiac repair of complex DORV.
• This method minimizes risks associated with traditional surgical routes, including damage to the aortic cusps and the bundle of His.
• This technique holds promise for improving outcomes in DORV VSD PS surgical management.