Double-outlet right ventricle with extremely rare conotruncal morphology

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiac Surgery

Background:

• Double-outlet right ventricle (DORV) is a complex congenital heart defect.
• L-malposition of the great arteries presents unique anatomical challenges.
• False Taussig-Bing heart is an extremely rare variant requiring specialized surgical approaches.

Observation:

• A 1-year-old boy presented with an extremely rare form of DORV.
• The patient exhibited l-malposition of the great arteries with conotruncal features consistent with a false Taussig-Bing heart.
• The complex morphology presented multiple surgical considerations.

Findings:

• Definitive anatomical repair was achieved through an arterial switch operation.
• A baffle was utilized to connect the ventricular septal defect to the pulmonary artery.
• This approach successfully addressed the abnormal great artery connections.

Implications:

• This case highlights the feasibility of anatomical repair in complex DORV variants.
• The described surgical technique offers a potential solution for similar rare congenital heart defects.
• Successful surgical outcomes underscore the importance of tailored approaches in pediatric cardiac surgery.