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Double-outlet right ventricle with complete atrioventricular canal
M Imamura1, J J Drummond-Webb, G E Sarris
1Department of Pediatric and Congenital Heart Surgery, The Cleveland Clinic Foundation, Ohio 44195-5066, USA.
The Annals of Thoracic Surgery
|October 13, 1998
Summary
This study details a staged surgical approach for complex congenital heart defects, including double-outlet right ventricle and atrioventricular canal defects. The treatment involved initial repairs followed by a biventricular repair, demonstrating a successful strategy for these challenging conditions.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiovascular Medicine
Background:
- Complex congenital heart disease presents significant surgical challenges.
- Double-outlet right ventricle (DORV) with atrioventricular canal (AVC) defects requires intricate management.
- Staged surgical interventions are often necessary for severe neonatal cardiac anomalies.
Observation:
- A neonate presented with multiple complex cardiac malformations: DORV, unbalanced complete AVC, hypoplastic aortic arch, coarctation of the aorta, and atrioventricular valve regurgitation.
- The patient underwent initial palliative procedures at 4 days of life, including aortic arch repair, atrial septectomy, atrioventricular valve repair, and pulmonary artery banding.
- A definitive biventricular repair was successfully performed at 14 months of age, comprising a two-patch complete AVC repair and arterial switch procedure.
Findings:
- The staged surgical approach successfully addressed the complex anatomy.
- The initial palliative procedures facilitated subsequent biventricular repair.
- The arterial switch procedure and two-patch AVC repair were key components of the definitive repair.
Implications:
- This case highlights the feasibility and effectiveness of staged surgical management for complex DORV with AVC defects.
- The findings support tailored, multi-stage surgical strategies for neonates with severe congenital heart disease.
- Successful biventricular repair in such complex cases offers improved long-term outcomes for affected children.