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Double outlet right ventricle associated with common atrioventricular canal: report of nine anatomic specimens
Insights
Double outlet right ventricle with complete atrioventricular canal is a rare congenital heart defect. This study details its unique anatomical features, including leaflet anomalies and ventricular septal defects, in nine pediatric cases.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Anatomy
Background:
- Double outlet right ventricle (DORV) is a rare congenital heart defect.
- Complete atrioventricular (AV) canal is an uncommon co-occurring anomaly.
- Understanding this combined malformation is crucial for diagnosis and management.
Purpose of the Study:
- To describe the specific anatomical characteristics of DORV associated with complete AV canal.
- To analyze variations in conal morphology and septal defects.
- To highlight imaging findings, particularly from radiograms.
Main Methods:
- Retrospective case series analysis.
- Review of nine cases with DORV and complete AV canal.
- Detailed anatomical description based on clinical and imaging data.
Main Results:
- The anterior AV leaflet was undivided and free-floating in all cases.
- Aortic and pulmonary orifices were in the same frontal plane.
- Pulmonary stenosis was present in eight of nine cases.
- Ventricular septal defects extended anteriorly, with mitral-aortic continuity in five cases.
Conclusions:
- DORV with complete AV canal presents unique anatomical features.
- Radiographic findings are key to understanding the ventricular septal defect's extent.
- Accurate anatomical description aids in surgical planning and patient care.
Abstract:
Double outlet right ventricle associated with complete atrioventricular (AV) canal is a rare malformation. Nine cases are reported. In this abnormality the anterior AV leaflet is undivided and free floating and the aortic and pulmonary orifices are in the same frontal plane. In four of the nine cases, the conus was bilateral, but it was slightly hypoplastic in two. In five cases there was no subaortic conus and there was mitral-aortic continuity through the ventricular septal defect. Pulmonary stenosis was present in eight cases. Radiograms may best demonstrate the anatomy of the ventricular septal defect; it extends far anteriorly below the aortic valve, and its inferior rim is deviated by the trabecula septomarginalis.