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Double outlet left ventricle with intact ventricular septum
International Journal of Cardiology
|February 1, 1984
Summary
This report details two rare cases of double outlet left ventricle, a congenital heart defect causing neonatal cyanosis and outflow tract obstruction. The findings highlight a unique presentation with hypoplastic right ventricles and specific aortic positioning.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Double outlet left ventricle (DOLV) with intact ventricular septum is a rare congenital heart malformation.
- This condition presents significant challenges in diagnosis and management due to complex intracardiac anatomy.
Observation:
- Two pediatric patients presented with neonatal cyanosis and clinical signs of right ventricular outflow tract obstruction.
- Both cases exhibited hypoplastic right ventricles with suprasystemic pressures.
- Key anatomical features included pulmonary to mitral valve continuity and a left-sided aorta.
Findings:
- The reported cases represent the second and third documented instances of this specific variant of double outlet left ventricle.
- The consistent presentation of cyanosis, outflow tract obstruction, and specific ventricular/aortic morphology is noteworthy.
Implications:
- Further documentation of rare congenital heart defects like this DOLV variant is crucial for understanding disease spectrum.
- These cases may inform diagnostic criteria and surgical approaches for similar complex pediatric cardiac malformations.
- Increased awareness can aid in earlier recognition and improved outcomes for affected neonates.