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Double-outlet right ventricle with intact ventricular septum
Y Ikemoto1, S Nogi, M Teraguchi
1Department of Pediatrics, Kansai Medical University, Osaka, Japan.
Acta Paediatrica Japonica : Overseas Edition
|April 1, 1997
Summary
This case report details a rare form of double-outlet right ventricle (DORV) with intact ventricular septum, diagnosed using magnetic resonance imaging (MRI). Surgical atrial septectomy is recommended over repeated balloon atrial septostomy (BAS) due to the high risk of septal defect closure.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Medical Imaging
Background:
- Double-outlet right ventricle (DORV) with an intact ventricular septum (VS) is a rare congenital heart defect.
- Associated anomalies include hypoplastic left ventricle and mitral valve atresia.
- Accurate diagnosis and management are crucial for patient outcomes.
Observation:
- This report presents the first documented case of this specific DORV variant diagnosed via magnetic resonance imaging (MRI).
- The infant experienced progressive cyanosis, necessitating three balloon atrial septostomy (BAS) procedures by 4 months of age.
- A prominent tendency for functional closure of the atrial septal defect was observed.
Findings:
- The study highlights the diagnostic utility of MRI in complex congenital heart disease.
- Repeated BAS was insufficient due to the restrictive nature and tendency for closure of the atrial septal defect.
- The specific DORV morphology presented challenges in maintaining adequate interatrial communication.
Implications:
- Surgical atrial septectomy is proposed as a superior alternative to repeated BAS in similar DORV cases.
- This approach may prevent recurrent cyanosis and improve long-term management.
- Understanding the pathophysiology of septal defect closure in this condition is vital for surgical planning.