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Dor procedure for pulmonary atresia with intact ventricular septum in an infant
Yoonjin Kang1, Jae Gun Kwak1, Eung Rae Kim1
1Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul, South Korea.
Insights
Infants with pulmonary atresia and right ventricle-dependent coronary circulation face high risks of heart attack and left ventricle aneurysms. Surgical repair, including the Dor procedure and Fontan operation, can be successful for these complex cases.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation is a severe congenital heart defect.
- This condition predisposes patients to myocardial infarction and ischemic complications, such as left ventricle aneurysms.
Observation:
- A case report of an infant diagnosed with pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation.
- The infant presented with a left ventricle apical aneurysm.
Findings:
- The infant successfully underwent the Dor procedure, a surgical technique for ventricular septal defects.
- A subsequent Fontan operation was also successfully performed.
Implications:
- Surgical intervention for postinfarction left ventricle aneurysms in infants with this complex cardiac anatomy is feasible.
- This case highlights the potential for surgical management of ischemic complications in neonates with critical congenital heart disease.
Abstract:
Pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation confers a high risk for myocardial infarction and complications of ischaemia, including left ventricle aneurysms. We describe an infant with pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation and a left ventricle apical aneurysm who successfully underwent the Dor procedure and a subsequent Fontan operation. Surgery for postinfarction left ventricle aneurysm can be considered in infants.
