Outcome in infants with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary
A J Powell1, J E Mayer, P Lang
1Department of Cardiology, Children's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA. powell@cardio.tch.harvard.edu
The American Journal of Cardiology
|November 25, 2000
Insights
Staged surgery for pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation achieved 83% 5-year survival. Early deaths were linked to coronary ischemia in infants under 4 months.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
Background:
- Pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation presents unique surgical challenges.
- Optimal management strategies for this complex congenital heart defect are critical for long-term outcomes.
Purpose of the Study:
- To evaluate the outcomes of staged surgical palliation, specifically Fontan palliation, in patients with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation.
Main Methods:
- A cohort of 12 patients with this specific cardiac anomaly underwent staged surgical management.
- The surgical approach was directed towards achieving Fontan palliation.
Main Results:
- An 83% 5-year actuarial survival rate was observed in the studied cohort.
- The two patient deaths occurred within the first four months of life and were presumed to be related to coronary ischemia.
Conclusions:
- Staged surgery aiming for Fontan palliation offers a viable treatment option for pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation.
- Early identification and management of potential coronary ischemia are crucial for improving survival in neonates with this condition.
Abstract:
Management of all patients with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation (n = 12) with staged surgery directed toward a Fontan palliation resulted in an 83% 5-year actuarial survival. Both deaths in the study were presumably related to coronary ischemia and occurred in the first 4 months of life.
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