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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
PubMed

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.

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