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Definitive repair in patients with pulmonary atresia and intact ventricular septum
A M Billingsley1, H Laks, S W Boyce
1Department of Cardiothoracic Surgery, University of California, Los Angeles Medical Center 90024.
Insights
Biventricular repair is successful for mild/moderate right ventricular hypoplasia in pulmonary atresia with intact ventricular septum. Severe cases benefit from the Fontan procedure, offering improved outcomes for complex congenital heart disease.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiac Surgery
Background:
- Pulmonary atresia with intact ventricular septum (PA/IVS) presents a surgical challenge.
- Right ventricular hypoplasia is a key determinant of surgical strategy in PA/IVS.
Purpose of the Study:
- To evaluate the outcomes of biventricular repair versus Fontan procedure in patients with PA/IVS.
- To determine the impact of right ventricular hypoplasia severity on surgical approach selection.
Main Methods:
- Retrospective review of 22 patients with PA/IVS undergoing definitive repair between 1977-1988.
- Categorization of patients based on right ventricular (RV) hypoplasia severity (mild/moderate vs. severe).
- Analysis of surgical techniques including biventricular repair and Fontan procedures, and associated outcomes.
Main Results:
- Biventricular repair was performed in 15 patients with mild/moderate RV hypoplasia, with one operative death (7%).
- Fontan procedure was performed in 7 patients with severe RV hypoplasia, with two operative deaths (29%).
- Patients with RV-coronary sinusoids showed varied surgical approaches with mixed outcomes.
Conclusions:
- Adequate early palliation enables successful biventricular repair for mild/moderate RV hypoplasia in PA/IVS.
- The Fontan procedure is a viable option for patients with severe RV hypoplasia, improving outcomes in complex congenital heart disease.
Abstract:
Between 1977 and 1988, 22 patients underwent definitive repair for pulmonary atresia with intact ventricular septum. Fifteen underwent biventricular repair (mean age 24 months). All had mild to moderate right ventricular hypoplasia at the time of definitive repair. Repair consisted of closure of the atrial septal defect with enlargement of the right ventricular cavity and outflow tract with a patch in eight, insertion of a valved homograft in three, and superior vena cava-pulmonary artery connection in four. There was one operative death (7%). Seven patients had severe right ventricular hypoplasia at the time of definitive repair and underwent a Fontan procedure (mean age 46 months). In two a valved connection was made to the right ventricle and in five a right atrial-pulmonary artery nonvalved connection. There were two operative deaths (29%). Three patients had right ventricular-coronary sinusoids: Two of them underwent a Fontan procedure and one a biventricular repair. We conclude that with adequate early palliation a biventricular repair may be successfully performed for patients with mild or moderate right ventricular hypoplasia, and the Fontan procedure may be used for those with severe right ventricular hypoplasia.