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Long-term results in neonates with pulmonary atresia and intact ventricular septum
J G Coles1, R M Freedom, N E Lightfoot
1Division of Cardiovascular Surgery and Cardiology, Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Pulmonary atresia with intact ventricular septum patients with ventriculocoronary connections face higher surgical risks. Successful right ventricle decompression improves survival, but is contraindicated with right ventricle-dependent coronary circulation.
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
- Congenital Heart Disease
Background:
- Pulmonary atresia with intact ventricular septum (PA/IVS) is a complex congenital heart defect.
- Ventriculocoronary connections (VCCs) are a significant anomaly in PA/IVS patients.
- Understanding risk factors is crucial for surgical outcomes.
Purpose of the Study:
- To analyze the institutional experience with PA/IVS from 1965-1987.
- To identify risk factors for mortality in PA/IVS patients.
- To evaluate the impact of VCCs on surgical outcomes.
Main Methods:
- Retrospective review of 115 PA/IVS patients.
- Angiographic assessment for VCCs.
- Multivariate analysis of risk factors for postoperative death.
Main Results:
- Early mortality was 27.2% in surgical patients (n=99).
- Actuarial survival was 24.7% at 13 years.
- VCCs, low RV/LV pressure ratio, and lower weight were risk factors for death.
Conclusions:
- Right ventricle decompression improves survival in PA/IVS without complex VCCs.
- Decompression is contraindicated in patients with VCCs and RV-dependent coronary circulation.
- Reoperations are common for long-term survivors.
Abstract:
Our entire institutional experience with pulmonary atresia and intact ventricular septum (1965 through 1987) included 115 patients, 16 of whom died before surgical intervention. Fifty-six percent of surgical patients (n = 99) had angiographic evidence of right ventricle-coronary arterial connections. The early mortality in the surgical group was 27.2%, and the actuarial survival was 24.7% +/- 6% at 13 years postoperatively. Multivariate analysis indicated that the presence of ventriculocoronary connections (p = 0.037), a decreasing ratio between right ventricular and left ventricular pressure at the initial cardiac catheterization (p = 0.007), and lower weight at operation (p = 0.001) were incremental risk factors for postoperative death; the presence of Ebstein's anomaly was an additional risk factor in the overall experience (including patients not surgically treated) (p = 0.01). Nearly all long-term survivors underwent at least one reoperation, including right ventricular outflow tract reconstruction (n = 39) and thromboexclusion of the right ventricle (n = 9). The presence of severe stenosis or interruption of the proximal left anterior descending coronary artery system was a uniformly lethal risk factor for patients undergoing these procedures (p = 0.0003). We conclude that surgical procedures that successfully decompress the right ventricle will usually result in biventricular circulation in and long-term survival of patients with pulmonary atresia with intact ventricular septum not complicated by Ebstein's anomaly or extensive ventriculocoronary connections. Decompression or thromboexclusion of the right ventricle is contraindicated in patients with ventriculocoronary connections and a right ventricle-dependent coronary circulation.