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Predictors for biventricular repair in pulmonary atresia with intact ventricular septum
J Cleuziou1, C Schreiber, A Eicken
1Department of Cardiovascular Surgery, German Heart Center Munich, Munich, Germany. cleuziou@dhm.mhn.de
Insights
Right ventricle decompression in pulmonary atresia with intact ventricular septum (PA-IVS) improves biventricular repair success. Key predictors include RV morphology and absence of coronary fistulae, leading to good long-term survival.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Cardiac Morphology
Background:
- Pulmonary atresia with intact ventricular septum (PA-IVS) presents diverse right heart morphologies.
- Understanding these variations is crucial for surgical planning.
Purpose of the Study:
- To identify predictors for successful biventricular repair in PA-IVS patients.
- To evaluate the impact of initial surgical strategies on repair outcomes.
Main Methods:
- Retrospective review of 86 PA-IVS patients.
- Emphasis on angiographic findings and initial surgical procedures.
- Analysis of right ventricular morphology, tricuspid valve size, and coronary anomalies.
Main Results:
- Biventricular repair was achieved in 65% of patients.
- Right ventricle decompression as an initial procedure was associated with higher biventricular repair rates (P < 0.001).
- Significant predictors for biventricular repair included tripartite right ventricle, larger tricuspid valve size (z-score -3.6), and absence of coronary fistulae.
Conclusions:
- Initial right ventricle decompression enhances the likelihood of biventricular repair in PA-IVS.
- Right ventricular size and lack of coronary fistulae are critical morphological predictors.
- Biventricular repair offers promising long-term survival (80% at 25 years).
Background:
Pulmonary atresia with intact ventricular septum (PA-IVS) is a complex congenital heart defect with a large variety of right heart-sided morphologies.
Methods:
We undertook a retrospective review of 86 patients with PA-IVS with a special emphasis on the angiographic findings. The aim of the study was to determine predictors for biventricular repair. Initial surgical procedures depended on the right ventricular morphology, the tricuspid valve size and coronary anomalies.
Results:
Fifty-five patients (64%) underwent decompression of the right ventricle (RV) as an initial procedure; 16 of them required an additional systemic-to-pulmonary artery shunt. Twenty-six patients (30%) had only a systemic-to-pulmonary artery shunt as their initial procedure. Five patients underwent interventional procedures performed by pediatric cardiologists. Biventricular repair was possible in 56 patients (65%). Univentricular palliation was achieved in 16 patients. Fourteen patients had only palliation with a systemic-to-pulmonary artery shunt. Mean tricuspid valve size was significantly bigger in patients with biventricular repair (z-score -3.6 +/- 2.6) than in patients who did not undergo biventricular repair (-5.2 +/- 1.7, P = 0.003). Predictors for biventricular repair were right ventricular decompression with or without systemic-to-pulmonary artery shunt ( P < 0.001), tripartite right ventricle ( P < 0.001) and the absence of coronary fistulae ( P < 0.001). Long-term survival was 80% +/- 13% at 25 years for patients undergoing biventricular repair.
Conclusions:
Decompression of the RV as an initial surgical procedure improves the possibility of achieving biventricular repair with good long-term results. However, morphological factors such as right ventricular size and the absence of coronary fistulae are significant predictors for biventricular repair.
