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| Determinants of Outcome after Surgical Treatment of Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries |
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Adriano Carotti1, Sonia B. Albanese1, Sergio Filippelli1, Lucilla Rava'2, Paolo Guccione1, Giacomo Pongiglione1, Roberto M. Di Donato1; 1Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesu' Children's Hospital, Rome, Italy; 2Epidemiology Unit, Bambino Gesu' Children's Hospital, Rome, Italy Objective: Analysis of results and identification of variables influencing surgical outcome in a 15-year series of patients treated for pulmonary atresia, ventricular septal defect, and multiple aorto-pulmonary collateral arteries. Methods: Ninety consecutive patients aged 37±61 months (range: 20 days-35 years) primarily underwent either one-stage complete unifocalization (n=69) or palliation to promote native pulmonary arterial development (n=21), the latter limited to patients with hypoplastic dominant pulmonary arteries. Chromosome 22q11.2 microdeletion occurred in 37% of the cases. Ventricular septal defect closure was accomplished in 70 patients (78%), with a mean postoperative pRV/pLV ratio of 0.48±0.14 (range: 0.2-0.8). End-points of univariate and multivariate analysis were suitability to one-stage unifocalization, suitability to simultaneous VSD closure, postoperative pRV/pLV ratio, and survival. Results: Fourteen-year survival, freedom from conduit reintervention, and freedom from percutaneous intervention on pulmonary arteries were 75%, 46%, and 52% respectively. At a median follow-up of 46 months pRV/pLV ratio did not significantly change compared to early postoperative value (paired t test on 35 observations: early postoperative, 0.51±0.14 vs follow-up, 0.53±0.18: p=ns). Patients with confluent intrapericardial pulmonary arteries had a worse postoperative pRV/pLV ratio after ventricular septal defect closure than those without (p=0.04). Kaplan-Meier survival estimates showed age below 30 days (p=0.0004), weight below 3 kgs (p=0.0004), and 22q11.2 microdeletion (p=0.001) significantly affecting survival. Microdeletion was significantly associated with mortality even at Cox regression model (p=0.006). Finally, simultaneous ventricular septal defect closure at the time of unifocalization significantly correlated with both early (p=0.0013) and overall survival (p=0.013). Conclusion: Results of surgery are satisfactory and durable, in spite of the need of repeated percutaneous or surgical interventions on pulmonary arteries or conduit. Avoiding neonatal age and body weight below 3 kg may increase the chance of positive outcome in terms of survival, as it does the simultaneous closure of the ventricular septal defect, when feasible, whereas presence of intrapericardial pulmonary arteries does not improve the hemodynamic result after ventricular septal defect closure. Finally, chromosome 22q11.2 microdeletion remains a variable significantly affecting survival. Back to 2010 Annual Meeting Back to Program Outline Back to Main Program |
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