Abstract
Objective: To assess the long-term outcomes of multistage surgical approach in 18 surviving patients in areas of mortality, requirement for postoperative interventional cardiac catheterization, saturations on air, right ventricular (RV) pressures in relation to the systemic pressures, RV and left ventricular (LV) functions and the adequacy of pulmonary arborisation. Method: Data of all surviving patients who underwent complete repair at Southampton University Hospital were reviewed. Data were obtained using case notes and computer based patient records. No data were gathered by direct patient contacts. LV and RV ejection fractions were measured by cardiac magnetic resonance imaging. Pulmonary arborisation patterns were assessed using cardiac catheter data. Results: A total of 26 patients had undergone complete repair. There were 8 (30.8%) deaths and the 18 survivors constituted the study group. Eleven (61.1%) had undergone three or more surgeries to attain complete repair. Fifteen (83.3%) maintained resting saturations above 80% after surgery. Nine (50%) had undergone three or more catheter interventions after complete repair. Eleven (61.1%) had undergone one or more pulmonary ballooning. Nine (50%) had undergone pulmonary artery (PA) stenting. Four (22.2%) had a RV ejection fraction (EF) 50% of LV pressures. Certain zones of the lungs were significantly under-filled on complete repair. Conclusions : Children with pulmonary atresia, ventricular septal defect and major aorto-pulmonary collaterals can be treated using a staged repair. There is a high mortality (31%) with average functional results. Sri Lanka Journal of Child Health , 2014; 43 (2): 84-87 (Key words: Pulmonary atresia; ventricular septal defect; major aorto-pulmonary collaterals) DOI: http://dx.doi.org/10.4038/sljch.v43i2.7008
Highlights
Pulmonary atresia with ventricular septal defect (VSD) and major aorto-pulmonary collateral arteries (MAPCAs) is an uncommon form of complex congenital heart disease with marked heterogeneity of pulmonary blood supply[1,2,3]
Nine (50%) had undergone three or more catheter interventions after complete repair
The outcome for patients born with pulmonary atresia, VSD, and MAPCAs was extremely poor, with surgically treated patients having a probability of only 20% of being alive at age 30 years[1,2,3,4,5]
Summary
Pulmonary atresia with ventricular septal defect (VSD) and major aorto-pulmonary collateral arteries (MAPCAs) is an uncommon form of complex congenital heart disease with marked heterogeneity of pulmonary blood supply[1,2,3]. According to the natural history of this lesion, an estimated 65% of patients survive to 1 year of age and 50% to 2 years, regardless of surgical intervention[1,2,3,4]. Traditional management has involved staged unifocalization of pulmonary blood supply[1,2,3]. The outcome for patients born with pulmonary atresia, VSD, and MAPCAs was extremely poor, with surgically treated patients having a probability of only 20% of being alive at age 30 years[1,2,3,4,5]. The oldest living person was 26 years while the youngest was 2 years in this series
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