title: Surgical reconstruction of pulmonary stenosis with ventricular septal defect and major aortopulmonary collaterals.
abstract: BACKGROUND
  Pulmonary stenosis with ventricular septal defect and major aortopulmonary collaterals (PS/VSD/MAPCAs) is an extremely rare form of congenital heart defect. Although it has been assumed that PS/VSD/MAPCAs would be similar to pulmonary atresia (PA) with VSD/MAPCA, there are currently no data to support this conjecture. This study reviewed our surgical experience with reconstruction of PS/VSD/MAPCA.
  
  
  METHODS
  This was a retrospective review of 25 patients (14 boys, 11 girls) who were born with PS/VSD/MAPCA and underwent surgical reconstruction. Preoperative pulmonary angiography was used to define the central branch pulmonary arteries and MAPCA. Patients were a median age of 4 months at the first operation.
  
  
  RESULTS
  There was one operative death (4%) in this cohort of 25 patients, and complete repair was achieved in the 24 survivors (96%). There were two distinct subgroups of patients: 11 demonstrated cyanosis in the neonatal timeframe and underwent an initial procedure to augment pulmonary blood flow (+PBF). The remaining 14 patients formed the second group (-PBF). The median age at the first operation was 0.8 months in the +PBF group and 5.2 months in the -PBF group (p<0.005). Complete repair was achieved in 91% of patients in the +PBF group and in 100% in the -PBF group; however, the average number of procedures to achieve complete repair was 2.8 in the +PBF group vs 1.0 in the -PBF group (p<0.005).
  
  
  CONCLUSIONS
  Outcomes for PS/VSD/MAPCAs as a whole were excellent, with a low surgical mortality and high rate of complete repair. There were two identifiable subgroups with distinctive differences required in their surgical management. These results provide a prognostic outlook for patients with PS/VSD/MAPCAs that can be compared and contrasted with PA/VSD/MAPCAs.
authors: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital/Stanford University, Stanford, California 94305, USA. mainwaring@stanford.edu
authors: Richard D
authors: RD
authors: Mainwaring
authors: Rajesh
authors: R
authors: Punn
authors: V Mohan
authors: VM
authors: Reddy
authors: Frank L
authors: FL
authors: Hanley
mesh_terms: D000792:Angiography; D006348:Cardiac Surgical Procedures / Q000379:methods*; D003097:Collateral Circulation; D005260:Female; D005500:Follow-Up Studies; D006345:Heart Septal Defects, Ventricular / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D011666:Pulmonary Valve Stenosis / Q000151:congenital / Q000000981:diagnostic imaging / Q000601:surgery*; D019651:Plastic Surgery Procedures / Q000379:methods*; D012189:Retrospective Studies