title: Hemodynamic assessment after complete repair of pulmonary atresia with major aortopulmonary collaterals.
abstract: BACKGROUND
  Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex form of congenital heart defect. There are limited data regarding late hemodynamics of patients after repair of PA/VSD/MAPCAs. This study evaluated the hemodynamics of patients who underwent complete repair of PA/VSD/MAPCSs and subsequently returned for a conduit change.
  
  
  METHODS
  This was a retrospective review of 80 children undergoing a right ventricle (RV)-to-pulmonary artery conduit replacement after complete repair of PA/VSD/MAPCAs. All patients underwent preoperative cardiac catheterization to define the cardiac physiology. Patients were an average age of 6.5±1.2 years, and the average interval between complete repair and conduit change was 4.5±1.1 years.
  
  
  RESULTS
  The preoperative cardiac catheterization demonstrated an average RV right peak systolic pressure of 70±22 mm Hg and pulmonary artery pressure of 38±14 mm Hg. This pressure gradient of 32 mm Hg reflects the presence of conduit obstruction. After conduit change, the intraoperative RV systolic pressure was 34±8 mm Hg, similar to 36±9 mm Hg at the conclusion of the previous complete repair. The corresponding RV/aortic pressure ratios were 0.36±0.07 and 0.39±0.09, respectively.
  
  
  CONCLUSIONS
  The data demonstrate that patients who underwent complete repair of PA/VSD/MAPCAs had nearly identical pulmonary artery pressures when they returned for conduit change some 4.5 years later. This finding indicates that the growth and development of the unifocalized pulmonary vascular bed is commensurate with visceral growth. We would hypothesize that complete repair, along with low RV pressures, will confer a long-term survival advantage.
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: V Mohan
authors: VM
authors: Reddy
authors: Lynn
authors: L
authors: Peng
authors: Calvin
authors: C
authors: Kuan
authors: Michal
authors: M
authors: Palmon
authors: Frank L
authors: FL
authors: Hanley
mesh_terms: D000293:Adolescent; D001011:Aorta / Q000503:physiopathology* / Q000601:surgery; D006328:Cardiac Catheterization; D006348:Cardiac Surgical Procedures; D002648:Child; D002675:Child, Preschool; D003097:Collateral Circulation / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics / Q000502:physiology*; D006801:Humans; D007223:Infant; D008297:Male; D011184:Postoperative Period; D011651:Pulmonary Artery / Q000503:physiopathology* / Q000601:surgery; D018633:Pulmonary Atresia / Q000503:physiopathology* / Q000601:surgery; D012189:Retrospective Studies