title: Aortic valve disease in pulmonary atresia and major aortopulmonary collaterals.
abstract: BACKGROUND
  Aortic valve disease in association with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals (PA/VSD/MAPCAs) is an extremely rare combination of congenital heart defects. The presence of aortic stenosis or insufficiency or both imposes an additional physiologic burden that complicates the management of PA/VSD/MAPCAs. This report summarizes our experience with 7 patients who underwent surgical repair of this rare combination of defects.
  
  
  METHODS
  This was a retrospective review of patients who had both aortic valve disease and PA/VSD/MAPCAs. Four patients had predominantly aortic stenosis, 2 patients had aortic insufficiency, and 1 had mixed stenosis and insufficiency. Anatomically, this corresponded to a dysplastic trileaflet valve in 4, bicuspid valve in 2, and quadricuspid valve in 1.
  
  
  RESULTS
  Four of the 7 patients underwent aortic valve surgery at the time of their first operation for PA/VSD/MAPCAs. The remaining 3 patients had aortic valve surgery at subsequent operations. All 7 patients are alive, with a median duration of follow-up of 3 years. One patient has required aortic valve replacement 2 years after repair of an insufficient bicuspid valve.
  
  
  CONCLUSIONS
  This manuscript summarizes our experience with 7 patients who had the rare combination of aortic valve disease and PA/VSD/MAPCAs. Although aortic valve disease has been reported for other conotruncal defects, we believe this is the first report of its presence in association with PA/VSD/MAPCAs.
authors: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital, Stanford University, Stanford, California, USA. mainwaring@stanford.edu
authors: Richard D
authors: RD
authors: Mainwaring
authors: George
authors: G
authors: Dimeling
authors: Rajesh
authors: R
authors: Punn
authors: Frank L
authors: FL
authors: Hanley
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000601:surgery*; D000293:Adolescent; D001021:Aortic Valve / Q000601:surgery; D000082882:Bicuspid Aortic Valve Disease; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000379:methods*; D002648:Child; D003097:Collateral Circulation / Q000502:physiology*; D015150:Echocardiography, Doppler / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery*; D006349:Heart Valve Diseases / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D021621:Imaging, Three-Dimensional / Q000379:methods; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011300:Preoperative Care / Q000379:methods; D018633:Pulmonary Atresia / Q000175:diagnosis / Q000601:surgery*; D035583:Rare Diseases; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D013997:Time Factors; D016896:Treatment Outcome