title: ALCAPA syndrome: an example of chronic myocardial hypoperfusion?
abstract: OBJECTIVE
  The purpose of this study was to evaluate functional variables and morphologic correlates of chronically hypoperfused myocardium before and after revascularization.
  
  
  BACKGROUND
  Neonates with congenital anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) develop some myocardial necrosis shortly after birth. The survivors of this event are left with a localized infarction and an almost entirely collateral circulation-dependent perfusion of the left ventricle that results in poor global left ventricular function. Survival beyond infancy is uncommon because of severe left heart failure. Revascularization, however, brings about functional recovery with good clinical outcome. The ALCAPA syndrome is thus characterized by chronic collateral circulation-dependent low perfusion, low contraction matching and potential revivability.
  
  
  METHODS
  Five patients with ALCAPA syndrome are presented, with preoperative and postoperative clinical findings and histologic data obtained from intraoperative transmural biopsy specimens.
  
  
  RESULTS
  The angiographically assessed preoperative ejection fraction was 33 +/- 19% (mean +/- SD). Postoperative echocardiographic follow-up revealed normal left ventricular function in all patients. Histologic study of the biopsy specimens taken from the region perfused by the anomalous artery showed a variable degree of fibrosis (51 +/- 32%). The ultrastructure of the remaining myocytes revealed viable characteristics, but a substantial percent (46 +/- 26%) showed a markedly reduced fraction of contractile material.
  
  
  CONCLUSIONS
  These ultrastructural studies suggest delayed subcellular adaptive responses in the chronically hypoperfused myocardium of patients with ALCAPA syndrome.
authors.affiliation: Department of Cardiac Surgery, Katholieke Universiteit Leuven, Belgium.
authors.forename: B
authors.initials: B
authors.lastname: Shivalkar
authors.forename: M
authors.initials: M
authors.lastname: Borgers
authors.forename: W
authors.initials: W
authors.lastname: Daenen
authors.forename: M
authors.initials: M
authors.lastname: Gewillig
authors.forename: W
authors.initials: W
authors.lastname: Flameng
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003097:Collateral Circulation / Q000502:physiology; D003330:Coronary Vessel Anomalies / Q000473:pathology* / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D007223:Infant; D008854:Microscopy, Electron; D017202:Myocardial Ischemia / Q000503:physiopathology*; D009206:Myocardium / Q000648:ultrastructure*; D011651:Pulmonary Artery / Q000002:abnormalities*; D013577:Syndrome; D016277:Ventricular Function, Left / Q000502:physiology