title: Treatment of right ventricle to coronary artery connections in infants with pulmonary atresia and intact ventricular septum.
abstract: OBJECTIVE
  At the severe end of the spectrum of infants with pulmonary atresia and intact ventricular septum, the likelihood of significant right ventricle to coronary artery connections increases. Our purpose is to present the first series of right ventricle to coronary artery connections ligated off bypass before right ventricular decompression and to evaluate the consequences of this approach.
  
  
  METHODS
  From 1988 to 2007, 19 patients with pulmonary atresia and intact ventricular septum had a total of 69 right ventricle to coronary artery connections identified preoperatively, and 10 more were located intraoperatively. Of these, 71 were judged large enough to warrant off-pump direct ligation. Preoperative diagnosis was by transthoracic echocardiography and angiography. Transesophageal and surface echocardiography were used for intraoperative location. Direct visualization and echocardiographic assessment for regional wall motion abnormalities determined the effects of ligation. Right ventricular decompression was done in all patients.
  
  
  RESULTS
  After ligation, coronary flow converted from moderately or largely retrograde to antegrade perfusion. Ligation produced no visual myocardial consequences or immediate local wall motion abnormalities. For 3 patients, however, apical-septal wall motion abnormalities appeared from 2 hours to 3 days postoperatively. Serial studies were done to assess the later effects in the 16 of 19 30-day survivors. No evidence for myocardial injury was found, and all continued on a 2-ventricle repair course.
  
  
  CONCLUSIONS
  The location and ligation of right ventricle to coronary artery connections can be reliably accomplished off bypass. Coronary flow became antegrade, improving myocardial oxygenation. No myocardial damage was observed. Inapparent right ventricle to coronary artery connections occasionally enlarge secondarily after right ventricular decompression, making early follow-up evaluation necessary after ligation. Despite the initial presence of significant right ventricle to coronary artery connections, 2-ventricle repairs are possible with long-term benefits.
authors.affiliation: Division of Cardiothoracic Surgery, University of Minnesota, Minneapolis, Minnesota 55455, USA. foker001@umn.edu
authors.forename: John E
authors.initials: JE
authors.lastname: Foker
authors.forename: Shaun P
authors.initials: SP
authors.lastname: Setty
authors.forename: James
authors.initials: J
authors.lastname: Berry
authors.forename: Prachi
authors.initials: P
authors.lastname: Jain
authors.forename: Kirsti
authors.initials: K
authors.lastname: Catton
authors.forename: Adriana C
authors.initials: AC
authors.lastname: Gittenberger-de-Groot
authors.forename: Lee A
authors.initials: LA
authors.lastname: Pyles
mesh_terms: D006348:Cardiac Surgical Procedures / Q000379:methods; D002675:Child, Preschool; D017023:Coronary Angiography; D003330:Coronary Vessel Anomalies / Q000601:surgery*; D003331:Coronary Vessels / Q000601:surgery*; D004452:Echocardiography; D017548:Echocardiography, Transesophageal; D006346:Heart Septum; D006352:Heart Ventricles / Q000002:abnormalities / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008026:Ligation; D018633:Pulmonary Atresia / Q000150:complications / Q000601:surgery*; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000002:abnormalities