title: Significance of the residual aortic obstruction in multistage repair of hypoplastic left heart syndrome.
abstract: OBJECTIVE
  The population of children who received right ventricle-to-pulmonary artery shunt (RV-PA) at Norwood procedure (NP) is growing, but limited data are available regarding the long-term outcome. The aim of this study was to present operative outcomes and mid-term hemodynamics and to assess the impact of the residual aortic obstruction on the results in patients undergoing three-staged surgery with RV-PA application at NP.
  
  
  METHODS
  Between June 2001 and June 2009, 229 children with hypoplastic left heart syndrome (HLHS) and variants underwent NP with RV-PA; 172 have proceeded to stage II and 95 to stage III. The medical records (clinical data, echocardiographic records, cardiac catheterization reports, electrocardiograms, and surgical notes) were retrospectively reviewed.
  
  
  RESULTS
  The later era of NP was associated with significantly better outcome (2001-2004: 1- and 5-years' survival were 64.3% and 59.8%, respectively; 2005-2009: 1- and 4-years' survival were 93.1% and 86.9%, respectively) (p<0.001). There was no association between long-term survival and diagnosis (HLHS/HLHS variant) (p=0.39). The incidence of depressed ventricular function and moderate or severe systemic atrioventricular valve regurgitation among the children who required balloon aortoplasty (BA) before stage II surgery was significantly higher than in children without aortic arch obstruction (p=0.027, p=0.008, respectively). In midterm follow-up, BA had no significant influence on the actuarial survival (p=0.089). No ventricular arrhythmias were noticed at any stage.
  
  
  CONCLUSIONS
  The RV-PA shunt is a safe technique that does not seem to impair systolic or electrical ventricular function; its outcomes continue to improve with growing experience. Combined cardiologic interventional and surgical procedures are required to optimize the outcomes.
authors.affiliation: Department of Cardiac Surgery, Klinikum Grosshadern, Ludwig Maximilians University, Munich, Germany. mijanusz@cyf-kr.edu.pl
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Januszewska
authors.forename: Rainer
authors.initials: R
authors.lastname: Kozlik-Feldmann
authors.forename: Zbigniew
authors.initials: Z
authors.lastname: Kordon
authors.forename: Simon
authors.initials: S
authors.lastname: Urschel
authors.forename: Heinrich
authors.initials: H
authors.lastname: Netz
authors.forename: Bruno
authors.initials: B
authors.lastname: Reichart
authors.forename: Edward
authors.initials: E
authors.lastname: Malec
mesh_terms: D001024:Aortic Valve Stenosis / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D006328:Cardiac Catheterization; D002404:Catheterization / Q000379:methods; D004812:Epidemiologic Methods; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects / Q000379:methods; D006352:Heart Ventricles / Q000601:surgery; D006439:Hemodynamics; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000503:physiopathology / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D058327:Norwood Procedures / Q000009:adverse effects / Q000379:methods*; D011651:Pulmonary Artery / Q000601:surgery; D016896:Treatment Outcome; D014262:Tricuspid Valve Insufficiency / Q000209:etiology; D018754:Ventricular Dysfunction / Q000209:etiology