title: Improving results of the Fontan procedure in patients with heterotaxy syndrome.
abstract: BACKGROUND
  The Fontan operation in patients with heterotaxy syndrome has been associated with a poor prognosis. We studied whether the outcome of those patients with heterotaxy syndrome improved compared with those who did not have the syndrome after an extracardiac Fontan operation.
  
  
  METHODS
  A retrospective review was performed on 185 patients who had undergone an extracardiac Fontan operation between 1996 and 2005.
  
  
  RESULTS
  Sixty-two of the patients had heterotaxy syndrome (33.5%). Forty-one had right isomerism and 21 had left isomerism. Heterotaxy syndrome was commonly associated with a morphologic right ventricle (59.7%), a common atrioventricular valve (72.6%), an interrupted inferior vena cava (25.8%), a separate hepatic vein (30.6%), and extracardiac pulmonary venous drainage (16.1%). The hospital mortality rate was higher in the heterotaxy syndrome than nonheterotaxy (4.8% vs 2.4%; p = 0.05). Eight-year survivals were 91.9 +/- 3.2% in the nonheterotaxy group and 89.3 +/- 4.2 % in the heterotaxy group (p = 0.39). At 8 years, freedom from reoperation was 90.2 +/- 3.2% in the nonheterotaxy group and 78.5 +/- 6.2% in the heterotaxy group (p = 0.15). The outcomes (other than those of arrhythmia) were no different between the two groups. The incidences of early and late postoperative arrhythmia were 29.0% and 25.4%, respectively, in heterotaxy patients, and 15.4% and 10.8% in nonheterotaxy patients (p < 0.05). Bradyarrhythmia was found to be more common. During follow-up, atrioventricular valve regurgitation of more than mild was more common in heterotaxy patients (33.9% vs 18.9%; p = 0.05).
  
  
  CONCLUSIONS
  Midterm outcomes after an extracardiac Fontan operation in heterotaxy and nonheterotaxy patients are similar, except arrhythmia and atrioventricular valve regurgitation.
authors.affiliation: Department of Pediatric Cardiology and Cardiovascular Surgery, Sejong General Hospital, Bucheon, Korea.
authors.forename: Soo-Jin
authors.initials: SJ
authors.lastname: Kim
authors.forename: Woong-Han
authors.initials: WH
authors.lastname: Kim
authors.forename: Hong Gook
authors.initials: HG
authors.lastname: Lim
authors.forename: Chang-Ha
authors.initials: CH
authors.lastname: Lee
authors.forename: Jae Young
authors.initials: JY
authors.lastname: Lee
mesh_terms: D001145:Arrhythmias, Cardiac / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D012086:Reoperation; D012189:Retrospective Studies; D016019:Survival Analysis; D013577:Syndrome; D016896:Treatment Outcome