title: Outcomes of multistage palliation of infants with functional single ventricle and heterotaxy syndrome.
abstract: BACKGROUND
  Management of infants with heterotaxy syndrome and functional single ventricle is complicated due to associated cardiac and extracardiac anomalies. We report current era palliation results.
  
  
  METHODS
  Between 2002 and 2012, 67 infants with heterotaxy syndrome underwent multistage palliation. Competing risks analyses modeled events after surgery (death vs Glenn procedure) and examined factors associated with survival. In addition, early and late outcomes following first-stage palliation surgery were compared with a matched contemporaneous control group of patients with nonheterotaxy single ventricle anomalies.
  
  
  RESULTS
  Fifty-eight patients (87%) required neonatal palliation, including a modified Blalock-Taussig shunt (n = 34; 51%), Norwood operation (n = 12; 18%) or pulmonary artery band (n = 12; 18%), whereas 9 patients (13%) underwent a primary Glenn procedure. Competing risks analysis showed that at 1 year after first-stage palliation surgery, 29% of the patients had died or undergone transplantation and 63% had undergone a Glenn procedure. By 5 years after the Glenn procedure, 64% of patients had undergone a Fontan procedure. The overall 8-year survival rate was 66%. On multivariable analysis, factors associated with mortality were unplanned reoperation (hazard ratio [HR], 2.9; 95% confidence interval [CI], 1.1-7.3; P = .005) and total anomalous pulmonary venous connection repair (HR, 2.3; 95% CI, 1.0-5.6; P = .056). Comparison with the contemporaneous matched patients with nonheterotaxy single ventricle anomalies showed that first-stage palliation in the patients with heterotaxy was associated with a higher rate of in-hospital death (27% vs 10%; P = .022), and significantly longer durations of ventilation and intensive care unit stay. Interstage mortality, survival after the Glenn procedure, and progression to the Fontan procedure were comparable in the 2 groups.
  
  
  CONCLUSIONS
  The management of infants with heterotaxy and a functional single ventricle remains challenging. First-stage palliation is associated with high operative mortality and increased resource utilization owing to surgical morbidity. Nonetheless, outcomes beyond hospital discharge are comparable to those for patients with other single ventricle anomalies. Efforts to improve survival in those patients should focus on perioperative care.
authors.affiliation: Division of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga. Electronic address: balsoufi@hotmail.com.
authors.forename: Bahaaldin
authors.initials: B
authors.lastname: Alsoufi
authors.affiliation: Division of Cardiology, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: Courtney
authors.initials: C
authors.lastname: McCracken
authors.affiliation: Division of Cardiology, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: Brian
authors.initials: B
authors.lastname: Schlosser
authors.affiliation: Division of Cardiology, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: Ritu
authors.initials: R
authors.lastname: Sachdeva
authors.affiliation: Division of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: Andrew
authors.initials: A
authors.lastname: Well
authors.affiliation: Division of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: Brian
authors.initials: B
authors.lastname: Kogon
authors.affiliation: Division of Cardiology, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: William
authors.initials: W
authors.lastname: Border
authors.affiliation: Division of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: Kirk
authors.initials: K
authors.lastname: Kanter
mesh_terms: D058366:Blalock-Taussig Procedure / Q000379:methods / Q000401:mortality; D015331:Cohort Studies; D016001:Confidence Intervals; D016208:Databases, Factual; D015199:Extracorporeal Membrane Oxygenation / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods* / Q000401:mortality; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery; D059446:Heterotaxy Syndrome / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D017052:Hospital Mortality / Q000639:trends; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D058327:Norwood Procedures / Q000379:methods* / Q000401:mortality; D010166:Palliative Care / Q000379:methods*; D011182:Postoperative Care / Q000379:methods; D012086:Reoperation / Q000379:methods; D012189:Retrospective Studies; D018570:Risk Assessment; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome
keywords: Fontan; Glenn; heterotaxy syndrome; single ventricle