title: Surgical palliation in patients with a single ventricle and dextrocardia.
abstract: BACKGROUND
  Dextrocardia is found in a significant proportion of patients undergoing a single-ventricle repair. Surgical outcomes in this cohort are unclear.
  
  
  METHODS
  The records of 41 consecutive patients with single-ventricle physiology and dextrocardia presenting in a single institution from 1990 to 2008 were reviewed. Of this cohort, 19 patients had heterotaxy syndrome. Twenty-five of the 41 patients had atrioventricular valve regurgitation (AVVR) on presentation (mild, 13; moderate, 9; severe, 3).
  
  
  RESULTS
  One patient died before intervention. Initial surgical palliation was performed in 31 patients. Four patients died postoperatively and 4 interim deaths occurred between initial palliation and bidirectional cavopulmonary shunt (BCPS). Thirty of the surviving 32 patients underwent BCPS, with 2 perioperative deaths. There were 4 additional deaths before Fontan surgery. Twenty-two of the surviving 25 patients underwent a Fontan procedure. There was 1 postoperative mortality. Survival to the age of 15 years was 56% (95% confidence interval [CI], 39%-70%). Patients with moderate or severe regurgitation had higher mortality if they were managed conservatively rather than by surgery (5 of 6 vs 2 of 6; P=.24). Patients with bilateral BCPS had better operative outcomes and survival compared with peers with unilateral anastomosis (odds ratio, 27; P=.005; 95% CI, 2.7-269). The side of the systemic venous pathway did not seem to influence outcomes.
  
  
  CONCLUSIONS
  Surgical outcomes of single-ventricle palliation seem poor in patients with dextrocardia. Aggressive management of congenital AVVR might improve the long-term prognosis.
authors.affiliation: University of Melbourne, Victoria, Australia; Department of Cardiac Surgery, Royal Children's Hospital, Victoria, Australia.
authors.forename: Chin L
authors.initials: CL
authors.lastname: Poh
authors.affiliation: University of Melbourne, Victoria, Australia; Murdoch Children's Research Institute, Victoria, Australia.
authors.forename: Mary
authors.initials: M
authors.lastname: Xu
authors.affiliation: The Clinical Epidemiology and Biostatistics Unit, Murdoch Children's Research Institute, Victoria, Australia; Department of Mathematics and Statistics, La Trobe University, Victoria, Australia.
authors.forename: John C
authors.initials: JC
authors.lastname: Galati
authors.affiliation: University of Melbourne, Victoria, Australia; Murdoch Children's Research Institute, Victoria, Australia.
authors.forename: Ajay J
authors.initials: AJ
authors.lastname: Iyengar
authors.affiliation: Department of Cardiology, Royal Children's Hospital, Victoria, Australia.
authors.forename: Michael
authors.initials: M
authors.lastname: Cheung
authors.affiliation: Murdoch Children's Research Institute, Victoria, Australia; Department of Cardiac Surgery, Royal Children's Hospital, Victoria, Australia; Department of Pediatrics, University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: Christian P
authors.initials: CP
authors.lastname: Brizard
authors.affiliation: Murdoch Children's Research Institute, Victoria, Australia; Department of Cardiac Surgery, Royal Children's Hospital, Victoria, Australia; Department of Pediatrics, University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: Igor E
authors.initials: IE
authors.lastname: Konstantinov
authors.affiliation: Murdoch Children's Research Institute, Victoria, Australia; Department of Cardiac Surgery, Royal Children's Hospital, Victoria, Australia; Department of Pediatrics, University of Melbourne, Melbourne, Victoria, Australia. Electronic address: yves.dudekem@rch.org.au.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003914:Dextrocardia / Q000401:mortality / Q000601:surgery*; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care; D011379:Prognosis; D015996:Survival Rate; D016896:Treatment Outcome