title: Risk factor analysis for second-stage palliation of single ventricle anatomy.
abstract: BACKGROUND
  Single ventricle hearts can be surgically palliated by a series of operations culminating in the Fontan procedure, which establishes a total cavopulmonary connection. The second-stage procedure creates a physiologic connection between the superior vena cava and the pulmonary artery.
  
  
  METHODS
  From 1998 to 2010, 557 patients with single ventricle heart disease underwent second-stage surgical palliation. This cohort was retrospectively analyzed to assess patient outcome by a number of anatomic, physiologic, and procedural factors. The analysis excluded patients undergoing hybrid first-stage procedures.
  
  
  RESULTS
  The median age at operation was 165 days (range, 59 days to 49 years). The most common anatomic subtypes were hypoplastic left heart syndrome (52%), tricuspid atresia (12%), unbalanced atrioventricular septal defect (10%), double inlet left ventricle (9%), or other (17%). Left ventricular hypoplasia was present in 70%. A hemi-Fontan procedure was done in 89%, and 11% received a bidirectional Glenn. Concomitant atrioventricular valve repair was necessary in 9%. Early mortality was 4.7%, and 5.9% died after discharge but before Fontan. No early or late deaths occurred in patients with tricuspid atresia and double inlet left ventricle. Multivariate analysis demonstrated ventricular dysfunction, atrioventricular valve regurgitation, and unbalanced atrioventricular septal defect were significant adverse risk factors for survival to Fontan.
  
  
  CONCLUSIONS
  Second-stage palliation can be performed at low risk for patients with left ventricular dominance, but significant risk remains for patients with left ventricular hypoplasia and unbalanced atrioventricular septal defect. Atrioventricular valve insufficiency is a persistent problem that has not been neutralized by repair strategies.
authors.affiliation: Division of Pediatric Cardiovascular Surgery, Section of Cardiac Surgery, University of Michigan Medical School, Ann Arbor, Michigan 48109, USA.
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Lee
authors.forename: Ranjit
authors.initials: R
authors.lastname: Aiyagari
authors.forename: Jennifer C
authors.initials: JC
authors.lastname: Hirsch
authors.forename: Richard G
authors.initials: RG
authors.lastname: Ohye
authors.forename: Edward L
authors.initials: EL
authors.lastname: Bove
authors.forename: Eric J
authors.initials: EJ
authors.lastname: Devaney
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000706:statistics & numerical data; D006343:Heart Septal Defects / Q000453:epidemiology / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation / Q000706:statistics & numerical data; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery; D059446:Heterotaxy Syndrome / Q000453:epidemiology / Q000601:surgery; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000453:epidemiology / Q000601:surgery; D000860:Hypoxia / Q000209:etiology / Q000601:surgery; D007223:Infant; D008297:Male; D008875:Middle Aged; D008944:Mitral Valve Insufficiency / Q000209:etiology / Q000601:surgery; D010166:Palliative Care; D011183:Postoperative Complications / Q000401:mortality / Q000601:surgery; D011651:Pulmonary Artery / Q000601:surgery*; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D016896:Treatment Outcome; D018785:Tricuspid Atresia / Q000453:epidemiology / Q000601:surgery; D014262:Tricuspid Valve Insufficiency / Q000209:etiology / Q000601:surgery; D014683:Vena Cava, Superior / Q000601:surgery*; D055815:Young Adult