title: Influence of Morphology and Initial Surgical Strategy on Survival of Infants With Tricuspid Atresia.
abstract: BACKGROUND
  Tricuspid atresia (TA) is a heterogeneous single-ventricle anomaly in which initial presentation and, consequently, timing and mode of palliation vary based on morphology and degree of pulmonary or systemic outflow obstruction. We report current era palliation outcomes and examine whether morphologic and, subsequently, surgical factors influence survival.
  
  
  METHODS
  From 2002 to 2012, 105 infants with TA underwent surgical palliation. Competing risks analyses modeled events after first-stage surgery (Glenn versus death) and after Glenn (Fontan versus death) and examined risk factors affecting outcomes.
  
  
  RESULTS
  Seventy-eight patients (74%) required neonatal first-stage palliation, including modified Blalock-Taussig shunt (n = 46, 44%), Norwood (n = 18, 17%), and pulmonary artery band (n = 14, 13%), whereas 27 (26%) received primary Glenn as their initial surgery. Hospital mortality was 5 patients (4.8%). Competing risks models showed that by 1 year after first-stage surgery, 15% of patients had died and 83% had undergone Glenn. By 5 years after Glenn, 2% of patients had died and 80% had undergone Fontan. Overall 8-year survival was 84%. On multivariable analysis, risk factors for mortality were genetic/extracardiac anomalies (hazard ratio 7.0, 95% confidence interval: 2.4 to 20.6, p < 0.001) and pulmonary atresia (hazard ratio 4.4, 95% confidence interval: 1.6 to 12.2, p = 0.004). Survival was not affected by initial palliation type (p = 0.36), ventriculoarterial discordance (p = 0.25), systemic outflow obstruction (p = 0.84), or arch obstruction (p = 0.62).
  
  
  CONCLUSIONS
  Despite morphologic and physiologic variations necessitating different palliative sequences, multistage palliation outcomes of various TA subtypes are comparable and generally good, with the exception of patients with associated genetic/extracardiac anomalies. The bulk of mortality is interstage, indicating continued opportunity for improvement in monitoring and managing patients during this critical period.
authors.affiliation: Division of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia. 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, Georgia.
authors.forename: Brian
authors.initials: B
authors.lastname: Schlosser
authors.affiliation: Division of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia.
authors.forename: Makoto
authors.initials: M
authors.lastname: Mori
authors.affiliation: Division of Cardiology, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia.
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, Georgia.
authors.forename: Timothy
authors.initials: T
authors.lastname: Slesnick
authors.affiliation: Division of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia.
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, Georgia.
authors.forename: Christopher
authors.initials: C
authors.lastname: Petit
authors.affiliation: Division of Cardiology, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia.
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, Georgia.
authors.forename: Kirk
authors.initials: K
authors.lastname: Kanter
mesh_terms: D006348:Cardiac Surgical Procedures / Q000379:methods; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D018785:Tricuspid Atresia / Q000401:mortality* / Q000473:pathology / Q000601:surgery*