title: Surgical management of competing pulmonary blood flow affects survival before Fontan/Kreutzer completion in patients with tricuspid atresia type I.
abstract: OBJECTIVE
  To determine the association between surgical management of pulmonary blood flow (PBF) at initial and staged procedures with survival to Fontan/Kreutzer operation (Fontan) in patients with tricuspid atresia.
  
  
  METHODS
  Infants aged <3 months with tricuspid atresia type I (n = 303) were enrolled from 34 institutions (1999-2013). Among those who underwent surgical intervention (n = 302), initial procedures were: systemic to pulmonary artery shunt (SPS; n = 189; 62%); pulmonary artery banding (PAB; n = 50; 17%); and superior cavopulmonary connection (SCPC; n = 63; 21%). Multiphase parametric-hazard models were used to analyze competing outcomes.
  
  
  RESULTS
  Risk-adjusted 6-year survival was lower after SPS (85%; P = .04) versus PAB (93%) or SCPC (93%). Survival after SPS when the main pulmonary artery (MPA) was closed (n = 21) or banded (n = 4) was 60%, versus 93% without MPA intervention (P = .02). After SPS, survival before SCPC was lower with an open ductus arteriosus (n = 7; 76% vs 97%; P = .02). Similarly, after SPS, risk-adjusted survival was similar to that for patients who had an initial PAB or SCPC when MPA intervention was avoided and the ductus arteriosus either closed spontaneously before SPS, or was closed during SPS. For all patients reaching SCPC (n = 277), survival to Fontan was not significantly influenced by whether PBF persisted through the MPA.
  
  
  CONCLUSIONS
  Tricuspid atresia patients with SPS represent a high-risk subgroup. Avoiding an open ductus arteriosus and concomitant MPA intervention during SPS may help mitigate the risk associated with SPS. The presence of antegrade PBF through the MPA, at initial and staged operations, did not significantly influence survival to Fontan operation.
authors.affiliation: Congenital Heart Surgeons' Society Data Center, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Travis J
authors.initials: TJ
authors.lastname: Wilder
authors.affiliation: Department of Surgery, Division of Cardiac & Thoracic Surgery, University of Chicago Medicine, Chicago, Ill.
authors.forename: Gerhard
authors.initials: G
authors.lastname: Ziemer
authors.affiliation: Department of Cardiovascular Surgery, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Edward J
authors.initials: EJ
authors.lastname: Hickey
authors.affiliation: University of Iowa Carver College of Medicine, Iowa City, Iowa.
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Gruber
authors.affiliation: Division of Pediatric Cardiac Surgery, Benioff Children's Hospital, University of California San Francisco, San Francisco, Calif.
authors.forename: Tara
authors.initials: T
authors.lastname: Karamlou
authors.affiliation: Division of Cardiothoracic Surgery, Connecticut Children's Medical Center and Yale-New Haven Children's Hospital, Hartford and New Haven, Conn.
authors.forename: Paul M
authors.initials: PM
authors.lastname: Kirshbom
authors.affiliation: Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Eugene H
authors.initials: EH
authors.lastname: Blackstone
authors.affiliation: Department of Cardiothoracic Surgery, Arnold Palmer Hospital for Children, Orlando, Fla.
authors.forename: William M
authors.initials: WM
authors.lastname: DeCampli
authors.affiliation: Congenital Heart Surgeons' Society Data Center, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: William G
authors.initials: WG
authors.lastname: Williams
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, University of Toronto and The Hospital for Sick Children, Toronto, Canada. Electronic address: brian.mccrindle@sickkids.ca.
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
mesh_terms: D002170:Canada; D018729:Fontan Procedure / Q000009:adverse effects / Q000401:mortality; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D011183:Postoperative Complications / Q000209:etiology; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000503:physiopathology / Q000601:surgery*; D011652:Pulmonary Circulation; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D018785:Tricuspid Atresia / Q000145:classification / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D014261:Tricuspid Valve / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D014481:United States
keywords: accessory pulmonary blood flow; congenital heart disease; systemic to pulmonary artery shunt; tricuspid atresia