title: Effect of Fontan-Associated Morbidities on Survival With Intact Fontan Circulation.
abstract: Although survival after the Fontan operation has improved, little is known about the burden of major medical morbidities associated with the modern total cavopulmonary connection (TCPC). A total of 773 consecutive patients who underwent a first Fontan operation at our institution between 1992 and 2009 were retrospectively reviewed. All subjects underwent TCPC (53% lateral tunnel, 47% extracardiac conduit). Median length of follow-up was 5.3 years (interquartile range 1.4 to 11.2), and 30% had follow-up >10 years. Freedom from a composite medical morbidity outcome (protein-losing enteropathy, plastic bronchitis, serious thromboembolic event, or tachyarrhythmia) was 47% at 20 years (95% confidence interval [CI] 38 to 55). Independent risk factors for morbidity included pre-Fontan atrioventricular valve regurgitation (hazard ratio [HR] 1.7, 95% CI 1.2 to 2.4, p = 0.001), pleural drainage >14 days (HR 1.5, 95% CI 1.01 to 2.2, p = 0.04), and longer cross-clamp time (HR 1.2 per 10 minutes, 95% CI 1.06 to 1.3, p = 0.004) at the time of TCPC. Surgical era, Fontan type, and ventricular morphology were not associated with the composite outcome. Presence of Fontan-associated morbidity was associated with a 36-fold increase in the risk of subsequent Fontan takedown, heart transplantation, or death (95% CI 17 to 76, p <0.001). For patients without any component of the composite outcome, freedom from Fontan failure was 98% at 20 years (95% CI 96 to 99). Medical morbidities after TCPC are common and significantly reduce the longevity of the Fontan circulation. However, for those patients who remain free from the composite morbidity outcome, 20-year survival with intact Fontan circulation is encouraging.
authors.affiliation: Division of Pediatric Cardiology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois; Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Kiona Y
authors.initials: KY
authors.lastname: Allen
authors.affiliation: Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Tacy E
authors.initials: TE
authors.lastname: Downing
authors.affiliation: Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Andrew C
authors.initials: AC
authors.lastname: Glatz
authors.affiliation: Division of Pediatric Cardiology, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, Texas.
authors.forename: Lindsay S
authors.initials: LS
authors.lastname: Rogers
authors.affiliation: Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Chitra
authors.initials: C
authors.lastname: Ravishankar
authors.affiliation: Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
authors.affiliation: Department of Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Fuller
authors.affiliation: Department of Anesthesiology and Critical Care, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Lisa M
authors.initials: LM
authors.lastname: Montenegro
authors.affiliation: Department of Anesthesiology and Critical Care, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: James M
authors.initials: JM
authors.lastname: Steven
authors.affiliation: Department of Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Thomas L
authors.initials: TL
authors.lastname: Spray
authors.affiliation: Department of Anesthesiology and Critical Care, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Susan C
authors.initials: SC
authors.lastname: Nicolson
authors.affiliation: Department of Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: James William
authors.initials: JW
authors.lastname: Gaynor
authors.affiliation: Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Electronic address: goldbergda@email.chop.edu.
authors.forename: David J
authors.initials: DJ
authors.lastname: Goldberg
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure; D005544:Forecasting; D006330:Heart Defects, Congenital / Q000453:epidemiology / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D009017:Morbidity / Q000639:trends; D010414:Pennsylvania / Q000453:epidemiology; D011183:Postoperative Complications / Q000453:epidemiology*; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D016896:Treatment Outcome; D055815:Young Adult