Functional state following the Fontan procedure

Ismee A. Williams, Lynn A. Sleeper, Steven D. Colan, Minmin Lu, Elizabeth A. Stephenson, Jane W. Newburger, Welton M. Gersony, Meryl S. Cohen, James F. Cnota, Andrew M. Atz, Richard V. Williams, Renee Margossian, Andrew J. Powell, Mario P. Stylianou, Daphne T. Hsu, Gail Pearson, Judith Massicot-Fisher, Marsha Mathis, Victoria Pemberton, Paul MitchellDianne Gallagher, Patti Nash, Gloria Klein, Minmin Lu, Lynn Mahony, Stephen Roth, Roger Breitbart, Jonathan Rhodes, Jodi Elder, Ellen McGrath, Seema Mital, Beth Printz, Ashwin Prakash, Darlene Servedio, Victoria Vetter, Bernard J. Clark, Mark Fogel, Steven Paridon, Jack Rychik, Margaret Harkins, Jamie Koh, Page A W Anderson, Rene Herlong, Lynne Hurwitz, Jennifer S. Li, Ann Marie Nawrocki, J. Philip Saul, Andrew D. Blaufox, Girish Shirali, Jon Lucas, Amy Blevins, LuAnn A. Minich, Linda Lambert, Michael Puchalski, Brian McCrindle, Timothy Bradley, Kevin Roman, Jennifer Russell, Shi Joon Yoo, Elizabeth Radojewski, Nancy Slater, Tal Geva, Marcy Schwartz, Michael Artman, Dana Connolly, Timothy Feltes, Julie Johnson, Jeffrey Krischer, G. Paul Matherne, John Kugler, Kathryn Davis, David J. Driscoll, Mark Galantowicz, Sally A. Hunsberger, Thomas J. Knight, Catherine L. Webb, Lawrence Wissow

Research output: Contribution to journalArticlepeer-review

29 Scopus citations


Background: Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function. Methods and Results: We analyzed data from 476 survivors withthe Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study. Mean age at creation of the Fontan circulation was 3.4 plus or minus 2.1 years, with a range from 0.7 to 17.5 years, and time since completion was 8.7 plus or minus 3.4 years, the range being from 1.1 to 17.3 years. We calculated a functional score for the survivors by averaging the percentile ranks of ventricular ejection fraction, maximal consumption of oxygen, the physical summary score for the Child Health Questionnaire, and a function of brain natriuretic peptide. The mean calculated score was 49.5 plus or minus 17.3, with a range from 3 to 87. After adjustment for time since completion of the circulation, we found that a lower score, and hence worse functional state, was associated with: right ventricular morphology (p less than 0.001), higher ventricular end-diastolic pressure (p equals 0.003) and lower saturations of oxygen (p equals 0.047) prior to completion of the Fontan circulation, lower income for the caregiver (p equals 0.003), and, in subjects without a prior superior cavopulmonary anastomosis, arrhythmias after completion of the circulation (p equals 0.003). The model explained almost one-fifth (18%) of the variation in the calculated scores. The score was not associated with surgical centre, sex, age, weight, fenestration, or the period of stay in hospital after completion of the Fontan circuit. A validation model, using 71 subjects randomly excluded from initial analysis, weakly correlated (R equals 0.17, p equals 0.16) with the score calculated from the dataset. Conclusions: Right ventricular morphology, higher ventricular end-diastolic pressure and lower saturations of oxygen prior to completion of the Fontan circuit, lower income for the provider of care, and arrhythmias after creation of the circuit, are all associated with a worse functional state. Unmeasured factors also influence outcomes. © 2009

Original languageEnglish (US)
Pages (from-to)320-330
Number of pages11
JournalCardiology in the Young
Issue number4
StatePublished - 2009


  • Clinical outcomes
  • Single ventricle
  • Univentricular heart

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine


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