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Functional state following the Fontan procedure

  • Ismee A. Williams (a1), Lynn A. Sleeper (a2), Steven D. Colan (a3), Minmin Lu (a2), Elizabeth A. Stephenson (a4), Jane W. Newburger (a3), Welton M. Gersony (a1), Meryl S. Cohen (a5), James F. Cnota (a6), Andrew M. Atz (a7), Richard V. Williams (a8), Renee Margossian (a3), Andrew J. Powell (a3), Mario P. Stylianou (a9), Daphne T. Hsu (a1) and For the Pediatric Heart Network Investigators (see Appendix)...



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 with the 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.


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.


Corresponding author

Correspondence to: Ismee A. Williams, MD, MS, Columbia University College of Physicians & Surgeons, Morgan Stanley Children’s Hospital of New York, 2-North, 3959 Broadway, New York, NY 10032. Tel: (212) 342-1560; Fax: (212) 305-4429; E-mail:


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