Portal de Periódicos da CAPES

Functional state following the Fontan procedure

2009; Cambridge University Press; Volume: 19; Issue: 4 Linguagem: Inglês

10.1017/s1047951109990382

1467-1107

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

Coronary Artery Anomalies

Abstract 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 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. 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.