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MINI-FOCUS ISSUE: THE FONTAN PROCEDURE

A Cross-Sectional Study of Exercise Performance During the First 2 Decades of Life After the Fontan Operation

Stephen M. Paridon, MD^_*,_*, Paul D. Mitchell, MS, Steven D. Colan, MD^_**, Richard V. Williams, MD, Andrew Blaufox, MD, Jennifer S. Li, MD^||, Renee Margossian, MD^_**, Seema Mital, MD^¶, Jennifer Russell, MD^#, Jonathan Rhodes, MD^_** for the Pediatric Heart Network Investigators

^_* The University of Pennsylvania, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
New England Research Institutes, Watertown, Massachusetts
University of Utah, Primary Children's Medical Center, Salt Lake City, Utah
Medical University of South Carolina, Charleston, South Carolina
^|| Duke University Medical Center, Durham, North Carolina
^¶ Columbia University, Children's Hospital of New York, New York, New York
^# University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
^_** Harvard University, Children's Hospital of Boston, Boston, Massachusetts.

Manuscript received September 25, 2007; revised manuscript received February 4, 2008, accepted February 12, 2008.

^_* Reprint requests and correspondence: Dr. Stephen M. Paridon, Cardiology Division, Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104. (Email: Paridon{at}email.chop.edu).

Objectives: The aim of this study was to describe exercise performance during the first 2 decades of life in Fontan survivors by a cross-sectional study and to identify factors that influence exercise performance.

Background: Exercise performance after the Fontan procedure is reduced relative to performance in healthy subjects. Data on pre-adolescents are limited, and the patterns of exercise performance in different ages are unexplored.

Methods: Ramp cycle ergometry was performed with expired gas. Data were analyzed for the entire study population and for subpopulations that did and did not achieve a maximal aerobic capacity.

Results: Of 411 subjects tested (12.4 ± 3.2 years of age), 166 achieved a maximal aerobic capacity. Peak oxygen consumption (VO₂) was 26.3 ml/kg/min (65% of predicted for age and gender [% predicted]) for the entire population and was lower in the submaximal capacity subgroup compared with the maximal capacity subgroup (63% predicted and 67% predicted, respectively; p = 0.02). Oxygen consumption at ventilatory anaerobic threshold (VAT) was better preserved (78% predicted for the total population) than peak VO₂. Higher % predicted O₂ pulse at peak exercise was associated with greater % predicted peak VO₂, work rate, and VAT. Adolescence and male gender were associated with decreased % predicted peak VO₂. The relationship between echocardiographic indexes of ventricular function and exercise function was surprisingly weak.

Conclusions: In Fontan patients, maximal aerobic capacity is reduced compared with healthy subjects, with better preservation of submaximal performance. Higher O₂ pulse is associated with better exercise performance, whereas adolescence and male gender are associated with decreased performance compared with healthy subjects.

Key Words: exercise • Fontan operation • oxygen consumption • oxygen pulse

Abbreviations and Acronyms   BMI = body mass index   HR = heart rate   dP/dtic = rate of pressure change during isovolumic contraction   MVV = maximal voluntary ventilation   % predicted = percent of predicted for age and gender   RER = respiratory exchange ratio   VAT = ventilatory anaerobic threshold   VCO2 = minute carbon dioxide production   VE = minute ventilation   VO2 = oxygen consumption

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