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MINI-FOCUS ISSUE: THE FONTAN PROCEDURE

Contemporary Outcomes After the Fontan Procedure

A Pediatric Heart Network Multicenter Study

Page A.W. Anderson, MD^_*,_*, Lynn A. Sleeper, ScD, Lynn Mahony, MD, Steven D. Colan, MD, Andrew M. Atz, MD^||, Roger E. Breitbart, MD, Welton M. Gersony, MD^¶, Dianne Gallagher, MS, Tal Geva, MD, Renee Margossian, MD, Brian W. McCrindle, MD, MPH^#, Stephen Paridon, MD^_**, Marcy Schwartz, MD, Mario Stylianou, PhD, Richard V. Williams, MD, Bernard J. Clark, III, MD^_** for the Pediatric Heart Network Investigators

^_* Duke University Medical Center, Durham, North Carolina
New England Research Institutes, Watertown, Massachusetts
University of Texas Southwestern Medical Center, Dallas, Texas
Children's Hospital Boston, Boston, Massachusetts
^|| Medical University of South Carolina, Charleston, South Carolina
^¶ Columbia University Medical Center, New York, New York
^# The Hospital for Sick Children, Toronto, Ontario, Canada
^_** Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
National Heart, Lung, and Blood Institute, Bethesda, Maryland
Primary Children's Medical Center, Salt Lake City, Utah.

Manuscript received September 18, 2007; revised manuscript received January 17, 2008, accepted January 26, 2008.

^_* Reprint requests and correspondence: Dr. Page A. W. Anderson, Duke University Medical Center, Research Park Building 2, Room 113, Box 3218, Durham, North Carolina 27710. (Email: ander005{at}mc.duke.edu).

Objectives: We characterized a large cohort of children who had a Fontan procedure, with measures of functional health status, ventricular size and function, exercise capacity, heart rhythm, and brain natriuretic peptide (BNP).

Background: The characteristics of contemporary Fontan survivors are not well described.

Methods: We enrolled 546 children (age 6 to 18 years, mean 11.9 years) and compared them within pre-specified anatomic and procedure subgroups. History and outcome measures were obtained within a 3-month period.

Results: Predominant ventricular morphology was 49% left ventricular (LV), 34% right ventricular (RV), and 19% mixed. Ejection fraction (EF) was normal for 73% of subjects; diastolic function grade was normal for 28%. Child Health Questionnaire mean summary scores were lower than for control subjects; however, over 80% of subjects were in the normal range. Brain natriuretic peptide concentration ranged from <4 to 652 pg/ml (median 13 pg/ml). Mean percent predicted peak O₂ consumption was 65% and decreased with age. Ejection fraction and EF Z score were lowest, and semilunar and atrioventricular (AV) valve regurgitation were more prevalent in the RV subgroup. Older age at Fontan was associated with more severe AV valve regurgitation. Most outcomes were not associated with a superior cavopulmonary connection before Fontan.

Conclusions: Measures of ventricular systolic function and functional health status, although lower on average in the cohort compared with control subjects, were in the majority of subjects within 2 standard deviations of the mean for control subjects. Right ventricular morphology was associated with poorer ventricular and valvular function. Effective strategies to preserve ventricular and valvular function, particularly for patients with RV morphology, are needed.

Key Words: brain natriuretic peptide • cardiac magnetic resonance • diastolic function • echocardiography • exercise • Fontan • pediatric

Abbreviations and Acronyms   AV = atrioventricular   BNP = brain natriuretic peptide   BSA = body surface area   CMR = cardiac magnetic resonance   E = atrioventricular valve peak early diastolic inflow velocity   E' = tissue Doppler peak early diastolic velocity   EDV = end-diastolic volume   EF = ejection fraction   ESV = end-systolic volume   FP = systemic ventricular flow propagation rate   LV = left ventricle/ventricular   RV = right ventricle/ventricular   SV = stroke volume   VAT = peak oxygen consumption at anaerobic threshold   VO2 = oxygen consumption

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