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J Thorac Cardiovasc Surg 2008;136:100-107
© 2008 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Functional status, heart rate, and rhythm abnormalities in 521 Fontan patients 6 to 18 years of age

^a Medical University of South Carolina, Charleston, SC
^b New England Research Institutes, Watertown, Mass
^c University of Utah, Primary Children's Medical Center, Salt Lake City, Utah
^d Children's Hospital Boston and Harvard Medical School, Boston, Mass
^e Children's Hospital of New York, Columbia University, New York, NY
^f Duke University School of Medicine, Durham, NC
^g The Hospital for Sick Children, Toronto, Ontario, Canada
^h National Heart, Lung, and Blood Institute, Bethesda, Md
ⁱ Children's Hospital of Philadelphia, Philadelphia, Pa

Received for publication September 14, 2007; revisions received November 29, 2007; accepted for publication December 18, 2007.

^_* Address for reprints: Andrew D. Blaufox, MD, Children's Heart Center, Schneider Children's Hospital, 269-01 176th Ave, New Hyde Park, NY 11040. (Email: Ablaufox{at}nshs.edu).

Objectives: Our objective was to determine the relationship between functional outcome and abnormalities of heart rate and rhythm after the Fontan operation.

Methods: The National Heart, Lung, and Blood Institute Pediatric Heart Network conducted a cross-sectional analysis of patients who had undergone a Fontan procedure at the 7 network centers. Analysis was based on 521 patients with an electrocardiogram (n = 509) and/or bicycle exercise test (n = 404). The Child Health Questionnaire parent report and the oxygen consumption at the anaerobic threshold were used as markers of functional outcome.

Results: Various Fontan procedures had been performed: intracardiac lateral tunnel (59%), atriopulmonary connection (14%), extracardiac later tunnel (13%), and extracardiac conduit (11%). Prior volume unloading surgery was performed in 389 patients: bidirectional Glenn (70%) and hemi-Fontan (26%). A history of atrial tachycardia was noted in 9.6% of patients and 13.1% of patients had a pacemaker. Lower resting heart rate and higher peak heart rate were each weakly associated with better functional status, as defined by higher anaerobic threshold (R = –0.18, P = .004, and R = 0.16, P = .007, respectively) and higher Child Health scores for physical functioning (R = –0.18, P < .001, and R = 0.17, P = .002, respectively). Higher anaerobic threshold was also independently associated with younger age and an abnormal P-axis. Resting bradycardia was not associated with anaerobic threshold or Child Health scores.

Conclusions: In pediatric patients (6–18 years) after the Fontan procedure, a lower resting heart rate and a higher peak heart rate are each independently associated with better physical function as measured by anaerobic threshold and Child Health scores. However, these correlations are weak, suggesting that other, nonrhythm and nonrate, factors may have a greater impact on the functional outcome of pediatric patients after the Fontan operation.

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