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Exercise Capacity and Predictors of Performance After Fontan: Results from the Pediatric Heart Network Fontan 3 Study

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Abstract

Impaired exercise following Fontan is a surrogate of morbidity. Single-center longitudinal data exist, but there is a lack of contemporary multi-center data. Ramp cycle ergometry was re-performed in consented participants who had originally participated in the Pediatric Heart Network’s Fontan cross-sectional study. Annualized change was evaluated at maximal and submaximal exercise. Associations between these outcomes and patient characteristics were analyzed. There were 336 participants in Fontan 3, mean age 23.2 years. Paired measurements of peak oxygen consumption (peak VO2) were available for 95; peak exercise data at Fontan 3 were available for 275. Percent-predicted peak VO2 declined by 0.8 ± 1.7% per year (p < 0.001). At Fontan 3, the lowest performing peak VO2 tertile had the highest rate of overweight and obesity (p < 0.001). Female gender was more prevalent in the highest performing tertile (p = 0.004). Paired data at the ventilatory anaerobic threshold (VO2 at VAT) were available for 196; VAT data at Fontan 3 were available for 311. Percent-predicted VO2 at VAT decreased by 0.8 ± 2.6% per year (p < 0.001). At Fontan 3, VO2 at VAT was better preserved than peak VO2 across all tertiles, with higher rates of overweight and obesity in the lower performing group (p = 0.001). Female gender (p < 0.001) and left ventricular morphology (p = 0.03) were associated with better performance. Submaximal exercise is better preserved than maximal in the Fontan population, but declined at the same rate over the study period. The overall longitudinal rate of decline in exercise performance is slower than what has been described previously.

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Data Availability

The data for this study is housed at the Healthcore, which served as the data coordinating center.

Abbreviations

TCPC:

Total cavopulmonary connection

PHN:

Pediatric Heart Network

RER:

Respiratory exchange ratio

Peak VO2 :

Peak oxygen consumption

VO2 at VAT:

Oxygen consumption at the ventilatory anaerobic threshold

BMI:

Body mass index

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Funding

Funding for this project was provided by the National Heart, Lung, and Blood Institute (NHLBI U01 HL068270, HL109741, HL109781, HL109816, HL109818, HL109777, HL109778, HL109673, HL109743, HL109737, HL068270). The views expressed are those of the authors, and do not represent official positions of NHLBI or NIH.

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Authors and Affiliations

  1. Division of Cardiology, The Children’s Hospital of Philadelphia, Perelman School of Medicine, 34th Street and Civic Center Blvd, Philadelphia, PA, 19104, USA

    David J. Goldberg & Stephen M. Paridon

  2. Healthcore, Watertown, MA, 02472, USA

    Victor Zak, Hua Ni & Russell Gongwer

  3. The Hospital for Sick Children, University of Toronto, Toronto, ON, M5G 1X8, Canada

    Brian W. McCrindle

  4. Children’s Hospital Boston, Harvard Medical School, Boston, MA, 02115, USA

    Jonathan Rhodes & Renee Margossian

  5. Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NY, 10032, USA

    Robert P. Garofano

  6. Division of Cardiovascular Sciences, National Heart, Lung, and Blood Institute, NIH, Bethesda, MD, 20892, USA

    Jonathan R. Kaltman

  7. Primary Children’s Hospital, University of Utah, Salt Lake City, UT, 84132, USA

    Linda M. Lambert & Richard V. Williams

  8. Children’s Health Dallas, University of Texas Southwestern Medical School, Dallas, TX, 75390, USA

    Lynn Mahony

  9. Duke University School of Medicine, Durham, NC, 27710, USA

    Zebulon Z. Spector

  10. Medical University of South Carolina, Charleston, SC, 29425, USA

    Andrew M. Atz

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  1. David J. Goldberg
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  15. Stephen M. Paridon
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For the Pediatric Heart Network Investigators

Contributions

All authors contributed to the study conception and design and to manuscript preparation and review. Data collection and analysis were performed at Healthcore. The first draft of the manuscript was written by David J. Goldberg and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to David J. Goldberg.

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The authors declare that they have no conflict of interest.

Ethical Approval

This study was approved by the institutional review board at each participating institution and complied with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments.

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All participants and/or their guardian provided informed consent and / or assent, as indicated.

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Goldberg, D.J., Zak, V., McCrindle, B.W. et al. Exercise Capacity and Predictors of Performance After Fontan: Results from the Pediatric Heart Network Fontan 3 Study. Pediatr Cardiol 42, 158–168 (2021). https://doi.org/10.1007/s00246-020-02465-1

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  • DOI: https://doi.org/10.1007/s00246-020-02465-1

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