The Unique Clinical Phenotype and Exercise Adaptation of Fontan Patients With Normal Exercise Capacity.


Journal

The Canadian journal of cardiology
ISSN: 1916-7075
Titre abrégé: Can J Cardiol
Pays: England
ID NLM: 8510280

Informations de publication

Date de publication:
09 2020
Historique:
received: 26 09 2019
revised: 06 11 2019
accepted: 07 11 2019
pubmed: 15 4 2020
medline: 19 5 2021
entrez: 15 4 2020
Statut: ppublish

Résumé

Exercise limitation is almost universal among Fontan patients. Identifying unique clinical features in the small fraction of Fontan patients with normal exercise capacity (high-capacity Fontan [HCF]) provides potential to inform clinical strategies for those with low exercise capacity (usual Fontan). We performed a retrospective chart review of all patients with single-ventricle physiology palliated with a Fontan operation who underwent incremental cardiopulmonary exercise testing at Cincinnati Children's Hospital Medical Center from 2013 to 2018. Comparison was between patients with peak oxygen uptake < vs ≥ 80% predicted. A total of 22 of 112 patients were classified as HCF (68% were female; aged 18 ± 7 years). During incremental exercise, peak oxygen uptake (86.1% ± 6.1% vs 62% ± 12.2% predicted; P < 0.001) was greater in HCF vs usual Fontan despite similar chronotropic impairment, resulting in a greater oxygen pulse in HCF. Pulmonary function, breathing reserve, and ventilatory equivalent for CO Approximately 1 in 5 Fontan patients who undergo cardiopulmonary exercise testing have normal exercise capacity despite chronotropic impairment. This implies a better preserved stroke volume, perhaps due to greater muscle pump-mediated preload. Additionally, a complicated perioperative Fontan course is associated with eventual impaired functional capacity.

Sections du résumé

BACKGROUND
Exercise limitation is almost universal among Fontan patients. Identifying unique clinical features in the small fraction of Fontan patients with normal exercise capacity (high-capacity Fontan [HCF]) provides potential to inform clinical strategies for those with low exercise capacity (usual Fontan).
METHODS
We performed a retrospective chart review of all patients with single-ventricle physiology palliated with a Fontan operation who underwent incremental cardiopulmonary exercise testing at Cincinnati Children's Hospital Medical Center from 2013 to 2018. Comparison was between patients with peak oxygen uptake < vs ≥ 80% predicted.
RESULTS
A total of 22 of 112 patients were classified as HCF (68% were female; aged 18 ± 7 years). During incremental exercise, peak oxygen uptake (86.1% ± 6.1% vs 62% ± 12.2% predicted; P < 0.001) was greater in HCF vs usual Fontan despite similar chronotropic impairment, resulting in a greater oxygen pulse in HCF. Pulmonary function, breathing reserve, and ventilatory equivalent for CO
CONCLUSIONS
Approximately 1 in 5 Fontan patients who undergo cardiopulmonary exercise testing have normal exercise capacity despite chronotropic impairment. This implies a better preserved stroke volume, perhaps due to greater muscle pump-mediated preload. Additionally, a complicated perioperative Fontan course is associated with eventual impaired functional capacity.

Identifiants

pubmed: 32284163
pii: S0828-282X(19)31430-8
doi: 10.1016/j.cjca.2019.11.006
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

1499-1507

Informations de copyright

Copyright © 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

Auteurs

Adam W Powell (AW)

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA; The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. Electronic address: Adam.powell@cchmc.org.

Clifford Chin (C)

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA; The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Tarek Alsaied (T)

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA; The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Harry B Rossiter (HB)

Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center, Torrance, California, USA; Faculty of Biological Sciences, University of Leeds, Leeds, United Kingdom.

Samuel Wittekind (S)

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA; The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Wayne A Mays (WA)

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA; The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Adam Lubert (A)

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA; The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Gruschen Veldtman (G)

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA; The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

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