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

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)...

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Bibliographic Details
Published in:Canadian journal of cardiology 2020-09, Vol.36 (9), p.1499-1507
Main Authors: Powell, Adam W., Chin, Clifford, Alsaied, Tarek, Rossiter, Harry B., Wittekind, Samuel, Mays, Wayne A., Lubert, Adam, Veldtman, Gruschen
Format: Article
Language:English
Subjects:
Adaptation, Physiological - physiology
Adolescent
Exercise Tolerance - physiology
Female
Follow-Up Studies
Fontan Procedure
Heart Defects, Congenital - physiopathology
Heart Defects, Congenital - surgery
Humans
Male
Oxygen Consumption - physiology
Phenotype
Retrospective Studies
Stroke Volume - physiology
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Summary: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 CO2 output slope were not different between groups. Those in the HCF group were more likely to self-report exercise ≥ 4 days/week for at least 30 minutes (77% vs 10%, P < 0.001), have normal systolic function (95% vs 74%, P = 0.003), have fewer postoperative complications (8% vs 36%, P = 0.04), and have shorter post-Fontan length of stay (8 ± 2.8 vs 12.4 ± 0.9 days, P = 0.04). 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. Les patients ayant subi une intervention de Fontan ont presque tous une capacité à l’effort réduite. L’étude des caractéristiques cliniques uniques de la petite proportion de patients ayant subi une telle intervention qui conservent une capacité à l’effort normale (Fontan associé à une capacité à l’effort supérieure, FCPS) pourrait éclairer les stratégies cliniques auprès des patients dont la capacité à l’effort est réduite (Fontan type, FT). Nous avons examiné rétrospectivement les dossiers médicaux de tous les patients présentant une physiologie univentriculaire à laquelle on a remédié par une intervention de Fontan et ayant subi une épreuve d’effort cardiopulmonaire avec augmentation progressive de la charge au centre médical du Childre
ISSN:0828-282X
1916-7075
DOI:10.1016/j.cjca.2019.11.006