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Marked skeletal muscle deficits are associated with 6-minute walk distance in paediatric pulmonary hypertension

Published online by Cambridge University Press:  11 February 2021

Catherine M. Avitabile*
Affiliation:
Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA
Sofia Saavedra
Affiliation:
Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA
Nithya Sivakumar
Affiliation:
Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA
Elizabeth Goldmuntz
Affiliation:
Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA
Stephen M. Paridon
Affiliation:
Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA
Babette S. Zemel
Affiliation:
Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA Division of Gastroenterology, Hepatology, and Nutrition, Children’s Hospital of Philadelphia, Philadelphia, PA, USA
*
Author for correspondence: Catherine Avitabile, MD, Children’s Hospital of Philadelphia, 3401 Civic Center Blvd, 8NW49 Philadelphia, PA 19104. Phone: 215-590-4040. E-mail: avitabilec@email.chop.edu

Abstract

Background:

Poor growth is common in children with pulmonary hypertension; however, skeletal muscle deficits have not been described and the association between muscle deficits and functional status is unknown.

Methods:

Patients aged 8–18 years with pulmonary hypertension (diagnostic Groups 1, 2, or 3) and World Health Organization functional class I or II underwent dual-energy absorptiometry to measure leg lean mass Z-score (a surrogate for skeletal muscle). Muscle strength was assessed using dynamometry. Physical activity questionnaires were administered. Clinical data, including 6-minute walk distance, were reviewed. Relationships between skeletal muscle, physical activity score, and 6-minute walk distance were assessed by correlations and linear regression.

Results:

Sixteen patients (12.1 ± 3.2 years, 50% female, 56% Group 1, 56% functional class II) were enrolled. Leg lean mass Z-score was significantly less than reference data (−1.40 ± 1.12 versus 0.0 ± 0.9, p < 0.001) and worse in those with functional class II versus I (−2.10 ± 0.83 versus −0.50 ± 0.73, p < 0.01). Leg lean mass Z-score was positively associated with right ventricular systolic function by tricuspid annular plane systolic Z-score (r = 0.54, p = 0.03) and negatively associated with indexed pulmonary vascular resistance (r = −0.78, p < 0.001). Leg lean mass Z-score and forearm strength were positively associated with physical activity score. When physical activity score was held constant, leg lean mass Z-score independently predicted 6-minute walk distance (R2 = 0.39, p = 0.03).

Conclusions:

Youth with pulmonary hypertension demonstrate marked skeletal muscle deficits in association with exercise intolerance. Future studies should investigate whether low leg lean mass is a marker of disease severity or an independent target that can be improved.

Type
Original Article
Copyright
© The Author(s), 2021. Published by Cambridge University Press

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