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Association between pulmonary function and left ventricular volume and function in duchenne muscular dystrophy
Author(s) -
Khokhar Arshjot,
Nair Athira,
Midya Vishal,
Kumar Ashutosh,
Sinharoy Ankita,
Ahmad Tariq Ali,
AbuHasan Mutasim,
Mondal Pritish
Publication year - 2019
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.26623
Subject(s) - duchenne muscular dystrophy , medicine , cardiology , vital capacity , pulmonary function testing , ejection fraction , stroke volume , muscular dystrophy , dystrophin , heart failure , lung , lung function , diffusing capacity
ABSTRACT Introduction : Duchenne muscular dystrophy (DMD) is characterized by absence of the subsarcolemmal protein dystrophin, present in skeletal muscles and cardiomyocytes. We hypothesized that progressive respiratory and left ventricular (LV) insufficiencies in DMD could be parallel and interrelated phenomena. Methods : We conducted a retrospective chart review of 27 patients with DMD. Our primary objective was to compare the rates of decline between pulmonary function test (PFT) measures (forced expiratory volume in the first second, forced vital capacity, peak expiratory flow rate, maximal inspiratory/expiratory pressure) and echocardiographic estimates of LV end‐diastolic volume and LV ejection fraction. Results : The rates of decline/year of PFTs and LV estimates were not significantly different. Pulmonary function test measures of ventilatory efficiency and strength had strong intercorrelations. Pulmonary function tests and LV estimates had weak but statistically significant correlations. Discussion : A comparable rate of decline in PFTs and LV indices in DMD provides evidence for concurrently progressive deterioration in respiratory and LV functions. Muscle Nerve , 2019