The Association between Pulmonary Function and Left Ventricular Volume and Function in Duchenne Muscular Dystrophy.

Khokhar A, Nair A, Midya V, et al. The Association between Pulmonary Function and Left Ventricular Volume and Function in Duchenne Muscular Dystrophy. Muscle & nerve 2019.

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 inter-related phenomena.

METHODS: We conducted a retrospective chart review of 27 DMD patients. The primary objective was to compare the rates of decline between pulmonary function test (PFT) measures (forced expiratory volume in one 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. PFT measures of ventilatory efficiency and strength had strong inter-correlations. PFTs and LV estimates had weak but statistically significant correlations.

DISCUSSION: A comparable rate of decline in PFTs and LV indices in DMD suggest concurrently progressive deterioration in respiratory and LV functions.

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