February 2016 Vol. 4 No.2

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Original Research Article

Left ventricular function in Duchenne muscular dystrophy with relation to some clinical parameters

M. Gospodinova¹, T. Chamova², A. Todorova^4,5, S. Sarafov², I. Tournev*^2,3 and S. Denchev*¹

¹Clinic of Cardiology, Medical Institute of Ministry of Interior, Sofia, Bulgaria
²Clinic of Neurology, University Hospital Alexandrovska, Sofia, Bulgaria
³Department of cognitive science and psychology, New Bulgarian University, Sofia, Bulgaria
⁴Genetic laboratory “Genika”
⁵Department of biochemistry, Medical University, Sofia

*Corresponding Author’s E-mail: maryvg2009@yahoo.com
Tel: +3598982547382

Accepted February 04, 2016

Abstract

Duchenne muscular dystrophy (DMD) is a severe X-linked disease due to loss of dystrophin in skeletal and cardiac muscle. The aim of the study was to evaluate cardiac involvement in DMD patients and to correlate it with age, ventilatory function and muscle strength. Forty five male, genetically verified DMD patients at mean age of 11,5±4,3 years, underwent clinical examination, ECG and transthoracic еchocardiography. Forced vital capacity and forced expiratory volume in 1 s were assessed. North star assessment score and Medical Research Council grading method were used for muscle strength and functional disability evaluation. Left ventricular systolic (LV) dysfunction was found in 24,4% of the patients and some impairment in diastolic function in 53.3%. The earliest finding was decreased early diastolic myocardial velocities at lateral mitral annulus, registered in patients 10 years of age. A positive correlation was found between muscle weakness parameters and ventilatory function with some of the indices of LV function. LV dysfunction was a common finding in DMD and its prevalence and severity increased with age. The parallel decrease in peripheral, respiratory and cardiac muscle function confirmed the common pathological molecular mechanism of muscle impairment.

Key words: Cardiac involvement, Duchenne muscular dystrophy, Functional disability, LV function, Muscle strength, Ventilatory function