Predictors of early left ventricular systolic dysfunction in duchenne muscular dystrophy patients |
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| Authors: | Raphael Henrique Déa Cirino MD Rosana Herminia Scola MD PhD Renata Dal‐Prá Ducci MD PhD Ana Cristina Camarozano Wermelinger MD PhD Claudia Suemi Kamoi Kay MD Paulo José Lorenzoni MD PhD Lineu Cesar Werneck MD PhD Eliane Ribeiro Carmes MD MSc Claudio Leinig Pereira Da Cunha MD PhD |
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| Affiliation: | 1. Cardiology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Rua General Carneiro, 181–12?o andar Curitiba, Paraná, Brazil;2. Neuromuscular Service, Neurology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil;3. Department of Internal Medicine, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Paraná, Brazil |
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| Abstract: | Introduction: Early detection of left ventricular systolic dysfunction (LVSD) is important for therapeutic strategies for Duchenne muscular dystrophy (DMD) patients. We analyzed myocardial strain using echocardiography for early detection of LVSD and determined the predictors of early LVSD. Methods: This investigation was a cross‐sectional study of 40 DMD patients with normal left ventricular ejection fraction. Global longitudinal strain (GLS) was used to analyze subtle disturbances in longitudinal contraction of the myocardium. Patients were determined to have early LVSD (GLS > –18) or normal left ventricular systolic function (GLS ≤ –18). Results: Patients who had early LVSD were older and had a higher frequency of corticosteroid therapy and of mutations in exons 45, 46, 47, 48, 49, 50, and 52. Discussion: Myocardial strain measurements are useful for the early diagnosis of LVSD in DMD patients. Older age, use of corticosteroids, and mutations within the “hot‐spot” region of the DMD gene are associated with early LVSD. Muscle Nerve 58 : 84–89, 2018 |
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| Keywords: | Duchenne muscular dystrophy early diagnosis echocardiography left ventricular dysfunction myocardial strain speckle tracking |
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