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Predictors of early left ventricular systolic dysfunction in duchenne muscular dystrophy patients
Author(s) -
Cirino Raphael Henrique Déa,
Scola Rosana Herminia,
Ducci Renata DalPrá,
Wermelinger Ana Cristina Camarozano,
Kay Claudia Suemi Kamoi,
Lorenzoni Paulo José,
Werneck Lineu Cesar,
Carmes Eliane Ribeiro,
Da Cunha Claudio Leinig Pereira
Publication year - 2018
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.26102
Subject(s) - duchenne muscular dystrophy , medicine , cardiology , ejection fraction , muscular dystrophy , contraction (grammar) , heart failure
: 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