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- Title
Outcomes of Cardiac Involvement in Patients with Late-Stage Duchenne Muscular Dystrophy under Management in the Pulmonary Rehabilitation Center of a Tertiary Referral Hospital.
- Authors
Kwon, Sung Woo; Kang, Seong-Woong; Kim, Jong-Youn; Choi, Eui-Young; Yoon, Young Won; Park, Yoo Mi; Ma, Dae Won; Chung, Hyemoon; Kwon, Hyuck Moon; Rim, Se-Joong
- Abstract
Objectives: The purpose of this study was to investigate the clinical outcome as well as the sequential changes of cardiac function in late-stage Duchenne muscular dystrophy (DMD) patients by 2-dimensional echocardiography. Methods: A total of 31 individuals (initial age: 21.6 ± 5.0 years, range: 15-35 years) with late-stage DMD (Swinyard-Deaver's stage 7 or 8) were enrolled. All of these patients had respiratory insufficiency and were on ventilator support. Sequential echocardiographic data were collected over at least 3 years. Repeated measures analysis of variance was used to compare changes in left ventricular ejection fraction (LVEF) over time. Results: The sequential change in the mean LVEF showed no significant differences with initial, 1-, 2-, and 3-year follow-up LVEFs which were 42.2, 42.9, 43.8 and 42.6%, respectively (p = 0.320). In terms of the clinical outcome, all but 1 patient survived during the follow-up period of 46.5 ± 9.1 months. Conclusions: The cardiac function in late-stage DMD patients showed a stabilization of LVEF on adequate ventilatory support and optimal cardiac medication therapy until their mid-30s. In addition, considering the favorable clinical outcome in our study, the process of cardiac involvement in late-stage DMD may demonstrate that in some patients it is nonprogressive. Copyright © 2012 S. Karger AG, Basel
- Subjects
DUCHENNE muscular dystrophy; DILATED cardiomyopathy; ECHOCARDIOGRAPHY; RESPIRATORY insufficiency; ANALYSIS of variance; THERAPEUTICS; HEART diseases
- Publication
Cardiology, 2012, Vol 121, Issue 3, p186
- ISSN
0008-6312
- Publication type
Article
- DOI
10.1159/000336810