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Outcomes of cardiac involvement in patients with late-stage Duchenne muscular dystrophy under management in the pulmonary rehabilitation center of a tertiary referral hospital.

 Kwon S.W.  ;  Kang S.-W.  ;  Kim J.-Y.  ;  Choi E.-Y.  ;  Yoon Y.W.  ;  Park Y.M.  ;  Ma D.W.  ;  Chung H.  ;  Kwon H.M.  ;  Rim S.-J. 
 CARDIOLOGY, Vol.121(3) : 186-193, 2012 
Journal Title
Issue Date
Adolescent ; Adult ; Echocardiography ; Electrocardiography ; Female ; Heart Diseases/diagnostic imaging ; Heart Diseases/etiology* ; Heart Diseases/therapy ; Hospitalization ; Humans ; Male ; Muscular Dystrophy, Duchenne/complications* ; Muscular Dystrophy, Duchenne/diagnostic imaging ; Muscular Dystrophy, Duchenne/therapy ; Positive-Pressure Respiration ; Rehabilitation Centers ; Stroke Volume ; Ventricular Dysfunction, Left ; Young Adult
Duchenne muscular dystrophy ; Left ventricular ejection fraction ; Cardiac involvement ; Dilated cardiomyopathy ; Echocardiography
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.
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1. College of Medicine (의과대학) > Dept. of Rehabilitation Medicine (재활의학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Kang, Seong Woong(강성웅) ORCID logo https://orcid.org/0000-0002-7279-3893
Kwon, Sung Woo(권성우)
Kwon, Hyuck Moon(권혁문) ORCID logo https://orcid.org/0000-0001-9901-5015
Kim, Jong Youn(김종윤) ORCID logo https://orcid.org/0000-0001-7040-8771
Yoon, Young Won(윤영원) ORCID logo https://orcid.org/0000-0002-0907-0350
Rim, Se Joong(임세중) ORCID logo https://orcid.org/0000-0002-7631-5581
Choi, Eui Young(최의영) ORCID logo https://orcid.org/0000-0003-3732-0190
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