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.
abstract: OBJECTIVE
  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.
authors.affiliation: Division of Cardiology, Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.
authors.forename: Sung Woo
authors.initials: SW
authors.lastname: Kwon
authors.forename: Seong-Woong
authors.initials: SW
authors.lastname: Kang
authors.forename: Jong-Youn
authors.initials: JY
authors.lastname: Kim
authors.forename: Eui-Young
authors.initials: EY
authors.lastname: Choi
authors.forename: Young Won
authors.initials: YW
authors.lastname: Yoon
authors.forename: Yoo Mi
authors.initials: YM
authors.lastname: Park
authors.forename: Dae Won
authors.initials: DW
authors.lastname: Ma
authors.forename: Hyemoon
authors.initials: H
authors.lastname: Chung
authors.forename: Hyuck Moon
authors.initials: HM
authors.lastname: Kwon
authors.forename: Se-Joong
authors.initials: SJ
authors.lastname: Rim
mesh_terms: D000293:Adolescent; D000328:Adult; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006331:Heart Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000628:therapy; D006760:Hospitalization; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000000981:diagnostic imaging / Q000628:therapy; D011175:Positive-Pressure Respiration; D012047:Rehabilitation Centers; D013318:Stroke Volume; D018487:Ventricular Dysfunction, Left; D055815:Young Adult