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.
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 DMDpatients 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: Christopher F Spurney; Francis M McCaffrey; Avital Cnaan; Lauren P Morgenroth; Sunil J Ghelani; Heather Gordish-Dressman; Adrienne Arrieta; Anne M Connolly; Timothy E Lotze; Craig M McDonald; Robert T Leshner; Paula R Clemens Journal: J Am Soc Echocardiogr Date: 2015-04-21 Impact factor: 5.251
Authors: Larry W Markham; Jonathan H Soslow; Aryaz Sheybani; Kim Crum; Frank J Raucci; William B Burnette Journal: Pediatr Res Date: 2021-08-24 Impact factor: 3.756
Authors: Abdelrahim Abdrabou Sadek; Shaimaa Mohamed Mahmoud; Mohammed Abd El-Aal; Ahmed Ahmed Allam; Walaa Ibrahim Abd El-Halim Journal: Electron Physician Date: 2017-11-25
Authors: Sven Dittrich; Erika Graf; Regina Trollmann; Ulrich Neudorf; Ulrike Schara; Antje Heilmann; Maja von der Hagen; Brigitte Stiller; Janbernd Kirschner; Robert Dalla Pozza; Wolfgang Müller-Felber; Katja Weiss; Katja von Au; Markus Khalil; Reinald Motz; Christoph Korenke; Martina Lange; Ekkehard Wilichowski; Joseph Pattathu; Friedrich Ebinger; Nicola Wiechmann; Rolf Schröder Journal: Orphanet J Rare Dis Date: 2019-05-10 Impact factor: 4.123