Authors: Thomas D Ryan Michael D Taylor Wojciech Mazur Linda H Cripe Jesse Pratt Eileen C King Kathleen Lao Michelle A Grenier John L Jefferies D Woodrow Benson Kan N Hor
Publish Date: 2013/01/29
Volume: 34, Issue: 5, Pages: 1159-1165
Abstract
Advances in management of noncardiac issues in Duchenne muscular dystrophy DMD have improved such that DMDassociated cardiac disease has become the leading cause of death for such patients Cardiac dysfunction measured by standard transthoracic echocardiographic methods eg fractional shortening FS and ejection fraction EF is rarely present during the first decade of life The current study used transthoracic echocardiogram TTE to assess strain ε an indicator of regional ventricular function in young DMD patients A retrospective review of the TTE database was performed TTE results from DMD patients 8 years n = 63 performed during 2009 to 2010 were compared with TTE results from an unaffected control group n = 61 Feature tracking analysis software was used to measure total circumferential strain ε cc as well as segmental ε cc based on the American Society of Echocardiography 16segment model Although there were no differences in FS the absolute value for leftventricular LV ε cc at the midchamber level was decreased in DMD −217 ± 38 vs −198 ± 42 p 001 unaffected vs DMD Segmental ε cc was similarly affected in the anteroseptal segment −230 ± 61 vs −189 ± 70 p = 0001 controls vs DMD the inferior segment −207 ± 516 vs −177 ± 61 p = 0003 controls vs DMD and the inferolateral segment −183 ± 62 vs −159 ± 67 p = 004 controls vs DMD In the present study we demonstrate both total and segmental LV ε cc anteroseptal inferior and inferolateral segments abnormalities at the midchamber level in a large group of young DMD patients with normal FS These novel findings substantiate that the disease process is present and results in abnormal myocardial function before standard measures detect global dysfunctionThe authors thank the sonographers in the Heart Institute at the Cincinnati Children’s Hospital Medical Center for their work performing the studies used in this investigation Additional thanks are due to the Heart Institute Research Core for assistance in study design and approval
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