Acquired noncompaction in Duchenne muscular dystrophy

被引:55
作者
Finsterer, J
Stöllberger, C
Gaismayer, K
Janssen, B
机构
[1] Neurol Hosp Rosenhugel, Vienna, Austria
[2] Univ Heidelberg, Inst Human Genet, Heidelberg, Germany
关键词
mitochondriopathy; left ventricular hypertrabeculation; embryonic myocardium;
D O I
10.1016/j.ijcard.2005.01.018
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Acquired left ventricular hypertrabeculation/noncompaction (LVHT) is rare and has been described in patients with mitochondriopathy, Barth syndrome, and Becker muscular dystrophy. Here we report acquired LVHT in a 28-year-old man with Duchenne muscular dystrophy who required non-invasive, positive-pressure ventilation for muscular respiratory failure since age 16 years. Transthoracic echocardiography at age 22 years revealed enlarged left atrium and ventricle and reduced fractional shortening, but no LVHT. Transthoracic echocardiography at age 27 years revealed enlarged left atrium and ventricle, systolic dysfunction, mitral insufficiency, and, surprisingly, LVHT. The cause and pathomechanism of acquired LVHT in this patient remained speculative. (c) 2005 Elsevier Ireland Ltd. All fights reserved.
引用
收藏
页码:420 / 421
页数:2
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