Finsterer Josef, Gelpi Ellen, Stöllberger Claudia
Department of Neurology, Krankenanstalt Rudolfstiftung, Vienna, Austria.
Acta Cardiol. 2005 Aug;60(4):445-8. doi: 10.2143/AC.60.4.2004996.
Though cardiac involvement is a frequent finding in patients with Duchenne muscular dystrophy (DMD) at the wheel-chair-bound stage, left ventricular hypertrabeculation (LVHT) has not been reported. The patient is a 28-year-old male with the typical clinical features of end stage DMD. Since the age of 16 years he required ventilatory support by means of non-invasive positive-pressure ventilation (NIPPV), initially intermittently and since age 27 permanently. Since the age of 21 years he developed chronic heart failure, and since age 24 atrial flutter, successfully treated with amiodarone. After discontinuation of amiodarone because of hyperthyroidism, he developed atrial fibrillation. Echocardiography at age 28 revealed mitral insufficiency, enlarged left atrial diameter, and, surprisingly, LVHT. Since LVHT was absent at the initial echocardiographic examination, it was regarded acquired. The case shows that cardiac involvement in DMD may not only comprise rhythm abnormalities, valve abnormalities, and dilative cardiomyopathy, but also LVHT in single cases.
尽管在杜兴氏肌营养不良症（DMD）患者处于轮椅依赖阶段时，心脏受累是常见表现，但左心室肌小梁增多（LVHT）尚未见报道。该患者为一名28岁男性，具有典型的终末期DMD临床特征。自16岁起，他需要通过无创正压通气（NIPPV）进行通气支持，最初是间歇性的，从27岁起变为永久性的。自21岁起，他出现慢性心力衰竭，24岁起出现心房扑动，用胺碘酮成功治疗。因甲状腺功能亢进停用胺碘酮后，他发展为心房颤动。28岁时的超声心动图显示二尖瓣关闭不全、左心房直径增大，令人惊讶的是还有LVHT。由于初次超声心动图检查时未发现LVHT，故认为是后天获得性的。该病例表明，DMD患者的心脏受累不仅可能包括节律异常、瓣膜异常和扩张型心肌病，个别情况下还可能出现LVHT。
