Finsterer Josef, Stöllberger Claudia, Gaismayer Kurt, Janssen Bart
Int J Cardiol. 2006 Jan 26;106(3):420-1. doi: 10.1016/j.ijcard.2005.01.018.
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.
获得性左心室肌小梁增多/心肌致密化不全（LVHT）较为罕见，已在患有线粒体病、巴特综合征和贝克型肌营养不良症的患者中有所描述。在此，我们报告一名28岁的杜氏肌营养不良症男性患者出现获得性LVHT，该患者自16岁起因肌肉呼吸衰竭需要无创正压通气。22岁时经胸超声心动图显示左心房和左心室增大，缩短分数降低，但未发现LVHT。27岁时经胸超声心动图显示左心房和左心室增大、收缩功能障碍、二尖瓣关闭不全，且令人惊讶的是出现了LVHT。该患者获得性LVHT的病因和发病机制仍属推测。
