Kalavakunta Jagadeesh K, Tokala Hemasri, Gosavi Aparna, Gupta Vishal
Department of Internal Medicine: Division of Cardiology, Michigan State University/Kalamazoo Center for Medical Studies/Borgess Medical Center, Kalamazoo, MI, USA.
Int Arch Med. 2010 Sep 15;3:20. doi: 10.1186/1755-7682-3-20.
Left ventricular noncompaction (LVNC) is a rare congenital abnormality. It is currently classified as a genetic cardiomyopathy and results from early arrest of endomyocardial morphogenesis. The pathophysiology of left ventricular dysfunction, which becomes apparent beyond the 4th decade of life, is unclear.
We report a case of 60-year-old woman who presented with shortness of breath and showed noncompacted endocardium on echocardiography. Cardiac catheterization and viral studies were unremarkable. Histology revealed endomyocardial fibrosis without disarray. She was subsequently diagnosed with LVNC and treated with medications.
Cardiologists and other physicians should be aware of LVNC due to its high likelihood of misdiagnosis and associated high complication rates. Early diagnosis, intervention and screening among family members can decrease the morbidity and mortality associated with LVNC.
左心室心肌致密化不全（LVNC）是一种罕见的先天性异常。它目前被归类为遗传性心肌病，是心内膜形态发生早期停滞的结果。左心室功能障碍的病理生理学在生命的第四个十年后变得明显，目前尚不清楚。
我们报告一例60岁女性，她因呼吸急促就诊，超声心动图显示心内膜致密化不全。心导管检查和病毒学研究均无异常。组织学显示心内膜纤维化但无排列紊乱。她随后被诊断为LVNC并接受药物治疗。
心脏病专家和其他医生应了解LVNC，因为其误诊可能性高且相关并发症发生率高。早期诊断、干预以及对家庭成员进行筛查可降低与LVNC相关的发病率和死亡率。
