Gerecke B, Engberding R
I. Medizinische Klinik, Klinikum Wolfsburg, Sauerbruchstraße 7, 38440, Wolfsburg, Germany.
Herzschrittmacherther Elektrophysiol. 2012 Sep;23(3):201-10. doi: 10.1007/s00399-012-0226-6. Epub 2012 Sep 25.
Isolated noncompaction cardiomyopathy (NCCM) is a rare genetically determined myocardial disease caused by abnormal fetal development of the myocardium resulting in a thin compacted and a thicker noncompacted layer of the affected left ventricular (LV) wall. The genetic basis of NCCM is heterogenous. Diagnosis can be made using echocardiography or magnetic resonance imaging. The diagnostic criteria for NCCM are still under discussion. Afflicted patients may present with various symptoms caused by arrhythmias, heart failure and cardioembolic events. Severely reduced LV function as well as left bundle branch block and atrial fibrillation were shown to be linked to worse outcomes. Treatment in patients with NCCM should be targeted at individual symptoms and clinical findings. Therapy includes pharmacological treatment, and in individual cases ablation or device therapy, as well as consideration for heart transplantation in selected cases. Aside from regular clinical follow-up of patients with NCCM screening of first degree family members with assessment of medical history, physical examination, ECG recording, and echocardiography are recommended.
孤立性心肌致密化不全（NCCM）是一种罕见的遗传性心肌病，由心肌胎儿发育异常引起，导致受累左心室（LV）壁的致密层变薄，非致密层增厚。NCCM的遗传基础是异质性的。可使用超声心动图或磁共振成像进行诊断。NCCM的诊断标准仍在讨论中。患病患者可能会出现由心律失常、心力衰竭和心脏栓塞事件引起的各种症状。左心室功能严重降低以及左束支传导阻滞和心房颤动与较差的预后有关。NCCM患者的治疗应针对个体症状和临床发现。治疗包括药物治疗，个别情况下进行消融或器械治疗，以及在特定病例中考虑心脏移植。除了对NCCM患者进行定期临床随访外，建议对一级家庭成员进行筛查，评估病史、体格检查、心电图记录和超声心动图。
