Fazio Giovanni, Corrado Giovanni, Zachara Elisabetta, Rapezzi Claudio, Sulafa Ali K, Sutera Loredana, Stollberger Claudia, Sormani Luca, Finsterer Joseph, Benatar Abraham, Di Gesaro Gabriele, Visconti Claudia, D'angelo Luciana, Novo Giuseppina, Mongiovi Maurizio, Cavusoglu Yuksel, Baumhakel Magnus, Drago Fabrizio, Indovina Giuseppe, Ferrara Filippo, Carerj Scipione, Pipitone Salvatore, Novo Salvatore
Department of Cardiology, University of Palermo, Palermo, Italy.
J Cardiovasc Med (Hagerstown). 2008 Nov;9(11):1095-7. doi: 10.2459/JCM.0b013e328308da04.
Noncompaction of left ventricular myocardium is a rare congenital cardiomyopathy resulting from an incomplete myocardial morphogenesis that leads to the persistence of the embryonic myocardium. This condition is characterized by a thin compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep intertrabecular recesses. It is not clear, in noncompaction of myocardium, whether intertrabecular recesses could be responsible for thrombi formation and thromboembolic complications.
The prevalence of stroke and echocardiographic finding of thrombus was evaluated in a continuous series of 229 patients (men and women) affected by noncompaction of the left ventricular myocardium, who were included in the SIEC registry. We excluded patients affected by atrial fibrillation.
The mean age of the patients was 49.5 years. Fifty percent of the patients were affected by a ventricular systolic dysfunction. The mean period of follow-up was 7.3 years. Only four patients had a history of ischemic stroke. A large thrombus into the left ventricular chamber was observed in a 1-year-old child affected by Behcet's disease (high risk of thrombi formation).
Noncompaction of the left ventricular myocardium, by itself, does not seem to be a risk factor for stroke or embolic results, so there is no indication for oral anticoagulant therapy.
左心室心肌致密化不全是一种罕见的先天性心肌病，由心肌形态发生不完全导致胚胎心肌持续存在引起。这种疾病的特征是薄的致密心外膜和极度增厚的心内膜层，伴有明显的小梁和深陷的小梁间隐窝。在心肌致密化不全中，小梁间隐窝是否会导致血栓形成和血栓栓塞并发症尚不清楚。
在纳入SIEC登记处的连续229例（男性和女性）左心室心肌致密化不全患者中，评估中风患病率和血栓的超声心动图表现。我们排除了患有心房颤动的患者。
患者的平均年龄为49.5岁。50%的患者存在心室收缩功能障碍。平均随访期为7.3年。只有4例患者有缺血性中风病史。在一名患有白塞病（血栓形成高风险）的1岁儿童中观察到左心室内有一个大血栓。
左心室心肌致密化不全本身似乎不是中风或栓塞结果的危险因素，因此没有口服抗凝治疗的指征。
