Celiker Alpay, Kafali Gülden, Doğan Riza
Department of Pediatric Cardiology, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
Pacing Clin Electrophysiol. 2004 Jan;27(1):104-8. doi: 10.1111/j.1540-8159.2004.00395.x.
Isolated noncompaction of the ventricular myocardium is a rare unclassified cardiomyopathy and is thought to be due to arrest of myocardial morphogenesis. In fetal life, it is characterized by an excessively prominent trabecular meshwork and deep intratrabecular recesses, and occurs in the left ventricle in the absence of structural heart disease. Echocardiography provides evidence for the diagnosis. The noncompacted ventricular myocardium may be accompanied by depressed ventricular function, systemic embolism, Wolff-Parkinson-White syndrome, left bundle branch block, and ventricular arrhythmia. Although onset of symptoms is frequently delayed until adulthood, symptomatic children have a poor prognosis. In this report, we describe a case of 6-year-old girl who had a history of recurrent syncope. Transthoracic echocardiographic examination showed a localized prominent trabeculation and deep intratrabecular recesses at the inferoapical region of the left ventricle. She had several episodes of ventricular fibrillation which was refractory to pharmacological treatment. An implantable cardioverter defibrillator (ICD) was successfully operated three times during follow-up.
孤立性心室心肌致密化不全是一种罕见的未分类心肌病，被认为是由于心肌形态发生停滞所致。在胎儿期，其特征为小梁网络过度突出和小梁内深陷，且发生于无结构性心脏病的左心室。超声心动图为诊断提供依据。致密化不全的心室心肌可能伴有心室功能减退、系统性栓塞、预激综合征、左束支传导阻滞和室性心律失常。虽然症状通常延迟至成年期才出现，但有症状的儿童预后较差。在本报告中，我们描述了一名有反复晕厥病史的6岁女孩。经胸超声心动图检查显示左心室心尖下区域有局限性突出的小梁和深陷的小梁内凹陷。她发生了几次室颤，药物治疗无效。在随访期间，植入式心脏复律除颤器（ICD）成功运行了三次。
