Celiker Alpay, Ozkutlu Süheyla, Dilber Embiya, Karagöz Tevfik
Department of Pediatric Cardiology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Pacing Clin Electrophysiol. 2005 Nov;28(11):1198-202. doi: 10.1111/j.1540-8159.2005.09498.x.
Isolated ventricular noncompaction (IVNC) is a serious cardiomyopathy with a generally poor prognosis. It is characterized by the presence of prominent ventricular myocardial trabeculations and deep intertrabecular recesses, in the absence of other structural heart defects. This cardiomyopathy is usually associated with ventricular dysfunction, thromboembolic events, and rhythm problems.
This article describes 11 children who have rhythm abnormalities associated with IVNC. On admission, eight children had complaints including palpitation, syncope, heart failure, and bradycardia. Ventricular arrhythmias were diagnosed in four children, sinus node and atrioventricular conductance disturbances in six children, and Wolff-Parkinson-White syndrome and associated tachycardia in one child. Three children with ventricular arrhythmias received an automatic implantable cardioverter defibrillator (ICD). Three patients died of cardiac problems during the follow-up period.
Since many of rhythm abnormalities, including life-threatening ventricular arrhythmias, may be seen in patients with IVNC, children with IVNC should be screened for arrhythmias. An ICD may be the best treatment for some of these patients.
孤立性心室肌致密化不全（IVNC）是一种严重的心肌病，预后通常较差。其特征是存在显著的心室心肌小梁和深陷的小梁间隐窝，且无其他结构性心脏缺陷。这种心肌病通常与心室功能障碍、血栓栓塞事件及节律问题相关。
本文描述了11例与IVNC相关的节律异常儿童。入院时，8名儿童有包括心悸、晕厥、心力衰竭和心动过缓等主诉。4名儿童被诊断为室性心律失常，6名儿童为窦房结和房室传导障碍，1名儿童为预激综合征及相关心动过速。3名室性心律失常儿童接受了植入式自动心脏复律除颤器（ICD）治疗。3例患者在随访期间死于心脏问题。
由于IVNC患者可能出现多种节律异常，包括危及生命的室性心律失常，因此应对IVNC儿童进行心律失常筛查。ICD可能是其中一些患者的最佳治疗方法。
