Lawrenz Wolfgang, Krogmann Otto N, Wieczorek Marcus
1Clinic for Pediatric Cardiology,Congenital Heart Disease,Heart Center Duisburg,Duisburg,Germany.
2Clinic for Electrophysiology,Evangelic Hospital Dinslaken,Dinslaken,Germany.
Cardiol Young. 2014 Aug;24(4):741-4. doi: 10.1017/S1047951113001091. Epub 2013 Aug 28.
Catecholaminergic polymorphic ventricular tachycardia is a rare life-threatening arrhythmogenic disorder. An association with paroxysmal atrial fibrillation and other atrial arrhythmias has been described, but in all published cases the initial manifestation of the disease was ventricular arrhythmia. This is the first report about a patient who presented with complex atrial tachycardia and sinus node dysfunction about 1 year before the typical ventricular arrhythmias were observed, leading to the diagnosis of catecholaminergic polymorphic ventricular tachycardia. In this girl, a mutation of the ryanodine receptor type 2 gene, which has not been described so far, was discovered.
儿茶酚胺能多形性室性心动过速是一种罕见的危及生命的致心律失常性疾病。已有报道称其与阵发性心房颤动及其他房性心律失常有关,但在所有已发表的病例中,该疾病的初始表现均为室性心律失常。本文首次报道了一名患者,在观察到典型室性心律失常约1年前,先出现了复杂房性心动过速和窦房结功能障碍,最终诊断为儿茶酚胺能多形性室性心动过速。在这个女孩身上,发现了迄今尚未见报道的2型兰尼碱受体基因突变。