Long-Term Follow-Up of Patients with Catecholaminergic Polymorphic Ventricular Arrhythmia

Journal of Clinical Medicine

Siegfried Lang¹  Xiaobo Zhou¹  Ibrahim El-Battrawy¹  Laura Raschwitz¹  Martin Borggrefe¹  Rainer Schimpf¹  Ibrahim Akin¹  Michael Veith¹  Gretje Roterberg¹  Christian Wolpert¹ 

[1] First Department of Medicine, Faculty of Medicine, University Medical Centre Mannheim (UMM), University of Heidelberg, Mannheim 68167, Germany;

关键词: cpvt;    sudden cardiac death;    ventricular tachyarrhythmia;    icd;   

DOI  :  10.3390/jcm9040903

来源: DOAJ

【 摘 要 】

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited disorder causing life-threatening arrhythmias. Long-term outcome studies of the channelopathy are limited. Objective: The aim of the present study was to summarize our knowledge on CPVT patients, including the clinical profile treatment approach and long-term outcome. Methods: In this single center study, we retrospectively and prospectively collected data from nine CPVT patients and analyzed them. Results: We reviewed nine patients with CPVT in seven families (22% male), with a median follow-up time of 8.6 years. Mean age at diagnosis was 26.4 12 years. Symptoms at admission were syncope (four patients) and aborted cardiac arrest (four patients). Family history of sudden cardiac death was screened in five patients. In genetic analyses, we found five patients with ryanodine type 2 receptor (RYR2) mutations. Seven patients were treated with beta-blockers, and if symptoms persisted flecainide was added (four patients). Despite beta-blocker treatment, three patients suffered from seven adverse cardiac events. An implantable cardioverter defibrillator was implanted in seven patients (one primary, six secondary prevention). Over the follow-up period, three patients suffered from ventricular tachycardia (ten times) and five patients from ventricular fibrillation (nine times). No one died during follow-up. Conclusion: Our CPVT cohort showed a high risk of cardiac events. Family screening, optimal medical therapy and individualized treatment are necessary in affected patients in referral centers.

【 授权许可】

Unknown