Frommeyer G, Pott C, Schulze-Bahr E, Eckardt L
Abteilung für Rhythmologie Department für Kardiologie und Angiologie, Universitätsklinikum Münster, Albert-Schweitzer Campus 1, 48149, Münster, Germany.
Herzschrittmacherther Elektrophysiol. 2012 Sep;23(3):231-6. doi: 10.1007/s00399-012-0224-8. Epub 2012 Sep 18.
Catecholaminergic polymorphic ventricular tachycardia (PCVT) is a rare, congenital ventricular tachyarrhythmia which occurs in the setting of adrenergic activation. It potentially leads to syncope and/or sudden cardiac death (SCD). PCVT represents one of the most dangerous congenital ion channel diseases. Mutations of the ryanodine receptor gene (RYR2), the calsequestrin gene (CASQ2), and the triadin gene (TRDN) have been identified as an underlying correlate. β-Blockers are employed as therapy and are sometimes combined with class IC antiarrhythmic drugs, or calcium antagonists of the verapamil type. ICD implantation is recommended in case of persisting syncope in the presence of β-blocker therapy or survived SCD. Left thoracic sympathectomy represents a subsidiary interventional therapy for individual cases. In addition, modifications of the patient's lifestyle including avoidance of physical stress and heart rates> 120/min are recommended.
儿茶酚胺能多形性室性心动过速（PCVT）是一种罕见的先天性室性心律失常，发生于肾上腺素能激活的情况下。它可能导致晕厥和/或心源性猝死（SCD）。PCVT是最危险的先天性离子通道疾病之一。已确定ryanodine受体基因（RYR2）、肌集钙蛋白基因（CASQ2）和三联蛋白基因（TRDN）的突变是其潜在相关因素。β受体阻滞剂用作治疗药物，有时与IC类抗心律失常药物或维拉帕米型钙拮抗剂联合使用。在β受体阻滞剂治疗期间仍有持续性晕厥或心源性猝死幸存者，建议植入植入式心脏复律除颤器（ICD）。左胸交感神经切除术是个别病例的辅助介入治疗方法。此外，建议改变患者的生活方式，包括避免身体应激和心率>120次/分钟。
