儿茶酚胺敏感性多形性室性心动过速患者的长期预后及室性心律失常的预测

目的：目前对儿茶酚胺敏感性多形性室性心动过速（CPVT）的临床特征的认识相对不足，并缺少完善的评估疗效、预测室性心律失常的方法，本研究对CPVT的治疗及预后进行长期随访，评估室性心律失常的预测指标。方法：6例CPVT患者给予β受体阻滞剂，部分患者给予普罗帕酮，1例患者给予导管消融肾动脉去交感神经术治疗。观察心电图、心律失常特点及长期随访预后。结果：6例患者经治疗后1例未再发生晕厥，3例因停β受体阻滞剂一次而发生晕厥，1例因停服β受体阻滞剂猝死，1例仍反复晕厥后猝死。所有患者于胸导联出现ST段抬高、T波双峰或倒置，4例患者出现T电交替，部分患者可见明显的U波，β受体阻滞剂治疗后这些心电异常消失或减轻。所有患者室性心动过速发作前均有窦性心动过速及频发室性早搏。结论：T波双峰或倒置、T波电交替、U波增高可能是CPVT的心电图特点，观察这些心电异常及有无窦性心动过速、频发室性早搏有助于评估β受体阻滞剂的疗效及预测致死性室性心律失常。β受体阻滞剂的依从性是预测预后的强烈指标。

Long-term outcomes and factors for predicting ventricular arrhythmia in patients with catecholaminergic polymorphic ventricular tachycardia

OBJECTIVE: To evaluate factors for predicting ventricular arrhythmia, the clinical effect of drugs on patients with catecholaminergic polymorphic ventricular tachycardia (CPVT), and their long-term outcomes. METHODS: Six patients diagnosed with CPVT underwent a series of electrocardiograms and 24-hour Holter monitoring. β-blockers were recommended for all patients, while some patients were also prescribed propafenone and 1 patient underwent catheter-based renal sympathetic denervation (RDN). The characteristics of electrocardiogram, arrhythmia and long-term outcomes were monitored. RESULTS: Syncope episodes did not occur any longer in 1 patient on β-blocker, but recurred in 3 other patients and 2 patients died (one due to his cessation of metoprolol for 3 months). Inverted and/or bifid T waves and abnormal U wave were observed in the precordial leads. T wave alternans was observed in 4 patients in the precordial leads. These abnormal electrocardiogram features disappeared or diminished with β-blocker treatment. All spontaneous episodes of ventricular tachycardia occurred prior to sinus tachycardia and frequent polymorphic premature ventricular contractions. CONCLUSIONS: Bifid and/or inverted T waves, T wave alternans and abnormal U waves together with sinus tachycardia and frequent premature ventricular contractions are indicator for predicting ventricular arrhythmia and assessing the effect of β-blockers. Compliance with β-blocker treatment is a strong indicator of outcome.