Blich Miry, Marai Ibrahim, Suleiman Mahmoud, Lorber Avraham, Gepstein Lior, Boulous Monther, Khoury Asaad
Division of Pacing and Electrophysiology, Rambam Health Care Campus and Bruce Rappaport Faculty of Medicine, Haifa, Israel.
Pacing Clin Electrophysiol. 2015 Mar;38(3):398-402. doi: 10.1111/pace.12574. Epub 2015 Jan 28.
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare but highly malignant inherited arrhythmic disorder. Although a standardized exercise stress test (ST) is the most reliable way to diagnose CPVT, in 30% only single ventricular premature beats (VPCs) were recorded.
To evaluate whether electrocardiographic characteristics of VPCs during ST distinguish patients with CPVT from healthy subjects.
Electrocardiographic characteristics of VPCs during ST in 16 calsequestrin-2 (CASQ2) mutation carriers CPVT patients were compared with that in 36 healthy subjects.
CPVT patients had more VPCs (31 ± 14 vs 3 ± 4, P < 0.0001), longer QRS duration (139 ± 18 ms vs 121 ± 21, P = 0.004), and coupling interval (CI; 476 ± 58 ms vs 355 ± 61 ms, P < 0.0001). The most sensitive characteristics for CPVT were >10 VPCs/test (100% sensitivity, 100% negative predictive value [NPV]), left bundle branch block (LBBB) pattern with inferior axis (88% sensitivity, 94% NPV), and CI longer than 400 ms (88% sensitivity, 94% NPV). Bigeminy or trigeminy or LBBB pattern with inferior axis was most specific for CPVT at 100% (100% positive predictive value PPV, 92% NPV). First VPC during the recovery period and VPC recording more than 1 minute during the recovery period were most specific for healthy subjects (100% specificity, 100% PPV). In multivariate analysis, QRS duration >120 ms (odds ratio 4.2, 95% confidence interval 1-17.6, P = 0.04) and first VPC at ≥10 mets (odds ratio 9.1, 95% confidence interval 2.01-41.1, P = 0.004) each predicted the presence of CPVT.
Several electrocardiographic criteria can help distinguish VPCs originating from CPVT compared with healthy subjects.
儿茶酚胺能多形性室性心动过速（CPVT）是一种罕见但恶性程度很高的遗传性心律失常疾病。尽管标准化运动负荷试验（ST）是诊断CPVT最可靠的方法，但在30%的患者中仅记录到单个室性早搏（VPC）。
评估运动负荷试验期间室性早搏的心电图特征能否区分CPVT患者与健康受试者。
比较16例钙结合蛋白-2（CASQ2）突变携带者CPVT患者运动负荷试验期间室性早搏的心电图特征与36例健康受试者的情况。
CPVT患者有更多的室性早搏（31±14对3±4，P<0.0001）、更长的QRS时限（139±18毫秒对121±21，P=0.004）和联律间期（CI；476±58毫秒对355±61毫秒，P<0.0001）。CPVT最敏感的特征为每次试验>10个室性早搏（敏感性100%，阴性预测值[NPV]100%）、下轴的左束支传导阻滞（LBBB）图形（敏感性88%，NPV 94%）以及CI长于400毫秒（敏感性88%，NPV 94%）。成对或成三联律或下轴的LBBB图形对CPVT最为特异，为100%（阳性预测值[PPV]100%，NPV 92%）。恢复期的首个室性早搏以及恢复期室性早搏记录超过1分钟对健康受试者最为特异（特异性100%，PPV 100%）。在多变量分析中，QRS时限>120毫秒（比值比4.2，95%置信区间1-17.6，P=0.04）以及≥10梅脱时的首个室性早搏（比值比9.1，95%置信区间2.01-41.1，P=0.004）各自都可预测CPVT的存在。
与健康受试者相比，几种心电图标准有助于鉴别源自CPVT的室性早搏。
