Horner Justin M, Ackerman Michael J
Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Heart Rhythm. 2008 Dec;5(12):1690-4. doi: 10.1016/j.hrthm.2008.08.038. Epub 2008 Sep 4.
Long QT syndrome (LQTS) can present with sudden death during exertion.
The purpose of this study was to determine the diagnostic importance of exercise-induced ventricular ectopy in the evaluation of LQTS.
From 1998 to 2006, 381 patients with a referral diagnosis of LQTS underwent a treadmill exercise stress test. An investigator blinded to both genotype and rendered diagnosis scored the stress tests for the presence of exercise-induced ventricular ectopy.
The dismissal diagnosis was LQTS in 177 (46%), catecholaminergic polymorphic ventricular tachycardia (CPVT) in 16, miscellaneous cardiac disease in 17, and normal in 171. Exercise-induced ventricular ectopy was detected in 107 (28%) patients. However, only 34 patients (9% overall) had exercise-induced ventricular ectopy greater than single premature ventricular contractions (PVCs). Among the 171 patients dismissed as normal, only 2% had ectopy greater than single PVCs. Among the genotype-positive LQTS patients, no significant ectopy was recorded in 80 with LQT1, compared to 5 (8%) patients with LQT2 and 3 (20%) patients with LQT3 (P <.0001). In contrast, exercise-induced ventricular ectopy beyond single PVCs was far more common among patients with CPVT (14/16 [88%]; P <.0001) and included PVCs in bigeminy in 13 (81%), couplets in 7 (47%), and nonsustained ventricular tachycardia in 3 (19%). Of note, bidirectional VT was not present in any of the 16 patients diagnosed with CPVT, including the 10 with genetically proven, RYR2-mediated CPVT.
Exercise-induced ventricular ectopy exceeding single PVCs was observed in less than 10% of patients referred for LQTS evaluation, including 2% of patients ultimately dismissed as normal. Exercise-induced bigeminy is strongly associated with the presence of significant cardiovascular disease but is far more likely to indicate CPVT than LQTS.
长QT综合征(LQTS)可在运动时出现猝死。
本研究旨在确定运动诱发的室性早搏在LQTS评估中的诊断重要性。
1998年至2006年,381例被转诊诊断为LQTS的患者接受了平板运动负荷试验。一名对基因型和最终诊断均不知情的研究人员对运动负荷试验中是否存在运动诱发的室性早搏进行评分。
最终诊断为LQTS的有177例(46%),儿茶酚胺能多形性室性心动过速(CPVT)16例,其他心脏病17例,正常171例。107例(28%)患者检测到运动诱发的室性早搏。然而,只有34例患者(占总数的9%)的运动诱发室性早搏超过单个室性早搏(PVC)。在171例被判定为正常的患者中,只有2%的患者早搏超过单个PVC。在基因型阳性的LQTS患者中,80例LQT1患者未记录到明显早搏,而LQT2患者中有5例(8%),LQT3患者中有3例(20%)(P<.0001)。相比之下,运动诱发的超过单个PVC的室性早搏在CPVT患者中更为常见(16例中有14例[88%];P<.0001),包括13例(81%)成对出现的PVC、7例(47%)二联律和3例(19%)非持续性室性心动过速。值得注意的是,16例被诊断为CPVT的患者中均未出现双向室性心动过速,包括10例经基因证实为RYR2介导的CPVT患者。
在转诊进行LQTS评估的患者中,不到10%的患者观察到运动诱发的室性早搏超过单个PVC,包括最终被判定为正常的患者中的2%。运动诱发的成对早搏与严重心血管疾病密切相关,但更可能提示CPVT而非LQTS。