From the Hôpital Cardiologique du Haut-Lévêque, CHU Bordeaux, Université Victor Segalen, Bordeaux II, LIRYC Institute, Bordeaux, France.
Circ Arrhythm Electrophysiol. 2014 Aug;7(4):590-7. doi: 10.1161/CIRCEP.113.001224. Epub 2014 Jun 26.
Although the Task Force Criteria for arrhythmogenic right ventricular cardiomyopathy (ARVC) have recently been updated, the diagnosis remains challenging in the early stages. The aim of this study was to evaluate the diagnostic value of β-adrenergic stimulation in ARVC.
We evaluated 412 consecutive patients (213 men, age 41.5±16 years) referred for premature ventricular contractions evaluation or suspected ARVC. Isoproterenol testing was performed with continuous infusion of isoproterenol (45 μg/min) for 3 minutes. It was considered positive if there were either (1) polymorphic premature ventricular contractions with ≥1 couplet or (2) sustained or nonsustained ventricular tachycardia with left bundle branch block excluding right ventricular outflow tract ventricular tachycardia. ARVC was diagnosed in 35 patients at initial evaluation (23 men, aged 42±15 years). Isoproterenol testing was positive in 32 of 35 (91.4%) patients with ARVC and in 42 of 377 (11.1%) patients without ARVC (P<0.0001). Sensitivity, specificity, positive, and negative predictive values of isoproterenol testing to diagnose ARVC were 91.4%, 88.9%, 43.2%, and 99.1%, respectively. During a mean follow-up period of 5.6±4.4 years, 6 additional patients met diagnostic criteria for ARVC. Importantly, initial isoproterenol testing was positive in 6 of 6 (100%) of these patients. Survival free from ARVC diagnosis was significantly lower in the positive isoproterenol group than in the negative isoproterenol group (P<0.0001, exact log-rank test).
Ventricular arrhythmogenicity during isoproterenol testing is highly sensitive (sensitivity, 91.4%) for the diagnosis of ARVC, particularly in its early stages.
尽管心律失常性右室心肌病（ARVC）的工作组标准最近已经更新，但在早期阶段的诊断仍然具有挑战性。本研究旨在评估β-肾上腺素能刺激在 ARVC 中的诊断价值。
我们评估了 412 例连续患者（213 例男性，年龄 41.5±16 岁），这些患者因室性早搏评估或疑似 ARVC 而就诊。异丙肾上腺素测试通过异丙肾上腺素（45μg/min）持续输注 3 分钟进行。如果存在（1）≥1 对多形性室性早搏或（2）持续性或非持续性左束支传导阻滞伴排除右室流出道室性心动过速的室性心动过速，则认为是阳性。在初次评估时诊断为 35 例 ARVC（23 例男性，年龄 42±15 岁）。35 例 ARVC 患者中有 32 例（91.4%）和 377 例非 ARVC 患者中的 42 例（11.1%）异丙肾上腺素测试阳性（P<0.0001）。异丙肾上腺素测试诊断 ARVC 的敏感性、特异性、阳性预测值和阴性预测值分别为 91.4%、88.9%、43.2%和 99.1%。在平均 5.6±4.4 年的随访期间，又有 6 例患者符合 ARVC 的诊断标准。重要的是，这 6 例患者的初始异丙肾上腺素测试均为阳性。阳性异丙肾上腺素组的 ARVC 诊断无生存时间明显低于阴性异丙肾上腺素组（P<0.0001，确切对数秩检验）。
异丙肾上腺素测试期间的室性心律失常性对 ARVC 的诊断具有高度敏感性（敏感性为 91.4%），特别是在早期阶段。
