Division of Electrophysiology, Department of Cardiovascular Medicine, University Hospital of Münster, Albert-Schweitzer-Campus 1, D-48149 Münster, Germany.
Heart Rhythm. 2013 Feb;10(2):158-64. doi: 10.1016/j.hrthm.2012.10.019. Epub 2012 Oct 13.
Recent evidence suggests that cardiac sarcoidosis (CS) and arrhythmogenic right ventricular cardiomyopathy (ARVC) can manifest very similarly.
To investigate whether there are significant demographic and electrophysiological differences between patients with CS and ARVC.
We prospectively compared patients with proven CS or ARVC who underwent radiofrequency catheter ablation of ventricular tachycardias by using 3-dimensional electroanatomical mapping. Furthermore, we evaluated whether the diagnostic criteria for ARVC would have excluded ARVC in patients with CS.
Eighteen patients (13 men; mean age 44.9 years) were included. All 18 patients had mild to moderately reduced right ventricular ejection fraction. Patients with cardiac sarcoidosis (n = 8) had a significantly lower mean left ventricular ejection fraction (35.6±19.3 vs 60.6±9.4; P = .002). Patients with CS had a significantly wider QRS (0.146 vs 0.110s; P = .004). Five of 8 (63%) patients with CS fulfilled the diagnostic ARVC criteria. Ventricular tachycardias (VTs) with a left bundle branch block pattern were documented in all but one patient (with CS). Programmed ventricular stimulation induced an average of 3.7 different monomorphic VTs in patients with CS vs 1.8 in patients with ARVC (P = .01). VT significantly more often originated in the apical region of the right ventricle in CS vs ARVC (P = .001), with no other predilection sites. Ablation success and other electrophysiological parameters were not different.
The current diagnostic ARVC guidelines do not reliably exclude patients with CS. Clinical and electrophysiological parameters that were characteristic of CS in our patients include reduced left ventricular ejection fraction, a significantly wider QRS, right-sided apical VT, and more inducible forms of monomorphic VT.
最近的证据表明，心脏结节病（CS）和致心律失常性右心室心肌病（ARVC）的表现可能非常相似。
研究 CS 和 ARVC 患者之间是否存在显著的人口统计学和电生理差异。
我们前瞻性比较了经心腔内电生理标测系统进行射频导管消融治疗室性心动过速的确诊 CS 或 ARVC 患者。此外，我们评估了 ARVC 的诊断标准是否会将 CS 患者排除在外。
共纳入 18 例患者（13 例男性，平均年龄 44.9 岁）。所有 18 例患者的右心室射血分数均轻度至中度降低。CS 组（n=8）的平均左心室射血分数显著较低（35.6±19.3 比 60.6±9.4；P=0.002）。CS 组的 QRS 波较宽（0.146 比 0.110s；P=0.004）。8 例 CS 患者中有 5 例（63%）符合 ARVC 的诊断标准。除了 1 例（CS）患者外，其余患者均有左束支传导阻滞模式的室性心动过速。CS 患者的程控刺激平均诱发出 3.7 种不同形态的单形性室速，而 ARVC 患者为 1.8 种（P=0.01）。CS 患者的 VT 主要起源于右心室心尖部，而 ARVC 患者的 VT 起源于其他部位（P=0.001）。消融成功率和其他电生理参数无差异。
目前的 ARVC 诊断标准不能可靠地排除 CS 患者。我们患者的 CS 特征包括左心室射血分数降低、QRS 波明显增宽、右侧心尖部 VT 以及更多可诱发的单形性 VT。
