Clinical and Pathological Impact of Tissue Fibrosis on Lethal Arrhythmic Events in Hypertrophic Cardiomyopathy Patients With Impaired Systolic Function
Clinical and Pathological Impact of Tissue Fibrosis on Lethal Arrhythmic Events in Hypertrophic Cardiomyopathy Patients With Impaired Systolic Function
复制标题
DOI：
10.1253/circj.cj-15-0104
复制
发表时间：
2015-08-01
影响因子：
3.3
通讯作者：
Ogawa, Hisao
中科院分区：
文献类型：
--
作者：
Wada, Yuko;Aiba, Takeshi;Ogawa, Hisao
背景：肥厚性心肌病（HCM）的自然病程从无症状的良性病程到预后不良不等。心肌纤维化可能在室性心动过速（VT/VF）中起关键作用；然而，右心室（RV）活检组织纤维化在HCM患者长期预后中的临床意义尚不清楚。方法和结果：我们招募了185例HCM患者（平均年龄，57±14岁）。用数码显微镜定量测定纤维化量（%面积）。血流动力学、超声心动图和电生理参数也进行了评估。严重纤维化患者QRS持续时间较长，信号平均心电图迟电位（LP）阳性，导致VT/VF发生率较高。在5+/-4年随访中，31例（17%）患者发生VT/VF。多因素Cox回归分析显示，组织纤维化(风险比（HR）： 1.65；低左心室射血分数（HR: 0.64； P=0.001 / 10%）和阳性SAECG （HR: 3.14； P=0.04）导致VT/VF的风险增加。组织纤维化严重程度和低左室射血分数的结合可用于HCM患者致死性心律失常事件的风险分层。结论：RV活检标本心肌纤维化可能导致异常传导延迟和自发性VT/VF，导致HCM患者预后不良。
Background: The natural history of hypertrophic cardiomyopathy (HCM) varies from an asymptomatic benign course to a poor prognosis. Myocardial fibrosis may play a critical role in ventricular tachyarrhythmias (VT/VF); however, the clinical significance of tissue fibrosis by right ventricular (RV) biopsy in the long-term prognosis of HCM patients remains unclear.Methods and Results: We enrolled 185 HCM patients (mean age, 57+/-14 years). The amount of fibrosis (%area) was quantified using a digital microscope. Hemodynamic, echocardiographic, and electrophysiologic parameters were also evaluated. Patients with severe fibrosis had longer QRS duration and positive late potential (LP) on signal-averaged ECG, resulting in a higher incidence of VT/VF. At the 5+/-4 year follow-up, VT/VF occurred in 31 (17%) patients. Multivariate Cox regression analysis revealed that tissue fibrosis (hazard ratio (HR): 1.65; P=0.003 per 10% increase), lower left ventricular ejection fraction (HR: 0.64; P=0.001 per 10% increase), and positive SAECG (HR: 3.14; P=0.04) led to a greater risk of VT/VF. The combination of tissue fibrosis severity and lower left ventricular ejection fraction could be used to stratify the risk of lethal arrhythmic events in HCM patients.Conclusions: Myocardial fibrosis in RV biopsy samples may contribute to abnormal conduction delay and spontaneous VT/VF, leading to a poor prognosis in HCM patients.