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目的:分析9例经病理证实以左心室受累为主的致心律失常性右心室心肌病(ARVC)的临床病理特征,提高对左心室受累致ARVC的认识。方法:回顾49例心脏移植中9例经病理证实为左心室受累为主的ARVC患者的一般特征、猝死和心肌病家族史、临床症状和病程、药物治疗史、心电图、24小时动态心电监测(Holter)、超声心动图、心血池、静态心肌显像、心脏磁共振成像、冠状动脉造影及受体心脏大体和组织学病理特征。结果:9例患者平均年龄13~54(40.4±15.9)岁,女性5例,男性4例。心悸胸闷病史0.33~20年,平均(9.1±8.9)年,晕厥史2例,有电除颤史6例,胺碘酮治疗5例。2例有猝死家族史。ARVC特征性心电图异常包括V1~3/4~6导联QRS间期>1.2(占0%),有ε波(占44.4%),胸前导联T波倒置(占33.3%)。9例24小时动态心电监测中4例有阵发性心房颤动,其中2例合并心房扑动;2例未服胺碘酮者室性早搏>1000次/24h,其余7例(5例服用胺碘酮)室性早搏均<1000次/24h,室性早搏形态平均(3.3±1.1)种/24h。9例术前超声心动图显示平均右心室内径(32.6±6.7)mm,左心房内径(37.1±10.3)mm,左心室舒张末径(57.3±11.9)mm,左心室射血分数0.26±0.09。7例患者术前心脏磁共振成像显示,5例可见异常高强度信号区域,5例可见右心室和右心房扩大,4例可见右心室壁瘤样突出。8例术前用Swan-Gans导管作血流动力学监测:平均右心室压(19.3±8.3)mmHg(1mmHg=0.133kPa),平均肺毛细血管楔压(15.6±7.7)mmHg,平均心脏指数(2.0±0.4)L/(min.m2),平均肺血管阻力(267.8±167.6)dyn.s.cm-5。9例病理特征为右心室腔显著扩大7例,右心室壁整体变薄6例,右心室流出道最薄处<1mm。左心室均有受累,显著扩张8例。左、右心室壁均被不同范围的纤维和脂肪组织替代。结论:虽然ARVC累及双心室者难以与扩张型心肌病鉴别,但是结合临床和影像资料,特别是心脏磁共振成像,可提高ARVC诊断的准确性。
OBJECTIVE: To analyze the clinicopathological features of 9 cases of arrhythmogenic right ventricular cardiomyopathy (ARVC) with pathological confirmed by left ventricular involvement and to improve the cognition of ARVC induced by left ventricular involvement. Methods: Ninety-nine patients with ARVC were retrospectively analyzed. The general characteristics of ARVC patients with pathologically confirmed left ventricular involvement, family history of sudden death and cardiomyopathy, clinical symptoms and course of disease, history of drug treatment, electrocardiogram, 24-hour Holter monitoring (Holter), echocardiography, cardiomyocytes, myocardial perfusion imaging, cardiac magnetic resonance imaging, coronary angiography and cardiac and histological features of the recipient heart. Results: The average age of 9 patients ranged from 13 to 54 years old (40.4 ± 15.9 years), female 5 and male 4. Palpation chest tightness history 0.33 to 20 years, an average of (9.1 ± 8.9) years, 2 cases of syncope, a history of defibrillation in 6 cases, amiodarone in 5 cases. 2 cases of sudden death family history. The characteristic ECG abnormalities of ARVC included V1 ~ 3/4 ~ 6 lead QRS interval> 1.2 (0%), ε wave (44.4%) and thoracic T wave inversion (33.3%). Four patients had paroxysmal atrial fibrillation in 9 cases of 24-hour ambulatory ECG monitoring, including 2 cases with atrial flutter; 2 cases with ventricular premature beats without amiodarone> 1000 times / 24h and the remaining 7 cases Amiodarone) premature ventricular contractions were <1000 times / 24h, premature ventricular morphology average (3.3 ± 1.1) species / 24h. Preoperative echocardiography showed that the average diameter of the right ventricle (32.6 ± 6.7) mm, the diameter of the left atrium (37.1 ± 10.3) mm, the left ventricular end diastolic diameter (57.3 ± 11.9) mm, and the left ventricular ejection fraction 0.26 ± 0.09. Preoperative cardiac magnetic resonance imaging of 7 patients showed that 5 cases showed abnormal high-intensity signal area, 5 cases showed right ventricle and right atrium enlargement, and 4 cases showed right ventricular aneurysm-like protrusion. Eight patients underwent preoperative hemodynamic monitoring with a Swan-Gans catheter: mean right ventricular pressure (19.3 ± 8.3) mmHg (1 mmHg = 0.133 kPa), mean pulmonary capillary wedge pressure (15.6 ± 7.7) mmHg, mean cardiac index 2.0 ± 0.4) L / (min.m2), average pulmonary vascular resistance (267.8 ± 167.6) dyn.s.cm-5.9 The pathological features of the right ventricular cavity were significantly enlarged in 7 cases, the right ventricular wall thinning in 6 cases , Right ventricular outflow tract thinnest <1mm. Left ventricular involvement, significant expansion in 8 cases. Left and right ventricular walls were replaced by different ranges of fibers and adipose tissue. CONCLUSION: Although it is difficult to differentiate biventricular involvement from dilated cardiomyopathy in ARVC, the accuracy of ARVC diagnosis can be improved by combining clinical and imaging data, especially cardiac magnetic resonance imaging.