摘要
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（Hoher）、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￡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±O．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-t-O．4）L／（min·m^2），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 clinical and pathological characteristics of arrhythmogenic right ventricular cardiomyopathy （ARVC） and manifestations of severe left ventricular impairment in 9 out of 49 patients who had cardiac transplantation.
Methods ： Nine patients （4 males,5 females; mean [↑-x ± s ] age 40. 4± 15.9 ）, having a pathology diagnosis of ARVC with severe left ventricular involvement at heart transplantation,were studied by clinical data which included patient age, gender, family history of premature sudden deaths, symptoms, physical examinations, 12-lead electrocardiographic （ECG） changes, Hoher monitoring, echocardiography, radionuclide angiography, myocardial perfusion scintigraphy, magnetic resonance imaging （ MRI ）, coronary artery angiography.
Results ：The history of palpitation or shortness of breath averaged 9. 1± 8. 9 years. There were 2 with a family history of syncope,2 patients with a familial history of sudden death. The abnormal ECG included QRS duration 〉 110ms in V1 ead （100%）, ε aves （44.4%）and inversion of T waves in the right precordial leads （33.3 % ）. There were 4 with paroxysmal atrial flutter and 2 with paroxysmal atrial fibrillation by Hoher monitoring. There were 2 patients （ without antiarrhythmic drug treatment） with frequent ventricular extrasystoles（ 〉 1 000/24 hours）and the other 7 （5 on amiodarone）with ventricular extrasystoles （ 〈 1 000/ 24 hours）by Hoher monitoring. The two-dimensional echocardiography showed severe left ventricular impairment （average EF 0. 26 ± 0. 09）. The average left ventricular endodiastolic dimension （EDD）, left atrial EDD and right ventricular EDD were 57. 3 ± 11.9 mm and 37. 1 ± 10. 3 mm,retrospectively. Seven received MRI examination. Out of them high signal intensity over the myocardial areas was found in 5, dilation of right ventricle and atrium in 5 and the right ventricle aneurysm in 4. Eight received SwanGans catheter examination. Average right ventricular pressure was 19. 3 ± 8.3 mmHg,average pulmonary capillary wedge pressure （PCWP） was 15.6±＋ 7.7 mmHg, average cardiac index was 2.0± 0.4 L/（ min ·m2）, and average pulmonary vessel resistance was 267.8±167. 6 dyn·s·cm^5. The gross examination of 9 explanted hearts showed markedly dilated right ventricles in 7 patients and thinned right ventricle walls in 6 patients. Some areas of the anterior right ventricular wall were replaced by fibrofatty tissue. The subendocardial areas of both ventricles were replaced by fibrofatty tissue.
Conclusion ： ARVC with left ventricular impairment is rare. Accurate diagnosis is possible by combination of clinical data, imaging findings and pathological features.
出处
《中国循环杂志》
CSCD
北大核心
2007年第4期278-281,共4页
Chinese Circulation Journal
基金
北京市科技计划项目资助(D0906004040391)
关键词
心肌病
左心室受累
磁共振成像
Cardiomyopathy
Left ventricular impairment
Magnetic resonance imaging
