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患者男,52岁。因反复发作性抽搐、意识丧失于1987年10月收入本院治疗。发作时 ECG 呈完全右束支阻滞(CRBBB)型室速,多种药物治疗无效,电击复律后室速发作止。心脏超声成像示右室增大,右室条索状。右心室造影:右室腔扩大、右室肌小梁增粗、右室收缩功能不良,造影剂排空缓慢。ECG、血压、血脂正常,诊断为致心律失常性右室发育不良。1994年3月因抽搐、意识丧失发作3次再次入院,~(99m)Tc-MIBI 静息心肌灌注断层显像发现在心脏短轴和水平长轴位左室前外侧壁呈放射性稀疏,后外侧壁呈放射性缺损,前间壁放射性异常浓聚。核素心室显像:右室腔明显增大,右室壁运动异常减弱。右室射血分数(RVEF)为13.8%,快速射血率(PER)为1.03/s,高峰充盈率(PFR)为0.67/s,左室不大,左室射血分数(LVEF)为48%。PER为3.29/s,PFR为2.27/s。诊断:致心律失常性左、右室发育不良。致心律失常性左、右室发育不良较少见。Gerlis 等把本病的特点归纳为:可发生在任何年龄,多为散发,少数有家族史,其病因尚不清楚。病理改变主要为心肌完全或部分缺如,缺如处有脂肪或纤维脂肪组织浸润。一般多累及右室,左室也可受
Male patient, 52 years old. Due to recurrent seizures, loss of consciousness in October 1987 income hospital treatment. The onset of ECG showed complete right bundle branch block (CRBBB) VT, a variety of drug treatment is invalid, the electroshock cardioversion ventricular tachycardia only. Echocardiography showed right ventricular enlargement, right ventricular cord-like. Right ventricular angiography: right ventricular cavity expansion, right ventricle trabecular thickening, right ventricular systolic dysfunction, contrast agent emptying slowly. ECG, blood pressure, blood lipids, diagnosed as arrhythmogenic right ventricular dysplasia. March 1994 due to convulsions, loss of consciousness episodes of re-admission 3 times, ~ (99m) Tc-MIBI resting myocardial perfusion tomography found in the short axis of the heart and horizontal long axis of the left ventricular anterolateral wall was radioactive sparse, posterior lateral Wall was radioactive defect, the anterior wall of the radioactive concentration. Radionuclide imaging: right ventricular cavity was significantly increased, abnormal right ventricular wall motion weakened. The RVEF was 13.8%, the fast ejection rate (PER) was 1.03 / s, the peak filling rate (PFR) was 0.67 / s, the left ventricular volume was not large and the left ventricular ejection fraction (LVEF) was 48 %. PER was 3.29 / s and PFR was 2.27 / s. Diagnosis: induced arrhythmic left and right ventricular dysplasia. To arrhythmogenic left and right ventricular dysplasia less common. Gerlis and other characteristics of the disease can be summarized as: can occur at any age, mostly distributed, a small number of family history, the etiology is not yet clear. Pathological changes mainly for the complete or partial lack of myocardial, lack of fat or fibrous adipose tissue infiltration. Generally involving the right ventricle, left ventricular can also be affected