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1、肥厚型心肌病患者 晕厥发生的机制和处理对策,第三军医大学西南医院心内科 胡 厚 源,Hypertrophic cardiomyopathy: a 50th anniversary,In 1958, Donald Teare, a forensic pathologist in London, reported eight cases of sudden death caused by asymmetrical hypertrophy or benign tumour of the heart in the British Heart Journal. ( Teare D. Asymmetric
2、al hypertrophy of the heart in young adults. Br Heart J . 1958;20:18. ),HCM病理生理,Comparison of survival free from HCM-related cardiac death, in patients with obstructive and non-obstructive HCM.,Syncope in hypertrophic cardiomyopathy,Symptoms of impaired consciousness (syncope and presyncope) occur i
3、n 1525% of the patients with HCM. Syncope typically occurs in younger patients with smaller ventricles. Impaired consciousness is often provoked by exercise (either during or after) or by postural change.,Mechanisms for syncope in HCM,Arrhythmia Paroxysmal atrial fibrillation/supraventricular tachyc
4、ardia Complete heart block/sinus node dysfunction Sustained ventricular tachycardia Primary haemodynamic mechanism Left ventricular outflow tract obstruction (LVOTO) Abnormal vascular control mechanisms leading to episodes of hypotension due to inappropriate vasodilatation Hypotension due to impaire
5、d filling when preload is reduced in the setting of diastolic dysfunction,治 疗,猝死高危的患者 植入ICD，提高生存率： 持续性VT 胺碘酮 或 ICD 非持续性VT ，无SCD危险因素 胺碘酮 非持续性VT ，伴有SCD危险因素 ICD 阵发性AF 胺碘酮 ； 控制AF患者的HR 阻滞剂、维拉帕米； 心动过缓 起搏器植入（DDD）； 减轻LVOT梗阻 药物、室间隔酒精消融术 (Alcohol septal ablation, ASA)、肥厚室间隔切开/切除术（myectomy）、心脏移植术 。,A schematic
6、 summary of the pharmacologic therapy of HCM,丙吡胺（disopyramide）,为a类抗心律失常药； 也是另一种负性肌力药，负性肌力作用强，可用于治疗HOCM； 除抗心律失常外，还能显著改善血流动力学。,LVOTG before and after the intravenous administration of disopyramide to a patient with HOCM,KaplanMeier survival plots for all-cause cardiac mortality in disopyramide-treated
7、 and nondisopyramide-treated patients with HOCM.,丙吡胺的注意事项,应监测QT间期延长； 丙吡胺并不能改善HCM的舒张功能； 对有发生房颤可能的患者有益处； 副作用包括抗胆碱能作用，如口干、 眼干、便秘、排尿困难。,室间隔心肌酒精消融术 Alcohol septal ablation, ASA,The beneficial affect of ASA on symptoms as determined by the NYHA and the CCS scores for heart failure symptoms and angina, res
8、pectively and exercise tolerance as determined by the treadmill exercise time.,The beneficial affect of ASA on the resting (diamonds) and provoked gradients (circles).,The changes in septal thickness after alcohol ablation,The changes in LV ejection after alcohol ablation.,The Kaplan-Meier survival curve for all-cause mortality.,肥厚室间隔心肌切开-切除术 myectomy,静息LVOTG50mmHg 或 激发LVOTG100mmHg,谢 谢,