肥厚型心肌病患者暈厥發(fā)生的機(jī)制和處理對(duì)策_(dá)胡厚源.ppt

1、肥厚型心肌病患者暈厥發(fā)生的機(jī)制和處理對(duì)策,第三軍醫(yī)大學(xué)西南醫(yī)院心內(nèi)科 胡 厚 源,Hypertrophic cardiomyopathy: a50th 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. Asymmetrical

2、 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 in 152

3、5% 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 tachycardia

4、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 impaired fill

5、ing when preload is reduced in the setting of diastolic dysfunction,治 療,猝死高危的患者 植入ICD，提高生存率： 持續(xù)性VT 胺碘酮 或 ICD 非持續(xù)性VT ，無(wú)SCD危險(xiǎn)因素 胺碘酮 非持續(xù)性VT ，伴有SCD危險(xiǎn)因素 ICD 陣發(fā)性AF 胺碘酮 ； 控制AF患者的HR 阻滯劑、維拉帕米； 心動(dòng)過(guò)緩 起搏器植入（DDD）； 減輕LVOT梗阻 藥物、室間隔酒精消融術(shù) (Alcohol septal ablation, ASA)、肥厚室間隔切開(kāi)/切除術(shù)（myectomy）、心臟移植術(shù),A schematic summary

6、 of the pharmacologic therapy of HCM,丙吡胺（disopyramide,為a類抗心律失常藥； 也是另一種負(fù)性肌力藥，負(fù)性肌力作用強(qiáng)，可用于治療HOCM； 除抗心律失常外，還能顯著改善血流動(dòng)力學(xué),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 and nondi

7、sopyramide-treated patients with HOCM,丙吡胺的注意事項(xiàng),應(yīng)監(jiān)測(cè)QT間期延長(zhǎng)； 丙吡胺并不能改善HCM的舒張功能； 對(duì)有發(fā)生房顫可能的患者有益處； 副作用包括抗膽堿能作用，如口干、 眼干、便秘、排尿困難,室間隔心肌酒精消融術(shù)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, respectively 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