臨床藥Therapeutics of Clinical Drugs 物治療學參 病理生理學 一 左心室肥厚 表一影響肥厚性心肌症左心室出口通道壓力 1,10-11 差之因素 verapamil Inotropic agent (ex. digoxin) ( ) ( ) ( ) ( ) 1 二 舒張功

摘要 高雄長庚紀念醫院藥劑部藥師邱春吉 王郁青 李炳鈺 (hypertrophic cardiomyopathy HCM) HCM (left ventricular outflow tract LVOT) HCM HCM verapamil disopyramide HCM HCM HCM hypertrophic cardiomyopathy 壹 前言 1-6 3,6 HCM (left ventricular outflow tract LVOT) 1 7 LVOT 8 HCM 貳 流行病學與病因學 HCM 1-6 HCM (autosomal dominant) 70-80% beta-myosin heavy chain protein cardiac myosin-binding protein C cardiac troponin T 2,3,9 30 4 Dec. 31 2014 藥學雜誌 121 97 臨床藥物治療學肥厚性心肌症之評估與治療

臨床藥Therapeutics of Clinical Drugs 物治療學參 病理生理學 一 左心室肥厚 表一影響肥厚性心肌症左心室出口通道壓力 1,10-11 差之因素 verapamil Inotropic agent (ex. digoxin) ( ) ( ) ( ) ( ) 1 二 舒張功能不良 HCM 1,3,10 三 LVOT 阻塞 30% 50% HCM LVOT LVOT 1,3,10 LVOT ( ) 1,10,11 ( ) 3 四 心肌缺血 1,3 1,3,6,8,10 肆 臨床表徵 20 40 3 圖一肥厚性心肌症左心室收縮時之概略圖 LVOT (left ventricular outflow tract) 98 THE JOURNAL OF TAIWAN PHARMACY Vol.30 No.4 Dec. 31 2014

臨床藥物治療1-4 學伍 診斷一 基因檢測 (genetic testing) 二 心電圖 (electrocardiography) HCM 24 (Holter) HCM 三 壓力測試 (treadmill exercise testing) (stress testing) HCM 四 胸部 X 光檢查 HCM X (chest x-ray) LVOT 五 心臟超音波 (echocardiography) (two-dimensional and doppler echocardiography) HCM LVOT 六 心臟磁振造影 HCM (cardiac magnetic resonance imaging CMRI) 藥學雜誌 121 99 七 心導管檢查和血管造影 (cardiac catheterization and angiography) LVOT HCM 陸 預後 10% HCM HCM 1,3 HCM 30 mm 1-3,6 柒 治療 HCM 一 無症狀病人 HCM 1,3 1,8 HCM 30 4 Dec. 31 2014

Therapeutics of Clinical Drugs 臨床藥物治療學二 有症狀病人與藥物治療 圖二肥厚性心肌症之治療流程圖 1,8 ACE: angiotensin-converting enzyme; ARB: angiotensin receptor blocker; DM: diabetes mellitus; EF: ejection fraction; GL: guidelines; HCM: hypertrophic cardiomyopathy; HTN: hypertension; LV: left ventricular. HCM HCM (negative inotropic effect) LVOT 1-6,8-11 propranolol 40 mg 5,8,10 nadolol 80 mg 5,8 verapamil LVOT 1-3,5,8-11 Dihydropyridine nifedipine ( ) LVOT 3,8,10 ( ) 1,8 HCM (avoid vasodilator therapy) HCM Beta-blocker disopyramide 100 THE JOURNAL OF TAIWAN PHARMACY Vol.30 No.4 Dec. 31 2014

臨床藥物治療 學 1-3,10-11 LVOT 1,3-5,10-11 (dual-chamber pacing) LVOT LVOT 1-4,10,11 LVOT HCM (implantable cardioverter- 1,8 1,8 ( ) HCM defibrillator ICD) HCM (avoid high dose diuretics) 1,2,4 三 侵入性治療 ( 圖二 ) 1,8 捌 結論 (septal myectomy) LVOT 50 mmhg 1-3,10-11 (alcohol septal ablation) LVOT HCM HCM LVOT Assessment and Treatment of Hypertrophic Cardiomyopathy Chun-Chi Chiu, Yu-Chin Wang, Ping-Yi Lee Department of Pharmacy, Kaohsiung Chang Gung Memorial Hospital Abstract Hypertrophic cardiomyopathy (HCM) is a genetic cardiomyopathy. It is the most common cause of sudden cardiac death in young people. The pathophysiology includs asymmetric left ventricular hypertrophy, diastolic dysfunction, left ventricular outflow tract (LVOT) obstruction and myocardial ischemia. The clinical presentation varies widely, ranging from no symptoms to severe symptoms of angina, heart failure and sudden death. 藥學雜誌 121 101 30 4 Dec. 31 2014

臨床藥物治療學 Therapeutics of Clinical Drugs The treatment of HCM is designed to reduce symptoms, improve exercise tolerance, retard disease progression, and improve prognosis. Medical therapy should be considered the initial therapeutic approach to relieving symptoms of patients with HCM. Beta-blocker is the firstline drug of choice. Patients who have an inadequate response to beta-blocker may respond to verapamil. Disopyramide has been used for treating patients with HCM. For patients with severe LVOT obstruction who do not respond to medical management, an invasive therapy may be necessary. Septal myectomy, alcohol septal ablation and dual-chamber pacing have been used. HCM is a genetic heart disease, therefore, the genetic testing, screening and models are the basis of future therapy for patients with HCM. 參考資料 1. 2. 3. 4. 5. 102 Gersh BJ, Maron BJ, Bonow RO, et al: 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol 2011;58:e212-60. Maron BJ, Maron MS. Hypertrophic cardiomyopathy. Lancet 2013;381:242-55. Ommen SR, Nishimura RA, Tajik A. Chapter 33. Hypertrophic Cardiomyopathy. In: Fuster V, Walsh RA, Harrington RA, eds. Hurst's The Heart. 13th ed. New York: McGraw-Hill; 2011. http://www.accessmedicine.com/ content.aspx?aid=7811789. Accessed September 4, 2013. Jacoby DL, DePasquale EC, McKenna WJ. Hypertrophic cardiomyopathy: diagnosis, risk stratification and treatment. CMAJ 2013;185(2):127-34. Spoladore R, Maron MS, D'Amato R, et al: Pharmaco- THE JOURNAL OF TAIWAN PHARMACY Vol.30 No.4 Dec. 31 2014 6. 7. 8. 9. 10. 11. logical treatment options for hypertrophic cardiomyopathy: high time for evidence. European Heart Journal 2012;33(14):1724-33. Spirito P, Autore C. Management of hypertrophic cardiomyopathy. BMJ 2006;332:1251-5. Maron MS, Olivotto I, Betocchi S, et al: Effects of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N Engl J M 2003;348:295-303. Mckenna WJ. Medical therapy in hypertrophic cardiomyopathy. http://www.uptodate.com/contents/medical-therapy-in-hypertrophic-cardiomyopathy? Updated Octtober 10, 2012. Ho CY. Hypertrophic cardiomyopathy in 2012. Circulation 2012;125:1432-38. Fifer MA, Vlahakes GJ. Management of symptoms in hypertrophic cardiomyopathy. Circulation 2008;117:429-39. Ommen SR, Shah PM, Tajik AJ. Left ventricular outflow tract obstruction in hypertrophic cardiomyopathy: past, present and future. Heart 2008; 94:1276-81.