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題名 | 肥厚性心肌症之評估與治療=Assessment and Treatment of Hypertrophic Cardiomyopathy |
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作者 | 邱春吉; 王郁青; 李炳鈺; | 書刊名 | 藥學雜誌 |
卷期 | 30:4=121 2014.12[民103.12] |
頁次 | 頁97-102 |
分類號 | 415.3165 |
關鍵詞 | 肥厚性心肌症; 乙型阻斷劑; 侵入性治療; Hypertrophic cardiomyopathy; |
語文 | 中文(Chinese) |
中文摘要 | 肥厚性心肌症 (hypertrophic cardiomyopathy;HCM) 是一種遺傳性心肌病變,為 引起年輕人發生猝死的最常見原因。HCM 的病理生理學機轉包括非對稱性左心室肥 厚、舒張功能不良、左心室出口通道 (left ventricular outflow tract;LVOT) 阻塞和心 肌缺血。其臨床表現具多樣化,可能從無症狀到嚴重的心絞痛、心衰竭之症狀和猝 死。HCM 的治療主要是以減少症狀、改善運動耐受性、延緩疾病的進行和改善預後 為主,藥物治療主要亦是緩解 HCM 之症狀。乙型阻斷劑是第一線治療藥,若對乙型 阻斷劑療效不佳的病人可選擇 verapamil 為替代用藥,disopyramide 也可用於 HCM 的 治療。有嚴重左心室出口通道阻塞且對藥物治療療效不佳之病人,應執行侵入性治療 包括心室中隔心肌切除術、心室中隔酒精燒灼術或置放心房心室雙腔心律調整器。 HCM 是一種遺傳性心疾病,因此基因檢測、篩選和模式是未來 HCM 病人治療之基 礎。 |
英文摘要 | 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.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. |
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