肥厚型梗阻性心肌病治疗进展

肥厚型梗阻性心肌病（HOCM）是最常见的遗传性心血管疾病,对于存在药物难治性心力衰竭症状（纽约心脏病协会心功能分级Ⅲ～Ⅳ级）且静息状态或生理（运动）激发时左心室流出道压力阶差≥50 mmHg(1 mmHg=0.133 kPa)的患者,应考虑室间隔心肌切除术（SM）或酒精室间隔消融术（ASA）作为首选治疗方案。本文从HOCM的病理生理学、诊断、药物治疗、SM、ASA以及新兴治疗手段展开,对HOCM治疗进展作一综述。     

乙醇室间隔消融术治疗肥厚型梗阻性心肌病研究进展

肥厚型梗阻性心肌病是青年人心源性猝死的最常见原因。严重者应及时治疗,最适病人的筛选、影像技术的辅助、并发症的控制和操作技术的提升将使乙醇室间隔消融术成为治疗肥厚型梗阻性心肌病一种很有前途的方法。     

改良经皮经腔间隔心肌化学消融术治疗肥厚型梗阻性心肌病

目的　观察改良经皮经腔间隔心肌化学消融术 (PTSMA)治疗肥厚型梗阻性心肌病 (HOCM)的疗效。方法　 5例患者在常规PTSMA治疗HOCM方法基础上 ,改良如下操作 :1.简化测压方法 :采用大一号动脉鞘与小一号猪尾管同步测股动脉与左室流入道压。 2 .酒精用量指导 :采用球囊闭塞靶间隔支后造影能充分显示间隔支血管供血范围 (即造影剂呈云雾状渗入心肌 )所需的造影剂量 ,指导消融酒精用量。3.尽量避开靶间隔支高位靠右分支 (右前斜位 30°观察 )消融。结果　 5例患者均获成功 ,术后即刻心导管测静息左室流出道压力阶差由术前的 ( 78 4± 2 5 2 )mmHg降至 ( 11 6± 4 7)mmHg。酒精用量为 3～ 9( 4 8± 2 3)ml,2例发生一过性房室传导阻滞 ,术后半年超声心动图测量室间隔厚度由术前的 ( 19 2± 2 3)mm减至 ( 16 1± 2 4 )mm。结论　适当改良的PTSMA方法简便 ,指导消融酒精用量充分 ,消融疗效肯定 ,可减少并发症发生     

室间隔心肌消融术治疗梗阻性肥厚型心肌病的现状

经皮室间隔心肌消融术 (PTSMA)用于梗阻性肥厚型心肌病 (HOCM)治疗的概念源于外科心肌切除术的良好疗效 ,而真正促进其临床应用的是两项观察 :HOCM者自发前壁心肌梗死后症状改善 ;暂时阻断穿隔支血流导致跨左室流出道压差 (LVOG)的一过性下降。 1 994年Sigwart首次报道 3例PTS MA成功。该方法逐渐为人接受 ,并证明成功率高 ,并发症低。至 2 0世纪末 ,已有千余人接受了该手术 ,1 994～ 2 0 0 0年行PTMSA的病例数已超过了 2 0世纪 60年代以来行外科心肌切除手术的HOCM病例的总和。PTMSA的近期疗效令人振奋。 90 %以上的患者LV…     

间隔消融术对比心肌切除术治疗梗阻性肥厚型心肌病研究进展

先前认为室间隔心肌部分切除术是治疗药物难治性梗阻性肥厚型心肌病的金标准,近年来,经皮经腔间隔心肌消融术由于创伤小,操作简单,获得广泛开展并取得了较好的疗效。但究竟哪种是更好的治疗方法,一直存在争论。现就目前药物难治性梗阻性肥厚型心肌病的两种方法疗效比较作一综述。     

经皮经腔间隔心肌化学消融术治疗肥厚型梗阻性心肌病长期疗效观察

本研究旨在评价经皮经腔室间隔化学（无水酒精）消融术（PTSMA）治疗肥厚型梗阻性心肌病（HOCM）的长期疗效（3．5年）。     

外科室间隔切除术改善梗阻性肥厚型心肌病患者症状和预后文献综述

外科室间隔切除术(SSM)应用于严重梗阻性肥厚型心肌病(HCM)患者的治疗已经有60多年历史, 酒精室间隔消融术(ASA)应用于梗阻性HCM的治疗也有近30年时间。本文就SSM手术方式及并发症, 在改善左心室压差、临床症状方面的获益, 降低疾病死亡率及猝死率等方面进行综述, 并与ASA进行对比。     

经皮腔内室间隔化学消融术后行射频消融术治疗梗阻性肥厚型心肌病1例

<正>临床资料患者男性,63岁,3年前因“梗阻性肥厚型心肌病”于我院住院行经皮腔内室间隔化学消融术,半年前无明显诱因出现胸闷、胸痛,30min缓解。入院查体：血压134/82mmHg (1 mmHg=0.133kPa),心率69次/min,呼吸19次/min,胸骨左缘3-4肋间闻及收缩期喷射性杂音。心电图：窦性心动过缓,ST-T改变。实验室检查：NT-pro BNP 499.70ng/L。     

肥厚型梗阻性心肌病无水酒精消融术靶血管选择方法的探讨

经皮经腔室间隔心肌化学消融术(PTSMA)治疗肥厚型梗阻性心肌病(HOCM )具有良好的即刻和近中期疗效[1 4 ] 。PTSMA成功的关键在于正确选择消融靶血管。我们探讨试注射极微量无水酒精结合超声心动图监测的方法在靶血管选择中的价值。资料与方法1.对象:自1999年11月以来对31例HOCM患者实施PTSMA治疗,其中男19例,女12例:年龄2 3～6 9岁,平均(42±9)岁,平均病史5 6年,心功能NYHA分级为(3 1±0 4 )级。所有患者均符合中华心血管病杂志编辑委员会经皮腔内室间隔心肌化学消融术专题组建议的PTSMA适应证[5] 。2 .PTSMA方法:参照…     

肥厚型心肌病猝死相关危险因素的研究进展

肥厚型心肌病是一种相对常见的心脏疾病,在普通人群的发病率为0.2%。其临床特征异质性大,但大部分患者的寿命不受影响。尽管如此,心脏性猝死仍然是一部分患者尤其是年轻患者的首发症状。因而,目前迫切需要寻找合理的危险分层方法,将肥厚型心肌病中心脏性猝死高风险的患者鉴别出来。现重点对肥厚型心肌病的相关危险因素做一总结。     

肥厚型心肌病预后评估的研究进展

肥厚型心肌病是最常见的遗传性心脏病,其临床表现及预后极富多样性。该病患者的不良转归主要有:猝死、心力衰竭以及心房颤动所致的栓塞事件等。现就影响肥厚型心肌病预后的因素作一综述。     

ST Segment “Hump” during Exercise Testing and the Risk of Sudden Cardiac Death in Patients with Hypertrophic Cardiomyopathy

Background: The appearance of a discrete upward deflection of the ST segment termed “the ST hump sign” (STHS) during exercise testing has been associated with resting hypertension and exaggerated blood pressure response to exercise. Objective: We investigated the prevalence and clinical significance of this sign in a population of patients with hypertrophic cardiomyopathy. Methods: Eighty‐one patients with hypertrophic cardiomyopathy (HCM) who underwent cardiopulmonary exercise testing were followed in a retrospective cohort study for a mean period of 5.3 years. Results: The appearance of the STHS at the peak of exercise testing was observed in 42 patients (52%), particularly in the inferior and the lateral leads. Patients with the STHS had higher fractional shortening and maximum left ventricular wall thickness and exhibited more frequently outflow tract gradient >30 mmHg at rest. Furthermore, the presence of STHS was a strong independent predictor of the risk of sudden cardiac death (SCD), as the latter occurred in eight of the patients with this sign (8/42, 19%) and in none of the patients without it (0/39, 0%) (P < 0.001). Conclusion: The appearance of a “hump” at the ST segment during exercise testing appears to be a risk factor for SCD in patients with HCM. However, further studies are necessary to validate this finding in larger populations and to elucidate the mechanism of the appearance of the “hump.”     

Molecular Genetic Basis of Hypertrophic Cardiomyopathy:

Genetics of SCD in HCM. Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disease caused by mutations in sarcomeric proteins. The disease is characterized by left ventricular hypertrophy in the absence of an increased external load, and myofibrillar disarray. A large number of mutations in genes coding for the β-myosin heavy chain (β-MyHC), cardiac troponin T (cTnT), cardiac troponin I, α-tropomyosin, myosin binding protein C (MyBP-C), and myosin light chain 1 and 2 in patients with HCM have been identified. Genotype-phenotype correlation studies have shown that mutations carry prognostic significance. The Gly²⁵⁶Glu, Val⁶⁰⁶Met, and Leu⁹⁰⁸Val mutations in the μ-MyHC are associated with a benign prognosis. In contrast, Arg⁴⁰³Gln, Arg⁷¹⁹Trp, and Arg⁴⁵³Cys mutations are associated with a high incidence of sudden cardiac death (SCD). Mutations in cTnT are associated with a mild degree of hypertrophy, but a high incidence of SCD. Mutations in MyBP-C are associated with mild hypertrophy and a benign prognosis. However, it has become evident that factors other than the underlying mutations, such as genetic background and possibly environmental factors, also modulate phenotypic expression of HCM.     

How to Treat Obstructions in Patients with Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is characterized by the presence of increased thickness of the left ventricular wall that is not solely explained by abnormal loading conditions. Two-thirds of the patients with HCM have an obstruction in the left ventricle after provocation or even while at rest. This obstruction is associated with more symptoms and a worse prognosis. The two main therapeutic approaches for treating a left ventricular obstruction are alcohol septal ablation and surgical myectomy. Both these techniques are discussed in this article. Currently, the final decision concerning the optimal invasive therapy for patients with obstructive HCM must be individualized to each patient depending on his/her wishes and expectations, way of life, age, heart morphology, and hemodynamics, as well as the experience of the treating center.