肥厚性心肌病(HCM)

什么是肥厚性心肌病?

Hypertrophic cardiomyopathy is most often caused by abnormal genes in the heart muscle. These genes cause the walls of the heart chamber (left ventricle) to become thicker than normal.

The thickened walls may become stiff and this can reduce the amount of blood taken in and pumped out to the body with each heartbeat.

下载可列印的资料单张:

• 什么是肥厚性心肌病? (PDF)
• 什么是HCM? 西班牙语(PDF)

观看这期《沙巴足球体育平台》(视频).

梗阻性和非梗阻性HCM

梗阻性HCM, 心肌增厚的部分, 通常是两个底部腔室(心室)之间的壁(隔膜), 阻断或减少从左心室流向主动脉的血液. 大多数HCM患者都是这种类型.

In nonobstructive HCM, the heart muscle is thickened but doesn’t block blood flow out of the heart.

体征、症状和风险

Some people with hypertrophic cardiomyopathy don’t have symptoms while others may only feel symptoms with exercise or exertion. Some people may not have signs or symptoms in the early stages of the disease but may develop them over time. 

了解HCM的症状和体征很重要. 它可以帮助在治疗最有效的时候得到早期诊断.

HCM的症状和体征包括:

• 胸痛，尤指体力消耗时的胸痛
• 呼吸急促呼吸急促，尤指体力消耗时
• Fatigue
• 心律失常 (心律失常)
• Dizziness
• 头晕
• 晕倒(晕厥)
• 脚踝，脚，腿，腹部肿胀

HCM是一种慢性疾病，会随着沙巴足球体育平台的推移而恶化. 这会导致身体功能和生活质量下降, 长期并发症和更多的经济和社会负担. 

HCM患者可能需要改变生活方式, 比如限制他们的活动, 为了适应他们的疾病.  

随着HCM的发展，它会导致其他健康问题. 患有HCM的人患HCM的风险更高 心房纤颤会导致血栓， stroke 以及其他与心脏有关的并发症. HCM也可能导致 心脏衰竭. 它也可能导致 心脏骤停，但这种情况很少见. 

HCM has been regarded as the most common cause of sudden cardiac death in young people and competitive athletes in North America, 虽然这种情况很少见.

得到诊断 

Hypertrophic cardiomyopathy is most often inherited and is the most common form of genetic heart disease. 它可以发生在任何年龄，但大多数在中年被诊断出来. 

据估计，每500人中就有1人患有HCM, 但很大一部分患者没有得到诊断. Of those diagnosed, two-thirds have obstructive HCM and one-third have non-obstructive HCM.

心脏病专家或儿科心脏病专家经常诊断和治疗HCM. You may also be referred to a cardiomyopathy center where the health care team has specialized training.

HCM的诊断是基于你的病史, 家族病史, 体检和诊断测试结果.  

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病史和家族史

Knowing your medical history and any signs and symptoms you may have is an important first step. 因为HCM可以从父母遗传给孩子, your physician will also want to know if anyone in your family has been diagnosed with HCM, 心脏衰竭 or 心脏骤停. 如果有人被诊断患有HCM, 一级亲属，包括兄弟姐妹和父母, 也应该检查一下. 了解更多关于基因检测的信息 资料指引(PDF).

物理考试

你的医生会用听诊器听你的心脏和肺. 如果它们听到嗖嗖声或嗖嗖声，就叫杂音, that could mean there is problem with blood flow through the heart which may suggest HCM.  

诊断测试

诊断通常由 超声心动图. 它检查心肌的厚度和流经心脏的血液流量. 在某些情况下，另一种超声心动图， 技术回声 (或TEE)，可以执行. TEE是在病人处于镇静状态时用探针插入喉咙进行的.

其他诊断测试可能包括:

• 心电图(ECG)
• 心脏磁共振成像(MRI)
• 压力测试
• 霍尔特和沙巴体育平台点击进入监视器 检测异常心律(心律失常)
• 基因检测

HCM影像(PDF) | 西班牙语(PDF)

诊断程序

Confirming diagnosis or preparing for surgery may also involve one or more medical procedures during 心导管检查.

HCM的治疗和管理

There is currently only one disease-specific medication to treat hypertrophic cardiomyopathy. Mavacamten, 这种药物于2022年4月获得了FDA的批准, 用于治疗有症状的HCM的梗阻性形式.

对于没有症状的HCM患者，心脏健康 lifestyle 包括保持活跃, 健康饮食, 保持正常体重, 建议睡眠质量好，不要吸烟. 如果你有其他疾病，比如高血压或糖尿病, it’s important that these are managed to avoid heart complications which can develop if left unchecked. 

For those with symptoms, the focus is on symptom management using medications and procedures.

药物

It's important to take the medications that have been prescribed by your health care professional. 这些药物可能有助于改善症状和功能，但可能有副作用. 治疗HCM的处方药物包括:

可能改善症状

• β-受体阻滞药
• 钙通道阻滞剂
• Diuretics

能改善症状和功能吗

• 肌凝蛋白抑制剂*

不像其他HCM药物, the cardiac myosin inhibitor mavacamten (Camzyos) is a new type of medication used to improve symptoms and function in patients with the obstructive type of HCM who have mild to moderate symptoms with activity. Unlike other medicines use to treat HCM, mavacamten targets the underlying cause of obstructive HCM. 

*Mavacamten is not in the current 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients with Hypertrophic 心肌病, 因为它是FDA在2022年批准的.

程序

多种手术和非手术方法可用于治疗HCM:

• 隔肌切除术 -室间隔肌切除术，也称为室间隔缩小疗法，是一种开胸手术. 这是为患有阻塞性HCM的人考虑的, 尽管服用了HCM药物, 持续出现严重症状. 外科医生切除了一部分膨胀到左心室的增厚的隔膜. This eliminates the obstruction and restores blood flow within the heart and out to the body.
  
  
• 酒精室间隔消融术(非手术) – 也称为非手术性鼻中隔复位治疗，酒精性鼻中隔消融 is a procedure where ethanol (a type of alcohol) is injected through a tube into the small artery that supplies blood to the area of heart muscle thickened by HCM. 酒精会导致这些细胞死亡. 增厚的组织收缩到更正常的大小. 心脏手术的风险和并发症随着年龄的增长而增加. 出于这个原因, ablation may be preferred to septal myectomy in older patients with other medical conditions.
  
  
• 心脏植入式电子装置(cied) – Several types of devices can be implanted in the body to help the heart work better, including:
  • 植入式心律转复除颤器 – An ICD helps maintain a normal heartbeat by sending an electric shock to the heart if an irregular heartbeat is detected. 这降低了心源性猝死的风险.
  • Pacemaker – This small device uses electrical pulses to prompt the heart to beat at a normal rate. 这是用于心率过慢的人.
  • 心脏再同步化治疗(CRT)装置 -这个装置协调心脏左右心室之间的收缩.
• 心脏移植手术 – In HCM patients with advanced, end-stage disease, a heart transplant may be considered. 在这个过程中，一个人患病的心脏被一个健康的供体心脏所取代.

Prognosis

The long-term outcome for people with HCM is very good and most patients with HCM have normal life expectancy without significant limitations or complications.

少数HCM患者, however, 是否有心脏衰竭和猝死等并发症的风险. The risk of heart complications can vary between families and among different members of the same family, so it’s important that your health care team identifies people who are most at risk for these complications so preventative treatment can be provided.