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. 这些基因导致心腔(左心室)壁比正常情况下变厚.
增厚的壁可能会变得僵硬，这可能会减少每次心跳时吸入和排出身体的血液量.
下载可列印的资料单张:
Watch this episode of House Calls: Explaining HCM (Video).
梗阻性和非梗阻性HCM
梗阻性HCM, 心肌增厚的部分, usually the wall (septum) between the two bottom chambers (ventricles), blocks or reduces the blood flow from the left ventricle to the aorta. 三分之二的HCM患者属于这种类型.
在非梗阻性HCM中，心肌增厚但不阻碍血液流出心脏.
体征、症状和风险
一些肥厚性心肌病患者没有症状，而另一些人可能只有在运动或用力时才会感到症状. 有些人在疾病的早期阶段可能没有症状或体征，但随着线上电子游戏飞禽走兽的推移可能会出现症状.
Knowing the signs and symptoms of HCM is important. It can help with getting an early diagnosis when treatment may be most effective.
HCM的症状和体征包括:
- 胸痛，尤指体力消耗时的胸痛
- Shortness of breath, especially with physical exertion
- 乏力
- 心律失常 (心律失常)
- 头晕
- 头晕
- 晕倒(晕厥)
- Swelling in the lower part of your body (ankles, feet, legs) or in neck veins.
HCM is a chronic disease that can get worse over time. This can lead to poorer function and quality of life, long-term complications and more financial and social burden.
As HCM progresses, it can cause other health problems. People with HCM are at higher risk for developing 心房纤颤会导致血栓， 中风 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被认为是北美年轻人和竞技运动员心脏性猝死的最常见原因, 虽然这种情况很少见.
得到诊断
肥厚性心肌病通常是遗传性的，是一种常见的遗传性心脏病. It can happen at any age, but most receive a diagnosis in middle age.
据估计，在美国，每500名年轻人中就有1人患有HCM, but a large percentage of people are undiagnosed. 那些被确诊的, 据估计，三分之二的人患有阻塞性HCM，三分之一的人患有非阻塞性HCM.
A cardiologist or pediatric cardiologist often diagnoses and treats HCM. 你也可能被转介到心肌病中心，那里的医疗团队有专门的培训.
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
了解你的病史和你可能有的任何体征和症状是重要的第一步. Because HCM can be passed from parents to children, 你的医疗保健专业人员也会想知道你家里是否有人被诊断患有HCM, 心脏衰竭 or 心脏骤停. 如果有人被诊断患有HCM, first degree relatives which include siblings and parents, 也应该检查一下. 了解更多关于 基因检测.
物理考试
Your health care professional will listen to your heart and lungs with a stethoscope. If they hear a swishing or whooshing sound called a murmur, 这可能意味着心脏血液流动有问题，这可能意味着HCM.
诊断测试
诊断通常由 超声心动图. 它检查心肌的功能和厚度，以及血液如何流经心脏. 在某些情况下，另一种超声心动图， 技术回声 (或TEE)，可以执行. A TEE is done using a probe inserted in the throat while the patient is under sedation.
其他诊断测试可能包括:
- 心电图(ECG)
- Cardiac magnetic resonance imaging (cardiac MRI or CMR)
- 心脏CT
- 压力测试
- 霍尔特和线上娱乐电子游戏网站监视器 检测异常心律(心律失常)
- 基因检测
诊断程序
确认诊断或准备手术也可能涉及一个或多个医疗程序 心导管检查.
HCM的治疗和管理
目前只有一种疾病特异性药物治疗肥厚性心肌病. Mavacamten is used to treat the obstructive form of HCM in people who have symptoms.
对于HCM患者来说，心脏健康 生活方式 包括保持活跃, 健康饮食, 保持正常体重, getting good quality sleep and not smoking is recommended. If you have other medical conditions like high blood pressure or diabetes, 重要的是要设法避免心脏并发症，如果不加以控制，可能会发展.
对于那些有症状的人，重点是使用药物和程序来控制症状.
药物
遵医嘱服药是很重要的. These medications may help improve symptoms and function, but may have side effects. 药物 that may be prescribed for HCM include:
可能改善症状
能改善症状和功能吗
- 肌凝蛋白抑制剂
不像其他HCM药物, 心脏肌球蛋白抑制剂马伐camten用于改善有轻度至中度活动性症状的阻塞性HCM患者的症状和功能. 不像其他治疗HCM的药物, mavacamten targets the underlying cause of obstructive HCM.
程序
A range of surgical and nonsurgical procedures can be used to treat HCM:
- 隔肌切除术 – 隔肌切除术, also called septal reduction therapy, is open-heart surgery. It’s considered for people with obstructive HCM who, 尽管服用了HCM药物, 持续出现严重症状. A surgeon removes part of the thickened septum that’s bulging into the left ventricle. 这样可以消除阻塞，恢复心脏内部和身体的血液流动.
- 酒精室间隔消融术(非手术) – Also called nonsurgical septal reduction therapy, alcohol septal ablation 是一种将乙醇(一种酒精)通过一根管子注入小动脉的手术，小动脉向心肌增厚的区域供血. 酒精会导致这些细胞死亡. The thickened tissue shrinks to a more normal size. The risks and complications of heart surgery increase with age. 出于这个原因, 对于有其他疾病的老年患者，消融可能优先于室间隔肌切除术.
- 心脏植入式电子装置(cied) -视具体患者风险因素而定, there are devices that can be implanted in the body to help the heart work better, 包括:
- 植入式心律转复除颤器 —当检测到心跳不规律时，ICD通过对心脏进行电击来维持心跳正常. This may reduce the risk of sudden cardiac death.
- 起搏器 – This small device uses electrical pulses to prompt the heart to beat at a normal rate. This is used for people with a heart rate that is too slow.
- 心脏再同步化治疗(CRT)装置 – This device coordinates contractions between the heart’s left and right ventricles.
- 心脏移植手术 -晚期HCM患者可考虑心脏移植. In this procedure, a person’s diseased heart is replaced with a healthy donor heart.
预后
HCM患者的长期预后良好，大多数HCM患者的预期寿命正常，没有明显的限制或并发症.
少数HCM患者, 然而, are at risk for complications including 心脏衰竭 and sudden death. 心脏并发症的风险在不同的家庭和同一家庭的不同成员之间是不同的. 重要的是，你的医疗团队要确定哪些人最有可能出现这些并发症，这样才能提供预防性治疗.
共同决策
对于患有HCM或有HCM风险的人, shared decision-making is recommended in developing a care plan. 这包括关于基因评估、身体活动、生活方式和治疗选择的决定.
这应该包括患者、家属和他们的护理团队之间的深思熟虑的对话，讨论:
- 检测和治疗方案
- Risks and benefits of those options to the patient
- Patient’s personal preferences and goals for their treatment plan
- 照顾者的目标和关注点
儿童与HCM
大多数儿童没有症状, 而其他人可能有轻微到严重的症状，包括心力衰竭或心脏骤停. Children under age 1 more often have symptoms of congestive 心脏衰竭. Older children may be symptom-free and unaware they have HCM. Mild symptoms of 心脏衰竭 can also look like asthma.
Symptoms in infants may be more difficult to detect. 它们可能包括:
- 呼吸困难
- 可怜的增长
- 过度出汗
- 喂奶时啼哭和躁动
许多患有HCM的儿童一旦被诊断出来并开始适当的治疗，就可以过上相对正常的生活. However, an HCM diagnosis does affect several areas of a child’s life. Specific recommendations should be developed by the child’s health care team.
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