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什么是心肌病?

Cardiomyopathy is a disease of the heart muscle characterized by an abnormally large, thick or stiff heart muscle. It may affect only the heart’s lower chambers (ventricles) or both the lower and upper chambers (atria).

心肌病会损害心脏周围的组织以及心肌细胞。在严重的情况下,心脏变得如此虚弱,无法正确抽血。这会导致心力衰竭或心跳不规则(arrhythmias). In some cases, cardiomyopathy also involves a buildup of scar tissue or fat within the heart muscle. In rare cases, the heart muscle can’t relax and blood can’t fill the heart properly.

Cardiomyopathy is very often a “time-will-tell” disease. Symptoms can vary and the progression of the disease can be unpredictable.

心肌病的类型是什么?

心肌病有多种形式。主要类型包括:

Dilated cardiomyopathy (DCM)

DCM is the most common type and occurs when the main pumping chamber of the heart muscle is too stretched out (dilated). Dilated cardiomyopathy makes the heart unable to pump blood effectively.

肥厚性心肌病(HCM)

HCM使心肌太厚。通常,增厚发生在心脏左心室的肌肉中,通常涉及心脏两个心室之间的壁。

限制性心肌病

限制性心肌病is a rare type of cardiomyopathy that causes the heart muscle to become very rigid or stiff. This makes it difficult for the ventricles of the heart to properly fill with blood.

Arrhythmogenic right ventricular cardiomyopathy (ARVC)

ARVC is a rare form of cardiomyopathy that affects only one in 5,000 people. It occurs when the muscle of the heart’s right ventricle is replaced by thick or fatty scar tissue. The scarring “scrambles” electrical signals within the heart and can make it difficult for the heart to pump blood.

How we care for cardiomyopathy

At the Boston Children’s HospitalCardiomyopathy Program, our cardiologists, cardiac surgeons and nurses, cardiac imaging professionals and other clinicians have years of expertise in治疗not only the multiple forms of cardiomyopathy in children, but also the various stages of the disease.

Cardiomyopathy |Symptoms & Causes

心肌病的症状是什么?

Some children with cardiomyopathy have few to no signs of illness at first, while others may be seriously affected by shortness of breath, dizziness and other symptoms shortly after birth.

Dilated cardiomyopathy symptoms

Many children with dilated cardiomyopathy (DCM) don’t have any symptoms. Those who do may have one or more of the following symptoms, which may vary depending on the age of the child:

  • 腹痛
  • 胸痛
  • chronic fatigue
  • 长期失去食欲或无能为力
  • frequent irritability without a visible cause
  • frequent vomiting
  • 苍白或张开的皮肤
  • rapid breathing
  • rapid or “racing” heartbeat
  • 呼吸急促
  • slow or delayed growth

Hypertrophic cardiomyopathy symptoms

Children with hypertrophic cardiomyopathy (HCM) may have symptoms during physical activity, or their symptoms may come on suddenly and unpredictably. Common symptoms signs include:

  • 心律异常
  • 胸痛
  • dizziness
  • fainting

限制性心肌病symptoms

Symptoms of restrictive cardiomyopathy can vary greatly from child to child. Many children have no noticeable symptoms, others have very mild symptoms, while others have clear warning signs that become progressively worse if the disease is not treated.

症状可能包括:

  • fatigue
  • persistent coughing
  • 运动期间或运动后的呼吸急促
  • 气短晚上歇息的时候(或系统性红斑狼疮eping)
  • 呼吸急促while lying flat
  • swelling in the abdomen
  • swelling in the feet or ankles
  • poor growth or failure to gain weight

Arrhythmogenic right ventricular cardiomyopathy symptoms

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is often difficult to diagnose because many people never have symptoms or develop symptoms suddenly after not having any problems.

症状可能包括:

  • 心律异常
  • fainting
  • lightheadedness or dizziness
  • 赛车心跳
  • 呼吸急促
  • swelling in the abdomen
  • swelling in the legs

What are the causes of cardiomyopathy?

Some forms of cardiomyopathy run in families. Anyone with a family history of cardiomyopathy, heart attack or arrhythmias should be screened. Genetic testing can detect about 50 about percent of cases. In other cases, cardiomyopathy develops because of another medical condition. In many cases, however, there is no clear cause for cardiomyopathy (idiopathic cardiomyopathy).

Dilated cardiomyopathy causes

在许多情况下,DCM无需已知原因。

确定原因时,原因包括:

  • 心肌炎,心肌的炎症,通常源自
  • viral infection
  • 某些代谢疾病
  • certain genetic disorders, likemuscular dystrophy
  • 从父母那里继承条件

Some less common causes of DCM are:

  • connective tissue disorders, such as rheumatoid arthritis
  • coronary artery disease
  • 无法正确控制的糖尿病
  • 肥胖
  • 药物滥用
  • thyroid disease

Hypertrophic cardiomyopathy causes

肥厚性心肌病(HCM)has a strong inherited component. At least half of all children with HCM have a parent or sibling with some enlargement in their heart muscle, although these family members may not have any symptoms themselves.

HCM is caused by a defect in the genes that control the manufacture of the proteins of the heart muscle.

限制性心肌病causes

限制性心肌病通常不会遗传,许多儿童没有可识别的疾病原因。

When there is known cause, these may include:

  • 某些癌症的化学疗法治疗
  • 某些癌症对胸部的辐射处理
  • sarcoidosis
  • scleroderma
  • 心脏衬里的疾病,包括心内膜纤维化和Loeffler的疾病
  • 综合征
  • too much iron in the heart (hemochromatosis)
  • an abnormal protein buildup in the muscle of the heart (amyloidosis)
  • 心脏中的肿瘤
  • a buildup of scar tissue in the heart

心律失常右心肌病因

ARVC通常会在家庭中呈现类似情况。至少30到50 /cent of all people with ARVC have family members with the disease. Researchers are working to identify precisely which gene mutations and chromosomes are involved in the onset of ARVC.

Cardiomyopathy |诊断和治疗

如何诊断心肌病?

Because the symptoms of cardiomyopathy can be diverse, it is often misdiagnosed as asthma, an infection or a gastrointestinal problem.

在没有症状的儿童中,当孩子患有胸部X射线检查时,有时会诊断出心肌病,出于另一个原因显示心脏肿大或超声心动图。

如果您孩子的医生怀疑心肌病,他或她可能会订购以下一项测试:

心肌病有哪些治疗选择?

您的孩子的治疗选择将由他或她的心肌病类型以及特定的患者确定symptoms. A child with no symptoms might not need medication or other treatment right away. Instead, the cardiologist will monitor your child to gauge the progression of the disease.

A child with more serious symptoms may need additional tests to give the treatment team more detailed information about how the cardiomyopathy is affecting the heart and the rest of the body. .

Many children with cardiomyopathy do well with medication alone. Others need surgery to improve the function of valves, and in the most severe cases, some children need aheart transplant.

Medication for cardiomyopathy

There are several different types of medications for cardiomyopathy, depending on which type your child has and the symptoms.

  • 血管紧张病converting enzyme (ACE) inhibitors are drugs that dilate blood vessels in the body, fighting the constricting effect caused by heart failure.
  • 抗心律失常药物combat the abnormal heart rhythms caused by irregular electrical activity within the heart.
  • Beta blockersblock certain chemicals from binding to nerve receptors in the heart, slowing the heart rate and lowering blood pressure.
  • Blood thinners or anticoagulants有助于防止血凝块的形成,尤其是在心肌病的扩张形式的儿童中。
  • 利尿剂防止体内液体积聚,并通过减少肺部的液体来帮助呼吸。这些药物也可能有助于治疗心脏上的疤痕组织。

心肌病的手术治疗

There are several options for treating cardiomyopathy using surgery or minimally-invasive procedures.

Defibrillators

For some children with cardiomyopathy — particularly HCM — an implantable cardioverter-defibrillator (ICD) can be a life-saving option. This tiny instrument, about the size of a deck of cards, is placed in the chest to monitor the child's heartbeat. If the child has anarrhythmia, the defibrillator will administer a precise electrical pulse to restore normal heart rhythm.

起搏器

对于一些心肌病儿童来说,起搏器植入是最少的侵入性选择。起搏器(一种小型电子设备)直接在皮肤下插入,它将电信号发送到孩子的心脏,控制和监测心率。该过程可以在几个小时内在局部麻醉下进行。

射频消融

射频消融是另一种微创治疗方法,对于某些由于心律不齐而导致的心肌病儿童可能非常有效。将小针状的探针插入心肌的疤痕组织中,发出射频波,燃烧疤痕组织和心律不齐。

Surgical removal of some heart muscle

In serious cases of hypertrophic cardiomyopathy, the treatment team may perform a surgery called a septal myectomy or septal myomectomy. This procedure involves the removal of a portion of the thickened muscle in the heart, widening the channel in the heart's ventricle that leads to the aortic valve. The procedure has a very good success rate, and most children have improved blood flow throughout the heart and body.

心室辅助装置

In certain types of cardiomyopathy, a ventricular-assist device (VAD) can be used to help recover the heart and normalize the heart’s function. After the heart’s function has returned to normal, the VAD will be removed and the child will be monitored for any further heart problems. This approach using the VAD is called a “bridge to recovery.”

Heart transplant

心肌病例最严重的儿童可能需要heart transplantif other methods don’t manage symptoms. While your child is waiting for an available heart, a VAD may be used to support the heart. In many cases, children can return to school and other activities while waiting for a transplant.

Cardiomyopathy |程序和服务

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