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What is cardiomyopathy?

心肌病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causes damage to tissue around the heart, as well as heart muscle cells. In severe cases, the heart becomes so weak that it can’t pump blood properly. This can lead to heart failure or irregular heartbeats (心律不齐s)。在某些情况下,心肌病也涉及心肌内的疤痕组织或脂肪。在极少数情况下,心肌无法放松,血液无法正确充满心脏。

心肌病通常是一种“时间意见”疾病。症状可能会有所不同,疾病的进展可能是不可预测的。

What are the types of cardiomyopathy?

There are many forms of cardiomyopathy. The main types include:

Dilated cardiomyopathy (DCM)

DCM是最常见的类型,当心肌的主泵室太伸展时发生(扩张)。扩张的心肌病使心脏无法有效泵血。

Hypertrophic cardiomyopathy (HCM)

HCM makes the heart muscle too thick. Usually, the thickening occurs in the muscle of the left ventricle in the heart, often involving the wall between the heart’s two ventricles.

Restrictive cardiomyopathy

限制性心肌病是一种罕见的心肌病,会导致心肌变得非常僵硬。这使得心脏的心室难以正确充满血液。

心律失常右心肌病(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.

我们如何照顾心肌病

在波士顿儿童医欧宝彩票平台院心肌病计划, our cardiologists, cardiac surgeons and nurses, cardiac imaging professionals and other clinicians have years of expertise intreatingnot only the multiple forms of cardiomyopathy in children, but also the various stages of the disease.

心肌病|Symptoms & Causes

What are the symptoms of cardiomyopathy?

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:

  • abdominal pain
  • chest pain
  • chronic fatigue
  • chronic loss of appetite or inability to feed
  • 频繁烦躁而没有明显原因
  • 经常呕吐
  • pale or clammy skin
  • 快速呼吸
  • 快速或“赛车”心跳
  • shortness of breath
  • slow or delayed growth

肥厚的心肌病症状

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

  • abnormal heart rhythm
  • chest pain
  • dizziness
  • 晕倒

Restrictive cardiomyopathy 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.

Symptoms can include:

  • fatigue
  • persistent coughing
  • shortness of breath during exercise or right after exercise
  • 晚上呼吸急促(休息或睡觉时)
  • 平躺时的呼吸急促
  • 腹部肿胀
  • 脚或脚踝肿胀
  • 增长不佳或不足增加体重

心律失常右心室心肌病症状

心律失常右心肌病(ARVC)is often difficult to diagnose because many people never have symptoms or develop symptoms suddenly after not having any problems.

Symptoms can include:

  • abnormal heart rhythm
  • 晕倒
  • 头晕或头晕
  • racing heartbeat
  • shortness of breath
  • 腹部肿胀
  • 腿肿胀

What are the causes of cardiomyopathy?

某些形式的心肌病在家庭中进行。任何具有心肌病,心脏病或心律不齐的家族史的人都应筛查。基因检测可以检测约50%的病例。在其他情况下,心肌病由于另一种疾病而发展。但是,在许多情况下,没有明显的心肌病(特发性心肌病)的原因。

扩张的心肌病因

In many cases, DCM occurs without a known reason.

When the cause can be determined, reasons include:

  • myocarditis, an inflammation of the heart muscle that usually stems from a
  • 病毒感染
  • certain metabolic diseases
  • certain genetic disorders, like肌营养不良症
  • inheriting the condition from a parent

DCM的一些不常见原因是:

  • 结缔组织障碍,例如类风湿关节炎
  • coronary artery disease
  • diabetes that is not properly controlled
  • obesity
  • substance abuse
  • 甲状腺疾病

肥厚的心肌病因

Hypertrophic cardiomyopathy (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

Restrictive cardiomyopathy is not typically inherited, and many children have no identifiable cause for their disease.

当有已知原因时,这些可能包括:

  • chemotherapy treatments for certain cancers
  • radiation treatments to the chest for certain cancers
  • sarcoidosis
  • 硬皮病
  • diseases of the heart lining, including endomyocardial fibrosis and Loeffler’s
  • syndrome
  • 心脏过多铁(血色素沉着病)
  • 心脏肌肉中的异常蛋白质积聚(淀粉样变性)
  • tumors in the heart
  • a buildup of scar tissue in the heart

Arrhythmogenic right ventricular cardiomyopathy causes

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.

心肌病|Diagnosis & Treatments

How is cardiomyopathy diagnosed?

由于心肌病的症状可能多种多样,因此通常被误诊为哮喘,感染或胃肠道问题。

In children who have no symptoms, cardiomyopathy is sometimes diagnosed when the child has a chest x-ray that shows an enlarged heart or an echocardiogram for another reason.

如果你的孩子的医生怀疑心肌病,他or she may order one or more of the following tests:

What are the treatment options for cardiomyopathy?

Your child's treatment options will be determined by the type of cardiomyopathy he or she has, as well as the specific症状。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 a心脏移植

心肌病的药物

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

  • Angiotestinconverting enzyme (ACE) inhibitors are drugs that dilate blood vessels in the body, fighting the constricting effect caused by heart failure.
  • Antiarrhythmic medicationscombat the abnormal heart rhythms caused by irregular electrical activity within the heart.
  • beta阻滞剂阻止某些化学物质与心脏中的神经受体结合,从而减慢心率并降低血压。
  • 血液稀释剂或抗凝剂help prevent the formation of blood clots, especially in children with the dilated form of cardiomyopathy.
  • Diureticsprevent the buildup of fluid in the body and can help breathing by reducing fluid in the lungs. These drugs may also be helpful in treating scar tissue on the heart.

Surgical treatments for cardiomyopathy

有几种使用手术或微创手术治疗心肌病的选择。

除颤器

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 an心律不齐, the defibrillator will administer a precise electrical pulse to restore normal heart rhythm.

Pacemakers

Pacemaker implantation is minimally invasive option for some children with cardiomyopathy. The pacemaker — a small electronic device — is inserted directly under the skin, where it sends electric signals to the child's heart, controlling and monitoring the heart rate. The procedure can be performed under local anesthesia in a matter of hours.

Radiofrequency ablation

Radiofrequency ablation is another type of minimally invasive treatment that can be very effective for some children with cardiomyopathy due to arrhythmia. A small, needle-like probe is inserted into the scarred tissue of the heart muscle, sending out radiofrequency waves that burns away the scar tissue and the arrhythmia.

Surgical removal of some heart muscle

在严重的肥厚性心肌病病例中,治疗小组可能会进行称为中间肌切除术或间隔肌瘤切除术的手术。该过程涉及去除心脏中增厚的一部分肌肉,从而拓宽导致主动脉瓣的心室中的通道。该手术的成功率非常好,大多数儿童的血液流动在整个心脏和身体中都得到了改善。

Ventricular assist device

在某些类型的心肌病中,可以使用心室辅助装置(VAD)来帮助恢复心脏并使心脏的功能正常化。心脏的功能恢复正常后,将删除VAD,并将监视孩子的任何进一步的心脏问题。这种使用VAD的方法称为“恢复桥梁”。

心脏移植

Children with the most severe cases of cardiomyopathy may need a心脏移植如果其他方法无法管理症状。当您的孩子正在等待可用的心脏时,可以使用VAD来支撑心脏。在许多情况下,儿童可以在等待移植时重返学校和其他活动。

心肌病|程式& Services

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