what is cardiomyopathy?

Cardiomyopathy means: disease of the heart muscle . In medicine it is used as a generic term for various heart diseases in which the heart muscle is damaged.

The heart muscle (myocardium in technical terms) is the largest part of the heart wall that surrounds the heart. Its job is to pump the blood from the atria into the heart chambers and finally into the main artery (aorta) . From there, the blood reaches the entire body.

In all forms of cardiomyopathy , the structure of the heart muscle changes increasingly, which limits its performance . For example, if the heart muscle thickens, it can no longer contract properly and less blood flows into the heart chamber. As a result, the heart pumps less blood into the body's circulation. Cardiomyopathies can be congenital or develop in the course of life, including through another disease.

The forms of cardiomyopathy

According to the American Heart Association classification, cardiomyopathies are divided into primary and secondary heart muscle diseases:

Primary and secondary cardiomyopathies:
Doctors call primary cardiomyopathy a heart muscle disease that develops directly on the heart muscle and is limited to it. It can exist from birth or appear later in life.
A secondary cardiomyopathy, however, developed by other diseases :

• Autoimmune diseases (for example rheumatoid arthritis , systemic lupus erythematosus)
• Diseases that change the connective tissue (for example, scleroderma)
• Diseases that affect metabolism (for example Diabetes mellitus, hyperthyroidism) or in diseases in which harmful substances accumulate in the heart.
• In addition, it can also be caused by the effects of medicines, heavy metals, or drugs. Cancer treatment (such as chemotherapy or radiation therapy ) can also lead to cardiomyopathy.

Ischemic Cardiomyopathy
The term "ischemic" means poor blood flow. "Ischemic cardiomyopathy" is a cardiomyopathy that is caused by a circulatory disorder in the heart . The poor blood circulation could be caused by a narrowed coronary artery . This occurs, for example, in coronary heart disease.

Since cardiomyopathies, according to the American Heart Association, are defined as heart muscle diseases in which the heart muscle has not been directly damaged by another cardiovascular disease such as coronary artery disease , high blood pressure or valve disease , the term "ischemic cardiomyopathy" is strictly used incorrect. For this reason, the so-called hypertensive cardiomyopathy , which occurs as a result of high blood pressure (hypertension), is not a cardiomyopathy in the classic sense.

The World Health Organization (WHO) divides cardiomyopathy into five main forms :

• dilated cardiomyopathy (enlargement of the heart cavities)
• hypertrophic cardiomyopathy (thickening of the heart muscle)
• restrictive cardiomyopathy
• arrhythmogenic right ventricular cardiomyopathy
• the so-called unclassifiable cardiomyopathy

Description of the five main types:

Dilated cardiomyopathy (DCM)

In this disease, the structure of the heart muscle changes and it is increasingly interspersed with connective tissue. This leads to an expansion (this is called dilation in medicine) of the heart cavities and thus to an enlargement of the heart . At the onset of this heart disease, it is mainly the left ventricle that is affected.
The impaired heart muscle has a lower capacity , its structure becomes thinner and softer. As a result:

• the heart loses its elasticity , which in turn leads to decreased blood filling of the heart cavities
• the heart has a restricted pumping function and no longer carries enough blood into the main artery (aorta) and thus into the body's circulation
• as the blood flow in the body is disturbed, the blood builds up in the veins .

When the heart can no longer pump enough blood around the body, doctors call this forward failure . If the blood builds up in the veins on the return flow to the heart, this is called backward failure.

Symptoms of dilative cardiomyopathy
Due to the limited pumping capacity, those affected have the typical symptoms of heart failure :

• a limited capacity
• Difficulty breathing (dyspnoea) during physical exertion (at an advanced stage, it can also occur when resting)
• Build-up of fluid in the lung (pulmonary edema) which can sometimes be heard as rattle noises when you breathe
• a tightness in the chest area (doctors refer to this as angina pectoris, patients perceive it primarily as a heart condition), which mainly occurs when there is strong physical or emotional stress
• Cardiac arrhythmias arise because the changed cardiac muscle structure also disrupts the transmission of stimuli to the heart.

If the disease worsens, the right ventricle and the atria can also enlarge (if all four heart cavities are affected, doctors refer to this as global insufficiency). Then there may be other physical complaints:

• Fluid retention in other part of the body (especially in the legs, ankles and organs in the abdomen)
• a congestion of blood , which can be noticed, for example, by protruding jugular veins
• the disturbed blood flow also makes it easier for blood clots to form. If they get to other organs via the bloodstream, they can block a blood vessel there and trigger a heart attack.  

Dilated cardiomyopathy often develops after previous myocarditis ( inflammation of the heart muscle ). High alcohol consumption is also a major cause of the abnormal heart enlargement. There are also a number of other factors that play a role less often, including certain autoimmune diseases (such as systemic lupus erythematosus), thyroid malfunction, special drugs (such as chemotherapeutic agents) and malnutrition.

In up to 50 percent of cases, dilated cardiomyopathy is inherited; doctors refer to this as familial dilated cardiomyopathy (FDC for short).     

Peripartum cardiomyopathy (PPCM)

In rare cases, dilated cardiomyopathy can also develop during pregnancy. Doctors call this peripartum cardiomyopathy.

Here occurs within the last weeks of pregnancy months when the mother suddenly to six after the birth of a heart failure. In contrast to the common form of dilated cardiomyopathy, peripartum cardiomyopathy progresses rapidly, so that the affected woman can develop severe heart failure within a few days, which in an emergency even requires a heart transplant .
Around 80 percent of patients recover from cardiomyopathy, but only 30 percent of those affected heal, with the size and function of the left ventricle normalizing.

The symptoms that indicate peripartum cardiomyopathy are:

• Shortness of breath
• to cough
• Leg edema
• Exhaustion

Hypertrophic cardiomyopathy (HCM)

In the hypertrophic form of cardiomyopathy, a thickening of the heart muscle forms (an increase in the size of tissue or organs is called hypertrophy in medicine). It usually occurs in the left ventricle of the heart, less often in the right one. When the heart muscle is thickened, it can no longer contract as well, which affects the heart's ability to pump.

Normally, a heart muscle only thickens when a person is doing a lot of physical exertion (for example, doing intense exercise). It is different with hypertrophic cardiomyopathy, where the thickening occurs because the fibers of the heart muscle are incorrectly arranged. A genetic predisposition can be found in around 50 percent of patientsfor this heart muscle disease. It occurs frequently in some families. The genes that cause the heart muscle cells to develop are changed in them. As a result, the individual muscle components do not form correctly, but rather arrange themselves incorrectly and enlarge. Some diseases, most of which are hereditary, can damage the heart and cause cardiomyopathy, such as amyloidoses, Friedrich ataxia or Noonan's syndrome.

However, the heart muscle does not thicken equally at every point, but rather unevenly. The thickened part of the heart wall is stiffer than the other areas . Since not only the muscle cells grow, but also connective tissue is stored in the heart wall, this additionally restricts the elasticity of the left ventricle. This creates the following problems:

• Since the thickened heart wall is less elastic, it can no longer expand completely and absorb less blood in the phase of blood filling (diastole)
• the heart muscle can no longer tense sufficiently, which impairs the pumping function of the heart
• the thickened heart wall is poorly supplied with blood

Hypertrophic cardiomyopathy can come in two forms :

1. Hypertrophic Obstructive Cardiomyopathy (HOCM) : It is the most common type of cardiomyopathy. This is where the muscle thickening occurs in the upper area of ​​the heart septum and narrows the outflow path of the left ventricle. As a result of the constriction (which doctors call this obstruction), the blood can no longer flow unhindered from the heart into the main artery (aorta).

2. Hypertrophic non-obstructive cardiomyopathy (HNCM): If the thickening is elsewhere on the heart and it does not reduce blood flow, doctors call it hypertrophic non-obstructive cardiomyopathy.

Symptoms of hypertrophic cardiomyopathy
Hypertrophic cardiomyopathy causes little or no discomfort for many sufferers for a long time. Also, sometimes they happen, sometimes they don't. It has not yet been clarified why they are fluctuating. Usually symptoms are only clearly noticeable in the advanced stage : 

• reduced performance , fatigue.
• a tightness and chest pain (angina)
• Difficulty breathing during physical exertion (in advanced stages, it can also occur during rest and at night)
• Dizziness , which can also lead to brief loss of consciousness
• Build-up of fluid (edema), especially in the lungs and legs
• Cardiac arrhythmias : strong heartbeats can occur, the heart can also stumble and cardiac arrhythmias can develop. If the heart goes completely out of step for a short time, it can lead to fainting .

Since many sufferers have no symptoms for a long time, the heart disease often goes unnoticed. This can have fatal consequences: If cardiac arrhythmias develop, there is an increased risk of blood clots forming. If the thrombus clogs a blood vessel, it can cause a heart attack or stroke .

The arrhythmias can sometimes be so severe that the heart suddenly stops beating. In young people (under 30 years of age), especially competitive athletes, hypertrophic cardiomyopathy is the most common cause of sudden cardiac death.

Restrictive cardiomyopathy (RCM)

It is very rare. In contrast to other forms of cardiomyopathy, restrictive cardiomyopathy does not involve any enlargement of the heart. The organ is normal or even smaller in size. The restricted (restrictive) function of the heart arises because more connective tissue is deposited in the inner heart wall of the heart chamber (doctors call this fibrosis). This stiffens the heart wall , it can no longer stretch properly during the blood filling phase and take in less blood and the heart pumps a smaller amount of blood into the body as a result. The disturbed blood filling phase also leads to blood congestion in the atrium.

With restrictive cardiomyopathy, the typical symptoms of heart failure occur:

• Reduction in performance
• Difficulty breathing during exertion
• Fluid build-up in tissues and organs
• Blood congestion, especially in the jugular vein
• Racing heart ( tachycardia )

Restrictive cardiomyopathy often occurs as a result of endocarditis ( inflammation of the lining of the heart ) or myocarditis (inflammation of the heart muscles).

Arrhythmogenic right ventricular cardiomyopathy

Only the right ventricle (hence the name right ventricular) is affected. In the heart muscle there, heart muscle cells die and are increasingly being replaced by fat and connective tissue. As a result, the heart muscles becomes stiffer and can no longer contract sufficiently. The heart wall of the right ventricle becomes thinner, expands and becomes weaker, in severe cases it can completely lose its functionality.

The first warning signal is usually a racing heart (tachycardia) . Severe cardiac arrhythmias can also develop, which can lead to ventricular fibrillation and sudden death.

Arrhythmogenic right ventricular cardiomyopathy is genetic in half of all patients, but it can also result from another disease.

Other cardiomyopathies

In addition to the four main forms, there are other heart muscle  diseases , for example  non-compaction cardiomyopathy (NCCM), a genetic heart disease in which a tissue disorder of the heart muscle develops and as a result, the performance of the heart is restricted, as well as tako-tsubo -Cardiomyopathy.

Stress cardiomyopathy (also called tako tsubo cardiomyopathy or broken heart syndrome)

In broken heart syndrome, attack- like heart problems occur that feel similar to a heart attack : those affected suddenly feel tightness and a sore chest, have shortness of breath, sweat and faint, suffer nausea and are scared to death. It is estimated that one percent of all patients hospitalized for a suspected heart attack will have broken heart syndrome.   

But unlike in a heart attack, the coronary arteries of those affected are not clogged, but free. In broken heart syndrome, the ability of the heart muscle to move and the ability of the left ventricle to pump deteriorate . In addition, the shape of the left ventricle changes: it is inflated like a balloon at the lower end and narrowed at the upper end. It is reminiscent of a Japanese squid trap, which consists of a bulbous clay jug and a narrow neck and is called "Tako-Tsubo". Since it was Japanese researchers who first described broken heart syndrome in the early 1990s, they called this heart disease "Tako-Tsubo cardiomyopathy".

So far, research has not clearly clarified what the cause of this heart damage is. It is noticeable that Broken Heart Syndrome mainly occurs in people who have experienced an intense emotional stress situation before the heart attack , such as the death of a loved one, an ongoing relationship conflict, financial existential fear. Severe physical stress , for example due to an accident, an infection or other events that are stressful for the body, can also be the trigger.

In 90 percent of cases, women past menopause develop broken heart syndrome, but it also occurs in men. The strong stress “breaks” their hearts, so to speak. Researchers suspect that the stress hormones (such as adrenaline, noradrenaline, dopamine), which are constantly released during the stressful phase, accumulate in the heart of those affected and damage the organ.

For a long time it was assumed that broken heart syndrome was comparatively harmless, but today we know that it is not. In some people, heart function returns to normal within a few weeks, but 50 percent of patients experience complications such as arrhythmia or a blood clot in the first few days after the heart attack. Five percent of patients die from Tako Tsubo cardiomyopathy.
The long-term prognosis is also less positive than previously assumed: studies show that patients who have been affected by Tako Tsubo cardiomyopathy are more prone to other diseases of the heart and brain, such as repeated heart failure or a stroke.

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