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Myocarditis

By Brian D. Hoit, MD, Professor of Medicine and Physiology and Biophysics;Director of Echocardiography, Harrington HVI, Case Western Reserve University;University Hospitals, Case Medical Center

Myocarditis is inflammation of the muscle tissue of the heart (myocardium) that causes tissue death.

  • Myocarditis may be caused by many disorders, including infection, toxins and drugs that affect the heart, and systemic disorders such as sarcoidosis, but often the cause is unknown.

  • Symptoms can vary and can include fatigue, shortness of breath, swelling (edema), awareness of heart beats (palpitations), and sudden death.

  • Diagnosis is based on electrocardiography (ECG), measurement of cardiac biomarkers, imaging of the heart, and biopsy of the heart muscle..

  • Treatment depends on the cause and includes drugs to treat heart failure and arrhythmias and rarely surgery.

Inflammation can be spread throughout the heart muscle or confined to one or a few areas. If inflammation extends into the pericardium (the flexible two-layered sac that envelops the heart) , this causes myopericarditis. The extent of myocardial involvement and extension into the pericardium can determine the type of symptoms. Inflammation that is spread throughout the heart may cause heart failure, abnormal heart rhythms, and sometimes sudden cardiac death. Less widespread inflammation is less likely to cause heart failure but can still cause abnormal heart rhythms and sudden cardiac death. Involvement of the pericardium leads to chest pain and other symptoms typical of pericarditis. Some people have no symptoms.

Causes of Myocarditis

Myocarditis may result from infectious or noninfectious causes. Many cases are unable to be identified (idiopathic).

In the United States and most other developed nations, infectious myocarditis is most often caused by a viral infection. The most common viral causes in the United States are parvovirus B19 and human herpes virus 6. In developing nations, infectious myocarditis is most often caused by rheumatic fever, Chagas disease, or AIDS.

Noninfectious causes include substances that are toxic to the heart (such as alcohol and cocaine), certain drugs, and some autoimmune and inflammatory disorders. Myocarditis caused by drugs is termed hypersensitivity myocarditis.

Causes of Myocarditis

Cause

Examples

Autoimmune disorders

Bacterial infections

Cardiotoxins

Alcohol

Cocaine

Drugs

Clozapine

Penicillins

Some diuretics

Fungal infections

Idiopathic

Inflammatory disorders

Parasitic infections

Radiation therapy

Viral infections

Coxsackie B virus

HIV

Human herpes virus 6

Parvovirus B19

Giant cell myocarditis

Giant cell myocarditis is a rare, severe form of myocarditis that has a rapid onset. The cause is unclear but may be autoimmune. A biopsy is done for diagnosis. In people with giant cell myocarditis, the heart is suddenly unable to pump enough blood to support the body's functions (called cardiogenic shock). People also frequently have abnormal heart rhythms that are hard to correct. Giant cell myocarditis has a poor prognosis but immunosuppressive therapy can help improve survival.

Symptoms of Myocarditis

People may have only a few symptoms or have severe and rapidly progressing heart failure and severe heart rhythm abnormalities. Symptoms depend on the cause of the myocarditis as well as the extent and severity of inflammation.

Heart failure symptoms may include fatigue, shortness of breath and swelling (edema).

Some people may have an awareness of heartbeats (palpitations) or fainting. In some people, the first symptom is a sudden, severe abnormal heart rhythm..

When inflammation of the pericardium occurs along with myocarditis, people may have chest pain. Dull or sharp pain may spread to the neck, back, or shoulders. Pain ranges from mild to severe. Chest pain due to pericarditis is usually made worse by movement of the chest such as coughing, breathing, or swallowing food. Pain may be relieved by sitting up and leaning forward.

People with infectious myocarditis may have symptoms of the infection, such as fever and muscle aches before myocarditis develops. Drug-related or hypersensitivity myocarditis may be accompanied by a rash. Some people may have enlarged lymph nodes.

Myocarditis can be acute, subacute or chronic. In some cases, myocarditis can lead to dilated cardiomyopathy.

Diagnosis of Myocarditis

  • Electrocardiography (ECG) and measuring cardiac markers

  • Cardiac imaging

  • Sometimes, endomyocardial biopsy

  • Tests to identify cause

Doctors suspect myocarditis when otherwise healthy people with no risk factors for heart disease have with symptoms of heart failure or abnormal heart rhythms.

ECG is done to look for evidence of a heart problem.

Doctors measure levels of cardiac markers (substances that are present when the heart is damaged) in the blood.

Echocardiography can show abnormalities in people with myocarditis.

Cardiac magnetic resonance imaging may show a characteristic pattern of abnormalities in people with myocarditis.

Endomyocardial biopsy in which a doctor takes a sample of tissue from the inner wall of the heart to examine under a microscope, can be done to confirm myocarditis. However because a diagnosis depends on the doctor obtaining a tissue sample from an area that shows the disease, endomyocardial biopsy may not be the best test for diagnosing endocarditis. Therefore if endomyocardial biopsy shows evidence of myocarditis, the disorder is confirmed, but just because a tissue sample does not show signs of myocarditis does not mean this diagnosis can be eliminated. In addition, because endomyocardial biopsy carries risks of severe complications, including a tear in the wall of the heart and death, it is not routinely done unless doctors suspect giant cell myocarditis (because prompt treatment of giant cell myocarditis may be life saving) or if myocarditis causes severe heart failure or heart rhythm abnormalities.

Diagnosis of cause

After myocarditis is diagnosed, tests to determine cause are done. In a young, previously healthy adult who has a viral infection and myocarditis, an extensive evaluation is usually not necessary.

A complete blood count is helpful to look for certain types of white blood cells (eosinophils), which are present in large numbers in people with hypersensitivity myocarditis.

Cardiac catheterization may be useful for ruling out a decrease in blood flow to the heart because myocarditis can mimic a heart attack.

In other cases, a biopsy of heart tissue may be needed to establish a diagnosis.

Other tests, including tests for autoimmune disorders, human immunodeficiency virus infection, histoplasmosis, and other infections may be needed.

Treatment of Myocarditis

  • Treatment of heart failure and abnormal heart rhythms

  • Treatment of underlying disorder

Treatment of heart failure includes diuretics and nitrates for symptomatic relief. In some cases of heart failure, a surgery such as a left ventricular assist device (LVAD), or heart transplantation may be necessary. Long-term drug treatment of heart failure is needed.

Abnormal heart rhythms are treated with antiarrhythmic therapy. Sometimes a pacemaker is needed if abnormal heart rhythms persist.

Antibiotics or drugs to treat other types of infections may sometimes be given if myocarditis is caused by an infection.

When a drug or toxin causes myocarditis, the causative substance is stopped and corticosteroids are given.

Giant cell myocarditis is treated with corticosteroids and immunosuppressive therapy.

Myocarditis caused by sarcoidosis is treated with corticosteroids.

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