Arrhythmogenic Cardiomyopathies

Cardiomyopathy refers to progressive impairment of the structure and function of the muscular walls of the heart chambers.

There are three main types of cardiomyopathy:

Any dilated or hypertrophic cardiomyopathy (see Overview of Cardiomyopathy Overview of Cardiomyopathy Cardiomyopathy refers to progressive impairment of the structure and function of the muscular walls of the heart chambers. There are three main types of cardiomyopathy: Dilated cardiomyopathy... read more ) can cause changes in the heart that can lead to different abnormal heart rhythms Overview of Abnormal Heart Rhythms Abnormal heart rhythms (arrhythmias) are sequences of heartbeats that are irregular, too fast, too slow, or conducted via an abnormal electrical pathway through the heart. Heart disorders are... read more (arrhythmias), including excessively slow or fast rhythms and sudden death (cardiac arrest Cardiac Arrest and CPR Cardiac arrest is when the heart stops pumping blood and oxygen to the brain and other organs and tissues. Sometimes a person can be revived after cardiac arrest, particularly if treatment is... read more ). Certain cardiomyopathies are particularly likely to cause arrhythmias. These are referred to as arrhythmogenic cardiomyopathies, and include

Some of these disorders have genetic causes, which are typically inherited mutations.

The symptoms of the arrhythmogenic cardiomyopathies include symptoms of heart failure Symptoms Heart failure is a disorder in which the heart is unable to keep up with the demands of the body, leading to reduced blood flow, back-up (congestion) of blood in the veins and lungs, and/or... read more itself, along with those of a too slow or too rapid heart beat. Heart failure manifestations include shortness of breath Shortness of Breath Shortness of breath—what doctors call dyspnea—is the unpleasant sensation of having difficulty breathing. People experience and describe shortness of breath differently depending on the cause... read more during exertion, excessive fatigue, difficulty breathing while lying flat (orthopnea), and/or swelling of the legs and feet Swelling Swelling is due to excess fluid in the tissues. The fluid is predominantly water. Swelling may be widespread or confined to a single limb or part of a limb. Swelling is often in the feet and... read more . Abnormal heart rhythms may cause palpitations Palpitations Palpitations are the awareness of heartbeats. The sensation may feel like pounding, fluttering, racing, or skipping beats. Other symptoms—for example, chest discomfort or shortness of breath—may... read more , light-headedness, fainting Fainting Light-headedness (near syncope) is a sense that one is about to faint. Fainting (syncope) is a sudden, brief loss of consciousness during which the person falls to the ground or slumps in a... read more , cardiac arrest Cardiac Arrest and CPR Cardiac arrest is when the heart stops pumping blood and oxygen to the brain and other organs and tissues. Sometimes a person can be revived after cardiac arrest, particularly if treatment is... read more , and sudden death.

Evaluation typically includes family history, electrocardiography Electrocardiography Electrocardiography (ECG) is a quick, simple, painless procedure in which the heart’s electrical impulses are amplified and recorded. This record, the electrocardiogram (also known as an ECG)... read more (ECG), chest x-ray, echocardiography Echocardiography and Other Ultrasound Procedures Ultrasonography uses high-frequency (ultrasound) waves bounced off internal structures to produce a moving image. It uses no x-rays. Ultrasonography of the heart (echocardiography) is one of... read more , and magnetic resonance imaging (MRI) of the heart Magnetic Resonance Imaging (MRI) of the Heart With magnetic resonance imaging (MRI), a powerful magnetic field and radio waves are used to produce detailed images of the heart and chest. This expensive and sophisticated procedure is used... read more . Sometimes, doctors need other tests, including exercise testing, ambulatory electrocardiographic monitoring, chest computed tomography (CT), electrophysiologic testing, coronary angiography, and/or biopsy of the heart. Genetic testing is usually done when a genetic cause is suspected.

Close relatives of people with an arrhythmogenic cardiomyopathy may be at risk and should be examined by a doctor to look for evidence of heart disease. Relatives may need certain tests as well.

Treatment depends on the cause of the cardiomyopathy and the specific rhythm disturbance, but typically includes standard treatments for heart failure Treatment Heart failure is a disorder in which the heart is unable to keep up with the demands of the body, leading to reduced blood flow, back-up (congestion) of blood in the veins and lungs, and/or... read more , antiarrhythmic drugs Drugs to Treat Abnormal Heart Rhythms There are many causes of abnormal heart rhythms (arrhythmias). Some arrhythmias are harmless and do not need treatment. Sometimes arrhythmias stop on their own or with changes in lifestyle,... read more , and sometimes an implantable cardioverter-defibrillator Implantable Cardioverter-Defibrillator (ICD) There are many causes of abnormal heart rhythms (arrhythmias). Some arrhythmias are harmless and do not need treatment. Sometimes arrhythmias stop on their own or with changes in lifestyle,... read more (ICD).