Cardiomyopathy is a rare heart disease that affects the myocardium, causing the heart to function abnormally. There are four types: ischemic, hypertrophic, dilated, and restricted. Dilated cardiomyopathy is the most common and often requires transplantation. Hypertrophic cardiomyopathy causes additional heart muscle tissue to grow, while restricted cardiomyopathy causes the heart muscles to widen, making it difficult for the heart to fill properly. Arrhythmogenic right ventricular dysplasia is a genetic disease that can lead to sudden death in athletes. Gene therapy and transplant technology are being researched to improve treatment options.
There are several types of cardiomyopathy, all of which affect the myocardium or muscle tissue of the heart. The damage prevents the heart from functioning normally, or the tissue walls thicken or harden, causing the heart to resist filling to capacity. Cardiomyopathy progresses in most cases and is one of the major diseases requiring heart transplantation.
The different forms are all relatively rare with about one-tenth of a percentage point in the general population. One form of cardiomyopathy is called ischemic because it is caused by repeated heart attacks or strokes. Non-ischaemic cardiomyopathy includes three main types where the cause may not always be clear. These are hypertrophic cardiomyopathy, dilated cardiomyopathy, and restricted cardiomyopathy. Arrhythmogenic right ventricular dysplasia (ARVD) is a fourth rare type of genetic origin.
Dilated cardiomyopathy is the most common form, causing enlargement of both of the heart’s ventricles, or pumping chambers. The enlargement weakens the heart and can lead to eventual heart failure. Early stage dilated cardiomyopathy can be treated with drugs such as digitalis or drugs that lower blood pressure. Ultimately, however, this form of heart muscle disease normally requires transplantation. The main causes include extreme alcoholism, viral diseases or genetic predisposition.
When the family history includes others with dilated cardiomyopathy, genetics may be considered as the primary cause. The viruses indicated in dilated cardiomyopathy are rheumatic fever and Kawasaki syndrome. Often the causative viruses are mild and can go undetected.
Hypertrophic cardiomyopathy causes additional heart muscle tissue to grow on the septum, the dividing wall between the two ventricles. This reduces the size of the left ventricle, which is responsible for pumping blood around the body. Symptoms are usually mild at first but can progress over time.
Drugs can increase heart function, and an implanted pacemaker or defibrillator often monitors heart rhythms, as this condition carries a risk of sudden death from arrhythmia. When the risk is minimized, many with hypertrophic cardiomyopathy have an excellent life expectancy. However, if the disease progresses, cardiologists may perform ablation, which uses alcohol to burn off some of the extra heart muscle.
Restricted cardiomyopathy is relatively rare. The heart muscles widen causing the ventricles to become stiff and unable to allow the heart to fill properly. This type tends to progress, with many needing a transplant. Initially, the primary goals are to eliminate dangerous arrhythmias and to increase cardiac function.
Arrhythmogenic right ventricular dysplasia is a genetic disease. The right ventricle, which pumps blood to the lungs, loses muscle tissue, which is then replaced by fat tissue. This type of cardiomyopathy, although very rare, is a leading cause of sudden death in athletes. Usually, an affected person is asymptomatic until presenting with heart failure. Drugs and a pacemaker implant can be used together to reduce heart failure.
The risk of transmitting ARVD to children is high, approximately 50%. It is advisable if you have a parent with ARVD to see a cardiologist to evaluate if this condition is present in yourself. Early diagnosis can help slow the disease and is also important when considering having your own children. Both parents can pass the disease to a child, and pregnancy can be complicated if you have ARVD.
While there is currently no cure for cardiomyopathy, researchers hope that gene therapy may one day play a role in reducing abnormal heart tissue. Additionally, transplant technology continues to improve, increasing life expectancy. However, a lack of donor hearts often makes transplantation impossible, and many die while waiting for a heart. An increase in the donor registry is encouraged to help make transplantation possible for everyone in need of a heart.
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