Cardiomyopathy

The most common form of heart disease is coronary artery disease. In this instance the arteries are diseased and the heart muscle is normal until an artery gets blocked and a part of the heart dies in a heart attack. Because this is the common form of heart disease the patient with it has the small comfort of knowing other people is a similar situation. With less common illnesses patients are burdened both with the illness and a certain loneliness. It is important to write about these illnesses many of which are more common than commonly perceived.

One of the less common but not uncommon forms of heart disease occurs when the heart muscle itself is weak and it functions poorly in spite of an excellent blood supply. This is called a congestive cardiomyopathy. There are many different causes for this situation but increasingly it is felt that most of the cases occur on a genetic basis and the individual was pre-programmed from birth to eventually get the illness. The most common presentation is that the patient starts to feel short of breath when walking. Others can feel irregular heart beats and faint. It is never associated with chest pain.

This illness has become a subject of intense interest for two reasons. The first is a recognition that it is more common than generally believed. The second is that we now have a series of medications that seem to control symptoms and improve the prognosis of cardiomyopathy patients.

The first issue is to confirm the diagnosis when a patient appears with the relevant symptoms. This is usually done with an echocardiogram or a cardiac MRI. The next item is to look for any possible cause that can be corrected. On the rare occasions when a specific cause is identified it will have an profound impact on therapy

After these initial steps, symptomatic patients must be treated. If they contain too much salt and water the patient has to adopt a salt restricted diet and start pills to increase the output of urine. The loss of water weight will improve edema and breathing. Once the excess fluid is shed the pills are decreased to the minimum dose to keep the weight and water level stable. A common scale is a very useful way for a patient to monitor if the water is creeping back in and the pill’s dose can be quickly increased to return the patient to his/her dry weight.

The next stage in therapy involves starting three different types of pills that can arrest and frequently reverse the illness. The first is a pill called carvedilol that is slowly increased until a certain goal is achieved. This medicine is particularly successful in improving weak hearts.

The next medicine that used to be introduced belongs to a family of medicines nicknamed ACE inhibitors and if these caused side effects a substitute pill from the ARB class of medication could be used. Presently a new combination drug called Entresto has won the hearts of doctors because of its success in helping patients. This medication includes an ARB type of medicine. This is the only medicine discussed in this article that is costly, and not infrequently a diplomatic war has to be waged to get an insurance company to approve its use.

Lastly, there is a unique class of water pill in the spironolactone family that has proved useful for cardiomyopathy patients who have heart failure. Because all the pills except the evening carvedilol pill can be swallowed together in the morning , the schedule of pills is relatively simple.

The patients with cardiomyopathy have to be seen regularly to ascertain that the symptoms are well controlled and that medication is not causing any side effects. On each visit the question needs to be raised whether any possible causes have surfaced. Sometimes an unsuspected degenerating heart valve is revealed and repair of the valve may improve symptoms

Most patients who are careful with their diets and medications, and monitor their weights carefully stay out of trouble and hospital for very long periods of time. Some even have a total regression of their cardiomyopathy. This always raises the question of whether the medication has to be continued. Because the scientific evidence suggests that stopping the medication frequently causes the illness to return, I try to persuade my patients to continue the pills.

No situation is perfect and a small number of cardiomyopathy patients deteriorate in spite of the medication. There is a long list of devices that can help, spanning sophisticated pacemakers to Left Ventricular Assist Devices and eventually cardiac transplantation. The nature of these approaches is complex and each one deserves a post of its own. I list them not to scare patients but to encourage patients. A cardiomyopathy patient who is deteriorating can choose from a long hierarchy of successful medicines and devices for help. So, the patient with progressive symptoms should seek specialized heart failure care in the certain knowledge that there is a group of specialists and devices devoted to his/her welfare.