What is Cardiomyopathy?
An all-inclusive medical guide for a unique and potentially fatal heart condition.
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Cardiomyopathy refers to diseases of the heart muscles (myocardium) that are responsible for the heart’s ability to pump blood that are associated with functional and structural abnormalities, like heart growth or the interruption in the ability for the heart to pump blood.
Many people do not have any symptoms when they get cardiomyopathy, which has resulted in it being perceived as uncommon. Yet, it is more common than it is generally given credit for. It should be taken seriously, as many cases of cardiomyopathy progress into chronic heart failure.
The prognosis of most cardiomyopathies is not great, and its causes are very often out of the hands of the patients (e.g., genetics). Treatment, therefore, is tiered towards reducing the severity of symptoms and increasing the lifespan of the patient.
For the cases that are preventable, a heart-healthy lifestyle of exercise, nutritious eating, and stress reduction is a sure-fire way to do your best in preventing the development of cardiomyopathy.
Introduction
According to the CDC, heart disease is the leading cause of death in the United States, causing about 647,000 Americans to die each year. About 30.3 million Americans are diagnosed with heart disease each year. Generally speaking, the leading cause of heart disease is lifestyle factors (e.g. poor nutrition, being sedentary, and smoking).
One of the less common variations of heart disease is called cardiomyopathy. Despite the fact that cardiomyopathy is not as common as other varieties of cardiovascular disease, while it is generally said that in adults its prevalence is 1 in 2500, due to it frequently going undiagnosed and due to it contributing to a substantial amount of sudden deaths, it could be that its prevalence in adults is around 1 in 500. Indeed, even 50% of patients in childhood or adolescence that die suddenly are affected by cardiomyopathies.
Thus, despite its official designation as being common, but not too common, it is essential to give it the time of day, as many people might be suffering from it without even knowing it.
What is Cardiomyopathy
One thing to keep in mind is that the word “Cardiomyopathy” by itself can be taken as a plural word. Which is to say, there are many different cardiomyopathies. But, the overarching definition of this plural term is a disease of the myocardium (which, in layman’s terms, are the heart muscles which form the heart and are responsible for maintaining the heart’s ability to pump blood throughout the body) which are correlated with structural and/ functional irregularities in the absence of a clearer reason (such as a different cardiovascular disease) for such irregularities.
The Different Cardiomyopathies
There are a couple of different types of cardiomyopathies, and each of them has its own peculiar differences, both in terms of physiology and epidemiology. We’ll briefly go through the types so that you can get a sense of these differences.
Dilated Cardiomyopathy:
Dilated cardiomyopathy is the most common form of cardiomyopathy, composing about 60% of known cardiomyopathy cases. Fundamentally, this presents itself as dilated and/or poorly functioning left or both ventricles — the right ventricle, serving to pump blood to the lungs and the left, serving to pump blood to the rest of the body. In other words, the heart has become enlarged, and as a result, it can no longer pump blood efficiently. The cause of dilated cardiomyopathy is quite mixed (e.g., genetic, alcohol abuse, autoimmune, inflammatory, and/or excess of thyroid hormone), but it does seem that genetic predispositions play a crucial role in its development. The level of risk for developing this disease is highest in African Americans and in men. The prognosis for dilated cardiomyopathy is not great. Most who are afflicted with it end up with chronic heart failure and almost 50% of patients die within 5 years.
Hypertrophic Cardiomyopathy
Hypertrophic cardiomyopathy is characterized by myocardial (heart muscle) hypertrophy (growth) and a non-dilated left ventricle. Generally speaking, this is a condition that derives from a genetic mutation of genes that encode for cardiac contractile proteins. The result of this is an interference with the generation of force and the relaxation of the muscles of your heart, which makes your heart much less efficient in this process of pumping blood. According to the journal Nature, the prognosis of hypertrophic cardiomyopathy is not quite clear just yet. Some people can live with it asymptomatic for years, but others get severe cases that rapidly lead to heart failure. Of the latter cases, however, the survival rates are anywhere from 1 to 10 years depending upon the severity of the situation.
Restrictive Cardiomyopathy
Restrictive cardiomyopathy is a little bit different than dilated and hypertrophic cardiomyopathy, in the sense that it doesn’t entail the growth of the heart. Rather, restrictive cardiomyopathy is characterized by the ventricles becoming stiff and rigid, which causes them not to relax and in turn, they do not fill with the normal blood volume. Eventually, this leads to the heart being incapable of pumping blood as efficiently and a weakening of the heart muscles. This is caused by a few different factors. Most of these factors are actually other diseases: hemochromatosis (when too much iron builds up in your body), sarcoidosis (an immune disease, which might be caused by an abnormal immune response), amyloidosis (a disease whereby abnormal proteins build up in the body’s organs), and some cancer treatments like chemotherapy or radiation therapy. The prognosis of restrictive cardiomyopathy is rather poor. Having the worst prognosis of all cardiomyopathies, there is generally only a two to five-year survival rate.
Arrhythmogenic Right Ventricular Dysplasia
A quite rare (1 in 1000 or 1 in 1,250 people) version of cardiomyopathy, arrhythmogenic right ventricular dysplasia is when the muscle tissue in the right ventricle is broken down and/or damaged and is replaced by scar tissue/fatty or fibrous tissue. When this occurs, the electrical signals of the heart can be interfered with and that can cause arrhythmias (which is when your heart rate is beating too fast, too slow, or irregularly in some other manner.) Over time, this can cause the myocardium to weaken and break down (atrophy). Its causes are primarily genetic, whereby certain genes that provide instructions for cell structures called desmosomes become mutated. Desmosomes provide the muscles of the heart strength, but when the genes attached to these cells are mutated, their function in providing this strength diminishes. It must be stated that with this condition there is a danger in participating in rigorous exercise due to the increased risk of arrhythmias, which does require emergency care. Teens and young adults are most at risk for developing arrhythmogenic right ventricular dysplasia. The prognosis of this disease is quite difficult to make out. On the one hand, some people can live with this without a problem for decades, while on the other, some might suddenly die from it. But, generally speaking, it is said to have good long-term results, where patients rarely develop life-threatening issues.
Symptoms and Diagnosis
Generally speaking, the way almost all forms of cardiomyopathy are detected and diagnosed is when symptoms arise (symptoms being either none, or fatigue, chest pain, leg swelling, shortness of breath, and/or even fainting). When these symptoms arise, a doctor will conduct a physical exam and will check for heart murmurs (which is the sound of blood flowing) and if abnormalities are noticed in this exam, the doctor might take a chest x-ray, blood work, EKG, or echocardiography. These tests are reliable ways to see the growth in your heart.
Prevention
Not all forms of cardiomyopathy are preventable. In particular, those that are genetically/familially inherited are not preventable. However, with the non-genetic/non-familial forms, there are active steps that you can take in order to substantially lower your risk for developing cardiomyopathy. Likewise, these are steps that might reduce the severity of cardiomyopathy in patients that have already been diagnosed with it. Here are a few of these strategies:
Quit drinking and/or smoking: smoking increases the likelihood that you will develop heart disease, or escalate the severity of an already existing heart disease. The same goes for drinking alcohol. Long-term alcohol intake is especially correlated with developing cardiomyopathy.
Eat a nutritious diet: The standard American diet, which consists primarily of highly refined and processed foods, rather than naturally derived and nutritious foods, contributes to a sizable amount of heart disease. Focusing your food intake on foods that are high in vitamins and minerals like fruits and vegetables, and foods with healthy fats in them like fatty fish, eggs, nuts, and olive oil, will contribute in a positive manner in the prevention of developing cardiomyopathy. One other important factor to keep in mind is to limit your exposure to trans fats, as trans fats have been directly implicated in the development of heart disease.
Exercise: Regular exercises have been proven time and time again to reduce the risk of developing heart disease, including cardiomyopathy. There is an important caveat to keep in mind when it comes to exercise; however: don’t do too much of it and take rest days. Doing to much exercise and also abdicating rest can actually increase your risk of developing heart disease.
Manage Stress: It is sometimes very difficult to manage stress. Most people work all day, and when they get home, they might not have time for themselves. However, if you are worried about the risk of developing cardiomyopathy, doing whatever you can to lower stress is crucial. This is because chronically elevated cortisol levels can be directly implicated in the development of cardiomyopathy. To lower your stress you need to ensure that you are getting 7–9 hours of sleep per night, ensuring that such sleep is high-quality sleep (to do this, try to shut off all electronics an hour before going to bed and following a regular sleep schedule), try meditating (there are plenty of apps out there to help you learn), drink tea, take a walk, and/or spend time with loved ones.
Genetic Testing: Given that a fairly high-rate of cardiomyopathy is caused by genetic factors, it might be a good idea for you to get a genetic test. Getting your genes tested can help doctors determine what the right course of action is for you in terms of treatment.
Risk Factors
Some of the risk factors for developing cardiomyopathy have already been mentioned in our descriptions of the different types of this disease. To reiterate, these risk factors include genetics, alcohol abuse, poor diet, a sedentary lifestyle, autoimmune disorders, excess thyroid hormone, stress, and various diseases (hemochromatosis, sarcoidosis, or amyloidosis).
However, other major risk factors include having a family history with heart disease, diabetes, obesity, and long-term high blood pressure.
Treatment
The treatments for cardiomyopathy are quite wide-ranging. Some of these treatments are simply lifestyle changes, and those can be seen in the “prevention” section of this article.
In more severe cases, however, medications might be required for treatment. These could include aldosterone blockers to rebalance your electrolytes, antiarrhythmics to prevent arrhythmias, ACE inhibitors, angiotensin II receptor blockers, beta-blockers, and calcium channel blockers to help lower blood pressure, anticoagulants, or blood thinners to prevent blood clots, anti-inflammatory drugs to lower inflammation, and diuretics or water pills to remove excess sodium from the body. This course of action, of course, depends on which of the issues might be arising.
If the medicine is not working well and lifestyle factors have been ruled out, a doctor might order a surgical procedure. These procedures vary quite widely, depending on the situation. For instance, a septal myectomy (a form of heart surgery) might be used for individuals who are dealing with hypertrophic cardiomyopathy and are not responding well to medicine. Or, a wide variety of devices could be implanted into your heart, such as a pacemaker to help control arrhythmias, a left-ventricular assist device to help the heart pump blood in an efficient manner, a cardiac resynchronization device, or an implantable cardioverter-defibrillator to help prevent falling into cardiac arrest due to severe arrhythmias. The very last resort — which would be determined by heart failure that no other treatments could remedy — would be open-heart surgery.
Given that the prognosis of most forms of cardiomyopathy is poor, the goal of treatment is to reduce the severity of symptoms and to keep the patients alive longer.
This article does not provide medical advice. The information, including but not limited to, text, graphics, images and other material contained in this article are for informational purposes only. No material on this article is intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health care provider with any questions you may have regarding a medical condition or treatment and before undertaking a new health care regimen, and never disregard professional medical advice or delay in seeking it because of something you have read in this article.
