Cardiomyopathy

Table of Contents

Cardiomyopathy is a disease of the heart muscle. This disease enlarges your heart muscle or makes it become thicker. In rare cases, scar tissue can replace the muscle tissue.

Some people manage to live a long, healthy life even though they have cardiomyopathy. Meanwhile, some people do not even realise they have it. However, in other cases, cardiomyopathy can make the heart muscle work harder to pump blood to the rest of he body. This can lead to serious complications, including: 

  • Heart failure
  • Irregular heartbeat
  • Heart valve problems
  • Sudden cardiac arrest

Heart attacks, high blood pressure, infections, and other diseases can cause cardiomyopathy. Certain types of cardiomyopathy run in families. However, in most cases, the exact cause is unknown. Treatment may involve oral medications, surgery, various medical procedures, and lifestyle changes.

Overview

The main types of cardiomyopathy are:

  • Dilated cardiomyopathy
  • Hypertrophic cardiomyopathy
  • Restrictive cardiomyopathy
  • Arrhythmogenic right ventricular dysplasia

Another type of cardiomyopathy is sometimes referred to as “unclassified cardiomyopathy.”

Cardiomyopathy can be acquired or inherited. “Acquired” means that you were not born with the condition, but you developed it from other diseases/conditions and other factors.

“Inherited” means your parents passed the disease down to you. In most cases, the cause of cardiomyopathy is unknown.

Cardiomyopathy can affect people of all ages. However, people in certain age groups are more prone to have certain types of cardiomyopathy. This article focuses on cardiomyopathy in adults.

In some cases, cardiomyopathy does not come with any signs or symptoms and does not require treatment. In other cases, the disease may progress rapidly – symptoms turn severe, and serious complications occur.

Treatment for cardiomyopathy include lifestyle changes, medications, surgery, implanted devices to treat arrhythmias, and non-surgical procedures. These treatment types can control symptoms, reduce complications, and stop the disease from getting worse.

Types of Cardiomyopathy

Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy is very common and can affect people of all ages. Hypertrophic cardiomyopathy affects men and women equally – 1 in every 500 people has the disease.

Hypertrophic cardiomyopathy occurs when the heart muscle enlarges and thickens for no apparent reason. The ventricles, lower chambers of the heart, and the septum (the wall that separates the left and right sides of the heart) become thicker. The thickened area creates narrowing or blockage in the ventricles, making it harder for the heart to pump blood. Hypertrophic cardiomyopathy can also make the ventricles become more rigid, affect the mitral valve, and affect the cells in heart tissue.

Dilated Cardiomyopathy

Dilated cardiomyopathy develops when the ventricles enlarge and weaken. This condition usually starts in the left ventricle and can affect the right ventricle over time. The weakened chambers of the heart do not pump effectively, causing the heart muscle to work harder. The heart gradually loses its ability to pump blood optimally. Dilated cardiomyopathy can lead to heart failure, heart valve disease, irregular heartbeats, and blood clots in the heart.

Restrictive Cardiomyopathy

Restrictive cardiomyopathy occurs when the ventricles become more rigid, but the walls of the heart remain normal. As a result, the ventricles are neither relaxed nor filled with normal blood volume. As the disease progresses, the ventricles do not pump properly and the heart muscle weakens.

Arrhythmogenic Right Ventricular Dysplasia

Arrhythmogenic right ventricular dysplasia is a rare type of cardiomyopathy that occurs when the muscle tissue in the right ventricle is replaced with fatty or fibrous tissue. This can interfere with the heart’s sequence of electrical impulses and lead to arrhythmias. Arrhythmogenic right ventricular dysplasia usually affects adolescents or young adults and can cause sudden cardiac arrest in young athletes.

Unclassified Cardiomyopathy

Other types of cardiomyopathy fall into this category, as follows: 

  • Left ventricular noncompaction occurs when the left ventricle experiences trabeculations – the projection of muscle within the ventricle.
  • Takotsubo cardiomyopathy (broken heart syndrome) occurs when extreme stress causes heart failure. Although rare, this condition commonly occurs more in postmenopausal women.

Causes of Cardiomyopathy

When a condition of cardiomyopathy is “Inherited”, this means you got the disease from your parents. Researchers are still looking for genetic reasoning for cardiomyopathy and how the disease can cause or contribute to various types of diseases.

In many cases, the cause of cardiomyopathy is unknown. This often occurs when the disease affects young children. 

Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy is often inherited. This is due to gene mutations or changes in the cardiac muscle protein. Hypertrophic cardiomyopathy can also develop over time due to high blood pressure, aging, or other diseases – such as diabetes or thyroid disease. Sometimes the cause of the disease is unknown.

Dilated Cardiomyopathy

The cause of dilated cardiomyopathy is often unknown. About a third of people with dilated cardiomyopathy get the disease from their parents.

Certain diseases, conditions, and substances can also cause dilated cardiomyopathy, such as:

  • Alcohol — especially if you also have a poor diet
  • Certain toxins — such as poisons and heavy metals
  • Complications in the third trimester of pregnancy
  • Coronary heart disease, heart attack, high blood pressure, diabetes, thyroid disease, viral hepatitis, and HIV
  • Illegal drugs, such as cocaine and amphetamines, and cancer medications
  • Infections, especially viral infections that cause inflammation of the heart muscle

Restrictive Cardiomyopathy

Certain diseases, conditions, and factors can cause restrictive cardiomyopathy, including:

  • Amyloidosis: A disease, in which abnormal amounts of proteins build up in the body organs including the heart
  • Connective tissue disorders
  • Hemochromatosis: A disease, in which excess amounts of iron build up in the body. The extra iron can be toxic and damage organs, including the heart.
  • Sarcoidosis: A disease that causes inflammation and can affect various body organs. Researchers believe that an abnormal immune response can lead to sarcoidosis. This abnormal response can cause small lumps to form in the body organs, including the heart.
  • Cancer treatments, such as radiation and chemotherapy

Arrhythmogenic Right Ventricular Dysplasia

Researchers believe that arrhythmogenic right ventricular dysplasia is an inherited disease.

Risk Factors of Cardiomyopathy

People of all ages and races can be affected by cardiomyopathy. However, certain types of diseases more commonly occur in certain groups.

Dilated cardiomyopathy is more common to affect African-American people than white people. This type of disease also develops more in men than women.

Adolescents and young adults are more likely to have arrhythmogenic right ventricular dysplasia than older people, although it is a rare case in both groups.

Main Risk Factors

Certain diseases, conditions, or factors can increase your risk for cardiomyopathy. The main risk factors include:

  • Family history of cardiomyopathy, heart failure, or sudden cardiac arrest (SCA)
  • Diseases or conditions that can cause cardiomyopathy — such as coronary heart disease, heart attack, or viral infections that cause inflammation of the heart muscle
  • Diabetes or other metabolic disease, or severe obesity
  • Diseases that can damage the heart, such as hemochromatosis, sarcoidosis, or amyloidosis
  • Long-term alcohol addiction
  • Long-term high blood pressure

In some cases, cardiomyopathy does not show any signs or symptoms. Thus, it is important to identify people who may be at high risk for this disease. This can help prevent future problems, such as serious arrhythmias (irregular heartbeats) or serious cardiac arrest.

Prevention

You cannot prevent genetic cardiomyopathy. However, you can take several precautions to lower the risk of the diseases or conditions that can cause or complicate cardiomyopathy. For example, coronary heart disease, high blood pressure, and heart attacks.

Your doctor may recommend you to change your lifestyle, such as:

  • Implement a healthy diet and be more physically active
  • Quit smoking
  • Avoid alcohol and illegal drugs
  • Get enough sleep and rest
  • Reduce stress

Cardiomyopathy may be caused by an underlying disease or condition. If you treat the condition early on, you may be able to prevent complications of cardiomyopathy. For example, controlling high blood pressure, high blood cholesterol, and diabetes:

  • Get regular check-ups with your doctor.
  • Follow your doctor’s advice about changes in your lifestyle
  • Take all medications as prescribed by your doctor.

Doctors may be able to prevent sudden cardiac arrest (SCA) if they can identify people who are at high risk for this condition and treat them with an implanted cardioverter defibrillator (SCA is a complication of cardiomyopathy).

Signs and Symptoms

Some people who have cardiomyopathy never show signs or symptoms. Others have no signs or symptoms in the early stages of the disease.

When cardiomyopathy worsens and the heart weakens, signs and symptoms of heart failure usually occur. These signs and symptoms include:

  • Shortness of breath or difficulty breathing, especially with physical activity
  • Fatigue 
  • Swelling of the ankles, feet, legs, abdomen, and neck veins

Other signs and symptoms may include dizziness; fainting during physical activities; arrhythmia (irregular heartbeat); chest pain, especially after a heavy physical activity or a heavy meal; and heart murmurs (a heart murmur is an unusual sound heard during a heartbeat).

Diagnosis of Cardiomyopathy

Your doctor will diagnose cardiomyopathy based on your personal and your family’s medical history, a physical test, and the results of tests and procedures.

Specialist Doctors Involved

In many cases, cardiologists or pediatricians are the ones that diagnose and treat cardiomyopathy. Cardiologists specialize in diagnosing and treating heart disease. Meanwhile, pediatric cardiologists are cardiologists who treat children patients. 

Personal and Family’s Medical History

Your doctor will study your medical history. He or she needs to identify what signs and symptoms you are experiencing and for how long you have been experiencing them.

In addition, your doctor will also want to know if anyone in your family has had cardiomyopathy, heart failure, or sudden cardiac arrest.

Physical Tests 

The doctor will use a stethoscope to listen to your heart and lungs for sounds that may indicate cardiomyopathy. These sounds may even suggest a certain type of disease. For example, the loudness, timing, and location of a heart murmur may indicate obstructive hypertrophic cardiomyopathy. A “crackle” sound in the lungs may be a sign of heart failure (heart failure often occurs at a later stage of cardiomyopathy).

Physical signs also help doctors diagnose cardiomyopathy. Swelling of the ankles, feet, legs, abdomen, or blood vessels in your neck can indicate fluid buildup – which is a sign of heart failure.

Your doctor may also spot signs and symptoms of cardiomyopathy during routine check-ups. For example, there may be a heart murmur, or you could have abnormal test results.

Diagnostic Tests

Your doctor may recommend one or more of the following tests to diagnose cardiomyopathy.

Blood Test

During a blood test, a small amount of blood is taken from your body. Usually, the doctor will take the blood from your vein in the arm using a needle. The procedure is quick and simple – although it can cause short-term discomfort.

Blood tests can indicate your heart condition and help your doctor rule out other conditions.

Chest X-ray

Chest x-rays take pictures of organs and structures inside the chest, such as the heart, lungs, and blood vessels. This test can show if your heart is enlarged. A chest X-ray can also show if there is a fluid buildup in your lungs.

Electrocardiogram

An ECG is a simple test that records the electrical activity of your heart. The test shows how fast your heart is beating and its rhythm (regular or irregular). The ECG also records the strength and timing of electrical signals as they pass through each part of the heart.

This test is used to detect and study numerous heart problems, such as heart attacks, arrhythmias, irregular heartbeats, and heart failure. ECG results can also show other disorders that affect your heart function.

A standard ECG only records your heart rate for a few seconds and will not detect problems that do not occur during the test.

To diagnose heart problems that come and go, your doctor may have you wear a portable ECG monitor. The two most common types of portable ECGs are Holter and event monitors.

Holter and Event Monitor

Holter and event monitors are small and portable devices. This monitor records the electrical activity of the heart while you are doing normal daily activities. The Holter monitor records the electrical activity of the heart for a full 24 or 48 hours.

An event monitor records the heart’s electrical activity only at certain times when you are wearing it. The way event monitor devices work is that you press a button to start the monitor when you experience symptoms. Other event monitors start automatically when they feel an abnormal heart rhythm.

Echocardiography

Echocardiography (echo) is a test that uses sound waves to create a moving image of your heart. The image shows the condition, size, and shape of your heart.

There are several types of echo, including stress echo. This test is done as part of a stress test. A stress echo can show whether you have decreased blood flow to your heart – which is a sign of coronary heart disease.

Another type of echo test is transesophageal echo, or TEE. TEE provides an image of the back of the heart.

In this test, a sound wave stick is placed at the end of a special tube. The tube is gently inserted down your throat and passed down your esophagus (the tube that carries food from your mouth to your stomach). Since it is located behind the heart, TEE can create detailed pictures of the structure of your heart.

Prior to the TEE, you will be given medicine to help you relax, and your throat will be numbed.

Stress Test

Certain heart problems are easier to diagnose when the heart is working harder and beating faster. During a stress test, you will be asked to exercise (or given medication if you are unable to exercise) to make your heart work harder and beat faster during the test.

These tests may include a nuclear heart scan, echo, and positron emission tomography (PET) heart scan.

Diagnostic Procedures

You may have to undergo one or more medical procedures to confirm a diagnosis or to prepare for a surgery (if surgery is planned). These procedures may include cardiac catheterization, coronary angiography, or myocardial biopsy.

Cardiac Catheterization

This procedure checks the pressure and blood flow in the chambers of your heart. This procedure also allows the doctor to collect a blood sample and view the heart arteries using x-ray imaging.

In cardiac catheterization, a long, thin, flexible tube called a catheter is inserted into a blood vessel in your arm, groin (upper thigh), or neck and threaded into your heart. This allows the doctor to study the inside of the artery for any blockages.

Coronary Angiography

This procedure is usually performed with cardiac catheterization. During the procedure, a dye that can be seen on an x-ray is injected into your coronary arteries. The dye allows your doctor to study the blood flow through your heart and blood vessels.

Myocardial Biopsy

In this procedure, your doctor removes a strand of your heart muscle. This can be done during cardiac catheterization. The heart muscle is then examined under a microscope to identify any changes in cells. These changes can indicate cardiomyopathy.

A dye can also be injected into the chambers of your heart. This allows your doctor to examine your heart functions. 

Myocardial biopsy is useful for diagnosing several types of cardiomyopathy.

Genetic Testing

Certain types of cardiomyopathy may run in families. Thus, your doctor may recommend genetic testing to identify diseases found in your parents, brothers and sisters, or other family members.

Genetic testing can show how the disease runs in families. This test can also find out how likely parents will pass the disease to their children.

Genetic testing may also be useful if your doctor suspects you have cardiomyopathy, yet you show no signs or symptoms. If the test result confirms that you have the disease, your doctor may start treatment right away. 

Treatment for Cardiomyopathy

People with cardiomyopathy but show no signs or symptoms may not require treatment. In some cases, dilated cardiomyopathy that occurs suddenly may go away on its own. For other people who have cardiomyopathy, treatment is indeed required.

Treatment depends on the type of cardiomyopathy you have, the severity of your symptoms and complications, and your age and overall health. Treatments may include:

  • Heart-healthy lifestyle changes
  • Medications
  • Non-surgical procedures
  • Surgery and implant devices

The main objectives of cardiomyopathy’s treatments include:

  • Control the signs and symptoms experienced so you can live as normally as possible 
  • Manage any condition that causes or contributes to disease
  • Reduce complications and the risk of sudden cardiac arrest
  • Stop the disease from getting worse

Lifestyle Changes For Better Heart Health

Your doctor may suggest lifestyle changes to manage conditions that are causing your cardiomyopathy including:

  • Implement a heart-healthy diet
  • Achieve a healthy weight
  • Manage stress
  • Engage more in physical activities
  • Quit smoking

Medications

Numerous medicines are used to treat cardiomyopathy. Your doctor may prescribe medications to help you:

  • Balance electrolytes in your body. Electrolytes are minerals that help maintain fluid levels and acid-base balance in your body. In addition, it also helps muscles and nerve tissue to function properly. Abnormal electrolyte levels may be a sign of dehydration (lack of fluids in your body), heart failure, high blood pressure, or other disorders. Aldosterone blockers are an example of a medication used to balance electrolytes.
  • Maintain a normal heart rate. Medicines called antiarrhythmics, help prevent arrhythmias.
  • Lower your blood pressure. ACE inhibitors, angiotensin II receptor blockers, beta blockers, and calcium channel blockers are examples of medications that lower blood pressure.
  • Prevent the formation of blood clots. Anticoagulants, or blood thinners, are examples of drugs that prevent blood clots. Blood thinners are often used to prevent blood clots in people with dilated cardiomyopathy.
  • Reduce inflammation. Corticosteroids are an example of a medication used to reduce inflammation.
  • Remove excess sodium from your body. Diuretics, or water pills, are examples of medicines that help remove excess sodium from the body, which reduces the amount of fluid in your blood.
  • Slow down your heart rate. Beta blockers, calcium channel blockers, and digoxin are examples of medicines that slow down the heart rate. Beta blockers and calcium channel blockers are also used to lower blood pressure.

It is recommended that you consume all the prescribed medicines regularly. You should  never increase the dose or skip the medicine unless your doctor tells you to.

Surgery and Medical Implant Devices

There are several types of surgery procedures to treat cardiomyopathy, including septal myectomy, surgical implant devices, and heart transplants.

Septal Myectomy

Septal myectomy is an open-heart surgery used to treat people who have hypertrophic cardiomyopathy with severe symptoms. This surgery is generally used for younger patients and for people whose symptoms are not improved by medications. 

Your surgeon will remove a portion of the thickened septum that bulges into the left ventricle. This increases blood flow throughout your body. The removed tissue will not grow back. If necessary, the surgeon will also repair or replace the mitral valve at the same time. Septal myectomy is often successful and allows you to return to normal life without symptoms.

Implantable Devices

Surgeons can place several types of medical implant devices on the heart to improve function and symptoms, including:

  • Cardiac resynchronization therapy (CRT) device. The CRT device coordinates contractions between the left and right ventricles of the heart.
  • Implantable cardioverter defibrillator (ICD). The ICD helps control life-threatening arrhythmias that can cause sudden cardiac arrest. This small device is implanted in the chest or stomach and is connected to the heart with a cable. If the ICD senses dangerous changes in heart rhythm, it will send an electric shock to the heart to restore a normal heart rate.
  • Left ventricular assist device (LVAD). This device helps the heart pump blood to the body. The LVAD can be used as long-term therapy or as a short-term treatment for people waiting for a heart transplant.
  • Pacemaker. This small device is placed under the skin of the chest or abdomen to help control arrhythmias. This device uses electric pulses to promote a normal heartbeat rate

Heart transplant

In this procedure, the surgeon replaces a diseased heart with a healthy heart from a recently deceased donor. Heart transplantation is the last treatment option for patients with end-stage heart failure. “End stage” means the condition has become too severe that all treatments, apart from heart transplants, have failed.

Non-surgical procedures

A non-surgical procedure called alcohol septal ablation can be an option to treat cardiomyopathy. In this procedure, the doctor injects ethanol (a type of alcohol) through a tube into a small artery that supplies blood to the thickened area of ​​the heart muscle. The alcohol kills cells, and the thickened tissue shrinks to a normal size. This procedure allows blood to flow freely through the ventricles, which improves symptoms.

Home Care for Patients Diagnosed with Cardiomyopathy

Some people who have cardiomyopathy – especially those with hypertrophic cardiomyopathy (HCM) – may live a healthy life with few problems or symptoms. Other people may experience serious symptoms and complications.

If you have cardiomyopathy, there are some precautions that you can take to care for your heart. Lifestyle changes and continuous care can help you manage the disease.

Lifestyle Changes

A healthy diet and physical activity are part of a heart healthy lifestyle. Your doctor can help you decide the right diet plan for you. Talk to your doctor about the amounts and types of fluids that are safe and healthy for you. Too much fluid can worsen certain heart conditions.

Your doctor may suggest low-sodium and low-fat diets

Talk to your doctor about the right types of physical activities for you. People who have HCM should not engage in vigorous activity. However, light activity, for example, walking, is recommended.

Your doctor may also suggest other lifestyle changes such as:

  • Quit smoking
  • Lose weight
  • Avoid alcohol and illegal drugs
  • Get enough sleep and rest
  • Reduce stress

Continuous Care

If you have cardiomyopathy, it is important to get continuous care. Consult your doctor if you notice new or worsening symptoms, such as swelling in the ankles, feet, legs, stomach, or blood vessels in the neck. These symptoms can be signs of worsening disease.

You must also:

  • Consume the medicines as prescribed by your doctor.
  • Implement the lifestyle changes as recommended by your doctor 
  • Make sure you follow all your medical examinations.
  • Follow the recommended treatment plan for all underlying conditions, such as diabetes and high blood pressure.

Cardiomyopathy often runs in families. The doctor may recommend that your parents, brothers and sisters, and children be examined to see if they have the disease.

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