Diagnosis

Your doctor will conduct a physical examination, take a personal and family medical history, and ask when your symptoms occur — for example, whether exercise brings on your symptoms. If your doctor thinks you have cardiomyopathy, you might need to undergo several tests to confirm the diagnosis, including:

  • Chest X-ray. An image of your heart will show whether it's enlarged.
  • Echocardiogram. This uses sound waves to produce images of the heart, which show its size and its motions as it beats. This test checks your heart valves and helps your doctor determine the cause of your symptoms.
  • Electrocardiogram (ECG). In this noninvasive test, electrode patches are attached to your skin to measure electrical impulses from your heart. An ECG can show disturbances in the electrical activity of your heart, which can detect abnormal heart rhythms and areas of injury.
  • Treadmill stress test. Your heart rhythm, blood pressure and breathing are monitored while you walk on a treadmill. Your doctor might recommend this test to evaluate symptoms, determine your exercise capacity and determine if exercise provokes abnormal heart rhythms.
  • Cardiac catheterization. A thin tube (catheter) is inserted into your groin and threaded through your blood vessels to your heart. Doctors might extract a small sample (biopsy) of your heart for analysis in the laboratory. Pressure within the chambers of your heart can be measured to see how forcefully blood pumps through your heart.

    Doctors might inject a dye into your blood vessels so that they show on X-rays (coronary angiogram). This test can be used to ensure there are no blockages in your blood vessels.

  • Cardiac MRI. This test uses magnetic fields and radio waves to create images of your heart. Cardiac MRI might be used in addition to echocardiography, particularly if the images from your echocardiogram aren't helpful in making a diagnosis.
  • Cardiac CT scan. You lie on a table inside a doughnut-shaped machine. An X-ray tube inside the machine rotates around your body and collects images of your heart and chest to assess the heart size and function and heart valves.
  • Blood tests. Several blood tests might be done, including those to check your kidney, thyroid and liver function, and to measure your iron levels.

    One blood test can measure B-type natriuretic peptide (BNP), a protein produced in your heart. Your blood level of BNP might rise when your heart is in heart failure, a common complication of cardiomyopathy.

  • Genetic testing or screening. Cardiomyopathy can be hereditary. Discuss genetic testing with your doctor. He or she might recommend family screening or genetic testing for your first-degree relatives — parents, siblings and children.

Treatment

The goals of cardiomyopathy treatment are to manage your signs and symptoms, prevent your condition from worsening, and reduce your risk of complications. Treatment varies by which type of cardiomyopathy you have.

Medications

Your doctor might prescribe medications to improve your heart's pumping ability, improve blood flow, lower blood pressure, slow your heart rate, remove excess fluid from your body or keep blood clots from forming.

Be sure to discuss possible side effects with your doctor before taking any of these drugs.

Surgically implanted devices

Several types of devices can be placed in the heart to improve its function and relieve symptoms, including:

  • Implantable cardioverter-defibrillator (ICD). This device monitors your heart rhythm and delivers electric shocks when needed to control abnormal heart rhythms. An ICD doesn't treat cardiomyopathy, but watches for and controls abnormal rhythms, a serious complication of the condition.
  • Ventricular assist device (VAD). This helps blood circulate through your heart. VAD usually is considered after less-invasive approaches are unsuccessful. It can be used as a long-term treatment or as a short-term treatment while waiting for a heart transplant.
  • Pacemaker. This small device placed under the skin in the chest or abdomen uses electrical impulses to control arrhythmias.

Nonsurgical procedures

Other procedures used to treat cardiomyopathy or arrhythmia include:

  • Septal ablation. A small portion of the thickened heart muscle is destroyed by injecting alcohol through a long, thin tube (catheter) into the artery supplying blood to that area. This allows blood to flow through the area.
  • Radiofrequency ablation. To treat abnormal heart rhythms, doctors guide long, flexible tubes (catheters) through your blood vessels to your heart. Electrodes at the catheter tips transmit energy to damage a small spot of abnormal heart tissue that is causing the abnormal heart rhythm.

Surgery

Types of surgery used to treat cardiomyopathy include:

  • Septal myectomy. In this open-heart surgery, your surgeon removes part of the thickened heart muscle wall (septum) that separates the two bottom heart chambers (ventricles). Removing part of the heart muscle improves blood flow through the heart and reduces mitral valve regurgitation.

Mayo Clinic Myectomy Expertise

Mayo Clinic heart surgeons have performed more than 3,000 myectomy procedures, with more than 250 performed each year on average. In the hands of expert centers, complication rates are very low and success rates very high. Recent data suggests that at low, medium and even "high" volume centers there is a gradient of mortality, meaning it's highest at the low volume centers and lowest at the high volume centers. But even those so-called high volume centers have mortality rates that are dramatically higher than is reported at the true expert centers. This is a procedure that should be done by those familiar with performing it at a high volume.

Hypertrophic cardiomyopathy and treatment options

Steve R. Ommen: The hypertrophic cardiomyopathy is a condition that has been under-diagnosed and overly feared throughout the world. In the United States alone, there are over a half a million people that have hypertrophic cardiomyopathy, many of whom are completely asymptomatic and unaware of their diagnosis. Some people can die suddenly. Sudden cardiac death occurs randomly without warning.

Hartzell V. Schaff: Over 2/3 of the patients will have obstruction. And the obstruction to the left ventricular outflow tract is an indication for operation in patients that have symptoms. So we know now that 2/3 of the patients with hypertrophic cardiomyopathy and obstruction are candidates for surgery.

Steve R. Ommen: Hypertrophic cardiomyopathy is the most common inherited cardiomyopathy or heart muscle disease. People are born with the genetics for it, but the hypertrophy doesn't appear to start developing until adolescence, growth spurts, or beyond. It is possible for infants to born with thick heart muscles, but that's really quite rare and usually more severe expressions of the disease. And it's also been described as not coming on until people were in their fifth or sixth decade of life. So really, the onset can be at any time of life. And certainly the symptoms can occur throughout life.

Hartzell V. Schaff: The common symptoms that patients have when they have obstructive hypertrophic cardiomyopathy are shortness of breath, angina-like chest pain and syncope. And unfortunately, some of these symptoms develop so slowly and over such a long time that the patients don't really understand how limited they are.

Steve R. Ommen: For patients who have symptoms due to hypertrophic cardiomyopathy, the first line of therapy is always to use medical management, medications. Usually, that's adding specific medications, but sometimes patients are on medications that can make their situation worse. And so some of the most effective therapy is removing the wrong agents, and then perhaps having to add in the right agents to help them with their symptoms down stream.

For patients who don't respond to those medical changes, or for whom those medications caused side effects that are intolerable, then that's when we move to things like surgical myectomy, which can more definitively relieve their symptoms.

Hartzell V. Schaff: The patients who are referred for surgery almost always have either failed medical treatment or have side effects from the medicines that limit them just as much as the symptoms from hypertrophic cardiomyopathy. So operation to relieve outflow tract obstruction is to relieve the symptoms. And in some patients, to allow them to get off of the medications that are having unwanted side effects.

Steve R. Ommen: Surgical myectomy has been a very successful operation for many of our patients. However, it is not utilized as much as it maybe could be in part owing to prior perceptions about increased risk with the operation, lack of universal availability of surgeons who can do it. But in the hands of expert centers, the complication rates are very low and our success rates are very high.

Hartzell V. Schaff: We now do a more extensive septal myectomy that extends towards the apex of the heart. And we've learned over the years that it's this distal portion of myectomy that's the most important in terms of relieving symptoms. The few patients who have had a second operation, who have been referred to us after having an operation that was unsuccessful, we found that the myectomy was not carried far enough into the ventricle. It's really not a regrowth of muscle. It's just an inadequate initial operation.

Steve R. Ommen: With surgical myectomy, the surgeon removes a portion of the hypertrophied septum, which is narrowing the path of blood, out of the heart. By doing this, it changes the direction the blood flows through the ventricle. It allows the mitral valve to function normally. And allows blood to leave the heart without increasing pressures or increasing forces. This muscle doesn't regrow over time. It is a permanent fix.

Hartzell V. Schaff: We found that it's rarely necessary to do something to the mitral valve. And the hazard with doing something to the mitral valve, where it turns out to be unnecessary, is there is a chance of injury. So we would prefer to do a septal myectomy, come off bypass, assess the mitral valve with an echocardiogram during surgery, before then addressing the mitral valve if there is residual regurgitation.

We can tell if the mitral regurgitation is relieved immediately after the myectomy once the aorta is closed and the heart is restarted. The echocardiogram is done in the operating room and we know right away whether the mitral regurgitation has been relieved. An operation is available for some patients that have non-obstructive hypertrophic cardiomyopathy. And these are patients with the apical distribution of hypertrophy.

Some of those patients have diastolic heart failure related to very small ventricular cavities. And in these patients, doing a transapical myectomy to enlarge the ventricle can improve their symptoms of heart failure.

Steve R. Ommen: While we see great results from the surgical myectomy as performed now, it is still something that should only be performed at true centers of excellence. Recent data that have come out have suggested that at low, medium, and even quote unquote "high volume" centers, there is a gradient of mortality, meaning it's highest at the low volume centers and lowest at the high volume centers.

But even those so-called high volume centers have mortality rates that are dramatically higher than what is reported from the true expert centers. And this is a procedure that should be done by those who are very familiar with this procedure, and doing a lot of them.

Hartzell V. Schaff: At Mayo Clinic, we've done over 3,000 operations for hypertrophic cardiomyopathy. We do 200 to 250 operations each year. The mortality for the procedure is less than 1%, especially for patients who are otherwise healthy.

Steve R. Ommen: One of the biggest parts of every interaction that I have with patients is helping them understand what their individual risk for sudden cardiac death might be, and whether they might consider having an implantable defibrillator. Our patients who have had surgery have a lower rate of sudden cardiac death and a lower rate for their defibrillators to discharge among those who have had them.

Hartzell V. Schaff: One of the things we've learned after doing septal myectomy is that actually the incidence of ventricular arrhythmia seems to be reduced. And this is shown in studies that look at defibrillator discharges and rates of sudden death.

Steve R. Ommen: The inheritance pattern of hypertrophic cardiomyopathy is autosomal dominant, which means each of the children of a patient with HCM has a 50/50 chance of inheriting this disease. We do recommend screening for all first-degree relatives, which is either genetic testing or echocardiographic-based surveillance. When a family has chosen to use echocardiography as their screening tool, we recommend that adult first-degree relatives get screened every five years. First-degree relatives who are adolescents or athletes, we usually screen every 12 to 18 months.

Hartzell V. Schaff: Septal myectomy cures the symptoms of hypertrophic cardiomyopathy when it relieves the obstruction. But of course, patients still have hypertrophic cardiomyopathy, still need to be followed by their physician for the other problems related to hypertrophic cardiomyopathy. But hopefully, are relieved of the shortness of breath, chest pain, or light-headedness that leads to the operation.

Cardiology Clinics

  • Heart transplant. You might be a candidate for a heart transplant if medications and other treatments are no longer effective and you have end-stage heart failure.

Clinical trials

Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this disease.

Lifestyle and home remedies

These lifestyle changes can help you manage cardiomyopathy:

  • Quit smoking.
  • Lose weight if you're overweight.
  • Eat a healthy diet, including a variety of fruits and vegetables and whole grains.
  • Reduce the amount of salt in your diet, and aim for less than 1,500 milligrams of sodium daily.
  • Get modest exercise after discussing with your doctor the most appropriate program of physical activity.
  • Eliminate or minimize the amount of alcohol you drink. Specific recommendations will depend on the type of cardiomyopathy you have.
  • Try to manage your stress.
  • Get enough sleep.
  • Take all your medications as directed by your doctor.
  • Go to your doctor for regular follow-up appointments.

Preparing for your appointment

If you think you may have cardiomyopathy or are worried about your risk because of a family history, make an appointment with your primary care doctor. He or she may refer you to a heart specialist (cardiologist).

Here's information to help you get ready for your appointment.

What you can do

Be aware of any pre-appointment restrictions. When you make the appointment, ask if there's anything you need to do in advance, such as restrict your diet.

Make a list of:

  • Your symptoms, including any that may seem unrelated to cardiomyopathy, and when they began
  • Key personal information, including a family history of cardiomyopathy, heart disease, stroke, high blood pressure or diabetes, and any major stresses or recent life changes
  • All medications, vitamins or other supplements you take, including doses
  • Questions to ask your doctor

Take a family member or friend along, if possible, to help you remember the information you're given.

For cardiomyopathy, some basic questions to ask your doctor include:

  • What's the most likely cause of my symptoms?
  • What are other possible causes?
  • What tests do I need?
  • What treatment options are available, and which do you recommend for me?
  • How often should I be screened?
  • Should I tell my family members to be screened for cardiomyopathy?
  • I have other health conditions. How can I best manage these conditions together?
  • Are there brochures or other printed material I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor is likely to ask you questions, including:

  • Do you have symptoms all the time, or do they come and go?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?

Cardiomyopathy care at Mayo Clinic

Jan. 23, 2019
  1. Cooper LT. Definition and classification of the cardiomyopathies. https://www.uptodate.com/contents/search. Accessed Dec. 9, 2017.
  2. What is cardiomyopathy in adults? American Heart Association. http://www.heart.org/HEARTORG/Conditions/More/Cardiomyopathy/What-Is-Cardiomyopathy-in-Adults_UCM_444168_Article.jsp#.Wi_ieEtrwmI. Accessed Dec. 9, 2017.
  3. Cardiomyopathy. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health-topics/cardiomyopathy. Accessed Dec. 9, 2017.
  4. Arrhythmia. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health-topics/arrhythmia#Treatment. Accessed Dec. 12, 2017.
  5. Kasper DL, et al., eds. Cardiomyopathy and myocarditis. In: Harrison's Principles of Internal Medicine. 19th ed. New York, N.Y.: McGraw-Hill Education; 2015. http://www.accessmedicine.mhmedical.com. Accessed Dec. 12, 2017.
  6. Ommen SR (expert opinion). Mayo Clinic, Rochester, Minn. Dec 21, 2018.
  7. Kim LK, et al. Hospital volume outcomes after septal myectomy and alcohol septal ablation for treatment of obstructive hypertrophic cardiomyopathy US nationwide inpatient database, 2003-2011. JAMA Cardiology 2016;1(3):333.
  8. Ommen SR, et al. Hypertrophic cardiomyopathy—one case per year? a clarion call to do what is right.. JAMA Cardiology 2016;1(3):333-334.
  9. Morrow ES. Allscripts EPSi. Mayo Clinic, Rochester, Minn. Jan. 7, 2019.
  10. Nguyen A, et al. Surgical Myectomy: Subaortic, Midventricular, and Apical. Cardiology Clinics. 2019;37(1):95.