Hypertrophic cardiomyopathy (HCM) is a heart condition in which the heart muscle in the left ventricle — the heart’s lower left chamber — gets thick and stiff, making it difficult for the heart to pump blood properly. Apical HCM is a specific type of HCM that affects the left ventricle apex — the bottom point of the left ventricle.

What Is Apical Hypertrophic Cardiomyopathy?

HCM is an umbrella term for conditions that cause hypertrophy (thickening) of the left ventricle wall. The most common type of HCM is asymmetric septal, also known as classic HCM. This type is diagnosed when the interventricular septum (the wall that separates the heart’s two ventricles) thickens.

Apical HCM is diagnosed when the apex of the left ventricle becomes thickened. The apex is the very tip of the heart, located at the bottom left side. It’s like the pointed end of a cone.

Apical HCM represents about 3 percent of all cases of HCM in the United States and is more common in certain ethnic groups. It’s most common among Japanese populations, affecting around 15 percent of all people there who are living with HCM, according to the Journal of Community Hospital Internal Medicine Perspectives.

Apical HCM also occurs in around 25 percent of HCM cases among Asian people and up to 10 percent of cases among non-Asians, according to a study in the Journal of the American Heart Association. The researchers note that the condition affects men more often than women. The average age at diagnosis is around 41 years old.

There are three types of apical HCM:

• Pure apical HCM is diagnosed when there is thickening only in the apex.
• Mixed apical HCM has thickening in both the apex and the septum. Even though thickening occurs in both areas, there’s usually more seen in the apex.
• Relative apical HCM is diagnosed when a person doesn’t meet all the criteria for proper diagnosis but has some features. Researchers believe that relative apical HCM is an early form of the disease.

Family history is a risk factor for HCM, meaning that family members who have it are likely to pass it on. First-degree relatives (children of biological parents) have a 50 percent chance of inheriting the genetic risk for classic HCM. However, studies show that apical HCM is less likely to be passed on in families. Only 13 percent to 25 percent of apical HCM cases are caused by known gene mutations, according to the Journal of the American Heart Association.

Compared to classic HCM, apical HCM is associated with more cases of atrial fibrillation (AFib). This condition occurs when the atria (top chambers of the heart) beat rapidly and irregularly, which increases the chance of blood clots. Apical HCM is also associated with more strokes — both from apical aneurysms and from AFib — but overall survival is better than with some other forms of HCM.

What Are the Symptoms of Apical Hypertrophic Cardiomyopathy?

The symptoms of apical HCM often overlap with those of other heart conditions, which can make diagnosis difficult. Many people with the condition aren’t diagnosed until a few years after their symptoms begin.

Common signs and symptoms of apical HCM include:

• Angina (chest pain)
• Dyspnea (trouble breathing)
• AFib
• Syncope (passing out or fainting)
• Heart palpitations (fluttering or pounding heartbeat)
• Difficulty exercising or performing strenuous activities

In some cases, stroke, heart attack, or congestive heart failure is the first noticeable sign of apical HCM that leads to a diagnosis.

How Is Apical Hypertrophic Cardiomyopathy Diagnosed?

Apical HCM is diagnosed using a few imaging techniques that help your cardiologist take a closer look at your heart. Your doctor might also use electrocardiography to measure your heart rhythm. No one test is used to diagnose apical HCM — instead, doctors use findings from several tests to make a final diagnosis.

Echocardiogram

Echocardiogram tests use ultrasound waves to create live images of the heart. The sound waves bounce off the heart’s structures and tissues and are read by a hand-held wand, called a transducer, that turns them into images.

Cardiologists may choose to use either a transthoracic echocardiogram (TTE) or a transesophageal echocardiogram (TEE) to look at your heart. TTE takes pictures by placing a device called a transducer over your chest. In some cases, your ribs or lungs may block the ability to get clear images. When this happens, you may have a TEE, which uses an ultrasound probe placed into your esophagus.

Cardiologists use echocardiography to look for heart abnormalities, including thickening of the ventricular wall at the apex. Apical HCM is diagnosed when the left ventricle wall thickness is greater than 15 millimeters.

A specific type of echocardiographic exam, known as Doppler echocardiography, can also be used to measure the blood flow through the heart. For people with HCM, cardiologists use the Doppler technique to check the difference in pressure between the bottom of the heart and the top with blood, where blood flows through the aortic valve into the body. This can show if you’re at risk of other complications, including irregular heartbeats in the ventricles and thromboembolism (blockage of blood vessels with blood clots).

Cardiac Magnetic Resonance Imaging

Cardiac magnetic resonance imaging (MRI) is another imaging technique for taking highly detailed pictures of your heart. MRIs use strong magnets and radio waves to generate images of the muscles, valves, and chambers in your heart.

Cardiac MRIs have an advantage over echocardiograms in that they can show the entire left ventricle and apex, helping your cardiologist look for left ventricle thickening. One study found that echocardiograms missed 40 percent of apical HCM cases that were later diagnosed with cardiac MRIs.

Before a cardiac MRI, you may have a contrast agent injected into your blood via an IV line. This helps your heart stand out in images. Gadolinium is a common contrast agent used, and it can help cardiologists look for fibrosis (scar tissue) in the heart. In people with apical HCM, fibrosis can cause arrhythmias (irregular heartbeat) and may lead to heart failure.

Electrocardiogram

During an electrocardiogram (ECG), patches containing electrodes are stuck to your skin to measure your heart’s electrical activity. Specifically, ECGs measure your heart’s rate and rhythm to find any abnormalities that point to a heart condition.

ECGs read your heart rhythm through electrical signals as the different parts of the heart contract (squeeze) and relax. This creates a distinct pattern, or tracing, on the electrocardiogram that lets your cardiologist know how well your heart is functioning.

One function read during an ECG is a T wave, which shows the heart’s ventricles repolarizing — preparing to beat again after a contraction. One study found that more than 90 percent of people with apical HCM have abnormal T waves.

Talk to Your Doctor About Apical Hypertrophic Cardiomyopathy

If you notice any symptoms of apical HCM, talk to your doctor or cardiologist. They’ll likely run some imaging tests and perform an ECG to look for any abnormalities and rule out other heart conditions.

Once you receive a diagnosis, your cardiology team will work with you to develop a treatment plan. Many treatments for apical HCM are also used for classic HCM, including beta-blockers and calcium channel blockers. If you have AFib, your doctor may also prescribe AFib treatments, such as blood thinners to help prevent blood clots.

Most people with apical HCM aren’t candidates for surgical or alcohol septal ablation because the septum usually isn’t involved in the disease. Your cardiologist will be able to explain the best options to manage your condition.

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