What is HCM...

HCM is a genetic disease which results in a thickening of the heart muscle and is the leading cause of sudden death in children and young adults.  While these young people have many things in common, the most unfortunate is that they all died needlessly from HCM, but if properly diagnosed, it's treatable. It is the leading cause of sudden cardiac death among young athletes; both professional and amateur.

1 in 500 North Americans have HCM and it can be detected and treated allowing patients to live long lives. HCM is a not an uncommon heart disease. HCM is nearly seven times more common than Cystic Fibrosis.

Symptoms...

There is no particular symptom or complaint which is unique to HCM and symptoms may occur at any stage in a person's life, even though the condition may have been present for some time. Unfortunately, when sudden death strikes, as a result of HCM, there are usually NO Symptoms!

The reason for the onset of symptoms is often not clear. However, they include shortness of breath, chest pain, palpitations, light-headedness and blackouts. The most commonly seen misdiagnosis is asthma, specifically "athletically induced asthma" as a first sign or symptom. This is likely due to transient shortness of breath often seen in HCM. Unfortunately, in some instances, the first symptom may be sudden cardiac arrest.

Here are some frightening facts about kids and heart disease:

• Heart disease kills more children than any other disease except cancer.

• 7,000 to 14,000 children die each year from heart disease.

• One in 500 people have life-threatening HCM and most are unaware.

• Out of 25 million youth in this country, 2,500,000 are at risk for Sudden Cardiac Arrest from undetected heart problems.

What is Long QT Syndrome

Long QT syndrome (LQTS) causes an abnormality of the heart's electrical system. The mechanical function of the heart is entirely normal. The electrical problem is due to defects in heart muscle cell structures called ion channels. These electrical defects predispose affected persons to a very fast heart rhythm (arrhythmia) called "Torsade de Pointes" (TdP) which leads to sudden loss of consciousness (syncope) and may cause sudden cardiac death.

How is LQTS Diagnosed

The diagnosis is commonly suspected or made from the electrocardiogram (ECG). All children and young adults should have an ECG as part of their evaluation before playing sports.

Diagnosing HCM and Arrhythmias

An echocardiogram is the Gold Standard for diagnosing HCM. Echocardiography uses sound waves to produce an image of the heart and to see how it is functioning. Doctors can learn about the size, shape, and movement of your heart muscle. This test can also show how the heart valves are working and how blood is flowing through your heart. Echocardiography can also give doctors information about your arteries. Echocardiography uses high-frequency sound waves (also called ultrasound) that can provide a moving picture of your heart. The sound waves are sent through the body with a device called a transducer. The sound waves bounce off of the heart and return to the transducer as echoes. The echoes are converted into images on a television monitor to produce pictures of your heart. An echocardiogram is completely painless.



An electrocardiogram (ECG or EKG) is a routine test that is used to look at the electrical activity of the heartbeat. An electrocardiogram can tell our cardiologist a lot about your heart and how it is working. This test can help our doctor learn more about your heart's rhythm, the size and function of the chambers of your heart, and your heart muscle. A healthy person's electrocardiogram has a certain pattern. When there are changes in that pattern, your doctor can tell that there is a problem with your heart.

Distinguishing from "Athlete's Heart"

For athletes, there can sometimes be confusion about the diagnosis of HCM. This stems from the fact that well-trained athletes often have thickening of the ventricular walls that is simply a physiologic consequence of training. The distinction between HCM and athlete's heart can be particularly problematic for individuals with ventricular wall thickness between 1.0 and 1.5 cm. Athletes in this situation would be well-served by consultation with a cardiologist with particular expertise in this area. Features that would favor athlete's heart over HCM include: left ventricular cavity size >55 mm in diameter, and decrease in wall thickness with de-conditioning. Features that would favor HCM include: family history of HCM and/or abnormal ECG.