What is hypertrophic cardiomyopathy?
Hypertrophic Cardiomyopathy (HCM or HOCM) is the leading cause of death in young people. HCM is a genetic disease that causes the thickening of the myocardium (heart muscle) – the heart muscle becomes abnormally thick, making it harder to pump blood throughout the body. Sudden death may occur from the onset of ventricular tachycardia (a type of rapid heart rate) or other dangerous arrhythmias. Although it can occur in any age group, sudden death is most shocking when it happens to young adults or athletes. While these tragic deaths are often given prominence in the media, sudden death is rare (1% or less per year in adults with hypertrophic cardiomyopathy). One death is too many. HCM cannot be cured but once detected can be managed under the care of a physician.
A dynamic interdisciplinary team is recommended to work with anyone who suspects they may have the disease or to offer effective treatment to diagnosed HCM patients.
Age of Onset
HCM can be confusing and elusive, even to the most experienced medical professionals. A young person who suddenly has a catastrophic cardiac event caused by HCM most likely had a perfectly normal heart at birth and for the first few years of life. That is why it is so confounding when a highly trained – healthy (appearing) young athlete dies of sudden cardiac death (SCD). Studies show that one third of SCD among young athletes can be attributed to HCM.
As reported, one in five hundred children have the gene and the disease generally expresses itself during puberty – the time of greatest growth. Most cases of disease are apparent by the late teens or early twenties. However, the HCM can occur at any age – from birth to 90’s. The most important part of the HCM age issues is the fact that the disease is five times more fatal in adolescents than when it presents itself in later years. The risk of SCD from HCM decreases with age. Therefore, as an individual gets older, on set of HCM frequently causes only mild disability or no disability at all. Many patients achieve normal life expectancy (some without even being aware of their disease).