School Athletics and Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy is a genetic disorder of the heart, characterized by abnormal thickness of the heart muscle, in the absence of hypertension or valvular heart disease. The heart is the most important muscle in the body, responsible for delivering oxygenated blood to the rest of the body. It is divided into a right and left side. The right side receives blood that has been used and pumps it to the lungs, to pick up more oxygen.Afterwards, it is returns from the lungs, to the left side and pumped back out to the body by the left ventricle. The flow of blood leaves the heart through something called the left ventricular outflow tract. When the left Ventricle is abnormally thickened, it sometimes decreases the area of the outflow tract, impeding the flow of blood to the body. As a result, the heart attempts to work harder. Like any other muscle in the body, the harder it works, the thicker it gets. This is where the problem comes in. When the area is decreased to the point, that blood can no longer leave the heart, it can result in something often referred to as Sudden cardiac death.

The most well known victim of this illness is the late Reggie Wilson, who played for the Boston Celtics. In 1993, at the age of 27, he dropped dead in the midst of a game. Of course there have also been a significant number of associated deaths in collegiate sports. Since then, in many states many high school athletes are often prescreened for this disease, prior to being allowed to participate in competitive sports. One would think that such a devastating disorder, would have some type of signs and symptoms. Most patients are asymptomatic but the most common signs are: Dyspnea(shortness of breath), angina(chest pain), fatigue, and syncope(passing out). Unfortunately, in many athletes the first and last symptom, is collapse and sudden death. It is for this reason, athletic physical exams are extremely important.

In the past, electrocardiograms were often used as a determinant for the potential of this disease. The heart has a separate electrical system supporting its mechanical activity. Often, structural abnormalities such as excessive thickening, will reveal itself in the tracing of an EKG. However, the gold standard is echocardiography. Echocardiography is an ultrasound procedure in which a two dimensional real time image of the heart can be visualized. Unlike an EKG, in which only the electrical activity is seen, the structural anatomy, cardiac function, and blood flow of the heart can be analyzed. If the measurements of the heart are within the upper limits of normal, a cardiologist may order what is called a stress echocardiogram to study the heart during exercise. With this study, both the EKG and Ultrasound study are combined to see if there is any obstruction to the outflow tract during exercise. If the studies come back positive for obstruction  there are a number of treatment options to control symptoms.

Medications such as Beta and calcium channel blockers,are used to decrease the heart rate and lower the demand on the heart. Septal ablation, is a catheterization procedure in which alcohol is used to necrose the thickness of the heart muscle near the outflow tract. Pacemakers and implantable defibrillators(AICD) can be implanted to control any electrical malfunctions of the heart, that would cause an increase in demand. As a last resort, open heart surgery is also an option. There they would perform a septal myectomy and remove the hypertrophied area.

Hypertrophic cardiomyopathy is a rare genetic disorder, occurring in .02 to .2 percent of the population as a whole. Many people live normal lives without symptoms. Yet it is especially important to be tested if there is a family history or signs and symptoms. If you feel as if you may be at risk, see your doctor and get tested. Early detection saves lives.