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Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is a common genetic cardiovascular disease affecting one in 500 Americans. Most people with hypertrophic cardiomyopathy have few or no symptoms. However, hypertrophic cardiomyopathy can result in heart failure, stroke and sudden death. In fact, it is the most common cause of sudden death in young patients, especially athletes.

Specialists at the Barnes-Jewish & Washington University Heart & Vascular Center provide comprehensive, state-of-the-art care to patients with hypertrophic cardiomyopathy, customizing treatment for each patient to get the best outcomes.

Understanding Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy occurs when the heart muscle becomes abnormally thick and affects the pumping action of the heart. If there is significant thickening just below the aortic valve, the blood flowing out of the heart will be obstructed in what is called hypertrophic obstructive cardiomyopathy (HOCM).

Illustration depicting the difference between a normal heart and hypertrophic cardiomyopathy

Symptoms include shortness of breath, chest discomfort, palpitations, fatigue, dizziness or passing out. Medications such as beta blockers, calcium channel blockers and diuretics may improve or alleviate chest pain, shortness of breath or palpitations.

Most people with hypertrophic cardiomyopathy live a normal lifespan. However, there is a small subset of patients who are at increased risk of sudden premature death.

Risk factors associated with an increased risk of sudden death include:

  • previous cardiac arrest
  • family history of sudden death
  • passing out episodes (syncope)
  • multiple runs of ventricular tachycardia seen on a monitor
  • massive thickening of the heart muscle, especially more than 30 mm
  • fall in blood pressure with exercise

Treating Hypertrophic Cardiomyopathy

The goal of treatment is to provide patients the opportunity to lead a normal life. For many people, medical management is sufficient, along with avoiding triggers such as strenuous sport or work activity.

When medical treatment fails, a myomectomy may be performed to surgically reduce the obstruction in the left ventricle. This delicate operation is done while the patient is on a heart-lung bypass machine. Muscle tissue just below the aortic valve is removed, increasing blood outflow. Myomectomy can result in significant reductions in shortness of breath, chest pain and instances of passing out.

Alcohol septal ablation can achieve similar results to a myomectomy without subjecting a patient to open heart surgery. This procedure consists of injecting alcohol into a selected vessel of the heart and inducing a controlled heart attack. The selected heart muscle dies, eliminating the obstruction of blood flow out of the heart.

Patients with risk factors for sudden death are evaluated for an internal cardioverter defibrillator (ICD). An ICD has a sophisticated computer and is able to monitor the heart's rhythm and deliver a shock if a life threatening rhythm is detected. The ICD is the best protection against sudden death.

Other treatments, such as alcohol septal ablation and a myomectomy may reduce the symptoms or reopen the blood flow but do not protect against sudden death.

Patients with HCM should avoid:

  • bursts of physical exertion
  • extreme temperatures
  • prolonged exercise
  • heavy lifting

Recent estimates suggest that 60-70 athletes die every year. Hypertrophic cardiomyopathy is the most common cause of sudden death in young athletes.

To make an appointment with a Washington University heart or vascular specialist at Barnes-Jewish Hospital, call 855.925.0631.