Hypertrophic Cardiomyopathy (HCM) is a primarily genetic disease that causes a thickening of the heart walls, usually on the left side, without any apparent reason. A normal heart has a left ventricular wall and septal thickness of 1.2 centimeters or less. With HCM, the left ventricular wall and septal thickness are 1.3 cm or greater. The prevalence of HCM is estimated at 1:500 in the general population and is the leading cause of sudden cardiac death in children and young adults in the U.S.
Once genetic cardiac disease or early sudden death has been identified in a family, it is imperative that all immediate and extended family members be screened at once and again at regular intervals by a cardiologist to monitor for the potential development of cardiac disease.
- Under 12 years old: If they are involved with athletic activities should have yearly cardiologist screenings.
- Ages 12 - 21: Should have yearly cardiologist screenings including an electrocardiogram and echocardiogram.
- Over 21 years: Every five years throughout life with a cardiologist screening, an echocardiogram, and an electrocardiogram.
Symptoms
- Heart murmur (present in 30-40% of individuals with HCM)
- Shortness of breath (dyspnea)
- Chest pain (angina)
- Palpitations, lightheadedness, and fainting
- Complications of HCM may lead to ventricular tachycardia, atrial fibrillation, endocarditis, stroke, and cardiac arrest.
Diagnosis
Diagnosis is made using results of an echocardiogram and an electrocardiogram. Risk assessment is made using results of Holter monitor and exercise testing.
Treatment
Treatment may include medications such as beta-blockers, calcium channel blockers, antiarrhythmic drugs, diuretics, anticoagulants and antibiotics. Surgical management may include ventricular septal myotomy-myectomy. Devices which may be necessary to manage HCM, are pacemakers and implantable cardioverter defibrillators (ICD).
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