Genetic condition awaiting consideration - Familial Hypertrophic Cardiomyopathy-4 (CMH4)
Hypertrophic cardiomyopathy (HCM) is a primary disease of the myocardium in which a portion of the myocardium is hypertrophied (thickened) without any obvious cause. It is perhaps best known as a leading cause of sudden cardiac death in young athletes.
The occurrence of hypertrophic cardiomyopathy is a significant cause of sudden unexpected cardiac death in any age group and a cause of disabling cardiac symptoms. Younger people are likely to have a more severe form of hypertrophic cardiomyopathy.
HCM is frequently asymptomatic until sudden cardiac death, and for this reason some suggest routinely screening certain populations for this disease. The clinical course of HCM is variable. Many patients are asymptomatic or mildly symptomatic.
The symptoms of HCM include dyspnea (shortness of breath), chest pain, palpitations, light headedness, fatigue, fainting and sudden cardiac death. Dyspnea is largely due to increased stiffness of the left ventricle, which impairs filling of the ventricles and leads to elevated pressure in the left ventricle and left atrium.
Major risk factors for sudden death in individuals with HCM include prior history of cardiac arrest or ventricular fibrillation, spontaneous sustained ventricular tachycardia, family history of premature sudden death, unexplained syncope, left ventricle thickness greater than or equal to 30 mm, abnormal exercise blood pressure and nonsustained ventricular tachycardia
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Page last updated: 18 April 2013