Thursday 2 November 2017

Hypertrophic Cardiomyopathy

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Hypertrophic cardiomyopathy (HCM) is a genetic disease that causes the heart muscle to become thickened, or hypertrophied. The heart cells in HCM are also abnormal, arranged in a disorganized pattern with fibrosis/scarring often present between the cells. In addition, the mitral valve can be affected and in some patients may briefly obstruct blood flow out from the main pumping chamber of the heart, which can result in regurgitation of blood back to the left atrium and higher pressures than normal in the heart.

The prevalence of HCM in the general population is 1 in 500 adults, although the majority of these cases remain undiagnosed. The disease is associated with normal longevity in the vast majority of diagnosed patients. In addition, most patients with HCM are asymptomatic and are not at risk of developing adverse complications related to HCM. Among those patients who do develop symptoms, the most common complaints include: chest pain, shortness of breath with exertion, fatigue, palpitations, and lightheadedness. A very small minority of patients are at risk of sudden death, which occurs most commonly in young patients.

The above is taken from Patient education: Hypertrophic cardiomyopathy (Beyond the Basics), by Martin S Maron, MD. This is an excellent article, link here. Other very good articles about HCM are linked from that page, at left, under "Related Articles".

Stanford Hospital is a centre of excellence for the study and treatment of HCM. The following videos by the director of Stanford Hypertrophic Cardiomyopathy Centre, Euan Ashley, MD, are highly enlightening -

As it's a genetic disease, other family members are at risk of having HCM -

Relationship to person with HCMChances the relative will have HCMChances that they don't have HCM
Identical twin100%0%
Child, Parent, Sibling50%50%
Grandparent, Uncle, Aunt, Niece, Nephew25%75%
First Cousin12.5%87.5%
Family members who are concerned should see their GP, for possible referral to get an echocardiogram and ECG. 

More excellent resources concerning HCM are -

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