Fast Five Quiz: Hypertrophic Cardiomyopathy

Jamshid Shirani, MD


April 13, 2022

The clinical course for patients with HCM is highly variable. While some patients remain asymptomatic with little need for treatment, others exhibit progressive disease requiring more aggressive treatment to improve morbidity and mortality. Pharmacological therapy is administered to patients with the goal of alleviating symptoms of exertional dyspnea, palpitation, and angina. Both beta-blockers and calcium channels blockers improve exertional dyspnea and chest pain. Invasive treatment, such as surgical myectomy, is indicated in patients with an LVOT gradient of 50 mmHg who remain symptomatic despite optimal medical therapy.

Since the introduction of genetic testing, over 2000 pathogenic variants in at least 11 genes encoding cardiac sarcomere proteins have been identified in association with this disease. Despite these data, owing to genetic heterogeneity, it has been well established that mutations do not reliably predict outcomes, and it is not recommended as a guide to management. However, genetic testing plays an important role in family screening.

Depending on the severity and progression of symptoms, patients diagnosed with HCM should avoid high-intensity activity. Nonstrenuous activity is not a risk factor for sudden cardiac death.

Learn more about HCM.

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