A 35-year-old man undergoes genetic testing after his brother dies suddenly. He is found to carry a MYBPC3 mutation. Echocardiogram shows maximal wall thickness of 22 mm, LVOT gradient 42 mmHg at rest. He is asymptomatic. What is the primary risk stratification tool for sudden cardiac death (SCD) in HCM?

• A HCM Risk-SCD calculator (ESC 5-year SCD risk score) ✓
• B Holter monitoring for non-sustained VT
• C Exercise stress testing with BP monitoring
• D Maximum wall thickness alone (>30 mm = high risk)

Explanation

The ESC HCM Risk-SCD calculator (2014) incorporates age, family history of SCD, unexplained syncope, max LV wall thickness, LA diameter, LVOT gradient, and NSVT to estimate 5-year SCD risk, guiding ICD implantation decisions (ICD if ≥6% 5-year risk). This is validated as the primary tool in ESC 2014/2020 HCM guidelines. Wall thickness >30 mm alone is a recognised independent risk factor but insufficient as the sole tool. Holter for NSVT and exercise BP are components that feed into the calculator.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.