Hypertrophic Cardiomyopathy Medical Slides

Present the AHA/ACC major risk factors: family history of SCD, unexplained syncope, massive LVH ≥30 mm, NSVT on Holter, abnormal BP response to exercise, and extensive LGE on CMR. Discuss the ESC HCM Risk-SCD calculator as an alternative quantitative tool. Emphasize that ICD is recommended for 5-year risk ≥6% (ESC) or ≥1 major risk factor with shared decision-making (AHA/ACC).

Use diagrams showing systolic anterior motion (SAM) of the mitral valve contacting the septum, creating a dynamic gradient that increases with decreased preload (Valsalva, standing, dehydration) and decreases with increased preload (squatting, leg elevation). Show the dagger-shaped CW Doppler signal and provocative testing with Valsalva or amyl nitrite during echo.

Reference the EXPLORER-HCM trial showing that mavacamten (a cardiac myosin inhibitor) reduced LVOT gradient to <30 mmHg in 74% of patients and improved NYHA class and exercise capacity. Present the VALOR-HCM trial demonstrating that mavacamten reduced the need for septal reduction therapy. Emphasize the REMS program requirement for monitoring LVEF (risk of excessive myocardial suppression) and the CYP2C19 interaction.