Autosomal dominant. Chest pain, dyspnea, syncope, arrhythmia, pulses bisferiens, systolic murmur LLSE (↑ Valsalva & standing), triple apex. › CVA, HF, SCD.
Screen 1st-degree relatives › TTE. AF › DOAC.
ICD: sustained VA · prior arrest · FH SCD · wall >30mm · unexplained syncope · apical aneurysm · LVEF <50% · extensive LGE · NSVT on Holter.
Rx: BBs/CCBs (non-DHP) › disopyramide/mavacamten › myomectomy/EtOH ablation (LVOT gradient ≥50).
Avoid: ACEi/ARBs, vasodilators, diuretics, PDE5i, digoxin, nitrates.
ECG: LVH, LAE, deep symmetric TWI V3–V6 (apical), QW, repol abnormalities.
Echo: asymmetric septal hypertrophy, MV SAM, LVOTO (gradient <50 › stress echo), eccentric MR. Repeat 1–2 yearly.