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HCM with Apical Aneurysm — EECC MCQ

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HardCardiomyopathyHCM with Apical AneurysmEECC

A 50-year-old man with HCM is found to have an LV apical aneurysm on CMR, a feature not typical of classic HCM. LGE shows transmural scar in the apex. What is the clinical significance?

Educational content. Not a substitute for clinical judgement or local policy.

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Correct answer: C — LV apical aneurysm in HCM is associated with high arrhythmic risk (sustained VT/VF) and thromboembolic risk (mural thrombus), and should prompt consideration of ICD and anticoagulation regardless of the HCM Risk-SCD score

LV apical aneurysm occurs in approximately 2-5% of HCM patients and represents a distinct, high-risk subphenotype. It is characterised by an apical aneurysm/dyskinesia with thinned apical wall, often with transmural LGE, surrounded by hypertrophied mid-ventricular segments. The 2023 ESC Cardiomyopathy Guidelines identify it as a risk modifier for SCD that is NOT captured by the standard HCM Risk-SCD calculator (which may underestimate risk in these patients). Complications include: (1) re-entrant VT from the scar-hypertrophy border zone; (2) thrombus formation in the aneurysm; (3) progressive systolic dysfunction. ICD should be considered (Class IIa as risk modifier) and anticoagulation for thrombus prevention.

Reference: ESC (2023): Guidelines on Cardiomyopathies