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Annual mortality related to HCM is 1–2% in most studies with contemporary management strategies,128,715 and may be as low as 0.5%.727 The annual rate of SCD or appropriate ICD therapy is about 0.8% but is largely dependent on age and risk profile.85,728–730 HCM patients are at risk for heart failure, AF, and stroke.128,715 Most HCM-related deaths at age ≤60 years occur suddenly, while older patients die more often of stroke or heart failure.731 SCD is often related to VA, which can be a consequence of ischaemia, left ventricle outflow tract (LVOT) obstruction, or supraventricular arrhythmias. SCD may also be triggered by exercise, and participation in competitive sport has been discouraged.732 However, recent data suggest that even vigorous exercise in HCM patients without risk markers may not be associated with VA.733,734

7.1.3.3.1 Risk stratification and primary prevention of sudden car-

diac death. In primary prevention, the challenge remains to identify the relatively small group of patients with the highest risk of SCD. NSVT is identified on continuous (24/48h) ambulatory ECG monitoring in 20–25% of patients.128,715 Its prevalence increases with age and correlates with LV wall thickness and LGE on CMR.716–721,735 The prognostic value of NSVT for SCD is more important in young patients (,30 years).735 The relation between the duration, frequency, or rate of NSVT and HCM prognosis remains unclear.735

ECG sinus rhythm – Negative T wavesV2-V6 and inferior leads

ECG VT – RBBB-like, superior axis (apical LV origin)

CMR – Apical aneurysm with LGE

4-chamber 2-chamber

I

II

III V1

V2

V3

aVR

aVL

aVF V4

V5

V6

I

II

III V1

V2

V3

aVR

aVL

aVF V4

V5

V6

Figure 22 Typical features of hypertrophic cardiomyopathy associated with sustained monomorphic ventricular tachycardia. CMR, cardiac magnetic resonance; ECG, electrocardiogram; LGE, late gadolinium enhancement; LV, left ventricular; RBBB, right bundle branch block; VT, ventricular tachycardia. Downloaded from academic.oup.com/eurheartj/article/43/40/3997/6675633 by guest on 07 October 2026