Extract

Hypertrophic cardiomyopathy (HCM) is one of the most frequent inherited cardiac diseases and is one of the leading causes of sudden cardiac death (SCD) in the young with an annual incidence for SCD of 1%.1 Estimation of SCD risk is a major part of the clinical management of HCM; therefore, the ESC guidelines recently proposed a new SCD risk prediction model. This model includes clinical data (age, unexplained syncope, familial history of SCD), arrhythmic data (non-sustained ventricular tachycardia—NSVT), and echocardiographic evaluation (maximal wall thickness, left atrial diameter, and left ventricular outflow tract gradient).2 This model can help the clinician to select the patient in whom implantation of an ICD should be implanted; but still there is a need for an improvement in risk stratification.

The investigation of cardiac tissue structure with late gadolinium enhancement (LGE)-CMR seems to be a promising tool in predicting cardiovascular mortality in HCM patients; nevertheless, it cannot be performed in every patient and is less available than echocardiography. Alteration of longitudinal function evaluated by speckle tracking is associated with NSVT3 and with LGE-CMR.3–5 The use of strain analysis is now recommended in HCM as a clinical tool for the evaluation of LV systolic function.6

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