Electrical storm in hypertrophic cardiomyopathy: Cardiac magnetic resonance and sudden cardiac death risk stratification: a case report

BACKGROUND: Risk stratification for sudden cardiac death (SCD) is a key factor in the management of patients with hypertrophic cardiomyopathy (HCM). Cardiac magnetic resonance (CMR) has a unique role in the evaluation of HCM and offers superior diagnostic and prognostic information to assess the indication for a prophylactic implantable cardioverter-defibrillator (ICD). CASE SUMMARY: A 39-year-old patient with non-obstructive HCM with a low ESC HCM Risk-SCD score underwent a CMR revealing a left ventricular apical aneurysm and extensive late gadolinium enhancement; a prophylactic ICD was thus implanted. A month later, the patient was admitted in refractory electrical storm with over 50 appropriate ICD shocks due to sustained ventricular tachycardia. Despite anti-arrhythmic therapy and mechanical ventilation, the evolution was unfavourable with haemodynamic instability; veno-arterial extracorporeal membrane oxygenation was implanted. The patient was submitted to CMR-guided epicardial VT catheter ablation with complications of LV thrombus and severe pericardial effusion. DISCUSSION: This case details the complex risk stratification for SCD in patients with HCM, highlighting the important role of CMR in the integrated approach to risk stratification.