SPC365

Electrical storm in Hypertrophic cardiomyopathy: Risk stratification and prevention of sudden death

Session: Sessão de casos clínicos
Speaker: Pedro Brás

Congress: CPC 2021
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Session Type: Sessão de Casos Clínicos
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Authors: Pedro Garcia Brás; Sílvia Aguiar Rosa; Guilherme Portugal; Bruno Valente; Pedro Silva Cunha; Alexandra Castelo; Vera Ferreira; Luísa Moura Branco; Mário Oliveira; Rui Cruz Ferreira
<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">The authors present the case of a 39 year-old male patient with non-obstructive hypertrophic cardiomyopathy (HCM) with previous history of an embolic stroke due to left ventricular (LV) thrombus, under oral anticoagulation with rivaroxaban. He had a family history of one brother with HCM and two cousins with sudden cardiac death (SCD) at the age of 40.<br />
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The 12-lead ECG showed pathological Q waves in leads V4-V6 and QRS fragmentation in the limb leads. The cardiac magnetic resonance (CMR) showed a maximum wall thickness of 30 mm, LV apical aneurysm and extensive late gadolinium enhancement, 29% of LV mass. There was no history of syncope or nonsustained ventricular tachycardia (NSVT) in 24h holter.</span></span></span></p>

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">According to the ESC HCM SCD risk score, the risk for SCD was low (3%) but while there was no family history of SCD in first-degree relatives there were two cousins with SCD at the age of 40. Moreover, the patient&rsquo;s ECG revealed Q waves and QRS fragmentation in several leads and the CMR showed a LV apical aneurysm and extensive LGE indicating considerable myocardial fibrosis, with a superior risk for SCD.</span></span></span></p>

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">After discussion with the Electrophysiology team, considering the aforementioned SCD high risk markers, the patient consented to an urgent implantable cardioverter-defibrillator (ICD) in primary prevention.<br />
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30 days after ICD implantation, the patient was transferred to the Intensive Care Unit in refractory electrical storm (ES), with over 50 appropriate ICD shocks due to sustained VT. Immediate anti-arrhythmic therapy was started, with amiodarone, esmolol and lidocaine perfusion, with no success. Patient sedation, mechanical ventilation and ICD-mediated overdrive pacing therapies were performed, also without significant response. With refractory ES, and a long period of hemodynamic instability, decision for LV assistance with veno-arterial ECMO was made.<br />
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The patient&rsquo;s evolution under ECMO support was favourable, maintaining however periods of NSVT and a significant LV intracavitary gradient (96 mmHg). Upon ECMO decannulation there was acute femoral artery thrombosis and surgical thromboembolectomy was performed.</span></span></span></p>

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">In this case of an aggressive presentation of refractory ES with a high risk of recurrence, the patient was successfully submitted to VT catheter ablation, guided by CMR imaging (extensive scar region in apical LV).</span></span></span></p>

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">In the following days the patient developed severe pericardial effusion (27.5 mm), with limited response to anti-inflammatory drugs, improving with corticosteroid therapy.</span></span></span></p>

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">The patient was discharged 2 weeks later in sinus rhythm with PE regression, under therapy with prednisolone, amiodarone, bisoprolol and diltiazem. </span></span></span></p>

<p><span style="font-size:11pt"><span style="font-family:&quot;Calibri&quot;,sans-serif"><span style="font-size:12.0pt">At 1 year follow-up there was no VT recurrence, ICD therapies or pericardial effusion.</span></span></span></p>

<p><span style="font-size:11pt"><span style="font-family:&quot;Calibri&quot;,sans-serif"><span style="font-size:12.0pt">While not contemplated on the ESC SCD risk score, CMR can provide key findings of superior SCD risk, which can be crucial in guiding an integrated approach to predict SCD risk.</span></span></span></p>