Advertisement
Case Report|Articles in Press

Refractory electrical storm in a patient with hypertrophic cardiomyopathy and an implantable cardioverter-defibrillator

Published:February 09, 2023DOI:https://doi.org/10.1016/j.jccase.2023.01.008

      Abstract

      A 63-year-old man with hypertrophic cardiomyopathy (HCM), mid-ventricular obstruction, and an apical aneurysm had an episode of cardiac arrest due to sustained ventricular tachycardia (VT). He was resuscitated and an implantable cardioverter-defibrillator (ICD) was implanted. In the following years, several episodes of VT and ventricular fibrillation were successfully terminated by antitachycardia pacing or ICD shocks. Three years after ICD implantation, he was re-admitted because of refractory electrical storm (ES). Since aggressive pharmacological treatments, direct current cardioversions, and deep sedation were not effective, he underwent epicardial catheter ablation which was successful to terminate ES. However, because of the recurrence of refractory ES after one year, he proceeded to surgical left ventricular myectomy with apical aneurysmectomy which provided him a relatively stable clinical course for six years. Although epicardial catheter ablation may be an acceptable option, surgical resection of apical aneurysm seems to be most efficacious for ES in patients with HCM and an apical aneurysm.

      Learning objectives

      In patients with hypertrophic cardiomyopathy (HCM), implantable cardioverter-defibrillators (ICDs) are the gold standard of therapy for prophylaxis against sudden death. Electrical storm (ES) caused by recurrent episodes of ventricular tachycardia can cause sudden death even in patients with ICDs. Although epicardial catheter ablation may be an acceptable option, surgical resection of apical aneurysm is most efficacious for ES in patients with HCM, mid-ventricular obstruction, and an apical aneurysm.

      Abbreviations:

      ES (electrical storm), HCM (hypertrophic cardiomyopathy), ICD (implantable cardioverter-defibillator), NSVT (non-sustained ventricular tachycardia), VT (ventricular tachycardia)

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Journal of Cardiology Cases
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Maron B.J.
        Clinical course and management of hypertrophic cardiomyopathy.
        N Engl J Med. 2018; 379: 655-668
        • Maron B.J.
        • Shen W.K.
        • Link M.S.
        • Epstein A.E.
        • Almquist A.K.
        • Daubert J.P.
        • Brady G.H.
        • Favale S.
        • Rea R.F.
        • Boriani G.
        • Estes N.A.M.
        • Spirito P.
        Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy.
        N Engl J Med. 2000; 342: 365-373
        • Maron B.J.
        • Rowin E.J.
        • Maron M.S.
        Paradigm of sudden death prevention in hypertrophic cardiomyopathy.
        Circ Res. 2019; 125: 370-378
        • Ommen S.R.
        • Mital S.
        • Burke M.A.
        • Day S.M.
        • Deswal A.
        • Elliott P.
        • Evanovich L.L.
        • Hung J.
        • Joglar J.A.
        • Kantor P.
        • Kimmelstiel C.
        • Kittleson M.
        • Link M.S.
        • Maron M.S.
        • Martinez M.W.
        • et al.
        2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American Heart Association Joint Committee on practice guidelines.
        Circulation. 2020; 142: e558-e631
        • Rowin E.J.
        • Maron B.J.
        • Haas T.S.
        • Garberich R.F.
        • Wang W.
        • Link M.S.
        • Maron M.S.
        Hypertrophic cardiomyopathy with left ventricular apical aneurysm: implications for risk stratification and management.
        J Am Coll Cardiol. 2017; 69: 761-773
        • Green J.J.
        • Berger J.S.
        • Kramer C.M.
        • Saleno M.
        Prognostic value of late gadolinium enhancement in clinical outcomes for hypertrophic cardiomyopathy.
        JACC Cardiovasc Imaging. 2012; 5: 370-377
        • Carbucicchio C.
        • Santamaria M.
        • Trevisi N.
        • Maccabelli G.
        • Giraldi F.
        • Fassini G.
        • Riva S.
        • Moltrasio M.
        • Cireddu M.
        • Veglia F.
        • Della Bella P.
        Catheter ablation for the treatment of electrical storm in patients with implantable cardioverter-defibrillators: short-and long-term outcomes in a prospective single-center study.
        Circulation. 2008; 117: 462-469
        • Hang D.
        • Schaff H.V.
        • Ommen S.R.
        • Dearani J.A.
        • Nishimura R.A.
        Combined transaortic and transapical approach to septal myectomy in patients with complex hypertrophic cardiomyopathy.
        J Thorac Cardiovasc Surg. 2018; 155: 2096-2102
        • Kunkala M.R.
        • Schaff H.V.
        • Nishimura R.A.
        • Abel M.D.
        • Sorajja P.
        • Dearani J.A.
        • Ommen S.R.
        Transapical approach to myectomy for midventricular obstruction in hypertrophic cardiomyopathy.
        Ann Thorac Surg. 2013; 96: 564-570
        • Sun D.
        • Schaff H.V.
        • Nishimura R.A.
        • Geske J.B.
        • Dearani J.A.
        • Ommen S.R.
        Transapical septal myectomy for hypertrophic cardiomyopathy with midventricular obstruction.
        Ann Thorac Surg. 2021; 111: 836-844