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Journal Article Ahead of print 1 citation

PubMed: 42639666 DOI: 10.1161/CIRCULATIONAHA.125.078597

International Experience With Implantable Cardioverter Defibrillators for the Prevention of Sudden Death in High-Risk Patients With Hypertrophic Cardiomyopathy.

Circulation

Authors: Ethan J Rowin, Barry J Maron, Konstantinos C Siontis, Albree F Tower-Rader, Daniele Massera, Benjamin Koethe, Ozlem Bilen, Dermot Phelan, Ahran Arnold, Jagdeep S Mohal, Amanda M Varnava, Maria Alessandra Schiavo, Raffaello Ditaranto, Elena Biagini, Hisham Ahamed, Aparna Hari, Sofian Johar, Bee-Ngo Lau, Domenico Corrado, Jackson O'Neill, Chris Semsarian, Susan A Casey, Scott Sharkey, Jiri Bonaventura, Jakub Honek, Alice Krebsova, Marketa Adamova, Dawn McCrystal, Erica Scherer, Ashwin Pillai, Megan Scalzo, Abhishek Jaiswal, Jorge Alberto Lax, Ruxandra Jurcut, Hiroaki Kitaok, Marit Kristine Smedsrud, Amanda Kirshkaln-Leahy, Pietro Francia, Beatrice Musumeci, Tatevik Hovakimyan, Omnia Kamel, Magdi H Yacoub, Oddný Brattberg Gunnarsdóttir, Gunnar Thor Gunnarsson, Berglind Adalsteinsdottir, Enrique A Berrios Barcenas, Michael A Fifer, Steve R Ommen, Mark V Sherrid, Martin S Maron

Affiliations

  • Hypertrophic Cardiomyopathy Center, Lahey Hospital and Medical Center, Burlington, MA (E.J.R. B.J.M. A.K.-L. M.S.M.).
  • Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN (K.C.S. S.R.O.).
  • Cardiology Division, Mass General Brigham, Boston, MA (A.F.T.-R. M.A.F.).
  • Hypertrophic Cardiomyopathy Program, Leon H. Charney Division of Cardiology, NYU Langone Health, New York, NY (D. Massera, M.V.S.).
  • Biostatistics, Epidemiology, and Research Design (BERD) Center, Tufts Medical Center, Boston, MA (B.K.).
  • Division of Cardiology, Department of Internal Medicine, Emory University, Atlanta, GA (O.B.).
  • Atrium Health, Sanger Heart & Vascular Institute, Charlotte, NC (D.P. E.S.).
  • National Heart and Lung Institute, Imperial College, London, UK (A.A. J.S.M. A.M.V.).
  • Cardiology Unit, Cardiac Thoracic and Vascular Department, IRCCS Azienda Ospedaliero- Universitaria di Bologna, Italy (M.A.S. R.D. E.B.).
  • European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (M.A.S. R.D. E.B.).
  • Amrita Hypertrophic Cardiomyopathy Center, Amrita Institute of Medical Sciences and Research, Kochi, India (H.A. A.H.).
  • Department of Cardiology, RIPAS Hospital, Brunei Darussalam (S.J. L.B.-N.).
  • Department of Cardiac, Thoracic and Vascular Sciences, University of Padua Medical School, Padova, Italy (D.C.).
  • Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia (J.O. C.S.).
  • Joseph F. Novogratz Family Heart Rhythm Science Center, Minneapolis Heart Institute Foundation, MN (S.A.C. S.S.).
  • Department of Cardiology, Motol University Hospital, Prague, Czech Republic (J.B. J.H.).
  • Department of Cardiology, Institute for Clinical and Experimental Medicine (IKEM), Prague, Czech Republic (A.K. M.A.).
  • Hartford HealthCare, Heart and Vascular Institute, CT (D. McCrystal, A.P. M.S. A.J.).
  • Division of Cardiology, Hospital Argerich, Buenos Aires, Argentina (J.A.L.).
  • Expert Center for Rare Genetic Cardiovascular Diseases, Emergency Institute for Cardiovascular Diseases, Bucharest, Romania (R.J.).
  • Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Japan (H.K.).
  • ProCardio Center for Research Based Innovation, Department of Cardiology, Oslo University Hospital, Rikshospitalet, Norway (M.K.S.).
  • Department of Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Italy (P.F. B.M.).
  • Department of Cardiac Arrhythmology, Nork-Marash Medical Center, Yerevan, Armenia (T.H.).
  • Aswan Heart Center, Egypt (O.K. M.H.Y.).
  • Department of Medicine, Akureyri Hospital, Iceland (O.B.G. G.T.G.).
  • Faculty of Medicine, University of Iceland, Reykjavik (O.B.G. G.T.G.).
  • Division of Cardiology, Landspitali-The National University Hospital of Iceland, Reykjavik (B.A.).
  • Instituto Nacional de Cardiologia Ignacio Chavex, Mexico City, Mexico (E.A.B.B.).

Abstract

BACKGROUND

Implanted cardioverter-defibrillators (ICDs) have been used in patients with hypertrophic cardiomyopathy (HCM) to prevent sudden death, and have proven lifesaving for many patients. However, experience with ICD therapy has largely been derived from relatively small HCM cohorts confined to specific countries or regions of the world. Therefore, we sought to determine the effectiveness of ICDs in preventing sudden death due to life-threatening ventricular arrhythmias in a large international multicenter HCM population.

METHODS

Databases from 25 HCM centers (8 in the United States, 9 in Europe, 4 in Asia, and 1 each in Australia, Africa, Mexico, and South America) were retrospectively interrogated to identify consecutive patients with HCM with ICDs (1992 to 2024) followed for 7±6 years (up to 32 years) for clinical outcomes.

RESULTS

A total of 3387 patients were identified (63% men). They had a mean left ventricular thickness of 22±7 mm. The participants had received ICDs at a mean age of 47±17 years. Over follow-up, 550 patients (16%) experienced ≥1 appropriate ICD therapy (2.6%/y), including 86 of the 247 implanted for secondary prevention (35% [6.4%/y]) and 464 of the 3140 implanted for primary prevention (15% [2.2%/y]). Appropriate therapy occurred in the 464 primary prevention patients at a mean age of 49±17 years, with a median time to first appropriate therapy of 4 years after ICD implantation; 16% of these received their first appropriate therapy ≥10 years after implantation, and 47% experienced multiple interventions. Independent predictors of appropriate ICD therapy included unexplained syncope, left ventricular apical aneurysms, left ventricular systolic dysfunction, and nonsustained ventricular tachycardia on ambulatory monitoring. Of the 3140 primary prevention patients, 2946 survived (94%) and 194 died (6%) (0.8%/y), including 68 due to HCM (0.3%/y), predominantly of end-stage heart failure (n=43) or stroke (n=9). In contrast, 11 patients (0.4%) died suddenly, with device failures occurring in 2.4% of those with life-threatening ventricular tachyarrhythmias. Survival free from HCM-related mortality at 10 and 20 years was 97% and 91%, respectively.

CONCLUSIONS

In this international multicenter study, the largest to date, including >3300 consecutive patients with HCM and ICDs, device therapy terminated potentially lethal ventricular tachyarrhythmias in 1 of 6 patients, with low HCM-related mortality rates for patients with ICDs. These novel data demonstrate the effectiveness of the ICD initiative, which has probably favorably altered the natural history of many patients with HCM worldwide.

MeSH

Tachycardia, Ventricular, Death, Sudden, Mortality, Cardiovascular System, Cardiomyopathy, Hypertrophic, Primary Prevention, Therapeutics, Defibrillators, Implantable, Blood Circulation, Cardiomyopathies, Defibrillators