Barry J Maron1, Susan A Casey2, Iacopo Olivotto2, Mark V Sherrid2, Christopher Semsarian2, Camillo Autore2, Aisha Ahmed2, Giuseppe Boriani2, Pietro Francia2, Stephen L Winters2, Michael Giudici2, Anna Koulova2, Ross Garberich2, Ethan J Rowin2, Samuel F Sears2, Martin S Maron2, Paolo Spirito2. 1. HCM Institute, Division of Cardiology, Tufts Medical Center, Boston, MA (B.J.M., E.J.R., M.S.M.); Department of Psychology & Cardiovascular Sciences, East Carolina University, Greenville, NC (S.F.S.); Royal Prince Alfred Hospital and Centenary Institute, University of Sydney, Australia (C.S.); Minneapolis Heart Institute Foundation, MN (S.A.C., A.A., R.G.); University of Iowa Hospitals, Iowa City (M.G.); Division of Cardiology, Department of Clinical & Molecular Medicine, Sant' Andrea Hospital, University of Sapienza, Rome (C.A., P.F.); Cardiology Division, Department of Diagnostics, Clinical and Public Health Medicine, University of Modena, and Reggio Emilia, Policlinico di Modena, Italy (G.B.); Policlinico di Monza, Italy (P.S.); St. Lukes - Roosevelt Hospital Center, NYU Lan gone Medical Center (M.V.S., A.K.); Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy (I.O.); Atlantic Health Systems, Morristown Memorial Hospital and Medical Center, NJ (S.L.W.). barrymaron1@gmail.com. 2. HCM Institute, Division of Cardiology, Tufts Medical Center, Boston, MA (B.J.M., E.J.R., M.S.M.); Department of Psychology & Cardiovascular Sciences, East Carolina University, Greenville, NC (S.F.S.); Royal Prince Alfred Hospital and Centenary Institute, University of Sydney, Australia (C.S.); Minneapolis Heart Institute Foundation, MN (S.A.C., A.A., R.G.); University of Iowa Hospitals, Iowa City (M.G.); Division of Cardiology, Department of Clinical & Molecular Medicine, Sant' Andrea Hospital, University of Sapienza, Rome (C.A., P.F.); Cardiology Division, Department of Diagnostics, Clinical and Public Health Medicine, University of Modena, and Reggio Emilia, Policlinico di Modena, Italy (G.B.); Policlinico di Monza, Italy (P.S.); St. Lukes - Roosevelt Hospital Center, NYU Lan gone Medical Center (M.V.S., A.K.); Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy (I.O.); Atlantic Health Systems, Morristown Memorial Hospital and Medical Center, NJ (S.L.W.).
Abstract
BACKGROUND: High-risk patients with hypertrophic cardiomyopathy (HCM) are identified by contemporary risk stratification and effectively treated with implantable cardioverter-defibrillators (ICDs). However, long-term HCM clinical course after ICD therapy for ventricular tachyarrhythmias is incompletely understood. METHODS AND RESULTS: Cohort of 486 high-risk HCM patients with ICDs was assembled from 8 international centers. Clinical course and device interventions were addressed, and survey questionnaires assessed patient anxiety level and psychological well-being related to ICD therapy. Of 486 patients, 94 (19%) experienced appropriate ICD interventions terminating ventricular tachycardia/ventricular fibrillation, 3.7% per year for primary prevention, over 6.4±4.7 years. Of 94 patients, 87 were asymptomatic or only mildly symptomatic at the time of appropriate ICD interventions; 74 of these 87 (85%) remained in classes I/II without significant change in clinical status over the subsequent 5.9±4.9 years (up to 22). Among the 94 patients, there was one sudden death (caused by device failure; 1.1%); 3 patients died from other HCM-related processes unrelated to arrhythmic risk (eg, end-stage heart failure). Post-ICD intervention, freedom from HCM mortality was 100%, 97%, and 92% at 1, 5, and 10 years, distinctly lower than in ischemic or nonischemic cardiomyopathy ICD trials. HCM patients with ICD interventions reported heightened anxiety in expectation of future shocks, but with intact general psychological well-being and quality of life. CONCLUSIONS: In HCM, unlike ischemic heart disease, prevention of sudden death with ICD therapy is unassociated with significant increase in cardiovascular morbidity or mortality, or transformation to heart failure deterioration. ICD therapy does not substantially impair overall psychological and physical well-being.
BACKGROUND: High-risk patients with hypertrophic cardiomyopathy (HCM) are identified by contemporary risk stratification and effectively treated with implantable cardioverter-defibrillators (ICDs). However, long-term HCM clinical course after ICD therapy for ventricular tachyarrhythmias is incompletely understood. METHODS AND RESULTS: Cohort of 486 high-risk HCM patients with ICDs was assembled from 8 international centers. Clinical course and device interventions were addressed, and survey questionnaires assessed patientanxiety level and psychological well-being related to ICD therapy. Of 486 patients, 94 (19%) experienced appropriate ICD interventions terminating ventricular tachycardia/ventricular fibrillation, 3.7% per year for primary prevention, over 6.4±4.7 years. Of 94 patients, 87 were asymptomatic or only mildly symptomatic at the time of appropriate ICD interventions; 74 of these 87 (85%) remained in classes I/II without significant change in clinical status over the subsequent 5.9±4.9 years (up to 22). Among the 94 patients, there was one sudden death (caused by device failure; 1.1%); 3 patients died from other HCM-related processes unrelated to arrhythmic risk (eg, end-stage heart failure). Post-ICD intervention, freedom from HCM mortality was 100%, 97%, and 92% at 1, 5, and 10 years, distinctly lower than in ischemic or nonischemic cardiomyopathy ICD trials. HCM patients with ICD interventions reported heightened anxiety in expectation of future shocks, but with intact general psychological well-being and quality of life. CONCLUSIONS: In HCM, unlike ischemic heart disease, prevention of sudden death with ICD therapy is unassociated with significant increase in cardiovascular morbidity or mortality, or transformation to heart failure deterioration. ICD therapy does not substantially impair overall psychological and physical well-being.
Authors: Martin S Maron; Ethan J Rowin; Benjamin S Wessler; Paula J Mooney; Amber Fatima; Parth Patel; Benjamin C Koethe; Mikhail Romashko; Mark S Link; Barry J Maron Journal: JAMA Cardiol Date: 2019-07-01 Impact factor: 14.676
Authors: Barry J Maron; Martin S Maron; Mathew S Maurer; Ethan J Rowin; Bradley A Maron; Nazzareno Galiè Journal: J Am Heart Assoc Date: 2021-10-08 Impact factor: 5.501
Authors: Lieke M van den Heuvel; Tanya Sarina; Joanna Sweeting; Laura Yeates; Kezia Bates; Catherine Spinks; Catherine O'Donnell; Samuel F Sears; Kevin McGeechan; Christopher Semsarian; Jodie Ingles Journal: Heart Rhythm O2 Date: 2022-02-08
Authors: Anastasia Miron; Myriam Lafreniere-Roula; Chun-Po Steve Fan; Katey R Armstrong; Andreea Dragulescu; Tanya Papaz; Cedric Manlhiot; Beth Kaufman; Ryan J Butts; Letizia Gardin; Elizabeth A Stephenson; Taylor S Howard; Pete F Aziz; Seshadri Balaji; Virginie Beauséjour Ladouceur; Lee N Benson; Steven D Colan; Justin Godown; Heather T Henderson; Jodie Ingles; Aamir Jeewa; John L Jefferies; Ashwin K Lal; Jacob Mathew; Emilie Jean-St-Michel; Michelle Michels; Stephanie J Nakano; Iacopo Olivotto; John J Parent; Alexandre C Pereira; Christopher Semsarian; Robert D Whitehill; Samuel G Wittekind; Mark W Russell; Jennifer Conway; Marc E Richmond; Chet Villa; Robert G Weintraub; Joseph W Rossano; Paul F Kantor; Carolyn Y Ho; Seema Mital Journal: Circulation Date: 2020-05-18 Impact factor: 29.690
Authors: Razvan Capota; Sebastian Militaru; Alin Alexandru Ionescu; Monica Rosca; Cristian Baicus; Bogdan Alexandru Popescu; Ruxandra Jurcut Journal: Health Qual Life Outcomes Date: 2020-10-30 Impact factor: 3.186