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Clinical Course and Quality of Life in High-Risk Patients With Hypertrophic Cardiomyopathy and Implantable
Barry J Maron1, Susan A Casey2, Iacopo Olivotto2
1HCM 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.
Insights
Implantable cardioverter-defibrillators (ICDs) effectively treat high-risk hypertrophic cardiomyopathy (HCM) patients. Long-term outcomes show ICDs prevent sudden death without worsening heart failure, though some patients experience anxiety.
Area of Science:
- Cardiology
- Electrophysiology
- Medical Devices
Background:
- Hypertrophic cardiomyopathy (HCM) poses a high risk for sudden cardiac death.
- Implantable cardioverter-defibrillators (ICDs) are used for risk stratification and treatment in high-risk HCM.
- Long-term outcomes and patient well-being after ICD therapy in HCM are not fully understood.
Purpose of the Study:
- To evaluate the long-term clinical course of high-risk HCM patients treated with ICDs.
- To assess device interventions, mortality, and psychological impact in HCM patients with ICDs.
Main Methods:
- A cohort of 486 high-risk HCM patients with ICDs was studied across 8 international centers.
- Clinical data and device interventions were analyzed.
- Patient-reported anxiety and psychological well-being were assessed via surveys.
Main Results:
- 19% of patients experienced appropriate ICD interventions for ventricular tachyarrhythmias.
- Most patients remained asymptomatic or mildly symptomatic post-intervention.
- HCM mortality freedom was 100% at 1 year, 97% at 5 years, and 92% at 10 years.
- Patients reported heightened anxiety but maintained general psychological well-being and quality of life.
Conclusions:
- ICD therapy in HCM effectively prevents sudden death without increasing cardiovascular morbidity or mortality.
- Unlike in ischemic heart disease, ICDs do not typically lead to heart failure deterioration in HCM.
- ICD therapy in HCM patients 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 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.
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