Clinical Course and Quality of Life in High-Risk Patients With Hypertrophic Cardiomyopathy and Implantable Cardioverter-Defibrillators

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.

Psychological Issues in Managing Families with Inherited Cardiovascular Diseases

“…For those who choose to receive an ICD, overwhelmingly the psychological and social outcomes are favorable (Sweeting et al 2017;Maron et al 2018). Yet, there are certain patients who do not fare as well and would benefit from psychological counseling.…”

Can positron emission tomography help stratify the risk of sudden cardiac death in patients with hypertrophic cardiomyopathy?

“…6 The only established therapy to prevent sudden cardiac death in patients with HCM is the implantable cardioverter-defibrillator (ICD). [7][8][9] The generally accepted indications for prevention of SCD with an ICD in HCM are based on recognized risk factors, including a prior history of ventricular fibrillation or sustained ventricular tachycardia, maximum LV wall thickness C 30 mm, 10 SCD in a first degree relative with HCM, 11 unexplained syncope within the past 6 months, 12 nonsustained VT (especially if [ 150 bpm), 13 or an abnormal blood pressure response to exercise (failure to increase systolic pressure [ 20 mm Hg). 1,2,6,7,9,11,14 The risk of lethal arrhythmic events is higher for patients presenting with HCM in childhood, 5 those having Troponin I or T mutations, 5 and those with higher serum BNP concentrations.…”

“…4,6 The prevention of SCD is made more challenging by the fact that living with an ICD can be a significant burden to patients, including the risk of inappropriate shocks, lead malfunction, and requirement for pulse generator replacement. 8 Because of this, any technique that can reduce the need for ICD implantation in patients who are truly at low risk of SCD would be of high clinical value. 18 A welcome addition to risk assessment for patients with HCM has been the identification of late gadolinium enhancement (LGE) on cardiac MRI as an independent predictor of SCD.…”

Does septal myectomy reduce risk of sudden cardiac death in patients with hypertrophic cardiomyopathy?

“…Hartzell V. Schaff, MD, and Anita Nguyen, MBBS Arguably, the 2 most important developments in the clinical management of patients with hypertrophic cardiomyopathy (HCM) are the understanding that left ventricular outflow tract (LVOT) obstruction is present in 60% to 70% of symptomatic patients and that implantable cardioverter defibrillators (ICDs) can dramatically reduce risk of sudden cardiac death (SCD) in appropriately selected patients. [1][2][3] The challenge in preventing SCD is identification of patients at increased risk of fatal arrhythmias to justify the cost and potential morbidity of device implantation. 3,4 Although there is some evidence that LVOT obstruction may be a risk factor for SCD, 5,6 this is not universally accepted, and the 2011 American Heart Association/ American College of Cardiology guidelines state only that marked LVOT obstruction might be a potential SCD risk modifier when estimated risk remains borderline after documentation of conventional predictive factors.…”

“…[1][2][3] The challenge in preventing SCD is identification of patients at increased risk of fatal arrhythmias to justify the cost and potential morbidity of device implantation. 3,4 Although there is some evidence that LVOT obstruction may be a risk factor for SCD, 5,6 this is not universally accepted, and the 2011 American Heart Association/ American College of Cardiology guidelines state only that marked LVOT obstruction might be a potential SCD risk modifier when estimated risk remains borderline after documentation of conventional predictive factors. 7 In contrast, the European Society of Cardiology (ESC) guidelines list LVOT obstruction as a major clinical feature associated with SCD and include LVOT gradient in their risk model.…”