Psychosocial Implications of Living with Catecholaminergic Polymorphic Ventricular Tachycardia in Adulthood

Abstract

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited arrhythmogenic disease with a high risk of sudden cardiac death. The impact on health-related quality of life (HR-QoL) and psychosocial outcomes is not known. We sought to provide the first description of HR-QoL and psychosocial wellbeing of adults with CPVT, parents of affected children and at-risk relatives. Participants were recruited through the Australian Genetic Heart Disease Registry and invited to complete a cross-sectional survey comprising a number of validated scales and open-ended questions. Thirty-five participants completed surveys (response rate 65%), including 19 with CPVT, 10 unaffected parents of a child with CPVT, and 7 at-risk relatives (one participant considered patient and parent). Young patients <40 years were significantly more likely to report anxiety (p = 0.04), depression (p = 0.03) and posttraumatic stress symptoms (p = 0.02) compared to older CPVT patients. Further, young patients with an implantable cardioverter defibrillator (ICD) reported significantly worse device-related distress (p = 0.04) and shock anxiety (p = 0.003). Patients with a genetic diagnosis had worse psychological adaptation than those patients without a gene result. Parents perceived their affected children to have poor quality of life across all subdomains compared to healthy age-matched children, however quality of life of parents and at-risk relatives was comparable to population norms. Ongoing psychosocial care is required for young people with CPVT. Those with an ICD and/or undergoing genetic testing may require additional support. The challenges of CPVT management should extend beyond the clinical and genetic aspects of care to incorporate greater psychosocial support, and further reinforces the need for a multidisciplinary approach to care.

Measuring ICD shock anxiety: Status update on the Florida Shock Anxiety Scale after over a decade of use

“…27 Patients with catecholaminergic polymorphic ventricular tachycardia-a specific genetic cardiovascular condition commonly affecting younger patients-who are ages 40 and younger experienced lower device acceptance and greater shock anxiety on FSAS than patients older than 40 years. 15 Patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy, also commonly affecting younger-aged individuals, indicated higher mean FSAS scores (M = 22.9) than previously reported normative. 28,29 Racial/ethnic differences may also be present, as African American ICD patients reported higher levels of shock anxiety than Caucasian ICD patients.…”

“…Spouses, mainly women, endorsed higher levels of shock anxiety on FSAS than ICD patients, highlighting the fear of disruption even for spouses . Patients with catecholaminergic polymorphic ventricular tachycardia–a specific genetic cardiovascular condition commonly affecting younger patients–who are ages 40 and younger experienced lower device acceptance and greater shock anxiety on FSAS than patients older than 40 years . Patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy, also commonly affecting younger‐aged individuals, indicated higher mean FSAS scores (M = 22.9) than previously reported normative .…”

“…Population differences in FSAS scores reveal subgroups who may be in greater need of psychological intervention-notably, women and younger-aged recipients. 5,12,15,16,21,[27][28][29] Individuals with poor ICDrelated knowledge also may be appropriate targets of intervention given the correlation between low device education and high FSAS score. 19…”

“…FSAS has demonstrated good discriminant and convergent validity as reflected by negative correlations with general emotional wellbeing, general health, and quality of life and positive correlations with quantity and disruptiveness of ICD shocks. 9 Further, the FSAS has demonstrated utility in diverse, international populations including Chinese, Dutch, Turkish, Australian, and Japanese ICD patient samples [12][13][14][15][16] as well as good validity and reliability (Cronbach = 0.87) in Turkish populations. 17 The average rating on FSAS items is 1.5, between "not at all" and "rarely.…”

Psychological Issues in Managing Families with Inherited Cardiovascular Diseases

“…Among those with ARVC, having an ICD shock, younger age, poor functional capacity, and shorter time since implant all predicted greater device-specific distress, and poor adjustment was associated with greater reported anxiety and depressive symptoms (James et al 2012). In a cohort of patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) who had an ICD, those who were younger were more likely to report device-related distress and shock anxiety and even greater body image concerns than the ARVC cohort described above (Richardson et al 2018).…”