General and disease-specific psychosocial adjustment in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy with implantable cardioverter defibrillators: A large cohort study

Cynthia A. James, Crystal Tichnell, Brittney Murray, Amy Daly, Samuel F. Sears, Hugh Calkins

Research output: Contribution to journalArticle


Background-Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized by frequent life-threatening ventricular arrhythmias, diagnosed on average in the teens to mid-50s and commonly treated by implantable cardioverter defibrillators (ICDs). As younger age and high frequency of ICD discharges are risk factors for difficulties in psychosocial adjustment, we developed a study to assess psychosocial adjustment among patients with ARVD/C and to determine risk factors for poor adjustment in this high-risk population. Methods and Results-Eighty-six adults enrolled in the Johns Hopkins ARVD Registry (38 male; mean age, 45.4=12.9 years), with an ICD in place for a median 3.2 years (range, 0.2 to 20.1 years), completed a set of questionnaires measuring ICD-specific anxiety (Florida Shock Anxiety Scale), device acceptance (Florida Patient Acceptance Survey), anxiety and depression (Hospital Anxiety and Depression Scale), and functional capacity (Duke Activity Status Index). Although overall device acceptance (Florida Patient Acceptance Survey mean, 76.7±15.3) was normative, patients with ARVD/C had substantially elevated body image concerns (Florida Patient Acceptance Survey subscale mean, 17.9±23.5) and device-related distress (subscale mean, 26.5±19.2), particularly among younger patients (P<0.01). Patients with ARVD/C had elevated ICD-specific (Florida Shock Anxiety Scale mean, 22.9±7.8) and general clinical anxiety (Hospital Anxiety and Depression Scale anxiety subscale mean, 6.2±3.9). Device-specific anxiety (Florida Shock Anxiety Scale) was predicted by younger age (P<0.0001), poorer functional capacity (P=0.016), having an ICD shock (P=0.003), and shorter time since ICD implant (P=0.007). Participants with poor device adjustment had an increased likelihood of clinically significant anxiety (P=0.006) and depression (P=0.008). Conclusions-Patients with ARVD/C are at elevated risk for anxiety, and young patients face challenges with device acceptance. Risk factors for poor device adjustment may be used clinically to identify patients at high-risk of psychological distress.

Original languageEnglish (US)
Pages (from-to)18-24
Number of pages7
JournalCirculation: Cardiovascular Genetics
Issue number1
StatePublished - Feb 1 2012



  • Cardiomyopathy
  • Defibrillation
  • Genetics

ASJC Scopus subject areas

  • Genetics
  • Cardiology and Cardiovascular Medicine
  • Genetics(clinical)

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