Abstract 16124: Implantable Cardioverter Defibrillators in Patients With Hereditary Transthyretin Cardiac Amyloidosis

Introduction: Implantable cardioverter defibrillator (ICD) implantation in patients with cardiac amyloidosis is controversial, with no clear guidelines for clinical decision-making.. The role of ICD implantation in hereditary Transthyretin Amyloid Cardiomyopathy (hATTR-CM) is unclear. We sought to establish the burden of ventricular arrhythmias and the outcomes of ICD implantation in a single center cohort of hATTR-CM patients. Methods: A total of 69 patients with a confirmed diagnosis of hATTR-CM from genetic testing, and technetium pyrophosphate (PYP) scanning, or endomyocardial biopsy underwent retrospective chart review for demographic, clinical, and arrhythmia data. Results: Seventy-four percent of the cohort was male, with a mean age at diagnosis of 68 (SD=18 years). Sixty-five patients (94.2%) patients were African-American; all of whom carried the Valine 122 Isoleucine mutation. Most had systolic heart failure (New York Heart Association Staging II [18, 26%] and III [40, 58.8%]); 37 (54%) patients had an LVEF ≤ 35%. Thirty-six (52.2%) patients had documented episodes of non-sustained ventricular tachycardia (NSVT), three (4.3%) with ventricular tachycardia (VT), and one (1.5%) with ventricular fibrillation (VF). A total of 15 (21.7%) patients had ICDs placed for prevention of sudden cardiac death in the setting of low LVEF (EF Conclusions: In a cohort of patients with hATTR-CM, we observed a high incidence of NSVT, yet only 41% of patients with severe systolic heart failure had ICDs implanted. A high rate of successful defibrillation and no inappropriate ICD shocks were noted, suggesting that ICDs should be strongly considered, and may be underutilized, in patients with systolic heart failure and/or arrhythmias in the setting of hATTR-CM.