Abstract 17122: Role of a Dedicated Program in Early Recognition and Treatment of Transthyretin Cardiac Amyloidosis

Introduction: Clinical recognition of Transthyretin Cardiac amyloidosis(ATTR-CA) is rising. Lack of treatment options with disease reversing agents makes early diagnosis and institution of treatment crucial to clinical improvement. A dedicated ATTR-CA program might facilitate such goals. Aim: We report our early experience with initiation of a cardiac amyloidosis program on diagnostic and therapeutic intervals and clinical outcomes.

Methods: Retrospective review of patients who are being treated with Tafamidis in our cardiac amyloidosis management program(CAMP) from March 2019 through May 2022

Results: 55 patients with ATTR-CA (47-male) aged 80±9 years; 45 with clinical HFpEF and NYHA class 2-3 limitations and at least one prior hospitalization related to heart failure(HF) were seen in our CAMP(Figure). Initial mean LVEF was 50±12%. 42 had LVH and 11 had biventricular hypertrophy. Mean LV septal & posterior wall thickness were 1.54±0.37cm & 1.44±0.29cm respectively. The median interval between onset of HF and diagnosis, and from diagnosis to initiation of Tafamidis were 4 months (Inter Quartile Range[IQR] 1-9) and 1 month (IQR 0-3) respectively. For HF management, 35 patients were maintained on loop diuretics, 34 on mineralocorticoid receptor antagonists, 21 on SGLT2 inhibitors, 12 on beta blockers, 7 on ARNIs, 6 on ACEI/ARBs, and 1 on digoxin. Medications were tolerated well with no reported adverse effects. At follow-up, 16 patients reported NYHA class 1, 15 class 2, 14 class 3 limitations. 3 patients required PCI after enrollment in the program. Follow-up echocardiograms were available for 36 patients with a mean EF of 50±11%.

Conclusions: Given the irreversible nature of ATTR-CA, early diagnosis and initiation of treatment is vital to improving long-term outcomes. Instituting a dedicated CAMP results in shorter diagnostic and therapeutic lag times than those historically described.