138 - Increasing Frequency of Diagnosis of Transthyretin Cardiac Amyloidosis and Changing Survival Over Time

Abstract

Background: Cardiac amyloidosis is a group of rare diseases resulting from extracellular deposition of amyloid protein, principally due to light chain (AL) amyloid or acquired or hereditary changes in TTR protein. Transthyretin cardiac amyloidosis (TTR) is increasingly recognized as an important cause of heart failure with preserved ejection fraction; the frequency of diagnosis has noted to be increasing. We aimed to investigate the changing phenotype and survival over time of patients diagnosed with cardiac amyloidosis at the Center for Advanced Cardiac Care (CACC). Methods: A retrospective study of 314 patients presenting to CACC between 1997–2016 with diagnosed cardiac amyloid was conducted. 29 patients underwent heart transplant and were excluded from further analyses, 151 TTR and 134 AL cardiac amyloidosis patients were included. Patients were divided into equal tertiles of 95 patients based on date of diagnosis. The first third were diagnosed between 1997 and 2010, the next third between 2010 and 2013 and the remaining third were diagnosed between 2013 and 2016. Baseline characteristics and Kaplan Meir survival curves were performed for each tertile, subdivided by amyloid phenotype. Results: The frequency of diagnosis of TTR cardiac amyloid increased overtime (Fig. 1, panel A). In Kaplan Meir analysis, survival of TTR amyloid was significantly improved over time (Fig. 1, panel B, Log-rank P = .024). For TTR patients, later tertile diagnosed was associated with significantly lower BNP, creatinine and LA size at baseline (P < .05) and the greater use of technetium 99m pyrophosphate (PYP) cardiac imaging rather than biopsy for diagnosis (P < .05). Conclusions: Over time, TTR cardiac amyloidosis is seen with increased frequency compared to AL amyloid. Survival for TTR amyloid is significantly increased in patients diagnosed more recently and at earlier presentation perhaps in part due to PYP scanning.