Changing Patterns of Diagnosis and Survival in Transthyretin Cardiac Amyloidosis: A Multicenter Cohort Study

作者：Brett W. Sperry, Ahmad Masri, Prem Soman, A. Ejaz, Priyanka Bhattacharya, Brent Medoff, Pranav Chandrashekar, Lauren Ives, Alfonsina Mirabal Santos, Sergio Teruya, Yanjun Wu, Yuanzi Zhao, Xiaofeng Wang, Mathew S. Maurer, Mazen Hanna · 发表于：Journal of the American Heart Association · 年份：2025 · DOI：10.1161/jaha.125.042530 · 被引用次数：3 · 研究领域：Amyloidosis: Diagnosis, Treatment, Outcomes、Parathyroid Disorders and Treatments、Pericarditis and Cardiac Tamponade

Background Diagnostic rates of transthyretin cardiac amyloidosis have increased due to growing awareness, noninvasive diagnoses, and treatments that improve morbidity and mortality. We sought to investigate real‐world trends in baseline characteristics and survival in the United States. Methods Patient‐level data at 5 cardiac amyloid referral centers were used, and mortality (defined as death or surgical heart failure therapies) was compared by eras (pre‐2010, 2010–2013, 2014–2017, and 2018–2021) and National Amyloidosis Center (NAC) stage. Results Among 1165 subjects, diagnosis of transthyretin cardiac amyloidosis in a more recent era was associated with older age, white race, wild‐type disease, and tafamidis treatment ( P <0.001). NAC stage was similar ( P =0.866) while Columbia stage was lower ( P =0.006) in recent eras, driven by lower New York Heart Association class and loop diuretic dose. Over a median follow‐up of 44.5 (interquartile range, 21–60) months, 630 subjects died, and diagnosis in a more recent era was associated with a lower mortality. Tafamidis was protective (hazard ratio [HR], 0.71; P =0.002), and NAC stage >1 portended an increased risk of death (stage 2: HR, 2.00; P <0.001; stage 3: HR, 4.02; P <0.001). There were interactions between tafamidis and both NAC stage and age, suggesting that the beneficial effects of tafamidis were attenuated at higher NAC stages (stage 2: P =0.041; stage 3: P =0.012) and older age ( P =0.001). Conclusions In t...