부산대학교 도서관

Background:ATTR cardiomyopathy is classically described in patients with heart failure with preserved ejection fraction (HFpEF). We sought to understand the prevalence, characteristics, and survival of patients at the time of diagnosis, had heart failure with HFpEF (≥50%), HfmrEF: (41-49%), HFrEF ( ≤40%).Methods:A retrospective institutional amyloid registry study of 585 patients diagnosed with wild-type (WT) or variant ATTR-CM from 2011-2020. Cox proportional hazards models were used to assess probability of transplant-free survival.Results:In our cohort, (median age 77 [71,83], 84% male, 25% African-American (AA), median eGFR 58 [46,73], 28% had HFrEF (n= 165), 17% HFmrEF (n=97), and 55% with HFpEF (n=323). After adjusting for Age, sex, race, NT-proBNP, and eGFR, HFrEF patients had worse survival than those with HFpEF (HR, 0.47[95% CI, 0.33-0.68, p<0.001]) and HmrEF (HR, 0.47[95% CI, 0.29-0.77, p=0.002]) (Fig 1a)AA patients had higher rates of HTN, DM, and variant TTR (72% vs. 9%, p<0.001) as well as lower rates of CAD compared to Caucasian patients. AA patients were predominantly V122I variant (n=109/146, 74%) and more likely to present with HFrEF (n=60/146, 41%) than Caucasian patients (n=102/440, 23%), p<0.001. AA WT (n=18/41, 44%) patients were also more likely to present with HFrEF than Caucasian WT (n=98/400, 25%), p=0.017 (Fig 1b).AA variant (43/108,41%) were more likely to present as HFrEF than Caucasian variants (4/41, 9.8%), p=0.001.Conclusions:Forty-five percent of patients diagnosed with ATTR-CM presented with LVEF ≤50% at the time of diagnosis, confirming that clinical suspicion for diagnosis of ATTR-CM should extend beyond HFpEF presentation. Reduced LVEF is an independent prognostic factor in patients with ATTR-CM. Racial disparities in the LVEF at presentation are present in both variant and WT ATTR. Further investigation into co-morbid, genetic, and racial factors associated with the presenting phenotype of ATTR amyloid is needed.