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Early Diagnosis and Outcome in Patients With Wild-Type Transthyretin Cardiac Amyloidosis

2021; Elsevier BV; Volume: 96; Issue: 8 Linguagem: Inglês

10.1016/j.mayocp.2021.04.021

1942-5546

Carlo Fumagalli, Mattia Zampieri, Federico Perfetto, Chiara Zocchi, Niccolò Maurizi, Luigi Tassetti, Andrea Ungar, Martina Gabriele, Giulia Nardi, Guido Del Monaco, Katia Baldini, Alessia Tomberli, Benedetta Tomberli, Niccolò Marchionni, Carlo Di Mario, Iacopo Olivotto, Francesco Cappelli,

Parathyroid Disorders and Treatments

Whether diagnostic timing in transthyretin (TTR) cardiac amyloidosis (CA) predisposes patients to worse outcomes is unresolved. We aimed to describe the long-term association of diagnostic timing (time from first onset of symptoms consistent with CA leading to medical contact to definitive diagnosis) with mortality in patients with wild-type TTR-CA (ATTRwt-CA). Overall, we reviewed the medical records of 160 patients seen at a tertiary care amyloidosis unit from January 1, 2016, to January 1, 2020 (median [interquartile range] follow-up, 21 [10 to 34] months), and compared them by survival. Median diagnostic timing was 4 (2 to 12) months and was longer in nonsurvivors (9 [3 to 15] vs 3 [1 to 7] months; P<.001). Patients diagnosed 6 or more months after symptom onset had higher mortality, with a median survival of 30 months (95% CI, 22 to 37 months). On Cox multivariable analysis, timing was independently associated with all-cause mortality (hazard ratio per month increase, 1.049 [95% CI, 1.017 to 1.083]) together with age at diagnosis, disease stage, New York Heart Association class, and coronary artery disease. In conclusion, diagnostic timing of ATTRwt-CA is associated with mortality. Timely diagnosis is warranted whenever "red flags" are present.