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.

Early Diagnosis and Outcome in Patients With Wild-Type Transthyretin Cardiac Amyloidosis / Fumagalli, Carlo; Zampieri, Mattia; Perfetto, Federico; Zocchi, Chiara; Maurizi, Niccolò; Tassetti, Luigi; Ungar, Andrea; Gabriele, Martina; Nardi, Giulia; Del Monaco, Guido; Baldini, Katia; Tomberli, Alessia; Tomberli, Benedetta; Marchionni, Niccolò; Di Mario, Carlo; Olivotto, Iacopo; Cappelli, Francesco. - In: MAYO CLINIC PROCEEDINGS. - ISSN 0025-6196. - ELETTRONICO. - 96:(2021), pp. 2185-2191. [10.1016/j.mayocp.2021.04.021]

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

Fumagalli, Carlo;Zampieri, Mattia;Perfetto, Federico;Zocchi, Chiara;Maurizi, Niccolò;Tassetti, Luigi;Ungar, Andrea;Gabriele, Martina;Nardi, Giulia;Del Monaco, Guido;Tomberli, Alessia;Tomberli, Benedetta;Marchionni, Niccolò;Di Mario, Carlo;Olivotto, Iacopo;Cappelli, Francesco
2021

Abstract

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.
96
2185
2191
Goal 3: Good health and well-being
Fumagalli, Carlo; Zampieri, Mattia; Perfetto, Federico; Zocchi, Chiara; Maurizi, Niccolò; Tassetti, Luigi; Ungar, Andrea; Gabriele, Martina; Nardi, Giulia; Del Monaco, Guido; Baldini, Katia; Tomberli, Alessia; Tomberli, Benedetta; Marchionni, Niccolò; Di Mario, Carlo; Olivotto, Iacopo; Cappelli, Francesco
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2158/1242579
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