• Paolo Milani, Francesca Fabris, Roberta Mussinelli, Giuseppe Damiano Sanna, Marco Basset, Pietro Benvenuti, Claudia Bellofiore, Martina Nanci, Mario Nuvolone, Andrea Attanasio, Gianluigi Guida, Stefano Perlini, Andrea Foli, Giampaolo Merlini, and Giovanni Palladini.
• Department of Molecular Medicine, University of Pavia, Pavia, Italy.
• Amyloid. 2024 Sep 1; 31 (3): 220225220-225.

BackgroundEarly identification of immunoglobulin light-chain amyloidosis (AL) is crucial due to its rapid progression. Monoclonal light-chain (M-LC) testing is the first step in the diagnostic workup for patients with suspected cardiac amyloidosis (CA). We aimed to determine whether the time interval between the first CA suspicion and M-LC testing can be related to AL amyloidosis survival outcomes.MethodsAll patients (n = 94) with isolated cardiac AL amyloidosis diagnosed at our center between 2016 and 2020 were included. Those with pre-existing known monoclonal protein (monoclonal gammopathy of undetermined significance or smoldering multiple myeloma) were excluded. Time intervals to diagnostic tests and diagnosis were calculated and assessed for their survival prediction ability.ResultsThe time interval between first CA suspicion (on echocardiography) and M-LC testing correlated with early mortality, and the best cutoff predicting survival, was 6 weeks. The 26 patients (∼28% of entire cohort) who underwent M-LC-studies >6 weeks after first suspicion more frequently presented Mayo stage IIIb (65% vs. 35%, p = .008), showing poorer overall survival than those (n = 68, 72%) referred for early M-LC studies (median 3 vs. 14 months, p = .039).ConclusionsMonoclonal protein testing should be the first-step in the diagnostic workup for patients with echocardiographic/other instrumental red flags raising CA suspicion.

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