Amyloid light-chain (AL) amyloidosis is a rare condition characterized by the abnormal production of immunoglobulin light chain that misshape and form insoluble aggregates known as amyloid fibrils. Over time, these amyloid deposits can accumulate slowly, causing dysfunction in organs and tissues. [1]

Currently, the literature does not provide a complete understanding of the risk factors for the development of AL amyloidosis. Monoclonal gammopathy of undetermined significance (MGUS) and MM are the only well-documented precursor conditions. Specifically, 10–15% of MM patients will develop AL amyloidosis. [1]

Although all organs, except the brain, can be affected, systemic AL amyloidosis most commonly affects heart and kidneys. [1]

Despite the availability of newer effective therapies, approximately 30% of patients with AL amyloidosis still die within 1 year from diagnosis due to advanced, irreversible cardiac involvement at presentation. [[2], [3], [4], [5]]

Therefore, the latest consensus document emphasizes the early identification of cardiac involvement through a routine assessment of elevated Nt-pro-BNP levels and the detection of unexplained interventricular septal diameter >12 mm [3,4]. However, first-level echocardiographic parameters such as left ventricular ejection fraction and biatrial enlargement are frequently only slightly affected in patients who do not exhibit heart failure symptoms [[6], [7], [8], [9], [10]]. Additional echocardiographic parameter, such as relative apical sparing of myocardial longitudinal strain (RELAPS), left ventricular global longitudinal strain (LVGLS), and the AL-SCORE, has been suggested to enhance the accuracy of the ultrasound evaluation in cardiac amyloidosis [[11], [12], [13], [14]]. Based on these results, we aim to identify a better marker of cardiac amyloidosis, using advanced echocardiography, in plasma cell disorders (PCD) to improve diagnosis and the timing of available treatments.