Staging classification of transthyretin cardiac amyloidosis based on the extent of cardiac damage at echocardiography: a large multicentric Registry.
D Tomasoni, G Bonfioli, A Aimo, P Milani, A Cipriani, M Senni, M Serenelli, G Foti, M Cameli, G M De Ferrari, G Limongelli, M Emdin, G Sinagra, G Palladini, M MetraAbstract
Background
Current risk stratification systems in patients with transthyretin cardiac amyloidosis (ATTR-CA) rely mainly on circulating biomarkers.
Purpose
The present study aimed to develop an echocardiography-based staging system for ATTR-CA.
Methods
Data were obtained from the CROSS-ATTR study, a retrospective, multicentre investigation including patients diagnosed with ATTR-CA between 2016 and 2024 across 12 Italian high-volume centers. Patients were classified into three echocardiographic stages according to the extent of cardiac involvement: stage 1, isolated left ventricular involvement; stage 2, left atrial and/or mitral valve impairment; and stage 3, right-sided impairment (Figure 1). The stages were mutually exclusive so that a patient was classified according to his most severe stage.
Results
A total of 591 patients were included (median age 79, IQR 75-83, 88.8% males; 95.6% with wild-type form). Sixty (10.1%) patients were classified as stage 1, 329 (55.7%) as stage 2 and 202 (32.2%) as stage 3. Patients in stage 3 exhibited more severe heart failure (HF) as compared with those in earlier stages. Over a median follow-up of 819 days (IQR 518–1215), rates of all-cause mortality (0%, 5%, and 13% in stages 1, 2, and 3, respectively) and the composite outcome of all-cause death or cardiovascular hospitalization (7%, 22%, and 43%, respectively) increased significantly across stages (Figure 2). At multivariable analysis, after adjustment for age, sex, NAC staging, genotype, and disease modifying treatments, the echo-staging system remained an independent predictor of all-cause mortality (adjusted HR [HRadj] 2.52, 95% CI 1.40-4.55, p-value 0.002 per each stage increase), and of the composite outcome (HRadj 2.15, 95% CI 1.62-2.85, p-value <0.001).
Conclusions
This novel and simple echocardiography-based staging system is strongly associated with clinical outcomes and may provide incremental prognostic value in patients with ATTR-CA.For image description, please refer to the figure legend and surrounding text.For image description, please refer to the figure legend and surrounding text.