Utility of Prognostic Staging Systems in Transthyretin Amyloidosis Cardiomyopathy in the Era of Available Disease-Modifying Treatment.
Prognostic staging systems in transthyretin amyloid (ATTR) cardiomyopathy were developed before the introduction of disease-modifying treatment. We therefore evaluated contemporary risk stratification according to different staging systems in patients with ATTR cardiomyopathy.
All patients with newly diagnosed ATTR cardiomyopathy and baseline biomarker measurements from 2019 to 2023 were included from Mayo Clinic, Rochester, MN. Prognostic outcomes were calculated with Kaplan-Meier analysis for 3e staging systems in the main analysis: the National Amyloidosis Centre (NAC) staging system; a modified Mayo staging system using hsTNT (high-sensitivity troponin T) cutoff >65 ng/L; the extended NAC model with a fourth stage (NT-proBNP [N-terminal pro-B-type natriuretic peptide] >10 000 ng/L).
The study population consisted of 441 patients with a median age of 76 years, 93% were men, 85% were initiated on tafamidis, and 94% had wild-type ATTR. Median follow-up was 3.4 years, and 109 deaths occurred during follow-up. The 3-year survival for the Mayo stages were 92.3% (88.7-95.9) for stage I, 71.6% (63.0-81.3) for stage II, and 43.5% (32.4-58.5) for stage III. For the NAC system, 3-year survival were 92.0% (89.0-95.7), 67.5% (59.0-77.3), and 47.0% (34.7-63.5) for stages I, II, and III, respectively. The 3-year survival by the extended NAC system was 92.0 (69.0-79.2), 69.0% (60.1-79.2), 53.2% (39.9-70.8) and 34.4% (17.4-68.0) for stages I, II, III, and IV, respectively.
In a contemporary cohort of patients with ATTR cardiomyopathy, the NAC and modified Mayo staging systems remained equally effective in delineating clinically meaningful prognostic groups, whereas the extended NAC system identified a stage IV subgroup with high mortality.
All patients with newly diagnosed ATTR cardiomyopathy and baseline biomarker measurements from 2019 to 2023 were included from Mayo Clinic, Rochester, MN. Prognostic outcomes were calculated with Kaplan-Meier analysis for 3e staging systems in the main analysis: the National Amyloidosis Centre (NAC) staging system; a modified Mayo staging system using hsTNT (high-sensitivity troponin T) cutoff >65 ng/L; the extended NAC model with a fourth stage (NT-proBNP [N-terminal pro-B-type natriuretic peptide] >10 000 ng/L).
The study population consisted of 441 patients with a median age of 76 years, 93% were men, 85% were initiated on tafamidis, and 94% had wild-type ATTR. Median follow-up was 3.4 years, and 109 deaths occurred during follow-up. The 3-year survival for the Mayo stages were 92.3% (88.7-95.9) for stage I, 71.6% (63.0-81.3) for stage II, and 43.5% (32.4-58.5) for stage III. For the NAC system, 3-year survival were 92.0% (89.0-95.7), 67.5% (59.0-77.3), and 47.0% (34.7-63.5) for stages I, II, and III, respectively. The 3-year survival by the extended NAC system was 92.0 (69.0-79.2), 69.0% (60.1-79.2), 53.2% (39.9-70.8) and 34.4% (17.4-68.0) for stages I, II, III, and IV, respectively.
In a contemporary cohort of patients with ATTR cardiomyopathy, the NAC and modified Mayo staging systems remained equally effective in delineating clinically meaningful prognostic groups, whereas the extended NAC system identified a stage IV subgroup with high mortality.
Authors
Skov Skov, Clemmensen Clemmensen, Scott Scott, Abou Ezzedine Abou Ezzedine, Chang Chang, Dispenzieri Dispenzieri, Poulsen Poulsen, Grogan Grogan
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