Key result
Significant valvular heart disease in ATTR-CM linked to ~2-fold higher risk of death or HF hospitalization.
Why the study?
Significant valvular heart disease (VHD) is associated with adverse prognosis in transthyretin amyloidosis cardiomyopathy (ATTR-CM), but data were limited to isolated VHD rather than differences in outcomes between isolated and multiple VHD, with and without aortic stenosis (AS).
Does the presence of isolated or multiple significant valvular heart disease, particularly with aortic stenosis, increase the risk of heart failure hospitalizations and all-cause death in patients with transthyretin amyloidosis cardiomyopathy?
Cohort (n=694)
Yes
Does the presence of isolated or multiple significant valvular heart disease, particularly with aortic stenosis, increase the risk of heart failure hospitalizations and all-cause death in patients with transthyretin amyloidosis cardiomyopathy?
In patients with transthyretin cardiac amyloidosis, significant valvular heart disease is present in 44% of cases and independently predicts mortality and heart failure hospitalization, especially when multiple valves and aortic stenosis are involved.
Significant valvular disease was associated with higher HF hospitalization or death risk in ATTR-CM; leaves open whether intervention improves outcomes.
Background Significant valvular heart disease (VHD) is associated with adverse prognosis in transthyretin amyloidosis cardiomyopathy (ATTR-CM). However, data is limited to isolated VHD. We aimed at evaluating the difference in outcome between isolated and multiple VHD, and particularly with and without aortic stenosis (AS).Methods Patients with ATTR-CM were retrospectively included in a multicentric registry. The endpoint was a composite of heart failure hospitalizations and all-cause death.Results Of the 694 patients (mean age 75 ± 13 years,74%male), 302(44%) presented with significant VHD. Of these, 98 had VHD including AS (isolated AS in 45%;multiple VHD with AS in 55%), while 204 had VHD without AS (isolated in 64%, multiple VHD in 36%). During a median follow-up of 22(8–38) months, 217(31%) patients reached the endpoint. Significant VHD was associated with worse outcomes than no-VHD (log-rank p < 0.001; HR:3.275; 95%CI (2.472–4.337), remaining significant after adjustment (HR2.006 (1.193–3.047);p = 0.007). Stratification by the presence of AS, and according to isolated or multiple VHD, revealed that isolated significant AS ((HR: 2.462; 95%CI (1.233–4.917); p = 0.011), and multiple VHD including significant AS, had the highest risk for adverse outcomes ((HR: 3.385; 95%CI (1.744–6.573);p < 0.001).Conclusion In ATTR-CM, significant VHD is strongly associated with poor prognosis, with the highest risk for adverse events in significant multiple VHD including AS.
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Sarrazyn et al. (2026) conducted a cohort in Transthyretin amyloidosis cardiomyopathy (ATTR-CM) (n=694). Significant valvular heart disease (isolated or multiple, with or without aortic stenosis) vs. No significant valvular heart disease was evaluated on Composite of heart failure hospitalizations and all-cause death (HR 2.006, 95% CI 1.193-3.047, p=0.007). Significant valvular heart disease in ATTR-CM increased risk of HF hospitalization or death (adjusted HR 2.006, 95% CI 1.193-3.047; p=0.007), highest in multiple VHD with AS (HR 3.385; p<0.001).
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