Prevalence and prognostic impact of valvular heart disease in transthyretin cardiac amyloidosis.

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.