Prevalence, Incidence and Clinical Outcomes of Epicardial Coronary Artery Disease Among Transthyretin Amyloidosis Cardiomyopathy Patients

Abstract Background: Transthyretin amyloidosis cardiomyopathy (ATTR-CM) patients are often older and may be at risk for obstructive epicardial coronary artery disease (oeCAD). While ATTR-CM may cause small vessel coronary disease, the prevalence and clinical significance of oeCAD is not well described.Methods and Results: The prevalence and incidence of oeCAD and its association with all-cause mortality and hospitalization among 133 ATTR-CM patients with ≥1-year follow-up was evaluated. The mean age was 78±9 years, 119 (89%) were male, 116 (87%) had wild-type and 17 (13%) had hereditary subtypes. Seventy-two (54%) patients underwent oeCAD investigations, with 30 (23%) receiving a positive diagnosis. Among these patients, 23 (77%) were diagnosed prior to ATTR-CM diagnosis, 6 (20%) at the time of ATTR-CM diagnosis, and 1 (3%) after ATTR-CM diagnosis. Baseline characteristics between patients with and without oeCAD were similar. Among patients with oeCAD, only 2 (7%) required additional investigations, intervention or hospitalization after ATTR-CM diagnosis. After a median follow-up of 27 months there were 37 (28%) deaths occurring in 5 patients with oeCAD (17%) and 32 patients without oeCAD (31%). Fifty-six (42%) patients required hospitalization, including 10 patients with oeCAD (33%) and 46 patients without oeCAD (45%). There was no significant difference in the rates of death or hospitalization among ATTR-CM patients with and without oeCAD, and oeCAD was not significantly associated with either outcome by regression analysis.Conclusions: While oeCAD is prevalent in ATTR-CM patients, this diagnosis is frequently known at time of ATTR-CM diagnosis and is not associated with subsequent death or hospitalization.