OBJECTIVES

Transthyretin (TTR) Amyloid Cardiomyopathy (ATTR-CM) is a rare, progressively debilitating, fatal disease caused by the deposition of amyloid fibrils of the serum TTR protein in the extracellular matrix of target organs such as the heart. Data which describe its epidemiology in the general population are limited. We aimed to estimate the prevalence and the incidence of ATTR-CM in France between 2011 and 2017 and to describe demographic characteristics of incident cases.

METHODS

We used real-world data from the SNDS nationwide database, which collects health insurance claims and hospital discharge information from 99% of the French population. As there is no specific ICD-10 marker code for ATTR-CM, the prevalent cases were defined as patients with diagnosis codes or procedure for both cardiac conditions possibly related to ATTR-CM and amyloidosis (E85), older than 50 years of age when diagnosed. Patients who were affected with myeloma or angiopathy amyloid or who received at least one of the drugs usually prescribed to treat AL Amyloidosis were excluded.

RESULTS

From 2011 to 2017, incidence increased from 0.5 to 1.8 cases per 100,000 residents per year, and prevalence increased from 0.8 to 4.2 cases per 100,000 residents per year. Over the 2011-2017 period, 4,815 incident cases were identified (incidence rate: 1.0/100,000 residents) of whom, 3.221 (66.9%) were males and sex ratio was 2.0. Median age at diagnosis was 81 years old (interquartile range: 13) and 70.9% of subjects were between 70 and 90 years old. Age was similar between men and women.

CONCLUSIONS

This study is the first nationwide estimate of ATTR-CM incidence and prevalence using French real-world data. ePACT algorithm identified only the ICD-10 coded diagnosed cases and thus may have underestimated the full prevalence and incidence of the disease in France. Other data sources are needed to assess the robustness of these estimates.