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. We aimed at describing clinical characteristics of ATTR-CM individuals in France.

METHODS

A cohort of individuals diagnosed with ATTR-CM between 2011 and 2017 was constituted using real-world data from the SNDS nationwide database, which collects health insurance claims and hospital discharge information from 99% of the French population. Their medical characteristics were identified over the 2007-2017 period through long-term disease codes, hospital discharge diagnosis codes and therapeutic procedures.

RESULTS

4815 patients were identified (mean age of 78.7 (10.0) years old, 66.9% of men). Codes and/or procedures related to arrhythmia or conduction disorders, heart failure, and cardiomyopathies were found in the history of 86.3%, 81.0% and 62.7% of patients, respectively.

68.4% of the individuals were affected with other cardiac conditions including non-rheumatoid valvulopathy for 21.7% and aortic stenosis for 18.0%.

Concerning the non-cardiac conditions which belong to the amyloidosis clinical spectrum, 18.8% and 13.4% of the included individuals were treated at least once for carpal tunnel syndrome and neurological disorders, respectively, over the 2007-2017 period.

A median of 30.7 months elapsed between the appearance of the first event related to the disease – a cardiac event for 85.4% of individuals - and ATTR-CM diagnosis date (date on which a diagnosis of amyloidosis and cardiac damage related to transthyretin cardiac amyloidosis was identified), indicating the complexity of the disease and the difficulty in establishing early a diagnosis of ATTR-CM.

CONCLUSIONS

ePACT is the first study using a nationwide real-world data to describe the medical features of individuals with ATTR-CM; results are consistent with those already published worldwide on smaller patient cohorts.