Baseline characteristic and prognosis of patients with cardiac amyloidosis referred in the French National Referral Center for Cardiac Amyloidosis (filière CARDIOGEN)

Introduction Amyloidosis is a disease caused by infiltration of tissues by an amyloid protein. The most frequent cardiac amyloidosis is light chain (AL) amyloidosis, hereditary transthyretin (TTR-h) and wild transthyretin (w-TTR). Objective Describe baseline clinical, biological, echocardiographic characteristics and prognostic of patients referred for cardiac amyloidosis in the Amyloidosis expert center. Methods From 2010 to July 2016, all patients referred to the expert center of our Hospital for suspected cardiac amyloidosis were included. Baseline demographic, clinical, laboratory and ultrasound characteristics were recorded and patients were followed-up for major events including death, cardiac transplantation and left ventricular assistance implant. Results Of the 942 patients referred for suspicion of CA, 502 had confirmed CA of whom162 had AL amyloidosis, 203 h-TTR and 141 w-TTR. CA were men in 60%, the median age was 69 years w-TTR amyloidosis were older (83 [79; 87], P P P P P P P Conclusion Cardiac amyloidosis is more frequent than initially thought and has poor prognosis. The challenge is to achieve earlier diagnosis to improve prognosis and treatment.