Cardiac amyloidosis is a fatal disease. It may represent 10% of non-ischemic heart diseases. Conduction and rhythm disorders are common causes of death. There are no real guidelines about PM or ICD indications in cardiac amyloidosis.

Cardiac devices (PM/ICD) prolong survival by decreasing MACE (cardiovascular death; sudden cardiac death; delivered therapies by ICD; cardiac decompensation; heart transplant) by treating rhythmic and conductive events and improving cardiac hemodynamics. The aim was to demonstrate the benefit in terms of cardiovascular mortality or morbidity of implantation of cardiac devices (PM/DAI) in patients with different types of cardiac amyloidosis.

A total of 1053 patients were screened for cardiac amyloidosis between 10/2010 and 12/2016 within Réseau Amylose Mondor (CHU Henri-Mondor–Créteil). A total of 186 AL amyloidosis (AL), 225 hereditary amyloidosis (hTTR) and 168 senile amyloidosis (wtTTR) were included in our study and followed from inclusion in the database until their death or until 12/2016.

Overall a total of 70/186 PM/ICD were implanted in AL amyloidosis (38%), 99/225 in hTTR (44%) and 96/168 in wtTTR (55%). Patients implanted with a PM/ICD had a 24-month risk of developing MACE almost twice as large as non-implanted patients, for any type of amyloidosis (HR 1.73 [1.28; 2.34]; P<0,001). A propensity score was created to identify factors associated with prosthesis implantation. In multivariate analysis, incorporating the propensity score, the best statistical model for identifying the risk factors for MACE occurrence showed that the presence of a PM/ICD was not associated with the occurrence of MACE at 24 months (C-stat 0.75) (Figure 1).

This study demonstrates that PM/ICD are not associated with an overdose of major cardiovascular complications, as suggested in some studies. Our data suggest that these prostheses have a place in this disease, provided to find the good criteria of selections of the patients to implant.