Abstract 20478: Mortality outcomes of Cardiac Implantable Device therapy In Patients With AL type Of Amyloidosis
Amyloid Light (AL) chain, a sub-type of amyloidosis which can affect the heart and may be associated with sudden cardiac death. Cardiac implantable electronic devices (CIED) use in AL is contentious due to a lack of data. Prior studies have suggested possible mortality benefit of implantable cardioverter defibrillators (ICD) in AL even in the absence of traditional risk factors.
Hypothesis: We sought to determine whether patients with AL who receive CIED or ICD therapy have improved survival than AL patients without CIED or ICD.
Methods: Using the Mayo Clinic Amyloid registry of biopsy proven AL, we reviewed all cases diagnosed between Jan 1st 1985 to Sep 30 2015. All data including follow-up was collected prospectively including in-person visits, mailed surveys or tele-interviews. We obtained death status, cause of death, follow-up duration and type of CIED implant each patient. Absolute risk reduction(ARR) and relative risk reduction(RRR) were calculated for each group (vs mortality in non-CIED AL).
Results: A total of 4,303 patients were seen, of which 3,402 had biopsy and/or mass spectrographically proven AL amyloid. The mean age at diagnosis was 63.0 (SD 10.73, SEM 0.18) years, with a median (25th, 75th) follow-up of 2.4 (0.6, 5.9) years. 164(4.8%) had a CIED implant (table 1). Absolute risk reduction(ARR) was greatest in those treated with ICDs at 14.8% vs. 9.8% for PPM vs. 7.8% for CRT (p=0.13). Kaplan-Meier analysis of those with an ICD showed no difference in mortality (fig. 1, p=0.11). Univariate Cox proportional hazards modelling for ICD vs no ICD showed a HR 0.92 (95%CI 0.7-1.2, p=0.49).
Conclusion: CIED use (of any type) in AL amyloidosis showed no significant benefit to all-cause mortality. ICD therapy showed the best ARR with mortality, but this did not reach statistical significance. Whether there is any morbidity benefit and survival advantage due to preventing cardiac related deaths (as opposed to all-cause mortality) remains to be determined.
Author Disclosures: A. Chahal: None. D. Padmanabhan: None. N. Tandon: None. A.M. Sugrue: None. V.K. Somers: None. A. Dispenzieri: None. M. Grogan: None. G. Lin: None. P. Brady: None.
- © 2016 by American Heart Association, Inc.