Abstract 1610: Device Therapy for Cardiac Amyloidosis Does not Impact Survival
Background: Cardiac amyloidosis (CA) is associated with sinus node dysfunction (SND) and conduction system disease (CSD) and increased risk of sudden cardiac death. Whether SND and CSD predict worse outcome, and the impact of device therapy (permanent pacemaker-PPM and ICD), is unknown.
Methods: To determine this we reviewed all clinical, pathological, and echocardiographic data on CA patients (Pts) referred to Mayo Clinic between 2000 and 2005. Outcome of CA Pts with devices was compared to CA Pts without indication for devices.
Results: A total of 387 Pts of which 63 (17%; age 66 ± 11 years, 82% male) had a device implanted and were included. Of these, AL form was present in 42 (66%), senile in 15 (23%), and familial in 7 (11%). All had restrictive physiology (EF 47% ± 15%), with increased septal thickness (≥ 15 mm) in 33 (53%). PPM was implanted in 26 Pts (42%) versus 37 (58%) who received ICD. No difference in age, EF, severity of restrictive physiology, or type of CA was observed between groups (p>0.05 for all). SND was present in 20 Pts (31%) and CSD in 16 Pts (25%), and atrial fibrillation was present in 31 (48%). Indication for ICD was primary prevention in 9 Pts (14%) and secondary (syncope or sustained ventricular arrhythmia) in 28 Pts (44%). Survival at one year of Pts with PPM was similar to Pts with ICDs and to that of CA Pts without devices (p>0.05).
Conclusions: SND and CSD are not uncommon in CA. Device therapy (PPM or ICD) has a neutral effect on survival, suggesting that CA Pts who present with symptomatic bradycardia have outcomes to similar to those without devices. ICD therapy is not associated with improved survival.