Abstract 16030: Heterogeneity of Electrocardiographic Findings in Cardiac Transthyretin (ATTR) Amyloidosis and Impact on Survival
Introduction: Cardiac transthyretin (ATTR) amyloidosis is an increasingly diagnosed cause of heart failure. Wild-type ATTR amyloidosis (ATTRwt) is a disease of older Caucasian men. Hereditary (mutant) cardiac ATTR amyloidosis (ATTRm) is most commonly associated with the V122I transthyretin (TTR) variant carried by 3-4% of African Americans or the T60A TTR variant in patients of Irish ancestry, the latter presenting with a mixed cardiac and neurologic phenotype. Most published data consider electrocardiographic (ECG) findings in ATTR amyloidosis as a single entity. Prognostic factors for survival in cardiac ATTR amyloidosis are poorly defined.
Methods and Results: We analysed ECGs from 158 patients with cardiac ATTR amyloidosis (ATTRwt=75; V122I=49; T60A=34) in a longitudinal retrospective study.
Results are shown in Table 1.
63 patients died; 29 ATTRwt, 24 V122I, 10 T60A, during follow-up of 22.7±14.6, 23.8±12.8, and 31.3±20.4 months respectively. Overall median survival: ATTRwt 3.1yr (95%CI:2.2-4.0); V122I 2.6 yr (95%CI:2.2-3.0); T60A not reached. In ATTRwt, broad QRS (≥120ms) was associated with worse survival (1.6yr; 95%CI:1.3-1.9 vs not reached). No other ECG parameter in ATTRwt, and none in either ATTRm group affected survival. On univariate analysis of the ATTRwt cohort, broad QRS, age, systolic BP, TropT and NTproBNP were significant variables at the <1% level, and eGFR at the 5% level; LVEF, septal S’ TDI and global longitudinal strain were not significant. On multivariate analysis only broad QRS (HR: 3.0, CI 1.2-7.5; P=0.024) and NTproBNP (HR: 3.0, CI 1.1-7.4; P=0.026) predicted mortality. Among ATTRwt patients, there were no significant differences of other characteristics between those with narrow and those with broad QRS complexes.
Conclusions: The ECG in cardiac ATTR amyloidosis is heterogeneous, greater awareness of which might help prevent diagnostic delays. Broad QRS complexes and NTproBNP are independent predictors of mortality in ATTRwt.
Author Disclosures: K.S. Patel: None. M. Fontana: None. S. Sachchithanantham: None. R. Sayed: None. A.D. Wechalekar: None. H.J. Lachmann: None. P.N. Hawkins: None. C.J. Whelan: None. J.D. Gillmore: None.
- © 2014 by American Heart Association, Inc.