Abstract 16359: Impact of Left Ventricular Diastolic Function on Occurrence of Cardiovascular Events in Cardiac Amyloidosis: Comparison of Myocardial Biopsy-Proven Transthyretin With Immunoglobulin Light Chain Amyloidosis
Background: Although transthyretin (TTR) cardiac amyloidosis was considered to show more favorable outcomes than those with immunoglobulin light chain (AL) amyloidosis, few data exist regarding the occurrence of cardiovascular events in myocardial biopsy-proven patients, particularly in terms of changes in left ventricular function. Therefore, we elucidated serial changes in left ventricular function associated with clinical outcomes of TTR and AL cardiac amyloidosis.
Methods and Results: Of 82 patients for the further evaluation of myocardial hypertrophy, we identified 21 patients (20 men, mean age 78 years) with TTR and 12 patients (8 men, mean age 66 years) with AL amyloidosis by endomyocardial biopsy (EMBx). At the time of enrollment, thickness of interventricular septum was greater in TTR (15±2.2 mm) than that in AL (12±1.9, p<0.001). E/e’ was significantly greater in AL (28±11) than that in TTR (21±8, p<0.05) associated with elevated plasma BNP levels (1118±697pg/ml vs 538±594,p<0.05), although left ventricular EF was not different in both groups. Interestingly, for 12 months, left ventricular end diastolic dimension in TTR increased from 40±1mm to 42±1mm (p<0.001) associated with substantially unchanged BNP levels from the base line. Under these conditions, the cumulative incidence of composite of death or readmission for heart failure in AL at 6 months and 12 month were 83.3% and 100%, respectively, and much greater than those in TTR amyloidosis (28.6% and 43.6%, p<0.001), suggesting highly impaired left ventricular diastolic function in AL is related to the higher occurrence of cardiac events than those with TTR.
Conclusions: These results demonstrate that EMBx-proven TTR cardiac amyloidosis was less associated with left ventricular diastolic as well as systolic dysfunction both of which could preserve for 12 months contributing to favorable clinical outcomes in comparison with AL cardiac amyloidosis. Whether aggressive treatment of AL could result in preserving diastolic function should further be sought.
- © 2013 by American Heart Association, Inc.