Abstract 11455: Predictors of Survival in Patients with Cardiac Amyloidosis
Backgrounds: Cardiac amyloidosis is an infiltrative cardiomyopathy that carries the worst prognosis. Although therapeutic options have extended recently, treatment is often overlooked and predictors of survival are not well known in cardiac amyloidosis.
Methods: Using our prospectively collected registry data from 1999 to 2010, we studied 89 consecutive patients (61 men, age; 60±10 years) with cardiac amyloidosis. Cardiac amyloidosis was suspected in patients with heart failure and typical echocardiographic findings, and the diagnosis was confirmed by a positive biopsy. Cardiac amyloidosis was associated with a plasma cell dyscrasia in 55 (62%) patients and 41(46%) received chemotherapy. Study patients were classified into 3 groups according to the treatment; 48 (54%) patients not treated with chemotherapy (Group 1), 24 (27%) without response to chemotherapy (Group 2) and 17 (19%) with response to chemotherapy (Group 3). The response to chemotherapy was defined as a decrease of light chain or improvement of heart failure.
Results: During a median follow-up of 4 months (interquartile range, 2-13 months), 79 (89%) patients died and 1 underwent cardiac transplantation. Presence of pericardial effusion (p=0.025), ejection fraction (p=0.042), and response to chemotherapy (p<0.001) were significantly different between patients who survived ≥ 1 year and those who survived < 1 year. The survival rates were significantly different among treatment groups and the actuarial 1 year survival rates of Group 1 (19±6%) and Group 2 (0%) were significantly lower than Group 3 (82±9%) (p<0.001) (Figure). On multivariate Cox analysis, presence of response to chemotherapy was the only independent predictor related to survival (hazard ratio=0.173, p<0.001).
Conclusions: Early diagnosis of cardiac amyloidosis by echocardiography and timely institution of chemotherapy are critical to an improved survival of cardiac amyloidosis.
- © 2012 by American Heart Association, Inc.