Predictors of survival among 60 patients with tissue proven primary cardiac amyloidosis

Background: Cardiovascular involvement among patients with primary systemic amyloidosis (AL) is common and predicts poor prognosis. Different parameters have been used to predict outcome. We studied the prognostic significance of clinical, ECG and echocardiographic parameters of 60 patients with tissue proven primary cardiac amyloidosis. Method and Results: Records of 60 patients with primary amyloidosis and cardiac involvement documented by endomyocardial tissue biopsy were retrospectively evaluated. Patients mean age was 57.9 ± 10.2 years. 71.6% were male and 86.6% Caucasian. Patients’ median survival was 12.2 ± 4.4 months with only 50% of patients survived for more than 1 year. 60% of patients had CHF (NYHA II-IV). CHF (NYHA II-IV), IVS, LVPW and LVEF were significant on univariate survival analysis (p < 0.05). On multivariate analysis only CHF (p = 0.027, HR 3.04 [95% CI: 1.1 - 8.1]) and IVS < 1.5 cm (p = 0.012, HR: 3.51 [95% CI: 1.3 - 9.3]) were predictors of survival. Patients with CHF had a median survival of 7.58 ± 1.74 months contrary to those without CHF who had a median survival of 31.2 ± 11.41 months. Patients with IVS ≥ 1.5 cm had a median survival of 7.0 ± 1.1 months, contrary to those with an IVS < 1.5 cm who had a median survival of 31.9 ± 12.4 months. Conclusion: In patients with primary amyloidosis and cardiac involvement, length of survival is strongly associated with CHF (NYHA II-IV) and IVS compared to other electrographic and echocardiographic parameters