Prediction of Long-Term Survival in Patients with Transfusion-Dependent Hemoglobinopathies: Insights from Cardiac Imaging and Ferritin.

Aims: The current study evaluated the association of echocardiography, cardiac magnetic resonance (CMR), and ferritin data with 10-year survival in thalassemia patients. Methods: Demographics, ferritin, echocardiography, and CMR parameters of stable consecutive thalassemia patients were prospectively collected. Results: In total, 75 patients (mean age 37 +/- 11 years, 45% male) with thalassemia were included and dichotomized based on their survival status after a median follow-up period of 10.3 [9.6-10.9] years. Older age (HR: 1.071, p = 0.001), ferritin >= 2000 ng/ml (HR: 4.682, p = 0.007) and >= 1700 ng/ml (HR: 7.817, p = 0.002), elevated LV end-diastolic pressure (HR: 1.019, p = 0.044), TR Vmax >2.8 m/s (HR: 6.845, p = 0.005), and CMR T2* <= 20 msec (HR: 3.602, p = 0.043) and <= 34 msec (HR: 5.854, p = 0.026) were associated with increased all-cause mortality (primary endpoint). A baseline model including age was created and became more predictive of worse survival by adding TR Vmax >2.8 m/s instead of elevated LV end-diastolic pressure (C index 0.767 vs. 0.760, respectively), ferritin >= 1700 ng/ml instead of >= 2000 ng/ml (C index 0.890 vs. 0.807, respectively), or CMR T2* <= 34 msec instead of <= 20 msec (C index 0.845 vs. 0.839, respectively). Parameters associated with the combined endpoint of cardiac mortality/cardiac hospitalization (secondary endpoint) after adjusting for age were ferritin >= 1700 ng/ml (HR 3.770, p = 0.014), ratio E/A wave >2 (HR 3.565, p = 0.04), TR Vmax >2.8 m/s (HR 4.541, p = 0.049), CMR T2* <= 20 ms (HR 9.462, p = 0.001), and CMR T2* <= 34 ms (HR 11.735, p = 0.002). The model including age and T2* <= 34 ms instead of T2* <= 20 ms was more predictive of the secondary endpoint (C-index 0.844 vs 0.838, respectively). Conclusions: In thalassemia patients, TR Vmax >2.8 m/s, ferritin >= 2000 ng/ml, and CMR T2* <= 20 ms were associated with worse long-term survival. In the current era of advanced chelation therapy, aiming for ferritin <= 1700 ng/ml and CMR T2* >= 34 ms may improve their prognosis. (C) 2021 Hellenic Society of Cardiology. Publishing services by Elsevier B.V.