Prediction of long-term survival in haemoglobinopathies: insights from cardiac imaging and ferritin

Abstract

Type of funding sources: None. Aims.The data on echocardiography, cardiac magnetic resonance (CMR) and ferritin predicting long-term survival in haemoglobinopathies are scarce. The current study evaluated the association of these parameters with the 10-years survival in haemoglobinopathies. Methods.This prospective study included stable consecutive haemoglobinopathy patients .Demographics, ferritin, echocardiography and CMR parameters were prospectively collected. Results. In total, 83 patients (mean age 38.4 ± 12.0 years, 46% male) with haemoglobinopathies were included and dichotomized based on their survival status after a follow-up of 9.8 ± 1.4 years. Patients who died were older (45.3 ± 11.6 vs 37.1 ± 11.7 years, p = 0.025), had higher ferritin levels (2498 vs 754 ng/ml, p = 0.001), higher right ventricular systolic pressure (RVSP) (41 ± 10 vs 31 ± 11mmHg, p = 0.001), more frequently elevated left ventricular (LV) end-diastolic pressure (70 vs 35%, p = 0.039) and lower CMR T2* values (23 ± 12 vs 35 ± 12ms, p = 0.007). Older age (HR: 1.053, p = 0.018), ferritin >2000ng/ml (HR: 3.517, p = 0.03), and >950ng/ml (HR: 11,135, p = 0.02), elevated LV end-diastolic pressure (HR: 3.977, p = 0.046), RVSP >34mmHg(HR: 10,134, p = 0.003), CMR T2* <20msec (HR: 4.900, p = 0.018) and <36msec (HR: 9.376, p = 0.035) were associated with increased all-cause mortality. A baseline model including age was created andit became more predictive of worse survival by adding RVSP >34mmHg than elevated LV end-diastolic pressure (C index 0.777 vs. 0.757 respectively) or ferritin >950ng/ml than >2000ng/ml (C index 0.805 vs. 0.770 respectively) or CMR T2*<36msec than <20msec (C index 0.825 vs. 0.810 respectively). Conclusions. In haemoglobinopathy patients, RVSP >34mmHg, ferritin >2000ng/ml and CMR T2* <20ms were associated with worse long term survival.In the current era of advanced chelation therapy, aiming for ferritin <950ng/ml and CMR T2* >36ms appears to improve their prognosis. Abstract Figure.