Prediction of long-term survival in patients with transfusion dependent haemoglobinopathies: Insights from cardiac imaging and ferritin.

ABSTRACT Aims. The current study evaluated the association of echocardiography, cardiac magnetic resonance (CMR) and ferritin data with the 10-years survival in thalassaemia patients. Methods Demographics, ferritin, echocardiography and CMR parameters of stable consecutive thalassaemia patients were prospectively collected. Results In total, 75 patients (mean age 37±11 years, 45% male) with thalassaemia 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 ≥2000ng/ml (HR: 4.682, p=0.007), and ≥1700ng/ml (HR: 7.817, p=0.002), elevated LV end-diastolic pressure (HR: 1.019, p=0.044), TR Vmax >2.8m/s (HR: 6.845, p=0.005), CMR T2* ≤20msec (HR: 3.602, p=0.043) and ≤34msec (HR: 5.854, p=0.026) were associated with increased all-cause mortality (primary end-point). A baseline model including age was created and it became more predictive of worse survival by adding TR Vmax >2.8m/s than elevated LV end-diastolic pressure (C index 0.767 vs. 0.760 respectively) or ferritin ≥1700ng/ml than ≥2000ng/ml (C index 0.890 vs. 0.807 respectively) or CMR T2*≤34msec than ≤20msec (C index 0.845 vs. 0.839 respectively). Parameters associated with the combined end-point of cardiac mortality/cardiac hospitalization (secondary end-point) after adjusting for the age were: ferritin ≥1700ng/ml (HR 3.770, p=0.014), ratio E/A wave >2 (HR 3.565, p=0.04), TR Vmax >2.8m/s (HR 4.541, p=0.049), CMR T2* ≤20ms (HR 9.462, p=0.001) and CMR T2* ≤34ms (HR 11.735, p=0.002). The model including age and T2* ≤34ms than T2* ≤20ms was more predictive of the secondary end-point (C-index 0.844 vs 0.838, respectively). Conclusions In thalassaemia patients, TR Vmax >2.8m/s, 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 ≤1700ng/ml and CMR T2* ≥34ms may improve their prognosis.