Alteration of contractile function in sickle-cell disease: ETENDARD’ cohort analysis

Left ventricular dysfunction (LVD) accompanied induced an raise of mortality for patients with Sickle-cell disease (SCD). In this study, we will analyze the frequency and the clinic parameters associated with this LVD. We analyze the French Multicentre cohort Etendard, which was established between 1999 and 2011 in order to analyze the prevalence of PHT, who included patients with stable type SS or S-β(0)-Thal SCD. Patients with severe renal, hepatic or pulmonary dysfunction were excluded. The studied parameters (clinical, ultrasound, biological) were collected prospectively. The cohort included 392 patients. Thirty-seven (9.4%) of these patients had a LVEF  P  = 0.55) for the patients with a LVD and the patients with a normal one. Sex ratio was also identical. No significant differences were find in arterial hypertension's frequency, (10.8% vs. 4.7%, P  = 0.125), nor cardiac frequency (72 vs. 74, P  = 0.17). BMI (21.89 vs. 21.7 kg/m 2 ( P  = 0,34) and creatinine clearance(126 vs.130 mL/min) were also identical ( P  > 0.10 for all).Echocardiography shows an increased LV volume (82.6 vs 71.7 mL/m 2 ) and an increased E/Ea ratio (8.3 vs 6.7) for the patients with a LVD ( P  2 ) and the TRV maximum speed are unchanged. On the functional level, these elements were unchanged: NYHA3 dyspnea frequency (13.51 vs. 6.76%), the distance traveled during the walking test (523 ± 76 vs. 512 ± 88), Borg's rating (2.01 vs 2.66), ProBNP value (1.74 log vs 1.83 log) ( P  > 0.10 for all of them). The LVD for the patients with SCD is uncommon, is not accompanied with clinical or biological differences, which could explain the raise of mortality highlighted in previous studies, and does not show functional consequences. However, a high cardiac output is necessary in each group. This cardiac output is obtained without any increase of heart rate, but with differences in left ventricular remodeling, whose prognostic character remains to be assessed.