Left ventricular dysfunction (LVD) defined as LVEF <55% is associated with an excess of death in patients with sickle-cell disease (SCD)^{1Damy et al EHJ 2016.} . In this study, we analyzed the frequency and clinic characteristics of LVD in the French SCD multicentric cohort initiated from 1999 to 2011 to measure the prevalence of pulmonary hypertension. Patients included had SS or S-β Thal SCD and were in stable condition. Patients with severe renal, hepatic or pulmonary dysfunction were excluded. The study variables were collected prospectively.

The French cohort included 392 patients. Thirty-seven patients (9.4%) had a LVEF <55%. Their average age was similar to those without LVD (35 vs. 34 years P=0.55). Sex ratio was also identical. No significant difference was found in the frequency of systemic hypertension (10.8% vs. 4.7%), systolic (118.05mmHg vs. 116.7mmHg) or diastolic blood pressure (67.3mmHg vs. 66.0mmHg), or heart rate (72 vs. 74), all P>0.15. BMI (21.89 vs. 21.7kg/m² (P=0.34) and creatinine clearance (126 vs.130mL/min) were also similar (all P>0.10). LV diastolic volume (82.6 vs. 71.7mL/m²) and E/E’ ratio (8.3 vs. 6.7) were increased (all P<0.001), but not cardiac output (6.3 vs. 6.3 L/min), left atrial volume (42.2 vs. 40.8mL/m²), and the maximal velocity of tricuspid regurgitation (all P>0.10). Frequency of class 3 NYHA (13.5 vs. 6.8%), 6minutes walk test distance (523±76 vs. 512±88m), Borg score (2.0 vs. 2.7), ProBNP value (log-expressed, 1.74 vs. 1.83), were similar (all P>0.10).

LV dysfunction is uncommon in patients with SCD and is not associated with clinical or biological differences which could explain the excess mortality previously observed. It does not result in functional impairment. However a similarly high cardiac output is observed, owing not to an increase in heart rate in those with LV dysfunction, but rather to a LV dilatation whose prognostic value needs further assessment.