Left ventricular dysfunction in patients with sickle cell disease

Left ventricular dysfunction (LVD) defined as LVEF 1 . 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. Results The French cohort included 392 patients. Thirty-seven patients (9.4%) had a LVEF  0.15. BMI (21.89 vs. 21.7 kg/m2 (P = 0.34) and creatinine clearance (126 vs.130 mL/min) were also similar (all P > 0.10). LV diastolic volume (82.6 vs. 71.7 mL/m2) and E/E’ ratio (8.3 vs. 6.7) were increased (all P   0.10). Frequency of class 3 NYHA (13.5 vs. 6.8%), 6 minutes walk test distance (523 ± 76 vs. 512 ± 88 m), Borg score (2.0 vs. 2.7), ProBNP value (log-expressed, 1.74 vs. 1.83), were similar (all P > 0.10). Conclusion 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.