Pulmonary hypertension in sickle-cell disease: comorbidities and echocardiographic findings

Our aim is to determine comorbidities associated with pulmonary hypertension (PHT) in clinically stable sickle-cell disease (SCD) patients and to evaluate left ventricular (LV) and right ventricular (RV) function in those patients.

Echocardiography was performed in 87 SCD patients that were divided into group I (without PHT) and group II (with PHT). Both groups were compared with healthy controls.

A history of retinopathy and leg ulcer was more frequent in group II than group I (p < 0.01). Haemoglobin levels were lower (p < 0.05), whereas blood urea nitrogen, lactate dehydrogenase and total bilirubin levels were higher in group II (p < 0.01). Although group II patients had larger LV end-diastolic, LV end-systolic and RV diastolic diameters compared with group I patients and controls (p < 0.05), LV ejection fraction was similar in the three groups. The mitral peak early diastolic inflow velocity to peak late diastolic inflow velocity (E/A) ratio was similar in group I, group II and the control group. The tricuspid E/A ratio was lower in group II than group I and controls (p < 0.05).

End organ damage occurs more often and haemolysis is severer in SCD patients with PHT than SCD patients without PHT. Although LV systolic and diastolic function is well preserved, RV diastolic function is disturbed in those patients with PHT.