High prevalence of pulmonary hypertension in homozygous sickle cell patients with leg ulceration

Abstract

Pulmonary hypertension is a common complication and is a risk factor for death in adult patients with sickle cell disease. Chronic leg ulceration is a major cause of morbidity in homozygous sickle cell disease. We aimed to determine prevalence of pulmonary hypertension in homozygous sickle cell patients and if there is any relation of pulmonary hypertension with leg ulceration. A total of 88 patients, asymptomatic for pulmonary hypertension, were enrolled in the study. Doppler echocardiography was performed on homozygous sickle cell patients with and without leg ulceration. 12 patients (10 male, 2 female) had active ulcer or healed scar (group I) and 76 patients (40 male, 36 female) had no active leg ulcer or history of (group II). The prevalence of pulmonary hypertension in group I and group II were 91.6% (n = 11) and 31.6% (n = 24), respectively (p = 0.0001). Patients with leg ulceration had increased left atrium and right ventricular diameters at diastole and also had increased left ventricular end-diastolic and end-systolic diameters. We determined an increased prevalence of pulmonary hypertension in patients with leg ulceration. Patients with homozygous sickle cell disease, especially those with leg ulcers should be screened for pulmonary hypertension, since pulmonary hypertension is a frequent and generally asymptomatic complication and a risk factor of mortality.