Relative systemic hypertension in patients with sickle cell disease is associated with risk of pulmonary hypertension and renal insufficiency.

We analyzed entry data from 163 adult hemoglobin SS and Sbeta(0) thalassemia patients enrolled in the prospective Sickle Cell Pulmonary Hypertension Screening Study and stratified their ECHO-determined tricuspid regurgitant jet velocity (TRV) and serum creatinine concentration according to three systemic blood pressure categories. TRV was >or= 2.5 m/sec in 27% of the patients with systolic blood pressure (SBP) <120 mmHg and diastolic blood pressure (DBP) <70 mmHg, in 37% with SBP 120-139 mmHg or DBP 70-89 mmHg, and in 93% with SBP 140 mmHg or DBP 90 mmHg or higher (P<0.0005 for trend). Serum creatinine concentration was 1.0 mg/dL or higher in 7% of patients with SBP <120 mmHg and DBP <70 mmHg, in 17% with SBP 120-139 mmHg or DBP 70-89 mmHg and 50% with SBP 140 mmHg or DBP 90 mmHg or higher (P<0.0005 for trend). Over 2 years of follow-up, there were trends for more frequent progression to elevated TRV (P=0.073) or creatinine (P=0.037) values according to the higher systemic blood pressure categories. Our findings suggest that systemic SBP 120-139 mmHg or DBP 70-89 mmHg defines a category of relative systemic hypertension in patients with sickle cell disease that is associated with increased risk for pulmonary hypertension and renal dysfunction. Whether antihypertensive and/or nitric oxide donor therapy in sickle cell disease patients with relative hypertension prevents these and other complications should be determined by clinical trials.