Abstract

Background: The prevalence of pulmonary arterial hypertension (PAH) in Saudi adults with sickle cell disease (SCD), the mechanism of its development, and its prospective prognostic significance are unknown.

Objective: To assess the prevalence of PAH among sickle cell disease patients attended hematology outpatient clinic at King Fahad Hospital, Al Hassa, Saudi Arabia.

Methods: Doppler echocardiography was performed for assessments of pulmonary- arterial systolic pressure (PASP) on 203 consecutive patients (102 men and 101 women) aged > 11 years, attending hematology clinic at King Fahad Hospital, Al Hassa, Saudi Arabia. Pulmonary hypertension was prospectively defined as a tricuspid regurgitant jet velocity (TRJV) of at least 2.5 m per second which can be estimate PASP equal or more than 25 mmHg.

Results: Doppler-defined pulmonary arterial hypertension was diagnosed in 37.1% among 202 patients included in study (after one female patient was excluded) using a cutoff of PASP >=25 mmHg.

Conclusion: The prevalence of PAH among adults Saudis with SCD is higher than that reported from the developed countries. Further assessment using invasive techniques is required coupled employing analytical study design to predict the factors that favor the development of PAH among Saudi patients are required.

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