The Relationship between Sickle Cell Disease and Pulmonary Hypertension in Adults

International Journal of Medical Science
© 2022 by SSRG - IJMS Journal
Volume 9 Issue 1
Year of Publication : 2022
Authors : Mariam Alabdo, Firas Hussein, Basem Marouf
How to Cite?

Mariam Alabdo, Firas Hussein, Basem Marouf, "The Relationship between Sickle Cell Disease and Pulmonary Hypertension in Adults," SSRG International Journal of Medical Science, vol. 9,  no. 1, pp. 14-20, 2022. Crossref,


Sickle cell disease (SCD) is an inherited hemoglobinopathy associated with high morbidity and mortality. Pulmonary hypertension (PH) is reported to play a significant role. There is very limited literature on PH in SCD in Syria. This study aimed to assess the prevalence of pulmonary hypertension and its relationship with clinical and laboratory parameters in sickle cell disease patients. This prospective case-control study included 50 patients with sickle cell disease and 50 controls matched for age and sex at Tishreen University Hospital at Lattakia between July 2019 – and July 2020. Both the patients and controls were subjected to echocardiography. Pulmonary hypertension was prospectively defined as a tricuspid regurgitant jet velocity of at least 2.5 m per second. Sickle cell disease patients had a greater mean TRV than controls. Doppler-defined pulmonary hypertension occurred in 32% of patients. Using the dichotomous variable of a TRV < 2.5 m/s or TRV ≥ 2.5 m/s, multiple logistic regression analysis identified an increased age, high LDH, and indirect bilirubin levels (markers of hemolysis) as significant correlates of pulmonary hypertension. The white-cell count, reticulocytes, sickle complications, clinical measures, or hydroxyurea therapy were unrelated to pulmonary hypertension. In this study of adults with sickle cell disease, the prevalence of pulmonary hypertension, as confirmed by echocardiographic evaluation, was 32%. It appears to be a complication of chronic hemolysis and is resistant to hydroxyurea therapy. Clinical assessment of adult patients with sickle cell disease should include echocardiography.


Pulmonary hypertension, Sickle cell disease, Echocardiography.


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