The risk of pulmonary hypertension in sickle cell disease in relation to the disease severity

Abstract: BACKGROUND: Sickle cell disease (SCD) is a heterogeneous disease which can induce fatal complications, especially cardiovascular complications. The development of a classification system to classify patients with SCD helps to identify those with high risk for further preventive measures and follow-up. OBJECTIVES: This study evaluates the relationship between SCD severity and the risk of pulmonary hypertension (PH) development aiming to identify the group of SCD patients who are at high risk of PH development for further preventive measures. PATIENTS AND METHODS: Retrospective analysis for SCD patients retrieved from records from 2009 to 2021. Five hundred and seventeen patients were selected; all patients were assessed for the risk of PH by following the guidelines of the European Society of Respiratory and European Society of Cardiology using echocardiography. Patients were then classified according to the Severity Classification System for SCD into three classes from the least severe to the most severe. This study was held at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. RESULTS: Among participants, 93% have a low risk of PH. Fifty-eight (11.2%) patients have a severe SCD, and 458 (88.5%) patients have mild SCD. Twenty-nine (80.5%) of individuals with SCD who have a high or moderate risk of PH were found to have severe SCD (Class III). CONCLUSION: SCD severity is strongly correlated with the risk of PH development. The severity classification system is easily applicable to identify those with a high risk of PH development to apply further preventive strategies.