Pulmonary Hypertension in Sickle Cell Disease: A Case Series

Pulmonary hypertension (PH) is a serious and increasingly recognized complication in sickle cell disease (SCD), particularly in individuals with the homozygous genotype. Elevated tricuspid regurgitant velocity (TRV) increases the potential for future development of PH, yet no standardized management approach currently exists, likely because of the varied pathophysiology of PH in these patients. Transthoracic echocardiogram-derived elevated TRV of more than 2.7 m/second in patients with SCD is associated with increased mortality and remains the only non-invasive approach to diagnosis. PH in patients with SCD can be due to various etiologies and can be considered either precapillary or post-capillary PH. This case series highlights the need to diagnose the underlying etiology of SCD-induced PH, which in turn will dictate appropriate management. Additionally, this case series will review recent management strategies available for treating patients with established PH and address significant risk factors for patients at risk for the development of PH.