Pulmonary Hypertension in Children with Sickle Cell Disease: a Review of the Current Literature

Purpose of ReviewPulmonary hypertension (PH) is a well-recognized complication of sickle cell disease (SCD) and is one of the strongest predictors of increased morbidity and mortality in adult patients. There is evidence that PH can develop in children with SCD and the clinical implications of this finding are an area of active research. We review the current literature examining the association of SCD and PH in childhood.Recent FindingsThe recent literature has focused on elucidating the multifactorial mechanisms for the development of PH in SCD with the goal of developing targeted therapies. In addition, there has been a focus on understanding the significance of echocardiographic evidence of PH in children with SCD, a finding that has recently been associated with adverse clinical factors. While still based on limited evidence, the increased understanding of the important prognostic implications of echocardiographic evidence of PH has led to the development of guidelines that recommend screening echocardiograms beginning in childhood in children with SCD.SummaryPH can develop in children with SCD and, while the exact clinical implications of this finding are still being elucidated, current guidelines and research are aimed at early identification and treatment to improve outcomes.