Pulmonary hypertension in sickle cell disease

Abstract

This review highlights the prevalence of pulmonary hypertension as a chronic complication of sickle cell disease (SCD) and its importance in the prognosis. In recent years, the limitations of echocardiogram for the appropriate diagnosis of SCD-associated pulmonary hypertension have been demonstrated, emphasizing the need of invasive hemodynamics assessment before any specific treatment for pulmonary hypertension is considered. The hemodynamic profile observed in this clinical situation is characterized by elevated cardiac output and low pulmonary vascular resistance that differs considerably from what is seen in pulmonary arterial hypertension. Furthermore, both hemodynamic profiles, precapillary and postcapillary, can be equally found in this setting stressing the need for a better understanding of the multiple pathophysiological mechanisms involved in the development of pulmonary hypertension before considering those patients for targeted therapies. Nevertheless, the presence of any form of pulmonary hypertension clearly denotes worse prognosis in SCD. Pulmonary hypertension is an important and prevalent complication of SCD with multiple associated mechanisms. A more aggressive approach of the baseline condition might be necessary, although the data supporting this assumption and also the use of targeted pulmonary arterial hypertension therapy are still lacking.