The Cardiopulmonary Complications of Sickle Cell Disease

Abstract

Sickle cell disease (SCD) is a genetic hemoglobinopathy associated with extensive morbidity and early mortality. While there have been recent improvements in available disease-modifying therapies for SCD, cardiopulmonary complications remain a major risk factor for death in this population. We provide an overview of current knowledge regarding several of the major acute and chronic cardiopulmonary complications in SCD, including: acute chest syndrome, airway disease, lung function abnormalities, nocturnal hypoxemia and sleep disordered breathing, pulmonary vascular disease, and sickle cell cardiomyopathy.

Keywords: Acute chest syndrome; Lung function; Pulmonary hypertension; Sickle cell cardiomyopathy; Sickle cell disease; Sleep-disordered breathing.