Cardiovascular complications of sickle cell disease: A primer for the general clinician

Abstract

Sickle cell disease (SCD) is the most common hereditary hemoglobinopathy and mainly affects individuals of African ancestry. As survival has improved especially in high-income countries, increased rates of cardiopulmonary complications such as pulmonary hypertension, heart failure with diastolic dysfunction, and sudden death are encountered in clinical practice. These complications are the leading causes of morbidity and mortality as these individuals survive into adulthood. Understanding the need for, early identification, timely intervention, and implementation of preventive strategies are critical in reversing this trend and improving quality of life and survival rates. This manuscript aims to provide a comprehensive review of the pathogenesis of cardiovascular complications associated with sickle cell disease and equip the clinician with tools to facilitate the early diagnosis and management of patients with SCD as increasing numbers survive into adulthood.