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

Chibuzo Ilonze; Gift C Echefu; Alexandria L Broadnax; Adedoyin Johnson; Aniekeme Etuk; Onyedika J Ilonze

doi:10.1016/j.jnma.2023.11.010

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

J Natl Med Assoc. 2023 Dec 14:S0027-9684(23)00146-3. doi: 10.1016/j.jnma.2023.11.010. Online ahead of print.

Authors

Chibuzo Ilonze¹, Gift C Echefu², Alexandria L Broadnax¹, Adedoyin Johnson³, Aniekeme Etuk⁴, Onyedika J Ilonze⁵

Affiliations

¹ Division of Hematology and Oncology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, United States.
² Division of Cardiovascular Diseases, Department of Internal Medicine, University of Tennessee Health Science Center, Memphis, TN, United States.
³ Department of Internal Medicine, Indiana University School of Medicine, Indianapolis, IN, United States.
⁴ Department of Internal Medicine, Infirmary Health Thomas Hospital, Mobile, AL, United States.
⁵ Division of Cardiovascular Medicine, Indiana University School of Medicine, Indianapolis, IN, United States. Electronic address: oilonze@iu.edu.

PMID: 38101960
DOI: 10.1016/j.jnma.2023.11.010

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.

Keywords: Diastolic dysfunction; Heart failure; Pulmonary hypertension; Sickle cell disease.