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Updated: Jul 8, 2025
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Cardiovascular complications of sickle cell disease: A primer for the general clinician
Chibuzo Ilonze1, Gift C Echefu2, Alexandria L Broadnax1
1Division of Hematology and Oncology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, United States.
Insights
Sickle cell disease (SCD) survivors face increased cardiopulmonary issues. Early diagnosis and intervention are key to improving quality of life and survival rates for these patients.
Area of Science:
- Hematology
- Cardiology
- Genetics
Background:
- Sickle cell disease (SCD) is a common inherited blood disorder, primarily affecting individuals of African ancestry.
- Improved survival in high-income nations has led to a rise in cardiopulmonary complications in adult SCD patients.
- These complications, including pulmonary hypertension and heart failure, are major causes of death and disability.
Purpose of the Study:
- To review the pathogenesis of cardiovascular complications in sickle cell disease.
- To provide clinicians with tools for early diagnosis and management of SCD patients.
- To address the growing challenge of managing adult SCD survivors with cardiac issues.
Main Methods:
- Comprehensive literature review.
- Analysis of current clinical practices and research findings.
- Synthesis of information on SCD pathophysiology and cardiovascular manifestations.
Main Results:
- Cardiopulmonary complications are increasingly prevalent in adult SCD patients.
- These complications significantly contribute to morbidity and mortality.
- Understanding pathogenesis is crucial for effective management.
Conclusions:
- Early identification and timely intervention are critical for managing SCD-related cardiopulmonary complications.
- Preventive strategies are essential to improve outcomes for adult SCD survivors.
- This review equips clinicians to better manage cardiovascular health in sickle cell disease.
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.
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