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Updated: Dec 27, 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
Vandana Sachdev1, Douglas R Rosing1, Swee Lay Thein1
1National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Insights
Sickle cell disease (SCD) causes organ damage and cardiopulmonary issues like pulmonary hypertension. Early diagnosis of these cardiac complications is vital for managing this inherited blood disorder.
Area of Science:
- Hematology
- Cardiology
- Pulmonology
Background:
- Sickle cell disease (SCD) is a prevalent inherited blood disorder globally and in the US.
- Abnormal hemoglobin (HbS) leads to microvascular occlusion and hemolytic anemia, causing progressive organ damage.
- Increased survival has shifted SCD to a chronic condition in adults, marked by multi-organ degeneration, particularly affecting the heart and lungs.
Purpose of the Study:
- To highlight the significance of cardiopulmonary complications in sickle cell disease.
- To emphasize the need for awareness regarding sickle-related cardiovascular phenotypes.
- To promote early screening, diagnosis, and intervention for cardiac issues in SCD patients.
Main Methods:
- Review of existing literature on sickle cell disease and its cardiopulmonary manifestations.
- Analysis of clinical data pertaining to cardiovascular phenotypes in adult SCD patients.
- Synthesis of information on morbidity and mortality associated with cardiac complications.
Main Results:
- Cardiopulmonary complications are the primary causes of morbidity and mortality in sickle cell disease.
- Common complications include cardiomyopathy, diastolic dysfunction, pulmonary hypertension (PH), and sudden cardiac death.
- Adult SCD patients experience chronic degenerative changes impacting multiple organs, especially the heart and lungs.
Conclusions:
- Cardiopulmonary complications represent a critical aspect of sickle cell disease management in adults.
- Recognizing sickle-related cardiovascular phenotypes is essential for timely medical intervention.
- Proactive screening and diagnosis can improve outcomes for patients with sickle cell disease.
Abstract:
Sickle cell disease (SCD) is the most common inherited blood disorder in the United States, and a global health problem. Pathological features of the abnormal hemoglobin (HbS) result in 2 hallmarks of the disease - recurrent episodes of acute microvascular occlusion and chronic hemolytic anemia - that inflict continuous and insidious damage to multiple organs. With improved childhood survival, SCD in adults has evolved into a chronic degenerative disease with underlying damage to multiple organs including the heart and lungs. Cardiopulmonary complications, including cardiomyopathy, diastolic dysfunction, pulmonary hypertension (PH), and sudden cardiac death are the most common causes of morbidity and mortality. Awareness of the sickle-related cardiovascular phenotypes is important for screening, early diagnosis, and intervention of cardiac complications in this disorder.
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