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Related Experiment Video

Updated: May 23, 2026

Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry

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Published on: November 5, 2019

Cardiovascular abnormalities in sickle cell disease.

Mark T Gladwin¹, Vandana Sachdev

¹Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh, 3459 Fifth Avenue, Montefiore Hospital, Pittsburgh, PA 15213, USA. gladwinmt@upmc.edu

Journal of the American College of Cardiology

|March 24, 2012

Summary

Sickle cell disease survival is improving, but cardiovascular issues like pulmonary hypertension and diastolic dysfunction are rising concerns. These conditions significantly increase mortality risk and require further investigation for better patient outcomes.

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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload

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Published on: March 14, 2017

Area of Science:

Cardiology
Hematology
Pulmonology

Background:

Sickle cell disease (SCD) involves organ damage from ischemia-reperfusion and chronic hemolytic anemia.
Improved treatments have increased patient survival, leading to a rise in cardiovascular complications.
Pulmonary hypertension (PH) and left ventricular diastolic dysfunction are increasingly prevalent in long-lived SCD patients.

Purpose of the Study:

To highlight the growing burden of cardiovascular complications in sickle cell disease.
To emphasize pulmonary hypertension and diastolic dysfunction as significant risk markers for mortality.
To underscore the need for further cardiac investigations in high-risk SCD patients.

Main Methods:

Review of autopsy and clinical studies on sickle cell disease patients.
Epidemiological analysis of factors associated with PH development.
Examination of the relationship between anemia, cardiac remodeling, and diastolic dysfunction.

Main Results:

Pulmonary hypertension is a common finding in SCD, linked to hemolysis, organ dysfunction, and mortality.
Chronic anemia causes cardiac dilation and increased left ventricular mass, often leading to diastolic dysfunction.
Both PH and diastolic dysfunction are associated with reduced exercise capacity and are strong predictors of mortality.

Conclusions:

Cardiovascular complications, particularly PH and diastolic dysfunction, are major concerns in the aging sickle cell disease population.
These conditions are critical risk factors for mortality and impaired quality of life.
Early recognition and management of cardiac issues are essential for improving outcomes in sickle cell disease.