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Cardiomyopathy in Sickle Cell Disease
Harsimran Kaur1, Fahad Aurif2, Mahdi Kittaneh3
1Internal Medicine, California Institute of Behavioural Neurosciences and Psychology, Fairfield, USA.
Cureus
|September 14, 2020
Summary
Sickle cell disease (SCD) causes red blood cells to sickle, obstructing blood flow and leading to anemia. This results in unique cardiac changes, including left ventricular dilation, but typically without systolic dysfunction.
Area of Science:
- Hematology
- Cardiology
- Genetics
Background:
- Sickle cell disease (SCD) is an inherited blood disorder caused by a point mutation in the beta-globin gene, leading to abnormal hemoglobin S.
- Sickle-shaped red blood cells (RBCs) obstruct blood vessels, causing organ ischemia, tissue fibrosis, and chronic anemia.
- Chronic anemia in SCD necessitates frequent blood transfusions, increasing risks of iron overload and volume overload.
Purpose of the Study:
- To elucidate the unique mechanisms driving cardiac remodeling in sickle cell disease.
- To characterize the specific features of SCD-related cardiomyopathy.
Main Methods:
- This study reviews the pathophysiology of SCD and its impact on cardiovascular health.
- Analysis of the relationship between chronic anemia, blood transfusions, and cardiac structural changes.
Main Results:
- SCD leads to chronic anemia and a high cardiac output state, causing cardiac remodeling.
- Blood transfusions in SCD patients contribute to iron and volume overload, promoting left ventricular (LV) dilation.
- SCD-related cardiomyopathy presents with features of restrictive cardiomyopathy and LV dilation, but typically lacks systolic dysfunction.
Conclusions:
- SCD-related cardiomyopathy is a distinct condition characterized by LV dilation and restrictive features, driven by chronic anemia and transfusion-related complications.
- Despite LV dilation, systolic function is generally preserved in sickle cell disease patients.
Keywords:
cardiomyopathychronic anemiadiastolic dysfunctioniron overload cardiomyopathymicrovascular occlusionpulmonary hypertenionrestrictive cardiomyopathysickle cell diseaseMore Related Videos
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