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Cardiomyopathy in Sickle Cell Disease

Authors
  • Kaur, Harsimran1
  • Aurif, Fahad2
  • Kittaneh, Mahdi3
  • Chio, Jeoffrey Patrick G4
  • Malik, Bilal Haider1
  • 1 Internal Medicine, California Institute of Behavioural Neurosciences and Psychology, Fairfield, USA
  • 2 General Surgery, California Institute of Behavioural Neurosciences and Psychology, Fairfield, USA
  • 3 General and Laparoscopic Surgery, California Institute of Behavioural Neurosciences and Psychology, Fairfield, USA
  • 4 Family Medicine, California Institute of Behavioural Neurosciences and Psychology, Fairfield, USA
Type
Published Article
Journal
Cureus
Publisher
Cureus
Publication Date
Aug 08, 2020
Volume
12
Issue
8
Identifiers
DOI: 10.7759/cureus.9619
PMID: 32923220
PMCID: PMC7478929
Source
PubMed Central
Keywords
License
Green
External links

Abstract

Sickle cell disease (SCD) is an inherited disorder that occurs due to point mutation in the beta-globin chain resulting in the production of hemoglobin S that tends to become rigid and sickle-shaped under low oxygen concentration. These sickle-shaped red blood cells (RBCs) obstruct the blood vessels leading to reduced blood flow to the organs, causing ischemia and tissue fibrosis. These sickle RBCs being abnormal in shape are frequently sequestered by the spleen, creating a state of chronic anemia in the body. This chronic anemia leads to a high cardiac output state causing cardiac remodeling. To tackle chronic anemia, patients are frequently treated with blood transfusions that makes them more prone to the risk of iron overload (from newly transfused RBCs and iron release from the RBCs that just got sequestered as well as from volume overload) and volume overload causing left ventricular (LV) dilation. The above-mentioned mechanism of cardiac hypertrophy, along with LV dilation together, makes SCD-related cardiomyopathy unique cardiomyopathy with features of restrictive cardiomyopathy with LV dilation. It is interesting to note here that even though there is a presence of LV dilatation, Systolic dysfunction is very uncommon in SCD-related cardiomyopathy.

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