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Sickle-cell disease and the heart: review of the current literature.

Abstract

Sickle cell disease (SCD) is an inherited chronic haemolytic anaemia whose clinical manifestations arise from the tendency of the haemoglobin to polymerize and deform red blood cells into the characteristic sickle shape due to a single nucleotide change in the β-globin. Vascular occlusion of small and large vessels can lead to chronic damage of multiple organs including brain, lung, bone, kidney, liver, spleen, and retina. However, the extent to which SCD impacts myocardial function is not very clear. Cardiovascular manifestations include both right and left ventricular systolic and diastolic dysfunction, elevated cardiac output, cardiomegaly and myocardial ischaemia. Progressive heart damage from iron overload occurs in patients requiring routine transfusion therapy. Pulmonary hypertension resulting from intravascular haemolysis has also been recognized as a major complication that independently correlates with survival. This review summarizes all available data for the heart complications in SCD to update the physicians for their appearance, diagnostic procedures and possible management.

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  • Publisher Full Text
  • Authors

    Voskaridou E, Christoulas D, Terpos E

    Institution

    Thalassaemia Centre, Laikon General Hospital, Athens, Greece. ersi_voskaridou@yahoo.com

    Source

    British journal of haematology 157:6 2012 Jun pg 664-73

    MeSH

    Anemia, Sickle Cell
    Erythrocytes, Abnormal
    Humans
    Hypertension, Pulmonary
    Iron Overload
    Myocardial Ischemia
    Myocardium
    beta-Globins

    Pub Type(s)

    Journal Article
    Review

    Language

    eng

    PubMed ID

    22530942