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