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

Br J Haematol. 2012 Jun;157(6):664-73. doi: 10.1111/j.1365-2141.2012.09143.x. Epub 2012 Apr 25.

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.

Publication types

  • Review

MeSH terms

  • Anemia, Sickle Cell / metabolism*
  • Anemia, Sickle Cell / mortality
  • Anemia, Sickle Cell / pathology
  • Erythrocytes, Abnormal / metabolism
  • Erythrocytes, Abnormal / pathology
  • Humans
  • Hypertension, Pulmonary / metabolism
  • Hypertension, Pulmonary / mortality
  • Hypertension, Pulmonary / pathology
  • Iron Overload / metabolism*
  • Iron Overload / mortality
  • Iron Overload / pathology
  • Myocardial Ischemia / metabolism*
  • Myocardial Ischemia / mortality
  • Myocardial Ischemia / pathology
  • Myocardium / metabolism
  • Myocardium / pathology
  • beta-Globins / metabolism

Substances

  • beta-Globins