Cardiovascular Abnormalities in Sickle Cell Disease

Sickle cell disease is characterized by recurrent episodes of ischemia-reperfusion injury to multiple vital organ systems and a chronic hemolytic anemia, both contributing to progressive organ dysfunction. The introduction of treatments that induce protective fetal hemoglobin and reduce infectious c...

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Bibliographic Details
Published in:	Journal of the American College of Cardiology 2012-03, Vol.59 (13), p.1123-1133
Main Authors:	Gladwin, Mark T., MD, Sachdev, Vandana, MD
Format:	Article
Language:	eng
Subjects:	Anemia, Sickle Cell - complications Anemia, Sickle Cell - physiopathology Anemias. Hemoglobinopathies Biological and medical sciences Cardiology Cardiology. Vascular system Cardiovascular Cardiovascular Diseases - diagnosis Cardiovascular Diseases - etiology Cardiovascular Diseases - physiopathology cell disease Diseases of red blood cells Emergency medical care Heart attacks Hematologic and hematopoietic diseases Humans Internal Medicine Medical sciences Mortality sickle Sickle cell anemia
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Summary:	Sickle cell disease is characterized by recurrent episodes of ischemia-reperfusion injury to multiple vital organ systems and a chronic hemolytic anemia, both contributing to progressive organ dysfunction. The introduction of treatments that induce protective fetal hemoglobin and reduce infectious complications has greatly prolonged survival. However, with increased longevity, cardiovascular complications are increasingly evident, with the notable development of a progressive proliferative systemic vasculopathy, pulmonary hypertension (PH), and left ventricular diastolic dysfunction. Pulmonary hypertension is reported in autopsy studies, and numerous clinical studies have shown that increased pulmonary pressures are an important risk marker for mortality in these patients. In epidemiological studies, the development of PH is associated with intravascular hemolysis, cutaneous leg ulceration, renal insufficiency, iron overload, and liver dysfunction. Chronic anemia in sickle cell disease results in cardiac chamber dilation and a compensatory increase in left ventricular mass. This is often accompanied by left ventricular diastolic dysfunction that has also been a strong independent predictor of mortality in patients with sickle cell disease. Both PH and diastolic dysfunction are associated with marked abnormalities in exercise capacity in these patients. Sudden death is an increasingly recognized problem, and further cardiac investigations are necessary to recognize and treat high-risk patients.
ISSN:	0735-1097 1558-3597