Document Detail


Cardiovascular abnormalities in sickle cell disease.
MedLine Citation:
PMID:  22440212     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Mark T Gladwin; Vandana Sachdev
Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Journal of the American College of Cardiology     Volume:  59     ISSN:  1558-3597     ISO Abbreviation:  J. Am. Coll. Cardiol.     Publication Date:  2012 Mar 
Date Detail:
Created Date:  2012-03-23     Completed Date:  2012-07-02     Revised Date:  2014-01-16    
Medline Journal Info:
Nlm Unique ID:  8301365     Medline TA:  J Am Coll Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1123-33     Citation Subset:  AIM; IM    
Copyright Information:
Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
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MeSH Terms
Descriptor/Qualifier:
Anemia, Sickle Cell / complications,  physiopathology*
Cardiovascular Diseases / diagnosis,  etiology,  physiopathology*
Humans
Grant Support
ID/Acronym/Agency:
P01 HL103455/HL/NHLBI NIH HHS; P01HL103455/HL/NHLBI NIH HHS; R01 HL096973/HL/NHLBI NIH HHS; R01 HL098032/HL/NHLBI NIH HHS; R01HL096973/HL/NHLBI NIH HHS; R01HL098032/HL/NHLBI NIH HHS
Comments/Corrections

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