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Evidence of endothelial dysfunction in patients with functionally univentricular physiology before completion of the Fontan operation

Published online by Cambridge University Press:  14 April 2005

Maria A. Binotto ,
Nair Y. Maeda  and
Antonio A. Lopes
Maria A. Binotto
Affiliation:
Department of Paediatric Cardiology and Adult Congenital Heart Disease, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil
Nair Y. Maeda
Affiliation:
Pró-Sangue Foundation, São Paulo, Brazil
Antonio A. Lopes
Affiliation:
Department of Paediatric Cardiology and Adult Congenital Heart Disease, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil
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Abstract

Introduction: Postoperative thrombosis after a cavopulmonary connection has been widely described. Abnormalities in coagulation seem to occur early in the course of patients with functionally univentricular physiology, and may precede surgery. Endothelial abnormalities due to chronic hypoxia, and hyperviscosity, may contribute to this scene. The purpose of our study was to investigate if patients with a superior cavopulmonary connection have altered levels of endothelial and coagulative markers in the plasma. Methods: We compared findings in 10 patients, aged from 4 to 19 years, with 6 age-matched normal controls. We measured levels of von Willebrand factor antigen, thrombomodulin, tissue-type plasminogen activator, plasminogen activator inhibitor-1 and d-dimer in the plasma using enzyme-linked immunosorbent assay. Results: We found increased levels of von Willebrand factor antigen (p = 0.01), tissue-type plasminogen activator (p = 0.01), and decreased levels of thrombomodulin (p = 0.008) in the patients when compared to controls, while levels of plasminogen activator inhibitor-1 were not different. Values of d-dimer were within the reference range. Levels of tissue-type plasminogen activator had a positive correlation with von Willebrand factor antigen (r = 0.66, p = 0.008). Conclusions: Altered levels of endothelial markers in the plasma, in the presence of normal levels of d-dimer, suggest that endothelial dysfunction may precede the occurrence of intravascular coagulation and thrombosis in patients with functionally univentricular physiology. These observations may have therapeutical implications.

Keywords

Functionally single ventricleendotheliumcavopulmonary connection
Type
Original Article
Information
Cardiology in the Young , Volume 15 , Issue 1 , January 2005 , pp. 26 - 30
Copyright
2005 Cambridge University Press

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