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Markers of endothelial dysfunction and severity of hypoxaemia in the Eisenmenger syndrome

Published online by Cambridge University Press:  16 September 2005

Rosangela de P. S. Soares ,
Nair Y. Maeda ,
Sérgio P. Bydlowski  and
Antonio Augusto Lopes
Rosangela de P. S. Soares
Affiliation:
Pro-Sangue Foundation, Heart Institute, School of Medicine, University of São Paulo, São Paulo, Brazil
Nair Y. Maeda
Affiliation:
Pro-Sangue Foundation, Heart Institute, School of Medicine, University of São Paulo, São Paulo, Brazil
Sérgio P. Bydlowski
Affiliation:
Pro-Sangue Foundation, Heart Institute, School of Medicine, University of São Paulo, São Paulo, Brazil
Antonio Augusto Lopes
Affiliation:
Department of Pediatric Cardiology and Adult Congenital Heart Disease, Heart Institute, School of Medicine, University of São Paulo, São Paulo, Brazil
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Abstract

Endothelial dysfunction has been reported in hypoxaemic patients with the Eisenmenger syndrome, but a direct correlation between levels of endothelial markers and the severity of hypoxaemia has not been explored. With this in mind, we compared the levels in the plasma of tissue-type plasminogen activator, thrombomodulin, and von Willebrand factor in 25 patients with the Eisenmenger syndrome. They had a median age of 31 years, and were divided into 2 groups according to their recent clinical history. Thus, 18 patients were stable, being in functional class II or III, seen as outpatients, and having peripheral saturations of oxygen of 89 plus or minus 5 percent. In contrast, 7 patients were unstable, showing episodes of symptoms placing them in functional class IV, requiring care in hospital, and manifesting saturations of oxygen of 77 plus or minus 5 percent. We were able to follow 12 patients, 8 who were stable and 4 unstable, for 24 months. At baseline, levels of von Willebrand factor were higher in the unstable patients when compared to those who were stable, at 142 plus or minus 29 and 110 plus or minus 25 units per decilitre, respectively (p equal to 0.013). This correlated positively with oxygen desaturation (p less than 0.020). The structural abnormalities also correlated positively with the magnitude of hypoxaemia (p less than 0.020). Levels remained higher in the unstable patients throughout the period of follow-up (p equal to 0.006). Tissue-type plasminogen activator was also increased, at 14.3 plus or minus 8.4 versus 6.5 plus or minus 2.7 nanograms per millilitre in controls (p less than 0.001), whereas thrombomodulin was decreased, with values of 14.4 versus 34.6 nanograms per millilitre in controls (p for median values of less than 0.001). There was no correlation with saturations of oxygen. We conclude that measurement of von Willebrand factor, as compared with tissue-type plasminogen activator and thrombomodulin, will prove a better marker of endothelial response to hypoxaemia in patients with the Eisenmenger syndrome.

Keywords

Endotheliumpulmonary hypertensionvon Willebrand factorthrombomodulintissue-type plasminogen activator
Type
Original Article
Information
Cardiology in the Young , Volume 15 , Issue 5 , October 2005 , pp. 504 - 513
Copyright
© 2005 Cambridge University Press

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