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[Von Willebrand factor and pulmonary endothelial dysfunction. Prognostic implications]

A A Lopes¹, N Y Maeda, S P Bydlowski

¹Instituto do Coração, Hospital das Clínicas da FMUSP e Fundação Pró-Sangue Hemocentro de São Paulo.

Arquivos Brasileiros De Cardiologia

|July 23, 1998

Summary

Elevated levels of von Willebrand factor (vWF) and its low molecular weight multimers (LMWM%) in pulmonary hypertension patients indicate endothelial dysfunction and predict poor one-year survival.

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Area of Science:

Cardiovascular Research
Hematology
Pulmonary Medicine

Context:

Pulmonary hypertension (PH) is a severe condition characterized by high blood pressure in the pulmonary arteries.
Endothelial cell dysfunction plays a critical role in the pathophysiology of PH.
Understanding biomarkers associated with PH prognosis is crucial for patient management.

Purpose:

To investigate quantitative and structural alterations in circulating von Willebrand factor (vWF) in patients with precapillary pulmonary hypertension.
To explore potential correlations between endothelial cell dysfunction, indicated by vWF changes, and short-term survival (one year) in these patients.

Summary:

Plasma antigenic activity of vWF (vWF:Ag) and low molecular weight multimers (LMWM%) were significantly increased in PH patients compared to healthy individuals.

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Degradation of the vWF subunit was also observed.

Higher baseline vWF:Ag and LMWM% levels were found in non-survivors compared to survivors.

LMWM% emerged as a significant predictor of one-year mortality via logistic regression analysis.

Impact:

The study highlights significant vWF abnormalities in PH, suggesting widespread pulmonary vascular endothelial cell dysfunction.
These vWF changes are associated with a poorer short-term prognosis in patients with pulmonary hypertension.
Findings may contribute to improved risk stratification and therapeutic strategies for PH.