Circulating von Willebrand factor antigen as a predictor of short-term prognosis in pulmonary hypertension

To determine the potential value of plasma von Willebrand factor antigenic activity (vWF:Ag) and other commonly measured clinical variables for predicting which patients with precapillary pulmonary hypertension would be unlikely to survive 1 year.

Prospective clinical study. The data obtained at the beginning of the study were analyzed at the end of the first year of follow-up.

Forty patients aged 1.2 to 45 years (median, 24 years) entered the study. Eleven patients had primary pulmonary hypertension, and in the remaining ones, pulmonary vascular disease was associated with antiphospholipid syndrome (n = 1), collagen vascular disease (n = 1), schistosomiasis (n = 3), or congenital heart defects (Eisenmenger's syndrome) (n = 24). Plasma vWF:Ag was determined by electroimmunodiffusion, and the results were expressed as the percentage of activity.

Seven of 11 patients with primary pulmonary hypertension but only 4 of 29 patients with secondary pulmonary hypertension died during the follow-up period (p < 0.005). Initial vWF:Ag values were significantly higher in the nonsurvivor group in comparison with the survivors (256.6+/-85.3% and 132.0+/-59.3% activity, respectively; p < 0.0001). The likelihood of fatal outcome as a function of plasma vWF:Ag levels was estimated for primary and secondary pulmonary hypertensive patients using logistic regression analysis. Decreased life expectancy was significantly related to high vWF:Ag levels and an established diagnosis of primary pulmonary hypertension. A plasma vWF:Ag of >240% (p = 0.003) was 54% sensitive and 93% specific for identifying patients who were unlikely to survive 1 year, with an overall predictive value of 75%. No other variables correlated significantly with survival.

Plasma vWF:Ag seems to be a useful biochemical index for predicting short-term prognosis in patients with pulmonary hypertension. In contrast to hemodynamic and histopathological predictors of survival, vWF:Ag does not require invasive techniques to be determined. In light of the possibility of false-negative results, serial measurements should be performed over time in patients with vWF:Ag of <240%. This observation proved helpful in two patients in this study.