Background: Changes in circulating von Willebrand factor (vWF) have been widely used for evaluating the severity of endothelial dysfunction in vascular disorders. In pulmonary hypertension, quantitative and structural abnormalities in circulating von Willebrand factor have been identified. We therefore hypothesized that these abnormalities could have prognostic implications.

Patients and methods: We studied 30 consecutive medically treated patients with primary (n = 11) or secondary precapillary pulmonary hypertension associated with congenital heart disease (n = 16) or schistosomiasis (n = 3). Plasma antigenic activity of vWF (vWF:Ag) was measured by electroimmunodiffusion. The relative concentration of low molecular weight vWF multimers (vWF:LMW/Total) was determined by Western immunoblotting. Results of initial evaluation were analyzed at the end of the first and third years of follow-up.

Results: Baseline vWF:Ag activity (P <0.0002) and the vWF:LMW/Total ratio (P <0.005) were higher in patients who died during the first year than in survivors. All patients with vWF:Ag activity >250% or a vWF:LMW/Total ratio >70% died in the first year. All 7 patients with vWF:Ag activity <100% were alive at the end of 3 years of follow-up. A vWF:LMW/Total ratio >68% was 67% sensitive and 95% specific for 1-year mortality, with an overall predictive value of 80%. Both vWF:Ag levels and mortality were greater in the patients with primary pulmonary hypertension than in patients with secondary pulmonary hypertension.

Conclusion: Patients with pulmonary hypertension who have abnormalities in circulating vWF have reduced 1-year survival. This might affect decisions such as patient assignment to lung transplantation.