Ohuchi H, Hayama Y, Miike H, et al. - To gain clarity regarding the prognostic significance of plasma levels of von Willebrand factor (vWF) antigen (vWF:Ag) in adults with congenital heart disease (ACHD), researchers undertook this investigation. The participants included 382 consecutive patients (20 unrepaired cyanotic ACHD, 172 Fontan patients and 190 ACHD following biventricular repair), in whom, measurements of vWF:Ag (%) were obtained. The results were compared with the clinical profiles and prognosis. Findings revealed that high vWF:Ag may indicate right-sided heart failure (RHF) severity as well as associated liver dysfunction with a strong prognostic worth for all-cause mortality in ACHD. A substantially higher risk of all-cause mortality was observed in patients with high vWF:Ag (≥165%) and the observed prognostic value was found to be independent of plasma levels of brain-type natriuretic peptide. Overall, to monitor ACHD with RHF, vWF:Ag might afford an excellent biomarker.