Prognostic value of von Willebrand factor in adult patients with congenital heart disease

Abstract

Objectivesvon Willebrand factor (vWF) has prognostic value in patients with heart failure (HF) and in those with liver disease. Liver congestion, due to right-sided HF (RHF), is one of the major clinical pathophysiologic manifestations in adults with congenital heart disease (ACHD). The present study’s purpose was to clarify the prognostic value of plasma levels of vWF antigen (vWF:Ag) in ACHD.MethodsWe measured vWF:Ag (%) in 382 consecutive patients (20 unrepaired cyanotic ACHD, 172 Fontan patients and 190 ACHD after biventricular repair) and compared the results with the clinical profiles and prognosis.ResultsThe plasma vWF:Ag level was 130±53 (normal range: 55%–190%), and 48 patients (13%) showed high levels of vWF:Ag (≥190%). Older age, Fontan circulation, higher central venous pressure, lower arterial oxygen saturation and lower plasma levels of albumin were independently associated with high log (vWF:Ag) (p<0.05–0.0001). During the follow-up of 2.4±1.4 years, 15 patients died. High log (vWF:Ag) predicted the all-cause mortality (HR 1.63 per 0.1, 95% CI 1.40 to 1.96, p<0.0001). Specifically, patients with high vWF:Ag (≥165%) had a substantially higher risk of all-cause mortality (HR 56.4, 95% CI 11.4 to 1020, p<0.0001), and this prognostic value was independent of plasma levels of brain-type natriuretic peptide.ConclusionsHigh vWF:Ag may reflect RHF severity and related liver dysfunction with a strong prognostic value of all-cause mortality in ACHD. Thus, vWF:Ag might be an excellent biomarker for monitoring ACHD with RHF.

Intensive Care Unit Management of the Adult with Congenital Heart Disease

“…A retrospective cohort study showed MELD-XI remained a significant predictor of MACE in ACHD patients after adjusting for patient characteristics, including sex, functional status, estimated glomerular filtration rate, and cardiac function, while predictive modeling revealed it to be most important variable predicting MACE in this population [70]. Biomarkers may also assist in risk stratification of patients with ACHD and liver complications, with prime candidates including galectin-3 and von Willebrand factor antigen [71,72].…”

Is vWF really a very wonderful factor for risk stratification in adults with congenital heart disease?

“…Plasma von Willebrand factor (vWF) is certainly a nice candidate for a prognostic marker(figure 1) As Ohuchi et al 6 outline, in this issue of Heart , vWF is easily and inexpensively obtainable as a plasma sample and is known to be elevated with endothelial dysfunction or hepatic fibrosis. This latter is an important consideration in some CHD pathologies, where significant right ventricular dysfunction is a key feature and contributes to morbidity and mortality.…”