Prognostic value of von Willebrand factor in adult patients with congenital heart disease
- 18 March 2020
- Vol. 106 (12), 910-915
- https://doi.org/10.1136/heartjnl-2019-316007
Abstract
Objectives von 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. Methods We 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. Results The 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) (pConclusions High 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.Keywords
This publication has 28 references indexed in Scilit:
- Hepatocellular Carcinoma in an Adult with Repaired Tetralogy of FallotCongenital Heart Disease, 2012
- Von Willebrand factor as new noninvasive predictor of portal hypertension, decompensation and mortality in patients with liver cirrhosisJournal of Hepatology, 2012
- Plasma von Willebrand Factor Levels Are an Independent Risk Factor for Adverse Events Including Mortality and Major Bleeding in Anticoagulated Atrial Fibrillation PatientsJournal of the American College of Cardiology, 2011
- Bacterial DNA translocation is associated with systemic circulatory abnormalities and intrahepatic endothelial dysfunction in patients with cirrhosisJournal of Hepatology, 2010
- Levels of von Willebrand factor antigen and von Willebrand factor cleaving protease (ADAMTS13) activity predict clinical events in chronic heart failureThrombosis and Haemostasis, 2009
- von Willebrand factor, endothelial dysfunction, and cardiovascular diseaseJournal of Thrombosis and Haemostasis, 2006
- Systemic Endothelial Dysfunction in Adults With Cyanotic Congenital Heart DiseaseCirculation, 2005
- Vascular Endothelial Dysfunction and Mortality Risk in Patients With Chronic Heart FailureCirculation, 2005
- Nitric Oxide Regulates Exocytosis by S-Nitrosylation of N-ethylmaleimide-Sensitive FactorCell, 2003
- High plasma levels of von Willebrand factor as a marker of endothelial perturbation in cirrhosis: Relationship to endotoxemiaJournal of Hepatology, 1996