Von Willebrand’s Factor as Long-Term Prognostic Factor in Adult Congenital Heart Disease

Objectives: Von Willebrand’s Factor (vWF) as a marker of endothelial dysfunction is associated with adverse outcome in cardiovascular disease, such as coronary artery disease, atrial fibrillation, pulmonary, or arterial hypertension and heart failure due to noncongenital heart disease. We aimed to evaluate the prognostic value of vWF on heart failure and death (HF/Death) in adult congenital heart disease (ACHD).

Methods: We evaluated 190 patients (mean age at inclusion 33.5 ± 11.0 years, 59.9% male) Mean follow-up was 7.5 ± 2.2 years. Cox’s proportional survival analysis was used to assess the relationship between baseline characteristics and adverse outcome. Receiver operative characteristics were calculated from independent predictors. Youden’s index was calculated in parameters with significant discriminative power. Corresponding odds ratios are provided.

Result: In the total cohort, 33 (17.3%) patients experienced HF/Death. For multivariate analysis we used a model including vWF, NT-proBNP, age, albumin, creatinine, hemoglobin, hs-CRP, NYHA class, and disease complexity. In this model vWF (HR = 1.03 [CI: 1.018–1.037]), NT-proBNP (HR = 1.001 [CI: 1.001–1.001]), and creatinine (HR = 0.97 [CI: 0.96–0.99]) remained independent predictors, even when adjusted for NYHA-class, age, and disease complexity. Discriminative power was significant in NT-proBNP (AUC = 0.809) and vWF (AUC = 0.779), but not in creatinine (AUC = 0.555). Optimal cut-off points were 300 ng/L for NT-proBNP (OR = 23.5 [CI: 3.9–20.2]) and 137% for vWF (OR = 8.8 [CI: 9.3–59.2]). Patients presenting with values beyond the cut-off points of NT-proBNP and vWF had the highest risk of HF/Death (OR = 28.2 [CI: 9.3–87.9]).

Conclusion: In ACHD, vWF is an independent predictor of HF/Death. Though NT-proBNP was the strongest single predictor of HF/Death, vWF provides additive information regarding risk assessment.