Frequency of acquired von Willebrand’s disease in children with congenital heart disease

Abstract

Objective — Bleeding tendency of paediatric patients with congenital heart disease has been well recognized.The underlying pathologies of this bleeding tendency have been studied extensively and many causes were identified. Among these causes, we aimed to find the frequency of acquired von Willebrand’s disease (AvWD) in children with congenital heart disease.

Material and methods — Forty-nine children with different forms of congenital cardiopathies who were assigned for surgery, are included in the study. Serum von Willebrand factor antigen level as well as ristocetin cofactor agglutination ratios were determined preoperatively and at one week and 6 months postoperatively.

Results — Six patients (12.2%) were found to have AvWD. However, we found no relation between bleeding tendency and AvWD status.

Conclusion — Although frequency of von Willebrand factor deficiency is higher in children with congenital heart disease than in the normal population, this condition does not result in adverse clinical outcomes like increased bleeding tendency during operation.

Key Words:

• acquired von Willebrand’s disease
• bleeding
• congenital heart disease