Acquired von Willebrand factor abnormalities in adults with congenital heart disease: Dependence upon cardiopulmonary pathophysiological subtype

Hemostasis is often abnormal in adults with congenital heart disease, and von Willebrand factor abnormalities have been reported in this patient population. We sought to determine the prevalence, type, and severity of the von Willebrand factor abnormality, and its relationship to three pathophysiological variables; cyanosis, pulmonary vascular disease, and turbulent blood flow. This prospective study comprised 76 unoperated congenital heart disease patients aged 20 to 68 years (mean = 34 years). There were 44 cyanotic and 32 acyanotic patients. Twenty-seven cyanotic and 6 acyanotic patients had pulmonary vascular disease, 31 cyanotic and 16 acyanotic patients had turbulent blood flow, and 11 patients were acyanotic without pulmonary vascular disease or turbulent flow. The largest plasma von Willebrand factor multimers were relatively decreased or absent in 77% of cyanotic versus 41% of acyanotic patients (p <.001); in 76% of patients with, versus 51% without, pulmonary vascular disease (p <.029); and in 72% of patients with, versus 45% without, turbulent flow (p <.016). Von Willebrand factor multimers were normal in all 11 acyanotic patients without pulmonary vascular disease or turbulent blood flow. Von Willebrand factor multimer abnormalities normalized after reparative surgery in five patients. Depletion of the largest plasma von Willebrand factor multimers is common in adults with congenital heart disease. Cyanosis, pulmonary vascular disease, and turbulent flow are determinants of the abnormality that is acquired and reversible.