Repeated Obstructive Prosthetic Valve Thrombosis Suspected to be Associated with Hypereosinophilic Syndrome in Complete Atrioventricular Septal Defect

Abstract

Hypereosinophilic syndrome is a rare condition defined as persistent eosinophilia (>1,500 cells/mm³) without a primary cause.Cardiac involvement such as valve insufficiency occurs in more than 75% of patients with this syndrome. The reports of adult patients with valve insufficiency related to this syndrome exist, but no pediatric patients are reported.
Herein is reported the case of a pediatric patient suffered from repeated obstructive valve thrombosis suspected to be associated with hypereosinophilic syndrome.
Case: Four-years-old girl with polysplenia, SA, CAVSD (Rastelli C), azygous continuation of IVC, and congenital biliary atresia received PA banding at 1 month, Kasai operation at 2 months, and CAVSD repair at 1 year and 1 month.
Left atrioventricular valve replacement (lt. AVVR) was performed at 4 years of age due to the persistent left atrioventricular regurgitation. Inspite of oral anticoagulation, the patient developed 5 recurrences of valve thrombosis, required 2 thrombolysis therapy sessions and 3 emergency operations. Blood examination showed elevation of eosinophil count to 6000-8000/mm³ from 60/mm³ preoperatively. The hypereosinophilia was strongly suspected as the cause of repeated valve thrombosis. We suspected the antibiotics as the cause of hypereosinophilia, and stopped administration 1 month after re-re-lt. AVVR. The eosinophil count was gradually normalized to 1000/mm³ and she was discharged in good condition.