Recurrent placental chorioangioma in the setting of Fontan circulation: A case report

Introduction: The incidence of cardiac disease in pregnancy continues to increase, particularly pregnancy with complex congenital heart diseases. Fontan circulation is a unique circulation surgically created for individuals with single ventricle physiology, effects of which on developing placenta and fetus are not completely understood. Chronic placental hypoxia in Fontan circulation might explain increased incidence of chorioangioma in those patients. Case Report: We present the case of a 28-year-old female with congenital pulmonary atresia and an extracardiac Fontan circulation with fenestrations and chronic right to left shunting, with baseline oxygen saturation of 87–91%. She had three successful pregnancies. Placentomegaly has been noted in all her pregnancies, with recurrent chorioangioma noted in her first and last pregnancy. Conclusion: Fontan circulation with chronic hypoxia may be associated with increased risk of giant chorioangioma with potential for adverse perinatal outcomes. Ultrasound screening for large chorioangioma therefore may be beneficial in this subset of patients.