P1452 A rare case of embolic blindness in an adult patient with univentricular heart after fontan operation

Abstract

Abstract Background Thromboembolic complications following Fontan procedure are one of the major causes of mortality and morbidity among patients with univentricular heart. We may classify thromboembolic complications into two groups: systemic- and nonsystemic. We present a case of a patient with a manifestation of both. Case report 24-year old female, foreign student was admitted to Ophthalmology Department due to sudden blindness of the left eye. Performed studies revealed embolus in the left central retinal artery. Because the patient had the history of congenital heart defect she was referred to our department. Analysis of medical documentation showed that the patient has combined congenital heart defect including right ventricle hypoplasia, tricuspid atresia, pulmonic stenosis, ventricular septal defect, atrial septal defect and persistent ductus arteriosus. She had a history of two surgeries: modified left-side Blalock-Tausing connection (in 4th month of life) and total cavo-pulmonary connection with the closure of Blalock-Tausing and excision of interatrial septum (in 2,5 year of life). On admission she presented with left-eye blindness. She had no dyspnoea or palpitations. However she reported very severe migraines from a few days preceding the admission to hospital. Echocardiography revealed tricuspid atresia, hypoplastic right ventricle, preserved systolic and diastolic function of left ventricle, atrial septal defect with no restriction. We observed enlarged diameter of vena cava inferior with no respirophasic variations and massive thrombus in inside with very low flow. The connection between inferior vena cava and right pulmonary artery was working properly with no visible fenestration. We also observed a winding vessel between ascending aorta and truncus pulmonalis/pulmonary artery with continuous flow of blood from left to right side. Computer tomography confirmed the diagnosis of massive thrombosis of vena cava inferior. Patient had anticoagulation started. Two days later we the flow in inferior vena cava returned – we did not observe any thrombus. Patient was referred to catheterization and had chronic anticoagulation initiated. Three months follow-up revealed no changes in clinical condition of a patient. Discussion The palliative Fontan operation predispose to the increased risk of thromboembolic complication. Despite the risk factors of such complications have been identified, there are no guidelines about prophylactic anticoagulation in that group of patients. As a result, chronic anticoagulation is initiated in patients who have other indications e.g. atrial fibrillation. It seems that it would be beneficial to define the group of patients after Fontan procedure with indications to chronic anticoagulation therapy despite arrhythmias.