Acute arterial embolism of left lower extremity caused by paradoxical embolism in Ebstein's anomaly

Abstract

Introduction:Ebstein's anomaly is a benign and stable congenital heart disease for asymptomatic patients. Despite a low incidence of Ebstein's anomaly (EA), patients’ quality of life can be badly affected by EA without positive surgical intervention. Especially EA is associated with other congenital heart disease, such as the atrial septal defect, patent foramen ovale, and arterial embolism exclude other reasons, it is often considered to be the consequence of paradoxical embolism, and surgical intervention must be conducted.Case report:An 11-year-old girl falling off the bed suffered pain from left lower extremity. Echocardiographic evaluation revealed an EA, severe tricuspid regurgitation, and secundum atrial septal defect. Both left leg amputation and cardiac surgery were conducted after recovery. Under the condition of anesthesia cardiopulmonary bypass extracorporeal circulation, atrial septal defect repair and Cone reconstruction of the tricuspid valve were performed. Patient recovered well and left hospital smoothly.Discussion:EA is a rare and complex congenital cardiac malformation. There are about 80% to 90% of EA patients with combined atrial septal defect and patent foramen ovale. Sudden arterial occlusion is very rare especially in childhood. When thoracic roentgenoscopy, arterial blood gas analysis, coagulation test, and echocardiographic of lower extremity deep venous system are all normal, one should consider the possibility of a paradoxical embolism. If patients have the paradoxical embolism or worsening tricuspid regurgitation, the most suitable therapeutic regimen should be chosen according to patients’ condition. With surgical techniques and methods renewed continuously, cone reconstruction of the tricuspid valve has been confirmed in clinical trials, which can use its own tissues to form not only central bloodstream, but also the coaption between leaflet and leaflet.

Recurrent venous thrombosis in a patient with Ebstein’s anomaly

“… 8 A common complication of Ebstein’s anomaly in adults includes paradoxical embolism, which is an originally venous thrombus that transverses through a right-to-left shunt and enters the systemic circulation. 5 …”

“… 3 , 4 Ebstein’s anomaly is a rare congenital heart disease that may be associated with right-to-left shunts that predisposes to paradoxical embolization. 5 Anticoagulant therapy is the initial treatment for venous thrombosis. Patients usually receive long-term anticoagulation therapy, such as warfarin or direct oral anticoagulants, which include dabigatran and rivaroxaban, to prevent recurrent thrombosis, embolism, and other late complications.…”