Aortic saddle embolism caused by right ventricle thrombus in a 2-year-old girl with Ebstein anomaly and Glenn shunt

A 2-year-old girl with Ebstein anomaly who had undergone Glenn operation presented with sudden onset of bilateral lower limb ischemia with severe pain and color changes (Fig. 1A). Transthoracic echocardiography showed an Ebstein anomaly and thrombus in the right ventricle. Arterial Doppler signals were inaudible but venous Doppler signals were audible in the both of the limbs. Multislice computed tomography showed obstruction at the iliac bifurcation of the aorta due to a saddle thrombus and a huge thrombus in the apex of right ventricle. In addition, there were occlusions in the right anterior and posterior tibial artery and right peroneal artery, and in the left external iliac artery (Fig. 2,3). After anticoagulation with intravenous heparin and urgenttransfemoral trombectomy distal perfusion and pulsation were completely restored in the left limb. However, pulsation did not return to normal in the dorsalis pedis and posterior distal arteries on the right side. Circulation of the right foot was not restored and gangrene developed (Fig. 1B). It was decided for amputation due to unsuccessful revascularization. However septicemia and pulmonary hemorrhage were developed and she died prior to amputation.

Sudden occlusion of the aorta is very rare especially in childhood and it usually occurs at its bifurcation and results in major threat to leg or patient survival, despite extensive collateralization. Prognosis in arterial occlusion is time dependent. The early recognition, supportive care, and prompt diagnosis are essential elements of management.