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Acute thrombosis of an extracardiac Fontan conduit
  1. J A E Kammeraad,
  2. N Sreeram

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A 5 year old girl had undergone a Fontan procedure for tricuspid atresia. An extracardiac conduit (16 mm diameter Vascutec) was used to make the inferior vena cava to pulmonary artery connection. Three months post-surgery, and within two weeks of discontinuing coumadin medication and commencing oral aspirin, she presented with acute abdominal pain and shock. Echocardiography and computed tomographic (CT) scans confirmed thrombosis of the inferior vena cava. At angiography, complete occlusion of the conduit was demonstrated (below left). The thrombus was passed with an 0.035 inch Cordis guidewire. Serial balloon dilation of the conduit was undertaken to re-establish patency. Additional thrombi in the left pulmonary arterial system were also crossed using various guidewire and balloon combinations, and patency of all segmental vessels was established (below centre). Finally, stent implantation was undertaken to relieve residual conduit stenosis, and intravenous streptokinase (1000 units/kg/hour for six hours) was administered. Repeat angiography 48 hours later confirmed resolution of all clots, and complete patency of the inferior caval and left pulmonary arterial system (below right). The patient was discharged from hospital after reinstituting oral coumadin treatment.

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Contrast injection via the femoral vein confirms complete obstruction of the inferior vena cava at its connection to the extracardiac conduit (arrow).

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The thrombus has been passed with an end hole catheter. Contrast injection in the superior vena cava confirms complete occlusion of the left pulmonary artery, in which two stents have been implanted (arrow).

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A stent has been placed in the Fontan conduit (arrow). The left pulmonary artery system is fully recanalised.