Background: Those with cyanotic heart disease have an elevated bleeding risk but also are hypercoaguable. Treating haemodynamically significant thrombi in this unique cohort poses a monumental challenge. Case A 29-year-old women with tricuspid atresia and left pulmonary artery atresia presented with superior caval vein syndrome. She had a right modified Blalock-Taussig shunt as a neonate. A left modified Blalock-Taussig shunt performed later failed to establish flow to her left lung. At age 5, she had a Fontan procedure to the right lung but could not tolerate the physiology and had a low cardiac output syndrome. The Fontan was taken down and she was left with a Glenn anastamosis to the right pulmonary artery. She did well for years until she had dyspnea, upper extremity oedema and "facial fullness". On examination she was tachycardic, hypotensive, and more desaturated than baseline. She also had facial plethora. Decision-making Echocardiogram showed a large 9 × 3 mm nearly occlusive thrombus in the superior caval vein at the bifurcation of the left and right innominate veins. An emergent venogram confirmed the location and size of the thrombus. Given the thrombus burden and potential for distal embolisation through the Glenn to the single functional lung, we chose to treat the patient with thrombolytics. She had uncomplicated ICU course and was sent home on warfarin. Follow-up echocardiogram showed complete resolution of clot.
Conclusion: This case shows the importance of history and physical exam in caring for this complex cohort of adult patients with CHD.
Keywords: Adult congenital; Fontan; cyanotic heart disease; thrombolytics.