2019 Volume 1 Issue 10 Pages 466-467
Although the etiology of Fontan-associated liver disease (FALD) remains unclear, increased central venous pressure (CVP) leads to increased hepatic afterload, which may ultimately result in cirrhosis, splenomegaly and portal hypertension.1 In contrast, portal hypertension is 1 possible risk factor for splenic artery aneurysm (SAA).2
A 27-year-old woman with polysplenia and single ventricle had undergone total cavopulmonary connection using a 14-mm conduit at the age of 6 years. Her clinical status deteriorated with massive ascites 15 years after Fontan completion. The conduit was exchanged for a 20-mm size at the age of 24 years, but ascites and esophageal varices developed. One year after the surgery, abdominal ultrasonography indicated a large SAA (Figure A–C), which was successfully treated with coil embolization. Two years later, computed tomography showed a new SAA (Figure D). Furthermore, multiple SAA were newly demonstrated on selective angiography (Figure E). SAA is very rare, but can develop as a fatal result of FALD, especially in patients who have high CVP, splenomegaly and portal hypertension.
(A) Abdominal ultrasonography showed a large splenic arterial aneurysm, which was 64.1×61.6 mm in size. (B) Coronal computed tomography (CT). (C) Selective angiography. (D) Coronal CT 2 years after treatment. (E) Selective angiography showed multiple small splenic aneurysms (red arrows).
The authors declare no conflicts of interest.