Congenital portosystemic shunt with multiple splenic artery aneurysms: Reversing pulmonary hypertension and preventing aneurysm rupture

We report an unusual occurrence of multiple splenic artery aneurysms and splenomegaly in a young woman with severe pulmonary hypertension, secondary to a congenital portosystemic shunt (CPS). The splenic artery was occluded using an Amplatzer Duct Occluder-II device, and closure of the large intrahepatic CPS was achieved using a muscular ventricular septal defect occluder. There was resolution of splenomegaly with normal pulmonary artery pressures, a few months after the procedure.