Article first published online: 7 SEP 2012 | DOI: 10.1111/j.1540-8175.2012.01816.x
Mini-Abstract
In a 28-year-old female, with symptoms and clinical signs of right heart failure, transthoracic echocardiography and abdominal, three-dimensional, contrast-enhanced, computed tomography revealed a rare type of portopulmonary hypertension. She had a severe pulmonary artery hypertension associated with an unusual (and, to our knowledge, not previously described) pattern of Abernethy malformation, with absence of the portal vein, but partial, extrahepatic portosystemic shunt, as the superior mesenteric vein went to the liver, but the splenic vein drained into the inferior vena cava.