Objective: Abernethy malformation (congenital extrahepatic portosystemic shunt) is a rare anomaly of the splanchnic venous system. Though rare, it is an important cause of pulmonary artery hypertension (PAH) which is often missed. All patients with PAH should be carefully evaluated for presence of Abernethy malformation before labelling them as Idiopathic PAH. Methods: This is a retrospective analysis of prospectively collected data. We reviewed the data of all patients referred to our center for evaluation of PAH.
10 patients were diagnosed to have an extra hepatic portocaval malformation. We reviewed their presentation, diagnosis, catheterization data, intervention and their outcome along with review of literature. Results: 10/104 patients with pulmonary hypertension and no intra or extracardiac shunt were found to have extrahepatic portocaval shunt (EHPCS). 3 patients had EHPCS type…
