Caring for the orphan's orphan: treatment of patients with portopulmonary hypertension.

If this were not enough, these patients also have pulmonary arterial hypertension (PAH). The coexistence of portal hypertension and PAH frequently makes patients with this rare (or ‘‘orphan’’) condition medical ‘‘untouchables’’. PPHTN patients with progressive hepatic dysfunction and other complications are often deemed to be too ‘‘high-risk’’ for placement of a transjugular intrahepatic portosystemic shunt or liver transplantation, making them destined for a poor outcome. Even those who may be acceptable candidates for liver transplantation commonly face a complicated perioperative course.