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Updated: May 5, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
Portopulmonary hypertension
Nadine Al-Naamani1, Kari E Roberts
1Tufts Medical Center, 800 Washington Street, #257, Boston, MA 02111, USA.
Abstract:
Diagnosis of portopulmonary hypertension (PoPH), is challenging because of the multitude of cardiac and pulmonary diseases that cosegregate with advanced liver disease. PoPH is unique in that its natural history is not wholly dependent on portal hypertension. Despite a dearth of randomized, prospective data, an ever-expanding clinical experience shows that patients with PoPH benefit from therapy with PAH-specific medications. Because of high perioperative mortality, transplantation should be avoided in those patients who have severe PoPH that is refractory to medical therapy. This article reviews the pathophysiology and pathogenesis of PoPH and discusses approaches to diagnosis and management.
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