Treatment for Hepatopulmonary Syndrome (HPS)

Definition and Diagnosis

Hepatopulmonary syndrome (HPS) is characterized by intrapulmonary vascular dilatations (IPVD) developing in the setting of portal systemic shunting, and is present in 4-29% of chronic liver disease patients of all ages 3
Diagnosis requires presence of hypoxia and evidence of IPVD through contrast-enhanced transthoracic echocardiography, technetium-labeled macro-aggregated albumin lung perfusion scan, or cardiac catheterization demonstrating severe shunting 3, 4

Liver transplantation is the only definitive treatment for HPS, resulting in complete reversal or significant improvement in >85% of patients, and is appropriate for HPS in both cirrhotic and non-cirrhotic liver disease 2, 3
The American Association for the Study of Liver Diseases recommends liver transplantation as the primary treatment for HPS, with five-year survival rates improved from 67% in pre-MELD era to 88% in MELD era for HPS patients undergoing transplantation 2
HPS is considered an indication for urgent transplantation due to its poor prognosis without intervention, with median survival without LT in adults with severe HPS (PaO2 <50 mmHg) being less than 12 months 3, 5

Long-term oxygen therapy is recommended for patients with severe hypoxemia to manage symptoms, particularly during periods of increased physical activity 2, 3

Multiple medical therapies, including beta-blockers, cyclooxygenase inhibitors, systemic glucocorticoids, cyclophosphamide, almitrine bismesylate, inhaled nitric oxide, and nitric oxide inhibitors, have been ineffective in treating HPS 2
The European Association for the Study of the Liver states that pentoxifylline has shown contradictory results with frequent GI side effects, and garlic administration has been associated with hepatotoxicity 2

Patients with severe HPS (PaO2 <60 mmHg) receive MELD exception points to prioritize them for transplantation, significantly improving post-transplant outcomes 2
Respiratory function may temporarily worsen in the first days after transplantation, but complete improvement and reversal of HPS may take months 1