The Canadian Hepatopulmonary Syndrome Program: A Longitudinal Liver Transplant Experience

Hepatopulmonary syndrome (HPS) is a pulmonary complication of liver disease comprised of liver dysfunction, intrapulmonary vascular dilatations, and abnormal oxygenation. Liver transplantation (LT) is the only curative treatment for HPS, but outcome reports in objectively diagnosed cohorts are mostly from single centers with mild/moderate disease populations and short-term follow-up.

This was a retrospective cohort study in which we sought to describe short- and long-term LT outcomes and their predictors in the Canadian HPS Program.

Among 53 patients with HPS receiving LT (partial pressure of arterial oxygen [PaO₂]: 50.6 ± 9.5 mmHg), mortality at 1, 5, and 10 years post LT was 4/53 (7.5%), 7/41 (17%), and 13/27 (48%), respectively. In a multivariable model, hazard of post-LT death was lower in patients with a higher baseline PaO₂ (HR: 0.93 [0.87-0.99]) and higher in those with obstructive lung disease (HR: 6.96 [1.29-37.43]). Oxygenation normalized in 46 of 47 (97.9%) patients 6 months after LT. Infectious (25/53; 47.2%) and biliary (26/53; 49.1%) complications were common. Malignancy occurred in 13 of 49 (26.5%) patients over a median follow-up 5.8 years.

We present LT data in the most severe and among the largest and longest-followed HPS cohorts reported to date. Our favorable outcomes compared to smaller centers and large databases suggest that high-volume centers with HPS expertise are preferable for LT in severe/very severe HPS. Heightened vigilance for specific short-term complications along with close monitoring of patients with comorbid obstructive lung disease is indicated. Our detailed analysis of post-transplant events and time course of disease resolution/oxygen cessation can inform patient/family counseling and provider expectations.