It is well known that hepatopulmonary syndrome (HPS) manifested with hypo-oxygenemia secondary to intra-pulmonary shunt has negative impacts on outcomes of liver transplantation in deceased liver transplantation (LT) but not in living donor LT (LDLT). We, Japanese multicenter study group, retrospectively reviewed clinical records of HPS patients undergoing LT and studied impacts of risk factors related to HPS on clinical outcomes in order to find strategies to overcome complications.

Method: When a patient had symptoms of hypo-oxygenemia and 99mTc-MAA lung perfusion scintigraphy revealed a shunt ratio greater than 15%, he or she was defined as HPS. Patients with anatomical shunts were excluded.

Results: Forty-eight patients (21 males and 27 females, age; 1 -63 with the median of 12 years) in 10 centers were enrolled among 73 patients with hypo-oxygenemia in 12 centers who were listed in a preliminary survey. Diseases were biliary atresia in 23, HCV cirrhosis in 6, NASH in 4, congenital hepatic fibrosis in 4, and others in 12. Grafts were right lobe in 12, left lobe in 35, and a whole liver in 1. Donors were parents in 34, sons or daughters in 6, spouses in 4 other relatives in 3, and a deceased donor in 1 and age ranged from 2 to 63 years. Blood loss ranged from 15 to 24169 grams. A manner of biliary reconstruction was duct-to-duct in 16 and hepatico-jejunostomy in 32. The length of ICU stay ranged from 2 to 170 days (median: 7). Respirator was continued in 20 patients on POD 3, 17 on POD 7, and 10 on POD14. Patient survival was 87% at 1 year and 82% at 5 year and 10 year. Causes of hospital mortality were sepsis in 2, TMA, intracranial bleeding, pulmonary fibrosis, rejection each in 1. Severe shunt and hypo-oxgenemia before operation and on POD 1 were significant risks for hospital mortality (n=6). The length of HPS suffering period was a significant risk for intraabdominal bleeding (n=6), biliary leakage (n=3), and fungal infection (n=1). Hypo-oxgenemia on POD 3 was a significant risk for biliary stenosis and that POD 14 was a significant risk for biliary leakage. Shunt ratio of all surviving patients was significantly improved.

Conclusion: Although LDLT is feasible for patients with HPS, early transplant and avoiding hypo-oxygenemia immediately after transplantation are important.

CITATION INFORMATION: Egawa H, Ogata S. Japanese Multicenter Study of Hepatopulmonary Syndrome in Living Donor Liver Transplantation. Am J Transplant. 2016;16 (suppl 3).

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