Outcomes of 50 Cases of Pediatric Liver Transplantation for Intrapulmonary Shunt While Considering the Deteriorated Prognosis of Severe Intrapulmonary Shunt Patients

Background. Intrapulmonary shunt (IPS) is recognized in 10% of chronic liver disease patients. Liver transplantation (LT) is associated with a high risk of morbidity and mortality in patients with IPS. Patients and methods. Of 519 pediatric LT cases between November 2005 and October 2018, 50 patients with IPS were enrolled in this study. The patients were divided into 3 groups, according to the shunt ratio, calculated by scintigraphy: mild (15%-20%, n = 26), moderate (20%-40%, n = 19), and severe (> 40%, n = 5). We compared the patients' characteristics before LT and the outcomes of LT between these groups. Results. The major original disease resulting in LT in the mild and moderate groups was biliary atresia (73.1% and 52.6%, respectively), while that in the severe group was congenital portosystemic shunt (60%). The median ages at LT were 7.5, 6.1, and 8.3 years in the mild, moderate, and severe groups, respectively. All of the mild and moderate IPS patients lived; however, 3 patients with severe IPS (60.0%) died within 3 months. The shunt ratios of the mild and moderate IPS patients normalized within 2 years after LT, while the 2 surviving severe IPS patients showed a slight improvement. The autopsy findings of the lung in 1 deceased severe IPS patient showed medial hypertrophy and proliferation of intimal cells of the pulmonary arteries, suggesting a diagnosis of portopulmonary hypertension. Conclusions. LT can be safely performed for mild and moderate IPS patients; however, LT for severe IPS patients should be carefully indicated because concomitant portopulmonary hypertension may be masked by IPS.