Hepatopulmonary Syndrome in Children with Cirrhotic and Non-Cirrhotic Portal Hypertension: A Single-Center Experience

Background Hepatopulmonary syndrome (HPS) is defined as an arterial oxygenation defect induced by intrapulmonary vascular dilatation (IPVD) associated with hepatic disease. The prevalence and clinical characteristics of HPS in portal hypertensive children is not well characterized. Aims The aim of this study was to investigate the prevalence and clinical characteristics of HPS in 40 portal hypertensive children. Methods We studied 40 children (11 girls and 29 boys; mean age, 111 months ± 52 months; range, 24–216 months) with portal hypertension (24 cirrhotic, 16 non-cirrhotic) for the presence of HPS using blood gas analysis, contrastenhanced echocardiography (CEE), and Tc99m-macroaggregated albumin scintigraphy. Clinical and laboratory characteristics of patientswere recorded.HPSwas considered to be present in a patient with hypoxemia and/or an elevated alveolar–arterial oxygen gradient (PAaO2) C15 mmHg) and positive CEE and/or scintigraphy. Results Elevated PAaO2 was detected in 7 of 24 patients with cirrhosis. Four of them also had IPVD with CEE. An intrapulmonary shunt in Tc99m-MAA with CEE was shown in one patient. A diagnosis of HPS was made in 16.7% of the patients with cirrhosis. Cirrhotic patients without IPVD had significantly better hepatic function and lower pediatric end-stage liver disease scores. Although, 2 of the 16 patients with non-cirrhotic portal hypertension had elevated PAaO2, none of them showed IPVD. One normoxemic patient in the non-cirrhotic portal hypertension group showed IPVD with CEE. None of the noncirrhotic patients fulfilled the diagnostic criteria of HPS. Conclusion Hepatopulmonary syndrome particularly occurs in cirrhotic portal hypertensive patients with severe hepatic dysfunction.