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016 THE PREVALENCE OF HEPATOPULMONARY SYNDROME IN CHILDREN K Noli, M Solomon1, M Charron2, F Golding3, SC Ling BACKGROUND: Hepatopulmonary syndrome (HPS) is defined as a triad of liver disease, hypoxemia and intrapulmonary vascular dilatation (IPVD). The reported prevalence of HPS in cirrhotic adults ranges from 4% to 29%. However, the prevalence of HPS and its outcome in children is unknown.
Divisions of Gastroenterology, Hepatology and Nutrition, 1Respiratory Medicine, 2Nuclear Medicine, and 3Cardiology, Hospital for Sick Children, Toronto, Ontario
AIM: To measure the prevalence of IPVD and HPS in children with liver disease.
METHODS: We recruited consecutive children <=18y with chronic liver diseases who attended the liver clinics, liver transplant clinics, or who were admitted as in-patients between January and June 2006. Children with congenital heart disease with intra-cardiac right to left shunt were excluded. All children underwent pulse oximetry. Children with cirrhosis or with oxygen saturation <=97% underwent contrast enhanced echocardiography (CEE) for detection of IPVD. If CEE was positive, 99mTc-labeled macroaggregated albumin (99mTc MAA) perfusion scan and arterial blood gas analysis were performed. HPS was considered present in a child with evidence of IPVD and a PaO2 less than 70 mmHg or an alveolar-arterial oxygen gradient (A-a) of greater than 20 mmHg.
RESULTS: Of 356 patients who met the inclusion criteria, 305 agreed to participate and 4 subsequently withdrew. Data on 301 patients were therefore analyzed. 8 children had SaO2 <=97%. Of 28 (9%) children who underwent CEE, 24 (86%) were cirrhotic, and 2 had nodular regenerative hyperplasia with portal hypertension. A positive CEE suggestive of IPVD was present in 7 (29%) children. Two of these patients had abnormal ABG. Thus, 8% of our cirrhotic cohort had HPS.
CONCLUSION: IPVD without hypoxia was common in this cohort of children with cirrhosis, whilst the minimal prevalence of HPS was 8%. Further follow-up in a larger cohort is required to determine the natural history of IPVD without hypoxia and the impact of HPS on clinical outcomes in children.