Diagnostic evaluation of hepatopulmonary syndrome in Egyptian children with chronic liver disease.

Abstract

There are few data on prevalence of hepatopulmonary syndrome (HPS) in children with chronic liver disease (CLD). This prospective study evaluated the prevalence and diagnostic procedures of HPS in Egyptian children with CLD. One hundred twenty (120) children with CLD were subjected to room-air pulse oximetry in supine and upright position, contrast enhanced echocardiography (CEE) and technetium-99m-labeled macroaggregated albumin (99mTc-MAA) perfusion lung scan. Arterial blood gas (ABG) analysis in upright position was performed for all children with identified intrapulmonary vascular dilatation (IPVD). Clinical, laboratory, imaging and endoscopic data were recorded and analyzed. Hypoxemia was found in 14 cases (11.7%) of the total cohort all of them had IPVD, whereas 6 cases (5%) of the patients had IPVD without hypoxemia. Therefore, HPS and subclinical HPS were diagnosed in 11.7% and 5% of CLD patients, respectively. Only 10 HPS patients had a pathological arterial oxygen saturation (SaO2) in the supine position (< or = 97%) but all showed a pathological SaO2 decrease (> or = 4%) after changing from supine to upright position. 99mTc-MAA perfusion lung scan revealed IPVD in 16.7% whereas CEE detected IPVD in 10% only of enrolled patients. There were strong correlations between shunt index estimated by lung scintiscan and oxygenation parameters in HPS patients. The characteristics of HPS patients were similar to that of non-HPS patients except for clubbing, dyspnea, cyanosis, orthodoexia and bleeding varices that were more associated with HPS patients as well as well as the Child-Pugh grades, which tended toward higher scores in HPS patients.