Pulmonary Vascular Complications in Asymptomatic Children With Portal Hypertension

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OBJECTIVES: : To determine the prevalence of portopulmonary hypertension, hepatopulmonary syndrome (HPS), and intrapulmonary vascular shunting (IPVS) in children with clinically stable portal hypertension and to assess the value of vasoactive peptide levels, biochemical tests and clinical signs or symptoms to predict these conditions. PATIENTS AND METHODS: : A prospective, cross-sectional analysis was conducted on 33 children, ages 4 to 17 years, with stable cirrhosis (n = 28) or extrahepatic portal hypertension (n = 5). The children were screened for IPVS and hypoxia with contrast-enhanced echocardiography (cECHO) and pulse oximetry, and screened for pulmonary hypertension with Doppler echocardiography. Chemistries, radiographs, physical examinations, and levels of vasoactive peptides were compared between subjects with IPVS and those with normal cECHO. RESULTS: : No subject had pulmonary hypertension. Six (19%) had IPVS, all of which had intrahepatic causes of portal hypertension, and 1 of whom had HPS. Compared with subjects with normal cECHO, those with IPVS had biochemical evidence of more advanced liver disease and higher b-type natriuretic peptide levels. CONCLUSIONS: : Prevalence of portopulmonary hypertension and HPS appear to be rare in clinically stable children with portal hypertension. Intrapulmonary vascular shunting was present in 19% of these patients. A novel finding of this study is the elevation of b-type natriuretic peptide in children with IPVS.

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