Sirolimus is safe and efficient for the treatment of BRBNS. Further prospective studies are needed to evaluate the long-term effectiveness of this drug. This is the first report that identifies a lymphatic component as part of BRBNS.

Objectives-To investigate the clinical presentation, manifestations, and response to therapy of portopulmonary hypertension (PPHTN) in pediatric patients.Study design-This study was a retrospective chart review describing the evaluation and course of 7 patients with PPHTN.Results-Causes of portal hypertension (HTN) included biliary atresia (3 cases), cavernous transformation of the portal vein (2 cases), and primary sclerosing cholangitis and cryptogenic cirrhosis (1 case each). The median interval from the diagnosis of portal HTN to PPHTN was 12.1 years. Four patients presented with a new heart murmur, 4 presented with syncope, and 3 presented with dyspnea. Although electrocardiograms (ECGs) and chest x-rays were normal in 3 and 2 patients, respectively, echocardiograms diagnosed pulmonary HTN in all 7 patients. Five patients had cardiac catheterizations; the average mean pulmonary artery pressure was 65 ± 20 mm Hg. Response to therapy was variable, and 4 patients died. Postmortem lung tissue examination revealed plexiform lesions and pulmonary arteriopathy.Conclusions-Because symptoms are subtle and may be overlooked, pediatric patients with portal HTN who develop a new heart murmur, dyspnea, syncope, or who are being evaluated for liver transplantation require evaluation for PPHTN. ECG and chest x-ray are insensitive screens for PPHTN. An echocardiogram and cardiology evaluation is essential for the diagnosis.Portopulmonary hypertension (PPHTN) is one of the pulmonary vascular disorders complicating chronic liver disease. 1 In 1998 the World Health Organization P(WHO) classified PPHTN as pulmonary arterial hypertension (HTN) associated with liver disease or portal HTN. 2 PPHTN is defined by elevated mean pulmonary arterial pressure (PAP) (> 25 mm Hg at rest), increased pulmonary vascular resistance (PVR) (>3 Wood units · 3 m 2 ), and normal pulmonary capillary wedge pressure (<15 mm Hg) in the presence of portal HTN. 3 Both hepatic and extrahepatic causes of portal HTN may lead to PPHTN. The prevalence of PPHTN in adult patients with cirrhosis is 0.25% to 0.73% based on autopsy series 1,4 and 3.5% to 8.5% in liver transplant candidates. 1,5 The diagnosis of PPHTN is usually made 4 to 7 years after the diagnosis of portal HTN in adults. 1,6 Copyright © 2005 Elsevier Inc. All rights reserved. METHODSA retrospective review of patient records in the Pediatric Liver Center, Children's Hospital identified 7 pediatric patients with PPHTN diagnosed between 1995 and 2004 ( Table I). All of these children had been previously enrolled in an institutional review board-approved protocol for prospective longitudinal evaluation of childhood pulmonary HTN, for which written informed consent was obtained from the parents or guardians. Data obtained from the medical record included age at diagnosis of portal HTN and PPHTN, cause of portal HTN, symptoms and/or signs precipitating the evaluation for pulmonary HTN, and additional review of chest x-ray, electrocardiogram (ECG), Doppler echocardiogram, right heart catheter...

Objectives To determine the prevalence of Portopulmonary Hypertension (PPHTN), 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. 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, x-rays, 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 one of whom had HPS. Compared to subjects with normal cECHO, those with IPVS had biochemical evidence of more advanced liver disease and higher b-type natriuretic peptide (BNP) levels. Conclusions PPHTN and HPS appear to be rare in clinically stable children with portal hypertension. IPVS was present in 19% of these patients. A novel finding of this study is the elevation of BNP in children with IPVS.

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