MII detects more reflux events than pH monitoring alone. The proportion of nonacid reflux to acid reflux events in infants is more similar to adults than previously reported. Combined pH-MII esophageal monitoring identifies more reflux events and improves clinical correlation with symptoms.

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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...