title: Intrapulmonary vascular dilation in children with chronic liver diseases: pre- and post-liver transplantation.
abstract: OBJECTIVE
  Chronic liver disease (CLD) can cause hepatopulmonary syndrome (HPS), defined as triad of liver disease, hypoxemia, and intrapulmonary vascular dilation (IPVD). The aim of this study was to determine the evidence of IPVD in a cohort of pediatric patients with CLD pre- and post-liver transplantation (LT).
  
  
  METHODS
  All pediatric patients with CLD listed for LT were studied. Pulse oxygen saturation (SpO(2)), technetium-99m-labeled macroaggregated albumin ((99m)Tc- MAA) perfusión scan (positive test: uptake of the isotope ≥ 6% in the brain), and echocardiography with saline bubble test (SBT) were performed. SBT was re-evaluated at 3-6 months after LT. Grading of SBT included grade 0 (no bubble), I (1-9 bubbles), grade II (10-20 bubbles), and grade III (> 20 bubbles).
  
  
  RESULTS
  Eighteen patients, median age 22.5 months (8-108), were enrolled. Most had biliary atresia (77.8%). Pre-LT, all patients had SpO(2) of 100% and none had positive (99)mTc- MAA perfusion scan. Two patients (11%) had negative SBT (grade 0), 1 (5.5%) had grade I, 3 (16.5%) had grade II, and 12 (67%) had grade III, respectively. Post-LT SBT became negative in all survivors (n = 16), (p = 0.0001).
  
  
  CONCLUSIONS
  Most cirrhotic children in this cohort study had evidence of IPVD by positive SBT. However, none of these met the criteria for diagnosis of HPS. This evidence of IPVD subsided after LT.
authors.affiliation: Department of Surgery. Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Anant
authors.initials: A
authors.lastname: Khositseth
authors.affiliation: Department of Pediatrics. Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Suporn
authors.initials: S
authors.lastname: Treepongkaruna
authors.affiliation: Department of Surgery. Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Khemika
authors.initials: K
authors.lastname: Khemakanok
authors.affiliation: Department of Surgery. Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Sumate
authors.initials: S
authors.lastname: Teeraratkul
authors.affiliation: Department of Surgery. Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Vichai
authors.initials: V
authors.lastname: Pansrimangkorn
authors.affiliation: Department of Surgery. Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Chollasak
authors.initials: C
authors.lastname: Thirapattaraphan
authors.affiliation: Department of Pediatrics. Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Surasak
authors.initials: S
authors.lastname: Leelaudomlipi
mesh_terms: D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D004452:Echocardiography; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008103:Liver Cirrhosis / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D010092:Oximetry; D055420:Perfusion Imaging / Q000379:methods; D011237:Predictive Value of Tests; D011651:Pulmonary Artery / Q000503:physiopathology*; D011652:Pulmonary Circulation; D019275:Radiopharmaceuticals / Q000008:administration & dosage; D012307:Risk Factors; D012965:Sodium Chloride / Q000008:administration & dosage; D013668:Technetium Tc 99m Aggregated Albumin / Q000008:administration & dosage; D016896:Treatment Outcome; D014664:Vasodilation
chemical_list: D019275:Radiopharmaceuticals; D013668:Technetium Tc 99m Aggregated Albumin; D012965:Sodium Chloride