start-ver=1.4 cd-journal=joma no-vol=52 cd-vols= no-issue=2 article-no= start-page=630 end-page=633 dt-received= dt-revised= dt-accepted= dt-pub-year=2020 dt-pub=20200331 dt-online= en-article= kn-article= en-subject= kn-subject= en-title= kn-title=Pediatric Living Donor Liver Transplantation for Congenital Absence of the Portal Vein With Pulmonary Hypertension: A Case Report en-subtitle= kn-subtitle= en-abstract= kn-abstract=Few reports of liver transplantation exist in patients with congenital absence of the portal vein and pulmonary hypertension. Living donor liver transplantation is usually performed before exacerbation of pulmonary hypertension. A 7-year-old girl (height: 131.5 cm; weight: 27.4 kg) with congenital absence of the portal vein was diagnosed with pulmonary hypertension (mean pulmonary artery pressure 35 mm Hg), and liver transplantation was planned before exacerbation of pulmonary hypertension. We successfully managed her hemodynamic parameters using low-dose dopamine and noradrenaline under monitoring of arterial blood pressure, central venous pressure, cardiac output, and stroke volume variation. Anesthesia was maintained using air-oxygen-sevoflurane and remifentanil 0.1 to 0.6 ƒÊg?kg-1?min-1. It is necessary to understand the potential perioperative complications in such cases and to adopt a multidisciplinary team approach in terms of the timing of transplantation and readiness to deal with exacerbation of pulmonary hypertension. en-copyright= kn-copyright= en-aut-name=MatsumotoNaohisa en-aut-sei=Matsumoto en-aut-mei=Naohisa kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=1 ORCID= en-aut-name=MatsusakiTakashi en-aut-sei=Matsusaki en-aut-mei=Takashi kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=2 ORCID= en-aut-name=HiroiKazumasa en-aut-sei=Hiroi en-aut-mei=Kazumasa kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=3 ORCID= en-aut-name=KakuRyuji en-aut-sei=Kaku en-aut-mei=Ryuji kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=4 ORCID= en-aut-name=YoshidaRyuichi en-aut-sei=Yoshida en-aut-mei=Ryuichi kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=5 ORCID= en-aut-name=UmedaYuzo en-aut-sei=Umeda en-aut-mei=Yuzo kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=6 ORCID= en-aut-name=YagiTakahito en-aut-sei=Yagi en-aut-mei=Takahito kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=7 ORCID= en-aut-name=MorimatsuHiroshi en-aut-sei=Morimatsu en-aut-mei=Hiroshi kn-aut-name= kn-aut-sei= kn-aut-mei= aut-affil-num=8 ORCID= affil-num=1 en-affil= Department of Anesthesiology and Resuscitology, Okayama University Hospital kn-affil= affil-num=2 en-affil= Department of Anesthesiology and Resuscitology, Okayama University Hospital kn-affil= affil-num=3 en-affil= Department of Anesthesiology and Resuscitology, Okayama University Hospital kn-affil= affil-num=4 en-affil= Department of Anesthesiology and Resuscitology, Okayama University Hospital kn-affil= affil-num=5 en-affil=Department of Gastroenterological Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences kn-affil= affil-num=6 en-affil=Department of Gastroenterological Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences kn-affil= affil-num=7 en-affil= kn-affil= affil-num=8 en-affil= Department of Anesthesiology and Resuscitology, Okayama University Hospital kn-affil= END