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Ten patients with moderate\/severe PAH underwent living\u2010donor liver transplantation (LDLT) at our institution; eight of these patients had BA and were the focus of this study. Preoperative therapies, including prostaglandin (PG)I<jats:sub>2<\/jats:sub>, were introduced. When mean pulmonary arterial pressure (<jats:styled-content style=\"fixed-case\">mPAP<\/jats:styled-content>) after treatment was &lt;40\u00a0mmHg or initial <jats:styled-content style=\"fixed-case\">mPAP<\/jats:styled-content> without therapy was &lt;35\u00a0mmHg, we performed an acute volume challenge test to evaluate right ventricular function. LDLT was performed when <jats:styled-content style=\"fixed-case\">mPAP<\/jats:styled-content> after anesthetic induction was confirmed at \u226435\u00a0mmHg. Six patients had favorable responses to preoperative treatment and catheter testing, but two patients showed poor responses. The two patients with poor responses had poor clinical courses with unstable <jats:styled-content style=\"fixed-case\">mPAP<\/jats:styled-content> after LDLT. The other six patients had successful courses with well\u2010controlled <jats:styled-content style=\"fixed-case\">mPAP<\/jats:styled-content>, and PGI<jats:sub>2<\/jats:sub> was withdrawn or weaned following LDLT. Survival did not significantly differ between the eight BA recipients with moderate\/severe PAH and 77 age\u2010matched BA recipients without PAH from the same time period. LDLT has major benefits for BA patients with well\u2010controlled PAH.<\/jats:p>","DOI":"10.1111\/ctr.12415","type":"journal-article","created":{"date-parts":[[2014,7,2]],"date-time":"2014-07-02T07:05:28Z","timestamp":1404284728000},"page":"1031-1040","source":"Crossref","is-referenced-by-count":9,"title":["Living\u2010donor liver transplantation for congenital biliary atresia with porto\u2010pulmonary hypertension and moderate or severe pulmonary arterial hypertension: Kyoto University experience"],"prefix":"10.1111","volume":"28","author":[{"given":"Eri","family":"Ogawa","sequence":"first","affiliation":[{"name":"Department of Pediatric Surgery Kyoto University Hospital Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomohide","family":"Hori","sequence":"additional","affiliation":[{"name":"Department of Hepato\u2010Pancreato\u2010Biliary and Transplant Surgery Kyoto University Hospital Kyoto 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