title: Living-donor liver transplantation for moderate or severe porto-pulmonary hypertension accompanied by pulmonary arterial hypertension: a single-centre experience over 2 decades in Japan.
abstract: BACKGROUND
  Candidates for orthotopic liver transplantation (OLT) often have porto-pulmonary hypertension (PPHTN) with pulmonary arterial hypertension (PAH). Poor outcomes of PPHTN contraindicate OLT. There are no guidelines for living-donor liver transplantation (LDLT) in PPHTN patients.
  
  
  METHODS
  We present our experiences of LDLT in six patients with moderate or severe PPHTN, along with our institutional guidelines. Three had liver cirrhosis and three were non-cirrhotic. Catheterization studies were undertaken before, during and after LDLT, and the mean pulmonary arterial pressure (mPAP), cardiac output (CO), pulmonary vascular resistance and total peripheral resistance (TPR) were monitored.
  
  
  RESULTS
  The results showed significant differences in CO and TPR between cirrhotic and non-cirrhotic patients before, during and after LDLT. Cirrhotic patients showed systemic hyperdynamic state. Two cirrhotic patients showed poor responses to pre-transplant treatment, and continued to have increased PAH and poor clinical courses after LDLT. LDLT has an advantage of flexible timing of LT. Currently in our institution, PPHTN patients with mPAP <40 mmHg are registered for LDLT after treatment and catheterization. However, LDLT is performed when mPAP is ≤35 mmHg, leading to improved outcomes.
  
  
  CONCLUSIONS
  PPHTN patients with well-controlled PAH, or secondary PAH resulting from porto-systemic shunts, may be appropriate candidates for LDLT after careful considerations.
authors.affiliation: Divisions of Hepato-Pancreato-Biliary and Transplant Surgery, Department of Surgery, Kyoto University Hospital, 54 Shogoinkawara-cho Sakyo-ku, Kyoto, 606-8507, Japan.
authors.forename: Eri
authors.initials: E
authors.lastname: Ogawa
authors.forename: Tomohide
authors.initials: T
authors.lastname: Hori
authors.forename: Hiraku
authors.initials: H
authors.lastname: Doi
authors.forename: Hajime
authors.initials: H
authors.lastname: Segawa
authors.forename: Shinji
authors.initials: S
authors.lastname: Uemoto
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D006976:Hypertension, Pulmonary / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D007564:Japan; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D017082:Portal Pressure; D011669:Pulmonary Wedge Pressure; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult