Living-donor liver transplantation for congenital biliary atresia with porto-pulmonary hypertension and moderate or severe pulmonary arterial hypertension: Kyoto University experience

Clin Transplant. 2014 Sep;28(9):1031-40. doi: 10.1111/ctr.12415. Epub 2014 Jul 28.


Porto-pulmonary hypertension with moderate or severe pulmonary arterial hypertension (PAH) is viewed as a contraindication to liver transplantation (LT) because of associated poor outcomes; however, patients with biliary atresia (BA) are generally good candidates for LT. Ten patients with moderate/severe PAH underwent living-donor liver transplantation (LDLT) at our institution; eight of these patients had BA and were the focus of this study. Preoperative therapies, including prostaglandin (PG)I2 , were introduced. When mean pulmonary arterial pressure (mPAP) after treatment was <40 mmHg or initial mPAP without therapy was <35 mmHg, we performed an acute volume challenge test to evaluate right ventricular function. LDLT was performed when mPAP after anesthetic induction was confirmed at ≤35 mmHg. 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 mPAP after LDLT. The other six patients had successful courses with well-controlled mPAP, and PGI2 was withdrawn or weaned following LDLT. Survival did not significantly differ between the eight BA recipients with moderate/severe PAH and 77 age-matched BA recipients without PAH from the same time period. LDLT has major benefits for BA patients with well-controlled PAH.

Keywords: congenital biliary atresia; living-donor liver transplantation; porto-pulmonary hypertension; prostaglandin; pulmonary arterial hypertension.

MeSH terms

  • Adolescent
  • Adult
  • Biliary Atresia / surgery*
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Graft Rejection
  • Graft Survival
  • Humans
  • Hypertension, Portal / surgery*
  • Hypertension, Pulmonary / surgery*
  • Liver Transplantation*
  • Living Donors*
  • Male
  • Postoperative Complications
  • Prognosis
  • Retrospective Studies
  • Risk Factors
  • Young Adult