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Successful living donor liver transplantation after stent implantation in a patient with Alagille syndrome and severe bilateral pulmonary artery stenosis

Published online by Cambridge University Press:  30 August 2018

Kentaro Akagi
Affiliation:
Department of Pediatrics, Graduate School of Medicine, Kyoto University Hospital, Kyoto, Japan
Toshikatsu Tanaka
Affiliation:
Department of Pediatric Cardiology, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Japan
Shiro Baba*
Affiliation:
Department of Pediatrics, Graduate School of Medicine, Kyoto University Hospital, Kyoto, Japan
*
Author for correspondence: S. Baba, MD, PhD, Department of Pediatrics, Graduate School of Medicine, Kyoto University Hospital, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto 606-8507, Japan. Tel: +81 75 751 3295 (3291); Fax: +81 75 752 2361; E-mail: [email protected]

Abstract

Severe pulmonary hypertension is a contraindication for liver transplantation owing to high mortality. However, decision-making regarding the treatment approach for patients with bilateral peripheral pulmonary artery stenosis, typically complicated by elevated main pulmonary artery and right ventricle pressures, can be challenging. Here, we report successful living donor liver transplantation after bilateral pulmonary artery stent implantation in a patient with Alagille syndrome, severe bilateral peripheral pulmonary artery stenosis, and extremely high main pulmonary artery and right ventricle pressures.

Type
Brief Report
Copyright
© Cambridge University Press 2018 

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References

1. Surani, SR, Mendez, Y, Anjum, H, Varon, J. Pulmonary complication of hepatic diseases. World J Gastroenterol 2016; 22: 60086015.Google Scholar
2. Krowka, MJ, Plevak, DJ, Findlay, DJ, et al. Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation. Liver Transpl 2000; 6: 443450.Google Scholar
3. Krowka, MJ, Mandell, MS, Ramsay, MA, et al. Hepatopulmonary syndrome and portopulmonary hypertension: a report of multicenter liver transplant database. Liver Transpl 2004; 10: 174182.Google Scholar
4. Ogawa, E, Hori, T, Doi, H, Segawa, H, Uemoto, S. Living-donor liver transplantation for congenital biliary atresia with porto-pulmonary hypertension and moderate or severe pulmonary arterial hypertension: Koto University experiences. Clin Transpl 2014; 28: 10311040.Google Scholar
5. Monge, MC, Mainwaring, RD, Sheikh, AY, et al. Surgical reconstruction of peripheral pulmonary artery stenosis in Williams and Alagille syndrome. J Thorac Cardiovasc Surg 2013; 145: 476481.Google Scholar
6. Cunningham, JW, McElhinney, DB, Gauvreau, K, et al. Outcome after primary transcatheter therapy in infants and young children with severe bilateral pheripheral pulmonary artery stenosis. Circ Cardiovasc Interv 2013; 6: 460467.Google Scholar
7. Hallbergson, A, Lock, JE, Marshall, AC. Frequency and risk of in-stent stenosis following pulmonary artery stenting. Am J Cardiol 2014; 113: 541545.Google Scholar
8. Ozcay, F, Varan, B, Tokel, K, et al. Severe peripheral pulmonary artery stenosis is not a contraindication to liver transplantation in Alagille syndrome. Pediatr Transplant 2006; 10: 108111.Google Scholar
9. Ovaert, C, Germeau, C, Berrea, C, et al. Elevated right ventricular pressure are not a contraindication to liver transplantation in Alagille syndrome. Transplantation 2001; 72: 345347.Google Scholar
10. Tzakis, AG, Reyes, J, Tepetes, K, et al. Liver transplantation for Alagille’s syndrome. Arch Surg 1993; 128: 337339.Google Scholar