Development of pulmonary hypertension in 5 patients after pediatric living-donor liver transplantation: de novo or secondary?

Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
Yasumasa ShirouzuKoichi Tanaka

Abstract

The development of portopulmonary hypertension (PH) in a patient with end-stage liver disease is related to high cardiac output and hyperdynamic circulation. However, PH following liver transplantation is not fully understood. Of 617 pediatric patients receiving transplants between June 1990 and March 2004, 5 (median age 12 yr, median weight 24.5 kg) were revealed to have portopulmonary hypertension (PH) after living-donor liver transplantation (LDLT), as confirmed by echocardiography and/or right heart catheterization. All children underwent LDLT for post-Kasai biliary atresia. In 2 patients with refractory biliary complications, PH developed following portal thrombosis; 2 with stable graft function, who had had intrapulmonary shunting (IPS) before LDLT, were found to have PH in spite of overcoming liver dysfunction due to hepatitis. PH developed shortly after distal splenorenal shunting in 1 patient, who suffered liver cirrhosis due to an intractable outflow blockage. The onset of PH ranged from 2.8 to 11 yr after LDLT, and mean pulmonary artery pressure (mPAP) estimated by echocardiography at the time of presentation ranged from 43 to 120 mmHg. Three of the 5 patients are alive under prostaglandin I2 (PGI2) treatment. Of the...Continue Reading

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Citations

Nov 26, 2013·Clinics in Chest Medicine·Nadine Al-Naamani, Kari E Roberts
May 22, 2007·American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons·R A FisherUNKNOWN A2ALL Study Group
Mar 28, 2009·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·David G KochAdrian Reuben
Jul 10, 2013·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Deirdre A KellyUNKNOWN American Society of Transplantation
Mar 1, 2017·Journal of Korean Medical Science·Kyung Jae LeeJae Sung Ko
Oct 31, 2007·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Benjamin L Shneider, George V Mazariegos
Apr 18, 2015·Journal of Pediatric Gastroenterology and Nutrition·Emmanuelle Ecochard-DugelayOlivier Bernard
Oct 15, 2008·Arquivos De Gastroenterologia·Sílvia BarcelosMariane Borba Monteiro

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