Combination therapy for severe portopulmonary hypertension in a child allows for liver transplantation

Ryan M. Serrano, Girish Subbarao, Richard Mangus, Greg Montgomery, Michael Johansen

Research output: Contribution to journalArticle

Abstract

Severe PPHTN is a contraindication to liver transplantation and predicts an abysmal 5-year outcome. It is defined as a resting mPAP >45 mm Hg with a mean pulmonary artery wedge pressure of <15 mm Hg and pulmonary vascular resistance of >3 wood units in the setting of portal hypertension. There have been limited reports of successful treatment of PPHTN leading to successful liver transplantation in adults, and one reported use of monotherapy as a bridge to successful liver transplant in pediatrics. To our knowledge, we describe the first use of combination therapy as a successful bridge to liver transplantation in a pediatric patient with severe PPHTN. This report adds to the paucity of data in pediatrics on the use of pulmonary vasodilator therapy in patients with severe PPHTN as a bridge to successful liver transplantation. Early diagnosis in order to mitigate or avoid the development of irreversible pulmonary vasculopathy that would preclude candidacy for liver transplantation is crucial, but our report demonstrates that combination therapy can be administered safely, quickly, and may allow for successful liver transplantation in patients with severe PPHTN.

Original languageEnglish (US)
Article numbere13461
JournalPediatric Transplantation
DOIs
StatePublished - Jan 1 2019

Fingerprint

Liver Transplantation
Hypertension
Pediatrics
Therapeutics
Lung
Pulmonary Wedge Pressure
Portal Hypertension
Vasodilator Agents
Early Diagnosis
Transplants
Liver

Keywords

  • congenital hepatic fibrosis
  • pediatric liver transplantation
  • portopulmonary hypertension

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

Cite this

Combination therapy for severe portopulmonary hypertension in a child allows for liver transplantation. / Serrano, Ryan M.; Subbarao, Girish; Mangus, Richard; Montgomery, Greg; Johansen, Michael.

In: Pediatric Transplantation, 01.01.2019.

Research output: Contribution to journalArticle

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abstract = "Severe PPHTN is a contraindication to liver transplantation and predicts an abysmal 5-year outcome. It is defined as a resting mPAP >45 mm Hg with a mean pulmonary artery wedge pressure of <15 mm Hg and pulmonary vascular resistance of >3 wood units in the setting of portal hypertension. There have been limited reports of successful treatment of PPHTN leading to successful liver transplantation in adults, and one reported use of monotherapy as a bridge to successful liver transplant in pediatrics. To our knowledge, we describe the first use of combination therapy as a successful bridge to liver transplantation in a pediatric patient with severe PPHTN. This report adds to the paucity of data in pediatrics on the use of pulmonary vasodilator therapy in patients with severe PPHTN as a bridge to successful liver transplantation. Early diagnosis in order to mitigate or avoid the development of irreversible pulmonary vasculopathy that would preclude candidacy for liver transplantation is crucial, but our report demonstrates that combination therapy can be administered safely, quickly, and may allow for successful liver transplantation in patients with severe PPHTN.",
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