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Home | CCC | Portopulmonary syndrome

Portopulmonary syndrome

by Dr Chris Nickson, last update April 25, 2019

OVERVIEW

  • Portopulmonary Syndrome is NOT considered contraindication to liver transplantation
  • affects ~20% of pre-transplant patients
  • usually due to increased blood flow through the lungs without increased resistance
  • ~5% have pulmonary HTN with high PVR, which is worse

DIAGNOSTIC CRITERIA

  • Portal hypertension
  • MPAP > 25 mmHg
  • PAOP < 15 mmHg
  • PVR > 120 dynes/s per cm-5

MANAGEMENT

  • liver transplantation if appropriate
  • epoprostenol post-op (PGI2 analogue) for 3 months -> remodelling
  • progressive thrombocytopenia and splenomegaly may occur

PROGNOSIS

  • can progress after transplantation
  • higher the MPAP and PVR (and TPG = transpulmonary gradient) -> higher the mortality
  • Some consider f MPAP >50 mmHg some advise cancellation of liver transplantation as close to 100% mortality

CCC 700 6

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About Dr Chris Nickson

An oslerphile emergency physician and intensivist suffering from a bad case of knowledge dipsosis. Key areas of interest include: the ED-ICU interface, toxicology, simulation and the free open-access meducation (FOAM) revolution. @Twitter | INTENSIVE| SMACC

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