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
Critical Care
Compendium
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