Portopulmonary syndrome

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