Portopulmonary hypertension is defined by the coexistence of portal and pulmonary hypertension.
- Pulmonary artery pressure: >25 mmHg
- Portal pressure: > 12 mmHg
- Portal hypertension and/or liver disease
- Clinical diagnosis: Ascites/varices/splenomegaly
- Mean pulmonary artery pressure (MPAP) > 25 mmHg at rest
- Pulmonary vascular resistance (PVR) > 240 dynes s cm−5
- Pulmonary artery occlusion pressure (PAOP) < 15mmHg or transpulmonary gradient (TPG) > 12 mmHg where TPG = MPAP − PAOP
Combination of medical therapy and liver transplantation.