PoPH | HPS | |
Portal hypertension | Always present | Always present |
Chronic liver disease | Sometimes present | Sometimes present |
PVR | High | Low |
Diagnostic characteristics | Demonstration of PAH: mPAP >20 mmHg; PVR >3 WU; PAWP <15 mmHg | Demonstration of intrapulmonary vascular dilatation and increased A–a gradient (≥15 or 20 mmHg) |
Key investigations | Right heart catheterisation | Arterial blood gas Contrast enhanced echocardiography and/or lung perfusion scanning |
Grading of severity | mPAP: Mild: 25–34 mmHg Moderate: 35–44 mmHg Severe: ≥45 mmHg | Partial pressure of oxygen in arterial blood: Mild: ≥80 mmHg Moderate: ≥60–80 mmHg Severe: ≥50–60 mmHg Very severe: <50 mmHg |
Treatment | Supportive measures# PAH specific therapy Liver transplantation¶ | Supportive measures# Embolisation/coiling of large IPVDs/AVMs Liver transplantation |
AVM: arteriovenous malformation; A–a: Alveolar–arterial. #: supportive measures including supplemental oxygen therapy, diuretics, nutrition and exercise; ¶: while PoPH may improve post liver transplant, severe PoPH is a contraindication to liver transplantation.