Distinction between hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN)
| HPS | PPHTN | |
| Symptomatology | Progressive dyspnoea | Progressive dyspnoea |
| Chest pain | ||
| Syncope | ||
| Clinical examination | Cyanosis | No cyanosis |
| Finger clubbing | RV heave | |
| Spider angiomas (?) | Pronounced P2 component | |
| ECG findings | None | RBBB |
| Rightward axis | ||
| RV hypertrophy | ||
| Arterial blood gas levels | Moderate-to-severe hypoxaemia | No/mild hypoxaemia |
| Chest radiography | Normal | Cardiomegaly |
| Hilar enlargement | ||
| CEE | Always positive; left atrial opacification for >3–6 cardiac cycles after right atrial opacification | Usually negative; however, positive for <3 cardiac cycles (if atrial septal defect or patent foramen ovale exists) |
| 99mTcMAA shunting index | ≥6% | <6% |
| Pulmonary haemodynamics | Normal/low PVR | Elevated PVR |
| Normal mPAOP | ||
| Pulmonary angiography | Normal/“spongy” appearance (type I) | Large main pulmonary arteries |
| Discrete arteriovenous communications (type II) | Distal arterial pruning | |
| OLT | Always indicated in severe stages | Only indicated in mild-to-moderate stages |
RV: right ventricle
ECG: electrocardiography
RBBB: right bundle-branch block
CEE: contrast-enhanced echocardiography
99mTcMAA: technetium-99m-labelled macroaggregated albumin
PVR: pulmonary vascular resistance
mPAOP: mean pulmonary artery occlusion pressure
OLT: orthotopic liver transplantation