Portopulmonary hypertension and hepatopulmonary syndrome

Table 1 Diagnostic criteria of portopulmonary hypertension

Criteria 1

Portal hypertension (15 mmHg, or portocaval gradient > 5 mmHg) and

Criteria 2

mPAP > 25 mmHg and mPAOP < 15 mmHg

mPAP - mPAOP (transpulmonary gradient) > 10 mmHg

PVR > 240 dyn.s.cm-5 = 3 UI WOOD

mPAP: Mean pulmonary artery pressure; mPAOP: Mean pulmonary artery occlusion pressure; PVR: Pulmonary vascular resistance.

Table 2 Hemodynamic patterns in cirrhotics with elevated mean pulmonary artery pressure

Hemodynamic pattern	mPAP	PVR	mPAOP	CO
Hyperdynamic state	↑	↓	→	↑↑
Left heart dysfunction	↑	↑	↑	↓
POPH	↑	↑	→	↓

Adapted from Machicao et al[15]. CO: Cardiac output; mPAOP: Mean pulmonary artery occlusion pressure; mPAP: Mean pulmonary artery pressure; POPH: Portopulmonary hypertension; PVR: Pulmonary vascular resistance.

Table 3 Severity score of pulmonary hypertension in portopulmonary hypertension patients based on liver transplantation mortality

Mild: 25 mmHg < mPAP < 35 mmHg

Moderate: 35 mmHg ≤ mPAP < 45 mmHg

Severe: mPAP ≥ 45 mmHg

mPAP: Mean pulmonary artery pressure.

Table 4 Portopulmonary hypertension treatments[69]

Molecule	Mechanism of action	Half-life	Administration	Adverse effects	Advantages	Drawbacks	Main results
Nitric Oxide	Selective dilation of the pulmonary vasculature	Seconds	Inhaled	Methemoglobinemia	Selective on pulmonary circulation	Endotracheal intubation	Improved hemodynamics
Epoprostenol	Vasodilation of all vascular beds/platelet inhibition	Minutes	Intravenous	Flushing, headache, nausea	Best studied drug	Long term central venous access/cost	Improved hemodynamics allowing LT[70,71]
Iloprost	Vasodilation of pulmonary vascular bed/platelet inhibition	Minutes	Inhaled	Flushing, headache, nausea	Selective on pulmonary circulation	Frequent administration/cost	Increased exercise tolerance and survival[26,27,30]
Bosentan	Endothelin receptor antagonist	5 h	Oral	Hepatotoxicity, peripheral edema	Oral administration, twice daily	Cost	Improved survival[29]
Sildenafil	Phosphodiesterase type 5 inhibitor	4 h	Oral	Flushing, headache, priapism	Oral administration, three times daily	Cost	Increased exercise tolerance[31]

LT: Liver transplantation.

Table 5 Diagnostic criteria of hepatopulmonary syndrome

Criteria 1: Chronic liver disease

Criteria 2: A-aDO2 ≥ 15 mmHg[7,72], or ≥ 20 mmHg[52], or ≥ to the age-adjusted value1[73]

Criteria 3: intrapulmonary vascular dilatation at CE-TTE[7] or 99mTcMAA[7]

¹Age-adjusted value: 0.26 (age) - 0.43. A-aDO₂: Alveolar-arterial oxygen pressure gradient; CE-TTE: Contrast-enhanced echocardiography;

^99mTcMAA: Technetium macroaggregated albumin lung perfusion scan.

Table 6 Severity score of hepatopulmonary syndrome[3]

Stages	PaO2, mmHg
Mild	≥ 80
Moderate	≥ 60 and < 80
Severe	≥ 50 and < 60
Very severe	< 50