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Copyright ©The Author(s) 2016.
World J Gastroenterol. Jul 14, 2016; 22(26): 6008-6015
Published online Jul 14, 2016. doi: 10.3748/wjg.v22.i26.6008
Table 2 Differences between the hepatopulmonary syndrome and portopulmonary hypertension
Hepatopulmonary syndromePortopulmonary hypertension
PathophysiologySevere vasodilatationSevere vasoconstriction
Production of endothelin-1 and tumor necrosis alpha and eNOS and iNOSConcentric intimal fibrosis, and smooth muscle hyperplasia and hypertrophy
Increase of COEndothelin-1, prostacyclin and thromboxane
Vasculoendothelial growth factor-A
Angiogenesis
Clinical featuresMost patients are asymptomaticDyspnea
DyspneaOrthopnea
PlatypneaFatigue
OrthodeoxiaSyncope
Significant sleep-time oxygen desaturationChest pain
Lightheadedness
Tricuspid regurgitation murmur, with a pronounced P2 sound
Increased jugular venous pressure
Peripheral edema
Ascites
DiagnosisCorrected alveolar-arterial oxygen gradient (Abnormal if > 15-20 mmHg) with or without hypoxemia (PaO2), all in sitting positionTransthoracic echocardiography.
Contrast-enhanced echocardiography
Degree of severity: Alveolar-Arterial oxygen gradient > 15 mmHg,mild with PaO2 > 80 mmHg, moderate > 60 mmHg to < 80 mmHg, severe > 50 mmHg to < 60 mmHg or very severe < 50 mmHg
Pulmonary angiography
TreatmentLiver transplantEndothelin receptor antagonist, phosphodiesterase type-5inhibitors, prostanoids, and combination therapy
Sildenafil alone or combined with prostacyclins
Transjugular intrahepatic portosystemic shunting
Liver transplant