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
Published online Jul 14, 2016. doi: 10.3748/wjg.v22.i26.6008
Hepatopulmonary syndrome | Portopulmonary hypertension | |
Pathophysiology | Severe vasodilatation | Severe vasoconstriction |
Production of endothelin-1 and tumor necrosis alpha and eNOS and iNOS | Concentric intimal fibrosis, and smooth muscle hyperplasia and hypertrophy | |
Increase of CO | Endothelin-1, prostacyclin and thromboxane | |
Vasculoendothelial growth factor-A | ||
Angiogenesis | ||
Clinical features | Most patients are asymptomatic | Dyspnea |
Dyspnea | Orthopnea | |
Platypnea | Fatigue | |
Orthodeoxia | Syncope | |
Significant sleep-time oxygen desaturation | Chest pain | |
Lightheadedness | ||
Tricuspid regurgitation murmur, with a pronounced P2 sound | ||
Increased jugular venous pressure | ||
Peripheral edema | ||
Ascites | ||
Diagnosis | Corrected alveolar-arterial oxygen gradient (Abnormal if > 15-20 mmHg) with or without hypoxemia (PaO2), all in sitting position | Transthoracic 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 | ||
Treatment | Liver transplant | Endothelin receptor antagonist, phosphodiesterase type-5inhibitors, prostanoids, and combination therapy |
Sildenafil alone or combined with prostacyclins | ||
Transjugular intrahepatic portosystemic shunting | ||
Liver transplant |
- Citation: Surani SR, Mendez Y, Anjum H, Varon J. Pulmonary complications of hepatic diseases. World J Gastroenterol 2016; 22(26): 6008-6015
- URL: https://www.wjgnet.com/1007-9327/full/v22/i26/6008.htm
- DOI: https://dx.doi.org/10.3748/wjg.v22.i26.6008