A useful review of “pulmonary complications in chronic liver disease” (Hepatology 2014; 59: 1627-37) has been published.
The main topics included hepatopulmonary syndrome (HPS) , portopulmonary hypertension (POPH), and hepatic hydrothorax (HH).
A few of the key points:
HPS is most common of these conditions and is identified in 5-30% of cirrhosis patients. It is identified with abnormal oxygenation (screening with pulse ox <96%) due to intrapulmonary vascular dilatations. There is no established medical therapy. It is reversible with liver transplantation.
The hallmark of POPH is the development of pulmonary arterial hypertension associated with portal hypertension. It occurs in 5-10% of cirrhosis patients and often presents as dyspnea on exertion/fatigue. There are numerous pharmacologic treatments that may be useful, include the following:
- prostacyclin analogs like epoprostenol
- endothelin receptor antagonists like boesentan
- phosphodiesterase-5 inhibitors like sildenafil, vardenafil, and tadalafil
Severe POPH is a relative contraindication for liver transplantation.
HH is a transudative pleural effusion seen in 5-10% of cirrhosis patients. Initial management includes salt restriction and diuretics. Transjugular intrahepatic portosystemic shunt and thoracentesis are second-line options. Liver transplantation is curative.
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