Hepatopulmonary Syndrome and Portopulmonary Hypertension in the Same Patient

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are the most common pulmonary vascular complications in patients with liver disease. We present a 71-year old woman with alcoholic cirrhosis and 6 month history of progressive shortness of breath with mild hypoxemia at rest. Results of spirometry was normal and diffusing capacity of the lung for carbon monoxide (DLCO) was severity reduced. Echocardiography with contrast enhancement revealed extra cardiac shunt. Three months after her initial presentation, a new echocardiography was performed with pulmonary systolic pressure increased and in a right heart catheterization (RHC) the mean pulmonary arterial pressure (PAPm) was 50 mmHg, this evidence supported the diagnosis of severe portopulmonary hypertension after HPS.