Portopulmonary Hypertension and Hepatopulmonary Syndrome: Is Transplant Always the Answer?

Portopulmonary hypertension (POPH) and hepatopulmonary syndrome (HPS) are two well-described liver–lung diseases. POPH is present in 5–10 % of patients with cirrhosis and is defined by the development of pulmonary arterial hypertension in the setting of portal hypertension. All patients who undergo a liver transplant (LT) evaluation are screened for this with a transthoracic echocardiogram, and if POPH is diagnosed, this may serve as a relative contraindication for a LT. If a patient with mild to moderate POPH responds to medical therapy, a LT may still be an option. HPS is more common affecting up to 30 % of patients with cirrhosis. These patients will have abnormal arterial oxygenation related to dilated intrapulmonary microvasculature. The development of HPS is an indication for a LT. Both of these conditions are recognized to increase morbidity and mortality, and a transplant center can apply for Model for End-Stage Liver Disease exception points in order to decrease a patient’s waitlist time.