Purpose of review: Increases in pulmonary artery pressure are seen in 20% of patients with liver cirrhosis due to a high cardiac output state together with circulatory volume overload. Some patients develop true pulmonary arterial hypertension with pathological changes in the pulmonary vasculature, an elevated pulmonary vascular resistance, and an increased transpulmonary gradient. Survival of patients with portopulmonary hypertension is poor once it becomes moderate to severe, whether or not liver transplant is undertaken. This increase in mortality is the result of right heart dysfunction.
Recent findings: Screening for portopulmonary hypertension occurs in most transplant centers using Doppler echocardiography to determine the right ventricular systolic pressure. The role of right heart function has been identified as a significant component in the survival of the new graft and the recipient during orthotopic liver transplant.
Summary: Our understanding of the management of patients with portopulmonary hypertension and the role of liver transplantation is improving as the results of multicenter databases are analyzed. Single center series are reaching a reasonable size, adding to our knowledge base, and helping in the development of evidence-based practice guidelines. The importance of right heart function and the use of perioperative echocardiography are better defined in the management of orthotopic liver transplant.