Pulmonary manifestations of chronic liver disease: a comprehensive review

Abstract

Hepatopulmonary syndrome (HPS) and porto-pulmonary hypertension (PoPH) represent relatively common pulmonary vascular complications of advanced liver disease. Despite distinct differences in their pathogenetic background, both clinical states are characterized by impaired arterial oxygenation and limited functional status, and are associated with increased pre-transplantation mortality. Accumulation of ascitic fluid in the pleural cavity, known as hepatic hydrothorax (HH), is another frequent manifestation of decompensated cirrhosis, which may cause severe respiratory dysfunction, depending on the volume of the effusion, the rapidity of its development and its resistance to therapeutic measures. Orthotopic liver transplantation constitutes the only effective treatment able to resolve the pulmonary complications of liver disease. A prioritization policy for liver transplantation has evolved over the past years regarding advanced stages of HPS, yielding favorable outcomes regarding post-transplantation survival and HPS resolution. In contrast, severe PoPH is associated with poor post-transplantation survival. Hence, liver transplantation is recommended only for patients with PoPH and an acceptable reduction in pulmonary pressure values, after receiving PoPH-targeted vasodilating therapy. This review focuses on basic pathogenetic and diagnostic principles and discusses the current therapeutic approaches regarding HPS, PoPH, and HH.

Keywords: Cirrhosis; chronic liver disease; hepatopulmonary syndrome; liver transplantation; porto-pulmonary hypertension.

Figure 1

Diagnostic algorithm for patients with chronic liver disease and dyspnea RVSP, right ventricular systolic pressure; mPAP, mean pulmonary arterial pressure; PVR, pulmonary vascular resistance; PAWP, pulmonary artery wedge pressure; IPVS, intrapulmonary vascular shunting; ECHO, echocardiography; P(A-a)O2, Alveolar-arterial oxygen pressure gradient; POPH, portopulmonary hypertension; HPS, hepatopulmonary syndrome