Pulmonary Vascular Complications of Liver Disease

• Published in: American journal of respiratory and critical care medicine 2013-01, Vol.187 (2), p.133-143
• Main Authors: FRITZ, Jason S, FALLON, Michael B, KAWUT, Steven M
• Format: Article
• Language: English
• Subjects: Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Angiogenesis; Biological and medical sciences; Blood; Blood vessels; Concise Clinical Review; Dyspnea; Growth factors; Hemodynamics; Hepatopulmonary Syndrome - diagnosis; Hepatopulmonary Syndrome - etiology; Hepatopulmonary Syndrome - pathology; Hepatopulmonary Syndrome - physiopathology; Hepatopulmonary Syndrome - therapy; Humans; Hypertension; Hypertension, Portal - complications; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - pathology; Hypertension, Pulmonary - physiopathology; Hypertension, Pulmonary - therapy; Intensive care medicine; Liver cirrhosis; Liver diseases; Liver Diseases - complications; Liver Transplantation; Lung - pathology; Lung - physiopathology; Medical sciences; Pathophysiology; Patients; Pneumology; Prognosis; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Pulse oximetry
• ISSN: 1073-449X; 1535-4970
• DOI: 10.1164/rccm.201209-1583CI

Hepatopulmonary syndrome and portopulmonary hypertension are two pulmonary vascular complications of liver disease. The pathophysiology underlying each disorder is distinct, but patients with either condition may be limited by dyspnea. A careful evaluation of concomitant symptoms, the physical examination, pulmonary function testing and arterial blood gas analysis, and echocardiographic, imaging, and hemodynamic studies is crucial to establishing (and distinguishing) these diagnoses. Our understanding of the pathobiology, natural history, and treatment of these disorders has advanced considerably over the past decade; however, the presence of either still increases the risk of morbidity and mortality in patients with underlying liver disease. There is no effective medical treatment for hepatopulmonary syndrome. Although liver transplantation can resolve hepatopulmonary syndrome, there appears to be worse survival even with transplantation. Liver transplantation poses a very high risk of death in those with significant portopulmonary hypertension, where targeted medical therapies may improve functional status and allow successful transplantation in a small number of select patients.