Abstract
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 isnoeffective medical treatment for hepatopulmonary syndrome. Although liver transplantation can resolve hepatopulmonary syndrome, there appears to be worse survival evenwith 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.
Original language | English (US) |
---|---|
Pages (from-to) | 133-143 |
Number of pages | 11 |
Journal | American journal of respiratory and critical care medicine |
Volume | 187 |
Issue number | 2 |
DOIs | |
State | Published - Jan 15 2013 |
Keywords
- Hepatopulmonary syndrome
- Hypertension
- Liver cirrhosis
- Pulmonary
- Pulmonary circulation
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
- Critical Care and Intensive Care Medicine