Pulmonary vascular complications of liver disease

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 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.

Figure 1.

Kaplan-Meier survival estimates of lung transplant candidates with and without hepatopulmonary syndrome (HPS). Reprinted by permission from Reference .

Figure 2.

Survival functions for portopulmonary hypertension (PoPH) versus idiopathic pulmonary arterial hypertension (IPAH), adjusted for age and race/ethnicity. Reprinted by permission from Reference .

Figure 3.

Kaplan-Meier survival estimates for portopulmonary hypertension (PoPH) versus idiopathic pulmonary arterial hypertension (IPAH) or familial pulmonary arterial hypertension (FPAH). Reprinted by permission from Reference .

Figure 4.

Kaplan-Meier survival estimates of patients with portopulmonary hypertension stratified by grade of cirrhosis. Reprinted by permission from Reference .