Therapeutic options in pulmonary hepatic vascular diseases

Abstract

Pulmonary-hepatic vascular disorders are frequent complications in patients with portal hypertension and cirrhosis. Both hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are associated with increased morbidity and mortality. The diagnosis of HPS should be confirmed early by arterial blood gas analysis and contrast enhanced echocardiography whereas POPH is finally diagnosed by presence of pulmonary arterial hypertension evaluated via right heart catheterization and presence of portal hypertension. Therapeutic options are initiation of long term oxygen therapy and liver transplantation in patients with severe HPS. Patients with POPH should receive targeted medical therapies with endothelin receptor antagonists, phosphodiesterase-5 inhibitors and/or prostanoids. In contrast, β-blockers should be avoided. It is unclear whether liver transplantation cures POPH or not. This review summarizes current knowledge of underlying conditions and focuses on therapeutic options in patients with pulmonary-hepatic vascular disorders.

Keywords::

• cirrhosis
• hepatopulmonary syndrome
• portopulmonary hypertension
• pulmonary-hepatic vascular disorders
• pulmonary hypertension

Financial & competing interests disclosure

V Fuhrmann has received research grants from Air Liquide, Vivisol & Actelion. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Pulmonary-hepatic vascular disorders as hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are frequent findings in patients with liver disease.

• Pulmonary-hepatic vascular disorders seem to be progressive diseases. Early diagnosis and early initiation of adequate therapeutic measures should be performed in experienced centers.

HPS

• HPS is defined by impaired arterial oxygenation due to intrapulmonary shunting and presence of acute or chronic hepatic disorder.

• HPS occurs in up to 33% of patients with cirrhosis.

• Contrast enhanced echocardiography is the gold standard for detection of intrapulmonary shunting.

• Mortality in HPS is more than twofold increased in comparison to cirrhotics without HPS.

• To date, liver transplantation is the only curative therapy for HPS.

POPH

• POPH is a kind of pulmonary arterial hypertension in patients with portal hypertension with significantly increased mortality.

• Transthoracic echocardiography is used for screening, the diagnosis is established by right heart catheterization.

• Medical treatment is the first therapeutic option in patients with moderate and severe POPH.

• Endothelin receptor antagonists (bosentan and ambrisentan), prostanoids (epoprostenol, terprostinil and iloprost), phosphodiesterase Type 5-inhibitors (sildenafil) are potential treatment options.

• Liver transplantation frequently does not lead to resolution of POPH.

Notes