Key result
Bosentan treatment in a 76-year-old male with portopulmonary hypertension improved 6-minute walking distance from 300 to 540 m and reduced NT-proBNP from 4928 to 640 ng/mL over 14 weeks.
Why the study?
Does bosentan improve exercise capacity and NT-proBNP in a patient with portopulmonary hypertension related to liver cirrhosis?
Case Report (n=1)
Does bosentan improve exercise capacity and NT-proBNP in a patient with portopulmonary hypertension related to liver cirrhosis?
Bosentan may be a promising therapeutic option for improving exercise capacity and reducing NT-proBNP in patients with portopulmonary hypertension.
Should not change practice yet in portopulmonary hypertension; single case leaves open need for controlled trials.
Pulmonary arterial hypertension (PAH) with coexisting portal hypertension has been defined as portopulmonary hypertension (PPHTN). It is often related to liver cirrhosis of various aetiologies and is associated with a high mortality rate. Endothelin-1 (ET) is supposed to play an important role in the pathogenesis of PAH as well as portal hypertension. Therefore, therapy with an ET(A)/ET(B) receptor antagonist might be of use in the treatment of PPHTN. We report the case of a 76-year-old male with liver cirrhosis owing to chronic hepatitis C virus infection and PPHTN who was treated with the dual ET(A)/ET(B) receptor antagonist bosentan. The patient showed remarkable improvement of 6-min walking distance from 300 to 480 m after 2 weeks and to 540 m after 14 weeks, respectively. In addition, a significant decline of N-terminal pro B-type natriuretic peptide fraction (NT-proBNP) from 4928 ng mL(-1) to 640 ng mL(-1) was observed. Bosentan might be a promising new therapeutical option for patients suffering from PPHTN.
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Grander et al. (2006) conducted a case report in Portopulmonary hypertension related to liver cirrhosis owing to hepatitis C (n=1). Bosentan was evaluated on 6-minute walking distance and NT-proBNP. Bosentan treatment in a 76-year-old male with portopulmonary hypertension improved 6-minute walking distance from 300 to 540 m and reduced NT-proBNP from 4928 to 640 ng/mL over 14 weeks.