Successful treatment of severe portopulmonary hypertension in a patient with Child C cirrhosis by Sildenafil

TO THE EDITORS: We have read the article of Kawut et al.1 about survival of patients with portopulmonary hypertension (PPHTN) and would like to refer a patient with chronic liver failure in Child-Pugh C stage and severe portopulmonary hypertension with successful treatment with sildenafil. A 55-year-old male with chronic liver failure due to alcohol was admitted to our hospital with rest dyspnea (New York Association class IV). Portal hypertension with esophageal varices had been previously diagnosed. There was no history of cardiorespiratory disease. On presentation he showed clinical signs of liver descompensation and Child-Pugh score revealed 11 points (Child C). Echocardiography showed right ventricular and atrial dilatation with severe tricuspid regurgitation and estimated pulmonary artery systolic pressure of 100 mmHg; no pulmonary shunting as a possible sign of hepatopulmonary syndrome was observed. Right heart catheterization revealed a mean pulmonary arterial pressure of 75 mmHg, a pulmonary capillary wedge pressure of 12 mmHg and PVR of 900 dynes.sec.cm-5; an adenosine test was not realized by inestability hemodinamic. Other causes of pulmonary hypertension were excluded. Silenafil at a dose of 50 mg three times a day was started. Seven days thereafter the patient felt better, dyspnea was relieved and liver function improved. After three months, repeated echocardiography demonstrated marked hemodynamic improvement, with pulmonary artery systolic pressure of 73 mmHg. He was in class II of NYHA and six-minute walk distance was 440 m. Child Pugh score was 5 points (Child A). Kawut et al.1 found that patients with PPHTN have a greater risk of death than that of idiopathic pulmonary arterial hypertension even after controlling for differences in liver function or therapeutics approach. However, the proportion of patients with treatment is low (only one patient with bosentan and none with sildenafil). Current therapy is based on treatment options proven in idiopathic pulmonary hypertension. Bosentan, a nonselective endothelin receptor antagonist, is used in the treatment of pulmonary artery hypertension. Bosentan is mainly eliminated from the body by hepatic metabolism and subsequent biliary excretion of metabolites formed by cytochrome P450; mild hepatic impairment (Child-Pugh class A) do not have a clinically relevant influence on the pharmacokinetics and should generally be avoided in patients with moderate or severe hepatic impairment and/or elevated liver aminotransferases.2 Data have been published on the efficacy and safety of Bosentan in portopulmonary hypertension in Child A and B cirrhosis;3-6 there no reports in C stage. Sildenafil, a selective phosphodiesterase-5 inhibitor that blocks the degradation of cyclic guanosine monophosphate, significantly improved exercise capacity according to the six-minute walking test and also significantly improved cardiopulmonary hemodynamics at 12 weeks in patients with pulmonary arterial hypertension; no hepatotoxicity was reported.7 Sildenafil has been used too successfully in portopulmonary patients with liver cirrhosis in Child Pugh A and B.8, 9 Our patient demonstrated marked symptomatic and hemodinamyc improvement with 3 months of treatment with sildenafil. To our knowledge this is the first time sildenafil has been used to treat this condition in Child- Pugh C. Sildenafil probably should be considered a therapeutic option in PPHTP with advanced cirrhosis. José Luis Callejas Rubio MD*, Javier Salmerón Escobar MD , Jorge González-Calvín MD , Norberto Ortego Centeno MD*, * Unit of Autoimmune Diseases, Service of Internal Medicine, Spain, Unit of Autoimmune Diseases, Service of Digestive Hospital Clínico San Cecilio Granada, Spain.