Functional improvement in a patient with cirrhosis and portopulmonary hypertension treated by sildenafil for 2 years

Portopulmonary hypertension (PPH) represents an uncommon form of pulmonary arterial hypertension (PAH) associated with portal hypertension and cirrhosis. PPH is a severe disease with a poor short-term prognosis, with a possibility of liver transplantation only for patients with mild to moderate PAH. Patients with PAH could be candidates for specific vasodilator-based treatment, the choice of which being guided by their toxicity profile. We report here the case of a 46-year-old woman with a cirrhosis (Child-Pugh C) complicated by a severe PPH (mean pulmonary arterial pressure: 49 mmHg, pulmonary vascular resistance: 688 dynes, right ventricular dysfunction), with NYHA class III dyspnea and an altered 6-min walk distance (6MWD). Treatment with sildenafil (20 mg t.i.d) was started. In the first 6 months, a functional improvement was indicated by dyspnea assessed as class II, an increased distance at the 6MWD (375 m vs. 250 m without oxygen desaturation), and a normalization of right ventricular function. After one year of therapy, the benefits were maintained (6MWD: 465 m) and transthoracic echocardiography showed a normalization of pulmonary arterial pressures. Sildenafil treatment was well tolerated. At two years, haemodynamic values measured by right heart catheterization were similar to the pre-treatment values, while clinical and functional parameters remained improved. At our knowledge, this is the first case delineating the long-term functional benefit of sildenafil monotherapy, making this drug a therapeutic option in patients with PPH and cirrhosis, the usefulness of which in the waiting period for liver transplantation needs to be evaluated.