Usefulness of acute pulmonary vasoreactivity test of sildenafil in treatment of portopulmonary hypertension. A case report

Abstract

A 50-year-old man diagnosed with liver cirrhosis type C was referred to our hospital because of right heart failure with pulmonary hypertension. Echocardiography revealed enlargement of the right atrium and ventricle with severe tricuspid regurgitation. The peak flow velocity of tricuspid regurgitation by continuous wave Doppler echocardiography was 452 cm/s. Right heart catheterization demonstrated severe pulmonary hypertension [pulmonary arterial pressure (PAP) systolic/diastolic/mean = 73/20/41 mmHg and pulmonary vascular resistance (PVR) = 509 dyn s cm^-5] with portal hypertension. We diagnosed the patient as having portopulmonary hypertension (PoPH). Although we treated the patient with a prostacyclin analog, tricuspid regurgitation velocity was increased to 480 cm/s four years after the start of the therapy. To select drugs for the treatment of PoPH, we performed an acute vasoreactivity test of sildenafil during right heart catheterization. Since single administration of sildenafil (20 mg) decreased PAP (93/30/55-77/27/44 mmHg) and PVR (908-833 dyn s cm^-5), we added sildenafil (20 mg, t.i.d.) to the prostacyclin analog. Tricuspid regurgitation velocity decreased to 403 cm/s one year after the addition of sildenafil. An acute vasoreactivity test of sildenafil during right heart catheterization was useful for the decision of the drug to be used in the treatment of PoPH.

Keywords: Acute pulmonary vasoreactivity test; Portopulmonary hypertension; Sildenafil.

Figure 1

Transthoracic echocardiography. Left panels: four years after the start of treatment with a prostacyclin analog and before sildenafil treatment. Right panels: one year after the addition of sildenafil. Parasternal short-axis view recorded in diastole (A and D) and end-systole (B and E). Tricuspid regurgitation velocity (C and F). Four years after the start of prostacyclin analog treatment before sildenafil treatment, left ventricle was D-shaped during systole and end-diastole due to the enlargement of the right ventricle. Tricuspid regurgitation velocity was 480 cm/s. One year after the addition of sildenafil, D-shape of left ventricle was improved and tricuspid regurgitation velocity decreased to 403 cm/s.