ABSTRACT: Terlipressin has been shown to improve both pulmonary and systemic hemodynamics in stable cirrhotic patients with pulmonary hypertension, whereas other vasoconstrictors may cause pulmonary pressures to deteriorate. We investigated the pulmonary and systemic hemodynamic effects of the first terlipressin dose (2 mg) in 7 cirrhotic patients with PH presenting with variceal bleeding (n=4) or hepatorenal syndrome (n=3). Terlipressin decreased pulmonary vascular resistance (158.8±8.9 vs 186.5±13.9 dynes • sec • cm-5; P=0.003) together with an increase in systemic vascular resistance (2143±126 vs 1643±126 dynes • sec • cm-5; P<0.001). Terlipressin should be the vasoconstrictor treatment of choice when patients present with variceal bleeding or HRS.