Combination therapy of bosentan and ambrisentan for portopulmonary hypertension

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terase 5 inhibitor) from 2012 to 2013. Results: After 1 year of oral triple combination therapy WHO-FC improved from III to II in all three patients and 6 min walking test improved from 273 ± 98 to 553 ± 31 (P b 0.05) and mean pulmonary arterial pressure decreased from 52.7 ± 9.5 to 31 ± 6.2 mmHg (P = 0.05) and cardiac index increased from 1.5 ± 0.2 to 2.82 ± 0.17 mmHg (p b 0.001). All three patients were tolerable with triple upfront combination therapy and had no severe side effect. Conclusions: Oral triple upfront combination therapy improved symptoms, exercise capacity and hemodynamics for the patients with severe I/HPAH in long-term without severe adverse effect.

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