Table of Contents Author Guidelines Submit a Manuscript
Canadian Respiratory Journal
Volume 22, Issue 1, Pages 42-46
Original Article

Effectiveness of Phosphodiesterase-5 Inhibitor Therapy for Portopulmonary Hypertension

Jolene H Fisher,1,2 Sindhu R Johnson,1,3 Cathy Chau,3 Amie T Kron,3 and John T Granton1,2

1University Health Network Pulmonary Hypertension Program, Canada
2Division of Respirology, Department of Medicine, Toronto General Hospital, Canada
3Division of Rheumatology, Department of Medicine, Toronto Western Hospital, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada

Copyright © 2015 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


BACKGROUND: Portopulmonary hypertension is associated with significant morbidity and mortality. Phosphodiesterase-5 inhibitor therapy is efficacious in other causes of WHO group I pulmonary arterial hypertension.

OBJECTIVE: To evaluate the efficacy and safety of phosphodiesterase-5 inhibitor therapy in patients with portopulmonary hypertension.

METHODS: A single-centre retrospective cohort study that included patients with a diagnosis of portopulmonary hypertension was performed. The primary outcome was change in pulmonary vascular resistance after six months of phosphodiesterase-5 inhibitor therapy. A secondary evaluation investigated the effect on other hemodynamic measurements, 6 min walk distance, functional class, safety outcomes and survival.

RESULTS: Of 1385 patients screened, 25 patients with portopulmonary hypertension were identified, of whom 20 received a phosphodiesterase-5 inhibitor. After six months, there was a significant decrease in pulmonary vascular resistance (−236 dyn·s·cm−5 [95% CI −343 dyn·s·cm−5 to −130 dyn·s·cm−5]; P<0.001), mean pulmonary artery pressure (−8.9 mmHg [95% CI −13.7 mmHg to −4.2 mmHg]; P=0.001) and an increase in Fick cardiac output (0.9 L/min [95% CI 0.1 L/min to 1.6 L/min]; P=0.02). There was no change in 6 min walk distance. The proportion of subjects with a WHO functional class III or IV was significantly reduced at six months compared with baseline (18% versus 61%; P=0.002). Safety outcomes did not reveal any adverse events.

CONCLUSIONS: Phosphodiesterase-5 inhibitor therapy improved hemodynamics and functional class at six months in a cohort of patients with portopulmonary hypertension.