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‘Long term use of Sildenafil in inoperable Chronic Thromboembolic Pulmonary Hypertension'

Jay Suntharalingam, Pulmonary Vascular Diseases Unit, Papworth Hospital, Email: jaysunth@btopenworld.com; Carmen M Treacy, Pulmonary Vascular Diseases Unit, Papworth Hospital, Email: Carmen.treacy@papworth.nhs.uk; Natalie J Doughty, Pulmonary Vascular Diseases Unit, Papworth Hospital, Email: natalie.doughty@papworth.nhs.uk; Kimberley Goldsmith, MRC Biostatistics Unit, Cambridge, Email: kimberley.goldsmith@papworth.nhs.uk; Elaine Soon, Pulmonary Vascular Diseases Unit, Papworth Hospital, Email: Elaine.soon@papworth.nhs.uk; Mark R Toshner, Pulmonary Vascular Diseases Unit, Papworth Hospital, Email: mark.toshner@papworth.nhs.uk; Karen K Sheares, Pulmonary Vascular Diseases Unit, Papworth Hospital, Email: karen.sheares@papworth.nhs.uk; Rodney Hughes, Pulmonary Vascular Diseases Unit, Papworth Hospital, Email: rodneyhughes@onetel.com; Nicholas W Morrell, University of Cambridge School of Clinical Medicine, Addenbrooke's Hospital, Cambridge, Email: nwm23@cam.ac.uk; Joanna Pepke-Zaba, Pulmonary Vascular Diseases Unit, Papworth Hospital, Email: joanna.pepkezaba@papworth.nhs.uk

joanna.pepkezaba{at}papworth.nhs.uk

Abstract

Background: There are currently no licensed medical therapies for inoperable chronic thromboembolic pulmonary hypertension (CTEPH).

Methods: In this double-blind, placebo-controlled pilot study 19 subjects with inoperable CTEPH were randomly assigned to sildenafil or placebo for 12 weeks. The primary endpoint was change in six minute walking distance (6MWD). Secondary endpoints included changes in WHO class, cardiopulmonary haemodynamics, quality of life (QOL) scores and NT-proBNP. All subjects were transferred to open-label sildenafil at the end of the study and offered repeat assessment at twelve months.

Results: There were no significant differences between the two groups with respect to change in exercise capacity. However significant improvements were seen in WHO class and pulmonary vascular resistance (PVR). 17 subjects were eligible for reassessment at 12 months and demonstrated significant improvements in 6MWD, activity and symptom components of QOL, cardiac index, PVR and NT-proBNP.

Conclusions: Although this pilot study was insufficiently powered to test the primary endpoint, it did suggest beneficial effects in favour of sildenafil in several secondary endpoints at both three and twelve months. Further larger-scale trials of sildenafil in inoperable CTEPH are required to confirm these findings and potentially increase the treatment options available for this devastating disease.

Trial registration: The study protocol was registered with the UK National Research Register database (Publication ID N0542136603).

Key Words: inoperable • pulmonary endarterectomy • pulmonary hypertension • sildenafil • thromboembolism