Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension

Paul M. Hassoun, Roham T. Zamanian, Rachel Damico, Noah Lechtzin, Rubina Khair, Todd M. Kolb, Ryan J. Tedford, Olivia L. Hulme, Traci Housten, Chiara Pisanello, Takahiro Sato, Erica H. Pullins, Celia P. Corona-Villalobos, Stefan L. Zimmerman, Mohamed A. Gashouta, Omar A. Minai, Fernando Torres, Reda E. Girgis, Kelly Chin, Stephen C. Mathai

Research output: Contribution to journalArticle

72 Scopus citations

Abstract

Background: Scleroderma-associated pulmonary arterial hypertension (SSc-PAH) is a rare disease characterized by a very dismal response to therapy and poor survival. We assessed the effects of up-front combination PAH therapy in patients with SSc-PAH. Methods: In this prospective, multicenter, open-label trial, 24 treatment-naive patients with SSc-PAH received ambrisentan 10 mg and tadalafil 40 mg daily for 36 weeks. Functional, hemodynamic, and imaging (cardiac magnetic resonance imaging and echocardiography) assessments at baseline and 36 weeks included changes in right ventricular (RV) mass and pulmonary vascular resistance as co-primary endpoints and stroke volume/pulmonary pulse pressure ratio, tricuspid annular plane systolic excursion, 6-minute walk distance, and N-terminal pro-brain natriuretic peptide as secondary endpoints. Results: At 36 weeks, we found that treatment had resulted in significant reductions in median (interquartile range [IQR])RVmass (28.0 g [IQR, 20.6-32.9] vs. 32.5 g [IQR, 23.2-41.4]; P

Original languageEnglish (US)
Pages (from-to)1102-1110
Number of pages9
JournalAmerican Journal of Respiratory and Critical Care Medicine
Volume192
Issue number9
DOIs
StatePublished - Nov 1 2015

Keywords

  • MRI
  • Pulmonary arterial hypertension
  • Therapy

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine

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    Hassoun, P. M., Zamanian, R. T., Damico, R., Lechtzin, N., Khair, R., Kolb, T. M., Tedford, R. J., Hulme, O. L., Housten, T., Pisanello, C., Sato, T., Pullins, E. H., Corona-Villalobos, C. P., Zimmerman, S. L., Gashouta, M. A., Minai, O. A., Torres, F., Girgis, R. E., Chin, K., & Mathai, S. C. (2015). Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension. American Journal of Respiratory and Critical Care Medicine, 192(9), 1102-1110. https://doi.org/10.1164/rccm.201507-1398OC