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

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

Research output: Contribution to journalArticle

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

Fingerprint

Pulmonary Hypertension
Brain Natriuretic Peptide
Therapeutics
Rare Diseases
Vascular Resistance
Stroke Volume
Echocardiography
Hemodynamics
Magnetic Resonance Imaging
Blood Pressure
Lung
Survival
ambrisentan
Tadalafil

Keywords

  • MRI
  • Pulmonary arterial hypertension
  • Therapy

ASJC Scopus subject areas

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

Cite this

Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension. / Hassoun, Paul M; Zamanian, Roham T.; Damico, Rachel L; Lechtzin, Noah; Khair, Rubina; Kolb, Todd Matthew; Tedford, Ryan J.; Hulme, Olivia L.; Housten, Traci; Pisanello, Chiara; Sato, Takahiro; Pullins, Erica H.; Corona Villalobos, Celia; Zimmerman, Stefan; Gashouta, Mohamed A.; Minai, Omar A.; Torres, Fernando; Girgis, Reda E.; Chin, Kelly; Mathai, Stephen.

In: American Journal of Respiratory and Critical Care Medicine, Vol. 192, No. 9, 01.11.2015, p. 1102-1110.

Research output: Contribution to journalArticle

Hassoun, Paul M ; Zamanian, Roham T. ; Damico, Rachel L ; Lechtzin, Noah ; Khair, Rubina ; Kolb, Todd Matthew ; Tedford, Ryan J. ; Hulme, Olivia L. ; Housten, Traci ; Pisanello, Chiara ; Sato, Takahiro ; Pullins, Erica H. ; Corona Villalobos, Celia ; Zimmerman, Stefan ; Gashouta, Mohamed A. ; Minai, Omar A. ; Torres, Fernando ; Girgis, Reda E. ; Chin, Kelly ; Mathai, Stephen. / Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension. In: American Journal of Respiratory and Critical Care Medicine. 2015 ; Vol. 192, No. 9. pp. 1102-1110.
@article{54167cef2a64498493ef80e7daac15ec,
title = "Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension",
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",
keywords = "MRI, Pulmonary arterial hypertension, Therapy",
author = "Hassoun, {Paul M} and Zamanian, {Roham T.} and Damico, {Rachel L} and Noah Lechtzin and Rubina Khair and Kolb, {Todd Matthew} and Tedford, {Ryan J.} and Hulme, {Olivia L.} and Traci Housten and Chiara Pisanello and Takahiro Sato and Pullins, {Erica H.} and {Corona Villalobos}, Celia and Stefan Zimmerman and Gashouta, {Mohamed A.} and Minai, {Omar A.} and Fernando Torres and Girgis, {Reda E.} and Kelly Chin and Stephen Mathai",
year = "2015",
month = "11",
day = "1",
doi = "10.1164/rccm.201507-1398OC",
language = "English (US)",
volume = "192",
pages = "1102--1110",
journal = "American Journal of Respiratory and Critical Care Medicine",
issn = "1073-449X",
publisher = "American Thoracic Society",
number = "9",

}

TY - JOUR

T1 - Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension

AU - Hassoun, Paul M

AU - Zamanian, Roham T.

AU - Damico, Rachel L

AU - Lechtzin, Noah

AU - Khair, Rubina

AU - Kolb, Todd Matthew

AU - Tedford, Ryan J.

AU - Hulme, Olivia L.

AU - Housten, Traci

AU - Pisanello, Chiara

AU - Sato, Takahiro

AU - Pullins, Erica H.

AU - Corona Villalobos, Celia

AU - Zimmerman, Stefan

AU - Gashouta, Mohamed A.

AU - Minai, Omar A.

AU - Torres, Fernando

AU - Girgis, Reda E.

AU - Chin, Kelly

AU - Mathai, Stephen

PY - 2015/11/1

Y1 - 2015/11/1

N2 - 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

AB - 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

KW - MRI

KW - Pulmonary arterial hypertension

KW - Therapy

UR - http://www.scopus.com/inward/record.url?scp=84946565060&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84946565060&partnerID=8YFLogxK

U2 - 10.1164/rccm.201507-1398OC

DO - 10.1164/rccm.201507-1398OC

M3 - Article

C2 - 26360334

AN - SCOPUS:84946565060

VL - 192

SP - 1102

EP - 1110

JO - American Journal of Respiratory and Critical Care Medicine

JF - American Journal of Respiratory and Critical Care Medicine

SN - 1073-449X

IS - 9

ER -