Calendar | For Your Patients | PHA Main Site | Contact Us | About Us | Not a registered user? Sign up here.

Resource Library

Differences Between Idiopathic Pulmonary Arterial Hypertension and Connective Tissue Disease-Associated Pulmonary Arterial Hypertension After 16 Weeks of Bosentan Monotherapy: the COMPASS-3 Study

Fernando Torres


Myung Park


Srinivas Murali


Himanshu Gupta

Francisco Soto


N. Frey

Raymond Benza


Reviews

  Sign in to add a review

0 comments
Leave a Comment

Conference: 2010 International PHA Conference and Scientific Sessions

Release Date: 06.24.2010

Presentation Type: Abstracts

Torres F1, Park MH2, Murali S3, Gupta H4, Soto FJ5, Frey N6, Benza RL3
1. University of Texas Southwestern Medical Center, Dallas, TX, USA 
2. University of Maryland School of Medicine, Baltimore, MD, USA 
3. Allegheny General Hospital, Pittsburgh, PA, USA 
4. University of Alabama at Birmingham, Birmingham, AL, USA 
5. Prevea Health, Green Bay, WI, USA 
6. Actelion Pharmaceuticals US, Inc., San Francisco, CA, USA

BACKGROUNDThough there are distinct differences in the clinical profile between idiopathic pulmonary arterial hypertension (IPAH) and connective tissue disease-associated PAH (CTD-APAH), it is less clear if there are meaningful differences in therapeutic response to bosentan therapy. Here we report on differences between these two PAH subgroups for 6-minute walk test distance (6MWD), functional class (FC) and right heart catheterization (RHC) at baseline and after 16 weeks of bosentan monotherapy.

METHODSOne hundred therapy-naïve PAH patients with baseline 6MWD 150-360m received bosentan for 16 weeks, including 27 CTD-APAH and 55 IPAH. 6MWD, FC, and RHC were assessed at baseline and Week 16.

RESULTS6MWD results were similar between the two subgroups (Table). 89% of CTD-APAH patients and 75% of IPAH patients were WHO functional class (FC) III-IV at baseline. At Week 16, 100% of CTD-APAH patients improved or maintained FC while 94.1% of IPAH patients improved or maintained FC. Overall, the CTD-APAH group demonstrated favorable change from baseline in RHC parameters similar to IPAH patients.

CONCLUSIONSThe treatment response to 16 weeks of bosentan monotherapy in terms of improvements in FC and 6MWD are not different in IPAH and CTD-APAH patients. The differential improvements in hemodynamics in these patient subgroups need further investigation.

Table: 6MWD distances among groups.

6MWD, m

CTD-APAH

IPAH

Total Cohort

 

Mean (SD)

n

Mean (SD)

n

Mean (SD)

n

Baseline

267.5 (64.7)

27

279.7 (54.2)

55

273.0 (55.9)

100

Week 16

291.7 (99.3)

25

303.6 (86.7)

54

293.0 (92.2)

94

Change from BL to Wk 16

27.6 (69.6)

25

23.4 (76.1)

54

22.2 (75.8)

94

P value (Change)

0.0111

 

0.0147

 

0.0008

 

There were no significant differences between CTD-APAH and IPAH groups.