Clinical trial NCT00909337: Early Therapy of Pulmonary Arterial Hypertension
Official title: Early Therapy of Pulmonary Arterial Hypertension
Study type: INTERVENTIONAL
Overall status: COMPLETED
Phase: NA
Conditions: Pulmonary Hypertension
Systemic Sclerosis
Lead sponsor: Medical University of Graz
Sponsor class: OTHER
Collaborators: name: Actelion
First posted: 2009-05-28
Enrollment: 10
Sex: ALL
Minimum age (years): 18
Maximum age (years): 90
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: NA
Intervention model: SINGLE_GROUP
Primary purpose: TREATMENT

Brief summary: Exercise-induced increase of the pulmonary arterial pressure may be an early sign of pulmonary arterial hypertension. It has been shown that patients with normal pulmonary arterial pressure at rest but elevated pulmonary arterial pressure during exercise have a decreased exercise-capacity and may have a worse prognosis compared to patients with normal pulmonary arterial pressure values at rest and during exercise. According to the currently used definition pulmonary hypertension can be diagnosed if the mean pulmonary arterial pressure is higher than 25mmHg at rest or 30mmHg during exercise. In this study patients with a risk for pulmonary arterial hypertension (connective tissue disease) and increased pulmonary arterial pressure values during exercise are receiving a therapy with a dual endothelin receptor antagonist - bosentan, a therapy established for pulmonary arterial hypertension. The therapy effect is than compared to the recorded changes before the introduction of this therapy.

Detailed description: Bosentan is an established therapy in pulmonary arterial hypertension, which may be diagnosed when the mean pulmonary arterial pressure is \>25mmHg at rest or \>30mmHg at exercise. Bosentan has shown efficacy, if mean pulmonary arterial pressure was elevated at rest. However, there are no data available of the effects of such a therapy, if the mean pulmonary arterial pressure is normal at rest but elevated during exercise. The purpose of this study is to provide additional data on this group of patients. Our hypothesis is that patients with exercise-induced pulmonary hypertension may show a gradual worsening of pulmonary hemodynamics and potentially a development of manifest pulmonary arterial hypertension (elevated resting pulmonary arterial pressure values) without treatment, while the introduction of Bosentan may improve hemodynamics and exercise capacity in this patient group.

Eligibility criteria: Inclusion Criteria:

* exercise-induced pulmonary hypertension
* systemic sclerosis

Exclusion Criteria:

* relevant pulmonary obstruction or restriction
* relevant left cardiac disease
* recent changes in medical therapy
* recent major operations
* recent major cardiovascular diseases
* inability to perform exercise

Primary outcomes:
measure: Change of mean pulmonary arterial pressure at 50W under therapy with Bosentan compared to change of mean pulmonary arterial pressure at 50W before the introduction of therapy with Bosentan
timeFrame: 12months before Bosentan therapy vs. at begin of bosentan therapy vs. 6months under bosentan therapy
Secondary outcomes:
measure: pulmonary vascular resistance, peak oxygen uptake
timeFrame: 12months before Bosentan therapy vs. at begin of bosentan therapy vs. 6months under bosentan therapy
Other outcomes:
Locations:
city: Graz
country: Austria
facility: Medical University of Graz, Pulmonology
Study officials:
affiliation: Medical University of Graz
name: Horst Olschewski, MD
role: PRINCIPAL_INVESTIGATOR
References:
citation: Kovacs G, Maier R, Aberer E, Brodmann M, Graninger W, Kqiku X, Scheidl S, Troster N, Hesse C, Rubin L, Olschewski H. Pulmonary arterial hypertension therapy may be safe and effective in patients with systemic sclerosis and borderline pulmonary artery pressure. Arthritis Rheum. 2012 Apr;64(4):1257-62. doi: 10.1002/art.33460. Epub 2011 Nov 11.