Early Therapy of Pulmonary Arterial Hypertension

Medical University of Graz

Status: Completed

Conditions: Systemic Sclerosis; Pulmonary Hypertension

Treatments: Drug: Bosentan

Study type: Interventional

Funder types: Other; Industry

Identifiers

NCT00909337

18-295 ex 06/07

Details and patient eligibility

About

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.

Full 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.

Enrollment: 10 patients

Sex: All

Ages: 18 to 90 years old

Volunteers: No Healthy Volunteers

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

Trial design

Primary purpose: Treatment

Allocation: N/A

Interventional model: Single Group Assignment

Masking: None (Open label)

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10 participants in 1 patient group

Bosentan

Other group

Description:

In this study, all participants have normal pulmonary arterial pressure at rest and elevated pulmonary arterial pressure during exercise. First, they were followed up for a year and were controlled after 1 year without specific therapy for pulmonary hypertension. Then Bosentan was introduced. A second control showing the effects of the therapy was done after 6 months. The changes in the "therapy period" can be compared with the changes in the "follow up period".

Treatment:

Drug: Bosentan