Bosentan in Treatment of Pulmonary Arterial Hypertension

• Register: ClinicalTrials.gov
• Last refreshed on: 19 February 2015
• Main ID: NCT00266162
• Date of registration: 15/12/2005
• Prospective Registration: No
• Primary sponsor: Competence Network for Congenital Heart Defects
• Public title: Bosentan in Treatment of Pulmonary Arterial Hypertension
• Scientific title: Therapy of Pulmonary Arterial Hypertension (PAH) With Bosentan in Patients With Eisenmenger Syndrome
• Date of first enrolment: August 2004
• Target sample size: 60
• Recruitment status: Completed
• URL: http://clinicaltrials.gov/show/NCT00266162
• Study type: Interventional
• Study design: Allocation: Non-Randomized, Endpoint Classification: Safety/Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment
• Phase: Phase 4

Countries of recruitment

Germany

Contacts

• Name: Ingram Schulze-Neick, MD
• Affiliation: German Heart Institute

Key inclusion & exclusion criteria

Inclusion Criteria:

- Non-specific:

- Written informed consent obtained

- Specific:

- Age at least 18 years

- Presence of cyanosis with < 93 % arterial oxygen saturation (measured by
transcutaneous pulse oximetry)

- Clinical indication for the invasive diagnostic procedures planned for the study
is given; this is evaluated on the basis of observation before, during and after
medicinal treatment)

- Presence of PAH as diagnosed by invasive methods with Rp:Rs > 0.75 measured at
rest, before testing of pulmonary vasodilatory reserve

- One of the following diagnoses:

- non-corrected large congenital shunting defect at atrial, ventricular or
arterial level: PAPVD, ASD, SVD, VSD, AVSD, TAC, APW, PDA, or a combination
of these.

- Surgically corrected shunting defect (diagnoses as above) with significant
residual defect

- Other diagnoses with univentricular physiology/haemodynamics.

Exclusion Criteria:

- Non-specific:

- pregnancy or lactation

- women of child-bearing age who are sexually active without practising reliable
methods of contraception

- any disease or impairment that, in the opinion of the investigator, excludes a
subject from participation

- substance abuse (alcohol, medicines, drugs)

- other medical, psychological or social circumstances that would adversely affect
a patient's ability to participate adequately in the study or increase the risk
to the patient or others in the case of participation.

- insufficient compliance

- subjects in whom MRI cannot be performed (contrast medium allergy,
claustrophobia, cardiac pacemaker)

- subjects who are not able to perform CPX

- Specific:

- pulmonary hypertension of any aetiology other than those specified in the
inclusion criteria

- subjects with known intolerance of NO or iloprost or their constituents

- acute decompensated heart failure within 7 days before the invasive procedure

- haemodynamic instability that would increase the risk of pulmonary arterial
reactivity testing

- arterial hypotension

- anaemia (Hb < 10 g/dl)

- decompensated symptomatic polycythaemia

- thrombocytopenia (< 50,000/µl)

- secondary impairment of organic (renal, hepatic) function

- other sources of pulmonary blood flow which render the measurement of the blood
flow to the lungs and pulmonary vascular resistance impossible

- obstruction of pulmonary blood outflow

- left ventricular diseases

- significant valvular diseases other than tricuspid or pulmonary regurgitation

- pericardial constriction

- history of stroke, myocardial infarction or life-threatening arrhythmia within 6
months before screening

- bronchopulmonary dysplasia or other chronic lung diseases

- history of significant pulmonary embolism

- other relevant diseases (e.g. HIV infection)

- trisomy 21

- Prohibited concomitant medication: Any medication listed below which has not
been discontinued at least 30 days prior to screening.

- Unspecified or other significant medication (e.g. medication for diabetes or
immunosuppression)

- Unstable medication, recent changes in dosage regimen

- Drugs to treat pulmonary hypertension (endothelin receptor antagonists, PDE-5
antagonists, prostanoids. (Specific pulmonary vasodilators during cardiac
catheterisation are allowed.)

- Other medication with vascular action

- Medication that is not compatible with bosentan or that interferes with its
metabolism (inhibitors of CYP2C9 or CYP3A4) or that, in the investigator's
opinion, may interfere with bosentan treatment



Age minimum: 18 Years
Age maximum: N/A
Gender: Both

Health Condition(s) or Problem(s) studied

Eisenmenger Syndrome

Intervention(s)

Drug: Bosentan administration

Primary Outcome(s)

maximal exercise tolerance (walking distance in the 6-minute walking test)

pulmonary-systemic ratio of arterial resistance (Rp:Rs)

peripheral oxygen saturation (SatO2)

Secondary Outcome(s)

NYHA class

normalisation of vasoactive mediators by bosentan therapy

increase in pulmonary reagibility by bosentan therapy

Secondary ID(s)

MP 3.2

01G10210

Source(s) of Monetary Support

Please refer to primary and secondary sponsors

Secondary Sponsor(s)

Actelion

German Federal Ministry of Education and Research