Bosentan in Treatment of Pulmonary Arterial Hypertension

NCT00266162 | Completed Phase 4

• 2 other identifiers: MP 3.201G10210
• Study Type: interventional
• Target: 60
• Locations: 1 country
• Sites: 8

Brief Summary

Eisenmenger's syndrome presents as a severe clinical picture of polymorbidity that constitutes a great burden at the individual as well as the familial and social level. The combination of critically increased pulmonary vascular resistance, progressive pressure load of the right ventricle and disturbance of pulmonary gas exchange result in long-term polymorbidity. The objective of this study is to look into the effects of medium-term pulmonary pressure-lowering treatment with oral bosentan in patients with congenital heart defects and clinically relevant pulmonary arterial hypertension (PAH), taking advantage of extensive diagnostic procedures.

Conditions

Eisenmenger Syndrome

Keywords

Heart Defects, Congenital; Eisenmenger Complex; Hypertension, Pulmonary; Pulmonary Heart Disease; Pulmonary Circulation; Vascular Resistance

Outcome Measures

Primary Outcomes (3)

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

• peripheral oxygen saturation (SatO2)

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

Secondary Outcomes (3)

• NYHA class

• increase in pulmonary reagibility by bosentan therapy

• normalisation of vasoactive mediators by bosentan therapy

Interventions

Bosentan administration DRUG

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• 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.

You may not qualify if:

• 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:
• 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)

Sponsors & Collaborators

• Competence Network for Congenital Heart Defects: lead
• German Federal Ministry of Education and Research: collaborator
• Actelion: collaborator

Study Sites (8)

Kinderkardiologie Universitätsklinikum Freiburg

Freiburg im Breisgau, Baden-Wurttemberg, D-79106, Germany

Location

Deutsches Herzzentrum Muenchen

Munich, Bavaria, D-80636, Germany

Location

Universitätsklinikum Giessen and Marburg

Giessen, Hesse, D-35385, Germany

Location

Herz-und Diabeteszentrum NRW

Bad Oeynhausen, North Rhine-Westphalia, D-32545, Germany

Location

Universitätsklinikum Schleswig-Holstein Campus Kiel

Kiel, North Rhine-Westphalia, D-24105, Germany

Location

Universitätsklinikum des Saarlandes

Homburg, Saarland, D-66421, Germany

Location

Universitätsklinikum der Martin-Luther-Universität Halle-Wittenberg

Halle, Saxony-Anhalt, D-06097, Germany

Location

Deutsches Herzzentrum Berlin

Berlin, State of Berlin, D-13353, Germany

Location

MeSH Terms

Conditions

Eisenmenger Complex; Heart Defects, Congenital; Hypertension, Pulmonary; Pulmonary Heart Disease

Condition Hierarchy (Ancestors)

Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases

Study Officials

• Ingram Schulze-Neick, MD

  German Heart Institute

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: phase 4
• Allocation: NON RANDOMIZED
• Masking: NONE
• Purpose: TREATMENT
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER GOV

Study Record Dates

• First Submitted: December 15, 2005
• First Posted: December 16, 2005
• Study Start: August 1, 2004
• Primary Completion: February 1, 2008
• Study Completion: February 1, 2008
• Last Updated: May 7, 2008

Record last verified: 2008-05

Locations

DE (8)