Therapy of Pulmonary Arterial Hypertension (PAH) - Treatment With Sildenafil in Eisenmenger Patients

NCT00586794 | Terminated Phase 3

• 1 other identifier: MP 3.1 Sildenafil
• Study Type: interventional
• Target: 24
• Locations: 1 country
• Sites: 20

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 provide evidence of improvement of patients exercise tolerance as well as general conditions by treatment with oral sildenafil as a specific pulmonary vasodilator.

Conditions

Pulmonary Arterial Hypertension (PAH)

Keywords

Hypertension; Eisenmenger

Outcome Measures

Primary Outcomes (1)

• To determine the distance of walking, which is performed during a 6- min walking test; oxygen saturation and relation of resistance Rp : Rs during the examination with "Herzkatheter", described as the difference between visit 1 (baseline) and visit 4

  visit 1 and visit 4 (after 26 weeks)

Secondary Outcomes (4)

• To provide normalization of pulmonary vascular function (reagibility and vasoactive mediators) in dependence on duration of the therapy

  78 weeks

• Parameters of MRI and Echo-diagnostic

  78 weeks

• Quality of life (SF-36)

  78 weeks

• Safety and tolerance of the treatment

  78 weeks

Study Arms (2)

A

ACTIVE COMPARATOR

Drug: Sildenafil

B

PLACEBO COMPARATOR

from the 26th weeks on open-label, all patients were treated with Sildenafil

Drug: Placebo

Interventions

Sildenafil DRUG

3x per day 20 mg TID

Also known as: Revatio®

A

Placebo DRUG

3x per day, 20 mg TID

B

Eligibility Criteria

• Age: 14 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

• Non-specific:
• Written informed consent obtained.
• No participation in another AMG driven study attendancing this treatment protocol
• Specific:
• Age at least 14 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 therapy)
• Presence of PAH as diagnosed by invasive methods with Rp:Rs \> 0.5 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

You may not qualify if:

• Non-specific:
• pregnancy or lactation
• women of child-bearing age who are sexually active without practising highly effective methods of contraception
• any diseases or impairment that, in the opinion of the investigator exclude 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 reliably in the study or increase the risk to themselves or others if they participated
• insufficient compliance
• missing willingness to storaging and transferring pseudonymous disease data within this study.
• subjects who are not able to perform Cardio-Pulmonary Exercise Testing (CPX).
• Specific:
• subjects with known intolerance of NO and iloprost or their constituents
• acute decompensated heart failure within the 7 days before the invasive diagnostic procedure
• clinically significant haemoptysis within the last 6 months
• hemodynamic instability which would represent an unjustifiable risk during testing of pulmonary arterial vasoreagibility
• arterial hypotension (as defined by age-specific values)

Sponsors & Collaborators

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

Study Sites (20)

Universitätsklinikum Freiburg, Klinik III Päd. Kardiologie

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

Location

Klinikum der Universität Heidelberg, Pädiatrische Kardiologie

Heidelberg, Baden-Wurttemberg, D-69120, Germany

Location

Medizinische Klinik und Poliklinik, Abteilung Innere Medizin III

Heidelberg, Baden-Wurttemberg, D-69120, Germany

Location

Klinikum Stuttgart, Olgahospital, Klinik für Kinderheilkunde und Jugendmedizin, Pädiatrie 3

Stuttgart, Baden-Wurttemberg, D-70176, Germany

Location

Universitätsklinikum Tübingen, Klinik für Kinderheilkunde und Jugendmedizin

Tübingen, Baden-Wurttemberg, D-72076, Germany

Location

Deutsches Herzzentrum München

Munich, Bavaria, D-80336, Germany

Location

Ludwig-Maximilian-Universität München, Klinikum Großhadern, Abt. Kinderkardiologie und Intensivmedizin

München-Großhadern, Bavaria, D-81377, Germany

Location

Universitätsklinikum Giessen and Marburg, Zentrum für Kinderheilkunde und Jugendmedizin

Giessen, Hesse, D-35385, Germany

Location

Medizinische Hochschule Hannover, Abt. für Kardiologie und Angiologie

Hanover, Lower Saxony, D-30625, Germany

Location

Medizinische Hochschule Hannover, Klinik für Kinderheilkunde, Pädiatrische Kardiologie und Pädiatrische Intensivmedizin

Hanover, Lower Saxony, D-30625, Germany

Location

Elisabeth Kinderkrankenhaus, Zentrum für Kinder- und Jugendmedizin

Oldenburg, Lower Saxony, D-26133, Germany

Location

Herz-und Diabeteszentrum NRW

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

Location

Evangelisches und Johanniter Klinikum Niederrhein gGmbH, Herzzentrum Duisburg

Duisburg, North Rhine-Westphalia, D-47137, Germany

Location

Universitätsklinikum Münster, EMAH-Zentrum

Münster, North Rhine-Westphalia, D-48149, Germany

Location

Universitätsklinikum des Saarlandes

Homburg/Saar, Saarland, D-66421, Germany

Location

Universitätsklinikum der Martin-Luther-Universität Halle-Wittenberg

Halle, Saxony-Anhalt, D-06120, Germany

Location

Universitätsklinikum Schleswig-Holstein Campus Kiel

Kiel, Schleswig-Holstein, D-24105, Germany

Location

Universitätsklinikum Charite, Campus Virchow-Klinikum, Otto-Heubner-Centrum für Kinder- und Jugendmedizin

Berlin, State of Berlin, D-13353, Germany

Location

Deutsches Herzzentrum Berlin, Abt. angeborener Herzfehler/Kinderkardiologie

Berlin, D-13353, Germany

Location

Universitätsklinikum Erlangen, Kinderkardiologische Abteilung, Kinder- und Jugendklinik

Erlangen, D-91054, Germany

Location

Related Links

• (Competence Network for Congenital Heart Defects)

MeSH Terms

Conditions

Pulmonary Arterial Hypertension; Hypertension; Eisenmenger Complex

Interventions

Sildenafil Citrate

Condition Hierarchy (Ancestors)

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

Intervention Hierarchy (Ancestors)

Sulfonamides; Amides; Organic Chemicals; Sulfones; Sulfur Compounds; Piperazines; Heterocyclic Compounds, 1-Ring; Heterocyclic Compounds; Purines; Heterocyclic Compounds, 2-Ring; Heterocyclic Compounds, Fused-Ring

Study Officials

• Siegrun Mebus, MD

  German Heart Institute Munich, Competence Network for Congenital Heart Defects

  PRINCIPAL INVESTIGATOR

• Ingram Schulze-Neick, MD

  Great Ormond Street Hospital for Sick Children,London

  STUDY CHAIR

Study Design

• Study Type: interventional
• Phase: phase 3
• Allocation: RANDOMIZED
• Masking: DOUBLE
• Who Masked: PARTICIPANT, INVESTIGATOR
• Purpose: TREATMENT
• Intervention Model: PARALLEL
• Sponsor Type: OTHER GOV

Study Record Dates

• First Submitted: December 21, 2007
• First Posted: January 4, 2008
• Study Start: December 1, 2007
• Primary Completion: May 1, 2012
• Study Completion: June 1, 2012
• Last Updated: June 6, 2012

Record last verified: 2010-10

Locations

DE (20)