Eisenmenger's Syndrome in Adults With CHD

Treatment of Eisenmenger's Syndrome in Adults With Congenital Heart Disease With Pulmonary Arterial Vasodilators

Status

Completed

Phases

Unknown

Study type

Observational

Source

ClinicalTrials.gov

Registry ID

NCT00266903

Enrollment

Registered

2005-12-20

Start date

1996-01-31

Completion date

2008-01-31

Last updated

2014-12-02

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Congenital Disorders

Keywords

adult, congenital heart disease, Eisenmenger's Syndrome, Bosentan, Sitaxsetan, Sildenafil

Brief summary

Review the outcome of treatment with endothelin receptor antagonists (bosentan or sitaxsetan) alone or in combination with Sildenafil (a PDE-5 inhibitor) in adult patients with pulmonary hypertension due to congenital heart disease.

Detailed description

The primary objective of this study is to examine medical records of patients with Eisenmenger's Syndrome in order to determine the effect of endothelin receptor antagonists, alone or in combination, with Sildenafil, on symptoms of pulmonary hypertension. This review will be conducted on Standard of Care procedures such as New York Heart Association (NYHA) classification, six-minute walk exercise capacity, pulmonary pressures and oxygens saturations. Pulmonary pressures determined by echo or cardiac catheterization will be analyzed where available. We hypothesize that patients with congenital heart defects who develop ES will have an improved six-minute walk distance, improved pulmonary arterial pressures and improved oxygenation after treatment with endothelin receptor antagonists and other pulmonary vasodilators.

Interventions

None listed

Study design

Time perspective

RETROSPECTIVE

Eligibility

Sex/Gender

ALL

Age

18 Years to No maximum

Healthy volunteers

Inclusion criteria

* Congenital heart disease (unoperated, palliated or repaired) Eisenmenger's syndrome Age 18 to 80

Exclusion criteria

* Those who do not meet inclusion criteria

Design outcomes

Primary

Measure	Time frame	Description
Review the outcome of treatment with endothelin receptor antagonists (bosentan or sitaxsetan) alone or in combination with Sildenafil (a PDE-5 inhibitor) in adult patients with pulmonary hypertension due to congenital heart disease	12 years	Our study demonstrates that endothelin receptor blockers can be used to improve exercise tolerance, oxygenation, and functional status in adults with Eisenmenger syndrome without adverse effects. The role of newer treatment modalities such as prostacyclins, PDE inhibitors, and nitric oxide remain promising and their role needs to be investigated to help improve quality of life and life expectancy in these patients.

Countries

United States

Outcome results