Eisenmenger's Syndrome in Adults With CHD

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

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.

Study Overview

• Status: Completed
• Conditions: Congenital Disorders

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.

• Study Type: Observational
• Enrollment (Actual): 48

Contacts and Locations

Study Locations

• United States
  • Georgia
    • Atlanta, Georgia, United States, 30322
      • Children's Healthcare of Atlanta

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (ADULT, OLDER_ADULT)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

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

Description

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

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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	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.	12 years

Collaborators and Investigators

• Sponsor: Children's Healthcare of Atlanta
• Investigators: Principal Investigator: Wendy M. Book, MD, Emory University SOM

Study record dates

Study Major Dates

• Study Start: January 1, 1996
• Primary Completion (Actual): January 1, 2008
• Study Completion (Actual): January 1, 2008

Study Registration Dates

• First Submitted: December 19, 2005
• First Submitted That Met QC Criteria: December 19, 2005
• First Posted (Estimate): December 20, 2005

Study Record Updates

• Last Update Posted (Estimate): December 2, 2014
• Last Update Submitted That Met QC Criteria: November 26, 2014
• Last Verified: November 1, 2014

More Information

Terms related to this study

• Keywords: adult; Bosentan; Sildenafil; congenital heart disease; Eisenmenger's Syndrome; Sitaxsetan
• Additional Relevant MeSH Terms: Heart Diseases; Cardiovascular Diseases; Congenital Abnormalities; Heart Defects, Congenital; Cardiovascular Abnormalities; Eisenmenger Complex
• Other Study ID Numbers: 05-118