Primary Outcome Measures:
- 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 [ Time Frame: 12 years ] [ Designated as safety issue: No ]
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.
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.