A Extension Study of Udenafil in Adolescents

Exclusion Criteria

• •1. Height \< 132 cm.
• •2. Weight \< 40 kg.
• •3. Hospitalization for acute decompensated heart failure within the last 12 months.
• •4. Current intravenous inotropic drugs.
• •5. Undergoing evaluation for heart transplantation or listed for transplantation.
• •6. Diagnosis of active protein losing enteropathy or plastic bronchitis within the last three years, or a history of liver cirrhosis.
• •7. Known Fontan baffle obstruction, branch pulmonary artery stenosis, or pulmonary vein stenosis resulting in a mean gradient of \> 4 mm Hg between the regions proximal and distal to the obstruction as measured by either catheterization or echocardiography.
• •8. Single lung physiology.
• •9. Maximal VO2 less than 50% of predicted for age and gender at enrollment.
• •10. Severe ventricular dysfunction assessed qualitatively by clinical echocardiography within six months prior to enrollment.
• •11. Severe valvar regurgitation, ventricular outflow obstruction, or aortic arch obstruction assessed by clinical echocardiography within six months prior to enrollment.
• •12. Significant renal, hepatic, gastrointestinal or biliary disorders that could impair absorption, metabolism or excretion of orally administered medications.
• •13. Inability to complete exercise testing at baseline screening.
• •14. History of PDE-5 inhibitor use (with the exception of FUEL participation) within 3 months before study onset.
• •15. Use of any other drug to treat pulmonary hypertension within 3 months before study onset.
• •16. Known intolerance to oral udenafil.
• •17. Frequent use of medications or other substances that inhibit or induce CYP3A4.
• •18. Current use of alpha-blockers or nitrates.
• •19. Ongoing or planned participation in another research protocol that would either prevent successful completion of planned study testing or invalidate its results.
• •20. Noncardiac medical, psychiatric, and/or social disorder that would prevent successful completion of planned study testing or would invalidate its results.
• •21. Cardiac care, ongoing or planned, at a non-study center that would impede study completion.
• •22. For females: Pregnancy at the time of screening, pregnancy planned before study completion, or refusal to use an acceptable method of contraception for study duration.
• •23. Unable to abstain or limit intake of grapefruit juice during the duration of the trial.
• •24. Refusal to provide written informed consent/assent.
• •25. In the opinion of the primary care physician, the subject is likely to be non-compliant with the study protocol.
• •26. History of clinically significant thromboembolic event, as adjudicated by study Investigators.