Clinical Study Assessing the Efficacy and Safety of Macitentan in Fontan-palliated Subjects

Prospective, Multi-center, Double-blind, Randomized, Placebo-controlled, Parallel-group Study Assessing the Efficacy and Safety of Macitentan in Fontan-palliated Adult and Adolescent Subjects

The primary objective is to assess the effect of macitentan 10 mg as compared to placebo on exercise capacity through cardiopulmonary exercise testing.

Inclusion Criteria:

• Written informed consent/assent from the subject and/or a legal representative prior to initiation of any study-mandated procedures
• Fontan-palliated subjects with either intra-atrial lateral tunnel total cavopulmonary connection (LT-TCPC), or extra cardiac tunnel TCPC (EC-TCPC) surgery > 1 year before Screening. Either LT- or EC-TCPC can be primary or secondary to atrio-pulmonary connection
• New York Heart Association (NYHA) functional class (FC) II or III (assessed by the investigator using the Specific Activity Scale
• Women of childbearing potential must have a negative serum pregnancy test use reliable contraception

Exclusion Criteria:

• Pattern of Fontan circulation severity
• Deterioration of the Fontan-palliated condition.
• Limitations to Cardiopulmonary exercise testing (CPET)
• Peak VO2 < 15 mL/kg/min.
• Any known factor or disease that may interfere with treatment compliance or full participation in the study