Main Inclusion Criteria

• Males or females ? 12 years old
• PAH due to PPH or secondary to sclerodermaor other connective tissue diseases
• WHO functional class III - IV
• Baseline 6-min walk test ? 150 m and ? 450/500 m
• Baseline hemodynamics
  • Mean PAP > 25 mmHg
  • PVR > 240 dyn•sec/cm5
  • PCWP < 15 mmHg

9017.01

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