An Extension Study for Patients Who Participated in the CAMN107X2201 Study

Inclusion Criteria:

1. Written informed consent must be obtained from the patient or legal guardian, before
any assessment is performed.

2. Patients who participated in CAMN107X2201 clinical trial and completed the study,
including all end-of-study (Study Completion) assessments of the Week 48 (Day 336) in
Cohort 1 and the Week 24 visit (Day 168) in Cohort 2 of the study protocol.

Exclusion Criteria:

Patients fulfilling any of the following criteria are not eligible for inclusion in this
study:

1. Women of child-bearing potential, defined as all women physiologically capable of
becoming pregnant UNLESS they are using two birth control methods. The two methods
can be a double barrier method or a barrier method plus a hormonal method. Adequate
barrier methods of contraception include: diaphragm, condom (by the partner),
intrauterine device (copper or hormonal), or sponge with spermicide. Hormonal
contraceptives include any marketed contraceptive agent that includes an estrogen
and/or a progestational agent

2. Pregnant or nursing (lactating) women, where pregnancy is defined as the state of a
female after conception and until the termination of gestation, confirmed by a
positive hCG laboratory test (> 5 mIU/mL)

3. Patients with a pulmonary capillary wedge pressure > 15 mm Hg at time of Study
Completion Assessments in CAMN107X2201. If pulmonary capillary wedge pressure is not
attainable, then a left atrial pressure measurement may be used in its place.

4. Patients with LVEF < 45%

5. Patients with thrombocytopenia, platelet count < 50 x109/L (50 x 103/µL)

6. Patients with uncontrolled systemic arterial hypertension, systolic > 160 mm Hg or
diastolic >90 mm Hg

7. Patients with a QTcF > 450 ms for males and > 470 ms for females in the absence of
right bundle branch block (based on Visit 1 ECG if required to be performed)

8. [In Canada Only: Liver function tests ALT or AST > 1.5 times ULN]

Other protocol-defined inclusion/exclusion criteria may apply.