Extension Study of Efficacy and Safety of LTP001 in Pulmonary Arterial Hypertension Participants

Inclusion Criteria:

• Written informed consent must be obtained before any assessment is performed.

• Participant is currently completing the Novartis-sponsored study CLTP001A12201 inPAH and completed key efficacy and safety procedures up to the end of treatment ofthe core study, without meeting discontinuation criteria in the core study.

• Willingness and ability to comply with scheduled visits, treatment plans and anyother study procedures.

• In the opinion of the Investigator would benefit from LTP001 treatment.

Exclusion Criteria:

• History of hypersensitivity to the study treatment.

• Sexually active males not committing to condom use precautions: sexually activemales must use a condom during intercourse while taking drug and for 24 hours afterstopping study medication and should not father a child in this period nor donatesperm. A condom is required to be used also by vasectomized men in order to preventdelivery of the drug via seminal fluid.

• Required or planned transplant or heart/lung surgery.

• Women of child-bearing potential, defined as all women physiologically capable ofbecoming pregnant, unless they are using highly effective methods of contraceptionwhile taking study treatment and until EOT visit (2 weeks post-last treatment).Highly effective contraception methods include:

• Total abstinence (when this is in line with the preferred and usual lifestyle of theparticipant. Periodic abstinence (e.g. calendar, ovulation, symptothermal,post-ovulation methods) and withdrawal are not acceptable methods of contraception.

• Female sterilization (have had surgical bilateral oophorectomy with or withouthysterectomy), total hysterectomy, or bilateral tubal ligation at least six weeksbefore taking study treatment. In case of oophorectomy alone, only when thereproductive status of the woman has been confirmed by follow up hormone levelassessment.

• Male sterilization (at least 6 months prior to screening). For female participantson the study, the vasectomized male partner should be the sole partner for thatparticipant

• Use of oral, estrogen and progesterone, injected, or implanted hormonal methods ofcontraception or placement of an intrauterine device (IUD) or intrauterine system (IUS), or other forms of hormonal contraception that have comparable efficacy (failure rate < 1%), for example hormone vaginal ring or transdermal hormonecontraception.

In case of use of oral contraception women should have been stable on the same pill for a minimum of 3 months before taking study treatment.

Women are considered post-menopausal if they have had 12 months of natural (spontaneous) amenorrhea with an appropriate clinical profile (e.g. age appropriate history of vasomotor symptoms). Women are considered not of child-bearing potential if they are post-menopausal or have had surgical bilateral oophorectomy (with or without hysterectomy) or total hysterectomy at least six weeks ago. In the case of oophorectomy alone, only when the reproductive status of the woman has been confirmed by follow up hormone level assessment is she considered not of child bearing potential.

• Pregnant or nursing (lactating) women, where pregnancy is defined as the state of afemale after conception and until the termination of gestation, confirmed by apositive hCG laboratory test.

• Acute or chronic impairment (other than dyspnea), which would limit the ability tocomply with study requirements, including interference with physical activity orexecution of study procedures such as 6MWT (e.g., angina pectoris, claudication,musculoskeletal disorder, need for walking aids).

• Permanent discontinuation of Novartis drug in the core efficacy study due totoxicity or disease progression despite active treatment, non-compliance to studyprocedures, withdrawal of consent or any other reason.