Patient description: A 62-year-old African-American man presented with left upper and lower extremity weakness, associated with dark visual spot in right eye, right facial numbness, facial drop and slurred speech. He denied dyspnea, headache, palpitations, chest pain, fever, dizziness, bowel or urinary incontinence, loss of consciousness. His medical history was significant for hypertension, hyperlipidemia and hypothyroidism. He smokes cigarette 1 pack per day for 40 years and alcohol consumption of 5 to 6 beers per week. He is not aware about his family history. He is using Levothyroxine, Atorvastatin and HTCZ. His vital signs were stable in the primary evaluation. Left-sided facial droop, dysarthria, and left-sided hemiplegia were seen in the physical exam. His National Institutes of Health Stroke Scale (NIHSS) score was calculated as 7. Initial CT angiogram of head and neck reported no acute intracranial findings. Intravenous recombinant tissue plasminogen activator (t-PA) was administered as well as high-dose statin therapy. The patient was admitted to the intensive care unit to be monitored for 24 hours. MRI of the head revealed an acute 1.7-cm infarct of the right periventricular white matter and posterior right basal ganglia.

Clinical trial eligibility criteria: eligible ages (years): 18.0-75.0,  Connective Tissue Disease-Associated Pulmonary Arterial Hypertension BMI > 18.5 kg/m2 Symptomatic pulmonary hypertension WHO/NYHA FC class II and III WHO Group I PAH associated with connective tissue disease Had a diagnostic right heart catheterization performed and documented within 36 months prior to Day 1 that confirmed a diagnosis of PAH according to all the following Mean pulmonary artery pressure ≥ 25 mm Hg (at rest) Pulmonary capillary wedge pressure (PCWP) ≤ 15 mm Hg Pulmonary vascular resistance > 240 dyn.sec/cm5 or > 3 mm Hg/liter (L)/minute Has BNP level ≤ 400 pg/mL Had an average 6MWD ≥ 150 meters on two consecutive tests performed on different days prior to randomization, with both tests measuring within 15% of one another Has been receiving no more than two (2) approved disease-specific PAH therapies. PAH therapy must have been at a stable dose for at least 90 days prior to Day 1. No additions or changes should be made to PAH therapies and doses should remain stable for the duration of the study  Participation in other investigational clinical studies involving interventional products being tested or used in a way different from the approved form or when used for an unapproved indication within 30 days prior to Day 1 Initiation of an exercise program for cardio-pulmonary rehabilitation within 90 days prior to Day 1 or planned initiation during the study Stopped receiving any PAH chronic therapy within 60 days prior to Day 1 Received a dose of prednisone > 20 mg/day (or equivalent dose if other corticosteroid) within 30 days prior to Day 1 Received intravenous (iv) or subcutaneous (sc) prostacyclin/prostacyclin analogues within 90 days prior to Day 1 Received intravenous inotropes within 30 days prior to Day 1 Has uncontrolled systemic hypertension as evidenced by sitting systolic blood pressure (BP) > 160 mm Hg or sitting diastolic BP > 100 mm Hg during Screening after a period of rest Has systolic BP < 90 mm Hg during Screening after a period of rest Has a history of clinically significant left-sided heart disease and/or clinically significant cardiac disease, including but not limited to any of the following Congenital or acquired valvular disease if clinically significant apart from tricuspid valvular insufficiency due to pulmonary hypertension