Pulmonary arterial hypertension | Symptoms WHO classification | Idiopathic pulmonary arterial hypertension | Familial pulmonary hypertension | Secondary to Connective Tissue Disease | Scleroderma, Limited | Diffuse Scleroderma | Mixed Connective Tissue Disease | Lupus Erythematosus, Systemic | Overlap syndrome | Persistent pulmonary hypertension | Surgical repair Atrial Septal Defects | Surgical repair Ventricular Septal Defects | Surgical repair Atrioventricular Septal Defect | Surgical repair Patent ductus arteriosus Persistent
Item
current diagnosis of pah (who group 1) with who class ii or iii symptoms in one of the following categories: idiopathic, heritable [familial], secondary to connective tissue disease (e.g., limited scleroderma, diffuse scleroderma, mixed connective tissue disease (ctd), systemic lupus erythematosus, or overlap syndrome), or persistent pah despite surgical repair (at least 6 months prior to the screening visit) of atrial septal defects, ventricular septal defects, atrio-ventricular septal defects, and persistent patent ductus.
boolean
C2973725 (UMLS CUI [1])
C1457887 (UMLS CUI [2,1])
C4267671 (UMLS CUI [2,2])
C3203102 (UMLS CUI [3])
C0340543 (UMLS CUI [4])
C0175668 (UMLS CUI [5,1])
C0009782 (UMLS CUI [5,2])
C0748540 (UMLS CUI [6])
C1258104 (UMLS CUI [7])
C0026272 (UMLS CUI [8])
C0024141 (UMLS CUI [9])
C0410000 (UMLS CUI [10])
C1135361 (UMLS CUI [11])
C0374711 (UMLS CUI [12,1])
C0018817 (UMLS CUI [12,2])
C0374711 (UMLS CUI [13,1])
C0018818 (UMLS CUI [13,2])
C0374711 (UMLS CUI [14,1])
C1389018 (UMLS CUI [14,2])
C0374711 (UMLS CUI [15,1])
C0013274 (UMLS CUI [15,2])
C0205322 (UMLS CUI [15,3])
Criteria Hemodynamic Fulfill | Catheterization of right heart | Mean pulmonary arterial pressure | Pulmonary Vascular Resistance | Left ventricular end-diastolic pressure measurement | Pulmonary Capillary Wedge Pressure
Item
have met the following hemodynamic criteria for subjects with right heart catheterization (rhc) when performed as part of the diagnosis or routine care: mean pulmonary arterial pressure (mpap) of >/=25 mmhg, pulmonary vascular resistance (pvr) of >/=240 dyne sec/cm5, left ventricular end diastolic pressure (levdp) or pulmonary capillary wedge pressure (pcwp) of ≤15 mmhg.
boolean
C0243161 (UMLS CUI [1,1])
C0019010 (UMLS CUI [1,2])
C1550543 (UMLS CUI [1,3])
C0189896 (UMLS CUI [2])
C3854605 (UMLS CUI [3])
C0456261 (UMLS CUI [4])
C1171395 (UMLS CUI [5])
C0086879 (UMLS CUI [6])
Therapy naive | Endothelin receptor antagonist Other Discontinued | Bosentan Discontinued | Etiology Increased liver function tests | Pharmacotherapy Stable Pulmonary arterial hypertension | Sildenafil Dose Stable | Epoprostenol Dose Stable
Item
be treatment naïve, have discontinued treatment with another era (e.g., bosentan) at least 1 month previously because of elevated liver function tests (lfts), or have been on a stable dose of drug therapy for pah (e.g., sildenafil or prostacyclin) for at least one month prior to the screening visit.
boolean
C0919936 (UMLS CUI [1])
C1134681 (UMLS CUI [2,1])
C0205394 (UMLS CUI [2,2])
C1444662 (UMLS CUI [2,3])
C0252643 (UMLS CUI [3,1])
C1444662 (UMLS CUI [3,2])
C0015127 (UMLS CUI [4,1])
C0877359 (UMLS CUI [4,2])
C0013216 (UMLS CUI [5,1])
C0205360 (UMLS CUI [5,2])
C2973725 (UMLS CUI [5,3])
C0529793 (UMLS CUI [6,1])
C0178602 (UMLS CUI [6,2])
C0205360 (UMLS CUI [6,3])
C0033567 (UMLS CUI [7,1])
C0178602 (UMLS CUI [7,2])
C0205360 (UMLS CUI [7,3])
Endothelin receptor antagonist Discontinued | Etiology Increased liver function tests | Liver Function Tests Result
Item
subjects who discontinued era treatment due to elevated lfts, must have lfts of <3 x upper limit of normal (uln).
boolean
C1134681 (UMLS CUI [1,1])
C1444662 (UMLS CUI [1,2])
C0015127 (UMLS CUI [2,1])
C0877359 (UMLS CUI [2,2])
C0023901 (UMLS CUI [3,1])
C1274040 (UMLS CUI [3,2])
Gender Childbearing Potential Absent | Childbearing Potential Pregnancy test negative | Childbearing Potential Breast Feeding Absent | Childbearing Potential Sexually active Contraceptive methods Quantity
Item
a female is eligible to participate in this study, as assessed by the investigator, if she is of: a. non-childbearing potential (i.e., physiologically incapable of becoming pregnant); or, b. child-bearing potential - has a negative pregnancy test and is not lactating at the screening and baseline/randomisation visits and, if sexually active, agrees to use 2 reliable methods of contraception from the screening visit until study completion and for at least 30 days following the last dose of study drug.
boolean
C0079399 (UMLS CUI [1,1])
C3831118 (UMLS CUI [1,2])
C0332197 (UMLS CUI [1,3])
C3831118 (UMLS CUI [2,1])
C0427780 (UMLS CUI [2,2])
C3831118 (UMLS CUI [3,1])
C0006147 (UMLS CUI [3,2])
C0332197 (UMLS CUI [3,3])
C3831118 (UMLS CUI [4,1])
C0241028 (UMLS CUI [4,2])
C0700589 (UMLS CUI [4,3])
C1265611 (UMLS CUI [4,4])
Informed Consent | Informed Consent Legal Guardian
Item
subject or subject's legal guardian is able and willing to give written informed consent. as part of the consent, female subjects of childbearing potential will be informed of the risk of teratogenicity and will need to be counselled in a developmentally appropriate manner on the importance of pregnancy prevention; and male subjects will need to be informed of potential risk of testicular tubular atrophy and aspermia.
boolean
C0021430 (UMLS CUI [1])
C0021430 (UMLS CUI [2,1])
C0023226 (UMLS CUI [2,2])
Endothelin receptor antagonist
Item
currently taking an era.
boolean
C1134681 (UMLS CUI [1])
Cyclosporine
Item
currently taking cyclosporine a.
boolean
C0010592 (UMLS CUI [1])
Body Weight
Item
body weight is less than 20 kg.
boolean
C0005910 (UMLS CUI [1])
Intolerance Therapy Pulmonary arterial hypertension | Etiology Adverse effects | Prostaglandins | Endothelin receptor antagonist | Phosphodiesterase 5 inhibitor | Exception Abnormality of the liver
Item
have not tolerated pah therapy due to adverse effects which may be related to their mechanism of action (e.g., prostanoids, era, pde-5 inhibitors) with the exception of liver abnormalities for those subjects who were receiving another era.
boolean
C0231199 (UMLS CUI [1,1])
C0087111 (UMLS CUI [1,2])
C2973725 (UMLS CUI [1,3])
C0015127 (UMLS CUI [2,1])
C0879626 (UMLS CUI [2,2])
C0033554 (UMLS CUI [3])
C1134681 (UMLS CUI [4])
C1318700 (UMLS CUI [5])
C1705847 (UMLS CUI [6,1])
C4021780 (UMLS CUI [6,2])
Pregnancy | Breast Feeding
Item
pregnant or breastfeeding.
boolean
C0032961 (UMLS CUI [1])
C0006147 (UMLS CUI [2])
Hepatitis | Hepatitis B Surface Antigens | Hepatitis C Antibodies | Elevated liver enzymes | Alanine aminotransferase increased | Aspartate aminotransferase increased | Alkaline phosphatase raised
Item
diagnosis of active hepatitis (hepatitis b surface antigen and hepatitis c antibody), or clinically significant hepatic enzyme elevation (i.e., alt, ast or ap >3xuln) at screening.
boolean
C0019158 (UMLS CUI [1])
C0019168 (UMLS CUI [2])
C0166049 (UMLS CUI [3])
C0235996 (UMLS CUI [4])
C0151905 (UMLS CUI [5])
C0151904 (UMLS CUI [6])
C0151849 (UMLS CUI [7])
Renal Insufficiency Severe | Creatinine clearance measurement
Item
severe renal impairment (creatinine clearance <30 ml/min) at screening.
boolean
C1565489 (UMLS CUI [1,1])
C0205082 (UMLS CUI [1,2])
C0373595 (UMLS CUI [2])
Body fluid retention
Item
clinically significant fluid retention in the opinion of the investigator.
boolean
C0268000 (UMLS CUI [1])
Anemia
Item
clinically significant anaemia in the opinion of the investigator.
boolean
C0002871 (UMLS CUI [1])
Hypersensitivity Investigational New Drugs | Hypersensitivity Metabolite | Hypersensitivity Formulation Excipient
Item
a known hypersensitivity to the study drug, the metabolites, or formulation excipients.
boolean
C0020517 (UMLS CUI [1,1])
C0013230 (UMLS CUI [1,2])
C0020517 (UMLS CUI [2,1])
C0870883 (UMLS CUI [2,2])
C0020517 (UMLS CUI [3,1])
C0524527 (UMLS CUI [3,2])
C0015237 (UMLS CUI [3,3])
Study Subject Participation Status | Clinical Trial | Investigational New Drugs
Item
have participated in another trial or have taken another investigational product during the previous 30 days.
boolean
C2348568 (UMLS CUI [1])
C0008976 (UMLS CUI [2])
C0013230 (UMLS CUI [3])
Alcohol abuse | Illicit medication use
Item
alcohol abuse, illicit drug use within 1 year.
boolean
C0085762 (UMLS CUI [1])
C0281875 (UMLS CUI [2])
Condition Affecting Patient safety | Pharmaceutical Preparations Affecting Patient safety
Item
any concurrent condition or concurrent use of medication that would affect subject safety in the opinion of the investigator.
boolean
C0348080 (UMLS CUI [1,1])
C0392760 (UMLS CUI [1,2])
C1113679 (UMLS CUI [1,3])
C0013227 (UMLS CUI [2,1])
C0392760 (UMLS CUI [2,2])
C1113679 (UMLS CUI [2,3])