Informed consent
Item
1. signed informed consent prior to initiation of any study mandated procedure
boolean
C0021430 (UMLS CUI [1])
Pulmonary arterial hypertension symptomatic | WHO functional class
Item
2. patients with symptomatic pah in world health organization (who) functional class (fc) ii to iv
boolean
C2973725 (UMLS CUI [1,1])
C0231220 (UMLS CUI [1,2])
C0043237 (UMLS CUI [2,1])
C0456387 (UMLS CUI [2,2])
C0205245 (UMLS CUI [2,3])
Pulmonary arterial hypertension classification | idiopathic pah | familial pah | drug-induced pah | toxin-induced pah | pah associated with connective tissue disease, congenital heart disease or HIV infection
Item
3. patients with pah belonging to one of the following subgroups of the dana point clinical classification group 1: a. idiopathic, or b. heritable, or c. drug or toxin induced, or d. associated with one of the following: i. connective tissue disease ii. congenital heart disease with simple systemic-to-pulmonary shunt at least one year after surgical repair iii. hiv infection
boolean
C0020542 (UMLS CUI [1,1])
C0008902 (UMLS CUI [1,2])
C3203102 (UMLS CUI [2])
C0340543 (UMLS CUI [3])
C0340544 (UMLS CUI [4])
C3697954 (UMLS CUI [5])
C3697982 (UMLS CUI [6])
C3697119 (UMLS CUI [7])
C3697673 (UMLS CUI [8])
PAH right heart catheterization | Mean pulmonary arterial pressure at rest | Pulmonary vascular resistance at rest | Pulmonary capillary wedge pressure | left ventricular end-diastolic pressure level
Item
4. documented hemodynamic diagnosis of pah by right heart catheterization - performed at any time prior to screening showing: a. resting mean pulmonary arterial pressure (mpap) ≥ 25 mmhg and b. resting pulmonary vascular resistance (pvr) > 240 dyn•s•cm-5 and c. pulmonary capillary wedge pressure (pcwp) or left ventricular end diastolic pressure (lvedp) ≤ 15 mmhg
boolean
C2973725 (UMLS CUI [1,1])
C0189896 (UMLS CUI [1,2])
C3854605 (UMLS CUI [2,1])
C0443144 (UMLS CUI [2,2])
C0456261 (UMLS CUI [3,1])
C0443144 (UMLS CUI [3,2])
C0086879 (UMLS CUI [4])
C0456190 (UMLS CUI [5])
6-minute walk test distance
Item
5. 6-minute walk distance (6mwd) ≥ 150 m at screening
boolean
C0430515 (UMLS CUI [1,1])
C0012751 (UMLS CUI [1,2])
Able to speak and read English
Item
6. able to fluently speak and read english
boolean
C0564215 (UMLS CUI [1,1])
C0376245 (UMLS CUI [1,2])
C0586740 (UMLS CUI [2,1])
C0376245 (UMLS CUI [2,2])
Phosphodiesterase type-5 inhibitors (pde5i), inhaled prostacyclin analogues, or calcium channel blockers stable medication dose duration
Item
7. for patients on phosphodiesterase type-5 inhibitors (pde5i), inhaled prostacyclin analogues, or calcium channel blockers, stable doses for at least 3 months prior to visit 2
boolean
C1318700 (UMLS CUI [1,1])
C3174092 (UMLS CUI [1,2])
C0205360 (UMLS CUI [1,3])
C0449238 (UMLS CUI [1,4])
C0205911 (UMLS CUI [2,1])
C0243071 (UMLS CUI [2,2])
C0205535 (UMLS CUI [2,3])
C3174092 (UMLS CUI [2,4])
C0205360 (UMLS CUI [2,5])
C0449238 (UMLS CUI [2,6])
C0006684 (UMLS CUI [3,1])
C3174092 (UMLS CUI [3,2])
C0205360 (UMLS CUI [3,3])
C0449238 (UMLS CUI [3,4])
C0449238 (UMLS CUI [3,5])
Stable medication dose of oral diuretics duration
Item
8. for patients on oral diuretics, stable doses for at least 4 weeks prior to visit 2
boolean
C0012798 (UMLS CUI [1,1])
C0442027 (UMLS CUI [1,2])
C3174092 (UMLS CUI [1,3])
C0205360 (UMLS CUI [1,4])
C0449238 (UMLS CUI [1,5])
Age
Item
9. men or women aged 18 or older
boolean
C0001779 (UMLS CUI [1])
Pre-pubertal | uterus not present | postmenopausal state | no period | status post ovariectomy | negative serum pregnancy test | negative urine pregnancy test | willingness urine pregnancy tests monthly | willingness contraceptive methods during clinical trial period | progesterone implant | IUD | tubal sterilization
Item
a. a woman is considered to be of childbearing potential unless she: has not yet entered puberty, or does not have a uterus, or has gone through menopause (has not had a period for at least 12 months for natural reasons, or who has had their ovaries removed) b. a women of childbearing potential is eligible only if she meets both criteria below: has a negative serum pregnancy test at screening and a negative urine pregnancy test at baseline and agree to perform monthly urine pregnancy tests, and agrees to use two methods of contraception (one method for patients with a progesterone implant or an intrauterine device or tubal sterilization) from the screening visit 1 until one month after study drug discontinuation
boolean
C1628325 (UMLS CUI [1])
C0042149 (UMLS CUI [2,1])
C0332197 (UMLS CUI [2,2])
C0232970 (UMLS CUI [3])
C0002453 (UMLS CUI [4])
C0231290 (UMLS CUI [5,1])
C0029936 (UMLS CUI [5,2])
C0430061 (UMLS CUI [6])
C0430057 (UMLS CUI [7])
C0600109 (UMLS CUI [8,1])
C0430056 (UMLS CUI [8,2])
C0332177 (UMLS CUI [8,3])
C0600109 (UMLS CUI [9,1])
C0700589 (UMLS CUI [9,2])
C0347984 (UMLS CUI [9,3])
C2347804 (UMLS CUI [9,4])
C0021102 (UMLS CUI [10,1])
C0033308 (UMLS CUI [10,2])
C0021900 (UMLS CUI [11])
C0038289 (UMLS CUI [12])
Moderate to severe obstructive lung disease: FEV1 / FVC percent post bronchodilator | FEV1 predicted percent post bronchodilator
Item
1. moderate to severe obstructive lung disease: forced expiratory volume in one second (fev1) / forced vital capacity < 70 percent and fev1 < 65 percent of predicted value after bronchodilator administration
boolean
C0600260 (UMLS CUI [1,1])
C0205081 (UMLS CUI [1,2])
C0600260 (UMLS CUI [2,1])
C0205082 (UMLS CUI [2,2])
C0730560 (UMLS CUI [3,1])
C2599594 (UMLS CUI [3,2])
C0730561 (UMLS CUI [4,1])
C2599594 (UMLS CUI [4,2])
Moderate to severe restrictive lung disease | percent predicted total lung capacity
Item
2. moderate to severe restrictive lung disease: total lung capacity < 60 percent of predicted value
boolean
C0085581 (UMLS CUI [1,1])
C0205081 (UMLS CUI [1,2])
C0205082 (UMLS CUI [2,1])
C0085581 (UMLS CUI [2,2])
C3828589 (UMLS CUI [3])
Hemoglobin
Item
3. hemoglobin < 75 percent of the lower limit of the normal range at screening
boolean
C0019046 (UMLS CUI [1])
Aspartate aminotransferase measurement | alanine aminotransferase measurement
Item
4. serum aspartate aminotransferase (ast) and/or alanine aminotransferase (alt) > 3 times the upper limit of normal (uln) at screening
boolean
C0201899 (UMLS CUI [1])
C0201836 (UMLS CUI [2])
Estimated creatinine clearance
Item
5. estimated creatinine clearance < 30 ml/min at screening
boolean
C1273051 (UMLS CUI [1])
Systolic blood pressure
Item
6. systolic blood pressure (sbp) < 90 mmhg at screening
boolean
C0871470 (UMLS CUI [1])
Body weight
Item
7. body weight < 40 kg at screening
boolean
C0005910 (UMLS CUI [1])
Comorbidity life threatening | life expectancy
Item
8. known concomitant life-threatening diseases with a life expectancy of < 12 months
boolean
C0009488 (UMLS CUI [1,1])
C2826244 (UMLS CUI [1,2])
C0023671 (UMLS CUI [2])
Disease preventing protocol compliance or adherence to therapy
Item
9. any condition that prevents compliance with the protocol or adherence to therapy
boolean
C0012634 (UMLS CUI [1,1])
C1292733 (UMLS CUI [1,2])
C0525058 (UMLS CUI [1,3])
C0012634 (UMLS CUI [2,1])
C1292733 (UMLS CUI [2,2])
C1510802 (UMLS CUI [2,3])
Endothelin receptor antagonists | exception macitentan
Item
10. treatment with endothelin receptor antagonists (eras) within 3 months prior to visit 2, or scheduled to receive any of these compounds, other than macitentan, during the trial
boolean
C1134681 (UMLS CUI [1])
C1705847 (UMLS CUI [2,1])
C2606556 (UMLS CUI [2,2])
Intravenous or subcutaneous prostacyclin or prostacyclin analogs recent or planned
Item
11. treatment with intravenous or subcutaneous prostacyclin or prostacyclin analogs within 3 months prior to visit 2, or scheduled to receive any of these compounds during the trial
boolean
C0205911 (UMLS CUI [1,1])
C1522726 (UMLS CUI [1,2])
C0332185 (UMLS CUI [1,3])
C0205911 (UMLS CUI [2,1])
C1522726 (UMLS CUI [2,2])
C1301732 (UMLS CUI [2,3])
C0205911 (UMLS CUI [3,1])
C0243071 (UMLS CUI [3,2])
C1522726 (UMLS CUI [3,3])
C0332185 (UMLS CUI [3,4])
C0205911 (UMLS CUI [4,1])
C0243071 (UMLS CUI [4,2])
C1522726 (UMLS CUI [4,3])
C1301732 (UMLS CUI [4,4])
C0205911 (UMLS CUI [5,1])
C1522438 (UMLS CUI [5,2])
C0332185 (UMLS CUI [5,3])
C0205911 (UMLS CUI [6,1])
C1522438 (UMLS CUI [6,2])
C1301732 (UMLS CUI [6,3])
C0205911 (UMLS CUI [7,1])
C0243071 (UMLS CUI [7,2])
C1522438 (UMLS CUI [7,3])
C0332185 (UMLS CUI [7,4])
C0205911 (UMLS CUI [8,1])
C0243071 (UMLS CUI [8,2])
C1522438 (UMLS CUI [8,3])
C1301732 (UMLS CUI [8,4])
Riociguat recent or planned
Item
12. treatment with riociguat within 3 months prior to visit 2, or scheduled to receive riociguat during the trial
boolean
C2717561 (UMLS CUI [1,1])
C0332185 (UMLS CUI [1,2])
C2717561 (UMLS CUI [2,1])
C1301732 (UMLS CUI [2,2])
Strong cytochrome p450 (cyp) 3a4 inducers or inhibitors recent
Item
13. treatment with strong cytochrome p450 (cyp) 3a4 inducers or inhibitors within 4 weeks prior to visit 2
boolean
C3850041 (UMLS CUI [1,1])
C0442821 (UMLS CUI [1,2])
C0332185 (UMLS CUI [1,3])
C3850053 (UMLS CUI [2,1])
C0442821 (UMLS CUI [2,2])
C0332185 (UMLS CUI [2,3])
Cardio-pulmonary rehabilitation program based on exercise recently started or planned
Item
14. recently started (< 8 weeks prior to visit 2) or planned cardio-pulmonary rehabilitation program based on exercise
boolean
C0332185 (UMLS CUI [1,1])
C0439659 (UMLS CUI [1,2])
C0700431 (UMLS CUI [1,3])
C0015259 (UMLS CUI [1,4])
C0700431 (UMLS CUI [2,1])
C0015259 (UMLS CUI [2,2])
C1301732 (UMLS CUI [2,3])
C0332185 (UMLS CUI [3,1])
C0439659 (UMLS CUI [3,2])
C0199529 (UMLS CUI [3,3])
C0015259 (UMLS CUI [3,4])
C0199529 (UMLS CUI [4,1])
C0015259 (UMLS CUI [4,2])
C1301732 (UMLS CUI [4,3])
Lactating | pregnancy | pregnancy test positive | pregnancy intention
Item
15. females who are lactating or pregnant (positive screening or baseline pregnancy test) or plan to become pregnant during the study
boolean
C0006147 (UMLS CUI [1])
C0032961 (UMLS CUI [2])
C0240802 (UMLS CUI [3])
C0032961 (UMLS CUI [4,1])
C0162425 (UMLS CUI [4,2])
Hypersensitivity macitentan | macitentan excipients | endothelin receptor antagonist
Item
16. known hypersensitivity to macitentan or its excipients or drugs of the same class
boolean
C0020517 (UMLS CUI [1,1])
C2606556 (UMLS CUI [1,2])
C0020517 (UMLS CUI [2,1])
C0015237 (UMLS CUI [2,2])
C2606556 (UMLS CUI [2,3])
C0020517 (UMLS CUI [3,1])
C1134681 (UMLS CUI [3,2])
Investigational new drugs recent
Item
17. treatment with another investigational drug within 3 months prior to visit 2
boolean
C0013230 (UMLS CUI [1,1])
C0332185 (UMLS CUI [1,2])
Factor or disease interferes with treatment compliance, completion of clinical trial or interpretation of research results | drug or alcohol dependence | mental disorders
Item
18. any known factor or disease that might interfere with treatment compliance, study conduct or interpretation of the results such as drug or alcohol dependence or psychiatric disease
boolean
C1521761 (UMLS CUI [1,1])
C0521102 (UMLS CUI [1,2])
C4319828 (UMLS CUI [1,3])
C1521761 (UMLS CUI [2,1])
C0521102 (UMLS CUI [2,2])
C2732579 (UMLS CUI [2,3])
C1521761 (UMLS CUI [3,1])
C0521102 (UMLS CUI [3,2])
C3526596 (UMLS CUI [3,3])
C0012634 (UMLS CUI [4,1])
C0521102 (UMLS CUI [4,2])
C4319828 (UMLS CUI [4,3])
C0012634 (UMLS CUI [5,1])
C0521102 (UMLS CUI [5,2])
C2732579 (UMLS CUI [5,3])
C0012634 (UMLS CUI [6,1])
C0521102 (UMLS CUI [6,2])
C3526596 (UMLS CUI [6,3])
C0038580 (UMLS CUI [7])
C0004936 (UMLS CUI [8])