Informed Consent
Item
signed informed consent prior to initiation of any study mandated procedure,
boolean
C0021430 (UMLS CUI [1])
Idiopathic pulmonary arterial hypertension Symptomatic | Familial pulmonary arterial hypertension | Pulmonary arterial hypertension associated with HIV infection | (Associated) (drug-induced) (toxin-induced) pulmonary arterial hypertension | New York Heart Association Classification
Item
patients with symptomatic idiopathic, or familial pulmonary arterial hypertension or pulmonary hypertension associated with human immunodeficiency virus (hiv) or drugs/toxins in nyha functional class ii to iv.
boolean
C3203102 (UMLS CUI [1,1])
C0231220 (UMLS CUI [1,2])
C1701939 (UMLS CUI [2])
C3697673 (UMLS CUI [3])
C4509209 (UMLS CUI [4])
C1275491 (UMLS CUI [5])
Childbearing Potential Urine pregnancy test negative | Childbearing Potential Contraceptive methods
Item
women of childbearing potential must have a negative urine pregnancy test and must use an adequate method of contraception during the study and for 28 days after discontinuation of the study drug.
boolean
C3831118 (UMLS CUI [1,1])
C0430057 (UMLS CUI [1,2])
C3831118 (UMLS CUI [2,1])
C0700589 (UMLS CUI [2,2])
Pulmonary arterial hypertension Relationship Condition Any | Exception Inclusion criteria
Item
pulmonary arterial hypertension related to any condition other than those specified in the inclusion criteria,
boolean
C2973725 (UMLS CUI [1,1])
C0439849 (UMLS CUI [1,2])
C0348080 (UMLS CUI [1,3])
C1552551 (UMLS CUI [1,4])
C1705847 (UMLS CUI [2,1])
C1512693 (UMLS CUI [2,2])
Pulmonary arterial hypertension Associated with Involvement Venous | Pulmonary arterial hypertension Associated with Involvement Capillary | Pulmonary Capillary Wedge Pressure
Item
pulmonary arterial hypertension associated with significant venous or capillary involvement (pulmonary capillary wedge pressure (pcwp) > 15 mmhg),
boolean
C2973725 (UMLS CUI [1,1])
C0332281 (UMLS CUI [1,2])
C1314939 (UMLS CUI [1,3])
C0348013 (UMLS CUI [1,4])
C2973725 (UMLS CUI [2,1])
C0332281 (UMLS CUI [2,2])
C1314939 (UMLS CUI [2,3])
C0006901 (UMLS CUI [2,4])
C0086879 (UMLS CUI [3])
Pulmonary Veno-Occlusive Disease | Pulmonary capillary hemangiomatosis
Item
known pulmonary veno-occlusive disease, or pulmonary capillary hemangiomatosis,
boolean
C0034091 (UMLS CUI [1])
C0340548 (UMLS CUI [2])
Obstructive Lung Disease Moderate | Obstructive Lung Disease Severe | FEV1/FVC percent post bronchodilator | Percent predicted FEV1 post bronchodilator
Item
moderate to severe obstructive lung disease: forced expiratory volume in 1 second/forced vital capacity (fev1/fvc) < 70% and fev1 < 65% 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])
Restrictive lung disease Moderate | Restrictive lung disease Severe | Percent Predicted Total Lung Capacity
Item
moderate to severe restrictive lung disease: total lung capacity (tlc) < 60% of predicted value,
boolean
C0085581 (UMLS CUI [1,1])
C0205081 (UMLS CUI [1,2])
C0085581 (UMLS CUI [2,1])
C0205082 (UMLS CUI [2,2])
C3828589 (UMLS CUI [3])
Pregnancy | Breast Feeding
Item
pregnant or breast-feeding women,
boolean
C0032961 (UMLS CUI [1])
C0006147 (UMLS CUI [2])
Systemic hypertension | Systolic Pressure Measurement Repeated | Diastolic blood pressure Measurement Repeated
Item
systemic hypertension (systolic blood pressure > 160 mmhg or diastolic blood pressure > 100 mmhg on repeated measurement),
boolean
C0020538 (UMLS CUI [1])
C0871470 (UMLS CUI [2,1])
C0242485 (UMLS CUI [2,2])
C0205341 (UMLS CUI [2,3])
C0428883 (UMLS CUI [3,1])
C0242485 (UMLS CUI [3,2])
C0205341 (UMLS CUI [3,3])
Systolic Pressure
Item
systolic blood pressure < 95 mmhg,
boolean
C0871470 (UMLS CUI [1])
Hepatic impairment Moderate Child-Pugh Classification | Hepatic impairment Severe Child-Pugh Classification
Item
moderate to severe hepatic impairment, i.e., child-pugh class b or c,
boolean
C0948807 (UMLS CUI [1,1])
C0205081 (UMLS CUI [1,2])
C4050412 (UMLS CUI [1,3])
C0948807 (UMLS CUI [2,1])
C0205082 (UMLS CUI [2,2])
C4050412 (UMLS CUI [2,3])
Chronic Kidney Insufficiency | Creatinine measurement, serum | Dialysis Continuous
Item
chronic renal insufficiency defined by serum creatinine > 2.5 mg/dl (221 μmol/l) or ongoing dialysis,
boolean
C0403447 (UMLS CUI [1])
C0201976 (UMLS CUI [2])
C0011946 (UMLS CUI [3,1])
C0549178 (UMLS CUI [3,2])
Blood Coagulation Disorder | Hemorrhage
Item
clinically relevant bleeding disorder or active bleeding,
boolean
C0005779 (UMLS CUI [1])
C0019080 (UMLS CUI [2])
Medical contraindication Bosentan | Medical contraindication Ambrisentan | Medical contraindication Sildenafil
Item
for those patients on monotherapy, any contraindication to bosentan, ambrisentan, or sildenafil, according to product label,
boolean
C1301624 (UMLS CUI [1,1])
C0252643 (UMLS CUI [1,2])
C1301624 (UMLS CUI [2,1])
C1176329 (UMLS CUI [2,2])
C1301624 (UMLS CUI [3,1])
C0529793 (UMLS CUI [3,2])
Hypersensitivity Iloprost | Hypersensitivity Iloprost Excipient
Item
known hypersensitivity to iloprost or any of its excipients.
boolean
C0020517 (UMLS CUI [1,1])
C0079594 (UMLS CUI [1,2])
C0020517 (UMLS CUI [2,1])
C0079594 (UMLS CUI [2,2])
C0015237 (UMLS CUI [2,3])