Informed consent
Item
1. voluntarily gives informed consent to participate in the study.
boolean
C0021430 (UMLS CUI [1])
Catheterization of right heart | Mean pulmonary arterial pressure | Pulmonary Vascular Resistance | Pulmonary Capillary Wedge Pressure
Item
2. right heart catheterization demonstrating conventional mean pulmonary artery pressure (mpap) >25, pulmonary vascular resistance (pvr) >3.0 wood units, pulmonary capillary wedge pressure (pcwp) <16 mmhg within one year of enrollment
boolean
C0189896 (UMLS CUI [1])
C3854605 (UMLS CUI [2])
C0456261 (UMLS CUI [3])
C0086879 (UMLS CUI [4])
Age
Item
3. subject is 16-75 years of age at screening.
boolean
C0001779 (UMLS CUI [1])
Idiopathic pulmonary arterial hypertension Symptomatic | Familial primary pulmonary hypertension | Pulmonary arterial hypertension associated with connective tissue disease | Pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunt | Pulmonary arterial hypertension associated with portal hypertension | Pulmonary arterial hypertension associated with HIV infection
Item
4. diagnosis of symptomatic idiopathic or heritable pah, pah associated with connective tissue disease (ctd), pah associated with repaired/unrepaired congenital systemic-to-pulmonary shunt, portopulmonary hypertension or pah associated with hiv infection.
boolean
C3203102 (UMLS CUI [1,1])
C0231220 (UMLS CUI [1,2])
C0340543 (UMLS CUI [2])
C3697982 (UMLS CUI [3])
C3698160 (UMLS CUI [4])
C1868851 (UMLS CUI [5])
C3697673 (UMLS CUI [6])
New York Heart Association Classification
Item
5. new york heart association functional class ii or iii
boolean
C1275491 (UMLS CUI [1])
ambrisentan | Therapeutic procedure Stable
Item
6. stable therapy with ambrisentan 5 or 10 mg every day for > 90 days.
boolean
C1176329 (UMLS CUI [1])
C0087111 (UMLS CUI [2,1])
C0205360 (UMLS CUI [2,2])
6 Minute Walk Functional Test Walking distance
Item
7. baseline 6-minute walk distance 50-450m
boolean
C3900196 (UMLS CUI [1,1])
C0429886 (UMLS CUI [1,2])
Lung disease Primary
Item
1. substantial primary lung disease
boolean
C0024115 (UMLS CUI [1,1])
C0205225 (UMLS CUI [1,2])
FEV1 | Forced vital capacity | Percent predicted FEV1
Item
forced expiratory volume at one second (fev-1)/forced vital capacity (fvc) <0.6 and fev-1 <70% predicted
boolean
C0748133 (UMLS CUI [1])
C1287681 (UMLS CUI [2])
C0730561 (UMLS CUI [3])
Carbon Monoxide Diffusing Capability Test
Item
diffusing capacity of lung for carbon monoxide (dlco) <30% predicted
boolean
C1516251 (UMLS CUI [1])
Pulmonary Fibrosis
Item
pulmonary fibrosis
boolean
C0034069 (UMLS CUI [1])
Left ventricular ejection fraction
Item
2. left ventricular ejection fraction < 50%
boolean
C0428772 (UMLS CUI [1])
Pulmonary Capillary Wedge Pressure
Item
3. pulmonary capillary wedge pressure > 16 mm hg
boolean
C0086879 (UMLS CUI [1])
Aortic valve disorder
Item
4. aortic valve disease
boolean
C1260873 (UMLS CUI [1])
Myocardial Ischemia
Item
5. ischemic heart disease
boolean
C0151744 (UMLS CUI [1])
Hypotension Systemic | Systolic Pressure
Item
6. systemic hypotension (sbp <90 mm hg)
boolean
C0020649 (UMLS CUI [1,1])
C0205373 (UMLS CUI [1,2])
C0871470 (UMLS CUI [2])
Concomitant Therapy | Endothelin receptor antagonist | Prostacyclin Analogue
Item
7. co-existing treatment with other endothelin receptor antagonists or prostacyclin analogues
boolean
C1707479 (UMLS CUI [1])
C1134681 (UMLS CUI [2])
C0033567 (UMLS CUI [3,1])
C0243071 (UMLS CUI [3,2])
New York Heart Association Classification
Item
8. new york heart association functional class iv
boolean
C1275491 (UMLS CUI [1])
Chronic thromboembolic pulmonary hypertension
Item
9. chronic thromboembolic pulmonary hypertension
boolean
C2363973 (UMLS CUI [1])
Pulmonary Veno-Occlusive Disease
Item
10. known or suspected pulmonary veno-occlusive disease
boolean
C0034091 (UMLS CUI [1])
Creatinine measurement, serum | Gender
Item
11. serum creatinine >2.0 mg/dl in women, serum creatinine >2.5 mg/dl in men
boolean
C0201976 (UMLS CUI [1])
C0079399 (UMLS CUI [2])
Serum potassium measurement
Item
12. baseline serum potassium >5.0 milliequivalent (meq)/l
boolean
C0302353 (UMLS CUI [1])
Study Subject Participation Status | Investigational New Drugs | Surgical and medical procedures
Item
13. participation in ongoing drug/intervention-based clinical trial
boolean
C2348568 (UMLS CUI [1])
C0013230 (UMLS CUI [2])
C1948041 (UMLS CUI [3])
Pregnancy
Item
14. pregnancy
boolean
C0032961 (UMLS CUI [1])
Informed Consent Unable
Item
15. unable to provide consent
boolean
C0021430 (UMLS CUI [1,1])
C1299582 (UMLS CUI [1,2])