Age
Item
1. age 18 and < 75 years at baseline visit.
boolean
C0001779 (UMLS CUI [1])
Idiopathic pulmonary arterial hypertension | Heritable pulmonary arterial hypertension | Hereditary hemorrhagic telangiectasia | Pulmonary arterial hypertension Association | Collagen-vascular disease | Exposure to Drugs | Exposure to Toxin | Congenital heart disease Repaired | Portopulmonary hypertension | HIV Infection | Protease Inhibitors Absent
Item
2. diagnosis of idiopathic pah, heritable pah (including hereditary hemorrhagic telangiectasia), associated pah (including collagen vascular disorders, drug+toxin exposure, repaired congenital heart disease repaired > 5 years, portopulmonary disease, and human immunodeficiency virus (hiv) infection not on protease inhibitor).
boolean
C3203102 (UMLS CUI [1])
C0340543 (UMLS CUI [2])
C0039445 (UMLS CUI [3])
C2973725 (UMLS CUI [4,1])
C0439849 (UMLS CUI [4,2])
C0262428 (UMLS CUI [5])
C0332157 (UMLS CUI [6,1])
C0242508 (UMLS CUI [6,2])
C0332157 (UMLS CUI [7,1])
C0040549 (UMLS CUI [7,2])
C0152021 (UMLS CUI [8,1])
C0205340 (UMLS CUI [8,2])
C1868851 (UMLS CUI [9])
C0019693 (UMLS CUI [10])
C0033607 (UMLS CUI [11,1])
C0332197 (UMLS CUI [11,2])
Pulmonary arterial hypertension Therapy naive | Prostacyclin Absent | Endothelin receptor antagonist Absent | Phosphodiesterase Inhibitors Absent
Item
3. pah treatment naïve including any prostacycline, endothelin receptor antagonist, or phosphodiesterase inhibitors within 12 months prior to enrollment.
boolean
C2973725 (UMLS CUI [1,1])
C0919936 (UMLS CUI [1,2])
C0033567 (UMLS CUI [2,1])
C0332197 (UMLS CUI [2,2])
C1134681 (UMLS CUI [3,1])
C0332197 (UMLS CUI [3,2])
C0031638 (UMLS CUI [4,1])
C0332197 (UMLS CUI [4,2])
Catheterization of right heart
Item
4. previous right heart catheterization that documented:
boolean
C0189896 (UMLS CUI [1])
Mean pulmonary arterial pressure
Item
1. mean pap; 25 mmhg.
boolean
C3854605 (UMLS CUI [1])
Pulmonary Capillary Wedge Pressure
Item
2. pulmonary capillary wedge pressure < 15 mmhg.
boolean
C0086879 (UMLS CUI [1])
Pulmonary Vascular Resistance Wood units | Pulmonary Vascular Resistance | 6 Minute Walk Functional Test Distance m
Item
3. pulmonary vascular resistance; 3.0 wood units or 240 dynes/sec/cm5 5.6mw distances; 150 m and < 450 meters.
boolean
C0456261 (UMLS CUI [1,1])
C0439525 (UMLS CUI [1,2])
C0456261 (UMLS CUI [2])
C3900196 (UMLS CUI [3,1])
C0012751 (UMLS CUI [3,2])
C0475209 (UMLS CUI [3,3])
WHO classification
Item
6. who functional class ii or iii as judged by principal investigators.
boolean
C4267671 (UMLS CUI [1])
Group Number Pulmonary Hypertension
Item
1. group ii - v pulmonary hypertension.
boolean
C0441833 (UMLS CUI [1,1])
C0237753 (UMLS CUI [1,2])
C0020542 (UMLS CUI [1,3])
Pulmonary arterial hypertension Associated with Congenital Heart Defects | Repair Lacking
Item
2. pah with unrepaired congenital heart defect.
boolean
C2973725 (UMLS CUI [1,1])
C0332281 (UMLS CUI [1,2])
C0018798 (UMLS CUI [1,3])
C4319951 (UMLS CUI [2,1])
C0332268 (UMLS CUI [2,2])
Therapy Pulmonary arterial hypertension | Investigational Therapy Pulmonary arterial hypertension | Protein-tyrosine kinase inhibitor | Rho Kinase Inhibitors | Phosphodiesterase Inhibitors | Epoprostenol | Cyclic GMP drug modulation
Item
3. current or prior pah treatments within the last 6-12 months including experimental pah therapies (including but not limited to tyrosine kinase inhibitors, rho-kinase inhibitors, phosphodiesterase inhibitors, prostacycline, or cgmp modulators).
boolean
C0087111 (UMLS CUI [1,1])
C2973725 (UMLS CUI [1,2])
C0949266 (UMLS CUI [2,1])
C2973725 (UMLS CUI [2,2])
C1268567 (UMLS CUI [3])
C4551434 (UMLS CUI [4])
C0031638 (UMLS CUI [5])
C0033567 (UMLS CUI [6])
C0018338 (UMLS CUI [7,1])
C0279532 (UMLS CUI [7,2])
Percent Predicted Total Lung Capacity
Item
4. tlc < 60% predicted; if tlc b/w 60 and 70% predicted, high resolution computed tomography must be available to exclude significant interstitial lung disease.
boolean
C3828589 (UMLS CUI [1])
Percent Predicted FEV1/FVC | Percent predicted FEV1
Item
5. fev1 / fvc < 70% predicted and fev1 < 60% predicted
boolean
C3828600 (UMLS CUI [1])
C0730561 (UMLS CUI [2])
Heart Disease Left sided Echocardiography
Item
6. significant left-sided heart disease (based on pre-trial echocardiogram):
boolean
C0018799 (UMLS CUI [1,1])
C0443246 (UMLS CUI [1,2])
C0013516 (UMLS CUI [1,3])
Aortic valve disorder | Disease of mitral valve
Item
1. significant aortic or mitral valve disease
boolean
C1260873 (UMLS CUI [1])
C0026265 (UMLS CUI [2])
Diastolic dysfunction Grade | Left ventricle Reduced systolic function
Item
2. diastolic dysfunction ; grade ii c.lv systolic function < 45%
boolean
C0520863 (UMLS CUI [1,1])
C0441800 (UMLS CUI [1,2])
C0225897 (UMLS CUI [2,1])
C4025008 (UMLS CUI [2,2])
Pericardial constriction | Restrictive cardiomyopathy | Coronary disease with Ischemia
Item
d. pericardial constriction e. restrictive cardiomyopathy f. significant coronary disease with demonstrable ischemia
boolean
C0240709 (UMLS CUI [1])
C0007196 (UMLS CUI [2])
C0010068 (UMLS CUI [3,1])
C0332287 (UMLS CUI [3,2])
C0022116 (UMLS CUI [3,3])
Chronic Kidney Insufficiency | Estimated creatine clearance MDRD formula
Item
7. chronic renal insufficiency defined as an estimated creatinine clearance < 30 ml/min (by mdrd equation)
boolean
C0403447 (UMLS CUI [1])
C1273051 (UMLS CUI [2,1])
C3839656 (UMLS CUI [2,2])
Atrial arrhythmia
Item
8. current atrial arrhythmias
boolean
C0085611 (UMLS CUI [1])
Uncontrolled hypertension | Systolic Pressure | Diastolic blood pressure
Item
9. uncontrolled systemic hypertension: sbp > 160 mm or dbp > 100mm
boolean
C1868885 (UMLS CUI [1])
C0871470 (UMLS CUI [2])
C0428883 (UMLS CUI [3])
Hypotension, severe | Systolic Pressure
Item
10. severe hypotension: sbp < 80 mmhg.
boolean
C2678378 (UMLS CUI [1])
C0871470 (UMLS CUI [2])
Pregnancy | Breast Feeding
Item
11. pregnant or breast-feeding
boolean
C0032961 (UMLS CUI [1])
C0006147 (UMLS CUI [2])
Mental disorders | Addictive Behavior | Other medical condition At risk Informed Consent | Other medical condition At risk Study Protocol | Other medical condition At risk Treatment Compliance
Item
12. psychiatric, addictive, or other disorder that compromises patient's ability to provide informed consent, follow study protocol, and adhere to treatment instructions
boolean
C0004936 (UMLS CUI [1])
C0085281 (UMLS CUI [2])
C3843040 (UMLS CUI [3,1])
C1444641 (UMLS CUI [3,2])
C0021430 (UMLS CUI [3,3])
C3843040 (UMLS CUI [4,1])
C1444641 (UMLS CUI [4,2])
C2348563 (UMLS CUI [4,3])
C3843040 (UMLS CUI [5,1])
C1444641 (UMLS CUI [5,2])
C4319828 (UMLS CUI [5,3])
Comorbidity Impairing Exercise | Comorbidity Impairing WHO classification | Chronic low back pain | Musculoskeletal problem Peripheral
Item
13. co-morbid conditions that would impair a patient's exercise performance and ability to assess who functional class, including but not limited to chronic low-back pain or peripheral musculoskeletal problems.
boolean
C0009488 (UMLS CUI [1,1])
C0221099 (UMLS CUI [1,2])
C0015259 (UMLS CUI [1,3])
C0009488 (UMLS CUI [2,1])
C0221099 (UMLS CUI [2,2])
C4267671 (UMLS CUI [2,3])
C0457949 (UMLS CUI [3])
C0026859 (UMLS CUI [4,1])
C0205100 (UMLS CUI [4,2])
Medical contraindication Magnetic Resonance Imaging | Claustrophobia | Metallic implant
Item
14. contraindications for magnetic resonance imaging, including significant claustrophobia, implanted metallic objects, or others as per appendix x).
boolean
C1301624 (UMLS CUI [1,1])
C0024485 (UMLS CUI [1,2])
C0008909 (UMLS CUI [2])
C3693688 (UMLS CUI [3])
Hypersensitivity Treprostinil | Hypersensitivity Tadalafil
Item
15. known allergy to treprostinil or tadalafil.
boolean
C0020517 (UMLS CUI [1,1])
C1145760 (UMLS CUI [1,2])
C0020517 (UMLS CUI [2,1])
C1176316 (UMLS CUI [2,2])
Nitrate Oral
Item
16. active oral nitrate use.
boolean
C0028125 (UMLS CUI [1,1])
C1527415 (UMLS CUI [1,2])
Diabetes Mellitus
Item
17. diabetes mellitus.
boolean
C0011849 (UMLS CUI [1])
Cardiac rehabilitation Planned | Pulmonary rehabilitation Planned
Item
18. planned initiation of cardiac or pulmonary rehabilitation during period of study.
boolean
C0700431 (UMLS CUI [1,1])
C1301732 (UMLS CUI [1,2])
C0199529 (UMLS CUI [2,1])
C1301732 (UMLS CUI [2,2])