gender, age
Item
male or female, age 3-18 years
boolean
C0079399 (UMLS CUI [1])
C0001779 (UMLS CUI [2])
pulmonary arterial hypertension due to idiopathic pulmonary arterial hypertension, connective tissue disease or congenital heart disease
Item
diagnosis of pah due to ipah, connective tissue disease, or congenital heart disease
boolean
C2973725 (UMLS CUI [1,1])
C3203102 (UMLS CUI [1,2])
C3697982 (UMLS CUI [1,3])
C3698160 (UMLS CUI [1,4])
cardiac catheterization, mpap, pcwp, lvedp, pvr
Item
pulmonary arterial hypertension confirmed by a cardiac catheterization, with mpap > 25 mmhg at rest, pcwp or lvedp ≤ 15 mmhg, and pvr ≥ 240 dynes/sec/cm-5 (ie, ≥ 3.0 units m2)
boolean
C0018795 (UMLS CUI [1,1])
C3854605 (UMLS CUI [1,2])
C0086879 (UMLS CUI [1,3])
C0456190 (UMLS CUI [1,4])
C0456261 (UMLS CUI [1,5])
nyha
Item
nyha/who functional class ii, iii, or iv
boolean
C1275491 (UMLS CUI [1])
6-minute walk test
Item
have had a 6-minute walk test (6-mwt) performed within the 3 months preceding the screening visit (if ≥ 8 years of age)
boolean
C0430515 (UMLS CUI [1])
cardiopulmonary exercise testing
Item
have had cardiopulmonary exercise testing (cpet) performed within the 3 months preceding the screening visit (if ≥ 8 years of age and > 130 cms in height. cpet testing will be conducted on nyha/who class iv patients at the discretion of the principal investigator)
boolean
C2959886 (UMLS CUI [1])
pulmonary arterial hypertension therapy
Item
if receiving therapy for pah, on a stable dose and regimen for at least 3 months prior to the screening visit
boolean
C2973725 (UMLS CUI [1,1])
C0087111 (UMLS CUI [1,2])
digoxin, diuretic, oxygen therapy
Item
if receiving digoxin, diuretic, or oxygen therapy, on a stable dose and regimen for at least 30 days preceding the screening visit
boolean
C0012798 (UMLS CUI [1])
C0012265 (UMLS CUI [2])
C0184633 (UMLS CUI [3])
nebulizer use
Item
must demonstrate the ability to appropriately use the nebulizer device as part of the screening process
boolean
C0027524 (UMLS CUI [1])
prostacyclins
Item
prior use of prostacyclins or prostacyclin analogues within 3 months prior to screening
boolean
C0205911 (UMLS CUI [1])
portal hypertension, hepatic disease
Item
portal hypertension or clinically relevant hepatic disease
boolean
C0020541 (UMLS CUI [1])
C0023895 (UMLS CUI [2])
eisenmenger syndrome, SpO2
Item
eisenmenger syndrome with resting spo2 <88% on room air
boolean
C0013743 (UMLS CUI [1,1])
C0428179 (UMLS CUI [1,2])
chronic lung disease, asthma, bronchopulmonary dysplasia, cystic fibrosis, congenital diaphragmatic hernia
Item
clinically relevant asthma or other chronic lung disease, such as bronchopulmonary dysplasia, cystic fibrosis, or repaired or unrepaired congenital diaphragmatic hernia
boolean
C0746102 (UMLS CUI [1,1])
C0004096 (UMLS CUI [1,2])
C0006287 (UMLS CUI [1,3])
C0010674 (UMLS CUI [1,4])
C0235833 (UMLS CUI [1,5])
lung infection, hemoptysis
Item
diagnosis or current evidence of active lung infection or clinically relevant hemoptysis
boolean
C0876973 (UMLS CUI [1])
C0019079 (UMLS CUI [2])
left-sided heart disease, valvular disease, heart failure
Item
left-sided heart disease, including valvular disease or heart failure
boolean
C0018799 (UMLS CUI [1,1])
C0225809 (UMLS CUI [1,2])
C0018824 (UMLS CUI [1,3])
C0018801 (UMLS CUI [1,4])
pulmonary arterial hypertension therapy dosage adjustment
Item
initiation or dosage adjustment of pah-specific therapy within 3 months prior to screening
boolean
C2973725 (UMLS CUI [1,1])
C0087111 (UMLS CUI [1,2])
C0420247 (UMLS CUI [1,3])
digoxin, diuretics, anticoagulation, oxygen therapy
Item
initiation of treatment with digoxin, diuretics, anticoagulation, or oxygen therapy within 30 days prior to screening
boolean
C0012265 (UMLS CUI [1])
C0012798 (UMLS CUI [2])
C0449867 (UMLS CUI [3])
C0184633 (UMLS CUI [4])