©2022 Stanford Medicine
Multi-center, Web Based Observational Study of Pulmonary Hypertension in Scleroderma Patients
Recruiting
I'm InterestedTrial ID: NCT00377949
Purpose
The purpose of this study is to determine the timeline of progression from pre-pulmonary
hypertension to diagnosable pulmonary hypertension based on right heart catheterization.
Moreover, to determine the timeline for progression from diagnosable pulmonary hypertension
to clinical worsening of disease as defined as death, hospitalization, or worsening of PHT
symptoms.
Official Title
The Natural History and Outcome of Patients With Scleroderma at High Risk for or With Early Pulmonary Hypertension
Eligibility
1. Global Inclusion Criteria
- Eligible patients must meet all of the following inclusion criteria:
- Patient ≥ 18 years with a clinical diagnosis of SSc (ACR criteria or the LeRoy
criteria for limited or diffuse scleroderma
2. Specific Inclusion Criteria
- Diagnosis of "pre" pulmonary arterial hypertension defined as:
- Echocardiogram with a resting sPAP of ≥ 40mmHg Or
- Pulmonary function test with FVC >70% and a DLCO <55% of predicted or a FVC/DLco
ratio >1.6. or
- Right heart catheterization which shows or a mean PA pressure > 30mmHg with
exercise (with a mPAP < 25mmHg at rest)
Patients entered as a 'pre'-pulmonary arterial hypertension who then undergo right heart
catheterization and are found to have pulmonary arterial hypertension, pulmonary venous
hypertension or diastolic dysfunction or pulmonary hypertension secondary to interstitial
lung disease will be followed as a definite PH patient and classified into the appropriate
category.
- Diagnosis of definite pulmonary hypertension Patients with pulmonary hypertension with
a right heart catheterization showing a mean PA pressure > 25mmHg, diagnosed in the
past 6 months.
Classification of PH Group 1 PAH - Patients with mPAP ≥ 25mmHg with a wedge < 15mmHg Group
2 PVH - Patients who have a mean PA pressure ≥ 25mmHg with a wedge pressure which is > 15
mmHg Group 3 PH-ILD Patients who have a mean PA pressure ≥ 25mmHg (on right heart
catheterization) who have moderate to severe interstitial fibrosis on HRCT scan with a FVC
and TLC < 65% predicted
b. Exclusion Criteria
- Diagnosis and treatment of pulmonary hypertension for > 6 months
- Patients with known severe interstitial fibrosis, pulmonary thrombotic disease, heart
failure, cardiomyopathy,history of coronary artery disease or other cardio-pulmonary
problems which could cause pulmonary hypertension are not eligible for the
'pre'-pulmonary hypertension but do qualify for the definite pulmonary hypertension
group if they have a right heart catheterization showing a mean PAH >25mmHg.
Recruiting
I'm InterestedContact Information
Stanford University
School of Medicine
300 Pasteur Drive
Stanford,
CA
94305
Jennifer Hughes