Multi-center, Web Based Observational Study of Pulmonary Hypertension in Scleroderma Patients

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Trial 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.

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Contact Information

Stanford University
School of Medicine
300 Pasteur Drive
Stanford, CA 94305
Jennifer Hughes