Institutional Review Board, Hospital for Special Surgery
February 04, 2011
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This is a study of scleroderma patients to determine the time of pathologic progression of pulmonary hypertension from pre-pulmonary hypertension, to diagnosable pulmonary hypertension, to clinical worsening of disease. Patients entered into the study will be followed in prospective fashion noting the clinical course of disease by both scheduled and event driven follow up. A total of 8-10 patients will be enrolled at this site and followed for a minimum period of 3-5 years (Approximately 15 sites will enroll 300-400 patients over the next 3 years).
(1) Global Inclusion Criteria
Eligible patients must meet all of the following inclusion criteria:
Patient ³ 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
Pulmonary function test with FVC >70% and a DLCO <55% of predicted or a FVC/DLco ratio >1.6.
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 catherterization showing a mean PA pressure > 25mmHg. diagnosed in the past 6 months or a mean PA pressure > 30 with exercise.
Patients who have a mean PA pressure > 25mmHg (on right heart catheterization) who have severe interstitial fibrosis will be entered as a definite pulmonary hypertension, and classified and followed as WHO Group III even though they would not have qualified for a pre pulmonary hypertension.
Patients who have a mean PA pressure > 25 with a wedge pressure which is > 15 will be entered and followed and classified as WHO Group II.
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.