Effects of Pulmonary Hypertension Therapy in Atypical Pulmonary Arterial Hypertension

Study Overview
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Brief Summary
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The purpose of this study is to characterize the clinical and hemodynamic response of Pulmonary Arterial Hypertension (PAH) therapy in patients with atypical PAH and risk factors for left heart disease.

Detailed Description
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This is an observational prospective study to better understand the clinical impact of Pulmonary Arterial Hypertension (PAH) specific therapy in patients with atypical PAH among those with risk factors for left heart disease The study involves detailed baseline clinical evaluation prior to initiation of PAH therapy, followed by repeat clinical assessment after 6 months of medical therapy

Official Title
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Effects of Pulmonary Hypertension Therapy in Atypical Pulmonary Arterial Hypertension: An Exercise Hemodynamic Study (TAPH Study)

Conditions
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Pulmonary Arterial Hypertension

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Age ≥ 18 years Pulmonary hypertension with mean PA pressure >20 mmHg and a planned initiation of pulmonary arterial hypertension therapy No active treatment for precapillary pulmonary hypertension Ambulatory (not wheelchair/scooter dependent) Presence of any risk factor for left heart disease will qualify for inclusion (either atrial fibrillation, body mass index>30 kg/m2, arterial hypertension, diabetes, coronary artery disease or age>60 years) Exclusion Criteria: Significant chronic obstructive pulmonary disease that is a primary contributor to symptoms in the opinion of the investigator Ischemia thought to contribute to dyspnea in the opinion of the investigator Obstructive hypertrophic cardiomyopathy Known infiltrative cardiomyopathy (amyloid) Constrictive pericarditis or tamponade Active myocarditis Complex congenital heart disease More than mild aortic or mitral stenosis Intrinsic (prolapse, rheumatic) valve disease with more than moderate mitral, tricuspid or aortic regurgitation Acute or chronic severe liver disease as evidenced by any of the following: encephalopathy, variceal bleeding, INR > 1.7 in the absence of anticoagulation treatment Terminal illness (other than HF) with expected survival of less than 1 year Enrollment or planned enrollment in another therapeutic clinical trial in next 3 months. Inability to comply with planned study procedures Pregnancy or breastfeeding mothers

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Describe prevalence of occult left heart disease during exercise in atypical pulmonary hypertension, as defined by pulmonary arterial wedge pressure during exercise cardiac catheterization. | Pulmonary arterial wedge pressure >25 mmHg during exercise cardiac catheterization will indicate occult left heart disease. | Baseline Catheterization | 
| Evaluate the therapeutic response of chronic pulmonary vasodilator therapy | Favorable pulmonary vasodilatory effect will be associated a change of > 10% reduction in pulmonary artery-pressure flow slope from baseline to 6 month catheterization | 6 Month Catheterization | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Right Heart Catheterization