Effects of Pulmonary Hypertension Therapy in Atypical Pulmonary Arterial Hypertension

Sponsor

Mayo Clinic (Other)

Overall Status

Recruiting

CT.gov ID

NCT04787445

Collaborator

(none)

Enrollment

Location

35.9

Anticipated Duration (Months)

0.6

Patients Per Site Per Month

Study Details

Study Description

Brief Summary

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.

Condition or Disease	Intervention/Treatment	Phase
Pulmonary Arterial Hypertension

Detailed Description

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

Study Design

Study Type:

Observational

Anticipated Enrollment :

20 participants

Observational Model:

Cohort

Time Perspective:

Prospective

Official Title:

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

Actual Study Start Date :

Mar 11, 2021

Anticipated Primary Completion Date :

Mar 9, 2024

Anticipated Study Completion Date :

Mar 9, 2024

Outcome Measures

Primary Outcome Measures

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

Other Outcome Measures

Identify predictors on baseline exercise catheterization of subsequent exercise capacity improvement following chronic pulmonary vasodilatory therapy. [6 Month Catheterization]
From paired exercise capacity measurements of peak VO2 from baseline to 6 month testing, patients with clinically meaningful change defined by improvement following treatment of >1 ml/kg/min will be identified. Measurements on baseline exercise catheterization, echocardiography, left atrial strain and clinical characteristics will be assessed for their ability to predict exercise capacity improvement by logistic regression.

Eligibility Criteria

Criteria

Ages Eligible for Study:

18 Years and Older

Sexes Eligible for Study:

All

Accepts Healthy Volunteers:

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