Pulmonary Hypertension Association Registry

Study Overview
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Brief Summary
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The PHA Registry (PHAR) is a national study about people who have pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). PHAR collects information from people with PAH and CTEPH who are cared for in participating PHA-accredited Pulmonary Hypertension Care Centers throughout the U.S. PHAR will determine how people with PAH and CTEPH are evaluated, tested, and treated, and will observe how well these participants do. The goal is to see if people with PH are treated according to recommended guidelines, and to see if there are certain factors that can lead to better or worse outcomes. PHAR will include information about people with PAH and CTEPH in the U.S. who are seen at participating PHA-accredited PH Care Centers. PHAR contains data about patient care and outcomes. Specifically, data in the PHAR includes information on diagnosis; clinical status; socioeconomic status; diagnosis test results; body size; treatment information; interest in participating in clinical trials; family health and social history; and information about smoking, alcohol, or drug use. Participants are followed over time, and provide updates such as changes in therapy, how often participants need to go to the hospital, and survival. Such information may help healthcare providers provide better care.

Official Title
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Pulmonary Hypertension Association Registry

Conditions
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Pulmonary Arterial Hypertension, Chronic Thromboembolic Pulmonary Hypertension, Pulmonary Hypertension

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: All age groups Written informed consent Pulmonary arterial hypertension (PAH), chronic thromboembolic pulmonary hypertension (CTEPH), or pediatric PH due to developmental lung disease Within 6 months of first outpatient visit at a PH Care Center Exclusion Criteria: Diagnosis of WSPH Group 2 pulmonary hypertension Diagnosis of WSPH Group 3 pulmonary hypertension, except PH due to developmental lung disease Diagnosis of WSPH Group 5 pulmonary hypertension
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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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Pulmonary arterial hypertension (PAH)<br>Patients with newly diagnosed or established PAH, within 6 months of first outpatient visit to a PH Care Center | |
| Chronic thromboembolic pulmonary hypertension (CTEPH)<br>Patients with newly diagnosed or established CTEPH, within 6 months of first outpatient visit to a PH Care Center | |
| Group 3 PH due to Developmental Lung Disease<br>Pediatric patients with newly diagnosed or established Group 3 PH due to developmental lung disease, within 6 months of first outpatient visit to a PH Care Center. | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Proportion of PAH and CTEPH patients completing all guideline-recommended diagnostics | PAH guideline-recommended diagnostics assessed include chest radiography, echocardiogram, ventilation-perfusion (V/Q) scan, pulmonary function tests, overnight oximetry, and right heart catheterization and identified as either completed or not completed | 10 years | 
| Impact of clinical predictors of disease worsening on patient outcomes | Prognostic importance of disease worsening predictors including changes in World Health Organization (WHO) / New York Heart Association (NYHA) functional class (I-IV), 6-minute walk distance in meters, and brain natriuretic peptide (BNP) in pg/mL on survival. | 10 years | 
| Survival |  | 10 years |