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.
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
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
New patients to a PHA-accredited PH Care Center (PHCC) with either newly diagnosed or established 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 the PHCC.
Cohort
Prospective
Not listed
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"
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.
Survival
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