PHA 2026 Research Room Studies

This tool was created for people living with pulmonary hypertension:

• To better understand PH and available resources.
• To think about what matters most in their care.
• To help guide conversations with their PH health care team.
• To find support in their community.

We want to learn from patients about how useful this tool is and how it can be improved.

Inclusion Criteria

• Adults 18-80 years old.
• Confirmed diagnosis of pulmonary hypertension.
• Ability to provide informed consent.

Exclusion Criteria

• Individuals under 18 or over 80 years old.
• Inability to provide informed consent.

• Complete the decision-making tool and provide feedback to researchers during a mini-interview.
• Researchers will be collecting feedback on usability, acceptability and implementation feasibility.
• Estimated time commitment: 10-15 minutes per participant.
• QR code available for independent completion.

Researchers are performing this study to:

• Determine if changes in blood inflammatory proteins over time are clinically useful in PAH and/or are associated with right ventricular function as assessed by cardiac MRI.
• Examine circulating immune cell gene expression differences in PAH patients that may improve diagnostic testing or clinical monitoring.
• Expand an integrated NIH PAH research program that includes a compliment of laboratory and clinical investigations.

Inclusion Criteria

• WHO Group 1 pulmonary hypertension; except those with cirrhosis and portal hypertension or with active infection.
• Patients 18 years or older.
• Ability to provide informed written consent.

Exclusion Criteria

• Pregnant or breastfeeding women.
• Age less than 18 years.
• Inability to provide informed written consent.

• Researchers will discuss the study with potentially interested participants and share written information.
• Contact information will be collected from interested participants for future involvement.
• Estimated time commitment: 10 minutes per participant for an informal discussion.

Researchers are performing the study to:

• To determine whether or not spironolactone is a beneficial therapy in PAH.
• To evaluate if spironolactone therapy in PAH has an effect on vascular inflammation.
• To discover if spironolactone therapy in PAH changes blood immune cell gene expression.

Inclusion criteria

• WHO Group 1 (PAH) except those with cirrhosis and portal hypertension or with active infection.
• Age 18 or older.
• Ability to provide informed written consent.
• Not pregnant.

Exclusion criteria

• Known or suspected allergy to spironolactone.
• Pregnant or breastfeeding.
• Age < 18 years.
• Inability to provide informed written consent for participation in the study.

• Researchers will discuss the study with potentially interested participants and share written information.
• Contact information will be collected from interested participants for future involvement.
• Estimated time commitment: 10 minutes per participant for an informal discussion.

This study aims to improve understanding of pulmonary arterial hypertension.

Researchers have found some genes that are associated with PAH and are actively looking for additional genes that may be involved in the disease. Identification of other gene changes, hormonal, and body chemistry abnormalities, and how these factors work together will help in understanding the disease process and may lead to improved therapy.

Researchers plan to accomplish this understanding by studying medical and family history along with blood samples from patients and family members.

Inclusion criteria

• Self-reported diagnosis of heritable pulmonary arterial hypertension or idiopathic pulmonary arterial hypertension.
• Age 0-90 years.

Exclusion Criteria

• Other diagnosis.
• Age greater than 90.

• Researchers will perform the consent and enrollment process.
• Researchers will explain the study and have eligible participants complete an electronic questionnaire.
• Under the supervision of the researchers, eligible enrolled patients will undergo a blood draw with phlebotomy team.
• QR code can be provided.
• Estimated time commitment: 15 minutes for questionnaire.

This remote Fitbit study helps adolescents with pulmonary arterial hypertension become more active by using a text Messaging (mHealth) program designed to encourage more daily steps.

The results will guide a larger future study to see if increasing daily activity can improve quality of life and health outcomes for adolescents with PAH.

Inclusion Criteria

• Adolescents between 10-21 years of age.
• Diagnosed with WHO Group 1 PAH or WHO Group 4 PH (CTEPH).
• WHO functional class I-III.
• Stable PAH-specific medication regimen for three months prior to enrollment.
• Subjects with only a single diuretic adjustment in the prior three months will be included. Adjustments in IV prostacyclin for side effect management are allowed.

Exclusion Criteria

• Diagnosis of PAH etiology other than idiopathic, heritable, or associated.
• Prohibited from normal activity due to wheelchair-bound status, bed-bound status, reliance on a cane/walker, activity-limiting angina, activity-limiting osteoarthritis, or other conditions that limit activity.
• Pregnancy.
• Functional class IV heart failure.
• Requirement of > 2 diuretic adjustments in the prior three months.
• Preferred form of activity is not measured by an activity tracker (swimming, yoga, ice skating, stair master, or activities on wheels such as bicycling or rollerblading).
• Can not be involved in any other investigational intervention.
• Does not own or have access to a personal smartphone device.

• Researchers will perform the consent and enrollment process
• Researchers will review medical records, explain the study and assist eligible participants with Fitbit set-up, if needed.
• Estimated time commitment: 10-20 minutes per participant.

This study aims to learn why some people with PAH have more fat around the right side of their heart than others.

Extra fat in this area may play a role in right-side heart disease.

Understanding this could help researchers find treatments that reduce heart fat and improve outcomes for people with PAH.

Inclusion Criteria

• Age > 18 years old.
• Diagnosed with heritable or connective tissue disease-associate PAH.
• WHO Functional Class I-III.
• Ambulatory.
• Able to have an MRI/MRS, perform a 6MWD test, and cardiopulmonary exercise test (bike test).

Exclusion Criteria

• Diagnosis of PAH etiology other than heritable or connective tissue disease-associated PAH.
• WHO Functional Class IV heart failure.
• Requirement for continuous oxygen.
• Unable to have an MRI/MRS, perform a 6MWD test, and cardiopulmonary exercise test.
• Patients with implanted/embedded ferromagnetic material that would preclude cardiac MRI.

• Researchers will discuss the study with potentially interested participants and share information.
• Contact information will be collected from interested participants for future scheduling and involvement.
• QR code can be provided.
• Estimated time commitment: 15 minutes per participant for an informal discussion.
• Study procedures will be completed at Vanderbilt Medical Center in Nashville, TN. The study may be able to provide flight and travel reimbursement to and from Vanderbilt for the study.

The goal of this study is to improve the understanding of the role of genetics in families impacted by pulmonary arterial hypertension. Identification of gene changes, hormonal and blood abnormalities and how these work together will help in understanding the disease process and may lead to improved therapy.

The study will be held over 32 months and may include up to three visits, depending on genetic testing results.

Depending on genetic testing results and study visits completed, participants in this study may receive $100 per visit, up to $300 total.

Inclusion criteria:

• Children and adults, ages 13 and up.
• Patients diagnosed with heritable pulmonary arterial hypertension
• Individuals without pulmonary arterial hypertension who are part of a family impacted by HPAH.
• Individuals who have had genetic testing and know they carry a HPAH-related genetic mutation (e.g. BMPR2, etc.)

Exclusion criteria

• Under 13 years old.

• Researchers will explain the study and have eligible participants complete an electronic questionnaire.
• Researchers will perform the consent and enrollment process and take patient vitals.
• Under the supervision of the researchers, eligible enrolled patients will undergo a blood draw with phlebotomy team.
• QR code can be provided.
• Estimated time commitment: 10 minute discussion followed by 15 minute questionnaire.
• Possible additional time commitment: one remote study visit a year.

We are testing a new tool (PHORA) that helps people with PAH and their caregivers understand their health information using clear visuals and explanations.

In a guided session, you’ll explore the tool with health data closely matched to your own, share what makes sense or feels confusing, and tell us how it might affect planning and conversations with your doctors and family.

Your feedback will help us improve the tool and design a future, longer-term study to see how it works in everyday life.

Inclusion criteria

• Patients: Aged 18 or above who report having been diagnosed with PAH.
• Caregivers: Aged 18 or above who report being a caregiver for an individual diagnosed with PAH.
• Clinicians: Aged 18 or above who report having worked with patients diagnosed with PAH.

Exclusion Criteria

• Under 18 years old.
• Inability to provide informed consent.
• Unable to interact with the prototype as needed.
• Those experiencing an acute medical crisis or not in a medical condition to participate safely will be excluded.

• Researchers will explain the study and select a case from the de-identified patient database that most closely reflects your diagnosis.
• Participants will complete tasks in the PHORA dashboard and think aloud & provide feedback to researchers.
• After completion of the PHORA tool, a brief questionnaire will be completed to capture overall perceptions of the tool.
• Estimated time commitment: 15-20 minutes per participant.

Researchers are conducting an anonymous survey to understand what matters most to people living with pulmonary hypertension when making treatment decisions, including which medication benefits and side effects are most important.

What you share will help researchers and doctors better understand patient priorities and could help patients and their doctors make more personalized treatment choices.

Inclusion criteria

• Adults 18 years of age or older.
• Self-reported diagnosis of pulmonary hypertension (any group/etiology).

Exclusion Criteria

• Under 18 years old.

• This study will only be conducted electronically through a link or QR code.
• Participants will complete a brief, anonymous Qualtrics survey on their mobile device or laptop.
• Estimated time commitment: 5-7 minutes per participant.

This study aims to understand individuals experience starting and using an inhaled treatment for pulmonary hypertension

A brief survey will focus on your lived experience of treatment, decision-making, education, titration experience, challenges, and day-to-day use of inhaled prostacyclin therapy.

Your feedback may help improve education and support for patients starting these treatments.

Inclusion criteria:

• Adults =18 years of age.
• Diagnosed with pulmonary arterial hypertension or pulmonary hypertension associated with interstitial lung disease.
• Currently using a dry powder inhaled (DPI) prostacyclin therapy (e.g., inhaled treprostinil DPI) or have used one within the past 12 months.
• Willing and able to provide informed consent and participate in a brief patient survey.

Exclusion criteria:

• Under 18 years old.
• Patients who have never used inhaled prostacyclin therapy.
• Patients using only nebulized or parenteral prostacyclin therapies without DPI experience.
• Individuals currently enrolled in interventional clinical trials that may influence treatment experience reporting.

• Eligible participants will complete a brief electronic questionnaire.
• Estimate time commitment: ~10 minutes per participant

To help researchers better understand the altered cellular pathway that is common to HHT and PAH, blood samples and/or cheek swabs will be collected from individuals who live with both HHT and PAH.

Data generated by this project may also help uncover novel approaches to treating the broader population of both diseases.

Inclusion criteria:

• Adults 18 years of age or older.
• A clinical diagnosis of PAH and HHT as defined by clinical criteria.
• Ability to provide informed consent.

Exclusion criteria:

• Under 18 years old.
• Individuals who do not have both PAH and HHT.
• Inability to provide informed consent.

• Researchers will explain the study and participants will complete a short questionnaire about their family and medical history related to HHT and PAH.
• Under the supervision of the researchers, eligible patients will undergo a blood draw and/or cheek swab.