Real World Observational Study of Sotatercept for Pulmonary Hypertension

Primary outcomes

1. EmPHasis10 Questionnaire

   Time frame: Baseline, Year 1, Year 2, Year 3, Year 4, Year 5, Year 6, Year 7, Year 8, Year 9, Year 10

   The EmPHasis10 Questionnaire consists of 10 questions to assess quality of life. The scores can range from 0-50, a higher score indicates a worse quality of life.

2. Number of pulmonary hypertension vasodilator medications taken by a subject

   Time frame: Baseline, Year 10

   Pulmonary hypertension vasodilator medications are used to decrease pressure in the pulmonary arteries.

3. Number of patients to have a change in dose of prostanoid medications

   Time frame: Baseline, Year 10

   A change in dose of prostanoid medications will be determined by a change from baseline dosage.

4. Number of patient hospitalizations

   Time frame: Baseline, Year 10

   Number of patient hospitalizations will be determined by the number of patients admitted into the hospital.

5. Number of patients to cease therapy sessions

   Time frame: Baseline, Year 10

   Cease of therapy session will be determined by a patient's termination of all therapy sessions

6. Number of patients to discontinue prostanoid therapy

   Time frame: Baseline, Year 10

   Number of patients to discontinue prostanoid therapy will be determined by a patients termination of all prostanoid therapy

7. Number of patients with bleeding complications

   Time frame: Baseline, Year 10

   Bleeding complications include nosebleeds not requiring ER visit or transfusion, epistaxis or GI hemorrhage requiring ED visit/hospitalization.

8. Number of patients to develop Skin telangiectasias

   Time frame: Baseline, Year 10

   Development of skin telangiectasias includes new development, worsening, or distribution

9. Change in 6 min walk distance

   Time frame: Baseline, Year 10

   6 minute walk distance will be measured in meters (m).

10. Number of patients to have a change in hemodynamics

    Time frame: Baseline, Year 10

    Change in hemodynamics include the change in Blood Flow, Blood Pressure, Cardiac Output, Vascular Resistance, Perfusion

11. Change in Right Ventricular Systolic Pressure (RVSP)

    Time frame: Baseline, Year 10

    Right Ventricular Systolic Pressure is the pressure in the right ventricle during contraction. RVSP will be measured in mmHg.

12. Right Ventricular Strain

    Time frame: Baseline, Year 10

    Right Ventricular Strain refers to strain on the right ventricle during contraction. Right Ventricular Strain will be determined by echocardiogram and measured as a percentage.

13. Change in Right Ventricle (RV) function

    Time frame: Baseline, Year 10

    Change in RV function will be determined by echocardiogram and measured as a percentage.

14. Change in capacitance

    Time frame: Baseline, Year 10

    Capacitance is the ability for blood vessels to store and release blood. capacitance will be determined by echocardiogram.

15. Change in tricuspid regurgitation

    Time frame: Baseline, Year 10

    Tricuspid regurgitation is the regurgitation of blood into the right atrium during contraction of the heart.

16. Change in Stroke Volume (SV)

    Time frame: Baseline, Year 10

    Stroke Volume is calculated by End-Diastolic Volume (EDV)-End-Systolic Volume (ESV) and measured in mL

17. Change in Pulse Pressure (PP)

    Time frame: Baseline, Year 10

    Pulse Pressure is calculated by PP=systolic blood pressure (SBP) - diastolic blood pressure (DBP) and measured in mmHg

18. Change in functional class

    Time frame: Baseline, Year 10

    Functional class refers to the severity of patients heart condition. A change in class would refer to the increase or decrease in severity of the condition.

19. Number of patients to survive without receiving a transplant

    Time frame: Baseline, Year 10

    A transplant is defined as receiving an organ or tissue from a donor.