The Living with Pulmonary Hypertension (LPH) questionnaire with Heart Failure questionnaire comprises 21 items, responded to on a 6-point Likert scale ranging from 0 'No' to 5 'Very much'. A total score ranging from 0 (best) to 105 (worst) is calculated by summing the responses to all 21 questions.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 66 patients were evaluable for LPH total score at Week 24.

The Living with Pulmonary Hypertension (LPH) questionnaire with Heart Failure questionnaire comprises 21 items, responded to on a 6-point Likert scale ranging from 0 'No' to 5 'Very much'. A total score ranging from 0 (best) to 105 (worst) is calculated by summing the responses to all 21 questions. In the below table, "N" signifies subjects who were evaluable for the specific parameter at that timepoint.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 72 patients were evaluable for LPH total score at Week 4.

The Living with Pulmonary Hypertension (LPH) questionnaire with Heart Failure questionnaire comprises 21 items, responded to on a 6-point Likert scale ranging from 0 'No' to 5 'Very much'. A total score ranging from 0 (best) to 105 (worst) is calculated by summing the responses to all 21 questions.

The Completers analysis set included patients valid for Safety/ITT analysis sets who had an LPH score at baseline (Visit 1), Week 4, Week 16, and Week 24. 58 patients in Completer analysis set were evaluable for LPH total score at Week 24.

The LPH derived from the Minnesota Living with Heart Failure questionnaire comprises 21 items, responded to on a 6-point Likert scale ranging from 0 'No' to 5 'Very much'. A total score ranging from 0 to 105 is calculated by summing the responses to all 21 questions. A physical dimension score (range 0-40, 8 items) and an emotional dimension score (range 0-25, 5 items) can also be calculated. For all LPH scores, a higher score indicates that patients are more affected by their medical condition. In the below table, "N" signifies subjects who were evaluable for the specific parameter at that timepoint.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 75 patients were evaluable for LPH physical dimension score at week 16 or before.

The LPH derived from the Minnesota Living with Heart Failure questionnaire comprises 21 items, responded to on a 6-point Likert scale ranging from 0 'No' to 5 'Very much'. A total score ranging from 0 to 105 is calculated by summing the responses to all 21 questions. A physical dimension score (range 0-40, 8 items) and an emotional dimension score (range 0-25, 5 items) can also be calculated. For all LPH scores, a higher score indicates that patients are more affected by their medical condition.

The Completers analysis set included patients valid for Safety/ITT analysis sets who had an LPH score at baseline (Visit 1), Week 4, Week 16, and Week 24. 57 patients in Completer analysis set were evaluable for LPH physical dimension score at Week 24.

The LPH derived from the Minnesota Living with Heart Failure questionnaire comprises 21 items, responded to on a 6-point Likert scale ranging from 0 'No' to 5 'Very much'. A total score ranging from 0 to 105 is calculated by summing the responses to all 21 questions. A physical dimension score (range 0-40, 8 items) and an emotional dimension score (range 0-25, 5 items) can also be calculated. For all LPH scores, a higher score indicates that patients are more affected by their medical condition. In the below table, "N" signifies subjects who were evaluable for the specific parameter at that timepoint.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 75 patients were evaluable for LPH emotional dimension score at week 16 or before.

The LPH derived from the Minnesota Living with Heart Failure questionnaire comprises 21 items, responded to on a 6-point Likert scale ranging from 0 'No' to 5 'Very much'. A total score ranging from 0 to 105 is calculated by summing the responses to all 21 questions. A physical dimension score (range 0-40, 8 items) and an emotional dimension score (range 0-25, 5 items) can also be calculated. For all LPH scores, a higher score indicates that patients are more affected by their medical condition.

The Completers analysis set included patients valid for Safety/ITT analysis sets who had an LPH score at baseline (Visit 1), Week 4, Week 16, and Week 24. 58 patients in Completer analysis set were evaluable for LPH emotional dimension score at Week 24.

For LPH total score, the MCID was an 11-point decrease from baseline. In the below table, "N" signifies subjects who were evaluable for the specific parameter at that timepoint.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 72 patients were evaluable for LPH total score at week 4.

For the physical and emotional dimension scores, the MCID was a 4-point decrease. In the below table, "N" signifies subjects who were evaluable for the specific parameter at that timepoint.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 72 patients were evaluable for LPH physical dimension score at week 4.

For the physical and emotional dimension scores, the MCID was a 4-point decrease. In the below table, "N" signifies subjects who were evaluable for the specific parameter at that timepoint.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 72 patients were evaluable for LPH emotional dimension score at week 4.

For LPH total score, the MCID was an 11-point decrease.

The Completers analysis set included patients valid for Safety/ITT analysis sets who had an LPH score at baseline (Visit 1), Week 4, Week 16, and Week 24. 58 patients in Completer analysis set were evaluable for LPH total score at Week 24.

For the physical and emotional dimension scores, the MCID was a 4-point decrease.

The Completers analysis set included patients valid for Safety/ITT analysis sets who had an LPH score at baseline (Visit 1), Week 4, Week 16, and Week 24. 57 patients were evaluable for LPH physical dimension score at week 24.

For the physical and emotional dimension scores, the MCID was a 4-point decrease.

The Completers analysis set included patients valid for Safety/ITT analysis sets who had an LPH score at baseline (Visit 1), Week 4, Week 16, and Week 24. 58 patients in Completer analysis set were evaluable for LPH emotional dimension score at Week 24.

The Work Limitations Questionnaire (WLQ) is an 8-item questionnaire that measures the degree to which employed individuals are experiencing limitations on-the-job due to their health problems, and health-related productivity loss (Presenteeism). The WLQ's terms are aggregated into four scales (i.e. Time Management, Physical demands, Mental-interpersonal demands, Output demands). Scale score range from 0 (limited none of the time) to 100 (limited all of the time) and represent the reported amount of time in the prior two weeks respondents were limited on-the-job. In the below table, "N" signifies subjects who were evaluable for the specific parameter at that timepoint.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 52 patients were evaluable for WLQ at week 4.

The WLQ is an 8-item questionnaire that measures the degree to which employed individuals are experiencing limitations on-the-job due to their health problems, and health-related productivity loss (Presenteeism). The WLQ's terms are aggregated into four scales (i.e. Time Management, Physical demands, Mental-interpersonal demands, Output demands). Using an algorithm, WLQ scale scores can be converted into an estimate of productivity loss. In the below table, "N" signifies subjects who were evaluable for the specific parameter at that timepoint.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 37 patients were evaluable for WLQ percentage of productivity loss at week 4.

The Work Limitations Questionnaire (WLQ) is an 8-item questionnaire that measures the degree to which employed individuals are experiencing limitations on-the-job due to their health problems, and health-related productivity loss (Presenteeism). The WLQ's terms are aggregated into four scales (i.e. Time Management, Physical demands, Mental-interpersonal demands, Output demands). Scale score range from 0 (limited none of the time) to 100 (limited all of the time) and represent the reported amount of time in the prior two weeks respondents were limited on-the-job.

The Completers analysis set included patients valid for Safety/ITT analysis sets who had an LPH score at baseline (Visit 1), Week 4, Week 16, and Week 24. 40 patients in Completer analysis set were evaluable for WLQ at Week 24.

The WLQ is an 8-item questionnaire that measures the degree to which employed individuals are experiencing limitations on-the-job due to their health problems, and health-related productivity loss (Presenteeism). The WLQ's terms are aggregated into four scales (i.e. Time Management, Physical demands, Mental-interpersonal demands, Output demands). Using an algorithm, WLQ scale scores can be converted into an estimate of productivity loss.

The Completers analysis set included patients valid for Safety/ITT analysis sets who had an LPH score at baseline (Visit 1), Week 4, Week 16, and Week 24. 40 patients in Completer analysis set were evaluable for WLQ percentage of productivity loss at week 24.

SF-12v2® Health Survey is a 12-item questionnaire to measure functional health and well-being from the patient's point of view. Physical and Mental Health Composite Scores (PCS & MCS) are computed using the scores of twelve questions and range from 0 to 100, where a zero score indicates the lowest level of health measured by the scales and 100 indicates the highest level of health. In the below table, "N" signifies subjects who were evaluable for the specific parameter at that timepoint.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 73 patients were evaluable for PCS and MCS at Week 4.

SF-12v2® Health Survey is a 12-item questionnaire to measure functional health and well-being from the patient's point of view. Physical and Mental Health Composite Scores (PCS & MCS) are computed using the scores of twelve questions and range from 0 to 100, where a zero score indicates the lowest level of health measured by the scales and 100 indicates the highest level of health.

The Completers analysis set included patients valid for Safety/ITT analysis sets who had an LPH score at baseline (Visit 1), Week 4, Week 16, and Week 24. 58 patients in Completer analysis set were evaluable for PCS and MCS at Week 24.

Functional class was determined by the WHO classification:

I: Patients with PH (pulmonary hypertension) but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain, or near syncope.

II: Patients with PH resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.

III: Patients with PH resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnea or fatigue, chest pain, or near syncope.

IV: Patients with PH with inability to carry out any physical activity without symptoms. These patients manifest signs of right-heart failure. Dyspnea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity.

In the below table, "N" signifies subjects who were evaluable for the specific parameter at that timepoint.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 75 patients were evaluable for WHO Function Class at Week 16 or Last Before.

Functional class was determined by the WHO classification:

I: Patients with PH (pulmonary hypertension) but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain, or near syncope.

II: Patients with PH resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.

III: Patients with PH resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnea or fatigue, chest pain, or near syncope.

IV: Patients with PH with inability to carry out any physical activity without symptoms. These patients manifest signs of right-heart failure. Dyspnea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity.

The Completers analysis set included patients valid for Safety/ITT analysis sets who had an LPH score at baseline (Visit 1), Week 4, Week 16, and Week 24. 57 patients in Completer analysis set were evaluable for WHO Function Class at Week 24.

The Modified Borg Dyspnea Scale assessed the intensity of the patient's dyspnea from 0 (best) to 10 (worst). In the below table, "N" signifies subjects who were evaluable for the specific parameter at that timepoint.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 58 patients were evaluable for Modified Borg Dyspnea Scale at week 24.

The Modified Borg Dyspnea Scale assessed the intensity of the patient's dyspnea from 0 (best) to 10 (worst).

The Completers analysis set included patients valid for Safety/ITT analysis sets who had an LPH score at baseline (Visit 1), Week 4, Week 16, and Week 24. 57 patients in Completer analysis set were evaluable for Modified Borg Dyspnea Scale at Week 24.

Six-minute walk distance (6MWD) was conducted to test the physical limitations of the patient by assessing the patient's exercise capacity. The distance walked by the patient in 6 minutes was measured. In the below table, "N" signifies subjects who were evaluable for the specific parameter at that timepoint.

The ITT analysis set was synonymous with the Safety analysis set definition which included all patients who received at least 1 dose of test drug. 67 patients were evaluable for 6MWD at week 24.

Six-minute walk distance (6MWD) was conducted to test the physical limitations of the patient by assessing the patient's exercise capacity. The distance walked by the patient in 6 minutes was measured.

The Completers analysis set included patients valid for Safety/ITT analysis sets who had an LPH score at baseline (Visit 1), Week 4, Week 16, and Week 24. 57 patients in Completer analysis set were evaluable for 6MWD at week 24.