The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR), a validated PAH-specific instrument consisting of 65 items used to assess symptoms, functioning and quality of life. The questionnaire is divided into three sections; Symptoms (Scores 0-25; high scores indicate more symptoms), Activity (Score 0-30; low score indicates good functioning)and Quality of Life (0-25; high scores indicate poor QoL). The sum of these scores equates to the Total score (0-80). In the CAMPHOR scores, lower scores indicate improvements.

The Treatment Satisfaction Questionnaire for Medication (TSQM), a validated generic measure of treatment satisfaction consisting of 14 Likert-response items comprising four domains: Effectiveness, Side Effects, Convenience, and Global Satisfaction. The TSQM was completed at baseline and at Week 8. The TSQM consists of 13 items that made up three specific scales (Effectiveness, Side effects, Convenience) and one global satisfaction scale. TSQM items are scaled using either a 5-point or 7-point scale. Five-point scales are used for unidimensional continua (e.g. extremely satisfied to not at all), while 7-point scales are used for bipolar continua(e.g., extremely positive to extremely negative. Non-neutral midpoints are used for 7-point scales, resulting in a greater range of positive response options than negative options for these items. Scale scores are transformed into scores ranging from 0 to 100, with a higher score indicating more satisfaction.

A Drug Administration Activities Diary, used by subjects to record in detail the amount of time (in minutes) spent on specifically-defined drug preparation/administration activities (e.g. diluting drug, preparing reservoir, and changing tubing), was completed over a 7-day period during the Screening period while on epoprostenol and repeated at Week 7 following transition to Remodulin. Drug Administration Activities Diary results are reported as average time per week spent on drug administration activities

The presence or absence of fatigue was documented. If present, the intensity of fatigue was rated mild, moderate, or severe.

The presence or absence of edema was documented. If present, the intensity of edema was rated mild, moderate, or severe.

The presence or absence of dyspnea was documented. If present, the intensity of dyspnea was rated mild, moderate, or severe.

The presence or absence of orthopnea was documented. If present, the intensity of orthopnea was rated mild, moderate, or severe.

The presence or absence of dizziness was documented. If present, the intensity of dizziness was rated mild, moderate, or severe.

The presence or absence of syncope was documented. If present, the intensity of syncope was rated mild, moderate, or severe.

The presence or absence of chest pain was documented. If present, the intensity of chest pain was rated mild, moderate, or severe.

A Patient Global Impression of Change Questionnaire, which consists of three items that ask the subject to rate changes (much better, somewhat better, about the same, somewhat worse, much worse) in their symptoms of PAH, the amount of time spent on activities associated with preparing and administering PAH therapy, and their satisfaction with their PAH therapy since transitioning from epoprostenol to intravenous Remodulin was conducted at Week 8 only and responses are reported as frequency distributions.

Class I: No limitation of physical activity. Class II: Slight limitation of physical activity. Class III: Marked limitation of physical activity. Class IV: Inability to carry out any physical activity without symptoms.

Class I: No limitation of physical activity. Class II: Slight limitation of physical activity. Class III: Marked limitation of physical activity. Class IV: Inability to carry out any physical activity without symptoms.

Two subjects had a Week 8 visit that was outside of the visit window and are included in the summary. One subject died prior to Week 8 assessments.

The Borg Dyspnea Score is a 10-point scale rating the maximum level of dyspnea experienced after the Six-Minute Walk Test. Scores range from 0 (for the best condition) to 10 (for the worst condition).

Two subjects had a Week 8 visit that was outside of the visit window and are included in the summary. One subject died prior to Week 8 assessments.

The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR), a validated PAH-specific instrument consisting of 65 items used to assess symptoms, functioning and quality of life. The questionnaire is divided into three sections; Symptoms (Scores 0-25; high scores indicate more symptoms), Activity (Score 0-30; low score indicates good functioning)and Quality of Life (0-25; high scores indicate poor QoL). The sum of these scores equates to the Total score (0-80). In the CAMPHOR scores, lower scores indicate improvements.

Two subjects with a Week 8 visit outside of the visit window are included in the summary. One subject died prior to completing the Week 8 visit. One subject had an incomplete Baseline questionnaire and the CAMPHOR Activity component could not be calculated, therefore N=5 Activity and Total Score Components, and N=6 for Symptom and Quality of Life.

The Treatment Satisfaction Questionnaire for Medication (TSQM), a validated generic measure of treatment satisfaction consisting of 14 Likert-response items comprising four domains: Effectiveness, Side Effects, Convenience, and Global Satisfaction. The TSQM was completed at baseline and at Week 8. The TSQM consists of 13 items that made up three specific scales (Effectiveness, Side effects, Convenience) and one global satisfaction scale. TSQM items are scaled using either a 5-point or 7-point scale. Five-point scales are used for unidimensional continua (e.g. extremely satisfied to not at all), while 7-point scales are used for bipolar continua(e.g., extremely positive to extremely negative. Non-neutral midpoints are used for 7-point scales, resulting in a greater range of positive response options than negative options for these items. Scale scores are transformed into scores ranging from 0 to 100, with a higher score indicating more satisfaction.

Two subjects had a Week 8 visit that was outside of the visit window and are included in the summary. One subject died prior to completing the Week 8 visit.

A Drug Administration Activities Diary, used by subjects to record in detail the amount of time (in minutes) spent on specifically-defined drug preparation/administration activities (e.g. diluting drug, preparing reservoir, and changing tubing), was completed over a 7-day period during the Screening period while on epoprostenol and repeated at Week 7 following transition to Remodulin. Drug Administration Activities Diary results are reported as average time per week spent on drug administration activities

Two subjects did not have Week 8 Drug Administration Activity Diaries completed.

Connect Drug and Total Time Components: N=4; One subject did not have data recorded for Connect drug activities. Total time could not be calculated for this subject.

The presence or absence of fatigue was documented. If present, the intensity of fatigue was rated mild, moderate, or severe.

Two subjects had a Week 8 visit that was outside of the visit window and are included in the summary. One subject died prior to Week 8 assessments.

The presence or absence of edema was documented. If present, the intensity of edema was rated mild, moderate, or severe.

The presence or absence of dyspnea was documented. If present, the intensity of dyspnea was rated mild, moderate, or severe.

The presence or absence of orthopnea was documented. If present, the intensity of orthopnea was rated mild, moderate, or severe.

The presence or absence of dizziness was documented. If present, the intensity of dizziness was rated mild, moderate, or severe.

The presence or absence of syncope was documented. If present, the intensity of syncope was rated mild, moderate, or severe.

The presence or absence of chest pain was documented. If present, the intensity of chest pain was rated mild, moderate, or severe.

A Patient Global Impression of Change Questionnaire, which consists of three items that ask the subject to rate changes (much better, somewhat better, about the same, somewhat worse, much worse) in their symptoms of PAH, the amount of time spent on activities associated with preparing and administering PAH therapy, and their satisfaction with their PAH therapy since transitioning from epoprostenol to intravenous Remodulin was conducted at Week 8 only and responses are reported as frequency distributions.

Two subjects had a Week 8 visit that was outside of the visit window and are included in the summary. One subject died prior to completing the Week 8 visit.

One subject was hospitalized for bacteraemia attributed to Remodulin.

Subject was hospitalized for abdominal pain, nausea, vomiting and dehydration and subsequently died. The investigator assessed the relationship to the study drug as not reasonably attributable to the study drug.

Subject was hospitalized for abdominal pain, nausea, vomiting and dehydration and subsequently died. The investigator assessed the relationship to the study drug as not reasonably attributable to the study drug.

Subject was hospitalized for abdominal pain, nausea, vomiting and dehydration and subsequently died. The investigator assessed the relationship to the study drug as not reasonably attributable to the study drug.

Quality of Life Score. Changes between Baseline and Week 8 were assessed using the Wilcoxon signed rank test. A two-sided p value of < 0.05 was considered statistically significant. No imputation was used for missing values.

Total Score N=5; Baseline Activity component score could not be calculated for one subject. Total Score could not be calculated for this subject.

Wilcoxon signed rank test was used to compare the values at Baseline to values at Week 8. A two-sided p value of < 0.05 was considered statistically significant. No imputation was used for missing values.

Convenience Score. Changes between Baseline and Week 8 were assessed using the Wilcoxon signed rank test. A two-sided p value of < 0.05 was considered statistically significant. No imputation was used for missing values.

Global Satisfaction Score. Changes between Baseline and Week 8 were assessed using the Wilcoxon signed rank test. A two-sided p value of < 0.05 was considered statistically significant. No imputation was used for missing values.

Connect Drug. Changes between Baseline and Week 8 were assessed using the Wilcoxon signed rank test. A two-sided p value of < 0.05 was considered statistically significant. No imputation was used for missing values.

Change Dressing. Changes between Baseline and Week 8 were assessed using the Wilcoxon signed rank test. A two-sided p value of < 0.05 was considered statistically significant. No imputation was used for missing values.

Total Time. Changes between Baseline and Week 8 were assessed using the Wilcoxon signed rank test. A two-sided p value of < 0.05 was considered statistically significant. No imputation was used for missing values.