| Primary | Number of Participants With Non-serious Treatment-emergent Adverse Events (Non-STEAEs) and Serious Treatment-emergent Adverse Events (STEAEs) | AE was defined as any untoward medical occurrence in participant or clinical investigation participant,temporally associated with use of medicinal product, whether or not considered related to medicinal product.SAE was defined as any untoward medical occurrence that, at any dose: results in death,is life threatening, requires hospitalization or prolongation of existing hospitalization,results in disability or incapacity,or is congenital anomaly or birth defect, important medical events that may not immediately life threatening or result in death or hospitalization but may jeopardize participant or may require medical or surgical intervention as per medical or scientific judgement or associated with drug-induced liver injury.TEAE is any event that was not present prior to initiation of study treatment or any event already present that worsens in either intensity or frequency following exposure to study treatment. TEAEs which were not serious TEAEs were considered as non serious TEAEs. | Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588) | Posted | | Count of Participants | | Participants | | Up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) | Participants received ambrisentan 7.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG003 | Ambrisentan 10 mg (Safety) | Participants received ambrisentan 10 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
| | | Title | Denominators | Categories |
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| Non-STEAEs | | |
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| Primary | Change From Baseline in Liver Function Parameters: Alanine Amino Transferase (ALT), Aspartate Amino Transferase (AST), Gamma Glutamyl Transferase (GGT), Total Bilirubin | Blood samples were collected from participants for analysis of following clinical chemistry parameters: ALT, AST, GGT, total bilirubin. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Millimoles per liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Chemistry Parameters: Calcium, Chloride, Carbon Dioxide (CO2) Content, Glucose, Potassium, Magnesium, Sodium, Phosphorus Inorganic, Blood Urea Nitrogen (BUN) | Blood samples were collected from participants for analysis of following clinical chemistry parameters: Calcium, chloride, CO2 content, glucose, potassium, magnesium, sodium, phosphorus inorganic, and BUN. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Millimoles per liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Chemistry Parameters: Alkaline Phosphatase (ALP), Creatine Kinase (CK), Lactate Dehydrogenase (LDH) | Blood samples were collected from participants for analysis of following clinical chemistry parameters: ALP, CK, LDH. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | International units per Liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | |
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| Primary | Change From Baseline in Chemistry Parameters: Creatinine, Uric Acid | Blood samples were collected from participants for analysis of following clinical chemistry parameters: Creatinine, uric acid. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Micromoles per liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 |
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| Primary | Change From Baseline in Chemistry Parameters: Albumin, Total Protein | Blood samples were collected from participants for analysis of following clinical chemistry parameters: Albumin, total protein. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Grams per liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) |
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| Primary | Change From Baseline in Hematology Parameters: Hemoglobin and Mean Corpuscle Hemoglobin Concentration (MCHC) | Blood samples were collected from participants for analysis of following hematology parameters: Hemoglobin and MCHC. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Grams per Liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 |
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| Primary | Change From Baseline in Hematology Parameters: Hematocrit | Blood samples were collected from participants for analysis of following hematology parameters: Hematocrit. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Proportion of red blood cells in blood | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) |
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| Primary | Change From Baseline in Hematology Parameters: Basophils, Eosinophils, Lymphocytes, Monocytes, Total Neutrophils, White Blood Cells (WBC), Platelet Count | Blood samples were collected from participants for analysis of following hematology parameters: Basophils, eosinophils, lymphocytes, monocytes, total neutrophils, WBC, platelet count. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Giga cells per Liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Hematology Parameter: Mean Corpuscle Hemoglobin | Blood samples were collected from participants for analysis of following hematology parameter: Mean Corpuscle Hemoglobin. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Picograms | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) |
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| Primary | Change From Baseline in Hematology Parameter: Mean Corpuscle Volume | Blood samples were collected from participants for analysis of following hematology parameter: Mean Corpuscle Volume. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Femtoliters | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) |
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| Primary | Change From Baseline in Hematology Parameters: Red Blood Cell Count, Reticulocytes | Blood samples were collected from participants for analysis of following hematology parameters: Red Blood Cell count, reticulocytes. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Trillion cells per liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 |
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| Primary | Number of Participants With Abnormal Values for Physical Examination Parameter: Liver Size | Physical examination included measurement of liver size. Any abnormal enlargement or reduction in the size of the liver is reported. Liver size was assessed as normal or abnormal. Data for abnormal (improved, worsened and unchanged) liver size is presented. End of study visit data is presented. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Count of Participants | | Participants | | Up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) |
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| Primary | Number of Participants With Abnormal Values for Physical Examination Parameter: Jugular Venous Pressure | Physical examination included measurement of Jugular venous pressure. Jugular venous pressure was assessed as normal or abnormal. Data for abnormal (improved, worsened and unchanged) jugular venous pressure is presented. End of study visit data is presented. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Count of Participants | | Participants | | Up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) |
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| Primary | Number of Participants With Abnormal Values for Physical Examination Parameters: Ascites | Physical examination included measurement of ascites. Ascites were assessed as present or absent. Data for ascites present with improved, worsened and unchanged is presented. End of study visit data is presented. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Count of Participants | | Participants | | Up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) | |
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| Primary | Number of Participants With Abnormal Values for Physical Examination Parameter: Peripheral Edema | Physical examination included measurement of peripheral edema. Peripheral edema were assessed as present or absent. Data for peripheral edema present with improved, worsened and unchanged is presented. End of study visit data is presented. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Count of Participants | | Participants | | Up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) | |
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| Primary | Percentage of Saturated Oxygen Level (Physical Examination Parameter) | Physical examination included measurement of saturated oxygen. End of study visit data is presented. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Percentage of oxygen saturation | | Up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) | Participants received ambrisentan 7.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Vital Signs Parameter: Systolic Blood Pressure (SBP) and Diastolic Blood Pressure (DBP) | SBP and DBP was measured for the participants at indicated time points. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Millimeters of mercury | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) |
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| Primary | Change From Baseline in Vital Signs Parameter: Heart Rate | Heart rate was measured for the participants at indicated time points. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Beats per minute | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) |
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| Primary | Change From Baseline in Vital Signs Parameter: Weight | Weight was measured for the participants at indicated time points. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Kilograms | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) | |
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| Primary | Change From Baseline in Vital Sign Parameter: Height | Height was measured for the participants at indicated time points. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Centimeters | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) |
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| Primary | Change From Baseline in Vital Sign Parameter: Body Mass Index | Body mass index was measured for the participants at indicated time points. Body mass index was calculated as weight in kilograms (kg) divided by the square of their height in meters (m^2). Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Kilogram per meter square | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | |
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| Primary | Change From Baseline in Vital Sign Parameter: Body Surface Area | Body surface area was measured for the participants at indicated time points. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Meter square | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) |
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| Primary | Number of Participants With Abnormal Electrocardiogram (ECG) Findings | 12-lead ECG was measured in a semi-supine position using an automated ECG machine. Abnormal findings were categorized as clinically significant (CS) and not clinically significant (NCS). Data for any time till end of study were presented. | | Posted | | Count of Participants | | Participants | | Up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) | Participants received ambrisentan 7.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Female: Follicle Stimulating Hormone (FSH) and Luteinizing Hormone (LH) at End of Study | FSH and LH level of participants were measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | International units per Liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Female: Follicle Stimulating Hormone (FSH) and Luteinizing Hormone (LH) at 20 Years of Age of Participants | FSH and LH level of participants were measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529.Change from Baseline was calculated by subtracting the Baseline value from the specified time point value. Only participants with data at 20 year visit is presented. When participants reached pubertal maturity prior to being 20 years of age then these tests were not repeated at 20-years of age of participants. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | International units per Liter | | Baseline (Day 1) and at 20 years of age of participants | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Female: Inhibin B at End of Study | Inhibin B level of participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. At end of study, participants did not receive Ambrisentan 7.5 mg dose for evaluation of Inhibin B hence N=0 | Posted | | Mean | Standard Deviation | Nanogram per liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Female: Inhibin B at 20 Years of Age of Participants | Inhibin B level of participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529.Change from Baseline was calculated by subtracting the Baseline value from the specified time point value. Only participants with data at 20 year visit is presented. When participants reached pubertal maturity prior to being 20 years of age then these tests were not repeated at 20-years of age of participants. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Nanogram per liter | | Baseline (Day 1) and at 20 years of age of participants | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Female: Sex Hormone Binding Globulin at End of Study | Sex hormone binding globulin level of participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Nanomoles per liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Female: Sex Hormone Binding Globulin at 20 Years of Age of Participants | Sex hormone binding globulin level of participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting Baseline value from the specified time point value. Only participants with data at 20 year visit is presented. When participants reached pubertal maturity prior to being 20 years of age then these tests were not repeated at 20-years of age of participants. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Nanomoles per liter | | Baseline (Day 1) and at 20 years of age of participants | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Female: Estrone at End of Study | Estrone level of female participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Picomole per milliliter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Female: Estrone at 20 Years of Age of Participants | Estrone level of female participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529.Change from Baseline was calculated by subtracting the Baseline value from the specified time point value. Only participants with data at 20 year visit is presented. When participants reached pubertal maturity prior to being 20 years of age then these tests were not repeated at 20-years of age of participants. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Picomole per milliliter | | Baseline (Day 1) and at 20 years of age of participants | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Female: Estriol at End of Study | Estriol level of female participants will be measured. Only those parameters having status as overall will be presented. Baseline is the last value recorded prior to start of study treatment from AMB112529. Change from Baseline is calculated by subtracting the Baseline value from the end of study post-dose visit value. Data for this endpoint will be available for this endpoint by June 2023 | | Not Posted | Jun 2023 | | | | | Baseline (Day 1) and up to 10 years and 11 months | | Participants | | | | |
| Primary | Change From Baseline in Plasma Endocrine Parameters - Female: Estriol at 20 Years of Age of Participants | Estriol level of female participants will be measured. Only those parameters having status as overall will be presented. Baseline is the last value recorded prior to start of study treatment from AMB112529.Change from Baseline is calculated by subtracting the Baseline value from the specified time point value. Only participants with data at 20 year visit is presented. When participants reached pubertal maturity prior to being 20 years of age then these tests were not repeated at 20-years of age of participants. Data for this endpoint will be available for this endpoint by June 2023 | | Not Posted | Jun 2023 | | | | | Baseline (Day 1) and at 20 years of age of participants | | Participants | | | | |
| Primary | Change From Baseline in Plasma Endocrine Parameters - Female: Estradiol at End of Study | Estradiol level of female participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Picomoles per liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Female: Estradiol at 20 Years of Age of Participants | Estradiol level of female participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529.Change from Baseline was calculated by subtracting the Baseline value from the specified time point value. Only participants with data at 20 year visit is presented. When participants reached pubertal maturity prior to being 20 years of age then these tests were not repeated at 20-years of age of participants. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Picomoles per liter | | Baseline (Day 1) and at 20 years of age of participants | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Male: FSH and LH at End of Study | FSH and LH level of participants were measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | International units per Liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Male: FSH and LH at 20 Years of Age of Participants | FSH and LH level of participants were measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529.Change from Baseline was calculated by subtracting the Baseline value from the specified time point value. Only participants with data at 20 year visit is presented. When participants reached pubertal maturity prior to being 20 years of age then these tests were not repeated at 20-years of age of participants. | Safety Population. Only those participants with available data at the specified time points were analyzed. No male participants received Ambrisentan 2.5mg dose at the time of attaining 20 years of age, hence N=0; No endocrinology laboratory tests were performed for 5 mg dose at the 20-year visit, hence N=0. | Posted | | Mean | Standard Deviation | International unit per Liter | | Baseline (Day 1) and at 20 years of age of participants | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Male: Inhibin B at End of Study | Inhibin B level of participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. No participants were analyzed for evaluation of Inhibin B in Ambrisentan 2.5 mg arm, hence N=0 | Posted | | Mean | Standard Deviation | Nanogram per liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Male: Inhibin B at 20 Years of Age of Participants | Inhibin B level of participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529.Change from Baseline was calculated by subtracting the Baseline value from the specified time point value. Only participants with data at 20 year visit is presented. When participants reached pubertal maturity prior to being 20 years of age then these tests were not repeated at 20-years of age of participants. | Safety Population. Only those participants with available data at the specified time points were analyzed. No male participants received Ambrisentan 2.5mg dose at the time of attaining 20 years of age, hence N=0; No endocrinology laboratory tests were performed for 5 mg dose at the 20-year visit, hence N=0. | Posted | | Mean | Standard Deviation | Nanogram per liter | | Baseline (Day 1) and at 20 years of age of participants | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Male: Sex Hormone Binding Globulin at End of Study | Sex hormone binding globulin level of participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. At end of study, no participants were analyzed for evaluation of Sex harmone binding globulin in 2.5 mg dose, hence N=0 | Posted | | Mean | Standard Deviation | Nanomoles per liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Male: Sex Hormone Binding Globulin at 20 Years of Age of Participants | Sex hormone binding globulin level of participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529.Change from Baseline was calculated by subtracting the Baseline value from the specified time point value. Only participants with data at 20 year visit is presented. When participants reached pubertal maturity prior to being 20 years of age then these tests were not repeated at 20-years of age of participants. | Safety Population. Only those participants with available data at the specified time points were analyzed. No male participants received Ambrisentan 2.5mg dose at the time of attaining 20 years of age, hence N=0; No endocrinology laboratory tests were performed for 5 mg dose at the 20-year visit, hence N=0 | Posted | | Mean | Standard Deviation | Nanomoles per liter | | Baseline (Day 1) and at 20 years of age of participants | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Male: Total Testosterone at End of Study | Total Testosterone level of participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Nanomoles per liter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 |
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| Primary | Change From Baseline in Plasma Endocrine Parameters - Male: Total Testosterone at 20 Years of Age of Participants | Total Testosterone level of participants was measured. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529.Change from Baseline was calculated by subtracting the Baseline value from the specified time point value. Only participants with data at 20 year visit is presented. When participants reached pubertal maturity prior to being 20 years of age then these tests were not repeated at 20-years of age of participants. | Safety Population. Only those participants with available data at the specified time points were analyzed. No male participants received Ambrisentan 2.5mg dose at the time of attaining 20 years of age, hence N=0; No endocrinology laboratory tests were performed for 5 mg dose at the 20-year visit, hence N=0. | Posted | | Mean | Standard Deviation | Nanomoles per liter | | Baseline (Day 1) and at 20 years of age of participants | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline of Pubertal Development in Male: Testicular Volume at End of Study | Testicular volume was assessed by Prader's orchiodometer and the assessment was performed by a pediatric endocrinologist using the Tanner's criteria. Only those parameters having status - overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. Data reported for left and right testicular volume. | Safety Population. Only those participants with available data at the specified time points were analyzed. No participants were analyzed for evaluation of testicular volume in 2.5 mg dose, hence N=0 | Posted | | Mean | Standard Deviation | Milliliter | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
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| Primary | Change From Baseline of Pubertal Development in Male: Testicular Volume at 20 Years of Age of Participants | Testicular volume was assessed by Prader's orchiodometer and the assessment was performed by a pediatric endocrinologist using the Tanner's criteria. Only those parameters having status as overall were presented. Baseline was the last value recorded prior to start of study treatment from AMB112529.Change from Baseline was calculated by subtracting the Baseline value from the specified time point value. Only participants with data at 20 year visit is presented. When participants reached pubertal maturity prior to being 20 years of age then these tests were not repeated at 20-years of age of participants. Data reported for left and right testicular volume. | Safety Population. Only those participants with available data at the specified time points were analyzed. No participants were analyzed for evaluation of testicular volume in 2.5 mg dose, hence N=0 | Posted | | Mean | Standard Deviation | Milliliter | | Baseline (Day 1) and at 20 years of age of participants | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | |
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| Primary | Time to Change in Dose of Ambrisentan or Other Targeted PAH Therapeutic Agents (Prostanoids, Phosphodiesterase Type 5 [PDE-5] Inhibitors) Due to Tolerability Issues | Time to change in dose of ambrisentan or other targeted PAH therapeutic agents (prostanoids, Phosphodiesterase type 5 [PDE-5] inhibitors) due to tolerability issues was defined as the time from randomization to the first occurrence of a dose change due to tolerability issues. | Safety Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Days | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 |
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| Secondary | Number of Participants With All-cause Death | Number of participants with all-cause death is presented. | | Posted | | Count of Participants | | Participants | | Up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (Safety) | Participants received ambrisentan 2.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG001 | Ambrisentan 5 mg (Safety) | Participants received ambrisentan 5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). | | OG002 | Ambrisentan 7.5 mg (Safety) | Participants received ambrisentan 7.5 mg tablet orally once daily. The Safety Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to the treatment group according to the highest dose received in the extension study (AMB114588). |
|
| Secondary | Change From Baseline in the 6 Minutes Walking Distance (6MWD) Test | Participant's 6 MWD data has been presented into three categories as overall, with oxygen use and without oxygen use. The 6-minute walk test measures the distance that a participant can walk in 6 minutes. All participants were given standardized instructions and the distance walked was measured. Baseline which is the last value recorded prior to start of study treatment in AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | ITT Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to their treatment group at the start of study AMB114588. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Meters | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
|---|
| OG000 | Ambrisentan 2.5 mg (ITT) | Participants received ambrisentan 2.5 milligrams (mg) dose of ambrisentan orally in tablet/s form once daily. The Intent-to-Treat (ITT) Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. | | OG001 | Ambrisentan 5 mg (ITT) | Participants received 5 mg dose of ambrisenten orally in tablet form once daily. The ITT Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. |
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| Secondary | Time to the First Clinical Worsening of PAH | Time to clinical worsening of PAH is defined as the time from randomization to first occurrence of death (all cause), placed on active list for lung transplant, and/or atrial septostomy, hospitalization due to PAH deterioration, addition of another targeted PAH therapeutic agents (prostanoids, PDE-5 inhibitors) due to deterioration of clinical condition, change in dose of ambrisentan or other targeted PAH therapeutic agents (prostanoids, PDE-5 inhibitors) due to deterioration of clinical condition, PAH related deterioration identified by increase in WHO functional class, deterioration in exercise testing (i.e., 20% decrease in 6MWD on two consecutive tests -1 week apart, clinical signs or symptoms of right sided heart failure (i.e., new peripheral edema, increase in liver size, ascites, increase in jugular venous pressure, pericardial effusion, increased dyspnea). | ITT Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Days | | Up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (ITT) | Participants received ambrisentan 2.5 milligrams (mg) dose of ambrisentan orally in tablet/s form once daily. The Intent-to-Treat (ITT) Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. | | OG001 | Ambrisentan 5 mg (ITT) | |
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| Secondary | Time to the Addition of Another Targeted PAH Therapeutic Agent Due to Deterioration of Clinical Condition | Time to addition of another targeted PAH therapeutics agents due to deterioration of clinical condition was defined as the time from randomization to the first occurrence of deterioration of clinical condition. | ITT Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Days | | Up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (ITT) | Participants received ambrisentan 2.5 milligrams (mg) dose of ambrisentan orally in tablet/s form once daily. The Intent-to-Treat (ITT) Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. | | OG001 | Ambrisentan 5 mg (ITT) | Participants received 5 mg dose of ambrisenten orally in tablet form once daily. The ITT Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. | | OG002 | Ambrisentan 7.5 mg (ITT) | |
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| Secondary | Time to the Addition of Another Targeted PAH Therapeutic Agent Due to Lack of Beneficial Effect With Previous Therapy | The time to addition of another targeted PAH therapeutic agents due to lack of beneficial effect with previous therapy was defined as the time from randomization to the first occurrence of lack of beneficial effect with previous therapy (not reaching set treatment goals). | ITT Population. Only those participants with available data at the specified time points were analyzed. Participants in higher dose group (7.5 and 10 mg) were not taking any additional therapeutic PAH agent hence N=0. | Posted | | Mean | Standard Deviation | Days | | Up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (ITT) | Participants received ambrisentan 2.5 milligrams (mg) dose of ambrisentan orally in tablet/s form once daily. The Intent-to-Treat (ITT) Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. | | OG001 | Ambrisentan 5 mg (ITT) | Participants received 5 mg dose of ambrisenten orally in tablet form once daily. The ITT Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. | |
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| Secondary | Time to Change in Dose of Ambrisentan or Other Targeted PAH Therapeutic Agents (Prostanoids, PDE-5 Inhibitors) Due to Deterioration of Clinical Condition | Time to change in dose of ambrisentan or other targeted PAH therapeutic agents (prostanoids, PDE-5 inhibitors) due to deterioration of clinical condition was defined as the time from randomization to the first occurrence of a dose change due to deterioration of clinical condition. | ITT Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Days | | Up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (ITT) | Participants received ambrisentan 2.5 milligrams (mg) dose of ambrisentan orally in tablet/s form once daily. The Intent-to-Treat (ITT) Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. | | OG001 | Ambrisentan 5 mg (ITT) | Participants received 5 mg dose of ambrisenten orally in tablet form once daily. The ITT Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. | | OG002 | Ambrisentan 7.5 mg (ITT) |
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| Secondary | Change From Baseline in Subject Global Assessment (SF-10) Health Survey for Children | The short-form 10 (SF-10) Health Survey for children is a 10-item, 4-week recall, parent-completed health assessment that measures physical and psychosocial functioning for children ages five and over. Two summary scores were calculated: a Physical Summary Score (PHS) and a Psychosocial Summary Score (PSS) with a range of 5 to 30 points for each 5-item score. The aggregate score was then standardized and transformed to a norm-based scoring metric in accordance with the developer's guidelines. This generated the final standardized norm-based scores for PHS (range -10.90 to 57.21) and for PSS (range 8.81 to 62.28), respectively. A higher value on each summary score indicates better functioning. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | ITT Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Mean | Standard Deviation | Scores on a scale | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (ITT) | Participants received ambrisentan 2.5 milligrams (mg) dose of ambrisentan orally in tablet/s form once daily. The Intent-to-Treat (ITT) Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. | | OG001 |
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| Secondary | Number of Participants With Change From Baseline in World Health Organization (WHO) Functional Class of PAH | PAH was classified by WHO functional class (FC) at specific time points. There were four WHO FC grades based on severity of PAH symptoms (Class I=none, Class IV=most severe). Grades were mapped to numeric scale for which scores ranged from 1-4 (i.e. Class I=1 and IV=4). Change categorization was based on change from Baseline scores: -2, -1, 0, +1, +2. Data was categorized as No Change (0), Improved (-1,-2), Deteriorated (+1,+2). Baseline was the last value recorded prior to start of study treatment from AMB112529. Higher score indicated higher severity.Change from Baseline was calculated by subtracting the Baseline value from the end of study post-dose visit value. | ITT Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Count of Participants | | Participants | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (ITT) | Participants received ambrisentan 2.5 milligrams (mg) dose of ambrisentan orally in tablet/s form once daily. The Intent-to-Treat (ITT) Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. | | OG001 | Ambrisentan 5 mg (ITT) | Participants received 5 mg dose of ambrisenten orally in tablet form once daily. The ITT Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. |
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| Secondary | Percentage Change From Baseline in Plasma N-terminal Pro-B-type Natriuretic Peptide (NT-Pro BNP) Concentration | Blood samples were collected to analyze NT-Pro BNP concentration at specific time points. Baseline was the last value recorded prior to start of study treatment from AMB112529. Change from Baseline was calculated by subtracting Baseline value from the specified time point value. | ITT Population. Only those participants with available data at the specified time points were analyzed. | Posted | | Geometric Mean | Full Range | Percentage Change | | Baseline (Day 1) and up to 10 years and 11 months | | | | ID | Title | Description |
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| OG000 | Ambrisentan 2.5 mg (ITT) | Participants received ambrisentan 2.5 milligrams (mg) dose of ambrisentan orally in tablet/s form once daily. The Intent-to-Treat (ITT) Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. | | OG001 | Ambrisentan 5 mg (ITT) | Participants received 5 mg dose of ambrisenten orally in tablet form once daily. The ITT Population consisted of all participants who received at least 1 dose of study drug. Participants were considered as belonging to ITT treatment group at the start of study AMB114588. | | OG002 | Ambrisentan 7.5 mg (ITT) |
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