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Trial record 23 of 66 for:    "Lung Disease" | "Bosentan"

Bosentan in Children With Pulmonary Arterial Hypertension Extension Study (FUTURE 2)

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details.
 
ClinicalTrials.gov Identifier: NCT00319020
Recruitment Status : Completed
First Posted : April 27, 2006
Results First Posted : March 29, 2017
Last Update Posted : June 14, 2017
Sponsor:
Information provided by (Responsible Party):
Actelion

Study Type Interventional
Study Design Intervention Model: Single Group Assignment;   Masking: None (Open Label);   Primary Purpose: Treatment
Condition Pulmonary Arterial Hypertension
Intervention Drug: Bosentan
Enrollment 33
Recruitment Details 36 Children ( >= 2 years and < 12 years) with idiopathic or familial pulmonary arterial hypertension were recruited from 11 centers across Europe and USA and enrolled in the FUTURE 1 trial (baseline). Only patients who completed FUTURE 1 (n=34) could be enrolled in FUTURE 2. Enrollment in FUTURE 2 started August 23, 2005.
Pre-assignment Details The actual number of patients enrolled in FUTURE 2 (F-2) was 33 because 2 patients did not complete FUTURE 1 (F-1) and one patient completed F-1 but was not enrolled in F-2.
Arm/Group Title Patients With Previous Bosentan Bosentan-naive Patients
Hide Arm/Group Description

This group included patients who already received bosentan (film-coated tablets) before enrollment in FUTURE 1, and then received the pediatric formulation of bosentan (dispersible tablets) during FUTURE 1 and FUTURE 2 (initiatied at 2 mg/kg b.i.d. for 4 weeks, then up-titrated to the maintenance dose of 4 mg/kg b.i.d. for the next 8 weeks of the FUTURE 1 trial (AC-052- 365) and to be continued in FUTURE 2. The dose could be down-titrated to 2 mg/kg b.i.d. if not tolerated).

Note: In this single arm trial, data are presented according to whether patients received bosentan or not before enrollment in FUTURE 1 but all the subjects received the study drug according to the same regimen.

This group included patients who were not treated with bosentan before enrollment in FUTURE 1, and received the pediatric formulation of bosentan (dispersible tablets) during FUTURE 1 and FUTURE 2 according to the same dosing regimen as described for "Patients with previous bosentan".

Note: In this single arm trial, data are presented according to whether patients received bosentan or not before enrollment in FUTURE 1 but all the subjects received the study drug according to the same regimen.

Period Title: Overall Study
Started 15 21
Enrollment in FUTURE 2 (F-2) 13 20
Completed 8 8
Not Completed 7 13
Reason Not Completed
Death             2             2
Withdrawal by Subject             4             1
F-1 completed but not enrolled in F-2             1             0
Administrative reason             0             5
Disease progression             0             2
Transplant             0             1
Treatment failure             0             1
Adverse Event             0             1
Arm/Group Title Patients With Previous Bosentan Bosentan-naive Patients Total
Hide Arm/Group Description This group included patients who already received bosentan (film-coated tablets) before enrollment in FUTURE 1, and then received the pediatric formulation of bosentan (dispersible tablets) during FUTURE 1 and FUTURE 2 (initiation at 2 mg/kg b.i.d. for 4 weeks, then up-titrated to the maintenance dose of 4 mg/kg b.i.d. for the next 8 weeks of the FUTURE 1 trial (AC-052- 365) and to be continued in FUTURE 2. The dose could be down-titrated to 2 mg/kg b.i.d. if not tolerated).

This group included patients who were not treated with bosentan before enrollment in FUTURE 1, and received the pediatric formulation of bosentan (dispersible tablets) during FUTURE 1 and FUTURE 2 according to the same dosing regimen as described for "Patients with previous bosentan".

Note: In this single arm trial, data are presented according to whether patients received bosentan or not before enrollment in FUTURE 1 but all the subjects received the study drug according to the same regimen.

Total of all reporting groups
Overall Number of Baseline Participants 15 21 36
Hide Baseline Analysis Population Description
[Not Specified]
Age, Continuous  
Median (Full Range)
Unit of measure:  Years
Number Analyzed 15 participants 21 participants 36 participants
7
(3 to 10)
7
(2 to 11)
7
(2 to 11)
Age, Customized  
Measure Type: Number
Unit of measure:  Participants
Number Analyzed 15 participants 21 participants 36 participants
2-3 years old 1 3 4
4-5 years old 3 6 9
6-11 years old 11 12 23
Sex: Female, Male  
Measure Type: Count of Participants
Unit of measure:  Participants
Number Analyzed 15 participants 21 participants 36 participants
Female
5
  33.3%
10
  47.6%
15
  41.7%
Male
10
  66.7%
11
  52.4%
21
  58.3%
Etiology of pulmonary arterial hypertension (PAH)   [1] 
Measure Type: Number
Unit of measure:  Participants
Number Analyzed 15 participants 21 participants 36 participants
Idiopathic PAH 12 19 31
Familial PAH 3 2 5
[1]
Measure Description: Children included in the study had a diagnosis of pulmonary arterial hypertension (PAH) belonging to one of the following two categories: 1- Idiopathic PAH, 2- Familal PAH
Duration of pulmonary arterial hypertension (PAH)  
Median (Full Range)
Unit of measure:  Months
Number Analyzed 15 participants 21 participants 36 participants
37.6
(1.2 to 82.6)
14
(0 to 133.5)
25.8
(0 to 133.5)
1.Primary Outcome
Title Change From Baseline to End of Study (EOS) in Height for Age.
Hide Description

In order to compare the growth data with those of healthy children, growth curves are calculated from height data collected throughout the follow-up period. For each patient, height measured at each study visit was converted to a z-score and expressed in standard deviations (SD) from WHO growth standards. The Z-score was calculated according to the following formula:

Z-score = (observed value of the study participant - median value of the reference population) / SD value of the reference population

Time Frame From baseline (FUTURE 1) up to 28 days after study treatment discontinuation (EOS or premature study treatment discontinuation), i.e. 32 months in average
Hide Outcome Measure Data
Hide Analysis Population Description
All-treated analysis set (all patients who received at least one dose of study drug in the combined FUTURE 1 / FUTURE 2 trial periods). Missing or incomplete data were treated as missing.
Arm/Group Title Patients With Previous Bosentan Bosentan-naive Patients Total
Hide Arm/Group Description:
This group included patients who already received bosentan (film-coated tablets) before enrollment in FUTURE 1, and then received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 (initiation at 2 mg/kg b.i.d. for 4 weeks, then up-titrated to the maintenance dose of 4 mg/kg b.i.d. for the next 8 weeks of the FUTURE 1 trial (AC-052- 365) and to be continued in FUTURE 2. The dose could be down-titrated to 2 mg/kg b.i.d. if not tolerated).

This group included patients who were not treated with bosentan before enrollment in FUTURE 1, and received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 according to the same dosing regimen as described for "Patients with previous bosentan".

Note: In this single arm trial, data are presented according to whether patients received bosentan or not before enrollment in FUTURE 1 but all the subjects received the study drug according to the same regimen.

All patients (bosentan-naive patients and patients treated with film-coated bosentan tablets before enrollment) who received at least one dose of study drug (bosentan dispersible tablets) in the combined FUTURE 1 / FUTURE 2 trial periods.
Overall Number of Participants Analyzed 10 14 24
Median (Full Range)
Unit of Measure: Z-score
Z-score at baseline
-0.8
(-3.32 to 3.72)
0.32
(-2.62 to 1.99)
-0.64
(-3.32 to 3.72)
Z-score at EOS
-0.74
(-3.52 to 2.78)
-0.08
(-2.44 to 1.84)
-0.36
(-3.52 to 2.78)
Z-score change from baseline to EOS
-0.05
(-0.94 to 0.91)
-0.01
(-0.77 to 1.08)
-0.01
(-0.94 to 1.08)
2.Primary Outcome
Title Change From Baseline to End of Study (EOS) in Body Weight
Hide Description The main study objective was to assess the long-term safety and tolerability of bosentan in children with PAH, including growth as measured by changes from baseline in body weight and height.
Time Frame From baseline (FUTURE 1) up to 28 days after study treatment discontinuation (EOS or premature study treatment discontinuation), i.e. 32 months in average
Hide Outcome Measure Data
Hide Analysis Population Description
All-treated analysis set (all patients who received at least one dose of study drug in the combined FUTURE 1 / FUTURE 2 trial periods). Missing or incomplete data were treated as missing.
Arm/Group Title Patients With Previous Bosentan Bosentan-naive Patients Total
Hide Arm/Group Description:
This group included patients who already received bosentan (film-coated tablets) before enrollment in FUTURE 1, and then received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 (initiation at 2 mg/kg b.i.d. for 4 weeks, then up-titrated to the maintenance dose of 4 mg/kg b.i.d. for the next 8 weeks of the FUTURE 1 trial (AC-052- 365) and to be continued in FUTURE 2. The dose could be down-titrated to 2 mg/kg b.i.d. if not tolerated).

This group included patients who were not treated with bosentan before enrollment in FUTURE 1, and received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 according to the same dosing regimen as described for "Patients with previous bosentan".

Note: In this single arm trial, data are presented according to whether patients received bosentan or not before enrollment in FUTURE 1 but all the subjects received the study drug according to the same regimen.

All patients (bosentan-naive patients and patients treated with film-coated bosentan tablets before enrollment) who received at least one dose of study drug (bosentan dispersible tablets) in the combined FUTURE 1 / FUTURE 2 trial periods.
Overall Number of Participants Analyzed 10 14 24
Median (Full Range)
Unit of Measure: kg
Weight at baseline
19.6
(12.5 to 30.2)
21.6
(11 to 39)
19.6
(11 to 39)
Weight change from baseline to EOS
8.2
(5 to 24.8)
8.5
(1.8 to 18.5)
8.3
(1.8 to 24.8)
3.Primary Outcome
Title Change From Baseline to End of Study (EOS) in Systolic Blood Pressure (SBP)
Hide Description The main study objective was to assess the long-term safety and tolerability of bosentan in children with PAH, including changes from baseline in blood pressure.
Time Frame From baseline (FUTURE 1) up to 28 days after study treatment discontinuation (EOS or premature study treatment discontinuation), i.e. 32 months in average
Hide Outcome Measure Data
Hide Analysis Population Description
All-treated analysis set (all patients who received at least one dose of study drug in the combined FUTURE 1 / FUTURE 2 trial periods). Missing or incomplete data were treated as missing.
Arm/Group Title Patients With Previous Bosentan Bosentan-naive Patients Total
Hide Arm/Group Description:
This group included patients who already received bosentan (film-coated tablets) before enrollment in FUTURE 1, and then received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 (initiation at 2 mg/kg b.i.d. for 4 weeks, then up-titrated to the maintenance dose of 4 mg/kg b.i.d. for the next 8 weeks of the FUTURE 1 trial (AC-052- 365) and to be continued in FUTURE 2. The dose could be down-titrated to 2 mg/kg b.i.d. if not tolerated).

This group included patients who were not treated with bosentan before enrollment in FUTURE 1, and received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 according to the same dosing regimen as described for "Patients with previous bosentan".

Note: In this single arm trial, data are presented according to whether patients received bosentan or not before enrollment in FUTURE 1 but all the subjects received the study drug according to the same regimen.

All patients (bosentan-naive patients and patients treated with film-coated bosentan tablets before enrollment) who received at least one dose of study drug (bosentan dispersible tablets) in the combined FUTURE 1 / FUTURE 2 trial periods.
Overall Number of Participants Analyzed 10 14 24
Median (Full Range)
Unit of Measure: mmHg
SBP at baseline
101.5
(87 to 115)
104
(79 to 121)
102.5
(79 to 121)
SBP change from baseline to EOS
-10.5
(-20 to 25)
4
(-21 to 28)
-4.5
(-21 to 28)
4.Primary Outcome
Title Change From Baseline to End of Study (EOS) in Diastolic Blood Pressure (DBP)
Hide Description The main study objective was to assess the long-term safety and tolerability of bosentan in children with PAH, including changes from baseline in blood pressure.
Time Frame From baseline (FUTURE 1) up to 28 days after study treatment discontinuation (EOS or premature study treatment discontinuation), i.e. 32 months in average
Hide Outcome Measure Data
Hide Analysis Population Description
All-treated analysis set (all patients who received at least one dose of study drug in the combined FUTURE 1 / FUTURE 2 trial periods). Missing or incomplete data were treated as missing.
Arm/Group Title Patients With Previous Bosentan Bosentan-naive Patients Total
Hide Arm/Group Description:
This group included patients who already received bosentan (film-coated tablets) before enrollment in FUTURE 1, and then received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 (initiation at 2 mg/kg b.i.d. for 4 weeks, then up-titrated to the maintenance dose of 4 mg/kg b.i.d. for the next 8 weeks of the FUTURE 1 trial (AC-052- 365) and to be continued in FUTURE 2. The dose could be down-titrated to 2 mg/kg b.i.d. if not tolerated).

This group included patients who were not treated with bosentan before enrollment in FUTURE 1, and received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 according to the same dosing regimen as described for "Patients with previous bosentan".

Note: In this single arm trial, data are presented according to whether patients received bosentan or not before enrollment in FUTURE 1 but all the subjects received the study drug according to the same regimen.

All patients (bosentan-naive patients and patients treated with film-coated bosentan tablets before enrollment) who received at least one dose of study drug (bosentan dispersible tablets) in the combined FUTURE 1 / FUTURE 2 trial periods.
Overall Number of Participants Analyzed 10 13 23
Median (Full Range)
Unit of Measure: mmHg
DBP at baseline
54.5
(47 to 94)
60
(52 to 75)
59
(47 to 94)
DBP change from baseline to EOS
-5
(-34 to 19)
-2
(-13 to 20)
-3
(-34 to 20)
5.Primary Outcome
Title Change From Baseline to End of Study (EOS) in Pulse Rate
Hide Description The main study objective was to assess the long-term safety and tolerability of bosentan in children with PAH, including changes from baseline in pulse rate.
Time Frame From baseline (FUTURE 1) up to 28 days after study treatment discontinuation (EOS or premature study treatment discontinuation), i.e. 32 months in average
Hide Outcome Measure Data
Hide Analysis Population Description
All-treated analysis set (all patients who received at least one dose of study drug in the combined FUTURE 1 / FUTURE 2 trial periods). Missing or incomplete data were treated as missing.
Arm/Group Title Patients With Previous Bosentan Bosentan-naive Patients Total
Hide Arm/Group Description:
This group included patients who already received bosentan (film-coated tablets) before enrollment in FUTURE 1, and then received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 (initiation at 2 mg/kg b.i.d. for 4 weeks, then up-titrated to the maintenance dose of 4 mg/kg b.i.d. for the next 8 weeks of the FUTURE 1 trial (AC-052- 365) and to be continued in FUTURE 2. The dose could be down-titrated to 2 mg/kg b.i.d. if not tolerated).

This group included patients who were not treated with bosentan before enrollment in FUTURE 1, and received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 according to the same dosing regimen as described for "Patients with previous bosentan".

Note: In this single arm trial, data are presented according to whether patients received bosentan or not before enrollment in FUTURE 1 but all the subjects received the study drug according to the same regimen.

All patients (bosentan-naive patients and patients treated with film-coated bosentan tablets before enrollment) who received at least one dose of study drug (bosentan dispersible tablets) in the combined FUTURE 1 / FUTURE 2 trial periods.
Overall Number of Participants Analyzed 11 14 25
Median (Full Range)
Unit of Measure: Beats per minutes
Pulse rate at baseline
87
(55 to 118)
94.5
(62 to 133)
88
(55 to 133)
Pulse rate change from baseline to EOS
-11
(-46 to 36)
-10
(-30 to 11)
-11
(-46 to 36)
6.Primary Outcome
Title Proportion of Patients With Treatment-emergent Liver Function Abnormalities
Hide Description

The main study objective was to assess the long-term safety and tolerability of bosentan in children with PAH, including laboratory abnormalities related to liver enzymes.

Proportion of patients with increase in alanine aminotransferase (ALT) or aspartate aminotransferase (AST) above 3 times upper limit of normal (ULN) is reported here.

Time Frame After baseline, up to 1 calendar day after study treatment discontinuation in FUTURE 1 or FUTURE 2, i.e. 31 months in average
Hide Outcome Measure Data
Hide Analysis Population Description
All-treated analysis set (all patients who received at least one dose of study drug in the combined FUTURE 1 / FUTURE 2 trial periods). Missing or incomplete data were treated as missing.
Arm/Group Title Patients With Previous Bosentan Bosentan-naive Patients Total
Hide Arm/Group Description:
This group included patients who already received bosentan (film-coated tablets) before enrollment in FUTURE 1, and then received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 (initiation at 2 mg/kg b.i.d. for 4 weeks, then up-titrated to the maintenance dose of 4 mg/kg b.i.d. for the next 8 weeks of the FUTURE 1 trial (AC-052- 365) and to be continued in FUTURE 2. The dose could be down-titrated to 2 mg/kg b.i.d. if not tolerated).

This group included patients who were not treated with bosentan before enrollment in FUTURE 1, and received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 according to the same dosing regimen as described for "Patients with previous bosentan".

Note: In this single arm trial, data are presented according to whether patients received bosentan or not before enrollment in FUTURE 1 but all the subjects received the study drug according to the same regimen.

All patients (bosentan-naive patients and patients treated with film-coated bosentan tablets before enrollment) who received at least one dose of study drug (bosentan dispersible tablets) in the combined FUTURE 1 / FUTURE 2 trial periods.
Overall Number of Participants Analyzed 15 21 36
Measure Type: Number
Unit of Measure: Percentage of participants
ALT > 3 x ULN 0 4.8 2.8
AST > 3 x ULN 0 4.8 2.8
7.Primary Outcome
Title Proportion of Patients With Treatment-emergent Hemoglobin Abnormalities
Hide Description

The main study objective was to assess the long-term safety and tolerability of bosentan in children with PAH, including hemoglobin abnormalities.

Proportion of patients with marked hemoglobin decreases (i.e., decrease of or above 15% of the lower normal limit (LL)) is reported here.

Time Frame After baseline, up to 1 calendar day after study treatment discontinuation in FUTURE 1 or FUTURE 2, i.e. 31 months in average
Hide Outcome Measure Data
Hide Analysis Population Description
All-treated analysis set (all patients who received at least one dose of study drug in the combined FUTURE 1 / FUTURE 2 trial periods). Missing or incomplete data were treated as missing.
Arm/Group Title Patients With Previous Bosentan Bosentan-naive Patients Total
Hide Arm/Group Description:
This group included patients who already received bosentan (film-coated tablets) before enrollment in FUTURE 1, and then received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 (initiation at 2 mg/kg b.i.d. for 4 weeks, then up-titrated to the maintenance dose of 4 mg/kg b.i.d. for the next 8 weeks of the FUTURE 1 trial (AC-052- 365) and to be continued in FUTURE 2. The dose could be down-titrated to 2 mg/kg b.i.d. if not tolerated).

This group included patients who were not treated with bosentan before enrollment in FUTURE 1, and received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 according to the same dosing regimen as described for "Patients with previous bosentan".

Note: In this single arm trial, data are presented according to whether patients received bosentan or not before enrollment in FUTURE 1 but all the subjects received the study drug according to the same regimen.

All patients (bosentan-naive patients and patients treated with film-coated bosentan tablets before enrollment) who received at least one dose of study drug (bosentan dispersible tablets) in the combined FUTURE 1 / FUTURE 2 trial periods.
Overall Number of Participants Analyzed 15 21 36
Measure Type: Number
Unit of Measure: Percentage of participants
13.3 9.5 11.1
8.Primary Outcome
Title Number of Subjects With Adverse Events Leading to Premature Discontinuation of Study Treatment
Hide Description [Not Specified]
Time Frame From the first study drug administration in FUTURE 1, for an average of 31 months
Hide Outcome Measure Data
Hide Analysis Population Description
[Not Specified]
Arm/Group Title Patients With Previous Bosentan Bosentan-naive Patients Total
Hide Arm/Group Description:
This group included patients who already received bosentan (film-coated tablets) before enrollment in FUTURE 1, and then received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 (initiation at 2 mg/kg b.i.d. for 4 weeks, then up-titrated to the maintenance dose of 4 mg/kg b.i.d. for the next 8 weeks of the FUTURE 1 trial (AC-052- 365) and to be continued in FUTURE 2. The dose could be down-titrated to 2 mg/kg b.i.d. if not tolerated).

This group included patients who were not treated with bosentan before enrollment in FUTURE 1, and received the pediatric formulation of bosentan (dispersible tablet) during FUTURE 1 and FUTURE 2 according to the same dosing regimen as described for "Patients with previous bosentan".

Note: In this single arm trial, data are presented according to whether patients received bosentan or not before enrollment in FUTURE 1 but all the subjects received the study drug according to the same regimen.

All patients (bosentan-naive patients and patients treated with film-coated bosentan tablets before enrollment) who received at least one dose of study drug (bosentan dispersible tablets) in the combined FUTURE 1 / FUTURE 2 trial periods.
Overall Number of Participants Analyzed 15 21 36
Measure Type: Number
Unit of Measure: Participants
1 5 6
Time Frame From the first administration of study treatment and for an average of 31 months for frequent adverse events (up to 1 day after study treatment discontinuation), and for an average of 32 months (up to 28 days after study treatment discontinuation) for serious adverse events
Adverse Event Reporting Description [Not Specified]
 
Arm/Group Title Patients With Previous Bosentan Bosentan_naive Patients Single Arm Bosentan_Total
Hide Arm/Group Description Patients who already received bosentan (film-coated tablets) before enrollment in FUTURE 1, and then received the pediatric formulation of bosentan during FUTURE 1 / FUTURE 2 Patients who were not treated with bosentan before enrollment in FUTURE 1, and received the pediatric formulation of bosentan during FUTURE 1 / FUTURE 2 All patients included in Future 1 / FUTURE 2 whether they received bosentan or not bosentan before enrollment in FUTURE 1
All-Cause Mortality
Patients With Previous Bosentan Bosentan_naive Patients Single Arm Bosentan_Total
Affected / at Risk (%) Affected / at Risk (%) Affected / at Risk (%)
Total   --/--      --/--      --/--    
Show Serious Adverse Events Hide Serious Adverse Events
Patients With Previous Bosentan Bosentan_naive Patients Single Arm Bosentan_Total
Affected / at Risk (%) # Events Affected / at Risk (%) # Events Affected / at Risk (%) # Events
Total   9/15 (60.00%)      9/21 (42.86%)      18/36 (50.00%)    
Blood and lymphatic system disorders       
IRON DEFICIENCY ANAEMIA  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
Cardiac disorders       
CARDIAC FAILURE  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
PERICARDIAL EFFUSION  1  1/15 (6.67%)  2 0/21 (0.00%)  0 1/36 (2.78%)  2
RIGHT VENTRICULAR FAILURE  1  1/15 (6.67%)  1 1/21 (4.76%)  1 2/36 (5.56%)  2
Gastrointestinal disorders       
ABDOMINAL PAIN  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
General disorders       
CHEST PAIN  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
FATIGUE  1  1/15 (6.67%)  1 1/21 (4.76%)  1 2/36 (5.56%)  2
INJECTION SITE NODULE  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
MEDICAL DEVICE COMPLICATION  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
Hepatobiliary disorders       
AUTOIMMUNE HEPATITIS  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
Infections and infestations       
BACTERAEMIA  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
BRONCHITIS VIRAL  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
CATHETER SITE INFECTION  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
CELLULITIS  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
DEVICE RELATED INFECTION  1  1/15 (6.67%)  1 2/21 (9.52%)  6 3/36 (8.33%)  7
EAR INFECTION  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
LOBAR PNEUMONIA  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
LUNG INFECTION  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
PNEUMONIA  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
PNEUMONIA VIRAL  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
VIRAL INFECTION  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
VIRAL RHINITIS  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
Investigations       
ARTERIAL CATHETERISATION  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
CATHETERISATION CARDIAC  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
HAEMOGLOBIN DECREASED  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
PULMONARY ARTERIAL PRESSURE  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
Musculoskeletal and connective tissue disorders       
FLANK PAIN  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
Nervous system disorders       
CONVULSION  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
DYSTONIA  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
SYNCOPE  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
Respiratory, thoracic and mediastinal disorders       
BRONCHIAL OBSTRUCTION  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
COUGH  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
DIAPHRAGMATIC HERNIA  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
PULMONARY ARTERIAL HYPERTENSION  1  2/15 (13.33%)  3 1/21 (4.76%)  1 3/36 (8.33%)  4
PULMONARY HYPERTENSION  1  1/15 (6.67%)  1 2/21 (9.52%)  2 3/36 (8.33%)  3
PULMONARY VEIN STENOSIS  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
RESPIRATORY FAILURE  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
WHEEZING  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
Surgical and medical procedures       
ADENOIDECTOMY  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
BALLOON ATRIAL SEPTOSTOMY  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
SYSTEMIC-PULMONARY ARTERY SHUNT  1  1/15 (6.67%)  1 1/21 (4.76%)  1 2/36 (5.56%)  2
Vascular disorders       
HYPERTENSION  1  0/15 (0.00%)  0 1/21 (4.76%)  1 1/36 (2.78%)  1
Indicates events were collected by systematic assessment
1
Term from vocabulary, MedDRA
Show Other (Not Including Serious) Adverse Events Hide Other (Not Including Serious) Adverse Events
Frequency Threshold for Reporting Other Adverse Events 5%
Patients With Previous Bosentan Bosentan_naive Patients Single Arm Bosentan_Total
Affected / at Risk (%) # Events Affected / at Risk (%) # Events Affected / at Risk (%) # Events
Total   12/15 (80.00%)      16/21 (76.19%)      26/36 (72.22%)    
Blood and lymphatic system disorders       
ANAEMIA  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
Cardiac disorders       
CYANOSIS  1  1/15 (6.67%)  1 1/21 (4.76%)  1 2/36 (5.56%)  2
PALPITATIONS  1  0/15 (0.00%)  0 2/21 (9.52%)  4 2/36 (5.56%)  4
Ear and labyrinth disorders       
EAR PAIN  1  0/15 (0.00%)  0 2/21 (9.52%)  3 2/36 (5.56%)  3
Eye disorders       
PHOTOPHOBIA  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
VISION BLURRED  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
Gastrointestinal disorders       
ABDOMINAL PAIN  1  1/15 (6.67%)  1 5/21 (23.81%)  6 6/36 (16.67%)  7
ABDOMINAL PAIN UPPER  1  0/15 (0.00%)  0 3/21 (14.29%)  4 3/36 (8.33%)  4
CONSTIPATION  1  1/15 (6.67%)  1 1/21 (4.76%)  1 2/36 (5.56%)  2
DIARRHOEA  1  2/15 (13.33%)  2 1/21 (4.76%)  1 3/36 (8.33%)  3
NAUSEA  1  1/15 (6.67%)  1 1/21 (4.76%)  1 2/36 (5.56%)  2
VOMITING  1  0/15 (0.00%)  0 4/21 (19.05%)  4 4/36 (11.11%)  4
DYSPEPSIA  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
TOOTH DISCOLOURATION  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
General disorders       
ADVERSE DRUG REACTION  1  1/15 (6.67%)  1 1/21 (4.76%)  2 2/36 (5.56%)  3
ASTHENIA  1  1/15 (6.67%)  1 2/21 (9.52%)  3 3/36 (8.33%)  4
CHEST PAIN  1  0/15 (0.00%)  0 3/21 (14.29%)  10 3/36 (8.33%)  10
FATIGUE  1  0/15 (0.00%)  0 2/21 (9.52%)  3 2/36 (5.56%)  3
PYREXIA  1  1/15 (6.67%)  1 2/21 (9.52%)  2 3/36 (8.33%)  3
CATHETER SITE PAIN  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
Immune system disorders       
ALLERGY TO PLANTS  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
Infections and infestations       
BRONCHITIS  1  2/15 (13.33%)  2 3/21 (14.29%)  4 5/36 (13.89%)  6
H1N1 INFLUENZA  1  2/15 (13.33%)  2 0/21 (0.00%)  0 2/36 (5.56%)  2
INFLUENZA  1  1/15 (6.67%)  1 1/21 (4.76%)  1 2/36 (5.56%)  2
NASOPHARYNGITIS  1  3/15 (20.00%)  5 4/21 (19.05%)  6 7/36 (19.44%)  11
OTITIS MEDIA  1  1/15 (6.67%)  1 1/21 (4.76%)  1 2/36 (5.56%)  2
PHARYNGITIS  1  1/15 (6.67%)  1 1/21 (4.76%)  1 2/36 (5.56%)  2
PNEUMONIA  1  1/15 (6.67%)  5 2/21 (9.52%)  2 3/36 (8.33%)  7
TONSILLITIS  1  1/15 (6.67%)  2 1/21 (4.76%)  1 2/36 (5.56%)  3
UPPER RESPIRATORY TRACT INFECTION  1  1/15 (6.67%)  1 4/21 (19.05%)  6 5/36 (13.89%)  7
VIRAL INFECTION  1  0/15 (0.00%)  0 2/21 (9.52%)  2 2/36 (5.56%)  2
GASTROINTESTINAL VIRAL INFECTION  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
PERTUSSIS  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
URINARY TRACT INFECTION  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
Injury, poisoning and procedural complications       
CONTUSION  1  0/15 (0.00%)  0 2/21 (9.52%)  2 2/36 (5.56%)  2
Musculoskeletal and connective tissue disorders       
PAIN IN EXTREMITY  1  0/15 (0.00%)  0 2/21 (9.52%)  2 2/36 (5.56%)  2
MUSCULOSKELETAL PAIN  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
Nervous system disorders       
DIZZINESS  1  1/15 (6.67%)  1 2/21 (9.52%)  3 3/36 (8.33%)  4
HEADACHE  1  1/15 (6.67%)  1 3/21 (14.29%)  6 4/36 (11.11%)  7
SYNCOPE  1  2/15 (13.33%)  2 1/21 (4.76%)  4 3/36 (8.33%)  6
Psychiatric disorders       
AGGRESSION  1  0/15 (0.00%)  0 2/21 (9.52%)  2 2/36 (5.56%)  2
Renal and urinary disorders       
ENURESIS  1  0/15 (0.00%)  0 2/21 (9.52%)  2 2/36 (5.56%)  2
Reproductive system and breast disorders       
NIPPLE SWELLING  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
Respiratory, thoracic and mediastinal disorders       
COUGH  1  1/15 (6.67%)  3 1/21 (4.76%)  1 2/36 (5.56%)  4
EPISTAXIS  1  1/15 (6.67%)  1 1/21 (4.76%)  1 2/36 (5.56%)  2
NASAL CONGESTION  1  0/15 (0.00%)  0 3/21 (14.29%)  4 3/36 (8.33%)  4
PULMONARY ARTERIAL HYPERTENSION  1  3/15 (20.00%)  3 1/21 (4.76%)  1 4/36 (11.11%)  4
PULMONARY HYPERTENSION  1  0/15 (0.00%)  0 3/21 (14.29%)  3 3/36 (8.33%)  3
OROPHARYNGEAL PAIN  1  1/15 (6.67%)  2 0/21 (0.00%)  0 1/36 (2.78%)  2
Skin and subcutaneous tissue disorders       
ECZEMA  1  1/15 (6.67%)  1 0/21 (0.00%)  0 1/36 (2.78%)  1
Vascular disorders       
FLUSHING  1  1/15 (6.67%)  1 3/21 (14.29%)  4 4/36 (11.11%)  5
Indicates events were collected by systematic assessment
1
Term from vocabulary, MedDRA
Certain Agreements
Principal Investigators are NOT employed by the organization sponsoring the study.
There IS an agreement between Principal Investigators and the Sponsor (or its agents) that restricts the PI's rights to discuss or publish trial results after the trial is completed.
Any study-related article or abstract written independently by investigators should be submitted to Actelion for review at least 60 days prior to submission for publication or presentation.
Results Point of Contact
Layout table for Results Point of Contact information
Name/Title: clinical trial disclosure desk
Organization: Actelion Pharmaceuticals Ltd
EMail: clinical-trials-disclosure@actelion.com
Layout table for additonal information
Responsible Party: Actelion
ClinicalTrials.gov Identifier: NCT00319020     History of Changes
Other Study ID Numbers: AC-052-367
2005-001967-70 ( EudraCT Number )
First Submitted: April 26, 2006
First Posted: April 27, 2006
Results First Submitted: February 9, 2017
Results First Posted: March 29, 2017
Last Update Posted: June 14, 2017