A Multicenter, Long-Term Extension Study to Assess Safety of Oral Sildenafil Citrate In The Treatment Of Subjects Who Have Completed Study A1481131

Number of Deaths Reported in the Study Prior to the Data Monitoring Committee (DMC) Recommendation of Dose Down Titration

时间窗: Pre-DMC Recommendation dose down titration (04 August 2011)

Deaths were reported immediately independent of the circumstances or suspected cause at any time during the study through the last follow-up visit or 30 days after the last administration of study drug, whichever comes later.

Downtitration in Dose Due to Intolerability.

时间窗: Pre-DMC recomendation (04 August 2011)

Based on review of the survival data, DMC concluded that the high dose of sildenafil was associated with a harmful effect on survival when compared to the low dose. The DMC also expressed concern as to the potential dose-response relationship between increasing dose and mortality. Therefore, on 04 August 2011, the DMC recommended discontinuation of the 40 mg and 80 mg three times a day (TID) doses, as well as the 20 mg TID dose in children with body weight ≤20 kg. The protocol was amended per DMC recommendations.

Pediatric Cognitive Development Status at Week 52.

时间窗: Week 52

Participant's cognitive development status was assessed at Week 52 using the physician assessment questions. Assessment question (i.e., compared to other children the participant's age group is this participant's cognitive development limited?) included the following criteria : severely limited, moderately limited, mildly limited and not limited.

Number of Participants Reporting at Least One Serious Adverse Event

时间窗: Up to Follow-Up visit (30 to 40 days after study completion or treatment discontinuation)

Safety was measured according to standard adverse event collection as described in the adverse event section of the results. Complete tables of the serious adverse events according to the A1481156 treatment groups are provided in the reported adverse event section.

Number of Participants Reporting Treatment-related Serious Adverse Events

时间窗: Up to Follow-Up visit (30 to 40 days after study completion or treatment discontinuation)

All serious adverse events regardless of treatment group or suspected relationship to study drug were reported. Investigators were to provide independent determination of possible causality of any serious adverse event.

Number of Deaths Reported During This Study

时间窗: Last follow-up visit or 30 days after the last administration of study drug

Deaths were reported immediately independent of the circumstances or suspected cause at any time during the study through the last follow-up visit or 30 days after the last administration of study drug, whichever comes later.

Discontinuation Due to Intolerability

时间窗: Throughout the treatment duration (median treatment duration 1689 to 1744 days)

Participant who experienced drug-related intolerance, the participant's dose was reduced by 50%. If, after a dose reduction, the participant continued to appear intolerant, they were discontinued from study treatment.

Number of Participants With Deterioration Post Baseline in Visual Acuity Safety Tests

时间窗: Week 36

Visual Acuity is measured either using the reduced Snellen test or via Teller cards, and was assessed in the left and right eyes separately. There were 9 lines on the reduced Snellen chart which were coded as 6/60, 6/36, 6/24, 6/18, 6/12, 6/9, 6/6, 6/5, 6/4 (where 6/60 was the easiest to read and 6/4 was the most difficult to read). If a participant experienced a visual adverse event the investigator was asked to perform additional ocular assessments either at the visit when the participant reported the visual adverse event or at an unplanned visit.

Pediatric Motor Development Status at Week 52

时间窗: Week 52

Participant's motor development status was assessed at Week 52 using the physician assessment questions. Assessment question (i.e., compared to other children the participant's age group is this participant's motor development limited?) included the following criteria : severely limited, moderately limited, mildly limited and not limited.

Number of Participants With Deterioration Post Baseline in Color Vision Monitoring Safety Tests.

时间窗: Week 36

Colour vision was measured where appropriate via the Farnsworth-Munsell D-15 Hue test. This test was performed in both eyes simultaneously or just in a single specific eye. If using a single eye the same eye was used throughout the study. In case of young participants an age-and-ability-appropriate evaluation such as the Ishihara Test for Unlettered Persons were conducted.

Pediatric Motor Development Status at Week 16.

时间窗: Week 16

Participant's motor development status was assessed at A1481156 baseline (Week 16 in A1481131; NCT00159913) using the physician assessment questions. Assessment question (i.e., compared to other children the participant's age group is this participant's motor development limited?) included the following criteria : severely limited, moderately limited, mildly limited and not limited.

Number of Participants Reporting at Least One Adverse Event

时间窗: Up to Follow-Up visit (30 to 40 days after study completion or treatment discontinuation)

Safety was measured according to standard adverse event collection as described in the adverse event section of the results. Complete tables of the adverse events according to the A1481156 treatment groups are provided in the reported adverse event section.

Number of Participants Reporting Treatment-related Adverse Events

时间窗: Up to Follow-Up visit (30 to 40 days after study completion or treatment discontinuation)

Safety was measured according to standard adverse event collection as described in the adverse event section of the results.

Pediatric Cognitive Development Status at Week 16.

时间窗: Week 16

Participant's cognitive development status was assessed at A1481156 baseline (Week 16 in A1481131; NCT00159913) using the physician assessment questions. Assessment question (i.e., compared to other children the participant's age group is this participant's cognitive development limited?) included the following criteria : severely limited, moderately limited, mildly limited and not limited.