MiscellaneousLong-Term Outcomes in Children With Pulmonary Arterial Hypertension Treated With Bosentan in Real-World Clinical Settings
Section snippets
Methods
This is a retrospective cohort study in 86 consecutive pediatric patients with PAH (≤18 years of age at bosentan initiation) who started bosentan from May 2001 to April 2003 with or without pre-existing continuous parenteral intravenous epoprostenol or subcutaneous treprostinil therapy at 2 tertiary referral centers in the United States (Columbia University Medical Center, New York, New York, and the Children's Hospital, Denver, Colorado). Follow-up data were collected retrospectively from
Results
In this cohort of 86 children with PAH, the median observation period, defined as time between initiation of bosentan and date of last clinical information capture before end of data collection, was 39 months (mean ± SD 35 ± 15, range 2 to 60).
Demographic characteristics at bosentan initiation have been presented previously7 (Table 1). Patients' ages ranged from 9 months to 18 years at the start of bosentan therapy. More patients with PAH-CHD were female and started bosentan as monotherapy.
Discussion
In this retrospective cohort study, we extended the observation period by 3 years in a previously identified cohort of 86 consecutive pediatric patients with PAH treated with bosentan monotherapy or as an add-on to pre-existing parenteral intravenous epoprostenol or subcutaneous treprostinil therapy.7 At the time these patients had bosentan initiated, bosentan was the only approved oral PAH therapy.
Historically, the prognosis for children with PAH appeared even worse than that for adults; the
Acknowledgment
The authors thank Sylvie Ertel, PhD, for medical writing assistance funded by Actelion Pharmaceuticals, Ltd. (Allschwil, Switzerland).
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Committee, and approved by the British Thoracic Society and the British Society of RheumatologyRecommendations on the management of pulmonary hypertension in clinical practice
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Cited by (0)
Dr. Ivy has served on advisory boards for Actelion, Basel, Switzerland; Gilead, Foster City, California; Pfizer, New York, New York; and United Therapeutics, Research Triangle Park, North Carolina; and receives grant support from Gilead. Dr. Berman Rosenzweig receives grant support from Actelion, Eli Lilly, Indianapolis, Indiana; Pfizer, Gilead and United Therapeutics and is a consultant for Actelion and United Therapeutics. Mr. Lemarié is a consultant for Actelion. Mrs. Brand and Dr. Rosenberg are employees of Actelion. Dr. Barst is a consultant for Actelion, Eli Lilly, Gilead, GSK, Middlesex, United Kingdom; Novartis, New York, New York; and Pfizer.
This research was supported by Grant M01 RR00069 from the General Clinical Research Centers Program, National Center for Research Resources, National Institutes of Health, Bethesda, Maryland, and by Actelion Pharmaceuticals, Ltd., Allschwil, Switzerland.