Author + information
- Received February 19, 2007
- Revision received August 22, 2007
- Accepted September 7, 2007
- Published online January 15, 2008.
- D. Dunbar Ivy, MD⁎,1,⁎ (, )
- Aimee K. Doran, CPNP⁎,3,
- Kelly J. Smith, MD†,
- George B. Mallory Jr, MD†,
- Maurice Beghetti, MD‡,
- Robyn J. Barst, MD§,4,
- Daniela Brady, RN§,
- Yuk Law, MD∥,
- Donna Parker, RRT⁎,
- Lori Claussen, RN⁎ and
- Steven H. Abman, MD⁎,2
- ↵⁎Reprint requests and correspondence:
Dr. Dunbar Ivy, Pediatric Heart Lung Center, Section of Pediatric Cardiology, The Children’s Hospital, 13123 East 16th Avenue, Denver, Colorado 80045.
Objectives This study investigated the short- and long-term outcome of children with pulmonary arterial hypertension (PAH) treated with inhaled iloprost.
Background Inhaled iloprost has been approved for the treatment of adults with PAH, but little is known about the effects in children with PAH.
Methods We evaluated the acute effects of inhaled iloprost on hemodynamic status and lung function and the response to long-term therapy in 22 children (range 4.5 to 17.7 years) with PAH (idiopathic, n = 12; congenital heart disease, n = 10). Cardiac catheterization, standard lung function testing before and after iloprost inhalation, 6-min walk test, World Health Organization functional class, and hemodynamic parameters were monitored.
Results Acute administration of inhaled iloprost lowered mean pulmonary artery pressure equivalent to the response to inhaled nitric oxide with oxygen. Acute iloprost inhalation reduced forced expiratory volume in 1 s and mid-volume forced expiratory flow by 5% and 10%, respectively, consistent with acute bronchoconstriction. At 6 months, functional class improved in 35%, decreased in 15%, and remained unchanged in 50% of children. Sixty-four percent of patients continued receiving long-term iloprost therapy, 36% stopped iloprost, due to lower airway reactivity, clinical deterioration, or death. In 9 patients on chronic intravenous prostanoids, 8 transitioned from intravenous prostanoids to inhaled iloprost, which continued during follow-up.
Conclusions Inhaled iloprost caused sustained functional improvement in some children with PAH, although inhaled iloprost occasionally induced bronchoconstriction. Most patients tolerated the transition from intravenous to inhaled prostanoid therapy. Clinical deterioration, side effects, and poor compliance, owing to the frequency of treatments, could limit chronic treatment in children.
↵1 Dr. Ivy is a consultant for Actelion, Gilead, and United Therapeutics.
↵2 Dr. Abman serves as a scientific advisor for INO Therapeutics.
↵3 Aimee Doran is a consultant for Actelion, Gilead, and United Therapeutics.
↵4 Dr. Barst is a consultant for Actelion, Gilead, United Therapeutics, INO Therapeutics, Biogen Idec, Pfizer, and Lilly.
Supported in part by grant M01-RR00069 from the General Clinical Research Center branch of the National Center for Research Resources, National Institutes of Health.
- Received February 19, 2007.
- Revision received August 22, 2007.
- Accepted September 7, 2007.
- American College of Cardiology Foundation