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CLINICAL RESEARCH: PEDIATRIC CARDIOLOGY

Short- and Long-Term Effects of Inhaled Iloprost Therapy in Children With Pulmonary Arterial Hypertension

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

^_* The Pulmonary Hypertension Program and Pediatric Heart Lung Center, Department of Pediatrics, The University of Colorado School of Medicine and The Children’s Hospital, Denver, Colorado
Pediatric Pulmonology, Texas Children’s Hospital, and Baylor University School of Medicine, Houston Texas
Pediatric Cardiology, Hospital of the University of Geneva and Children’s Hospital of Geneva, Geneva, Switzerland
Columbia University College of Physicians and Surgeons and New York Presbyterian Hospital, New York, New York
^|| Children’s Hospital & Regional Medical Center, Department of Pediatrics, University of Washington, Seattle, Washington.

Manuscript received February 19, 2007; revised manuscript received August 22, 2007, accepted September 7, 2007.

^_* 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. (Email: ivy.dunbar{at}tchden.org).

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.

Abbreviations and Acronyms   6MW = 6-min walk   FEV1 = forced expiratory volume in 1 s   FEF25–75 = mid-volume forced expiratory flow   FVC = forced vital capacity   IV = intravenous   NO = nitric oxide   PAH = pulmonary arterial hypertension   PAP = pulmonary arterial pressure   PVR = pulmonary vascular resistance   TLC = total lung capacity   WHO = World Health Organization