Short- and long-term effects of inhaled iloprost therapy in children with pulmonary arterial hypertension.

Abstract

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.

• Authors: D Dunbar Ivy, Aimee K Doran, Kelly J Smith, George B Mallory Jr, Maurice Beghetti, Robyn J Barst, Daniela Brady, Yuk Law, Donna Parker, Lori Claussen, Steven H Abman
• Journal: Journal of the American College of Cardiology (J Am Coll Cardiol) Vol. 51 Issue 2 Pg. 161-9 (Jan 15 2008) ISSN: 1558-3597 [Electronic] United States
• PMID: 18191742 (Publication Type: Journal Article, Multicenter Study, Research Support, N.I.H., Extramural)

Chemical References

• Vasodilator Agents
• Iloprost

Topics

• Administration, Inhalation
• Adolescent
• Age Factors
• Child
• Child, Preschool
• Cohort Studies
• Dose-Response Relationship, Drug
• Drug Administration Schedule
• Female
• Follow-Up Studies
• Humans
• Hypertension, Pulmonary (diagnosis, drug therapy)
• Iloprost (administration & dosage)
• Male
• Multivariate Analysis
• Probability
• Respiratory Function Tests
• Retrospective Studies
• Risk Assessment
• Severity of Illness Index
• Time Factors
• Treatment Outcome
• Vascular Resistance (drug effects)
• Vasodilator Agents (administration & dosage)