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

https://doi.org/10.1016/j.jacc.2007.09.031Get rights and content
Under an Elsevier user license
open archive

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

Cited by (0)

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.

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.