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Published on: May 11, 2015
Use of inhaled iloprost in children with pulmonary hypertension
Antonio Moreno-Galdó1, Alba Torrent-Vernetta, Inés de Mir Messa
1Unidad de Neumología Pediátrica y Fibrosis Quística, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
Insights
Inhaled iloprost showed good tolerability and effectiveness in children with pulmonary hypertension (PH). While some patients improved, others experienced deterioration, highlighting the need for further research in pediatric PH treatment.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Pharmacology
Background:
- Pediatric pulmonary hypertension (PH) is a severe condition requiring effective treatments to prevent vascular remodeling and improve survival.
- Iloprost is an approved treatment for adult PH, but its efficacy and safety in children remain under-evaluated.
Purpose of the Study:
- To assess the long-term effects of inhaled iloprost in pediatric patients diagnosed with pulmonary hypertension.
- To analyze treatment outcomes, including clinical status, functional capacity, and survival rates.
Main Methods:
- A retrospective study included 22 pediatric patients treated with inhaled iloprost between 2000 and 2012.
- Exclusion criteria included left-right cardiac shunt and persistent PH of the newborn.
- Patient diagnoses encompassed pulmonary arterial hypertension, pulmonary veno-occlusive disease, PH secondary to lung disease, and multifactorial PH.
Main Results:
- Iloprost demonstrated good tolerability in most patients, with only one case of abdominal pain.
- PH resolved in 2 patients, functional capacity improved in 10, and 3 showed stabilization.
- Six patients deteriorated, including two deaths and two lung transplant recipients.
Conclusions:
- This uncontrolled study suggests inhaled iloprost is effective and well-tolerated for treating pediatric pulmonary hypertension.
- Further research is necessary to confirm these findings due to the serious nature of pediatric PH and its potential for poor outcomes.
Abstract:
Pulmonary hypertension (PH) in children is a serious disorder, for which the major goal of treatment is to prevent progressive vascular remodeling, and improve clinical status and survival. Iloprost is approved for the treatment of PH in adults; however, few studies have evaluated its effects in children. The objective of this study is to analyze the long-term effects of inhaled iloprost treatment in children with PH. A retrospective study was conducted in patients treated with iloprost between 2000 and 2012. Patients with left-right cardiac shunt and persistent PH of the newborn were excluded. The cohort comprised 22 patients (15 females) with a median age of 2.6 years. Twelve patients had pulmonary arterial hypertension including idiopathic (n = 6), hereditary (n = 2) and associated (congenital heart disease [n = 3], and schistosomiasis [n = 1]). One patient had pulmonary veno-occlusive disease, six patients had PH secondary to lung disease and three had multifactorial PH. Median mean pulmonary arterial pressure was 55 mmHg and median pulmonary vascular resistance was 15.5 Wood units. Good tolerability was observed, with the exception of one case of recurring abdominal pain. PH resolved in two patients, with functional capacity improvement in 10 patients and stabilization in three patients. The clinical condition of six patients deteriorated; two died, and two received lung transplants. In conclusion, the results of this uncontrolled study showed that iloprost was effective and well tolerated in children. However, further research is needed to support this study, as PH is a serious condition that can require organ transplantation or result in death.
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