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Published on: May 11, 2015
Long-term inhaled iloprost use in children with pulmonary arterial hypertension
Dursun Alehan1, Işıl Yıldırım, Murat Sahin
1Section of Cardiology, Faculty of Medicine, Department of Pediatrics, Hacettepe University, Sıhhıye, Ankara, Turkey. dalehan@hacettepe.edu.tr
Insights
Inhaled iloprost is safe and effective for treating pediatric pulmonary arterial hypertension, improving walking distance and showing good patient compliance. Long-term use demonstrated favorable tolerability and efficacy in children.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension Research
- Pharmacology
Background:
- Pulmonary arterial hypertension (PAH) in children presents significant morbidity and mortality risks.
- Chronic inhaled iloprost therapy is a potential treatment for pediatric PAH.
- Long-term data on inhaled iloprost's safety and efficacy in children is limited.
Purpose of the Study:
- To evaluate the long-term tolerability, safety, and efficacy of inhaled iloprost in pediatric patients with pulmonary arterial hypertension.
- To assess the impact of inhaled iloprost on functional capacity and treatment outcomes in children with PAH.
Main Methods:
- Retrospective analysis of 20 pediatric patients with idiopathic or associated PAH treated with inhaled iloprost (2003-2010).
- Patients received iloprost as monotherapy or in combination with other vasodilators.
- Data collected included patient demographics, treatment regimens, follow-up duration, and 6-minute walking test (6MWT) results.
Main Results:
- The median age of patients was 3.8 years; 60% had PAH associated with congenital heart disease.
- The median 6MWT distance increased from 420m to 490m after iloprost therapy (p=0.028).
- Adverse events were mild (headache, rash) and did not require discontinuation; overall compliance was good.
Conclusions:
- Inhaled iloprost, alone or combined with other therapies, is safe and effective for treating pediatric pulmonary arterial hypertension.
- Timely combination therapy and careful patient assessment are crucial for improving clinical outcomes in pediatric PAH.
- This study supports inhaled iloprost as a valuable treatment option for children with PAH.
Background:
We performed a retrospective analysis of patients with pulmonary arterial hypertension receiving inhaled iloprost in a single centre to evaluate long-term tolerability, safety, and efficacy of chronic inhaled iloprost therapy in children.
Methods:
A total of 20 patients with either idiopathic or associated pulmonary arterial hypertension were treated with iloprost between April, 2003 and January, 2010. The median age and weight of the patients were 3.8 years--ranging from 4 months to 19 years--and 12.3 kilograms--ranging from 4 to 73 kilograms-- respectively. Pulmonary arterial hypertension was idiopathic or hereditary in eight patients (40%) and associated with congenital cardiac disease in 12 patients (60%).
Results:
Of the 20 patients, 15 had combined therapy--12 patients with two and three patients with three different classes of drugs. In all, six patients died during follow-up. The median follow-up time was 18 months, ranging from 6 to 74 months. The 6-minute walking test was performed in 7 out of 20 patients at baseline and on follow-up. The median 6-minute walking test increased from 420 to 490 metres after iloprost therapy (p = 0.028). After initiation of iloprost therapy, one patient complained of headache and another had a rash around his mouth, none necessitating discontinuation of therapy. Overall compliance with inhaled iloprost was good.
Conclusion:
Pulmonary hypertension is associated with significant morbidity and mortality. Careful assessment of each patient and timely combination of specific vasodilator therapy is needed to improve clinical outcomes. This study suggests that inhaled iloprost, with or without concomitant endotelin receptor antagonist and/or phosphodiesterase inhibitor, is safe and efficacious for treatment of pulmonary arterial hypertension in children.
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