Pulmonary arterial hypertension (PAH) is a rare disorder characterized by increased pulmonary arterial pressure and vascular resistance due to the impaired endothelial wall and smooth muscle functions of the pulmonary vessels [1,2]. The PAH in children is either idopathic (IPAH) or associated with congenital heart diseases (CHD), especially with left to right shunts [3]. In the treatment, nitric oxide, prostanoids, magnesium sulphate, endothelin receptor antagonists and phosphodiesterase-5 inhibitors are used. Prostacyclins vasodilate the pulmonary vessels and prevent the endothelial cell damage to control PAH [4]. The most common side effects of prostacyclins is headache, systemic hypotension, allergic reactions, chest pain, dyspnea, nausea and vomiting [2,4]. The optimum dosage with the minimum side effects is 0.3 ng/kg/min for the inhalation and 0.6 ng/kg/min. for the intravenous administration of iloprost (synthetic analog of prostacyclin) [4].

To evaluate the safety of inhaled iloprost in infants with PAH, we analyzed its side effects retrospectively in our pediatric cardiology and cardiovascular surgery clinic. We evaluated 52 infants (27 females) with PAH-CHD hospitalized for the surgical correction of their cardiac anomalies in the last year. Their mean age was 13.5±4.7 months (3-24 months) and the mean pulmonary arterial pressure (PAP) was 39±11.6 mmHg. (25-70 mmHg). They received iloprost inhalation (Ilomedin, Schering AG) 6 times/day at a dosage of 0.3 ng/kg/min for 7-15 days (mean 10.6 ± 2.8 days) preoperatively. Their mean PAP was 28 ± 12.3 mmHg after the iloprost treatment. The difference in the mean PAP values was statistically significant (P<0.05). The infants were monitorized during the inhalation. They did not develop systemic hypotension (mean arterial pressure was 72±8.7 mmHg) and their vital signs were stable.

4. Hawkins A, Tulloh R. Treatment of pediatric pulmonary hypertension. Vasc Health Risk Manag. 2009;5:509–24.