Methods: Between 2008 and 2011, 16 children (10 boys, 6 girls; mean age 4.97 years; range 8 months to 17 years) with a ventricular septal defect and associated severe pulmonary arterial hypertension who had a positive vasoreactivity were administered inhaled iloprost for a mean duration of 40 (range 7 to 90) days before the surgical repair. Inhaled iloprost was used for five days postoperatively. Pulmonary arterial hypertension was diagnosed by right and left heart catheterization and evaluated with echocardiography immediately before the operation, 30 days postoperatively, and annually for five years.

Results: The mean systolic and mean pulmonary artery pressures at the initial presentation were 81 (range 51 to 104) mmHg and 60 (range 41 to 77) mmHg, respectively. After the administration of inhaled iloprost, there was a decline of about 18 mmHg according to the echocardiographic findings (p<0.05). After surgery, the mean pulmonary arterial pressure additionally decreased by 14 mmHg (p<0.05). There was mortality in two patients perioperatively. The remaining patients recovered well and were uneventfully discharged. There was no increase in the postoperative pulmonary artery pressures in discharged children for five years.

Conclusion: Inhaled iloprost-induced pulmonary vasodilator response varies among children. The use of inhaled iloprost before and after the surgical repair assures a reduction in pulmonary arterial pressure and decrease cardiovascular complications.