Background-Inhaled nitric oxide (iNO) has been used to assess the vasodilator capacity of the pulmonary vascular bed in children with congenital heart disease and elevated pulmonary vascular resistance. Inhaled iloprost is a pulmonary vasodilator for the long-term treatment of pulmonary hypertension (PHT). Because these 2 vasodilators act through different pathways (release of cGMP or cAMP, respectively), we compared the pulmonary vasodilator capacity of each. Methods and Results-A total of 15 children with congenital heart disease and PHT who had elevated pulmonary vascular resistance (preoperative, nϭ10; immediately postoperative, nϭ5) were first given 20 ppm of iNO for 10 minutes; then, after baseline values were reached again, they were given aerosolized iloprost at 25 ng ⅐ kg Ϫ1 ⅐ min Ϫ1 for another 10 minutes. Finally, iNO and iloprost were given simultaneously for 10 minutes. With iNO, the pulmonary vascular resistance and systemic vascular resistance ratio decreased from 0.48Ϯ0.38 to 0.27Ϯ0.16 (PϽ0.001). Similarly, iloprost decreased the ratio from 0.49Ϯ0.38 to 0.26Ϯ0.11 (PϽ0.05). The combination had no additional effect on the resistance ratio. Plasma cGMP increased from 17.6Ϯ11.9 to 34.7Ϯ21.4 nmol/L during iNO (PϽ0.01), and plasma cAMP increased from 55.7Ϯ22.9 to 65.1Ϯ21.2 nmol/L during iloprost inhalation (PϽ0.05). Conclusions-In children with PHT and congenital heart disease, both iNO and aerosolized iloprost are equally effective in selectively lowering pulmonary vascular resistance through an increase in cGMP or cAMP, respectively. However, the combination of both vasodilators failed to prove more potent than either substance alone. Aerosolized iloprost might be an alternative to iNO for early testing of vascular reactivity and for the postoperative treatment of acute PHT.

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Objective: To evaluate immediate and midterm results after balloon valvoplasty in a paediatric population with congenital aortic stenosis, giving special consideration to aortic regurgitation. Design: Retrospective study. Setting: Two tertiary referral centres for paediatric cardiology. Patients: 70 consecutive patients, with an age range of 0-16.4 years. Group A infants , 3 months old (n = 21). Group B children . 3 months old (n = 49). Median follow up time was 19.8 months, range 0-158 months. Intervention: All patients underwent balloon aortic valvoplasty. The balloon to annulus ratio was selected at a mean of 0.90 (range 0.67-1.0). Main outcome measures: Doppler gradients and degree of aortic regurgitation. Results: The pressure gradient dropped significantly with the intervention and increased mildly at follow up. Freedom from relevant aortic regurgitation (that is, moderate and severe) was initially lower in group A (75% v 90% after one month) but after two years the difference between the two groups was not significant (50% v 61%). Freedom from reintervention was significantly lower in group A (with 35% v 80%) after three years. Conclusion: Aortic balloon valvoplasty is safe and effective but has a high rate of early reintervention in infants with critical aortic stenosis. The major long term problem is progressive aortic regurgitation, which does not seem to be prevented by the use of small balloons.

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Inhalation of NO reduced pulmonary artery pressure in children with severe pulmonary hypertension after cardiac surgery and this effect was maintained over several days at concentrations carrying little risk of toxicity.

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Functional results of aortic valve repair with cusp extension using fresh pericardium have been satisfactory at medium term, particularly in children with a small aortic anulus at the time of initial repair, because the expansion potential of fresh autologous pericardium is equivalent to that of the growing sinotubular junction and aortic anulus diameters.