Aerosolized iloprost for postoperative pulmonary hypertensive crisis in children with congenital heart disease

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Abstract

Introduction

Pulmonary hypertensive crisis (PHC) is a significant contributor to the morbidity and mortality of surgery for congenital heart defect. Management of such a potentially fatal complication has been evolving for the past decades. Inhaled iloprost has been reported as an alternative treatment for this condition. We evaluated the use of aerosolized iloprost as a rescue therapy for PHC in children undergoing congenital heart surgery.

Methods

In this clinical study, 12 high risk children were monitored in order to identify postoperative PHC after congenital heart repair. Factors being monitored included pulmonary artery pressure, systemic blood pressure, left atrial pressure, transcutaneous oximetry and heart rate. PHC was defined as an acute rise in pulmonary pressure which causes cardiopulmonary compromise as reflected by desaturation and hypotension. Despite conventional medical treatment to prevent postoperative PHC, children with PHC were therefore administered with aerosolized iloprost (0.5 μg/kg).

Result

Eight of the 12 children had one or more episodes of PHC, secondary to the pulmonary vasoreactivity. All responded to the aerosolized iloprost treatment, as demonstrated by a fall in their mean pulmonary pressure from 47.9 ± 14.9 to 30.2 ± 7.9 mmHg (p = 0.012) and a rise in the arterial saturation from 82.2 ± 16.7 to 93.4 ± 11.5 % (p = 0.012) while mean systemic blood pressure tended to increase from 59.4 ± 12.1 to 64 ± 10.3 mmHg (p = 0.16).

Conclusion

In medical setting with limited access to the nitric oxide, inhaled iloprost is consider to be an effective alternative treatment for postoperative PHC in children undergoing congenital heart surgery.

Section snippets

Materials and methods

This study was approved by the Institutional Review Board and written informed consent was obtained from the patient's parents prior to enrollment.

In this prospective open-label single arm study, patients at risk of developing PHC [3] were monitored with a transthoracic pulmonary artery catheter in order to measure the pulmonary pressure during PHC after surgery for congenital cardiac defects. Patients were eligible, if they had cardiac surgery under cardiopulmonary bypass for congenital heart

Statistical analysis

Data are expressed as mean ± standard error of the mean. The Wilcoxon Signed Rank's Test was used to compare the hemodynamic differences between pre- and post-iloprost treatment during PHC.

Hemodynamic data

The administration of inhaled iloprost significantly reduced the mean PAP from 47.9 ± 14.9 to 30.2 ± 7.9 mmHg (p = 0.012) and improved the oxygen saturation from 82.2 ± 16.7 to 93.4 ± 11.5% (p = 0.012) while being ventilated with 100% oxygen as shown in Fig. 1, Fig. 2. The mean systemic blood pressure tended to increase from 59.4 ± 12.1 to 64 ± 10.3 mmHg (p = 0.16) as shown in Fig. 3. There were no significant changes in the other hemodynamic measurements. All eight children continued to improve with iloprost

Discussion

Congenital heart defects causing increased pulmonary blood flow or pulmonary venous congestion frequently lead to pulmonary hypertension. The chronically increased blood flow and increased blood pressure in the pulmonary vascular system results in arterial smooth muscle cell hypertrophy and hyperplasia. This subsequently leads to pulmonary vasoconstriction followed by cellular remodeling involving endothelial cells and smooth muscle cells, which is eventually also impairing the

Conclusion

In this study iloprost was effective as a selective pulmonary vasodilator in treatment of the PHC in selected children undergoing congenital heart surgery.

Acknowledgement

We thank Axel Boehnke, M.D. for his editorial work with this manuscript.

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