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Neonatology

PULMONARY HYPERTENSION IN NEONATES AND CHILDREN

Pulmonary hypertension in neonates and children can result from various underlying disorders, e.g. persistent pulmonary hypertension of the newborn (PPHN), bronchopulmonary dysplasia (BPD), pulmonary hypertension associated with congenital heart defects, idiopathic pulmonary arterial hypertension (iPAH), and hypoxic lung failure. Inhaled nitric oxide (NO) ventilation accompanied by a suitable pharmacological therapy has been approved for the treatment of hypoxic lung failure and pulmonary hypertension in term neonates (> 37th week of pregnancy. Several recent reports contain observations concerning the use of approved adult pulmonary hypertension drugs in children. Persistent pulmonary hypertension after operations for congenital heart defects carries a high risk of morbidity and mortality. A clinical study reports that 15 children suffering from congenital heart disease were administered inhaled NO and iloprost pre- and immediately postoperatively. Both substances induced a comparable significant decrease in the quotient from pulmonary and systemic vascular resistance (PVR/SVR). The study also found no differences in pulmonary selectivity. Inhaled iloprost is described as a viable therapeutic alternative because of its toxicological harmlessness, ease of use, and ventilation-independent application.

Further publications report the successful use of inhaled iloprost after other treatment options such as epoprostenol or NO had failed in children who had undergone congenital heart surgery. Additional administration of inhaled iloprost established stable haemodynamics and allowed to transplant a heart into a patient who received an intensified pharmacotherapy for congenital heart defect and subsequently developed de-compensated right-ventricular failure. Positive effects of inhaled iloprost therapy have also been demonstrated in neonates with persistent pulmonary hypertension (PPHN) and in children suffering from severe respiratory failure. A pilot study addressing PPHN found that oxygenation and clinical condition improved so that mechanical ventilation was not necessary.

No rebound phenomenon was seen during the two hours of inhalation.

In some isolated cases, inhaled iloprost helped wean patients with bronchopulmonary dysplasia from mechanical ventilation and/or reduced outpatients´ need for supplemental oxygen. There are indications that inhaled iloprost is able to produce such improvements in physical function and quality of life not only in adult patients with pulmonary hypertension but also in children suffering from this disease. The „Pulmonary Hypertension“ work group within the German Society for Pediatric Cardiology gives preference to treatment with prostanoids. A practice-oriented survey discusses the advantages of inhaled iloprost as well as the problems associated with practising inhalation several times a day, and of other therapy options for children. The “Congenital Heart Defects” competence network recommends doing the treatment with inhaled iloprost using an ultrasonic nebulizer.

Excerpt from the leaflet „Inhaled iloprost in intensive medical care – Clinical data, November 2004, published by Schering Deutschland GmbH, Berlin