Atrial septal fenestration in children for treatment of end-stage pulmonary hypertension

Patients and methods: Atrial septostomy was carried out in three children with end-stage pulmonary arterial hypertension and recurrent syncope. Continuous intravenous epoprostenol infusion was started concomitantly, in association with oral bosentan therapy. Results: Within a few weeks, the functional New York Heart Association (NYHA) class improved from III to II in all three patients. No recurrent syncope occurred and the children remained in NYHA functional class II during follow-up. The atrial septostomy remained patent and there was no need to increase the dose of intravenous epoprostenol. Conclusion: This small case series demonstrates that children with end-stage pulmonary arterial hypertension may benefit from an aggressive management, including fenestration of the atrial septum associated with the combination of bosentan and epoprostenol.