Use of Amplatzer fenestrated atrial septal defect device in a child with familial pulmonary hypertension.

In a 4.5-year-old child with refractory pulmonary arterial hypertension, we performed atrial septostomy with the application of an Amplatzer fenestrated device designed to maintain patency. Continuous intravenous epoprostenol infusion was started concomitantly. Forty-two months after the procedure, the patient had no recurrent syncope and remained in New York Heart Association functional class II. Fenestration of the atrial septum is feasible in children with pulmonary artery hypertension. No conclusion regarding the patient's need for an atrial septal defect can be drawn since concomitant prostanoid therapy was administered. The long-term patency of the atrial communication needs further confirmation and the optimal timing for its application has to be determined.