Atrial flow regulator and atrial septostomy in pediatric pulmonary hypertension: when procedure and device match

Abstract

To the Editor, Despite recent improvements in the management of pediatric pulmonary hypertension (PH) and reduced overall mortality rates due to pathway-targeted therapy, there is currently no cure for most patients, and morbimortality remains high with lung transplant being the destination therapy for most.1,2 According to the clinical practice guidelines, the creation of a pre-tricuspid restrictive defect with atrial septostomy (AS) has no strong evidence but may be considered for patients in WHO/Ross III-IV functional class (FC) with syncope and/or severe right ventricular failure who remain unresponsive to maximal pharmacological treatment or as a bridging therapy to lung transplant.1,2 There is relatively little experience among pediatric patients. However, the AS/PH drug combo has improved short-term outcomes like FC, syncope or survival.3,4 When performed by experienced teams the rate of complications drops. However, it is still challenging in the context of high-risk patients. Main fatal events are related to a severe PH crisis or severe cyanosis due to unpredictable shunt size. Different approaches have been described to create a restrictive AS. Balloon (or blade) septostomy shows early spontaneous closure, so implanting a device in the atrial septum would be a common way to prevent it. The use of a customized fenestrated closure device has...