Introduction of transcatheter edge-to-edge repair in patients with congenital heart disease at a children's hospital

BackgroundAtrioventricular valve regurgitation (AVVR) is a devastating complication in children and young adults with congenital heart disease (CHD), particularly in patients with single ventricle physiology. Transcatheter edge-to-edge repair (TEER) is a rapidly expanding, minimally invasive option for the treatment of AVVR in adults that avoids the morbidity and mortality associated with open heart surgery. However, application of TEER in in CHD and in children is quite novel. We describe the development of a peri-procedural protocol including image-derived pre-intervention simulation, with successful application to four patients.AimsTo describe the initial experience using the MitraClip system for TEER of dysfunctional systemic atrioventricular valves in patients with congential heart disease within a pediatric hospital.MethodsA standardized screening and planning process was developed using cardiac magnetic resonance imaging, three dimensional echocardiography and both virtual and physical simulation. Procedures were performed using the MitraClip G4 system and patients were clinically followed post-intervention.ResultsA series of four CHD patients with at least severe AVVR were screened for suitability for TEER with the MitraClip system: three patients had single ventricle physiology and Fontan palliation, and one had repair of a common atrioventricular canal defect. Each patient had at least severe systemic AVVR and was considered at prohibitively high risk for surgical repair. Each patient underwent a standardized preprocedural screening protocol and image-derived modeling followed by the TEER procedure with successful clip placement at the intended location in all cases.ConclusionsThe early results of our protocolized efforts to introduce TEER repair of severe AV valve regurgitation with MitraClip into the CHD population within our institution are encouraging. Further investigations of the use of TEER in this challenging population are warranted. What is Known: Atrioventricular valve regurgitation (AVVR) is a devastating complication in children and young adults with congenital heart disease (CHD), particularly in patients with single ventricle physiology. Transcatheter edge-to-edge repair (TEER) is a rapidly expanding, minimally invasive option for the treatment of AVVR that avoids the morbidity and mortality associated with open heart surgery. AVVR in CHD patients is a complex problem. An organized approach is required if the potential of TEER therapy is ever to be realized in this heterogenous population. What is New: We demonstrate an image-derived modeling protocol and clinically successful application of TEER in four patients with CHD including three patients with palliated single ventricle physiology and a Fontan circulation. What is Next: The protocolized approach to TEER in CHD described in this report may serve as a foundation for the safe initiation of TEER therapy at other free-standing pediatric hospitals. Further collaborative investigations of the use of TEER in this challenging population are warranted. Ideally, a multicenter study will ensue.