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

Abstract

Background: 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 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.

Aims: To 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.

Methods: A 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.

Results: A 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.

Conclusions: The 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.

Keywords: congenital heart disease; echocardiography; valvular heart disease.

Figure 1.. Overview of Preoperative Modeling.

Cardiac Magnetic resonance images and 3D echocardiographic images were registered to create a combined model. These models were then used for virtual simulation using SlicerHeart, and physical simulation with the actual MitraClip Delivery System.

Figure 2.. Case 1 with Hypoplastic Left Heart Syndrome and a Fontan Circulation.

Top: 2D, 3D and 3D color images before intervention. Mid: Bottom: 2D, 3D and 3D color ages after delivery of first clip near the anterior-septal commissure with reduction in regurgitation. Bottom: 2D, 3D and 3D color ages after delivery of second clip centrally between the anterior and septal leaflets with significant reduction in regurgitation.

Figure 3.. Case 2 with Hypoplastic Left Heart Syndrome and a Fontan Circulation.

Top: 2D, 3D and 3D color images before intervention. Bottom: 2D, 3D and 3D color ages after delivery of a single clip between the anterior and septal leaflets with significant reduction in regurgitation.

Figure 4.. Case 3 with Complete Atrioventricular Canal and Left Atrioventricular Valve Regurgitation).

Top: 2D, 3D and 3D color images before intervention. Bottom: 2D, 3D and 3D color images after delivery of single clip between the superior and inferior bridging leaflet (to close the open “cleft”).

Figure 5.. Case 4 with Unbalanced Complete Atrioventricular Canal, Interrupted Inferior Vena Cava, and a Fontan Circulation.

Top: 2D, 3D and 3D color images before intervention. Mid: 2D, 3D and 3D color ages after delivery of first clip. Bottom: 2D, 3D and 3D color ages after delivery of second clip with significant reduction in regurgitation.