Cardiovascular anatomy in children with bidirectional Glenn anastomosis, regarding the transcatheter Fontan completion.

BACKGROUND: Transcatheter stent-secured completion of total cavopulmonary connection (TCPC) after surgical preparations during the Glenn anastomosis procedure has been reported, but complications from this approach have precluded its clinical acceptance.

AIMS: To analyse cardiovascular morphology and dimensions in children with bidirectional Glenn anastomosis, regarding the optimal device design for transcatheter Fontan completion without special surgical "preconditionings".

METHODS: We retrospectively analysed 60 thoracic computed tomography and magnetic resonance angiograms performed in patients with a median age of 4.1 years (range: 1.8-17.1 years). Additionally, we simulated TCPC completion using different intra-atrial stent-grafts in a three-dimensional model of the representative anatomy, and performed calculations to determine the optimal stent-graft dimensions, using measured distances.

RESULTS: Two types of cardiovascular arrangement were identified: left atrium interposing between the right pulmonary artery (RPA) and inferior vena cava, with the right upper pulmonary vein (RUPV) orifice close to the intercaval axis (65%); and intercaval axis traversing only the right(-sided) atrial cavity, with the RUPV located posterior to the atrial wall (35%). In the total population, the shortest median RPA-to-atrial wall distance was 1.9mm (range: 0.6-13.8mm), while the mean intra-atrial distance along the intercaval axis was 50.1±11.2mm. Regardless of the arrangement, 83% of all patients required a deviation of at least 5.9±2.4mm (range: 1.2-12.7mm) of the stent-graft centre at the RUPV level anteriorly to the intercaval axis to avoid covering or compressing this vein. Fixing the anterior deviation of the curved stent-graft centre at 10mm significantly decreased the range of bend angle per every given RUPV-RPA distance.

CONCLUSIONS: For both types of cardiovascular arrangement, after conventional bidirectional Glenn anastomosis, the intra-atrial curved stent-graft seemed most suitable for achieving uncomplicated TCPC completion percutaneously without previous surgical "preconditionings" in the majority of children. Experimental study is necessary to validate this conclusion.