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Improved Management of Systemic Venous Anomalies in Single Ventricle: New Rationale

Antonio Amodeo1, Mauro Grigioni2, Sergio Filippelli1, Giuseppe D'avenio2, Roberto M. Di Donato1; 1Ospedale Bambino Gesù, Rome, Italy; 2Istituto Superiore di Sanità, Rome, Italy


Objective: New technical solutions were conceived for two variants of systemic venous connections known to affect the functioning of conventional cavopulmonary anastomoses: 1) direct hepatic-to-azygos vein anastomosis in the setting of a Kawashima operation for cases of polysplenia syndrome and interruption of the inferior vena cava; 2) confluent bidirectional single cavopulmonary anastomosis in cases of bilateral superior venae cavae. Guiding principles were: a) reducing competition of venous flows; b) exploiting the flow velocity enhancement effect of confluent venous channels; c) avoiding prosthetic material.

Methods: We performed finite element numerical simulations and hydraulic efficiency assessments in two sets of comparative models: 1) classic Kawashima completion (CKC), with extracardiac connection of the isolated hepatic venous return to the pulmonary artery - vs - modified Kawashima completion (MKC), with direct hepatic-to-azygos vein anastomosis; 2) bilateral bidirectional Glenn anastomosis (BBGA) - vs - modified bilateral bidirectional Glenn (MBBGA), i.e. bilateral superior venae cavae merging into a single bidirectional cavopulmonary anastomosis both as isolated procedure and in association with Fontan completion. Since September 2003, these two innovative techniques were carried out in 7 patients: 1) MKC in 5 patients (mean age 4.8 years, mean weight 14.5 Kg) with one MKC case performed as a single stage procedure; 2) MBBGA in 2 patients, both 10 month-old and weighing about 7 kgs. Flow mapping MRI studies were also performed in 3 patients with MKC and in the 2 patients with MBBGA.

Results: Results: Vectorial maps of the velocity fields showed a higher computed hydraulic efficiency and less power loss in the MKC arrangement compared to the CKC model as well as in MBBGA layout compared to conventional BBGA model. All patients are alive and well up to 36 months with 2D-echo and MRI evidence of excellent function of the venous anastomoses. The 2 patients with MBBGA are awaiting Fontan completion.

Conclusion: Conclusion: Direct hepatic-to-azygos vein anastomosis associated with Kawashima operation and single confluent bilateral bidirectional Glenn anastomosis are safe and effective techniques for the management of systemic venous anomalies in single ventricle patients. Hydrodynamic studies prove that these techniques are superior to the respective conventional counterparts.


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