Complex Bi-ventricular Repair For Severely Unbalanced, Left Ventricle Dominant Atrioventricular Canal
Damien J. LaPar, MD MSc, Julija Dobrila, MD, Blaz Podgorsek, MD, Peter C. Chen, MD, Christopher E. Greenleaf, MD, Jorge D. Salazar, MD.
UTHealth McGovern Medical School, Houston, TX, USA.
Objective(s): Unbalanced atrioventricular (AV) canal defects remain a challenging surgical dilemma for pediatric and congenital cardiac surgeons. While single ventricle palliation remains the gold standard for cases with significant ventricle hypoplasia, there are several long-term disadvantages to the Fontan circulation. Consequently, bi-ventricular conversion for these patients provides an attractive alternative with improving outcomes and potential advantages.
Methods: The present video demonstrates a successful bi-ventricular repair/conversion for a severely unbalanced, left ventricle dominant AV canal in a 13-month-old (11 kg) child with trisomy 21 who had undergone previous pulmonary artery (PA) banding and AV valve repair. Pre-operative magnetic resonance imaging demonstrated limited inflow through the tricuspid valve, with an right ventricle (RV) volume indexed of 25 ml/m2 and moderate AV valve regurgitation. Biventricular repair included extensive RV overhaul, right AV valve repair with papillary muscle splitting, tricuspid anterior papillary muscle relocation and resuspension to the RV free wall with an adjustable tensioner, left AV valve repair, fenestrated atrial septal defect closure, main PA band removal, and pulmonary arterioplasty.
Results: Postoperative echocardiogram demonstrated low normal RV function, a closed VSD, mild left and right AV valve regurgitation, and bidirectional ASD shunting with a mean gradient of 3mmHg. The postoperative course was uneventful with extubation on postoperative day 2 and discharge home on postoperative day 9.
Conclusions: Complex bi-ventricular repair in patients with severely unbalanced, left ventricle dominant atrioventricular canal can be performed successfully. Detailed preoperative imaging and patient selection are critical to achieve good outcomes. Long-term follow up with observation is needed.
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