Anatomic Repair of Congenitally Corrected Transposition: 1.5V Repair with Hemi-Mustard and Rastelli Procedures

This operative technique video demonstrates an anatomic repair of congenitally corrected transposition (ccTGA) with ventricular septal defect (VSD), pulmonary atresia (PS), and mild right ventricular hypoplasia in a two-year-old who was palliated with a 3.5mm rmBTS (and coronary artery fistula ligation) in the neonatal period, followed by a rmBTS takedown and bidirectional Glenn shunt at four months old. 

Morphologic Left-Ventricle-to-Aorta (mLV-to-Ao) Baffling 

The VSD was inspected through a vertical ventriculotomy along the morphologic right ventricular outflow tract (RVOT). The mLV-to-Ao baffle was created with bovine pericardium. The inlet portion of the VSD was closed with interrupted, pledget-supported braided sutures to avoid tricuspid chords. The remainder of the baffle was completed with continuous 5-0 Prolene sutures that were placed on either side of the interrupted sutures. 

Hemi-Mustard 

The septum primum and limbus of the septum secundum were excised. Then the coronary sinus was unroofed to minimize chance of obstruction. A bovine patch was sewn from the lateral edge of the tricuspid valve across the atrial septum to around the IVC. After this, there was an anterior leaflet cleft in the mitral valve that was closed with interrupted GORE-TEX sutures. 

Rastelli 

The RVOT was reconstructed with a trileaflet ePTFE valved- conduit constructed during the operation. Next, the previous MPA stump patch was removed, and defect was extended onto LPA. Then the distal end was anastomosed to the branch pulmonary arteries on the left side of the aorta. Finally, the proximal end was anastomosed to the RVOT with an anterior bovine pericardial hood. 

Post-op transesophageal echocardiogram showed good biventricular function and no significant tricuspid or mitral regurgitation. There was no mLV-to-Ao or atrial baffle obstruction. There was no RVOT gradient or insufficiency. 

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