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In this video, the authors demonstrate their technique involving the combined Mustard procedure and en bloc rotation of the outflow tracts in a seven-year-old child with congenitally corrected transposition of the great arteries, ventricular septal defect, and severe pulmonary stenosis in a redo setting. The patient had previously undergone epicardial pacemaker implantation due to intermittent atrioventricular block at the age of four years. 
 
After redo sternotomy, the heart was thoroughly dissected and freed from all scar tissue. A pericardial patch was harvested, measured, and the contours of the patch were marked to serve as cutting lines. After trimming the patch, the first stitch was placed to ensure rapid reorientation and save clamp time. The patient was then placed on cardiopulmonary bypass.  
 
After instillation of crystalloid cardioplegic solution into the aortic root, the right atrium was obliquely incised, and the morphologic left ventricle was inspected.  

To begin the Mustard operation, the atrial septum was completely excised, beginning within the oval fossa. The septum secundum was also radically excised to ensure a straight path between the superior vena cava and the baffle. Next, the endocardium was approximated throughout the entire excision line, and the left atrial appendage was partially excluded to shorten the length of the suture line of the Mustard baffle. The coronary sinus was incised.  

The next step involved starting a continuous suture between the atrial wall and the pericardial patch, located between the tricuspid valve and the pulmonary veins at the level midway between the caval veins. The patch should run around the ostia of the caval veins, ensuring that blood will flow unobstructed into the tricuspid valve. A marking stitch was used on the pericardial patch to estimate the point at which the end of the caval vein circumference should be reached. In this case, the patch needed to be trimmed to prevent obstruction of pulmonary venous blood flow into the mitral valve. Both suture lines should meet again between the mitral and tricuspid valves. 

The result of the Mustard baffle was critically inspected once more before the right atrium was closed. After more than one hour of cross-clamp time, and with the en bloc rotation of the outflow tracts still needing to be performed, the decision was made to open the aortic cross-clamp and allow the heart to recover during approximately 30 minutes of reperfusion.  

After this time, the aorta was cross-clamped again. Both the aorta and the pulmonary artery were cut at the same level, inspected, and the ventricular septal defect (VSD) was probed with a right-angled clamp. Additional dissection of the aortic root was necessary.  

Marking sutures were placed to facilitate orientation after the excision of the outflow tract block. The buttons of the coronary arteries were excised and mobilized extensively. A subaortic incision in the left ventricular outflow tract initiates the en bloc excision of both roots. The previously placed marking stitches were divided, and first, the part of the aortic root and then the part of the pulmonary root were harvested. At this stage, caution must be taken to avoid losing orientation and prevent harming other structures, such as the coronary arteries, the atrioventricular (AV) valves, and the atria. 
 
Two balanced ventricles and the VSD were visible. Both roots were inspected, and a commissurotomy of the pulmonary valve was performed to augment the valve orifice. The tunnel-like subvalvular stenosis was also resected. Excessive muscle from the infundibular septum was trimmed to reduce the mismatch between the outflow tracts and their new roots. The wide base between both roots was approximated to facilitate easier anastomosis and reduce the risk of restenosis. 

The outflow tract was measured with a probe, and the VSD was closed with an autologous pericardial patch. The patch was carefully chosen to ensure that it was not too wide, as that could provoke aortic insufficiency in the future, but large enough to compensate for the mismatch between the outflow tract and the root. The sutures were anchored on both ends of the patch, and the patch was trimmed according to the height of the outflow tracts. 

Next, the outflow tracts were rotated by approximately 180 degrees and reimplanted, starting at the most posterior point and moving anteriorly until the remnants of the conal septum were reached. For all anastomoses—except for the one between the VSD patch and the area between both roots—self-dissolving monofilament sutures were used to allow for the growth of all structures. Although smaller bites on the root and larger bites on the outflow tract were used to adjust for the persisting mismatch, the VSD patch still needed to be incised, and another patch was used to further enlarge the circumference of the left ventricular outflow tract.  

Reconstruction of the left ventricular outflow tract was then possible. First, the left coronary button was reinserted, followed by the right coronary button. The LeCompte maneuver was performed, and after anastomosing the aortic root to the ascending aorta, the cross-clamp was opened following deairing of the left ventricle. 

To facilitate the anastomosis of the right ventricular outflow tract to the pulmonary root, a pericardial skirt was used to adjust for the discrepancy between the two lumens. The outflow tract was measured again with a probe. In this particular case, for the anastomosis of the pulmonary root to the pulmonary artery, the implantation site of the pulmonary root had to be slightly shifted to the left. Finally, the remaining incision, which was part of the original bifurcation, was closed. 

The child was weaned from cardiopulmonary bypass and was discharged uneventfully two weeks after the operation. 

References

1. Mair R, Kreuzer M, Sames-Dolzer E. En Bloc Rotation of the Outflow Tracts - Double Root Translocation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2024;27:63-68. doi: 10.1053/j.pcsu.2024.01.008. Epub 2024 Jan 23. PMID: 38522875.
2. Kreuzer M, Sames-Dolzer E, Tulzer A, Gierlinger G, Mair R, Nawrozi MP, Mair R. The right age for the en-bloc rotation of the outflow tracts: a single-centre experience. Eur J Cardiothorac Surg. 2023 Jun 1;63(6):ezad065. doi: 10.1093/ejcts/ezad065. PMID: 36810682.
3. Kreuzer M, Sames-Dolzer E, Mair R, Gierlinger G, Tulzer A, Saric D, Mair R. En Block Rotation of the Outflow Tracts: Intermediate Follow-up After 15 Years of Experience. Ann Thorac Surg. 2021 Aug;112(2):603-609. doi: 10.1016/j.athoracsur.2020.06.034. Epub 2020 Aug 20. PMID: 32828753.
4. Hosny H, Sedky Y, Romeih S, Simry W, Afifi A, Elsawy A, Khalek MA, Doss R, Elguindy A, Aguib H, Yacoub M. Revival and modification of the Mustard operation. J Thorac Cardiovasc Surg. 2020 Jan;159(1):241-249. doi: 10.1016/j.jtcvs.2019.03.027.
5. Yacoub MH, Radley-Smith R, Maclaurin R. Two-stage operation for anatomical correction of transposition of the great arteries with intact interventricular septum. Lancet. 1977;1:1275-8.
6. Zayed, Kareem; Alsalakawy, Amr; Afifi, Ahmed; Mahgoub, Ahmed; Hosny, Hatem; Yacoub, Magdi (2024). The New Modified Mustard Operation With an Introduction By Cardiothoracic Pioneer Professor Magdi Yacoub. CTSNet, Inc. Media. https://doi.org/10.25373/ctsnet.28067168.v1

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