Fourth Sternotomy for Late Sequelae Post-Repair TOF/PA With Criss-Cross PA

The authors present the surgical management of four different late sequelae post-repair of tetralogy of Fallot with pulmonary atresia and criss-cross pulmonary arteries.

The patient was an 11-year-old, 33.5 kg girl who was born with cyanosis due to pulmonary atresia and ventricular septal defect. She underwent an initial central shunt in the neonatal period, followed by complete repair at 7 months of age with placement of a 14 mm pulmonary homograft. Two years later, a stent was placed in her pulmonary conduit and at the age of 4 years, and she underwent a third-time sternotomy for explanation of the stent and augmentation of the right ventricular outflow tract (RVOT), but no prosthesis was placed at that time.

She presented with exertional shortness of breath that was worsening and frequent premature ventricular contractions (PVCs). Echocardiography revealed residual ventricular septal defect (VSD) at the superior margin of the patch, which was known previously and it was thought to be small. There was right ventricular chamber enlargement and free pulmonary regurgitation with a gradient of over 60 mm Hg across the conduit. Holter monitoring showed nonsustained ventricular tachycardia and frequent PVCs. Computed tomography scan showed multilevel obstruction in the RVOT, at the level of the conduit and right pulmonary artery. She also had criss-cross pulmonary arteries with a superior/inferior relationship between the left and right branches. On cardiac MRI, the right ventricular end-diastolic volume index was 177 ml/m2.

The decision was made to proceed with a fourth-time sternotomy to address all the current issues. Intraoperative transesophageal echocardiography showed the residual shunt to measure a little over 4 mm, and two jets were demonstrated at the superior margin of the patch. This was most likely an intramural VSD type. Cardiopulmonary bypass was initiated via central aortic and bicaval cannulation. After cardioplegic arrest, the RVOT was opened and cryoablation was performed to address the PVC burden. Three lesion sets were performed, each for 90 seconds at -60oC using a cryoprobe. The lesions were performed along the right ventricular free wall, vertically from the ventricular septum to the ventricular septal defect patch and along the pulmonary annulus. The authors could not visualize the residual shunt through the RVOT, so a transaortic approach was utilized. Multiple pledgeted 5/0 prolene sutures were placed in a horizontal mattress fashion from the superior margin of the previously placed VSD patch to the base of the right coronary sinus of Valsalva, and sutures were tied inside the sinus at the base of the right coronary cusp. The aortic transection enabled the authors to access the right main branch pulmonary artery easily, so it was augmented along its entire length up to the lobar branches using an appropriately sized photofix bovine pericardial patch. The aorta was reconstructed and standard pulmonary prosthesis (23 mm Inspiris Resilia) was placed in the RVOT and a second pericardial patch was placed to augment the main pulmonary artery and the RVOT and secure the prosthesis. She was then weaned off cardiopulmonary bypass without difficulty.

The patient was extubated in the operating room, received no transfusions, and the remaining postoperative course was uneventful. She was discharged three days later. Her PVCs burden was reduced down to 1.5% (20% preoperatively).

In conclusion, elimination of multilevel RVOTO should be the rule in reoperations to guarantee optimal hemodynamics, cryoablation of the RVOT should have its place during reoperations after initial repair of TOF, and transaortic access to residual VSD should be strongly considered for residual shunts after initial repair due to difficulty in assessment of the magnitude of the shunt.

References

1. Shin HJ, Song S, Shin YR, Park HK, Park YH. Concomitant right ventricular outflow tract cryoablation during pulmonary valve replacement in a patient with tetralogy of Fallot. Korean J Thorac Cardiovasc Surg. 2017 Feb;50(1):41–43
2. Belli E, Houyel L, Serraf A, Lacour-Gayet F, Petit J, Planché C. Transaortic closure of residual intramural ventricular septal defect. Ann Thorac Surg. 2000 May;69(5):1496-1498
3. Patel JK, Glatz AC, Ghosh RM, Jones SM, Natarajan S, Ravishankar C, et al. Intramural ventricular septal defect Is a distinct clinical entity associated with postoperative morbidity in children after repair of conotruncal anomalies. Circulation. 2015 Oct 13;132(15):1387-1394.

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