Modified Lecompte Combined With Pulmonary Valve Replacement and Reduction Pulmonary Arterioplasty for TOF/APV-Like Syndrome

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A 6-year-old, 24 kg boy with congenital pulmonary valve (PV) stenosis underwent an unsuccessful attempt at transcatheter PV balloon dilation, followed by open pulmonary valvectomy with monocusp PV reconstruction. He had a history of chronic asthma with frequent hospitalizations and has been on medical therapy. He presented recently with exertional fatigue and asthma exacerbation. A chest X-ray revealed hyperinflated lung fields with a large cardiac silhouette and increased pulmonary vascularity.  

Transthoracic echocardiogram showed widely opened pulmonary regurgitation. A preoperative cardiac MRI demonstrated significantly dilated central and main branch pulmonary arteries (PAs) with significant right ventricle enlargement. CT scan showed significant tracheobronchial compression secondary to the significantly dilated branch PAs. 

The decision was made to proceed with a repeat operation. ​Intraoperative bronchoscopy was performed and showed significant tracheobronchial compression. The procedure was performed on a beating heart and included bilateral reduction branch pulmonary arterioplasties and PV replacement with a bioprosthesis. The authors felt that a modified Lecompte maneuver with anterior translocation of the right PA was necessary to relieve the significant airway compression. 

The Surgery 

A repeat median sternotomy was performed, and cardiopulmonary bypass (CPB) was initiated with aortic and single right atrial cannulation. At normothermia and on a beating heart, the main PA was transected, and the branches were dissected thoroughly from hilum-to-hilum. The right and left branch PAs were disconnected, and the right branch was brought anterior to the ascending aorta but remained posterior to the superior vena cava (SVC) in a modified Lecompte maneuver. 

The right PA was then reconnected to the left branch to create a new arterial confluence. Excess PA wall tissue was resected. Reduction arterioplasty was performed for both main branch PAs using a Hegar dilator to adjust their new size and avoid excess narrowing of the branches. A 23 mm aortic bioprosthesis was then sewn in the pulmonary position and secured to the right ventricular outflow tract (RVOT) using running polypropylene sutures. A bovine pericardial patch was then used to reconstruct the anterior wall of the main PA and the RVOT.  

The patient was weaned off CPB without difficulty. Post-bypass evaluation showed a gradient across the right PA as it passed between the SVC and ascending aorta. 

Consequently, the CPB was reinstated, and the right PA was disconnected from the PA confluence and brought lateral to the SVC. This mandated the use of a short piece of an externally reinforced polytetrafluoroethylene (PTFE) graft to connect the right PA to the PA confluence and ensure a tension-free anastomosis. CPB was then weaned for the second time without difficulty, and a transesophageal echocardiogram showed a widely patent RVOT and branch PAs, with the prosthesis well-seated and no periprosthetic regurgitation, and good ventricular function.  

The total CPB time was 126 minutes. The patient was extubated in the operative room and received no transfusions. The remainder of his postoperative course was uneventful, and he was discharged on postoperative day seven. He continues to do well during his follow-up. The follow-up CT scan with 3D reconstruction showed widely patent branch pulmonary arteries with improved tracheobronchial compression​. 

References

1. Yong MS, Yim D, Brizard CP, et al. Long-term outcomes of patients with absent pulmonary valve syndrome: 38 years of experience. Ann Thorac Surg. 2014;97(5):1671-1677. doi:10.1016/j.athoracsur.2014.01.035
2. Swaminathan S, Agarwal A, Infante JC, Rosenkranz E. Tetralogy of Fallot With Absent Pulmonary Valve and Nonconfluent Pulmonary Arteries: A Management Conundrum. World J Pediatr Congenit Heart Surg. 2020;11(4):NP168-NP171. doi:10.1177/2150135118775661
3. Greene CL, Mainwaring RD, Sidell D, Palmon M, Hanley FL. Lecompte Procedure for Relief of Severe Airway Compression in Children With Congenital Heart Disease. World J Pediatr Congenit Heart Surg. 2019 Sep;10(5):558-564

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