Tetralogy of Fallot With Absent Pulmonary Valve: Repair by Anterior Translocation of Branch Pulmonary Arteries and Reduction Plasty

Tetralogy of Fallot with an absent pulmonary valve is physiologically distinctive from other forms of TOF due to tracheobronchial compression resulting from massive dilatation of the main pulmonary artery and branches and abnormal branching of segmental arteries. Symptomatic newborns and infants have a poor prognosis because of severe central pulmonary artery dilatation and bronchial compression.

One-stage early primary repair is the method of choice. Surgery must address the dilated pulmonary arteries to minimize the incidence of bronchial compression and peripheral lung damage. The traditional approach has been plication and reduction of the anterior and/or posterior wall of the normally positioned pulmonary arteries.

This alternative approach involves translocation of the pulmonary artery anterior to the aorta away from the trachea and bronchial tree, which may reduce or eliminate bronchial compression by the pulmonary artery.

This video demonstrates surgery for tetralogy of Fallot with absent pulmonary valve (TOF, APV) using anterior translocation of branch pulmonary arteries (the LeCompte maneuver) and reduction plasty. 



A three-month-old infant weighing 4 kg presented with respiratory distress. Echocardiography confirmed the diagnosis of tetralogy of Fallot. A CT angiogram demonstrated large, dilated branch pulmonary arteries with evidence of left bronchial compression. 

Under standard cardiopulmonary bypass, the procedure began with extensive mobilization of branch pulmonary arteries on either side followed by transection of the ascending aorta above the sinotubular junction and transection of the main pulmonary artery. Next, the LeCompte maneuver was performed with translocation of the branch pulmonary arteries anteriorly. The branch pulmonary arteries were then further mobilized to the lobar branches on either side.

Next, the muscle bundles in the right ventricular outflow tract (RVOT) were resected and a limited transannular ventriculotomy was performed. Aortic reconstruction was then performed using end-to-end anastomosis and the ventricular septal defect was closed. Anastomosis of the RVOT to the transected confluence was then completed and surgeons removed a part of the anterior wall of branch pulmonary arteries on either side.

Next, plication of the posterior wall was completed and the anterior wall was sutured back in place. A bicuspid pulmonary valve was placed in the RVOT with a polytetrafluoroethylene (PTFE) membrane. Finally, the anterior wall was reconstructed using a patch of autologous pericardium. A transesophageal echocardiogram was then completed to evaluate the adequacy of the repair.

This technique ensures not only an effective reduction plasty of the branch pulmonary arteries by anterior resection and posterior plication, but also brings them anteriorly away from the bronchi, thereby relieving the bronchial compression. The valve in the RVOT takes care of the pulmonary regurgitation.

References

1. Hraska V. A New Approach to Correction of Tetralogy of Fallot With Absent Pulmonary Valve . Ann Thorac Surg 2000;69:1601–3
2. Hraska V, Photiadis J, Schindler E, Sinzobahamvya N, Fink C, Haun C, Schneider M, Blaschczok HC, Asfour B. A novel approach to the repair of tetralogy of Fallot with absent pulmonary valve and the reduction of airway compression by the pulmonary artery. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009:59– 62. doi: 10.1053/j.pcsu.2009.01.022. PMID: 19349015.

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