Novel Approach to Repair of Tetralogy of Fallot With Absent Pulmonary Valve Syndrome and Severe Airway Compression

Objectives

Tetralogy of Fallot (TOF) with absent pulmonary valve syndrome and massively dilated pulmonary arteries may present with severe airway compression. Neonates who present with severe airway compression are difficult to manage and have a high mortality rate. The traditional approach of ventricular septal defect (VSD) closure and pulmonary artery plication is often insufficient to relieve severe airway compression. The authors present a new approach to relieving severe airway compression in TOF with absent pulmonary valve and massively dilated pulmonary arteries.

Methods

A 3.2 kg neonate was born at 39 weeks gestation with TOF with absent pulmonary valve syndrome and massively dilated pulmonary arteries. He was born in severe respiratory distress requiring immediate intubation. He was taken for operative repair at two days of life.

Results

The patient underwent four critical steps to alleviate his severe airway compression.

1. The VSD was closed to reduce the volume load on the heart.
2. The pulmonary arteries were plicated to reduce their size and relieve wall tension.
3. A competent pulmonary valve was placed to eliminate the to-and-fro motion of the main pulmonary artery.
4. A Le Compte maneuver was performed to move the pulmonary artery away from the bronchus.

The patient underwent delayed sternal closure on postoperative day seven. He was extubated on postoperative day 11. The first oral feed was on postoperative day 22. The patient was discharged home on postoperative 34. At the time of video preparation, he was at home on nasal cannula.

Conclusion

The traditional approach of VSD repair and pulmonary artery plication may not provide sufficient relief of airway compression in TOF with absent pulmonary valve syndrome and massively dilated pulmonary arteries. In this situation, two additional measures of placement of a competent pulmonary valve and a Le Compte maneuver may be utilized to improve airway compression and allow for extubation.

This educational video was originally presented during the STSA 65th Annual Meeting. This content is published with the permission of the STSA. For more information on the STSA and its next Annual Meeting, please click here.