Tetralogy of Fallot with restrictive ventricular septal defect by accessory tricuspid valve: repair with accessory valve preservation.

BACKGROUND

Restrictive ventricular septal defect resulting in suprasystemic right ventricular pressure is a rare entity in tetralogy of Fallot patients. It is often caused by accessory tricuspid valve tissue protruding through the defect. Surgical resection of the accessory tricuspid valve has been recommended, but the risk of tricuspid regurgitation caused by the resection has not been widely discussed.

CASE PRESENTATION: A 5-month-old male who had been diagnosed with tetralogy of Fallot was referred for progressive decline in oxygen saturation. Preoperative echocardiogram revealed restrictive ventricular septal defect and severe pulmonary stenosis, suggesting suprasystemic right ventricular pressure (> 160 mmHg). Intraoperatively, accessory tricuspid valve tissue was found to be attached to the superior edge of the ventricular septal defect and partially occlude the aortic valve. The risk of tricuspid regurgitation caused by accessory tricuspid valve resection was high due to the complicated relationship between the true tricuspid valve and the accessory tricuspid valve. Therefore, the accessory tricuspid valve was preserved on the right ventricular side of the ventricular septum to avoid the risk of tricuspid regurgitation. Postoperative echocardiogram did not show tricuspid regurgitation or left ventricular outflow tract obstruction, and the patient was discharged to home on postoperative day 35 without major complications.

CONCLUSIONS

We herein reported a high-risk patient of tetralogy of Fallot with restrictive ventricular septal defect caused by accessory tricuspid valve who successfully underwent definitive repair with accessory tricuspid valve preservation considering the risk of tricuspid regurgitation.