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. 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. 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.

Anatomically corrected malposition of the great arteries is a rare conotruncal anomaly that is often confused with congenitally corrected transposition of the great arteries. This case report describes the successful surgical management of an adolescent with severe right ventricular outflow obstruction caused by accessory tricuspid valve tissue in the presence of situs inversus with atrioventricular and ventriculoarterial concordance with malposed great arteries (anatomically corrected malposition) {I,L,D}.

Accessory tricuspid valve tissue is a rare entity associated with congenital cardiac anomalies. We report a seven-month-old child who underwent ventricular septal defect closure with excision of an accessory tricuspid valve leaflet and discuss their morphology, clinical presentation, diagnostic modalities, and treatment options. doi: 10.1111/jocs.12731 (J Card Surg 2016;31:324-326).

Ventricular septal defect (VSD) in Tetralogy of Fallot (TOF) is usually large and non-restrictive with equalization of right and left ventricular pressures. Restrictive VSD in TOF is rare. We present an unusual case of TOF with restriction to VSD caused by accessory tricuspid valve tissue that varied with respiration.