Surgical Strategy and Outcomes in Prenatally Diagnosed Neonates With Epstein's Anomaly and Circular Shunt

This study was performed to evaluate surgical outcomes in patients prenatally diagnosed with severe Ebstein's anomaly and a circular shunt.

This retrospective study included 13 patients diagnosed with severe Ebstein's anomaly accompanied by a circular shunt on foetal echocardiography between 2012 and 2024 at our institution. All patients were delivered by planned caesarean section. Foetal hydrops was present in 4 patients. At birth, chest radiographs showed cardiothoracic ratios ranging from 90% to 100%, and echocardiography demonstrated severe pulmonary and tricuspid regurgitation in all cases. Surgical strategies-including main pulmonary artery ligation and the modified Starnes procedure-were evaluated based on perioperative clinical course, incorporating chest X-rays, echocardiograms, laboratory data, and urine output. The median follow-up after the modified Starnes procedure was 3.46 years (interquartile range, 2.9 years).

All patients underwent surgical intervention on the day of birth: main pulmonary artery ligation in 7 and a modified Starnes procedure in 6. Of those who initially underwent ligation, 4 patients required conversion to the modified Starnes procedure on postoperative day 1, 2 patients on day 3, and 1 patient on day 6. One patient died of infection after the Glenn procedure. Of the remaining 12 patients, 7 completed Fontan circulation, and 5 were awaiting the Fontan. The 5-year postoperative survival rate was 92.3%.

Surgical outcomes for severe Ebstein's anomaly with a circular shunt were excellent. However, the main pulmonary artery ligation as an initial palliation did not stabilize haemodynamics. Early implementation of the modified Starnes procedure appears critical to overcoming the haemodynamically unstable period immediately after birth.