Outcomes of the Fontan Operation in Patients with Ebstein’s Anomaly: Findings from a Binational Fontan Registry

Abstract

Objectives: We sought to review the outcomes of patients with Ebstein’s anomaly (EA) after the Fontan operation. Methods: Patients with EA were identified from a large binational registry about the Fontan operation. Data were collected from hospital records, registry data, and clinical correspondence. Results: Of the 1601 patients who underwent a contemporary Fontan operation from 1991 to 2023, 34 patients had EA. Seven patients (21%) had concomitant congenitally-corrected transposition of great arteries (ccTGA). Prior Starnes procedure was performed in 18 (53%) patients. Survival after Fontan operation in patients with EA was 92% (95%CI: 70%-98%) and freedom from Fontan failure was 80% (95%CI: 53%-92%) at 10 years. Patients with EA had worse long-term survival (p=0.01) after Fontan operation and lower freedom from Fontan failure (p=0.004) compared to other patients with left-ventricle dominance. Patients with EA, who underwent prior Starnes procedure, had 100% survival and freedom from Fontan failure, albeit at a shorter follow-up (median 4.2 years, range 13 days – 17.7 years), with no difference between patients with prior Starnes and patients with tricuspid atresia (p=0.76 and p=0.69, respectively), although comparison was hindered by low numbers. Of the 7 patients with ccTGA and Ebstein’s anomaly, there were no mortalities, however, 2 patients had Fontan failure at 7.0 and 9.8 years post-Fontan. Conclusion: Patients with EA have worse long-term outcomes after the Fontan operation, as compared to other patients with left ventricular dominance. Patients with a prior Starnes procedure appear to have good post-Fontan outcomes, although bias may occur due to small numbers