Outcomes of the Fontan operation in patients with Ebstein anomaly: An Australia and New Zealand Fontan registry study

Abstract

OBJECTIVES: We sought to review the outcomes of patients with Ebstein 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. 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 = .01) after Fontan operation and lower freedom from Fontan failure (P = .004) compared with 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 = .76 and P = .69, respectively), although comparison was hindered by low numbers. Of the 7 patients with congenitally corrected transposition of great arteries and EA, there were no mortalities; however, 2 patients had Fontan failure at 7.0 and 9.8 years post-Fontan. CONCLUSIONS: Patients with EA have worse long-term outcomes after the Fontan operation compared with 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.