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

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.