Early timing of surgical intervention in patients with Ebstein's anomaly predicts superior long-term outcome☆

Abstract

Repair, as opposed to replacement, is feasible in the vast majority of patients presenting with Ebstein's anomaly with a low early mortality rate. Outcome, in terms of survival and freedom from re-operation in the long term is determined by the clinical state at the time of surgery. Therefore, timely operation is warranted before significant cardiomegaly develops and functional status deteriorates.

Behavior of Ebstein’s Anomaly: Single-Center Experience and Midterm Follow-Up

“…The mean time between initial surgery and redo in our series was 13.4 ± 10.6 years. Re-operation rates of 9 and 20% were reported by Chauvaud [5] and Badiu et al [8], respectively. Long-term follow-up in operated patients with Ebstein’s anomaly is mandatory, as redo surgery in adulthood is not rare.…”

“…The reoperation rate in this series was 9% [5]. Today, early mortality rates as low as 1.6% have been reported [8]. The majority of patients in our study underwent valve repair.…”

“…There was no significant difference in survival between the group with valve repair and the group with valve replacement. It is unclear whether tricuspid valve repair or valve replacement is superior in the long-term outcome [3,8,9]. However, Chauvaud [5] suggests a superiority of tricuspid valvuloplasty associated with a longitudinal right ventricular plication over valve replacement.…”

“…As our study consisted of a relatively low number of patients (n = 49), we presume it is underpowered to detect predictors of surgery. However, other series showed that the need for surgery is an individual matter, primarily based on functional status [5,8,9]. There is a benefit from early surgical therapy regarding survival and freedom from redo surgery in the long term [8].…”

Impact of Surgery in Ebstein’s Anomaly Using Current Surgical Criteria

“…Patients who had surgery at institutions other than the present one, and for whom data on the surgery, or echocardiography or clinical records were ity. 8 They suggested that, even in an asymptomatic or minimally symptomatic patient, early surgery must be performed before cardiomegaly develops and functional class worsens. In contrast, other authors consider that surgery should be delayed until symptoms appear, cyanosis becomes evident or paradoxical embolism occurs.…”