Abstract 18937: Ebstein’s Anomaly in Adults - Favourable Outcomes from a Single Specialist Centre Experience

Introduction: Ebstein’s anomaly (EA) accounts for less than 1% of all congenital heart disease (CHD) and outcome data in adults is scarce. In our adult CHD centre, we have practised an uniform approach of “watchful waiting” with surgery reserved for either those with refractory arrhythmia or worsening dyspnoea, with lower threshold for cases with tricuspid valves amenable to repair (rather than require replacement), especially with adequate biventricular volumes. We aimed to evaluate the long-term outcomes of our EA patients managed with this approach. Methods: All patients with EA and normal cardiac connections reviewed at least once in the adult CHD clinic were included. Outcome data was ascertained by reviewing the National Deaths registry and medical records. Results: 51 EA patients (17 males, median age at diagnosis 13 years (0-63), mean age at first adult CHD clinic review 33+/-13 years) were followed for a mean period of 21+/-14 years. Of these, 18 patients (35%) had supraventricular arrhythmia with Wolff-Parkinson-White syndrome in 5 patients (10%). 16 patients (31%) underwent catheter ablation with sustained freedom from arrhythmia in 9 (56%) patients. Five patients (10%) required pacemaker implantation. 16 patients (31%) underwent tricuspid valve (TV) surgery (9 repair and 7 replacement). 7 patients had anti-arrhythmia surgery at the time of TV surgery, with re-do surgery required in 3. Small LV (defined as end-diastolic diameter <40mm in adulthood) was present in 20 patients. There were three deaths; a 58 y.o. female with cardiogenic shock after third time sternotomy and valve surgery, a 45 y.o. female with progressive heart failure and a 72 y.o. male with mild EA and unknown cause of death. Kaplan-Meier analysis showed 100% survival to age 40, 95% to age 50 and 81% to age 60. Conclusion: EA in adulthood often has severe morphological abnormalities but with a conservative management strategy, we demonstrate good medium-term survival. ![][1] [1]: /embed/graphic-1.gif