EBSTEIN'S ANOMALY – OUR EARLY AND MID-TERM RESULTS WITH CONE REPAIR

Abstract

Introduction: Ebstein’s anomaly is a rare disease, comprising less than 1% of all cases of congenital heart disease. Da Silva et al, in 2004, described cone reconstruction of tricuspid valve, which can restore the tricuspid valve anatomy. This repair technique has shown promising early and intermediate results. We present our experience with cone repair done over a period of 6 years at the Department of Cardiothoracic and Vascular Surgery, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi.

            Material and Methods: 7 patients of Ebstein’s anomaly have been treated with cone repair in our hospital from 2017 to 2022. This was a retrospective study, where hospital records of all these patients were retrieved and studied. Patients were either traced from routine OPD follow-up or telephonically. Pre-operative, post-operative and follow-up parameters were analysed using various statistical tools.

            Results: Our study demonstrated good functional outcome after cone repair. There was significant improvement in tricuspid regurgitation (p = 0.0003), trans-annular plane systolic excursion (p = 0.007) and New York Heart Association functional class (p = 0.004).

            Conclusion: Cone repair for Ebstein’s anomaly provides good short and mid-term outcome in experienced hands. The low morbidity and mortality associated with cone repair, in addition to improvement in RV function and TR makes it the procedure of choice for Ebstein’s anomaly, irrespective of the anatomical type. However, long term studies with larger cohorts can provide better insight into the outcome of this novel procedure.