The cone procedure to ebstein's anomaly repair: clinical and echocardiographic outcomes

ABSTRACT

INTRODUCTION: Ebstein's anomaly occurs due to failure in the delamination of the septal and posterior leaflets with apical displacement of the functional annulus, dilatation of the atrialized portion of the right ventricle, thinning of the free wall, dilatation of the true annulus and redundant anterior leaflet with multiple fenestrations. The "Cone" reconstruction of the tricuspid valve, described by Silva et al, results in complete coaptation of the valves with excellent results in the medium and long term. OBJECTIVES: In this study, clinical and echocardiographic data of patients undergoing surgical repair using this technique was reviewed. METHODS: Pre and post-operative (PO) records and echocardiograms of 8 patients operated on from 2018 to 2022 were reviewed. RESULTS: The median age of the group was 22.5 (2 to 42 years). In the pre-operative 3 patients had Wolf-Parkinson-White ventricular pre-excitation and 6 had atrial septal defect associated, 6 had moderate to severe dyspenia, with only one being cyanotic. All had severe tricuspid regurgitation (TI) and 6 had moderate to severe right ventricular dilatation. In the immediate PO, moderate TI was detected in 2 and mild in 6. In 1 patient, Glenn anastomosis was added to the Cone procedure due to hemodynamic instability and hypoxemia immediately after bypass. In the post-operative follow-up time, median of 11.5 months, all patients were asymptomatic and TI increased from mild to moderate in only one case. One patient required pacemaker implantation due to complete atrioventricular block. CONCLUSION: In this small cohort, the Cone procedure resulted in significant improvement of the tricuspid regurgitation, no mortality and low morbidity with significant improvement of the clinical status.