Cone Reconstruction in Children with Ebstein Anomaly: The Mayo Clinic Experience

Abstract

CR in children and young adults with Ebstein anomaly can be performed with low early mortality and excellent durability at short-term follow-up. CR represents an important surgical option for young patients. It is applicable to patients with a broad range of anatomic variability and precludes valve replacement in the vast majority. CR should be considered prior to the deleterious effects of chronic right ventricular volume overload and the development of systolic dysfunction, which hamper long-term prognosis. Therefore, early referral for surgical evaluation is recommended.

Cone Reconstruction of Atypical Ebstein Anomaly Associated with Right Ventricular Apical Hypoplasia

“…Data from our institution have shown promising early results after CR. 1 The procedure, which involves reconstruction of the TV at the true anatomic annulus, imparts valvular function that is more like normal valvular anatomy than the function imparted by any previously described technique. 1 The CR technique can be applied to many anatomic variations of Ebstein anomaly, as in this instance of atypical Ebstein anomaly with RV apical hypoplasia.…”

“…1,2 We present the apparently unique case of a patient with atypical Ebstein anomaly and right ventricular (RV) apical hypoplasia who was spared palliative shunt surgery by our use of CR.…”

Arrhythmia after cone repair for Ebstein anomaly: The Mayo Clinic experience in 143 young patients

“…The current approach to intervention on the tricuspid valve in EA is the cone repair (CR) as first described by da Silva and adapted by Dearani to provide a more physiologic repair than prior techniques . Successful CR has been reported to be as high as 98% in young patients with EA who presented for surgery . This has led to earlier valve repair in many patients.…”

Management of Ebstein’s anomaly

“…A thin walled RV with poor function is a preoperative indication that a superior cavopulmonary shunt may be needed. If there is significant tricuspid stenosis or failure to wean from bypass with an LA pressure less than 10 and CVP greater than 15, with low systemic pressures, than a superior cavopulmonary shunt is performed (8). We prefer to do selective right sided cryo-ablation-Maze for patients with documented paroxysmal flutter or inducible atrial arrhythmias.…”