Cone procedure for Ebstein's anomaly

Brief Review

Cone procedure for Ebstein’s anomaly of tricuspid valve constructs a funnel like valve using the native valve leaflets. This avoids the problems associated with artificial valves including bioprosthetic valves, especially in children. In one study cone procedure group had 85% reduction in tricuspid regurgitation compared to 56% in the conventional group, at discharge from hospital [1]. The cone procedure involves detachment of tricuspid leaflets, division of chordae, plication of the tricuspid annulus, rotation of leaflets and final re-insertion of the leaflets in the modified tricuspid valve annulus [2].

Since Cone procedure has become an effective hemodynamic intervention for Ebstein’s anomaly, late mortality due to arrhythmias assume more importance. Periprocedural electrophysiological study and ablation of potential arrhythmic circuits have been suggested as a preventive strategy [3]. This aggressive protocol documented significant electrophysiological findings in 69% (29 of 42 patients), including 8 patients in whom there was no prior suspicion of arrhythmia. Seventeen of these patients underwent catheter ablation while the remainder had intra operative interventions. The strategy could prevent sudden death in those treated with this protocol.

Real time intraoperative three dimensional transesophageal echocardiographic guidance for Cone procedure has been used successfully as in case of mitral valve repair [4].

References

  1. Vogel M, Marx GR, Tworetzky W, Cecchin F, Graham D, Mayer JE, Pigula FA, Bacha EA, Del Nido PJ. Ebstein’s malformation of the tricuspid valve: short-term outcomes of the “cone procedure” versus conventional surgery. Congenit Heart Dis. 2012 Jan-Feb;7(1):50-8.
  2. Pizarro C, Bhat MA, Temple J. Cone reconstruction and ventricular septal defect closure for neonatal Ebstein’s anomaly. Multimed Man Cardiothorac Surg. 2012 Jan 1;2012:mms014.
  3. Shivapour JK, Sherwin ED, Alexander ME, Cecchin F, Mah DY, Triedman JK, Marx GR, del Nido PJ, Walsh EP. Utility of preoperative electrophysiologic studies in patients with Ebstein’s anomaly undergoing the Cone procedure. Heart Rhythm. 2014 Feb;11(2):182-6.
  4. Sujatha M, Gadhinglajkar S, Dharan BS, Sreedhar R. Role of Intraoperative Real-Time Three-Dimensional Transesophageal Echocardiography During Cone Procedure for Ebstein’s Anomaly. J Cardiothorac Vasc Anesth. 2014 Dec 19. pii: S1053-0770(14)00659-4. doi: 10.1053/j.jvca.2014.12.023. [Epub ahead of print]