Papillary Muscle Approximation to the Interventricular Septum in Cone Reconstruction of Ebstein’s Anomaly

Cone reconstruction of the tricuspid valve for Ebstein’s anomaly is a significant innovation in the management of Ebstein’s anomaly, and has shown favorable results (1). This repair entails significant delamination of tethered papillary muscles, with persistent significant right ventricular dilatation. Techniques for remodeling the right ventricle may improve long-term outcomes. Papillary muscle repositioning has been proposed to relieve tethering in adult functional mitral regurgitation. This video describes the technique of right ventricular papillary muscle approximation to the septum (RV-PMA) as an adjunct to cone reconstruction of Ebstein’s anomaly, and reviews some of the authors’ initial results.

This is the case of a 3-year-old child with Ebstein’s anomaly, severe tricuspid regurgitation, and severe right atrial and right ventricular dilatation. The patient had a history of atrial tachyarrythmias and attempted ablation, and was stable on medications. The heart was accessed through a lower midline sternotomy, and cardiopulmonary bypass was instituted with aortic and bicaval cannulation. The anterior leaflet was detached off its hinge points circumferentially to the inferior leaflet and tethering connections were taken down. The septal leaflet was delaminated off the septum and mobilized towards the atrioventricular groove. RV-PMA consisted of suturing the anterior papillary muscle to the ventricular septum, so as to bring the papillary muscle and anterior leaflet free edge closer to the posterior and septal leaflets, relieve tethering, and avoid delamination of the papillary muscles all the way to the apex. The anteroseptal commissure was closed, and the inferior leaflet was rotated and sewn side-to-side to the septal leaflet. The annulus was plicated anteriorly and inferiorly. The valve was then reattached to the atrioventricular groove. The valve was tested and functioned well by water insufflation and transesophageal echocardiography.

RV-PMA is simple, safe, and can improve coaptation, tricuspid valve function, and RV remodeling as an adjunct to cone reconstruction in Ebstein’s anomaly. Further evaluation and longer follow-up is currently ongoing.

References

1. Da Silva, J. P., Baumgratz, J. F., Da Fonseca, L., Franchi, S. M., Lopes, L. M., Tavares, G. M. P., et al. The cone reconstruction of the tricuspid valve in Ebstein’s anomaly. The operation: early and midterm results. The Journal of Thoracic and Cardiovascular Surgery, 2006;133(1):215–223. http://doi.org/10.1016/j.jtcvs.2006.09.018
2. 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;7(1):50-8. doi: 10.1111/j.1747-0803.2011.00603.x.

This video was presented at the AATS 2016 meeting in Baltimore, MD, USA.