Cone Repair for Ebstein's Anomaly in a Ten-Year-Old Girl

The patient was a ten-year-old, 43.3 kg girl who had a well-known diagnosis of Ebstein's anomaly and underwent a previous electrophysiologic study with catheter ablation of an accessory conduction pathway because of Wolff-Parkinson-White syndrome. She presented with exertional fatigue and increased work of breathing. A transthoracic echocardiogram showed severe tricuspid valve regurgitation and typical features of Ebstein's anomaly. There was significant enlargement of the right atrial and right ventricular chambers, in addition to a patent foramen ovale.

The Surgery

The decision was made to proceed with repair. Through a median sternotomy and aortic and bicaval cannulation, cardioplegic arrest was achieved and the tricuspid valve was assessed via an oblique right atriotomy. The tricuspid valve showed displacement of the septal leaflet and failure of delamination of all three leaflets. The anterior and inferior leaflets were heavily muscularized with no true chordal structures. The steps of the cone repair were followed, starting by detaching the anterior leaflet from its attachment, followed by completion of the delamination process for all three leaflets in a 360-degree fashion. The atrialized portion of the right ventricle was plicated with a two-layer running polypropylene suture, followed by reduction of the tricuspid valve annulus using multiple interrupted pledgeted polypropylene sutures in a horizontal mattress fashion. The mobilized anterior/inferior leaflet complex was then rotated and sewn to the septal leaflet to complete the reconstruction of the cone, which was subsequently reattached to the true tricuspid valve annulus using a combination of interrupted and running sutures. The valve was tested along this process multiple times and all fenestrations were closed to improve its competence.

The patent foramen ovale was then closed with a single 5-0 pledgeted polypropylene suture. The heart was de-aired, and the aortic cross-clamp was removed. The patient regained her normal sinus rhythm. A strip of the free wall of the right atrium was then resected, followed by closure of the right atriotomy and removal of caval snares. The patient was then ventilated and weaned off cardiopulmonary bypass without difficulty.

A post-bypass transesophageal echocardiogram showed good biventricular function with mild tricuspid valve regurgitation. The surgeons were satisfied with these results. The patient was decannulated and hemostasis was achieved, followed by chest closure in the standard fashion. She was extubated in the operating room and received no transfusion. The aortic cross-clamp time was 128 minutes, and the cardiopulmonary bypass time was 163 minutes.

The remaining postoperative course was uneventful, and she was discharged four days later.

A follow-up echocardiogram continued to show mild tricuspid regurgitation and good ventricular function.
 

References

1. Dearani JA, Said SM, O'Leary PW, Burkhart HM, Barnes RD, Cetta F. Anatomic repair of Ebstein's malformation: lessons learned with cone reconstruction. Ann Thorac Surg. 2013 Jan;95(1):220-6; discussion 226-8. doi: 10.1016/j.athoracsur.2012.04.146. Epub 2012 Nov 28.
2. Anderson HN, Dearani JA, Said SM, Norris MD, Pundi KN, Miller AR, Cetta ML, Eidem BW, O'Leary PW, Cetta F. Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience. Congenit Heart Dis. 2014 May-Jun;9(3):266-71. doi: 10.1111/chd.12155. Epub 2013 Dec 23/span>
3. Holst KA, Dearani JA, Said S, Pike RB, Connolly HM, Cannon BC, Sessions KL, O'Byrne MM, O'Leary PW. Improving Results of Surgery for Ebstein Anomaly: Where Are We After 235 Cone Repairs? Ann Thorac Surg. 2018 Jan;105(1):160-168. doi: 10.1016/j.athoracsur.2017.09.058. Epub 2017 Nov 24.
4. da Silva JP, Baumgratz JF, da Fonseca L, Franchi SM, Lopes LM, Tavares GM, Soares AM, Moreira LF, Barbero-Marcial M. The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg. 2007 Jan;133(1):215-23. doi: 10.1016/j.jtcvs.2006.09.018. Epub 2006 Dec 4.

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The author S.M.S is a consultant for Stryker, Abbott, and Artivion.

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