Original article
Congenital heart surgery
Improving Results of Surgery for Ebstein Anomaly: Where Are We After 235 Cone Repairs?

Presented at the Fifty-third Annual Meeting of The Society of Thoracic Surgeons, Houston, TX, Jan 21–25, 2017.
https://doi.org/10.1016/j.athoracsur.2017.09.058Get rights and content

Background

Ebstein anomaly has heterogeneous anatomy and numerous operative techniques are described. Cone repair provides a near anatomic tricuspid valve repair. The purpose of this study was to examine our experience with cone repair.

Methods

Cone repair was performed in 235 consecutive patients with Ebstein anomaly, 134 children (57%) and 101 adults (43%), from June 2007 to October 2015. Median age was 15.6 years (range, 6 months to 73 years). Cone repair was the first operation in 192 patients (82%), the second in 41 (17%), and the third in 2 (1%). Previous tricuspid valve repair had been performed in 27 (12%). Echocardiograms were obtained preoperatively and at hospital dismissal for all patients and for a subgroup of patients at least 6 months after cone repair (n = 81).

Results

Leaflet augmentation was done in 67 patients (28%), Sebening stitch in 57 (24.2%), neochordae in 49 (21%), and annuloplasty band in 158 (67%). Bidirectional cavopulmonary shunt was performed in 46 patients (20%). There was 1 early death (0.4%). Early reoperation was required in 14 patients (5.9%); re-repair was possible in 7 (50%). The majority of early reoperations (11 of 14; 79%) occurred in the first third of the series. Mean follow-up was 3.5 ± 2.5 years. There was sustained reduction in tricuspid regurgitation (p < 0.0001), a progressive decline in right ventricle size (p < 0.0001), and late increase in right ventricle fractional area change after initial decline (p < 0.0001). Freedom from late reoperation was 97.9% at 6 years.

Conclusions

Cone repair is safe, and the learning curve is significant. Sustained reduction in tricuspid regurgitation and favorable changes in the right ventricle at follow-up suggest that cone repair has an advantageous impact on right ventricular remodeling.

Section snippets

Patients and Methods

After approval by the Mayo Clinic Institutional Review Board (February 2016), we searched our surgical database for patients with Ebstein anomaly who underwent cone repair from June 20, 2007, to October 6, 2015.

Operative Details and Early Results

Operative procedures and perioperative data are summarized in Table 3. Cone repair was undertaken as a TV re-repair in 27 patients (11.5%). There was 1 early death, of a 50-year old man with endocarditis preoperatively related to pacemaker leads. Despite antibiotic coverage and successful cone repair with BCPS, he had low output syndrome, required extracorporeal membrane oxygenation postoperatively, had a stroke, and died 38 days after operation.

Extracorporeal membrane oxygenation was required

Ideal Repair Is Anatomic Repair

The initial repair techniques for Ebstein anomaly focused on TV monocusp repair [11]. The Carpentier technique 12, 13 incorporated surgical delamination of the anterior leaflet with annular reattachment so monocusp mobility was optimized for ventricular septal coaptation in contrast to the Danielson method, which did not incorporate surgical delamination but rather anterior papillary muscle approximation to the ventricular septum (modified Sebening stitch) [14]. Although monocusp repairs were

References (21)

There are more references available in the full text version of this article.

Cited by (74)

  • Ebstein Anomaly: We Should Do Better

    2023, Journal of the American College of Cardiology
  • Electrophysiology and surgery intertwined in complex treatment of Ebstein's anomaly in childhood

    2023, HeartRhythm Case Reports
    Citation Excerpt :

    Ebstein’s anomaly, a rare and highly variable congenital heart defect,1 still presents a treatment challenge. The currently used cone repair of the tricuspid valve has carried favorable results in suitable patients.2 Arrhythmogenic substrates including accessory pathways3,4 and right bundle branch block5 associated with electromechanical ventricular dyssynchrony present additional therapeutic targets.

View all citing articles on Scopus
View full text