Objectives: The aim of this study was to evaluate the outcome in a series of patients with Ebstein's anomaly and a failing right ventricle (RV) undergoing tricuspid valve (TV) repair and bidirectional Glenn cavopulmonary anastomosis (BDG).

Materials And Method: Between January 2006 and September 2013, 11 consecutive patients diagnosed with severe forms of Ebstein's anomaly and a failing RV underwent TV surgery and BDG. The mean age was 16.5 ± 7 years. Most frequently found symptoms were cyanosis, dyspnea, and arrhythmias. The azygos or hemiazygos veins were left open. The TV was repaired using the "peacock tail" technique, which consisted of total detachment of the anterior and posterior leaflets of the TV and rotation in both directions reimplanting them to the true annulus. The mean follow-up was 3.8 ± 2.4 years (range three months to six years).

Results: Hospital mortality was 9% (one patient). TV repair was possible in 10 patients. None of the patients had AV block postoperatively. At one year after surgery, the indexed RV and RA diameter were reduced significantly versus the preoperative data (p = 0.003 and p < 0.001). The mean TVR and indexed TV area were 1.2 ± 0.42 and 1.6 ± 0.6 (mm/m2), significantly lower than preoperatively (p = 0.001 and p = 0.008, respectively). The mean NYHA functional class, SaO2 , and cardiothoracic ratio were significantly improved.

Conclusions: The peacock tail technique for TV repair in combination with BDG in patients with Ebstein's malformation and depressed RV function results in TV preservation, a low incidence of recurrent regurgitation, favorable functional status and RV function, and resolution of cyanosis.

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http://dx.doi.org/10.1111/jocs.12321DOI Listing

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