Does addition of bi-directional cavo-pulmonary shunt to tricuspid repair in advanced cases of Ebstein anomaly result in better outcomes?

OBJECTIVES

Ebstein anomaly can be managed by single ventricular, bi-ventricular and one and a half ventricular repairs. We present midterm results of Comprehensive Tricuspid Valve repair (CTVR) with bi-directional cavo-pulmonary shunt (BCPS).

METHODS

In this prospective observational study (Jan2012-July2018), 69 patients underwent surgery for Ebstein anomaly. In Group I (n = 48; 69.6%), all patients got CTVR and a BCPS (one and a half ventricle repair). Group II (n = 15; 21.8%) consisted of a similar repair without BCPS (bi-ventricle repair). All patients were echocardiographed at six monthly intervals.

RESULTS

Median age of the cohort was 17 years (range 1-68). 12 (17.4%) patients were Carpentier type B, 51 (73.9%) were type C and 6 (8.7%) were type D. There were two early mortalities (2.89%). At a mean follow up of 3.2 ± 1.2 years, there were no late deaths and one delayed repair-failure in each group. Group I had significantly lower mean TR grade (1.2 ± 0.4vs1.6 ± 0.5, p = 0.03) as compared to Group II without a significant difference in the mean gradients (1.5 ± 0.5vs1.6 ± 0.6, p = 0.4). Mean indexed TAPSE (15.0 ± 6.7vs.16.6 ± 5.6 mm/m2, p = 0.21), NYHA class (1.2 ± 0.4vs1.3 ± 0.4) and six-minute walk distance (506 vs 507 m, p = 0.7) was similar in both groups.

CONCLUSION

One and a half ventricle repair of Ebstein anomaly gives a more functionally competent, non-stenotic and durable tricuspid valve as compared to a two-ventricle repair. BCPS doesn't result in facial swelling or AV malformations. Preload reduction by BCPS may allow the myopathic ventricle to remodel.