Abstract
ObjectiveEbstein's anomaly remains a relatively ignored disease. Lying in the 'No Man's land' between congenital and valve surgeons, it largely remains inadequately studied. We report our short-term results of treating it as a 'one and a half ventricle heart' and propose that the true tricuspid annulus (TTA) 'Z' score be used as an objective criterion for estimation of 'functional' right ventricle (RV).Methods22 consecutive patients undergoing surgery for Ebstein's anomaly were studied. Echocardiography was performed to assess the type and severity of the disease, tricuspid annular dimension and its 'Z' score. Patients were operated by a modification of the cone repair, with addition of annuloplasty, bidirectional cavopulmonary shunt (BCPS) and right reduction atrioplasty to provide a comprehensive repair. TTA 'Z' score was correlated later with postplication indexed residual RV volume.ResultsThere was one (4.5%) early and no late postoperative death. There was a significant reduction in tricuspid regurgitation grading (3.40±0.65 to 1.22±0.42, P<0.001). Residual RV volume reduced to 71.96±3.8% of the expected volume and there was a significant negative correlation (rho −0.83) between TTA 'Z' score and indexed residual RV volume. During the follow-up of 20.54±7.62 months, the functional class improved from 2.59±0.7 to 1.34±0.52 (P<0.001).ConclusionIn Ebstein's anomaly, a higher TTA 'Z' score correlates with a lower postplication indexed residual RV volume. Hence, a complete trileaflet repair with offloading of RV by BCPS (when the TTA 'Z' score is >2) is recommended. The short-term outcomes of our technique are promising.
Highlights
In 1866, Wilhelm Ebstein described a complex congenital cardiac anomaly during the autopsy of a 19-year-old cyanotic man[1]
There was a significant reduction in tricuspid regurgitation grading (3.40±0.65 to 1.22±0.42, P
Residual right ventricle (RV) volume reduced to 71.96±3.8% of the expected volume and there was a significant negative correlation between true tricuspid annulus (TTA) ‘Z’ score and indexed residual RV volume
Summary
In 1866, Wilhelm Ebstein described a complex congenital cardiac anomaly during the autopsy of a 19-year-old cyanotic man[1]. The complex lesion was named Ebstein’s anomaly and included septal and posterior leaflet adherence to the underlying myocardium with downward displacement of the functional tricuspid annulus, resulting in dilatation of atrialized portion of right ventricle (RV) and true tricuspid annulus (TTA; the right atrioventricular junction)[2]. It is a rare congenital cardiac anomaly, occurring in approximately 1 per 200,000 live births and accounting for
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