Clinical Spectrum and Long-term Outcome of 183 Cases of Ebstein's Anomaly, Experience of Mexican Population.

BACKGROUND: Ebstein's anomaly (EA) is a myopathy of the right ventricle that causes a variable spectrum of tricuspid valve delamination failure with diverse clinical and anatomical presentation. We reviewed our data of EA to establish an association between clinical and echocardiographic findings with mortality. METHODS: We divided patients in infants, Children/adolescents (Ch/A), and adults, according to age of presentation. Clinical and echocardiographic parameters were compared among groups. Multivariate analysis was performed for mortality. Survival analysis was plotted using Kaplan Meier curves. RESULTS: Cyanosis, severe forms of AE and heart failure were more frequent among infants, arrhythmias in Ch/A and stroke among adults. Surgery was performed in 71 patients; infants had higher mortality and early complications. We found that the predicted mortality at 40 years of age in the three groups was significantly different (log rank test, p <0.0001): Infants: 38%, Ch/A 16 and 4% in adults. Multivariate model in surgical group showed that progressive drop of right ventricular fractional shortening (RVFS) predicts a higher mortality risk. In the non-surgical group, low RVFS and cyanosis were significantly associated with mortality. CONCLUSION: EA in infants is linked to higher morbidity and mortality, while arrhythmias predominate in Ch/A and stroke in adults. In general, stroke is frequent in patients with EA, some prevention alternative must be implemented. Right ventricular dysfunction is very important in EA and is associated with high mortality. It must be subject of discussion the planning of the type of surgery or even in the decision of to preclude surgical treatment.

Impact of Surgery in Ebstein's Anomaly Using Current Surgical Criteria.

BACKGROUND: In Ebstein's anomaly (EA) current surgical criteria may not translate into better long-term survival. The aim of this study was therefore to determine if surgical treatment for EA increases survival, and to analyze factors associated with mortality.Methods and Results:A retrospective study was carried out involving 103 patients with surgical indication using current criteria, comparing operated (n=49; 47.5%) and non-operated patients (n=54; 52.4%); the severity of disease was similar in all cases. Overall follow-up was 12 years (range, 1-49 years). There were no differences in mortality: in the surgical and non-surgical groups, survival at 10 years was 92.8% vs. 90.7%; 20 years, 85.7% vs. 81.0%; and 30 years, 78.5% vs. 72.2%, respectively. On multivariate analysis right ventricular fractional shortening (RVFS) was associated with mortality in both groups. Decreasing RVFS was associated with worse survival according to severity: when RVFS was <20%, survival at 20, 40 and 60 years was 58%, 39%, and 12.5%, respectively (P<0.0013). Left ventricular ejection fraction also correlated with survival (P<0.0013). CONCLUSIONS: Surgery did not translate into benefit in terms of survival, and this was clearly associated with RV function; therefore this should be a key factor in the surgical decision making.