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.