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Predictors of long-term survival with Ebstein's anomaly of the tricuspid valve
T L Gentles1, A L Calder, P M Clarkson
1Department of Cardiology, Green Lane Hospital, Auckland, New Zealand.
Insights
Predictors of death in Ebstein's anomaly include male sex and a cardiothoracic ratio ≥0.65. Surgery is recommended before the cardiothoracic ratio reaches 0.65 to improve survival in these patients.
Area of Science:
- Cardiology
- Congenital Heart Disease
Background:
- Ebstein's anomaly is a rare congenital heart defect affecting the tricuspid valve.
- Predictors of mortality and surgical indications in Ebstein's anomaly remain unclear.
Purpose of the Study:
- To identify predictors of death in patients with Ebstein's anomaly.
- To establish surgical recommendations based on prognostic factors.
Main Methods:
- Retrospective review of 48 patients diagnosed with Ebstein's anomaly.
- Analysis of clinical data, including presentation, follow-up duration, and outcomes.
- Statistical analysis to determine significant predictors of mortality.
Main Results:
- 42% of patients died, with a 50% survival probability at 47 years.
- Significant predictors of death included male sex, cardiothoracic ratio ≥0.65, NYHA class III/IV, breathlessness, and absence of Wolff-Parkinson-White syndrome.
- Cardiothoracic ratio ≥0.65 predicted sudden death better than functional status; atrial fibrillation predicted death within 5 years.
Conclusions:
- Tricuspid valve surgery is recommended before the cardiothoracic ratio reaches 0.65, irrespective of symptoms.
- Early surgical intervention may improve outcomes in Ebstein's anomaly.
Abstract:
In patients with Ebstein's anomaly of the tricuspid valve, predictors of death and indications for surgery are poorly defined. We retrospectively reviewed 48 patients with Ebstein's anomaly, 17 (35%) of whom presented in the first week of life. Duration of follow-up extended to 32 years (greater than 10 years in 35%). Twenty of the 48 patients (42%) died, 6 in the first week of life and 1 at age 5 months. Thirteen of the 41 patients surviving to age 6 months subsequently died, 50% probability of survival being reached at 47 years. Significant (p less than or equal to 0.05) predictors of death in this group were: male sex, cardiothoracic ratio greater than or equal to 0.65, New York Heart Association class III or IV, breathlessness and the absence of Wolff-Parkinson-White syndrome. Eight patients died suddenly. A cardiothoracic ratio greater than or equal to 0.65 was a better predictor of sudden death than functional status. All who developed atrial fibrillation died within 5 years. Other atrial arrhythmias were not helpful in predicting sudden death. In view of these findings, tricuspid valve surgery is recommended before the cardiothoracic ratio reaches 0.65, regardless of the symptomatic state.