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Survival in atrioventricular discordance
Insights
Long-term survival for patients with corrected transposition is limited. Left atrioventricular valve insufficiency significantly impacts outcomes, suggesting valve replacement may be beneficial.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Adult Congenital Heart Disease
Background:
- Limited data exists on long-term survival for patients with atrioventricular discordance, ventriculoarterial discordance, and two ventricles (corrected transposition).
- Understanding prognostic factors is crucial for managing this complex congenital heart defect population.
Purpose of the Study:
- To evaluate the long-term survival rates of patients diagnosed with corrected transposition.
- To identify clinical variables associated with survival in this patient cohort.
Main Methods:
- Retrospective review of 107 patients with corrected transposition diagnosed between 1951 and 1981.
- Analysis of survival data and associated clinical variables including sex, age, ventricular septal defect, pulmonary stenosis, dextrocardia, atrioventricular valve insufficiency, and heart block.
Main Results:
- Overall survival was 70% at five years and 64% at ten years post-diagnosis.
- Left atrioventricular valve insufficiency was the only variable consistently associated with decreased long-term survival.
- Ventricular septal defect and pulmonary stenosis did not significantly impact overall survival, though pulmonary stenosis showed a protective effect when a VSD was present.
Conclusions:
- Left atrioventricular valve insufficiency is a significant negative prognostic factor in patients with corrected transposition.
- Atrioventricular valve replacement should be considered for patients with hemodynamically significant left atrioventricular valve insufficiency.
Abstract:
Limited information is available concerning the long-term survival of patients with atrioventricular discordance, ventriculoarterial discordance, and two ventricles (corrected transposition). The long-term follow-up of 107 patients examined at the Mayo Clinic over a 30-year period between 1951 and 1981 was reviewed. Overall survival from the date of Mayo Clinic diagnosis was 70% at five years and 64% at ten years. Associated variables were analyzed for their effect on survival including sex, age at diagnosis, presence of ventricular septal defect, pulmonary stenosis, dextrocardia, left atrioventricular valve insufficiency, and complete heart block. There was no significant difference between those with and without a ventricular septal defect (VSD). Pulmonary stenosis was protective when a VSD was present but was not a significant predictor of long-term survival. The only variable that consistently correlated with decreased survival was left atrioventricular valve insufficiency (p less than 0.04 for univariate and stepwise Cox, and p = 0.08 for multivariate analysis). A logistic model for survival after open-heart surgery failed to identify any significant variable. We conclude that the presence of left atrioventricular valve insufficiency in association with atrioventricular discordance significantly alters the long-term outcome. Atrioventricular valve replacement should be considered in such patients when insufficiency becomes hemodynamically significant.