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Death and other events after cardiac repair in discordant atrioventricular connection
Insights
Intracardiac repair offers good long-term survival for patients with atrioventricular discordant connection. Overcoming early risks like heart block can lead to excellent outcomes in this complex congenital heart disease population.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Atrioventricular discordant connection is a complex congenital heart defect.
- Intracardiac repair has become a viable treatment option.
Purpose of the Study:
- To evaluate the long-term outcomes and identify risk factors after intracardiac repair in patients with atrioventricular discordant connection.
Main Methods:
- Retrospective analysis of 99 consecutive patients undergoing intracardiac repair.
- Actuarial survival rates, hazard function analysis, and identification of risk factors for mortality and morbidity were assessed.
Main Results:
- 1, 5, and 10-year survival rates were 86%, 75%, and 68%.
- Mortality risk decreased significantly by 6 months post-operation.
- Risk factors for death included double-outlet right ventricle, complete heart block, and younger/older age.
- Tricuspid valve issues and heart block development were significant concerns.
Conclusions:
- Intracardiac repair yields favorable long-term survival for atrioventricular discordant connections.
- Addressing early risks, particularly heart block and tricuspid valve complications, is crucial for optimizing results.
- Outcomes can be excellent if early surgical challenges are mitigated.
Abstract:
The 1 month, 1 year, and 10 year actuarial survival rates after operation among 99 consecutive patients with discordant atrioventricular connection who underwent intracardiac repair are 86%, 75%, and 68%, respectively. The hazard function of death declines rapidly after operation to reach a low level 6 months postoperatively, and it becomes indistinguishable from that of a matched general population by 4 years. Risk factors for premature death include double-outlet right ventricle, complete heart block preoperatively or developing perioperatively, very young or older (greater than 25 years) age, a high hematocrit value, and earlier date of operation. The last indicates improvement in results in recent years. Eleven (11%) of 99 patients had complete heart block preoperatively, and 23 (26% of 88) developed it perioperatively. Patients with discordant ventriculoarterial connection (i.e., corrected transposition) had a lesser probability of developing heart block after repair than did those with other ventriculoarterial connections. Seventeen (17%) patients required tricuspid (pulmonary venous) valve replacement or repair at the original intracardiac operation, and eight additional patients developed important incompetence after repair. Among the risk factors for incompetence was an Ebstein-like anomaly of the tricuspid valve. Reoperations (three) for conduit obstruction have been no more common than in patients with concordant atrioventricular connection. Fifty-seven (81%) of 70 surviving patients are in New York Heart Association Class I and 12 (17%) in Class II. This, and the single-phase hazard function, indicate that the overall results of cardiac repair in patients with atrioventricular discordant connection could become excellent if the early risks were overcome.