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Electrophysiological studies after the Mustard and Senning operations for complete transposition. Do they have
M Haemmerli1, M Bolens, B Friedli
1Pediatric Cardiac Unit, Hôpital Cantonal Universitaire, Geneva, Switzerland.
Insights
Long-term survival is good after Mustard or Senning operations, but arrhythmias are a concern. Electrophysiological studies can identify some risks for sick sinus syndrome, though prediction remains challenging.
Area of Science:
- Pediatric Cardiology
- Cardiac Electrophysiology
- Congenital Heart Disease
Background:
- Long-term survival after Mustard or Senning operations for complete transposition is generally good.
- Late arrhythmias, particularly sick sinus syndrome, remain a significant concern in these patients.
- The utility of postoperative electrophysiological investigations in predicting these arrhythmias is not well-established.
Purpose of the Study:
- To evaluate the effectiveness of electrophysiological studies in identifying children at risk of developing arrhythmias after Mustard or Senning operations.
- To assess the predictive value of specific electrophysiological parameters for sick sinus syndrome.
Main Methods:
- A cohort of 16 children who underwent electrophysiological study post-Mustard or Senning operation were followed for up to 9 years.
- Electrophysiological parameters including sinus node function and atrial conduction were analyzed.
- Clinical outcomes, specifically the development of sick sinus syndrome, were monitored.
Main Results:
- Electrophysiological abnormalities were frequent, with sinus nodal dysfunction in 9/16 and atrial conduction/refractoriness issues in 7/13 patients.
- Seven children developed clinical sick sinus syndrome during follow-up.
- Predictive accuracy for sick sinus syndrome varied: sensitivity was 42% (specificity 66%) using corrected sinus node recovery time alone, improving to 71% sensitivity (55% specificity) with additional criteria, but overall prediction remained difficult.
Conclusions:
- Electrophysiological studies frequently reveal abnormalities in children post-Mustard or Senning operation.
- Accurately identifying patients at high risk for developing sick sinus syndrome based on these studies remains challenging.
- Further refinement of electrophysiological criteria may be needed to improve risk stratification.
Abstract:
Overall, long-term survival after the Mustard or Senning operation is good, but late arrhythmias remain a concern. Whether postoperative electrophysiological investigations can identify patients at risk of developing serious arrhythmias is unknown. In this study, 16 children who underwent electrophysiological study after the Mustard or Senning operation for complete transposition (the combination of a concordant atrioventricular and a discordant ventriculo-arterial connexion) were followed up for one month to 9 years (mean 4.33 years) after this investigation. At the electrophysiological study, sinus nodal dysfunction was diagnosed in 9/16 patients, and abnormalities of atrial conduction and refractoriness in 7/13. During follow-up, 7 children developed clinical evidence of the sick sinus syndrome. When considering corrected sinus node recovery time as the only electrophysiological parameter, the sensitivity of predicting sick sinus syndrome from the electrophysiological study was 42%, and specificity 66%. The sensitivity increased to 71% if additional electrophysiological criteria of sinus node dysfunction were included, such as sinuatrial conduction time or sinus node entrance block, but specificity dropped to 55%. Atrioventricular conduction disturbances were rare. Thus, abnormalities at electrophysiological studies after the Mustard or Senning operations are frequent, but identification of patients at risk of developing sick sinus syndrome remains difficult.