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Improvement in Dyssynchrony with Pharmacological Ablation of Right-Sided Accessory Pathway-Induced Cardiomyopathy in
Mai Sekine1, Satoshi Masutani1, Tomohiko Imamura2
1Department of Pediatrics, Saitama Medical Center, Saitama Medical University.
Insights
Right-sided accessory pathways in Wolff-Parkinson-White (WPW) syndrome can cause heart problems in infants. Flecainide successfully treated dilated cardiomyopathy in two infants, restoring heart function and avoiding early catheter ablation.
Area of Science:
- Pediatric Cardiology
- Electrophysiology
- Pharmacology
Background:
- Right-sided accessory pathways in Wolff-Parkinson-White (WPW) syndrome are associated with cardiac dyssynchrony and dilated cardiomyopathy.
- This cardiac dysfunction can manifest with a characteristic septal shape, independent of supraventricular tachycardia episodes.
Observation:
- Two infants, aged 13 and 5 months, presented with dilated cardiomyopathy attributed to right-sided accessory pathways.
- These infants experienced abnormal aneurysmal dilation of the basal interventricular septum and reduced ejection fraction.
Findings:
- Flecainide administration resulted in successful treatment of the dilated cardiomyopathy in both infants.
- Post-treatment, the abnormal septal dilation significantly improved, nearing normalization.
- Ejection fraction recovered following flecainide treatment.
Implications:
- Flecainide represents a novel and potentially crucial therapeutic option for managing right-sided accessory pathway-induced dilated cardiomyopathy in infants.
- This pharmacological approach may obviate the need for invasive catheter ablation procedures during early infancy.
- Early intervention with flecainide could prevent long-term cardiac complications associated with untreated WPW syndrome-related cardiomyopathy.
Abstract:
Right-sided accessary pathways in patients with Wolff-Parkinson-White (WPW) syndrome may cause cardiac dyssynchrony and dilated cardiomyopathy, with a characteristic septal shape, irrespective of any supraventricular tachycardia episodes. We report on two infants (13 and 5 months), whose right-sided accessary pathway-induced dilated cardiomyopathy was successfully treated by flecainide for the first time. After the flecainide administration, an abnormal aneurysmal dilation of the basal interventricular septum was almost restored to normal, and the decreased ejection fraction recovered. Flecainide use may be an important therapeutic option for this entity to avoid catheter ablation during infancy.
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