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Sinus node dysfunction in children: different aetiologies, similar clinical course in two-centre experience
Gulhan Tunca Sahin1, Cemil Cihad Kurt2, Hasan Candas Kafali1
1Department of Paediatric Cardiology, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, University of Health Sciences, Istanbul, Turkey.
Insights
Sinus node dysfunction in children, though rare, is increasingly diagnosed alongside structural heart disease, particularly after cardiac surgery. Permanent pacing effectively treats symptomatic cases, requiring ongoing patient monitoring.
Area of Science:
- Pediatric Cardiology
- Electrophysiology
- Congenital Heart Disease
Background:
- Sinus node dysfunction (SND) is uncommon in pediatric populations.
- Increasing frequency of diagnosis suggests a potential link with underlying cardiac conditions.
Purpose of the Study:
- To evaluate the clinical characteristics of pediatric patients diagnosed with sinus node dysfunction.
- To assess the outcomes and management of sinus node dysfunction in children.
Main Methods:
- Retrospective review of 77 pediatric patients diagnosed with sinus node dysfunction.
- Data collected from two tertiary pediatric cardiology centers in Turkey between January 2011 and June 2022.
Main Results:
- The majority of patients (64.9%) were male, with a mean age of 8.2 years.
- Age-incompatible bradycardia and pauses were common rhythm disturbances; syncope, presyncope, and dizziness were frequent initial symptoms (43%).
- Structural heart disease was present in 75.3% of patients, with congenital heart defects (CHDs) being most common, including transposition of the great arteries and atrial septal defects. Four patients had SCN5A mutations, and two had Emery-Dreifuss muscular dystrophy.
Conclusions:
- Sinus node dysfunction in children is often associated with structural heart disease, especially post-cardiac surgery.
- Continuous monitoring is crucial for these patients due to the potential for late-onset sinus node dysfunction.
- Permanent pacing is an effective treatment for symptomatic sinus node dysfunction in pediatric patients.
Aim:
This study aims to evaluate the clinical characteristics and outcomes of children diagnosed with sinus node dysfunction.
Methods:
This was a retrospective review of patients diagnosed with sinus node dysfunction in two tertiary paediatric cardiology centres in Turkey from January 2011 to June 2022.
Results:
In all, 77 patients (50, 64.9% males) were included, with a mean age of 8.2 ± 6.3 years and a mean weight of 28.2 ± 18.8 kg. While age-incompatible bradycardia and pauses were the most common rhythm disturbances, syncope, presyncope, and dizziness (n:33, 43%) were the most frequent initial symptoms. Structural heart disease was present in 58 (75.3%) of the 77 patients, 47 (61%) of whom were congenital. The most commonly associated CHDs were transposition of the great arteries (n:8), atrial septal defect (n:7), and atrioventricular septal defect (n:5). Seven of them also had left atrial isomerism. The remaining 19 patients were isolated. Four patients had SCN5A mutation (two of them were siblings) and two of them had Emery-Dreifuss muscular dystrophy.
Conclusion:
Although sinus node dysfunction is rare in children, it has been diagnosed with increasing frequency with structural heart disease, especially in patients who have undergone corrective cardiac surgery related to atrial tissue. Since sinus node dysfunction can occur at any time postoperatively, these patients should be kept under constant control. If symptomatic sinus node dysfunction is confirmed, permanent pacing is an effective therapeutic modality.
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