Sinus node dysfunction in children: different aetiologies, similar clinical course in two-centre experience

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.