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July 26, 2026Cardiology in the Young

High-degree atrioventricular block in a child with an ANK2 variant and long QT phenotype requiring leadless pacemaker implantation

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Key result

ANK2 variant linked to long-QT and high-degree AV block requiring a leadless pacemaker.

  • n=1

Population

10-year-old boy with recurrent syncope, seizure-like episodes, QT prolongation, and persistent high-degree…

Design

Case_report

Authors

JYJihye YouChonbuk National University Hospital

Discussion

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Implication

Supports genetic evaluation in unexplained pediatric AV block; leaves open broader ANK2 role pending larger studies.

Key Points

  • To report a case of high-degree atrioventricular block in a child associated with an ANK2 variant and long QT phenotype.
  • Detailed family phenotyping including genetic testing for ANK2 variant in members
  • Electrocardiography and continuous monitoring for detection of cardiac abnormalities
  • Assessment of arrhythmic manifestations and management strategies
  • A 10-year-old boy presented with recurrent syncope and seizure-like episodes.
  • Genetic testing revealed a heterozygous ANK2 variant of uncertain significance.
  • The case emphasizes the need for vigilant rhythm surveillance and individualized management in pediatric atrioventricular block.

Study Design

Type

Case Report (n=1)

Structured PICO

P
Population
A 10-year-old boy with recurrent syncope, QT prolongation, and persistent high-degree atrioventricular block.
E
Exposure
Leadless pacemaker implantation
O
Outcome
Clinical presentation and management of high-degree atrioventricular block

Highlights the rare presentation of clinically significant conduction disease requiring pacing in a pediatric patient with an ANK2 variant and long-QT phenotype.

Cite This Study

Jihye You (2026) conducted a case report in Ankyrin-B syndrome / long QT phenotype with high-degree atrioventricular block (n=1). ANK2 variant (NM_001148.4:c.5626G>A) was evaluated. A 10-year-old boy with a heterozygous ANK2 variant of uncertain significance presented with a long-QT phenotype and persistent high-degree atrioventricular block requiring a leadless pacemaker.

synapsesocial.com/papers/6a65a7b4d3aea3239cd7851dhttps://doi.org/10.1017/s1047951126123464
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