{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T12:34:06Z","timestamp":1786365246557,"version":"build-2736575974"},"reference-count":32,"publisher":"Cambridge University Press (CUP)","issue":"6","license":[{"start":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T00:00:00Z","timestamp":1781827200000},"content-version":"unspecified","delay-in-days":18,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":["cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec id=\"S1047951126113511_as1\">\n                    <jats:title>Introduction:<\/jats:title>\n                    <jats:p>Atrial standstill represents a rare cardiac arrhythmia characterised by the complete absence of atrial electrical and mechanical activity. Early diagnostic recognition coupled with comprehensive genetic counselling assumes paramount importance. Herein, we report a rare case of a patient who presented with bradycardia at an early age and demonstrated aggressive atrial standstill during a pacemaker upgrade, which challenged the therapeutic strategy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"S1047951126113511_as2\">\n                    <jats:title>Case presentation:<\/jats:title>\n                    <jats:p>\n                      A 5-year-old girl was initially diagnosed with bradycardia. Holter monitoring confirmed severe bradycardia with prolonged sinus arrest episodes. Subsequently, the patient underwent single-chamber pacemaker implantation. During a planned dual-chamber upgrade at age 11, despite systematic exploration of multiple anatomical sites within the right atrium, adequate atrial capture could not be achieved. Intracardiac electrophysiological assessment demonstrated a complete absence of electrical activity in the bi-atrium. While speckle-tracking echocardiography revealed mildly reduced global longitudinal strain with impairment noted in the lateral myocardial segments, indicating potential injuries from ventricular demand pacing. Genetic test identified a compound heterozygous variant of\n                      <jats:italic>SCN5A<\/jats:italic>\n                      c.2431C&gt;T and c.2893C&gt;T. The protein structure of SCN5A has been built and named AF-P21333-F1, and the molecular function of the variant site has been annotated. Additionally, murine scRNA-seq data (GSE132658) revealed cardiac\n                      <jats:italic>Scn5a<\/jats:italic>\n                      expression is confined to the conductive bundles and fibres rather than cardiomyocytes, and the loss-of-function caused aggressive atrial standstill.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"S1047951126113511_as3\">\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>\n                      This case provides valuable insights into genotype-phenotype correlations in\n                      <jats:italic>SCN5A<\/jats:italic>\n                      -associated atrial standstill, emphasises the importance of comprehensive electrophysiological assessment during device implantation, and underscores considerations for physiological pacing strategies in paediatric patients requiring lifelong device dependency.\n                    <\/jats:p>\n                  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