Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Familial Sick Sinus Syndrome

가족성 동기능 장애 증후군 1례

  • Nam, In-Hye (Department of Pediatrics, College of Medicine, Chungnam National University) ;
  • Cheon, Sung-Hee (Department of Pediatrics, College of Medicine, Chungnam National University) ;
  • Kil, Hong-Ryang (Department of Pediatrics, College of Medicine, Chungnam National University)
  • 남인혜 (충남대학교 의과대학 소아과학교실) ;
  • 천성희 (충남대학교 의과대학 소아과학교실) ;
  • 길홍량 (충남대학교 의과대학 소아과학교실)
  • Received : 2002.08.05
  • Accepted : 2002.09.25
  • Published : 2003.02.15
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Abstract

Sick sinus syndrome(SSS) constitutes a spectrum of cardiac arrhythmia, including sinus bradycardia, sinus pause-arrest, sinoatrial block, slow escape rhythm, bradyarrhythmia and tachyarrhythmia. SSS is relatively uncommon in children but its exact incidence is unknown because diagnostic criteria are not uniform and most children with SSS, in general are asymptomatic. SSS may be primary(organic sinus node disease) or secondary(cardiac surgery comprises much of SSS in children and adolescents), but it can hardly be caused by familial relations as well. We reports an occurrence of familial sick sinus syndrome. Mother was diagnosed as SSS, which was presented by symptoms of dizziness and treated by permanent pacemaker(DDD). Also, two daughters revealed SSS with non-compacted cardiomyopathy on neonatal screening and fetal echocardiography respectively. We concluded that familial SSS may occur, so familial screening should be suggested.

저자들은 비경화성 심근증을 동반한 두 자녀와 기질적 질환이 없이 발생한 동기능 장애 증후군 환자를 경험하였기에 문헌 고찰과 함께 가족성 동기능 장애 증후군 1례를 보고하는 바이다.

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