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Journal of the Korean Pediatric Society 2003;46(2):195-197.
Published online February 15, 2003.
Familial Sick Sinus Syndrome
In-Hye Nam, Sung-Hee Cheon, Hong-Ryang Kil
Department of Pediatrics, College of Medicine, Chungnam National University, Daejeon, Korea
가족성 동기능 장애 증후군 1례
남인혜, 천성희, 길홍량
충남대학교 의과대학 소아과학교실
Correspondence:  Hong-Ryang Kil,
Email: namine726@hanmail.net
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.
Key Words: Sick sinus syndrome, Cardiomyopathy


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