Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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상심실성 빈맥과 심장 종양으로 진단된 결절성 경화증 1예

Supraventricular tachycardia in a neonate with cardiac rhabdomyoma and tuberous sclerosis

  • 방인국 (계명대학교 의과대학 소아과학교실) ;
  • 김여향 (계명대학교 의과대학 소아과학교실) ;
  • 김천수 (계명대학교 의과대학 소아과학교실) ;
  • 이상락 (계명대학교 의과대학 소아과학교실) ;
  • 권태찬 (계명대학교 의과대학 소아과학교실)
  • Bang, In Kug (Department of Pediatrics, School of Medicine, Keimyung University) ;
  • Kim, Yeo Hyang (Department of Pediatrics, School of Medicine, Keimyung University) ;
  • Kim, Chun Soo (Department of Pediatrics, School of Medicine, Keimyung University) ;
  • Lee, Sang Lak (Department of Pediatrics, School of Medicine, Keimyung University) ;
  • Kwon, Tae Chan (Department of Pediatrics, School of Medicine, Keimyung University)
  • 투고 : 2008.03.08
  • 심사 : 2008.05.02
  • 발행 : 2008.07.15
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초록

심장의 원발성 종양은 소아에서는 매우 드문 질환이다. 횡문근종은 태아, 영아 및 소아 연령에서 가장 흔한 원발성 심장 종양으로 결절성 경화증이 동반되는 것으로 보고되고 있다. 결절성 경화증은 뇌, 심장, 피부 및 기타 장기들을 침범하는 신경피부 증후군으로, 결절성 경화증을 가진 영아의 50-64%에서 동반된다. 심장 횡문근종은 종괴는 심방을 비롯하여 심실 중격과 심실벽에 다발성으로 존재하며 종양의 위치와 크기, 침범 정도에 따라 심장 유출로 협착 및 심부전과 같은 여러 가지 임상 양상을 보일 수 있다. 드물지만 부정맥을 주증상으로 발현하는 경우도 보고되고 있다. 하지만 대부분 심장의 횡문근종이 특별한 문제를 일으키지 않고 성장과 함께 쇠퇴되는 경향을 보이므로 극히 일부분에서만 장기적인 항부정맥제 사용이 요구된다. 환자의 최종적인 예후는 동반되는 뇌병변에 의해 결정되는 경향이 있다. 저자들은 상심실성 빈맥으로 입원치료 중 진단된 심장 횡문근종을 가진 결절성 경화증 1예를 경험하였기에 보고하는 바이다.

Primary tumors of the heart are uncommon among pediatric patients. Rhabdomyoma is the most frequent cardiac tumor in infants and children, which is commonly associated with tuberous sclerosis. Tuberous sclerosis is a neurocutaneous syndrome affecting the brain, heart, skin, and other organs. Cardiac rhadomyomas are reported in 50-64% of infants with tuberous sclerosis. Tuberous sclerosis involves multiple locations in the atrium, ventricle and septum, and may induce mechanical obstruction of the outflow tract and heart failure depending on the location, number, size, and degree of invasion of tumors. Arrhythmias may also develop in infants with cardiac rhadomyomas, but only a few of these patients require prolonged anti-arrhythmic therapy because arrhythmia often disappears with spontaneous regression of the tumors, and the ultimate prognosis may be decided by the cerebral manifestations.

키워드

참고문헌

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피인용 문헌

  1. Prenatal Diagnosis of Tuberous Sclerosis by Fetal Echocardiography With an Unusual Clinical Course After Birth vol.1, pp.2, 2008, https://doi.org/10.5812/acvi.14113