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Cardiac Rhabdomyoma in Children-A Report of 26 Cases

Journal of the Korean Pediatric Society 1994;37(1):61-69.
Published online January 15, 1994.
Cardiac Rhabdomyoma in Children-A Report of 26 Cases
Ho Sung Kim, Youn Woo Kim, Wun Jung Bae, Chung Il Noh, Jung Yun Choi, Yong Soo Yun
Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
소아 심장 횡문근종 환아들에서 나이에 따른 종양의 수와 크기 및 임상 양상
김호성, 김연우, 배은정, 노정일, 최정연, 윤용수
서울대학교 의과대학 소아과학교실
We reviewed 26 cases of Cardiac rhabdomyoma in children which were diagnosed between 1986 and 1991 in Seoul National University Children's Hospital. Twenty-two cases were associated with tuberous sclerosis. Total tumor count was 58 and mean tumor count per patient was 2.23. Their Cardiac rhabdomyoma were diagnosed from fetal period through 12 years after birth. Median age was 9.5 months. Fifteen patients were males and rest 11 patients were females. Cardiac symtoms and signs at initial diagnosis were present in 12 cases. They consisted of cardiac murmur in 4, arrhythmia in 8 and congestive heart failure in 2 cases on chest X-ray and other 1 case showed abnormal left cardiac border. Electrocardiography and 24 hour Holter monitorings showed arrhythmia in 15 cases. Clinically significatn arrhythmias were found in 7 cases on 24 hour Holter monitorings. They consisted of 1 with frequent premature ventricular contractions, 3 with frequent premature atrial contractions, 2 with atrial tachycardia and 3 with atrio-ventricular block (2 cases of atrio-ventricular block had other arrhythmias also). The locations of tumors were left ventricle in 21, interventricular septum in 21, right ventricle in 13 and atrium in 3 tumors. Mean tumjor count per patient in fetal period and infancy was 2.86 and it was statistically much more than that after infancy which was 1.5. Mean tumor area which was calculated on two dimensional echocardiography was larger in fetal period and infancy(128.9¡¾38.2mm2) than that after infancy (47.8¡¾11.2mm2). Mean relative tumor area to aortic root dimension was also significantly larger in fetal period and infancy than that after infancy(2.07 vs 0.30). In tuberous sclerosis, mean mass area and mean relartive tumor area to aortic root dimension in fetal period and infancy was significantly larger than those in patients after infancy. Of 26 cases, tumorectomy was done in 3 cases due to hemodynamic obstruction and arrhythmia. One case died immediately after operation and the other 2 cases are still alive with the follow up period of 30 and 44 months each. Of the 7 patients with significant arrhythmia on 24 hour Holter monitorings, pacemaker insertion was done in 1 case who received tumorectomy and other 1 case was treated with anti-arrhythmic drugs. All the other patients did not need anti-arrhythmic drugs or other management.
Key Words: Cardiac rhabdomyoma, Tuberous sclerosis, Echocardiography

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