All issues > Volume 37(1); 1994

Original Article

J Korean Pediatr Soc. 1994;37(1):61-69. Published online January 15, 1994.

Cardiac Rhabdomyoma in Children-A Report of 26 Cases

Ho Sung HS Kim¹, Youn Woo YW Kim¹, Wun Jung WJ Bae¹, Chung Il CI Noh¹, Jung Yun JY Choi¹, Yong Soo YS Yun¹

¹Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea

Abstract

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.

Keywords :Cardiac rhabdomyoma, Tuberous sclerosis, Echocardiography