Yen Hung-Rong, Chu Shih-Ming
Department of Pediatrics, Chang Gung Children's Hospital, No. 5, Fu-Shin Street, Kweishan, Taoyuan 333, Taiwan.
Acta Paediatr Taiwan. 2003 Mar-Apr;44(2):112-5.
Cardiac rhabdomyoma, the most common cardiac tumor of infant and child, has been known to be associated with tuberous sclerosis complex. However, tuberous sclerosis complex with fetal supraventricular tachycardia is rarely reported. Herein, we report a female neonate diagnosed as tuberous sclerosis complex with cardiac rhabdomyoma presenting as supraventricular tachycardia. The newborn infant was initially treated with vagal maneuver, adenosine push, and digoxin. Although the tachycardia converted into normal sinus rhythm, frequent recurrence of PSVT was noted. At 16 days of age, the recurrent supraventricular tachycardia was refractory to vagal maneuver and drug therapy. Direct-current synchronized cardioversion converted the arrhythmia to normal sinus rhythm and oral digoxin effectively controlled the condition of the patient without further PSVT attacks. Subsequent 9-month follow-up revealed no further attack of arrhythmia. Regression of cardiac rhabdomyoma has not been visualized on echocardiogram at 9 months old.
心脏横纹肌瘤是婴幼儿最常见的心脏肿瘤，已知其与结节性硬化症相关。然而，结节性硬化症合并胎儿室上性心动过速的报道很少。在此，我们报告一名被诊断为结节性硬化症合并心脏横纹肌瘤并表现为室上性心动过速的女新生儿。该新生儿最初接受了迷走神经手法、腺苷推注和地高辛治疗。尽管心动过速转变为正常窦性心律，但仍注意到室上性心动过速频繁复发。在16日龄时，复发性室上性心动过速对迷走神经手法和药物治疗无效。直流电同步心脏复律将心律失常转变为正常窦性心律，口服地高辛有效控制了患者病情，未再发生室上性心动过速发作。随后9个月的随访显示未再发生心律失常。9个月大时，超声心动图未显示心脏横纹肌瘤消退。
