PDF(Pubmed)
Abstract：
Despite its rare incidence of 1/40,000, fetal cardiac rhabdomyoma (CR) represents the prevailing type of benign cardiac fetal tumors, which commonly affects the ventricles. Fetal CRs rarely occur in the right atrium. Thus, the presentation of atrial fibrillation and premature atrial contractions (PAC) due to a solitary cardiac rhabdomyoma is an extremely rare scenario. Our literature review found that only 2% (1 out of 61) of rhabdomyoma cases were found in the right atrium. The majority of fetal cardiac rhabdomyomas are associated with tuberous sclerosis complex (TSC).
A 7-day-old male neonate presented with arrhythmias and an atrial mass for further evaluation. Echocardiography revealed a hyperechoic, round, uniform right atrial mass (25 mm). An abdominal and testicular ultrasound showed multiple thin-walled cortical cysts in both kidneys and a scrotal hydrocele, respectively. His laboratory workup was insignificant except for hypomagnesemia. Electrocardiography revealed junctional rhythm and PACs with wave distortions. A brain magnetic resonance imaging scan revealed multiple subependymal lesions on the frontal and occipital horns of the lateral ventricles. These findings (Fig. 1), along with a family history of TSC, confirmed the diagnosis of TSC with associated CR. The patient was treated symptomatically with an anti-convulsant and monitored with regular follow-ups. Surgical resection was not required.
Despite CR\'s predominance in the ventricles, a diagnosis of rhabdomyoma should be kept in mind in the presence of a solitary atrial mass and PACs. Physicians should evaluate systemic findings related to TSC and provide appropriate follow-up and family screening. Surgical resection is not always required, and symptom management can be achieved through medical treatment alone.
A 7-day-old male neonate presented with arrhythmias and an atrial mass for further evaluation. Echocardiography revealed a hyperechoic, round, uniform right atrial mass (25 mm). An abdominal and testicular ultrasound showed multiple thin-walled cortical cysts in both kidneys and a scrotal hydrocele, respectively. His laboratory workup was insignificant except for hypomagnesemia. Electrocardiography revealed junctional rhythm and PACs with wave distortions. A brain magnetic resonance imaging scan revealed multiple subependymal lesions on the frontal and occipital horns of the lateral ventricles. These findings (Fig. 1), along with a family history of TSC, confirmed the diagnosis of TSC with associated CR. The patient was treated symptomatically with an anti-convulsant and monitored with regular follow-ups. Surgical resection was not required.
Despite CR\'s predominance in the ventricles, a diagnosis of rhabdomyoma should be kept in mind in the presence of a solitary atrial mass and PACs. Physicians should evaluate systemic findings related to TSC and provide appropriate follow-up and family screening. Surgical resection is not always required, and symptom management can be achieved through medical treatment alone.
摘要：
背景：尽管胎儿心脏横纹肌瘤（CR）的发病率为1/40,000，但它代表了良性心脏胎儿肿瘤的流行类型，这通常会影响心室。胎儿CR很少发生在右心房。因此，孤立性心脏横纹肌瘤引起的心房颤动和房性早搏(PAC)的出现是极为罕见的情况.我们的文献综述发现，在右心房中仅发现2％（61例中有1例）的横纹肌瘤病例。大多数胎儿心脏横纹肌瘤与结节性硬化症（TSC）有关。
方法：一名7天大的男性新生儿出现心律失常和心房肿块，用于进一步评估。超声心动图显示高回声，圆形,均匀右心房肿块（25毫米）。腹部和睾丸超声检查显示肾脏和阴囊鞘膜积液均有多个薄壁皮质囊肿，分别。除了低镁血症，他的实验室检查微不足道。心电图显示交界性心律和PAC伴有波畸变。脑部磁共振成像扫描显示，侧脑室额叶和枕骨角上有多个室管膜下病变。这些发现（图。1),有TSC家族史,证实TSC的诊断与CR相关。患者用抗惊厥药对症治疗，并定期随访监测。不需要手术切除。
结论：尽管CR在心室中占主导地位，在存在孤立性心房肿块和PAC的情况下,应牢记横纹肌瘤的诊断.医师应评估与TSC相关的系统性发现，并提供适当的随访和家庭筛查。手术切除并不总是需要的，症状管理可以通过单独的医疗来实现。
方法：一名7天大的男性新生儿出现心律失常和心房肿块，用于进一步评估。超声心动图显示高回声，圆形,均匀右心房肿块（25毫米）。腹部和睾丸超声检查显示肾脏和阴囊鞘膜积液均有多个薄壁皮质囊肿，分别。除了低镁血症，他的实验室检查微不足道。心电图显示交界性心律和PAC伴有波畸变。脑部磁共振成像扫描显示，侧脑室额叶和枕骨角上有多个室管膜下病变。这些发现（图。1),有TSC家族史,证实TSC的诊断与CR相关。患者用抗惊厥药对症治疗，并定期随访监测。不需要手术切除。
结论：尽管CR在心室中占主导地位，在存在孤立性心房肿块和PAC的情况下,应牢记横纹肌瘤的诊断.医师应评估与TSC相关的系统性发现，并提供适当的随访和家庭筛查。手术切除并不总是需要的，症状管理可以通过单独的医疗来实现。
