PDF(Pubmed)
Abstract：
Primary cardiac tumors in children are very rare and may be associated with severe arrhythmias and sudden infant death syndrome. These cardiac arrhythmias vary depending on the location and size of the tumor. Sixty-four percent of children with cardiac fibroma, the second most common benign cardiac tumor in children, have ventricular arrhythmias, affecting therapeutic management and risk profile of these children. We report on two siblings with cardiac fibromas whose clinical presentations differed depending on their locations and size of the tumors. The first child, a three-year-old girl, was diagnosed with a cardiac fibroma in the left ventricle at the age of 8 months after surviving resuscitation due to ventricular fibrillation. Secondary prophylactic implantation of an ICD was performed. On propranolol, no further malignant arrhythmias have occurred to date. The seven-month-old brother was diagnosed postnatally with a cardiac tumor adjacent to the right ventricle. A few weeks after birth, the boy had refractory supraventricular tachycardia and ventricular arrhythmia that only resolved with amiodarone. In genetic testing, Gorlin-Goltz syndrome was diagnosed in both children. Conservative pharmacological therapy is a therapeutic strategy for asymptomatic patients with cardiac fibromas. The anti-arrhythmic medication depends on the location of the tumor. Implantation of an ICD should be performed in cases of malignant arrhythmias. In rare cases, there is an association between cardiac tumors and genetic syndromes, such as Gorlin-Goltz syndrome. These should always be considered when such a tumor is diagnosed.
摘要：
儿童原发性心脏肿瘤非常罕见，可能与严重的心律失常和婴儿猝死综合征有关。这些心律失常根据肿瘤的位置和大小而变化。64%的儿童患有心脏纤维瘤，儿童第二常见的良性心脏肿瘤，有室性心律失常,影响这些儿童的治疗管理和风险状况。我们报告了两个患有心脏纤维瘤的兄弟姐妹，其临床表现因其位置和肿瘤大小而异。第一个孩子,一个三岁的女孩,在因心室纤颤而复苏后存活8个月时被诊断为左心室的心脏纤维瘤。进行ICD的二级预防性植入。关于普萘洛尔，迄今为止没有发生进一步的恶性心律失常.这位七个月大的兄弟在出生后被诊断出患有右心室附近的心脏肿瘤。出生几周后，该男孩患有难治性室上性心动过速和室性心律失常，仅使用胺碘酮即可缓解。在基因检测中,在两个孩子中都诊断出Gorlin-Goltz综合征。保守药物治疗是无症状心脏纤维瘤患者的治疗策略。抗心律失常药物取决于肿瘤的位置。在恶性心律失常的情况下，应进行ICD的植入。在极少数情况下,心脏肿瘤和遗传综合征之间有联系，比如Gorlin-Goltz综合征.当诊断这种肿瘤时，应始终考虑这些因素。
