Department of Cardiology, Children's Hospital Boston, Massachusetts 02115, USA.
J Am Coll Cardiol. 2011 Oct 25;58(18):1903-9. doi: 10.1016/j.jacc.2011.08.005.
The aim of this study was to describe a large experience with primary cardiac tumors in pediatric patients, characterize associated arrhythmias, and expand knowledge of natural history and treatment options.
Primary cardiac tumors in children are rare. The incidence of arrhythmias is not well-defined, and management plans vary widely.
We employed a retrospective single-center review of patients ≤21 years of age diagnosed with a primary cardiac tumor between 1968 and 2010. Clinically significant arrhythmias were defined as: 1) sudden cardiac arrest; 2) nonsustained and sustained ventricular tachycardia (VT); 3) pre-excitation; and 4) sustained supraventricular tachycardia of any mechanism.
A total of 173 patients were identified: 106 rhabdomyoma, 25 fibroma, 14 myxoma, 6 vascular, 4 teratoma, 3 lipoma, and 15 other. Median age at diagnosis was 7 months (prenatal to 21 years). Of these, 42 (24%) had clinically significant arrhythmias. Patients with large fibromas were the highest-risk group, with VT occurring in 64%. Among rhabdomyoma patients, 10% had pre-excitation, and 6% had VT. Over a mean follow-up of 6 years (1 day to 34 years, median 4 years), surgical excision was performed in 62 cases, with rhythm treatment being 1 of the indications in 20. Post-operatively, clinically significant arrhythmias were eliminated in 18 of these 20, including all 13 fibroma patients.
Clinically significant arrhythmias occurred in 24% of pediatric patients with cardiac tumors, VT being the most common type. Surgical excision for VT associated with rhabdomyomas and fibromas in selected patients is an important and effective management strategy in these patients.
本研究旨在描述小儿原发性心脏肿瘤的大量临床经验，阐述相关心律失常，并拓展对其自然病史和治疗选择的认识。
小儿原发性心脏肿瘤较为罕见。心律失常的发生率尚未明确，且治疗方案差异较大。
我们采用回顾性单中心研究方法，对 1968 年至 2010 年间诊断为原发性心脏肿瘤且年龄≤21 岁的患者进行了研究。有临床意义的心律失常定义为：1）心脏骤停；2）非持续和持续性室性心动过速（VT）；3）预激；4）任何机制引起的持续性室上性心动过速。
共纳入 173 例患者：106 例横纹肌瘤、25 例纤维瘤、14 例黏液瘤、6 例血管瘤、4 例畸胎瘤、3 例脂肪瘤和 15 例其他类型肿瘤。诊断时的中位年龄为 7 个月（从产前至 21 岁）。其中 42 例（24%）存在有临床意义的心律失常。大纤维瘤患者为最高危人群，64%出现 VT。横纹肌瘤患者中 10%存在预激，6%存在 VT。平均随访 6 年（1 天至 34 年，中位数 4 年）后，62 例患者接受了手术切除，20 例患者中有 1 例为节律治疗适应证。术后，20 例患者中有 18 例（包括 13 例纤维瘤患者）的心律失常得到消除。
24%的小儿心脏肿瘤患者存在有临床意义的心律失常，其中 VT 最为常见。对于某些特定患者的横纹肌瘤和纤维瘤相关性 VT，手术切除是一种重要且有效的治疗策略。
