Smythe J F, Dyck J D, Smallhorn J F, Freedom R M
Division of Cardiology, Hospital For Sick Children, Toronto, Ontario, Canada.
Am J Cardiol. 1990 Nov 15;66(17):1247-9. doi: 10.1016/0002-9149(90)91109-j.
Although spontaneous regression of cardiac rhabdomyoma has been reported, prognosis is still considered to be poor and surgery continues to be indicated. The experience with rhabdomyoma diagnosed in live infants over a 20-year period was reviewed. Diagnosis by angiography or echocardiography was accepted only if multiple tumors were present or if tuberous sclerosis was also diagnosed. Nine patients (3 diagnosed prenatally and the remaining 6 at age less than 8 months) were identified as having a total of 24 tumors. Measurements in 2 planes demonstrated at least some evidence of regression in 24 patients (100%), with 20 of 24 having complete resolution. One patient required delayed surgery for excision of a subaortic ridge that appeared at the site of a resolved tumor. Our findings suggest that pediatric cardiac rhabdomyoma is most often a benign condition in which spontaneous regression is the rule. Surgery is recommended only for patients with refractory dysrhythmias or severe hemodynamic compromise.
尽管已有心脏横纹肌瘤自发消退的报道,但预后仍被认为较差,手术治疗仍有必要。回顾了20年间活体婴儿诊断为横纹肌瘤的经验。仅当存在多个肿瘤或同时诊断为结节性硬化症时,血管造影或超声心动图诊断才被接受。9例患者(3例产前诊断,其余6例年龄小于8个月)共发现24个肿瘤。在两个平面上的测量显示,24例患者(100%)至少有一些消退的证据,24例中有20例完全消退。1例患者因切除位于已消退肿瘤部位出现的主动脉下嵴而需要延迟手术。我们的研究结果表明,小儿心脏横纹肌瘤通常是一种良性疾病,自发消退是常态。仅建议对难治性心律失常或严重血流动力学障碍的患者进行手术。