Cardiac rhabdomyomas and obstructive left heart disease: Histologically but not functionally benign
Cardiac rhabdomyomas and obstructive left heart disease: Histologically but not functionally benign
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DOI：
10.1016/s0003-4975(98)00180-5
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发表时间：
1998-05-01
影响因子：
4.6
通讯作者：
Freedom, RM
中科院分区：
文献类型：
--
作者：
Black, MD;Kadletz, M;Freedom, RM
背景。心脏横纹肌瘤是婴儿最常见的原发性心脏肿瘤。此类肿瘤自发消退很常见，尤其是较小的病变，随后症状会缓解。基于我们关于自发消退的数据，我们机构的理念是在没有危及生命的症状时采取期待疗法。然而，有时需要手术干预以切除左心室流出道的横纹肌瘤。
方法。对27年间诊断为横纹肌瘤的30名儿童进行了回顾性研究。
结果。23%（7/30）的儿童需要手术切除左心室流出道内的肿瘤，尽管共有94%的儿童有左心室受累。无死亡病例。到目前为止，没有儿童需要再次切除肿瘤。
结论。横纹肌瘤的自然病程是自发消退（23名未接受手术干预的儿童仍然存活，并继续接受医学随访）。我们建议手术切除以缓解急性流出道梗阻，并依靠肿瘤的自然消退病程来实现长期无需再次手术。尽管手术已被认为是救命的，但我们有些惊讶地发现，超过20%的儿科患者需要手术干预。（C）1998年，胸外科医师协会。
Background. Cardiac rhabdomyoma is the most common primary heart tumor in infants. Spontaneous regression of such tumors is common, particularly with Smaller lesions, followed by resolution of symptoms. Based on our data on spontaneous involution, our institutional philosophy has been one of expectant management in the absence of life-threatening symptoms. However, surgical intervention sometimes is required for the extirpation of a rhabdomyoma from the left ventricular outflow tract.Methods. A retrospective review was conducted of 30 children in whom a rhabdomyoma was diagnosed over a 27-year period.Results. Twenty-three percent (7/30) of the children required surgical extirpation of the tumor from within their left ventricular outflow tract, although a total of 94% had left ventricular involvement. There were no deaths. To date, no child has required reexcision of tumor.Conclusions. The natural history of rhabdomyoma is one of spontaneous regression (the 23 children who did not undergo surgical intervention are alive and continue to be followed up medically). We recommend surgical excision to alleviate acute outflow tract obstruction with reliance on the tumor's natural history of regression to achieve long-term freedom from reoperation. Although operation has been recognized as lifesaving, rye were somewhat surprised to find that greater than 20% of our pediatric population required operative intervention. (C) 1998 by The Society of Thoracic Surgeons.