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新生儿心脏横纹肌瘤致主动脉瓣梗阻的处理。

Management of neonatal cardiac rhabdomyoma obstructing the aortic valve.

机构信息

Department of Cardiovascular Surgery, Osaka Women's and Children's Hospital, Osaka, Japan.

出版信息

J Card Surg. 2022 Sep;37(9):2839-2841. doi: 10.1111/jocs.16702. Epub 2022 Jun 21.

Abstract

Rhabdomyomas associated with tuberous sclerosis are common cardiac tumors in children, and no surgical intervention is needed in most cases. However, when the tumor causes left ventricular outlet tract obstruction (LVOTO), immediate surgical intervention is indicated. Here, we report a newborn who was diagnosed antenatally with multiple cardiac rhabdomyomas, one of which blocked the aortic valve during systole. The tumor was resected early in the postnatal period with excellent outcomes. Until surgery, we maintained ductus arteriosus patency and systemic circulation using prostaglandin E1, which helped to reduce the risk of sudden death due to LVOTO. Postoperative two-dimensional echocardiography at discharge showed that surgery was effective in resolving LVOTO.

摘要

结节性硬化症相关的横纹肌瘤是儿童常见的心脏肿瘤,大多数情况下无需手术干预。然而,当肿瘤引起左心室流出道梗阻(LVOTO)时,则需要立即进行手术干预。在这里,我们报告了一例新生儿,其在产前被诊断为多发性心脏横纹肌瘤,其中一个肿瘤在收缩期阻塞了主动脉瓣。该肿瘤在新生儿期早期进行了切除,结果非常理想。在手术前,我们使用前列腺素 E1 维持动脉导管开放和体循环,这有助于降低因 LVOTO 导致猝死的风险。出院时的二维超声心动图显示,手术有效地解决了 LVOTO。

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