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新生儿心脏肌肉瘤——病例报告及文献综述

Cardiac Myosarcoma in a Newborn Infant-A Case Report and Literature Review.

作者信息

Vakrilova Liliya, Marinov Rumen, Hitrova-Nikolova Stanislava, Dobrev Dobri, Denev Maxim, Lazarov Stoyan

机构信息

Faculty of Medicine, Medical University of Sofia, Sofia, Bulgaria.

Department of Neonatology, University Obstetrics and Gynecology Hospital "Maichin Dom", Sofia, Bulgaria.

出版信息

Front Cardiovasc Med. 2021 Jul 14;8:675202. doi: 10.3389/fcvm.2021.675202. eCollection 2021.

Abstract

Malignant cardiac neoplasms are extremely rare in neonates. Prenatal diagnosis is often not available. Initial symptoms can mimic non-cardiac diseases. We present a pre-mature newborn, birth-weight 2,480 g, 34 gestational weeks, which underwent cardiac surgery due to a cardiac tumor. This was a 3-rd pregnancy after two spontaneous abortions to a mother with thrombophilia, diabetes, hydramnios, and retroplacental hematoma. The baby was admitted to NICU with transitory respiratory failure and inborn infection; hence oxygen-supplementation and antibiotics were initiated. On day 11 a deterioration with tachypnea, high oxygen requirements, significantly increasing C-reactive protein values were noted. Chest radiographs were unremarkable. On day 18 a life-threatening condition with clinical symptoms of shock was identified. Echocardiography showed a large tumor formation in the right atrium, reduced blood flow in the right ventricle and pulmonary artery. On day 19 cardiac surgery was performed: a large tumor with a myxomatous appearance that occupied the cavity of the right atrium and infiltrated the annulus of the tricuspid valve was extirpated. The front wall was restored with a patch. Hemodynamics was temporarily stabilized. On the following day, ventricular fibrillation and asystole occurred. Despite life support efforts, the outcome was lethal. The histological result confirmed leiomyosarcoma of the right atrium and right ventricular hypotrophy. Inborn cardiac sarcomas are extremely rare. The prognosis is poor. Due to fast progression in the third trimester, they can be missed by fetal echocardiography in earlier pregnancy. The postnatal clinical course is aggressive, not specific until invasive infiltration or obstruction by the tumor mass occurred. In our case, the sarcoma mimicked an inborn infection, followed by nosocomial infection and septic shock. Despite low incidence, cardiac tumors should be kept in mind and echocardiography should be conducted if there are unclear symptoms with progressive deterioration during the neonatal period.

摘要

恶性心脏肿瘤在新生儿中极为罕见。通常无法进行产前诊断。初始症状可能类似于非心脏疾病。我们报告一例早产新生儿,出生体重2480克,孕34周,因心脏肿瘤接受了心脏手术。这是母亲的第三次妊娠,前两次自然流产,母亲患有血栓形成倾向、糖尿病、羊水过多和胎盘后血肿。婴儿因短暂呼吸衰竭和先天性感染入住新生儿重症监护病房(NICU);因此开始给予吸氧和抗生素治疗。第11天,发现病情恶化,出现呼吸急促、高氧需求、C反应蛋白值显著升高。胸部X光片无异常。第18天,发现出现危及生命的状况,伴有休克的临床症状。超声心动图显示右心房有一个大的肿瘤形成,右心室和肺动脉血流减少。第19天进行了心脏手术:切除了一个外观呈黏液瘤样、占据右心房腔并浸润三尖瓣环的大肿瘤。前壁用补片修复。血流动力学暂时稳定。第二天,发生室颤和心搏停止。尽管进行了生命支持努力,但结局是致命的。组织学结果证实为右心房平滑肌肉瘤和右心室肥厚。先天性心脏肉瘤极为罕见。预后很差。由于在妊娠晚期进展迅速,早期妊娠的胎儿超声心动图可能会漏诊。出生后的临床病程进展迅速,在肿瘤块发生浸润或阻塞之前没有特异性。在我们的病例中,肉瘤最初表现为先天性感染,随后是医院感染和感染性休克。尽管发病率低,但在新生儿期如果出现不明原因的症状且病情进行性恶化,应考虑心脏肿瘤并进行超声心动图检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2511/8316586/14cde688cbc5/fcvm-08-675202-g0001.jpg

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Cardiac Myosarcoma in a Newborn Infant-A Case Report and Literature Review.新生儿心脏肌肉瘤——病例报告及文献综述
Front Cardiovasc Med. 2021 Jul 14;8:675202. doi: 10.3389/fcvm.2021.675202. eCollection 2021.

本文引用的文献

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Rare operations in pediatric heart surgery: Cardiac tumors in childhood.小儿心脏手术中的罕见手术:儿童心脏肿瘤
Turk Gogus Kalp Damar Cerrahisi Derg. 2018 Sep 16;26(4):544-549. doi: 10.5606/tgkdc.dergisi.2018.16147. eCollection 2018 Oct.
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Fetal heart rhabdomyomatosis: a single-center experience.胎儿心脏横纹肌瘤:单中心经验。
J Matern Fetal Neonatal Med. 2021 Mar;34(5):701-707. doi: 10.1080/14767058.2019.1613365. Epub 2019 May 15.
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Identification and clinical course of 166 pediatric cardiac tumors.166例儿童心脏肿瘤的识别与临床病程
Eur J Pediatr. 2017 Feb;176(2):253-260. doi: 10.1007/s00431-016-2833-4. Epub 2017 Jan 10.
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Pediatric cardiac tumors: a 45-year, single-institution review.小儿心脏肿瘤:一项为期45年的单机构回顾研究。
World J Pediatr Congenit Heart Surg. 2015 Apr;6(2):215-9. doi: 10.1177/2150135114563938.

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