Fetal supraventricular tachycardia with giant cardiac rhabdomyoma: Role of post natal sirolimus in a developing country

IF 0.6 Q4 PEDIATRICS PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2024-10-24 DOI:10.1016/j.ppedcard.2024.101762
Anis Munirah Mohd Kori , Nursyahirah Anum Mohd Radzi , Ammar Mohamad Ziyadi , Intan Juliana Abd Hamid , Nor Rosidah Ibrahim , Noraida Ramli , Mohd Rizal Mohd Zain
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Abstract

Primary cardiac tumors in fetuses are rare and mainly represent rhabdomyoma. The tumors can be clinically silent or cause hemodynamically significant obstructions. We present a case of giant neonatal cardiac rhabdomyoma with right inflow obstruction complicated by fetal supraventricular tachycardia. Fetal echocardiography revealed the presence of supraventricular tachycardia with multiple cardiac masses, one of which caused an obstruction of the right inflow of the heart. As the baby developed hemodynamic instability due to the mass effect, oral sirolimus was initiated postnatally. Despite the limitation of evidence on the usage of sirolimus in neonatal population, this case report revealed the benefits of postnatal sirolimus in a decrease in the size of the mass without any adverse medication effects. Sirolimus has demonstrated efficacy in neonatal cardiac rhabdomyoma, however, a large prospective study is needed to demonstrate the efficacy.
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胎儿室上性心动过速伴巨大心脏横纹肌瘤:产后西罗莫司在发展中国家的作用
胎儿的原发性心脏肿瘤非常罕见,主要是横纹肌瘤。这些肿瘤可能在临床上无症状,也可能导致血流动力学上的明显梗阻。我们报告了一例新生儿巨大心脏横纹肌瘤并发右心室流入道梗阻和胎儿室上性心动过速的病例。胎儿超声心动图显示,胎儿存在室上性心动过速和多个心脏肿块,其中一个肿块导致右心流入道梗阻。由于肿块效应导致婴儿血流动力学不稳定,产后开始口服西罗莫司。尽管在新生儿群体中使用西罗莫司的证据有限,但该病例报告显示了产后使用西罗莫司的益处,即在减少肿块大小的同时不会产生任何药物不良反应。西罗莫司对新生儿心脏横纹肌瘤有一定疗效,但还需要大型前瞻性研究来证明其疗效。
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来源期刊
CiteScore
0.90
自引率
11.10%
发文量
69
审稿时长
75 days
期刊介绍: Progress in Pediatric Cardiology is an international journal of review presenting information and experienced opinion of importance in the understanding and management of cardiovascular diseases in children. Each issue is prepared by one or more Guest Editors and reviews a single subject, allowing for comprehensive presentations of complex, multifaceted or rapidly changing topics of clinical and investigative interest.
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