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体外膜肺氧合治疗儿茶酚胺能多形性室性心动过速:一例报告及文献复习

Extracorporeal membrane oxygenation for catecholaminergic polymorphic ventricular tachycardia: a case report and literature review.

作者信息

Li Yuna, Wu Yao, Li Yumei, Zhang Zhen

机构信息

Department of PICU, Children's Medical Center, the First Hospital of Jilin University, 1 Xinmin Street, Changchun, Jilin Province, China.

出版信息

BMC Pediatr. 2025 Jan 7;25(1):13. doi: 10.1186/s12887-024-05357-y.

Abstract

BACKGROUND

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited ion channelopathy characterized by a structurally normal heart sensitive to catecholamines. It primarily presents as Bidirectional ventricular tachycardia (BiVT) and is a significant cause of sudden cardiac death in children.

CASE PRESENTATION

We report our experience with central Extracorporeal Membrane Oxygenation (ECMO) therapy in a 4-year-old boy with CPVT. Despite these measures, his CPVT was refractory to standard medical treatment and mechanical ventilatory support, with symptom progression. Consequently, ECMO support was initiated in addition to existing treatment. The patient was successfully weaned off ECMO on the 10th day of therapy and was discharged in a good condition. Follow-up after discharge showed favorable outcomes.

CONCLUSIONS

The successful outcome in this case was attributed to the application of ECMO, which helped maintain the patient's circulatory status and address progressively worsening cardiogenic shock and uncontrolled ventricular arrhythmia. In such situations, the early use of ECMO can provide essential circulatory support and stability for patients, as demonstrated in this case.

摘要

背景

儿茶酚胺能多形性室性心动过速(CPVT)是一种遗传性离子通道病,其特征是心脏结构正常但对儿茶酚胺敏感。它主要表现为双向性室性心动过速(BiVT),是儿童心脏性猝死的重要原因。

病例报告

我们报告了一名4岁CPVT男孩接受中心体外膜肺氧合(ECMO)治疗的经验。尽管采取了这些措施,但他的CPVT对标准药物治疗和机械通气支持无效,症状仍在进展。因此,在现有治疗基础上启动了ECMO支持。患者在治疗第10天成功撤离ECMO,并康复出院。出院后随访显示预后良好。

结论

该病例的成功归因于ECMO的应用,它有助于维持患者的循环状态,应对逐渐加重的心源性休克和无法控制的室性心律失常。在这种情况下,如本病例所示,早期使用ECMO可为患者提供必要的循环支持和稳定性。

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