Atrial Septal Defect Device Closure in Patients with Metabolic or Genetic Diseases

IF 0.4 4区医学 Q4 PEDIATRICS

Iranian Journal of Pediatrics Pub Date : 2023-12-05 DOI:10.5812/ijp-137508

Keyhan Sayadpour Zanjani, Morteza Heidari, Nima Nazari, Hosein Alimadadi, Reihaneh Mohsenipour

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引用次数: 0

Abstract

Background: The coexistence of a metabolic or genetic disease can complicate the course of an atrial septal defect device closure. Methods: The database of our hospital was searched for patients who had undergone atrial septal defect (ASD) device closure and had concurrent metabolic and genetic diseases. Out of 188 such patients, 11 were identified. Results: This cohort study included 11 patients with type 1 diabetes mellitus, insulin resistance, mitochondrial diseases, rickets, Seckel syndrome, Alagille syndrome, cystic fibrosis, Down syndrome, and Crigler-Najjar syndrome type II. The patients were followed for a median of 4 years. Two patients experienced thromboembolic events. One procedure failed as the device was embolized. Large devices with a waist circumference greater than 1.5 times the body weight were used in 3 patients. One patient died 19 days after the procedure due to multi-organ failure, which was not related to device closure. Conclusions: In patients with metabolic or genetic diseases, this procedure may be complicated by factors such as small patient size, hypercoagulation, organ failure (cardiac, renal, or hepatic), vascular abnormalities, and issues with anesthesia or transesophageal echocardiography. It is recommended that careful attention be given to the specific challenges associated with each disease. The utilization of large devices can be considered safe, particularly in patients beyond 4 years of age.

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代谢性或遗传性疾病患者的心房间隔缺损装置封堵术

背景:代谢性或遗传性疾病的共存可使房间隔缺损装置关闭的过程复杂化。方法:检索我院数据库中合并代谢性和遗传性疾病的房间隔缺损(ASD)装置闭合患者。在188名这样的患者中，有11人被确诊。结果:本队列研究纳入了11例1型糖尿病、胰岛素抵抗、线粒体疾病、佝偻病、Seckel综合征、Alagille综合征、囊性纤维化、唐氏综合征和Crigler-Najjar综合征II型患者。这些患者的随访时间中位数为4年。2例患者发生血栓栓塞事件。一个手术失败了，因为装置被栓塞了。3例患者使用了腰围大于体重1.5倍的大型器械。1例患者在手术后19天因多器官衰竭死亡，这与器械关闭无关。结论:对于患有代谢性或遗传性疾病的患者，该手术可能会因患者体型小、高凝、器官功能衰竭(心脏、肾脏或肝脏)、血管异常以及麻醉或经食管超声心动图问题等因素而复杂化。建议仔细注意与每种疾病相关的具体挑战。使用大型器械被认为是安全的，特别是在4岁以上的患者中。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Iranian Journal of Pediatrics 医学-小儿科

CiteScore

0.90

自引率

20.00%

发文量

审稿时长

6-12 weeks

期刊介绍： Iranian Journal of Pediatrics (Iran J Pediatr) is a peer-reviewed medical publication. The purpose of Iran J Pediatr is to increase knowledge, stimulate research in all fields of Pediatrics, and promote better management of pediatric patients. To achieve the goals, the journal publishes basic, biomedical, and clinical investigations on prevalent diseases relevant to pediatrics. The acceptance criteria for all papers are the quality and originality of the research and their significance to our readership. Except where otherwise stated, manuscripts are peer-reviewed by minimum three anonymous reviewers. The Editorial Board reserves the right to refuse any material for publication and advises that authors should retain copies of submitted manuscripts and correspondence as the material cannot be returned. Final acceptance or rejection rests with the Editors.