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唐氏综合征患者先天性心脏病外科治疗的结果:单中心经验。

The Outcome of Congenital Cardiac Surgery in Patients with Down Syndrome: Single-Center Experience.

机构信息

Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia.

Pediatric Cardiac Services, Madinah Cardiac Center MCC, 42210 Madinah, Saudi Arabia; Pediatric Department, Faculty of Medicine, Menoufia University, 32951 Menoufia, Egypt.

出版信息

Heart Surg Forum. 2023 Aug 24;26(4):E372-E380. doi: 10.59958/hsf.5795.

DOI:10.59958/hsf.5795
PMID:37679086
Abstract

OBJECTIVES

This study aimed to describe the outcomes of Down syndrome patients who underwent cardiac surgery for congenital heart defects and to develop risk prediction models for in-hospital mortality, recurrent hospital admission, and the need for catheter intervention among a cohort of patients.

METHODS

This single-centre retrospective cohort study included consecutive Down syndrome patients who underwent cardiac surgery for congenital heart defects between January 2018 and December 2021. We reviewed the electronic medical records. Two hundred patients fulfilled the eligibility criteria with complete data reporting. The patients' perioperative data and outcomes were recorded.

RESULTS

Females constituted 56.5%. Most (78.5%) patients showed accepted recovery. The incidence of all-cause in-hospital mortality was 3.0%. The rates of the need for a second operation, heart failure management, and permanent pacemaker insertion were 3.0%, 2.0%, and 2.5%, respectively. Only 8 (4.0%) patients stayed in the hospital for a long duration after chylothorax or tracheostomy (if intubated more than 2 weeks). The model had an accuracy of 99% and included the intraoperative transesophageal echocardiography (TEE) abnormalities (residual heart lesions) (adjusted odds ratio [AOR]: 26.541, p = 0.033), the duration of mechanical ventilation following the operation (AOR: 1.152, p = 0.009), and the occurrence of postoperative heart block (AOR: 76.447, p = 0.005).

CONCLUSION

Surgical treatment of congenital heart defects in Down syndrome patients had good outcomes with accepted recovery (without intra-hospital or during follow-up mortality or morbidity) of 78.5% and a 3% incidence of in-hospital mortality. Though, the occurrence of chylothorax was considerably high, and resulted in a long hospital stay (more than 10 days). Repair of tetralogy of Fallot and coarctation of the aorta were associated with an increased likelihood of catheter intervention following the operation.

摘要

目的

本研究旨在描述接受先天性心脏缺陷心脏手术的唐氏综合征患者的结局,并为该患者队列中的住院死亡率、再次住院率和导管介入需求建立风险预测模型。

方法

这是一项单中心回顾性队列研究,纳入了 2018 年 1 月至 2021 年 12 月期间接受先天性心脏缺陷心脏手术的唐氏综合征连续患者。我们查阅了电子病历。200 名患者符合纳入标准且数据完整。记录了患者围手术期数据和结局。

结果

女性占 56.5%。大多数(78.5%)患者表现出可接受的恢复。全因住院死亡率为 3.0%。再次手术、心力衰竭管理和永久起搏器植入的发生率分别为 3.0%、2.0%和 2.5%。仅 8 名(4.0%)患者在出现乳糜胸或气管切开(如果插管超过 2 周)后在医院停留时间较长。该模型的准确率为 99%,包括术中经食管超声心动图(TEE)异常(残余心脏病变)(调整后的优势比[OR]:26.541,p = 0.033)、术后机械通气时间(OR:1.152,p = 0.009)和术后发生心脏传导阻滞(OR:76.447,p = 0.005)。

结论

唐氏综合征患者先天性心脏缺陷的手术治疗效果良好,可接受的恢复率为 78.5%(无院内或随访期间死亡率或发病率),院内死亡率为 3%。然而,乳糜胸的发生率相当高,导致住院时间延长(超过 10 天)。法洛四联症和主动脉缩窄的修复与术后导管介入的可能性增加有关。

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