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婴幼儿终末期左心室扩张型心肌病的肺动脉环扎术:队列研究

Pulmonary artery banding in infants and young children with end-stage left ventricular dilated cardiomyopathy: cohort study.

作者信息

Zeng Min, Yang Fan, Yue Chao, Wei Wei, Ma Kai, Dou Zheng, Li Quanlin, Wang Xu, Li Shoujun

机构信息

Department of Pediatric Cardiac Center, National Center for Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College.

Department of Pediatric Intensive Care Unit, National Center for Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College.

出版信息

Int J Surg. 2025 Jan 1;111(1):146-152. doi: 10.1097/JS9.0000000000002061.

Abstract

BACKGROUND

Dilated cardiomyopathy (DCM) is the most common cardiomyopathy, and 40--50% of patients may die or need a heart transplant in 5 years after diagnosis. Although heart transplantation is the most effective life-saving option of end-stage DCM, scarcity of donors and series of complications prevent many patients from receiving timely treatment. Pulmonary artery banding (PAB) is recently described as an alternative strategy for end-stage DCM, with low left ventricular function (LVEF) but preserved right ventricular function, may potentially restore heart function and delay the need for heart transplantation, but current clinical evidence is still insufficient. On the other hand, the medication treatment of DCM in pediatric patients is mostly based on the experience of adults. It remains unclear whether PAB combined medication treatment could benefit infants and young children patients. The aim of this study was to assess the short-term efficacy of PAB combined with medication therapy in infants and young children with end-stage DCM, compared with medication therapy alone.

METHODS

This is a retrospective analysis of 18 consecutive pediatric patients aged ranging from 1 month to 44 months old who diagnosed with end-stage DCM (LVEF ≤30%) with preserved right ventricular function between 2019 and 2023 in our hospital. All patients had been treated with conventional medications for 2 months. Then they were divided in two groups: PAB surgery group (6/18), and nonsurgery group (12/18). Regardless of whether surgery was performed, both groups continued to receive medication treatment. Recovery of ventricular function was primary endpoints. Secondary endpoints included 180-day mortality and severe heart failure (LVEF ≤30%).

RESULTS

The authors found there were no differences in age, weight, height, BMI, renal function, liver function, pulmonary hypertension, tricuspid valve regurgitation, mitral valve regurgitation, and genetic abnormalities between those with and without PAB surgery. Comparing with nonsurgery group, five patients in surgery group regain the normal cardiac ejection fraction (LVEF ≥50%) (5/6, 83.3% vs. 4/12, 33.3%, P= 0.131). A total of three patients had sudden death in nonsurgery group, and there was no death in surgery group ( P= 0.180). Five patients (5/12, 41.7%) still remain the low heart failure (LVEF ≤40%) after 6 months of enrollment only given medical therapy, and none of patients present with LVEF ≤40% in PAB surgery group (0/6, 0% vs. 8/12, 67.7%, P= 0.034).

CONCLUSION

Pulmonary artery banding is safe and effective in infants and young children with end-stage DCM with preserved right ventricular function. Combined with conventional heart failure medication therapy, it may provide short-term benefits postoperatively, decrease the cardiogenic shock, act as a bridge to recovery, and potentially reduce the need for heart transplantation. Long-term effects remain further observation, and larger randomized controlled trials would be more persuasive in validating its efficacy.

摘要

背景

扩张型心肌病(DCM)是最常见的心肌病,40%-50%的患者在确诊后5年内可能死亡或需要进行心脏移植。尽管心脏移植是终末期DCM最有效的挽救生命的选择,但供体稀缺和一系列并发症使许多患者无法及时接受治疗。肺动脉环扎术(PAB)最近被描述为终末期DCM的一种替代策略,对于左心室功能低下(LVEF)但右心室功能保留的患者,可能潜在地恢复心脏功能并延迟心脏移植的需求,但目前的临床证据仍然不足。另一方面,儿科患者DCM的药物治疗大多基于成人经验。PAB联合药物治疗是否能使婴幼儿患者受益仍不清楚。本研究的目的是评估PAB联合药物治疗与单纯药物治疗相比,对终末期DCM婴幼儿患者的短期疗效。

方法

这是一项对2019年至2023年在我院诊断为终末期DCM(LVEF≤30%)且右心室功能保留的18例年龄在1个月至44个月的连续儿科患者的回顾性分析。所有患者均接受了2个月的常规药物治疗。然后将他们分为两组:PAB手术组(6/18)和非手术组(12/18)。无论是否进行手术,两组均继续接受药物治疗。心室功能恢复是主要终点。次要终点包括180天死亡率和严重心力衰竭(LVEF≤30%)。

结果

作者发现,接受PAB手术和未接受PAB手术的患者在年龄、体重、身高、BMI、肾功能、肝功能、肺动脉高压、三尖瓣反流、二尖瓣反流和基因异常方面没有差异。与非手术组相比,手术组有5例患者恢复了正常心脏射血分数(LVEF≥50%)(5/6,83.3%对4/12,33.3%,P=0.131)。非手术组共有3例患者猝死,手术组无死亡(P=0.180)。仅接受药物治疗6个月后,非手术组有5例患者(5/12,41.7%)仍处于低心力衰竭状态(LVEF≤40%),PAB手术组无患者LVEF≤40%(0/6,0%对8/12,67.7%,P=0.034)。

结论

肺动脉环扎术对右心室功能保留的终末期DCM婴幼儿患者安全有效。与传统心力衰竭药物治疗相结合,它可能在术后提供短期益处,减少心源性休克,作为恢复的桥梁,并可能减少心脏移植的需求。长期效果仍有待进一步观察,更大规模的随机对照试验在验证其疗效方面将更有说服力。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b758/11745709/cf40258602bb/js9-111-0146-g001.jpg

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