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小儿心脏移植术后病理学研究:11 年研究。人群特征及其对预后的影响。

Pathology of explanted pediatric hearts: An 11-year study. Population characteristics and implications for outcomes.

机构信息

The Cardiac Registry, Departments of Cardiology, Pathology, and Cardiac Surgery, Boston Children's Hospital, Boston, Massachusetts, USA.

Department of Thoracic and Cardiovascular Surgery, Mie University Graduate School of Medicine, Mie, Japan.

出版信息

Pediatr Transplant. 2024 Jun;28(4):e14742. doi: 10.1111/petr.14742.

Abstract

BACKGROUND

As more pediatric patients become candidates for heart transplantation (HT), understanding pathological predictors of outcome and the accuracy of the pretransplantation evaluation are important to optimize utilization of scarce donor organs and improve outcomes. The authors aimed to investigate explanted heart specimens to identify pathologic predictors that may affect cardiac allograft survival after HT.

METHODS

Explanted pediatric hearts obtained over an 11-year period were analyzed to understand the patient demographics, indications for transplant, and the clinical-pathological factors.

RESULTS

In this study, 149 explanted hearts, 46% congenital heart defects (CHD), were studied. CHD patients were younger and mean pulmonary artery pressure and resistance were significantly lower than in cardiomyopathy patients. Twenty-one died or underwent retransplantation (14.1%). Survival was significantly higher in the cardiomyopathy group at all follow-up intervals. There were more deaths and the 1-, 5- and 7-year survival was lower in patients ≤10 years of age at HT. Early rejection was significantly higher in CHD patients exposed to homograft tissue, but not late rejection. Mortality/retransplantation rate was significantly higher and allograft survival lower in CHD hearts with excessive fibrosis of one or both ventricles. Anatomic diagnosis at pathologic examination differed from the clinical diagnosis in eight cases.

CONCLUSIONS

Survival was better for the cardiomyopathy group and patients >10 years at HT. Prior homograft use was associated with a higher prevalence of early rejection. Ventricular fibrosis (of explant) was a strong predictor of outcome in the CHD group. We presented several pathologic findings in explanted pediatric hearts.

摘要

背景

随着越来越多的儿科患者成为心脏移植(HT)的候选者,了解影响移植后心脏结局的病理预测因素和移植前评估的准确性对于优化稀缺供体器官的利用和改善结局非常重要。作者旨在研究移植心脏标本,以确定可能影响 HT 后心脏移植物存活的病理预测因素。

方法

对 11 年间获得的小儿心脏标本进行分析,以了解患者的人口统计学特征、移植适应证以及临床病理因素。

结果

本研究共分析了 149 例移植心脏,其中 46%为先天性心脏病(CHD)。CHD 患者年龄较小,平均肺动脉压和阻力明显低于心肌病患者。21 例患者死亡或再次移植(14.1%)。在所有随访时间间隔内,心肌病组的存活率明显更高。在 HT 时年龄≤10 岁的患者中,死亡和 1、5、7 年生存率较低。暴露于同种异体组织的 CHD 患者的早期排斥反应明显更高,但晚期排斥反应并无差异。CHD 心脏伴有一个或两个心室过度纤维化的患者,死亡率/再次移植率显著更高,移植物存活率更低。病理检查的解剖诊断与临床诊断在 8 例中存在差异。

结论

心肌病组和 HT 时年龄>10 岁的患者的存活率更好。同种异体组织的使用与早期排斥反应的发生率更高相关。心室纤维化(移植标本)是 CHD 组的一个强烈预后预测因素。我们提出了一些在移植小儿心脏中发现的病理表现。

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本文引用的文献

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Immunity and inflammation: the neglected key players in congenital heart disease?
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