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成人单心室先天性心脏病心脏移植术后的生存情况。

Survival After Cardiac Transplantation in Adults With Single-Ventricle Congenital Heart Disease.

机构信息

Cardiovascular Outcomes Research Laboratories (CORELAB), Division of Cardiac Surgery, David Geffen School of Medicine at UCLA, Los Angeles, California, USA; Department of Surgery, University of Colorado Anschutz Medical Center, Aurora, Colorado, USA.

Cardiovascular Outcomes Research Laboratories (CORELAB), Division of Cardiac Surgery, David Geffen School of Medicine at UCLA, Los Angeles, California, USA.

出版信息

J Am Coll Cardiol. 2023 Sep 19;82(12):1226-1241. doi: 10.1016/j.jacc.2023.06.037.

Abstract

BACKGROUND

Without large-scale analyses of adults with single-ventricle congenital heart disease (CHD) undergoing heart transplantation, little evidence exists to guide listing practices and patient counseling.

OBJECTIVES

This study aims to evaluate survival after heart transplantation in adults with single and biventricular CHD and compare it to that of non-CHD transplant recipients.

METHODS

In this 15-year (2005-2020) retrospective analysis, outcome-blinded investigators used probability-linkage to merge the National (Nationwide) Inpatient Sample and Organ Procurement and Transplantation Network data sets.

RESULTS

Of 382 adult (≥18 years of age) heart transplant recipients with CHD, 185 (48%) had single-ventricle physiology. Compared to biventricular CHD, single-ventricle patients showed significantly reduced survival at 1 (80% vs 91%; HR: 2.50; 95% CI: 1.40-4.49; P = 0.002) and 10 years (54% vs 71%; HR: 2.10; 95% CI: 1.38-3.18; P < 0.001). Among patients who survived the first post-transplantation year, biventricular CHD patients exhibited similar 10-year survival as single-ventricle patients, except for those with hypoplastic left heart syndrome (79% vs 71%; HR: 1.58; 95% CI: 0.85-2.92; P = 0.15). Additionally, biventricular CHD transplant recipients showed significantly better 10-year conditional survival compared to their non-CHD counterparts (79% vs 68%; HR: 0.73; 95% CI: 0.59-0.90; P = 0.003).

CONCLUSIONS

Among adult CHD transplant recipients, single-ventricle physiology correlated with higher short-term mortality. However, 10-year conditional survival was similar for biventricular and most single-ventricle CHD patients, and notably better for biventricular CHD patients compared to non-CHD heart transplant recipients. These findings have significant implications towards patient selection and listing strategies, easing concerns related to heart transplantation in adults with CHD and destigmatizing most subtypes of single-ventricle CHD.

摘要

背景

由于缺乏对接受心脏移植的单心室先天性心脏病(CHD)成人进行大规模分析,因此几乎没有证据可以指导列表实践和患者咨询。

目的

本研究旨在评估单心室和双心室 CHD 成人接受心脏移植后的生存率,并与非 CHD 移植受者进行比较。

方法

在这项为期 15 年(2005-2020 年)的回顾性分析中,结果盲法研究人员使用概率链接将国家(全国)住院患者样本和器官获取与移植网络数据集合并。

结果

在 382 名(≥18 岁)患有 CHD 的成年心脏移植受者中,185 名(48%)具有单心室生理学。与双心室 CHD 相比,单心室患者在 1 年(80% vs 91%;HR:2.50;95%CI:1.40-4.49;P=0.002)和 10 年(54% vs 71%;HR:2.10;95%CI:1.38-3.18;P<0.001)的生存率显著降低。在幸存至移植后第一年的患者中,除了左心发育不良综合征患者外(79% vs 71%;HR:1.58;95%CI:0.85-2.92;P=0.15),双心室 CHD 患者在 10 年时的生存率与单心室患者相似。此外,与非 CHD 患者相比,双心室 CHD 移植受者的 10 年条件生存率显著提高(79% vs 68%;HR:0.73;95%CI:0.59-0.90;P=0.003)。

结论

在成年 CHD 心脏移植受者中,单心室生理学与较高的短期死亡率相关。然而,对于大多数双心室和单心室 CHD 患者,10 年条件生存率相似,与非 CHD 心脏移植受者相比,双心室 CHD 患者的生存率明显更好。这些发现对患者选择和列表策略具有重要意义,减轻了成年人 CHD 患者接受心脏移植的相关担忧,并使大多数单心室 CHD 亚型不再被污名化。

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