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患有先天性心脏病且接受1.5心室修复术的成年人的长期预后。

Long-term outcomes of adults with congenital heart disease and 1.5 ventricle repair.

作者信息

Egbe Alexander C, Miranda William R, Connolly Heidi M, Dearani Joseph A

机构信息

Department of Cardiovascular Medicine, Mayo Clinic Rochester, MN 55905.

Department of Cardiovascular Medicine, Mayo Clinic Rochester, MN 55905.

出版信息

J Heart Lung Transplant. 2025 Apr;44(4):511-520. doi: 10.1016/j.healun.2024.12.020.

DOI:10.1016/j.healun.2024.12.020
PMID:40113347
Abstract

BACKGROUND

The 1.5 ventricle repair is a palliative procedure for patients with congenital heart disease with advanced right heart failure. There are limited data about patient selection and outcomes of this procedure. The purpose of this study was to describe the clinical and hemodynamic characteristics, and outcomes of adults with congenital heart disease (CHD) and 1.5 ventricle repair.

METHOD

We studied adults with CHD that underwent 1.5 ventricle repair and received care at Mayo Clinic between January 1, 2003, and December 31, 2023. The patients were divided into two cohorts. Cohort #1 comprised of patients that had 1.5 ventricle repair prior to adulthood (age <18 years), while Cohort #2 comprised of patients that underwent 1.5 ventricle repair in adulthood (age ≥18 years).

RESULTS

There were 61 patients with 1.5 ventricle repair (Cohort #1 n=39, 64%; Cohort #2 n=22, 36%). In Cohort #1, the median age at the time of 1.5 ventricle repair was 7 years (interquartile range [IQR]: 1-12), while the median age at presentation to the CHD clinic was 20 years (IQR: 18-27). Of the 39 patients in Cohort #1, 4 (10%) required reintervention. The 10-year risk of death/transplant was 14%. In Cohort #2, the median age at the time of 1.5 ventricle repair was 33 years (IQR: 23-44), and the operative mortality was 4%. There was an improvement in cardiac output, functional status, and reduction in the proportion of patients with stage D heart failure at 1 year after 1.5 ventricle repair.

CONCLUSIONS

The 1.5 ventricle repair is a viable treatment option in patients with advanced heart failure and may delay the need for heart transplant.

摘要

背景

1.5心室修复术是针对患有晚期右心衰竭的先天性心脏病患者的一种姑息性手术。关于该手术的患者选择和预后的数据有限。本研究的目的是描述先天性心脏病(CHD)且接受1.5心室修复术的成人患者的临床和血流动力学特征及预后。

方法

我们研究了2003年1月1日至2023年12月31日期间在梅奥诊所接受1.5心室修复术并接受治疗的CHD成人患者。患者被分为两个队列。队列1由成年前(年龄<18岁)接受1.5心室修复术的患者组成,而队列2由成年期(年龄≥18岁)接受1.5心室修复术的患者组成。

结果

共有61例接受1.5心室修复术的患者(队列1 n = 39,64%;队列2 n = 22,36%)。在队列1中,1.5心室修复术时的中位年龄为7岁(四分位间距[IQR]:1 - 12),而到CHD诊所就诊时的中位年龄为20岁(IQR:18 - 27)。队列1的39例患者中,4例(10%)需要再次干预。10年死亡/移植风险为14%。在队列2中,1.5心室修复术时的中位年龄为33岁(IQR:23 - 44),手术死亡率为4%。1.5心室修复术后1年,心输出量、功能状态有所改善,D期心力衰竭患者比例降低。

结论

1.5心室修复术是晚期心力衰竭患者的一种可行治疗选择,可能会延迟心脏移植的需求。

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