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

IF 6 1区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Journal of Heart and Lung Transplantation Pub Date : 2025-04-01 Epub Date: 2025-03-18 DOI:10.1016/j.healun.2024.12.020
Alexander C. Egbe MD, MPH, MS, FACC , William R. Miranda MD , Heidi M. Connolly MD , Joseph A. Dearani MD
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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.
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成人先天性心脏病和1.5心室修复的长期预后
背景:1.5心室修复是先天性心脏病伴晚期右心衰患者的一种姑息性手术。关于该手术的患者选择和结果的数据有限。本研究的目的是描述成人先天性心脏病(CHD)和1.5心室修复的临床和血流动力学特征和结果。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心室修复并接受治疗的成人冠心病患者。患者被分为两组。队列1包括成年前接受1.5次心室修复的患者(年龄≥18岁),而队列2包括成年期接受1.5次心室修复的患者(年龄≥18岁)。结果61例患者行1.5心室修复术(队列1 n=39,占64%;队列2 n=22, 36%)。在队列1中,接受1.5次心室修复时的中位年龄为7岁(四分位数间距[IQR]: 1 - 12),而到冠心病诊所就诊时的中位年龄为20岁(IQR: 18-27)。在队列1的39例患者中,4例(10%)需要再干预。10年死亡/移植风险为14%。在队列2中,1.5心室修复时的中位年龄为33岁(IQR: 23-44),手术死亡率为4%。心排血量、功能状态均有改善,心室修复1.5次后1年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可行的治疗方案,可延缓心脏移植的需要。
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来源期刊
CiteScore
10.10
自引率
6.70%
发文量
1667
审稿时长
69 days
期刊介绍: The Journal of Heart and Lung Transplantation, the official publication of the International Society for Heart and Lung Transplantation, brings readers essential scholarly and timely information in the field of cardio-pulmonary transplantation, mechanical and biological support of the failing heart, advanced lung disease (including pulmonary vascular disease) and cell replacement therapy. Importantly, the journal also serves as a medium of communication of pre-clinical sciences in all these rapidly expanding areas.
期刊最新文献
Cover Front matter Long-Term Cardiac Allograft Vasculopathy Outcomes After Heart Transplantation: Donation After Circulatory Death vs Donation After Brain Death Donation After Circulatory Death Heart Retransplantation in Adults in the United States: Early Outcomes and Listing Implications Local Donor Heart Procurement is Associated with Cardioprotection and Less Primary Graft Dysfunction Than Remote Procurement
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