丰唐循环与全身性疾病--35年随访的回顾性队列分析：35年后的丰唐全身性疾病。

IF 3.7 2区医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS

American heart journal Pub Date : 2024-10-17 DOI:10.1016/j.ahj.2024.10.008

Gruschen R. Veldtman MBChB, FRCP , Ali Abualsaud MD , Sarah Cohen MD, PhD , Maria Victoria Ordonez MD , Liming Guo MSc , Chao Li MSc , Aihua Liu PhD , Jasmine Grewal MD , Michelle Gurvitz MD , Judith Therrien MD , Ariane Marelli MD, MPH

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引用次数: 0

摘要

背景：Fontan手术可挽救单心室（SV）生理患者的生命。鉴于近年来人们对丰坦循环患者全身性疾病（SD）的关注，我们试图：1）量化与丰坦循环相关的SD发生率的增加；2）确定SD的风险因素；3）评估丰坦循环患者SD与死亡率之间的关系：方法：采用匹配的回顾性队列研究设计。从魁北克先天性心脏病（CHD）数据库中筛选出随访时间长达35年、在丰坦手术后存活至少30天的患者。在每位丰坦患者中，随机抽取20名性别和年龄相同的孤立性室间隔缺损（VSD）患者组成对照组。心外并发症（包括肝脏、呼吸系统、胃肠道或肾脏疾病）导致至少一次住院即定义为存在室间隔缺损。通过卡普兰-梅耶曲线分析和考克斯比例危险模型等时间到事件分析，评估SD的累积风险、SD的风险因素以及SD与10年死亡率之间的关系：结果：共发现了533例Fontan循环患者，并与10280例VSD患者进行了配对。在10年和35年的随访中，Fontan循环患者发生SD的累积概率分别为59.02%和89.66%，是VSD患者的4-7倍（分别为8.68%和23.34%；LogRank检验P1心外器官受影响），与无SD相比，SD与10年死亡风险增加3.38倍（95%CI：1.73-6.60）有关：这项以人群为基础的研究表明，Fontan 循环患者发生 SD 的风险增加，进而导致更高的死亡风险。这些研究结果表明，有必要对Fontan手术后患者的心脏和全身疾病进行更系统的监测。

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Fontan circulation and systemic disease – a retrospective cohort analysis over 35 years of follow-up

Background

The Fontan operation provides lifesaving palliation for individuals with single ventricle (SV) physiology. Given recent concerns of systemic disease (SD) for patients with a Fontan circulation, we sought to (1) quantify the increase in SD incidence associated with the Fontan circulation; (2) identify the risk factor of SD; (3) assess the association between SD and mortality in patients with a Fontan circulation.

Methods

A matched retrospective cohort study design was adopted. From the Quebec Congenital Heart Disease (CHD) Database with up to 35 years of follow-up, patients who survived at least 30 days after the Fontan operation were identified. For each Fontan patient, patients with isolated ventricular septal defect (VSD) with the same sex and age were identified and 20 of them were randomly selected to form the control group. The presence of SD was defined as at least 1 hospitalization due to extra-cardiac complications including liver, respiratory, gastrointestinal or renal disease. Time-to-event analysis including Kaplan-Meier curve analysis and Cox proportional hazard models were conducted to assess the cumulative risk of SD, risk factors of SD, and the association between SD and 10-year mortality.

Results

A total of 533 patients with Fontan circulation were identified and matched with 10,280 VSD patients. The cumulative probabilities of SD at 10- and 35-years follow-up were 59.02% and 89.66% in patients with a Fontan circulation, 4 to 7 times of the probabilities in VSD patients (8.68% and 23.34%, respectively; LogRank tests P < .0001). In Fontan patients, cardiovascular complications were associated with a 4.1-fold (95% CI: 3.52-4.88) higher risk of developing SD. Multisystem disease (>1 extra-cardiac organ affected) disease was associated with a 3.38-fold (95%CI: 1.73-6.60) increase in 10-year mortality risk when comparing to the absence of SD.

Conclusions

This population-based study demonstrated that patients with a Fontan circulation had increased risk of SD, which in turn led to higher risk of mortality. These findings underscore the need for more systematic surveillance of cardiac and systemic disease for patients after Fontan operation.

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来源期刊

American heart journal 医学-心血管系统

CiteScore

8.20

自引率

2.10%

发文量

214

审稿时长

38 days

期刊介绍： The American Heart Journal will consider for publication suitable articles on topics pertaining to the broad discipline of cardiovascular disease. Our goal is to provide the reader primary investigation, scholarly review, and opinion concerning the practice of cardiovascular medicine. We especially encourage submission of 3 types of reports that are not frequently seen in cardiovascular journals: negative clinical studies, reports on study designs, and studies involving the organization of medical care. The Journal does not accept individual case reports or original articles involving bench laboratory or animal research.