International journal of cardiology. Congenital heart disease最新文献

英文中文

Outcome of ventricular assist devices in adults with congenital heart disease – A single center case series 成人先天性心脏病患者心室辅助装置的疗效-单中心病例系列

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2025-12-20 DOI: 10.1016/j.ijcchd.2025.100648

Ahmed Younis , Zeyad M. Kholeif , William R. Miranda , Heidi M. Connolly , Mauricio T. Villavicencio , Joseph A. Dearani , Alexander C. Egbe

引用次数: 0

Progression of stenosis severity and aortopathy in adult patients with congenital aortic stenosis 成人先天性主动脉狭窄患者的狭窄严重程度和主动脉病变进展

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2025-12-16 DOI: 10.1016/j.ijcchd.2025.100646

Zoë A. Keuning , Frederike Meccanici , Kevin M. Veen , Bibi Schreurs , Roland R.J. van Kimmenade , Joost P. van Melle , Monique R.M. Jongbloed , Michiel Voskuil , Berto J. Bouma , Famke Sneep , Jeroen F.A. Simons , Alexander Hirsch , Jolien W. Roos-Hesselink , Annemien E. van den Bosch

Background

Congenital aortic stenosis (AS) is a heterogeneous disease. However, repeated data describing disease progression is limited, especially in adults. Therefore, the objective of this study is to investigate progression of peak velocity and aortic dimensions in adult congenital AS patients, identifying markers for fast progression.

Methods

Adult patients (aged 18–55 years) with a native aortic valve and at least mild AS at baseline registered in the Dutch CONCOR registry between 2001 and 2019 from all six tertiary expert centers for congenital heart disease were included. Patients with severe aortic regurgitation at baseline or no available echocardiograms during follow-up were excluded. Data on ascending aortic dimensions and peak velocity changes over time were analyzed using mixed models until death, aortic valve replacement or March 1, 2023.

Results

402 patients (63 % male) were included with a median age of 26 [IQR 19–41] years and peak velocity of 3.1 [IQR 2.7–3.6] m/s. Median follow-up time was 6.8 [IQR 3.8–11.6] years. Peak velocity significantly progressed over time with 0.06 ± 0.10 m/s/year (p < 0.001), independent of baseline velocity. Older age and concentric left ventricular (LV) remodeling were associated with faster progression. Mean progression of ascending aortic dimension was 0.4 ± 0.5 mm/year (p < 0.001), with faster progression in younger patients (p = 0.002). No correlation between aortic growth rate and stenosis severity progression was seen (r = 0.001, p = 0.98).

Conclusion

Overall disease progression was slow in adult congenital AS patients, and independent of baseline peak velocity. Progression of stenosis should be monitored more carefully in older patients and patients with signs of concentric LV remodeling.

背景先天性主动脉瓣狭窄（AS）是一种异质性疾病。然而，描述疾病进展的重复数据有限，特别是在成人中。因此，本研究的目的是研究成人先天性AS患者的峰值流速和主动脉尺寸的进展，确定快速进展的标志物。方法纳入2001年至2019年期间在荷兰CONCOR登记的所有六个先天性心脏病三级专家中心登记的具有天然主动脉瓣且基线时至少轻度AS的成年患者（18-55岁）。排除基线时有严重主动脉反流或随访时无超声心动图的患者。使用混合模型分析升主动脉尺寸和峰值流速随时间变化的数据，直到死亡、主动脉瓣置换术或2023年3月1日。结果402例患者（63%为男性），中位年龄26 [IQR 19-41]岁，峰值流速3.1 [IQR 2.7-3.6] m/s。中位随访时间为6.8年[IQR 3.8 ~ 11.6]年。峰值速度随时间显著增加，为0.06±0.10 m/s/年（p < 0.001），与基线速度无关。年龄越大，左室同心重构越快。升主动脉直径的平均进展为0.4±0.5 mm/年（p < 0.001），年轻患者的进展更快（p = 0.002）。主动脉生长速率与狭窄严重程度进展无相关性（r = 0.001, p = 0.98）。结论成人先天性AS患者总体病情进展缓慢，且与基线峰值速度无关。老年患者和有同心性左室重构迹象的患者应更仔细地监测狭窄的进展。

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引用次数: 0

Strength and resistance training in adult congenital heart disease: A guide to exercise prescription in the clinic 成人先天性心脏病的力量和阻力训练：临床运动处方指南

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2025-12-27 DOI: 10.1016/j.ijcchd.2025.100650

Calum Dolan , Gemma Brown , Elaine Muirhead , Lorna Swan

引用次数: 0

Clinical worsening in adult congenital heart disease and heart failure: A single-centre, observational study 成人先天性心脏病和心力衰竭的临床恶化：一项单中心观察性研究

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2026-03-01 Epub Date: 2026-01-27 DOI: 10.1016/j.ijcchd.2026.100656

Thibault Bourgeois , Juliette Hubert , Pieter De Meester , Thibault Petit , Els Troost , Lucas Van Aelst , Filip Rega , Philip Moons , Werner Budts , Alexander Van De Bruaene

Background

Data on contemporary treatment approaches and reliable markers of clinical worsening in adult patients with congenital heart disease and heart failure (ACHD-HF) are scarce. This study aimed at evaluating (1) medical therapy within a contemporary ACHD-HF cohort and (2) the incidence of various (composite) endpoints across anatomical and pathophysiological subgroups.

Methods

Retrospective study including ACHD-HF patients (≥16 years) under active follow-up at a tertiary care center, monitored until last visit, death, ventricular assist device (VAD) implantation, or heart transplant (HTX). Medical therapy was documented at time of inclusion and final follow-up. Clinical endpoints were evaluated, after excluding patients with early events (<1 week of inclusion) or without follow-up. Endpoints included HF hospitalization, increase of diuretic treatment, NYHA class progression, all-cause mortality, HTX and VAD placement.

Results

Of the 256 ACHD-HF patients (mean age 50 ± 17 years; 52 % male), 56 were excluded for the final analysis. Two hundred patients were followed for a median of 30 (IQR 22–36) months. Changes in medical therapy included: increased use of mineralocorticoid receptor antagonists (p = 0.038), sodium-glucose transport protein 2 inhibitors (p = 0.002) and a trend towards increased use of angiotensin receptor-neprilysin inhibitors (p = 0.070). Furthermore, whereas HF hospitalization (p < 0.001) and progression of NYHA (p = 0.016) were associated with death, whereas HTX or VAD implant and escalation of diuretic therapy (p = 0.961) were not.

Conclusions

This study characterizes current ACHD-HF therapy and identifies NYHA progression and HF hospitalization as potential markers of clinical worsening as they relate to death, HTX, and VAD implantation.

关于成年先天性心脏病和心力衰竭（ACHD-HF）患者的当代治疗方法和临床恶化的可靠标志物的数据很少。本研究旨在评估(1)当代ACHD-HF队列的药物治疗和(2)跨解剖和病理生理亚组的各种（复合）终点的发生率。方法回顾性研究纳入三级保健中心积极随访的ACHD-HF患者（≥16岁），监测至最后一次就诊、死亡、心室辅助装置（VAD）植入或心脏移植（HTX）。在纳入和最后随访时记录药物治疗。在排除早期事件（纳入1周）或未随访的患者后，评估临床终点。终点包括HF住院、利尿剂治疗增加、NYHA分级进展、全因死亡率、HTX和VAD放置。结果256例ACHD-HF患者（平均年龄50±17岁，52%为男性），56例被排除在最终分析之外。200例患者随访时间中位数为30 （IQR 22-36）个月。药物治疗的变化包括：矿皮质激素受体拮抗剂的使用增加（p = 0.038），钠-葡萄糖转运蛋白2抑制剂（p = 0.002）和血管紧张素受体-萘普利素抑制剂使用增加的趋势（p = 0.070）。此外，HF住院（p < 0.001）和NYHA进展（p = 0.016）与死亡相关，而HTX或VAD植入和利尿剂治疗升级（p = 0.961）与死亡无关。本研究确定了当前ACHD-HF治疗的特点，并确定NYHA进展和HF住院是临床恶化的潜在标志，因为它们与死亡、HTX和VAD植入有关。

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引用次数: 0

Burden of diffuse myocardial fibrosis assessed by cardiac magnetic resonance in repaired tetralogy of Fallot: A systematic review and meta-analysis 通过心脏磁共振评估修复法洛四联症患者弥漫性心肌纤维化负担：一项系统回顾和荟萃分析

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-12-01 Epub Date: 2025-10-24 DOI: 10.1016/j.ijcchd.2025.100631

José Carlos P. Secco , Eduardo Schaustz , Adriana M. Innocenzi , Fernanda.P. Fernandes , Emiliano Medei , Andréa Silvestre-Sousa , Gabriel C. Camargo , Ronir R. Luiz , Jessica P.L. Moreira , Carlos A.S. Magliano , Renata Moll-Bernardes , Mariana Póvoa-Corrêa

Background

The assessment of myocardial fibrosis by cardiac magnetic resonance (CMR) in patients with repaired tetralogy of Fallot (rTOF) is not routinely performed, primarily due to technical challenges in measuring right ventricular native T1 and extracellular volume (ECV). Additionally, although native T1 can be assessed without contrast, offering broader applicability, its role in evaluating myocardial fibrosis in rTOF patients remains unclear.

Objectives

The aim of this systematic review and meta-analysis was to evaluate interstitial fibrosis in both the right and left ventricles using native T1 and ECV quantification in patients with rTOF.

Methods

This systematic review was conducted according to the 2020 PRISMA guidelines and was registered in PROSPERO. The PubMed, Scopus, and Embase databases were searched to identify studies in which patients with rTOF underwent CMR with native T1 and ECV assessment. Random-effect models were used to estimate pooled effect sizes.

Results

The sample comprised 14 studies that included 893 patients with rTOF and 284 controls. LV native T1 values did not differ significantly between groups, except in the studies with a 3.0 T scanner (d = 3.52; p < 0.01). RV values were significantly elevated in patients with rTOF (d = 1.36, p < 0.01). The left ventricle and right ventricle ECVs were significantly higher in these patients than in controls (d = 0.95 and 1.11, respectively; both p < 0.01).

Conclusion

Despite successful surgical repair, patients with rTOF frequently exhibit evidence of myocardial fibrosis, a marker of adverse remodeling that may progress long after anatomical correction.

通过心脏磁共振（CMR）评估修复法洛四联症（rTOF）患者的心肌纤维化并没有常规进行，主要是由于测量右心室原生T1和细胞外体积（ECV）的技术挑战。此外，虽然原生T1可以不进行对比评估，具有更广泛的适用性，但其在评估rTOF患者心肌纤维化中的作用尚不清楚。本系统综述和荟萃分析的目的是利用原生T1和ECV量化评估rTOF患者的左右心室间质纤维化。方法本系统评价按照2020年PRISMA指南进行，并在PROSPERO注册。检索PubMed、Scopus和Embase数据库，以确定rTOF患者接受CMR并进行原生T1和ECV评估的研究。随机效应模型用于估计合并效应大小。结果共纳入14项研究，包括893例rTOF患者和284例对照组。除了使用3.0 T扫描仪的研究外，各组间LV原生T1值无显著差异（d = 3.52; p < 0.01）。rTOF患者的RV值显著升高（d = 1.36, p < 0.01）。这些患者的左心室和右心室ecv明显高于对照组（d分别= 0.95和1.11，p均为0.01）。结论：尽管手术修复成功，但rTOF患者经常表现出心肌纤维化的迹象，这是一种不良重构的标志，可能在解剖矫正后很长一段时间内持续发展。

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引用次数: 0

Congenital heart disease in men of reproductive age: Nationwide cohort studies of prevalence, male fertility, and birth outcomes in offspring 育龄男性先天性心脏病：患病率、男性生育能力和后代出生结局的全国队列研究

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-12-01 Epub Date: 2025-11-06 DOI: 10.1016/j.ijcchd.2025.100637

Marit Sandberg , Nina Øyen , Tatiana Fomina , Ferenc Macsali , Gottfried Greve , Elisabeth Leirgul

Background

The potential for men with congenital heart disease (CHD) to father children and the impact of paternal CHD on offspring birth outcomes are not well understood.

Methods

Using Norwegian nationwide registries from 1994 to 2014, we identified two study populations: 1 829 877 men aged 18–50 years and 1 207 410 newborns. First, we reported the time trends of CHD in men and paternal CHD in newborns. Second, we compared the rate of becoming fathers in men with and without CHD. Third, we compared birth outcomes in offspring with and without paternal CHD.

Results

Between 1994 and 2014, the prevalence of CHD in men of reproductive age increased from 22.7 to 32.2 per 10 000, while the prevalence of paternal CHD in newborns increased from 21.6 to 26.9 per 10 000. Men with mild CHD had a similar rate of becoming fathers compared to men without CHD (rate ratio 0.97, 95 % confidence interval 0.90–1.05), but men with moderate/severe CHD had a lower rate (rate ratio 0.78, 95 % confidence interval 0.70–87). Newborns with paternal CHD had no increased risk of preterm birth or being small for gestational age, compared to newborns without paternal CHD.

Conclusion

The prevalence of CHD in men and paternal CHD in newborns was increasing. Moderate/severe CHD in men was associated with a lower rate of becoming fathers compared to the general male population. Paternal CHD was not associated with an increased risk of newborns being preterm or small for gestational age.

研究背景先天性心脏病（CHD）男性是否有可能成为孩子的父亲，以及父亲的CHD对后代出生结局的影响尚不清楚。方法利用1994 - 2014年挪威全国登记资料，确定了两个研究人群：18-50岁男性1 829 877人，新生儿1 207 410人。首先，我们报道了男性冠心病和新生儿父亲冠心病的时间趋势。其次，我们比较了有和没有冠心病的男性成为父亲的比率。第三，我们比较了有和没有父亲冠心病的后代的出生结果。结果1994 - 2014年育龄男性冠心病患病率从22.7 / 1万上升到32.2 / 1万，新生儿父亲冠心病患病率从21.6 / 1万上升到26.9 / 1万。与非冠心病男性相比，轻度冠心病男性成为父亲的比率相似（比率比0.97,95%可信区间0.90-1.05），但中度/重度冠心病男性的比率较低（比率比0.78,95%可信区间0.70-87）。与没有父亲冠心病的新生儿相比，父亲冠心病的新生儿早产或胎龄小的风险没有增加。结论男性冠心病患病率和新生儿父系冠心病患病率呈上升趋势。与一般男性人群相比，中度/重度冠心病男性成为父亲的几率较低。父亲患冠心病与新生儿早产或小于胎龄的风险增加无关。

{"title":"Congenital heart disease in men of reproductive age: Nationwide cohort studies of prevalence, male fertility, and birth outcomes in offspring","authors":"Marit Sandberg , Nina Øyen , Tatiana Fomina , Ferenc Macsali , Gottfried Greve , Elisabeth Leirgul","doi":"10.1016/j.ijcchd.2025.100637","DOIUrl":"10.1016/j.ijcchd.2025.100637","url":null,"abstract":"<div><h3>Background</h3><div>The potential for men with congenital heart disease (CHD) to father children and the impact of paternal CHD on offspring birth outcomes are not well understood.</div></div><div><h3>Methods</h3><div>Using Norwegian nationwide registries from 1994 to 2014, we identified two study populations: 1 829 877 men aged 18–50 years and 1 207 410 newborns. First, we reported the time trends of CHD in men and paternal CHD in newborns. Second, we compared the rate of becoming fathers in men with and without CHD. Third, we compared birth outcomes in offspring with and without paternal CHD.</div></div><div><h3>Results</h3><div>Between 1994 and 2014, the prevalence of CHD in men of reproductive age increased from 22.7 to 32.2 per 10 000, while the prevalence of paternal CHD in newborns increased from 21.6 to 26.9 per 10 000. Men with mild CHD had a similar rate of becoming fathers compared to men without CHD (rate ratio 0.97, 95 % confidence interval 0.90–1.05), but men with moderate/severe CHD had a lower rate (rate ratio 0.78, 95 % confidence interval 0.70–87). Newborns with paternal CHD had no increased risk of preterm birth or being small for gestational age, compared to newborns without paternal CHD.</div></div><div><h3>Conclusion</h3><div>The prevalence of CHD in men and paternal CHD in newborns was increasing. Moderate/severe CHD in men was associated with a lower rate of becoming fathers compared to the general male population. Paternal CHD was not associated with an increased risk of newborns being preterm or small for gestational age.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100637"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145578757","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Outcomes of a mental health screening program for adults with congenital heart disease 成人先天性心脏病患者心理健康筛查项目的结果

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-12-01 Epub Date: 2025-09-28 DOI: 10.1016/j.ijcchd.2025.100623

Alice Zhang , Matthew W. Buelow , Cheryl L. Brosig , Amber Craig , Lawrence A. Miller , Jane M. Sowinski , Patricia M. Stoll , Kristen Andersen , Scott B. Cohen

Background

Adult congenital heart disease (ACHD) patients face a significant burden of mental health challenges related to their primary cardiac disease.

Objectives

This study characterizes the outcomes of mental health follow up, the prevalence and associated risk factors for mental health concerns in our single center ACHD population.

Methods

The Wisconsin Adult Congenital Heart Disease (WAtCH) program began an outpatient mental health screening program in May 2021 using the Patient Health Questionnaire-4 (PHQ-4). Through a retrospective chart review of ACHD patients seen from May 3, 2021, to May 5, 2022, we report on outcomes of a mental health referral process for ACHD patients with positive mental health screens. We also examined associations between mental health screens and patient demographic and clinical variables.

Results

Among 778 ACHD patient encounters (from 681 patients), there were 213 (27.4 %) positive mental health screens. Of 157 patient encounters with a positive screen and a recorded mental health disposition, 55 (35 %) reported no preexisting mental health provider. Patients at 21 of these encounters accepted a mental health referral, resulting in 3 completed appointments with an in-house mental health specialist. Younger age, female gender, minority race, great complexity congenital heart disease (CHD), were independently associated with a positive screen.

Conclusions

We found limited established mental health care and a low rate of mental health follow up in our ACHD population with a positive mental health screen. Several demographic and disease related factors are related to a greater risk of mental health concerns in ACHD patients.

成人先天性心脏病（ACHD）患者面临着与其原发性心脏病相关的心理健康挑战的重大负担。目的探讨单中心ACHD人群的心理健康随访结果、患病率及相关危险因素。威斯康星州成人先天性心脏病（WAtCH）项目于2021年5月开始使用患者健康问卷-4 （PHQ-4）进行门诊心理健康筛查项目。通过对2021年5月3日至2022年5月5日期间ACHD患者的回顾性图表回顾，我们报告了心理健康筛查呈阳性的ACHD患者的心理健康转诊过程的结果。我们还研究了心理健康筛查与患者人口统计学和临床变量之间的关系。结果778例ACHD患者（来自681例患者）中，213例（27.4%）心理健康筛查呈阳性。在157名筛查呈阳性并有心理健康倾向记录的患者中，55名（35%）报告没有先前存在的心理健康提供者。其中21名患者接受了心理健康转诊，与内部心理健康专家完成了3次预约。年龄较小、女性、少数民族、高度复杂性先天性心脏病（CHD）与筛查阳性独立相关。结论：我们发现，在心理健康筛查呈阳性的ACHD人群中，现有的心理健康护理有限，心理健康随访率较低。一些人口统计学和疾病相关因素与ACHD患者更大的心理健康问题风险有关。

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引用次数: 0

Changes in the congenital heart disease spectrum at China national children's medical center: a review of 10-year data 中国国家儿童医疗中心先天性心脏病谱的变化：10年数据回顾

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-12-01 Epub Date: 2025-10-23 DOI: 10.1016/j.ijcchd.2025.100633

Jia Zheng, Zhiqiang Li, Song Bai, Jian Guo, Zhenjiang Song, Qilin Li, Nan Ding, Yaobin Zhu, Fan Fan, Zhangke Guo, Hanlu Yi

Background

With the decline in the birth rate and simultaneous advancements in medical technology in China, research on how birth population changes have affected congenital heart disease (CHD) surgery in the last 10 years has increased.

Method

We investigated the medical data of all patients diagnosed with CHD treated at the surgical department at our hospital from January 2013 to December 2022, including their age and the type of surgery. We classified patients into two groups based on their age: less than 1 year (younger) and greater than 1 year (older). We defined patent ductus arteriosus, atrial septal defect, and ventricular septal defect as simple CHDs and others as complex diseases. Vascular ring diseases, including double aortic arch, pulmonary artery sling, and aberrant left subclavian artery with diverticulum, were considered special conditions.

Result

We found that 6440 patients underwent surgery at our hospital in the period mentioned. The number of surgeries conducted annually increased significantly from 609 in 2016 to 941 in 2019 but gradually declined since 2020. The proportion of simple CHD cases increased annually, whereas the proportion of complex CHD cases declined. The proportion of patients with vascular ring disease gradually increased. The proportion of older children increased, whereas that of younger children decreased each year. All changes were statistically significant.

Conclusions

Owing to advancements in medical technology, economic factors, change of birth rate and abortion rate, the number of surgeries for CHDs has decreased significantly. It is challenging to predict future trends. Nevertheless, this phenomenon is specific to China. Policymakers, hospitals, and surgeons must introduce appropriate adjustments in response to this change.

随着中国出生率的下降和医疗技术的进步，近10年来出生人口变化对先天性心脏病（CHD）手术的影响研究越来越多。方法调查2013年1月至2022年12月在我院外科就诊的所有冠心病患者的医疗资料，包括年龄、手术类型等。我们根据患者的年龄将其分为两组：小于1岁（年轻）和大于1岁（年长）。我们将动脉导管未闭、房间隔缺损和室间隔缺损定义为单纯性冠心病，将其他疾病定义为复杂疾病。血管环疾病，包括双主动脉弓，肺动脉悬吊，左锁骨下动脉异常伴憩室，被认为是特殊情况。结果同期在我院接受手术治疗的患者为6440例。每年的手术数量从2016年的609例大幅增加到2019年的941例，但从2020年开始逐渐下降。单纯性冠心病所占比例逐年上升，而复合性冠心病所占比例逐年下降。血管环病患者比例逐渐增加。年龄较大的儿童所占比例逐年上升，而年龄较小的儿童所占比例逐年下降。所有变化均具有统计学意义。结论随着医疗技术进步、经济因素、出生率和流产率的变化，冠心病手术数量明显减少。预测未来的趋势是具有挑战性的。然而，这种现象是中国特有的。决策者、医院和外科医生必须针对这一变化进行适当的调整。

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引用次数: 0

Congenital uterine anomalies and congenital heart disease: An underappreciated association? 先天性子宫异常与先天性心脏病：一种未被充分认识的关联？

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-12-01 Epub Date: 2025-10-24 DOI: 10.1016/j.ijcchd.2025.100634

Harrison J. VanDolah , Kathy Mostajeran , Luis Goncalves , Wayne J. Franklin , Melissa Parks , Tabitha G. Moe

Background

Congenital uterine anomalies (CUAs), also known as Müllerian duct anomalies, can have an impact on gynecological care, pre-pregnancy counseling, and obstetrical outcomes. Patients with congenital heart disease (CHD), particularly of higher complexity, often have extracardiac manifestations. The relationship between CUAs and CHD is not well-described in the currently available literature.

Methods

A single center retrospective cohort study was performed of all patients born female with ICD-10 codes corresponding to congenital heart disease and a completed pelvic MRI or ultrasound. CT imaging was not included given the known shortcomings of CUA detection and classification with CT. CHD diagnoses were confirmed with echocardiography and were classified into “simple”, “moderate”, or “complex” per the 2018 ACHD guidelines. CUAs were categorized based on the 2021 American Society of Reproductive Medicine Guidelines for classification of Müllerian anomalies.

Results

197 patients met inclusion criteria. 33 (16.7 %) were found to have a CUA. 17 (20 %) patients with moderate or complex CHD had a CUA. Notable associations include VACTERL and partial anomalous pulmonary venous return. More complex CHD was associated with more complex CUA.

Conclusions

Within the limitations of a single center retrospective study, our data suggests there is an underappreciated association between CUA and CHD that requires further prospective multicenter study to assess prevalence data. There may ultimately be a role for targeted screening for CUA in patients with CHD, particularly those with genetic syndromes or moderate or complex CHD who are considering pregnancy or gynecologic intervention.

背景先天性子宫异常（CUAs），也被称为胆管异常，可对妇科护理、孕前咨询和产科结果产生影响。先天性心脏病（CHD）患者，特别是复杂性较高的患者，通常有心外表现。在目前的文献中，CUAs与冠心病之间的关系并没有得到很好的描述。方法采用单中心回顾性队列研究方法，对所有出生时患有先天性心脏病ICD-10编码并完成盆腔MRI或超声检查的女性患者进行研究。鉴于已知CT检测和分类CUA的缺点，未包括CT成像。通过超声心动图确诊冠心病，并根据2018年冠心病指南将其分为“简单”、“中度”和“复杂”。根据2021年美国生殖医学学会关于勒氏管异常分类的指南对cua进行分类。结果197例患者符合纳入标准。33例（16.7%）发现有CUA。17例（20%）中度或复杂冠心病患者发生CUA。值得注意的关联包括VACTERL和部分肺静脉异常回流。更复杂的冠心病与更复杂的CUA相关。结论：在单中心回顾性研究的限制下，我们的数据表明CUA和冠心病之间存在未被充分认识的关联，需要进一步的前瞻性多中心研究来评估患病率数据。对冠心病患者，特别是那些有遗传综合征或中度或复杂冠心病且考虑妊娠或妇科干预的患者，可能最终有针对性地筛查CUA。

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引用次数: 0

Machine learning-based predictive model for atrial arrhythmia following transcatheter atrial septal defect closure 基于机器学习的经导管房间隔缺损闭合后房性心律失常预测模型

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-12-01 Epub Date: 2025-11-05 DOI: 10.1016/j.ijcchd.2025.100639

Xander Jacquemyn , Alexander Van De Bruaene , Joris Ector , Peter Haemers , Pieter De Meester , Cedric Manlhiot , Werner Budts , Bert Vandenberk

Background

Atrial septal defects (ASDs) are frequently closed percutaneously. Despite successful closure, many patients still develop atrial arrhythmias. There is inconsistent data on the risk factors associated with these atrial arrhythmias. As such, we aimed to develop a machine learning (ML) model predicting atrial arrhythmias following transcatheter ASD closure in adolescents and adults.

Methods

Patients with secundum-type ASDs undergoing transcatheter closure between 2008 and 2024 at a single center were retrospectively analyzed. Patients with prior atrial arrhythmias were excluded. A deep neural network, adapted via transfer learning from a large external ECG dataset, was used to extract predictive features from preprocedural 12-lead ECGs. These features were combined with clinical, demographic, biochemical and hemodynamic variables in ensemble survival models. Model performance was assessed using the integrated Brier scores and the area under the receiver operating curve (AUC).

Results

A total of 148 adult patients (median 44.4 years [30.6–57.8], 105 females [70.9 %]) were eligible for included. There were a total of 1055 person-years of follow-up (median follow-up 7.3 [3.1–11.3]), during which 28 patients (18.9 %) developed atrial arrhythmias. The final ensemble ML model incorporating ECG-derived features demonstrated strong predictive performance (integrated Brier score 0.044, mean AUC 0.823). Subgroup and sensitivity analyses confirmed the robustness of the model across various patient profiles.

Conclusions

We developed a novel ML-based risk model using a transfer learning approach to predict atrial arrhythmias after transcatheter ASD closure. Further research and external validation are needed to refine the proposed risk stratification prior to clinical implementation.

背景：室间隔缺损（asd）通常是经皮缝合的。尽管成功关闭，许多患者仍然发生心房心律失常。关于与这些心房心律失常相关的危险因素的数据不一致。因此，我们旨在开发一种机器学习（ML）模型，预测青少年和成人经导管ASD关闭后的心房心律失常。方法回顾性分析2008 ~ 2024年在单中心行经导管闭合术的二次型asd患者。排除既往有心房心律失常的患者。通过从大型外部心电数据集进行迁移学习，采用深度神经网络从术前12导联心电图中提取预测特征。在整体生存模型中，这些特征与临床、人口统计学、生化和血流动力学变量相结合。采用综合Brier评分和受试者工作曲线下面积（AUC）评估模型性能。结果共纳入148例成人患者，中位年龄44.4岁[30.6 ~ 57.8岁]，女性105例[70.9%]。共随访1055人-年（中位随访7.3[3.1-11.3]），其中28例（18.9%）发生心房心律失常。结合心电图衍生特征的最终集成ML模型表现出较强的预测性能（综合Brier评分0.044，平均AUC 0.823）。亚组分析和敏感性分析证实了该模型在不同患者资料中的稳健性。我们开发了一种新的基于ml的风险模型，使用迁移学习方法来预测经导管ASD关闭后的心房心律失常。在临床实施之前，需要进一步的研究和外部验证来完善建议的风险分层。

{"title":"Machine learning-based predictive model for atrial arrhythmia following transcatheter atrial septal defect closure","authors":"Xander Jacquemyn , Alexander Van De Bruaene , Joris Ector , Peter Haemers , Pieter De Meester , Cedric Manlhiot , Werner Budts , Bert Vandenberk","doi":"10.1016/j.ijcchd.2025.100639","DOIUrl":"10.1016/j.ijcchd.2025.100639","url":null,"abstract":"<div><h3>Background</h3><div>Atrial septal defects (ASDs) are frequently closed percutaneously. Despite successful closure, many patients still develop atrial arrhythmias. There is inconsistent data on the risk factors associated with these atrial arrhythmias. As such, we aimed to develop a machine learning (ML) model predicting atrial arrhythmias following transcatheter ASD closure in adolescents and adults.</div></div><div><h3>Methods</h3><div>Patients with secundum-type ASDs undergoing transcatheter closure between 2008 and 2024 at a single center were retrospectively analyzed. Patients with prior atrial arrhythmias were excluded. A deep neural network, adapted via transfer learning from a large external ECG dataset, was used to extract predictive features from preprocedural 12-lead ECGs. These features were combined with clinical, demographic, biochemical and hemodynamic variables in ensemble survival models. Model performance was assessed using the integrated Brier scores and the area under the receiver operating curve (AUC).</div></div><div><h3>Results</h3><div>A total of 148 adult patients (median 44.4 years [30.6–57.8], 105 females [70.9 %]) were eligible for included. There were a total of 1055 person-years of follow-up (median follow-up 7.3 [3.1–11.3]), during which 28 patients (18.9 %) developed atrial arrhythmias. The final ensemble ML model incorporating ECG-derived features demonstrated strong predictive performance (integrated Brier score 0.044, mean AUC 0.823). Subgroup and sensitivity analyses confirmed the robustness of the model across various patient profiles.</div></div><div><h3>Conclusions</h3><div>We developed a novel ML-based risk model using a transfer learning approach to predict atrial arrhythmias after transcatheter ASD closure. Further research and external validation are needed to refine the proposed risk stratification prior to clinical implementation.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100639"},"PeriodicalIF":1.2,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145528524","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

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