International journal of cardiology. Congenital heart disease最新文献_第2页

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 : 2025-11-05 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。

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引用次数: 0

Clinical characteristics and outcomes of cardiovascular surgery and transcatheter procedures in adults with Down syndrome and congenital heart disease 成人唐氏综合征和先天性心脏病的心血管手术和经导管手术的临床特点和结果

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-11-05 DOI: 10.1016/j.ijcchd.2025.100638

Takumi Osawa , Tomoko Machino-Ohtsuka , Keitaro Kume , Takahiro Yano , Koshiro Kanaoka , Yoko Sumita , Yusuke Yano , Ruriko Numata , Naoto Kawamatsu , Hideyuki Kato , Yuji Hiramatsu , Tomoko Ishizu

Background

Advances in medical care have increased the number of adults with Down syndrome and congenital heart disease (CHD), including those with residual or unrepaired lesions. However, clinical outcomes following invasive cardiovascular procedures in this population remain poorly understood.

Methods

We extracted data on patients with Down syndrome aged ≥16 years and diagnosed with CHD from the Japanese Registry of All Cardiac and Vascular Diseases Diagnosis Procedure Combination (JROAD-DPC), covering the period from April 1, 2013, to March 31, 2023. Patients who underwent cardiovascular surgery or transcatheter procedures were included in the analysis.

Results

The study included 115 patients (median age: 21 [17–33] years; 47.8 % female), of whom 72 underwent surgery and 43 underwent transcatheter intervention. Procedures were most common among those aged 16–29 years. The main CHD subtypes were tetralogy of Fallot, atrial septal defects, and atrioventricular septal defects. Catheter-based interventions were associated with shorter hospital stay and no in-hospital mortality. In contrast, all three deaths (4.2 %) occurred in the surgical group, with pulmonary hypertension medications used in two of these patients. Among surgical patients receiving pulmonary hypertension medications, the in-hospital mortality rate reached 25 %. In multivariate analysis, emergency admission and the use of pulmonary hypertension medications were independently associated with longer hospital stays after surgery.

Conclusions

In adults with Down syndrome and CHD, invasive cardiovascular procedures had relatively acceptable in-hospital outcomes. Careful patient selection and perioperative management are crucial when planning cardiac surgery, especially in patients taking medication for pulmonary hypertension.

医疗保健的进步增加了患有唐氏综合征和先天性心脏病（CHD）的成年人的数量，包括那些有残留或未修复病变的人。然而，在这一人群中，有创心血管手术后的临床结果仍然知之甚少。方法我们从日本所有心血管疾病诊断程序组合登记处（JROAD-DPC）中提取年龄≥16岁且诊断为冠心病的唐氏综合征患者的数据，涵盖时间为2013年4月1日至2023年3月31日。接受心血管手术或经导管手术的患者被纳入分析。结果纳入115例患者，中位年龄21岁[17-33]，女性47.8%，其中手术72例，经导管介入43例。手术在16-29岁的人群中最为常见。冠心病主要亚型为法洛四联症、房间隔缺损和房室间隔缺损。基于导管的干预措施与更短的住院时间和无院内死亡率相关。相比之下，所有3例死亡（4.2%）发生在手术组，其中2例患者使用了肺动脉高压药物。在接受肺动脉高压药物治疗的外科患者中，住院死亡率达到25%。在多变量分析中，急诊入院和肺动脉高压药物的使用与术后住院时间的延长独立相关。结论：在患有唐氏综合征和冠心病的成年人中，有创心血管手术具有相对可接受的住院结果。仔细的病人选择和围手术期管理是至关重要的，当计划心脏手术，特别是在病人服用药物肺动脉高压。

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引用次数: 0

Cardiac autonomic function in patients with single ventricle physiology after Fontan palliation: A literature review Fontan姑息治疗后单心室患者的心脏自主神经功能：文献综述

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-30 DOI: 10.1016/j.ijcchd.2025.100636

Elizaveta Polyakova , Marieke Nederend , Philippine Kies , Anastasia D. Egorova , Monique R.M. Jongbloed

Background

The Fontan operation has significantly improved survival in patients with single ventricle physiology. This comes at a price of highly prevalent long-term complications. Autonomic dysfunction has been documented in patients after Fontan palliation. Although autonomic dysfunction is associated with a range of adverse outcomes, the exact clinical implications in patients with a Fontan circulation remain unclear.

Aims

The aims of this review are to address (1) the extent and characteristics of autonomic dysfunction in Fontan patients; (2) its association with clinical short- and long-term outcomes, and (3) identify key gaps in the literature.

Methods

A literature search was performed in PubMed using a dedicated query for single ventricle disease and cardiac autonomic (dys)function.

Results

Data from the literature consistently indicated overt autonomic dysfunction in patients after Fontan operation versus controls, marked by reduced heart rate variability and impaired parasympathetic tone. However, autonomic dysfunction showed different associations with clinical outcomes in Fontan patients. Several studies reported an association with diminished exercise capacity, arrhythmias, organ function markers, while other studies found no clear predictive value. Gaps in literature include data on adult patients, the influence of anatomical substrate, sex and lifestyle, as well as specific “driving” factors for the development of cardiac autonomic dysfunction.

Conclusion

Autonomic dysfunction in Fontan patients is a persistent and potentially progressive condition, characterized by reduced heart rate variability and reduced parasympathetic tone. Long-term studies are needed to clarify its clinical significance and address gaps in literature relating to contributing factors like sex, anatomy, and underlying mechanisms.

背景Fontan手术可显著提高单心室生理患者的生存率。这是以非常普遍的长期并发症为代价的。在Fontan姑息治疗后的患者中有植物神经功能障碍的记录。尽管自主神经功能障碍与一系列不良后果相关，但Fontan循环患者的确切临床意义尚不清楚。本综述的目的是解决(1)Fontan患者自主神经功能障碍的程度和特征；(2)其与临床短期和长期预后的关系；(3)确定文献中的关键空白。方法在PubMed上使用单心室疾病和心脏自主神经（dys）功能的专用查询进行文献检索。结果来自文献的数据一致表明Fontan手术后患者与对照组相比存在明显的自主神经功能障碍，其特征是心率变异性降低和副交感神经张力受损。然而，自主神经功能障碍与Fontan患者的临床结果有不同的相关性。一些研究报告了与运动能力下降、心律失常、器官功能指标的关联，而其他研究没有发现明确的预测价值。文献中的空白包括成年患者的数据，解剖基质、性别和生活方式的影响，以及心脏自主神经功能障碍发展的具体“驱动”因素。结论Fontan患者的自主神经功能障碍是一种持续的、潜在的进行性疾病，其特征是心率变异性降低和副交感神经张力降低。需要长期的研究来阐明其临床意义，并解决与性别、解剖学和潜在机制等因素相关的文献空白。

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引用次数: 0

How to set up a pregnancy heart team 如何成立孕心小组

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-28 DOI: 10.1016/j.ijcchd.2025.100635

K. Rijs , C.D. van der Marel , A.L.M.J. van der Knijff-van Dortmont , I.J.J. Dons-Sinke , V.F.R. Adriaens , J.M.J. Cornette , A.G.M.G.J. Mulders , J.J. Duvekot , M.M.P. Driessen , A.E. van den Bosch , J.A.A.E. Cuypers , R.M. Kauling , J.W. Roos-Hesselink

Cardiovascular disease (CVD) is the leading cause of maternal morbidity and mortality in developed countries, complicating 4–11 % of pregnancies. Women with CVD are at increased risk of maternal complications, including heart failure and arrhythmias, as well as adverse fetal outcomes such as preterm birth and perinatal mortality. Traditionally, care for pregnant women with CVD has been fragmented across specialties, leading to discontinuity and missed opportunities for optimization. To address this, recent European Society of Cardiology (ESC) guidelines strongly recommend the implementation of a pregnancy heart team: a dedicated multidisciplinary team providing integrated care for this high-risk population.

We describe the implementation and organization of a pregnancy heart team in our academic tertiary hospital. The core team consists of a cardiologist specialized in cardio-obstetrics, a maternal-fetal medicine specialist, and an obstetric anesthesiologist, with additional experts consulted as required. Patients are discussed at predefined time points: preconception, during pregnancy, and postpartum. Standardized risk assessment using the modified World Health Organization (mWHO) classification guides counseling, management, and delivery planning. Shared decision-making is central, balancing maternal and fetal risks with patient preferences. The team establishes individualized peripartum care plans covering timing, mode, and location of delivery, anesthesia strategies, anticoagulation management, and postpartum monitoring.

This structured, multidisciplinary model ensures continuity of care and offers a practical framework to improve outcomes in women with CVD during pregnancy.

心血管疾病（CVD）是发达国家孕产妇发病和死亡的主要原因，使4 - 11%的妊娠并发症。患有心血管疾病的妇女发生产妇并发症的风险增加，包括心力衰竭和心律失常，以及不良胎儿结局，如早产和围产期死亡。传统上，对患有心血管疾病的孕妇的护理分散在各个专科，导致不连续性和错失优化机会。为了解决这个问题，最近的欧洲心脏病学会（ESC）指南强烈建议实施妊娠心脏小组：一个专门的多学科小组，为这一高危人群提供综合护理。介绍了我院三级专科医院妊娠心脏科的实施与组织。核心小组由一名专门从事心脏产科的心脏病专家、一名母胎医学专家和一名产科麻醉师组成，并根据需要咨询其他专家。患者在预定的时间点进行讨论：孕前，怀孕期间和产后。使用经修订的世界卫生组织（mWHO）分类指南进行标准化风险评估，包括咨询、管理和交付计划。共同决策是核心，平衡产妇和胎儿的风险与患者的偏好。团队建立个性化的围产期护理计划，包括分娩的时间、方式和地点、麻醉策略、抗凝管理和产后监测。这种结构化的多学科模式确保了护理的连续性，并为改善妊娠期心血管疾病妇女的预后提供了实用的框架。

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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 : 2025-10-24 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 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 : 2025-10-24 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

Physical fitness in adults with isolated secundum atrial septal defects 孤立性房间隔缺损成人的身体健康状况

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-24 DOI: 10.1016/j.ijcchd.2025.100632

Linda Ashman Kröönström , Anna-Klara Zetterström , Mikael Dellborg , Åsa Cider , Peter Eriksson , Emelie Dahlin , Zacharias Mandalenakis

Background

Atrial septal defect (ASD) is one of the most common diagnoses in patients with adult congenital heart disease (ACHD). However, recent studies indicate that ASD is not so benign and may impede physical fitness. We aimed to assess physical fitness in patients with isolated secundum ASD compared with healthy reference values and to evaluate the results according to the absence or presence of repair.

Methods

Between April 2009 and March 2025, patients with ACHD performed physical fitness tests comprising five muscular fitness tests and one cardiorespiratory endurance test. Data were stored in a registry at the Physical Therapy Department, Sahlgrenska University Hospital/Östra. In total, 102 adults with isolated secundum ASD were included in this descriptive, register-based cohort study.

Results

Both women and men with ASD showed significantly lower isoinertial muscular fitness test results (heel lifts: p < 0.001 and shoulder flexions: p < 0.001) and maximal exercise capacity (p < 0.001) compared with healthy reference values. The shoulder flexion test revealed the lowest percentage of reference values for women and men with ASD (58.3 % and 53.3 %, respectively). Women who had undergone surgical procedures performed the timed-stands test significantly faster when compared with women who had undergone device procedures (13.0 s [10.2, 16.0] vs. 19.1 s [15.1, 26.2]; p = 0.014).

Conclusion

Patients with less complex ACHD, such as secundum ASD, may have impaired muscular fitness and cardiorespiratory endurance. Therefore, all patients with ACHD could be offered tests of physical fitness. These tests are necessary when prescribing individualized exercise, as suggested by guidelines.

背景：房间隔缺损（ASD）是成人先天性心脏病（ACHD）患者最常见的诊断之一。然而，最近的研究表明，自闭症谱系障碍并不是那么良性的，它可能会妨碍身体健康。我们的目的是评估孤立性继发性ASD患者的身体健康状况与健康参考值的比较，并根据是否存在修复来评估结果。方法2009年4月至2025年3月，对ACHD患者进行体能测试，包括5项肌肉体能测试和1项心肺耐力测试。数据存储在Sahlgrenska大学医院物理治疗科的登记处/Östra。这项描述性、基于登记的队列研究共纳入102例孤立性继发性ASD成人患者。结果与健康参考值相比，女性和男性ASD患者的等惯性肌肉健康测试结果（抬跟：p <； 0.001，肩关节屈曲：p <； 0.001）和最大运动能力（p < 0.001）均显著降低。肩关节屈曲试验显示，女性和男性ASD的参考值比例最低（分别为58.3%和53.3%）。接受外科手术的女性比接受器械手术的女性进行计时架测试的时间明显快（13.0 s [10.2, 16.0] vs. 19.1 s [15.1, 26.2]; p = 0.014）。结论继发性ASD等不太复杂的ACHD患者可能存在肌肉健康和心肺耐力受损。因此，所有的ACHD患者都可以进行体能测试。这些测试是必要的，当处方个体化运动，建议的指导方针。

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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 : 2025-10-23 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

Predictors of interruption in medical follow-up among patients with complex adult congenital heart disease 复杂成人先天性心脏病患者医学随访中断的预测因素

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-18 DOI: 10.1016/j.ijcchd.2025.100630

Ryotaro Maeda , Naohiko Nakanishi , Tomoyuki Nagai , Hideo Tsubata , Michiyo Yamano , Tetsuhiro Yamano , Takeshi Nakamura , Yoko Kawai , Yo Kajiyama , Kazuyuki Ikeda , Shuhei Fujita , Shinichiro Oda , Tomoko Iehara , Satoaki Matoba

Background

Many patients with adult congenital heart disease (ACHD) experience interruptions in medical follow-up, leading to a worsening life prognosis, particularly among those with moderate-to-severe complexity. However, the risk factors associated with interruption remain unclear.

Methods

In this questionnaire-based study, we sent postcards containing a QR code linking to the questionnaire to 1226 patients who underwent surgery for congenital heart disease between 1997 and 2023 and were >15 years old at registration. We analyzed the causative factors for interruption in medical follow-up among patients with moderate or severe ACHD complexity.

Results

Of 785 patients who received questionnaire, 211 patients provided valid responses. Among them, 144 patients with moderate-to-severe complexity were included in the analysis to identify factors associated with interruption in medical follow-up. We found that 19.4 % of patients experienced interruptions. 64.0 % of patients discontinued hospital visits between 15 and 19 years old. Multivariate analysis revealed that the perceived need for continued hospital visits was an independent factor associated with interruption.

Conclusions

Our study identified inadequate communication regarding the necessity of continued follow-up as an independent factor contributing to the interruption of medical care. These findings highlight the need for better education for both ACHD specialists and general healthcare providers.

背景：许多成人先天性心脏病（ACHD）患者在医学随访中经历中断，导致生活预后恶化，特别是那些具有中度至重度复杂性的患者。然而，与中断相关的风险因素仍不清楚。方法在这项以问卷为基础的研究中，我们向1997年至2023年间接受先天性心脏病手术的1226例患者发送了包含问卷链接的二维码的明信片，登记时年龄为15岁。我们分析了导致中度或重度ACHD复杂性患者医疗随访中断的原因。结果收到问卷的785例患者中，有211例患者提供有效回复。其中144例中至重度并发症患者纳入分析，以确定中断医学随访的相关因素。我们发现19.4%的患者经历过中断。64.0%的患者在15至19岁之间停止了医院就诊。多变量分析显示，持续就诊的感知需求是与中断相关的独立因素。结论我们的研究发现，关于继续随访必要性的沟通不足是导致医疗服务中断的一个独立因素。这些发现强调了对ACHD专家和一般医疗保健提供者进行更好的教育的必要性。

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引用次数: 0

Mortality rates in adults with severe congenital heart disease: insights from the National Congenital Heart Disease Registry of Australia 严重先天性心脏病的成人死亡率：来自澳大利亚国家先天性心脏病登记处的见解

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-17 DOI: 10.1016/j.ijcchd.2025.100629

Jason Chami MD , Calum Nicholson , David Baker , Rachael Cordina , Geoff Strange , David S. Celermajer

Background

Survival into adulthood with congenital heart disease (CHD) has improved, but premature mortality remains common. Long-term, multi-centre data are scarce. We used a validated complexity algorithm to select “severe CHD” patients from the National CHD Registry of Australia, quantifying mortality by ventricular physiology, residential remoteness and treating-centre location.

Methods

From 70185 CHD patient records, we identified 6557 patients classified as “severe” by 2020 European Society of Cardiology guidelines, born from 1980 onwards and alive at 16 years old. We found 2118 single-ventricle patients, 3674 two-ventricle and 765 with insufficient information for classification. Residence was grouped into Major Cities, Inner Regional, and Outer Regional/Remote. Vital status follow-up was to December 31, 2023. Kaplan–Meier curves were used to compare survival.

Results

Overall, 25-year survival was 96.9 % and 40-year survival was 89.0 %. Survival differed by physiology: single-ventricle survival was 96.4 % at 25 years and 88.2 % at 40 years, while two-ventricle survival was better, at 97.6 % at 25 years and 90.8 % at 40 years (p = 0.0015). Patients managed in Sydney/Melbourne fared better than those treated elsewhere, particularly after age 35 (25-year survival ∼97 % for both; 40-year survival 89.9 % vs 81.9 %; p = 0.027). Survival did not, however, vary with residential remoteness.

Conclusion

Adults with severe lesions, particularly single-ventricle physiology, remain at high risk of premature mortality. Patients managed in Sydney/Melbourne had better survival, possibly reflecting the influence of long-established specialist ACHD programs, although residual confounding cannot be excluded. Urban-rural outcome parity suggests that Australia's centralised care model mitigates geographical barriers.

成年期先天性心脏病（CHD）的生存率有所提高，但过早死亡仍然很常见。长期的、多中心的数据很少。我们使用一种经过验证的复杂性算法从澳大利亚国家冠心病登记处选择“严重冠心病”患者，通过心室生理学、居住距离和治疗中心位置量化死亡率。方法从70185例冠心病患者记录中，我们确定了6557例根据2020年欧洲心脏病学会指南分类为“严重”的患者，这些患者出生于1980年以后，16岁时存活。我们发现2118例单心室患者，3674例双心室患者和765例分类信息不足的患者。居住地分为主要城市、内陆地区和外围地区/偏远地区。重要状态随访至2023年12月31日。Kaplan-Meier曲线用于比较生存率。结果25年生存率为96.9%，40年生存率为89.0%。生存因生理因素而异：25年时单心室生存率为96.4%，40年时为88.2%，而双心室生存率更好，25年时为97.6%，40年时为90.8% （p = 0.0015）。在悉尼/墨尔本治疗的患者比在其他地方治疗的患者表现更好，特别是35岁以后（两者25年生存率为97%；40年生存率为89.9%对81.9%;p = 0.027）。然而，生存并没有因为居住地的偏远而有所不同。结论严重脑室病变，特别是单脑室生理病变的成人仍有较高的过早死亡风险。在悉尼/墨尔本接受治疗的患者生存率更高，这可能反映了长期建立的专科ACHD项目的影响，尽管不能排除残留的混杂因素。城乡结果均等表明，澳大利亚的集中护理模式减轻了地理障碍。

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引用次数: 0