International journal of cardiology. Congenital heart disease最新文献_第3页

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

Long-term outcome after surgical ventricular septal defect closure: Longitudinal follow-up into the fifth decade 室间隔缺损手术后的长期结果：纵向随访至第五个十年

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-10 DOI: 10.1016/j.ijcchd.2025.100624

Sahra Ünlütürk , Robert M. Kauling , Judith A.A.E. Cuypers , Annemien E. van den Bosch , Alexander Hirsch , Chiara Pelosi , Daniel J. Bowen , Raluca G. Chelu , Ad J.J.C. Bogers , Willem A. Helbing , Isabella Kardys , Jolien W. Roos-Hesselink

Objectives

To evaluate survival, clinical outcome and quality of life (QoL) of patients up to 49 years after surgical ventricular septal defect (VSD) closure.

Methods

Single-center, longitudinal cohort study evaluating consecutive patients with surgical VSD closure between 1968 and 1980 with extensive cardiac and QoL evaluation every decade.

Results

Of the original cohort of 174 patients, 39 died (22 %), 8 were lost to follow-up and 38 had not participated previously. Survival rate at 49 years follow-up was 77 % and 86 % when excluding early postoperative mortality. Of the 89 eligible survivors, 76 (85 %) were evaluated (59 % male, median age 49 [44–54] years) with a median follow-up of 44 (range 40–49) years after surgery. Event-free survival at 49 years was 50 %, with symptomatic arrhythmias (10 %), pacemaker implantation (8 %) and VSD-related interventions (3 %) being common complications. At last follow-up, 58 % had left atrial dilation, 25 % had aortic regurgitation and 5 patients (7 %) had a residual VSD. Early postoperative arrhythmias predicted mortality. Both left ventricular (LV) and right ventricular ejection fraction remained stable, with only 1 % having an LV ejection fraction below 45 % at last follow-up. Exercise capacity and VO₂max were mildly reduced in 33 % and 49 % of the patients while self-perceived QoL was stable and comparable with the general Dutch population.

Conclusion

Half of the patients with surgical VSD closure had an event-free survival at 49 years. Pacemaker implantation was often needed. Early postoperative arrhythmias predicted mortality. QoL was good and remained stable over time.

目的评价室间隔缺损（VSD）术后49年的生存率、临床预后和生活质量（QoL）。方法采用单中心、纵向队列研究，对1968 - 1980年间连续行室间隔缺损手术的患者进行评估，每十年进行一次广泛的心脏和生活质量评估。结果在最初的174例患者队列中，39例死亡（22%），8例失去随访，38例以前没有参加过。排除术后早期死亡率后，49年随访的生存率分别为77%和86%。在89例符合条件的幸存者中，76例（85%）接受了评估（59%为男性，中位年龄49[44 - 54]岁），术后中位随访44年（40-49年）。49年无事件生存率为50%，常见并发症有症状性心律失常（10%）、起搏器植入（8%）和vsd相关干预（3%）。最后随访，58%的患者左心房扩张，25%的患者主动脉瓣反流，5例（7%）患者残留室间隔缺损。术后早期心律失常可预测死亡率。左室（LV）和右室射血分数保持稳定，只有1%的左室射血分数在最后随访时低于45%。33%和49%的患者的运动能力和最大摄氧量轻度下降，而自我感知的生活质量稳定，与荷兰一般人群相当。结论一半的VSD手术患者在49年无事件生存。起搏器植入是经常需要的。术后早期心律失常可预测死亡率。生活质量很好，并且随着时间的推移保持稳定。

{"title":"Long-term outcome after surgical ventricular septal defect closure: Longitudinal follow-up into the fifth decade","authors":"Sahra Ünlütürk , Robert M. Kauling , Judith A.A.E. Cuypers , Annemien E. van den Bosch , Alexander Hirsch , Chiara Pelosi , Daniel J. Bowen , Raluca G. Chelu , Ad J.J.C. Bogers , Willem A. Helbing , Isabella Kardys , Jolien W. Roos-Hesselink","doi":"10.1016/j.ijcchd.2025.100624","DOIUrl":"10.1016/j.ijcchd.2025.100624","url":null,"abstract":"<div><h3>Objectives</h3><div>To evaluate survival, clinical outcome and quality of life (QoL) of patients up to 49 years after surgical ventricular septal defect (VSD) closure.</div></div><div><h3>Methods</h3><div>Single-center, longitudinal cohort study evaluating consecutive patients with surgical VSD closure between 1968 and 1980 with extensive cardiac and QoL evaluation every decade.</div></div><div><h3>Results</h3><div>Of the original cohort of 174 patients, 39 died (22 %), 8 were lost to follow-up and 38 had not participated previously. Survival rate at 49 years follow-up was 77 % and 86 % when excluding early postoperative mortality. Of the 89 eligible survivors, 76 (85 %) were evaluated (59 % male, median age 49 [44–54] years) with a median follow-up of 44 (range 40–49) years after surgery. Event-free survival at 49 years was 50 %, with symptomatic arrhythmias (10 %), pacemaker implantation (8 %) and VSD-related interventions (3 %) being common complications. At last follow-up, 58 % had left atrial dilation, 25 % had aortic regurgitation and 5 patients (7 %) had a residual VSD. Early postoperative arrhythmias predicted mortality. Both left ventricular (LV) and right ventricular ejection fraction remained stable, with only 1 % having an LV ejection fraction below 45 % at last follow-up. Exercise capacity and VO<sub>2</sub>max were mildly reduced in 33 % and 49 % of the patients while self-perceived QoL was stable and comparable with the general Dutch population.</div></div><div><h3>Conclusion</h3><div>Half of the patients with surgical VSD closure had an event-free survival at 49 years. Pacemaker implantation was often needed. Early postoperative arrhythmias predicted mortality. QoL was good and remained stable over time.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100624"},"PeriodicalIF":1.2,"publicationDate":"2025-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145361636","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

The Congenital Cardiac Diagnosis Translator (CCDT): Enhancing interoperability in congenital cardiac diagnosis terminology 先天性心脏诊断翻译（CCDT）：增强先天性心脏诊断术语的互操作性

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-09 DOI: 10.1016/j.ijcchd.2025.100628

Sarah Kübler, Ely Erez, Justin Cohen, Peter J. Gruber

Objectives

Congenital heart diseases (CHDs) are classified using diverse terminologies worldwide, creating barriers to effective communication and collaboration in both clinical practice and research. This project aimed to develop a tool to improve interoperability between commonly used CHD classification systems.

Methods and results

The Congenital Cardiac Diagnosis Translator (CCDT) was developed by manually cross-matching diagnostic terms across six coding systems: Fyler, HPO, IPCCC, STS, ICD-10, and ICD-11. Terms were reviewed one system at a time. When an exact match was unavailable, the most specific encompassing diagnosis was selected; in cases where no suitable match existed, no translation was provided. Mappings were consolidated into a curated master lookup table. The CCDT was implemented as a web-based tool with a graphical user interface, enabling term-specific searches. Translations were qualitatively validated by clinical experts in congenital cardiology to ensure accuracy and relevance. The CCDT includes 505 unique congenital heart diagnoses cross-mapped across six coding systems. Qualitative feedback indicated that the translator is intuitive, accurate, and easy to use.

Conclusions

The CCDT provides a scalable, expert-informed solution for improving interoperability across CHD classification systems. By standardizing diagnostic terminology, it supports global collaboration and data sharing in congenital cardiology. The translator is freely accessible and will continue to evolve alongside diagnostic systems.

目的先天性心脏病（CHDs）在世界范围内使用不同的术语进行分类，这给临床实践和研究中的有效沟通和合作造成了障碍。该项目旨在开发一种工具来提高常用CHD分类系统之间的互操作性。方法与结果先天性心脏诊断翻译器（CCDT）通过人工交叉匹配6个编码系统的诊断术语：Fyler、HPO、IPCCC、STS、ICD-10和ICD-11。术语是一次一个系统地审查。当无法获得完全匹配时，选择最具体的综合诊断；在没有适当匹配的情况下，不提供翻译。映射被合并到一个策划的主查找表中。CCDT是作为基于web的工具实现的，具有图形用户界面，支持特定术语的搜索。翻译由先天性心脏病学临床专家进行定性验证，以确保准确性和相关性。CCDT包括505个独特的先天性心脏病诊断，跨六个编码系统交叉映射。定性反馈表明，翻译直观，准确，易于使用。CCDT为提高冠心病分类系统的互操作性提供了一个可扩展的、专家知情的解决方案。通过标准化诊断术语，它支持先天性心脏病学的全球合作和数据共享。翻译器可以免费使用，并将与诊断系统一起继续发展。

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引用次数: 0

Clinical course of severe congenital aortic valve stenosis in children 儿童重度先天性主动脉瓣狭窄的临床过程

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-09 DOI: 10.1016/j.ijcchd.2025.100626

Maximiliaan L. Notenboom , Sencer Albayrak , Kevin M. Veen , Ingrid van Beynum , Rebecca Swens , Jolien W. Roos-Hesselink , Pieter C. van de Woestijne , Jonathan R.G. Etnel , Willem A. Helbing , Johanna J.M. Takkenberg , Ad J.J.C. Bogers

Background

Congenital valvular aortic stenosis (VAS) in children requires lifelong follow-up. An overview of age-specific treatment pathways from first diagnosis of severe VAS is lacking.

Objectives

To assess patient journeys and outcomes after severe pediatric VAS diagnosis, by describing its clinical course and treatment trajectories based on a 37-year single-center experience.

Methods

Baseline and time-related data of children diagnosed with severe congenital VAS between 1985 and 2022 were retrospectively collected. Time-related death (Kaplan-Meier estimator) and intervention occurrence (Aalen-Johansen estimator) were analyzed.

Results

245 children (73.1 % male, median age: 1.2 years (IQR:0.1–7.0)) were diagnosed with severe VAS (53 aged <30 days, 74 between 30d-1y, 84 between 1y-12y, 34 between 12y-18y). Median survival follow-up was 23.3 years (IQR:10.3–31.2) (99.0 % complete). Thirty-five-year incidence of death after diagnosis was 16.2 %(95 %CI:9.7–22.2 %). Of 245 patients, 211 patients (86.1 %) underwent an intervention and 34 (13.9 %) did not undergo an intervention. Of these 34 children, 7 children showed time-related Doppler gradient regression to mild-or-moderate VAS and 17 had stable severe VAS. These 24 children experienced a 6.0 % incidence of death at 30-years after diagnosis. The most common intervention over time (47.5 %) was balloon valvuloplasty, especially in neonates and infants, followed by aortic valve replacement (37.5 %), especially in older children.

Conclusions

This study highlights the vast heterogeneity of treatment pathways and outcomes in children diagnosed with severe VAS at different ages. The observed stabilization of severe VAS or regression of the serial peak Doppler gradient to mild-or-moderate VAS without symptoms in 24 children highlights the need for better insight into determinants of disease course.

儿童先天性瓣膜性主动脉瓣狭窄（VAS）需要终身随访。缺乏从首次诊断严重VAS开始的年龄特异性治疗途径的概述。目的根据37年的单中心经验，通过描述其临床过程和治疗轨迹，评估重症儿童VAS诊断后的患者历程和结果。方法回顾性收集1985 ~ 2022年诊断为重度先天性VAS患儿的基线及时间相关资料。分析时间相关死亡（Kaplan-Meier估计）和干预发生（aallen - johansen估计）。结果245例患儿（男性占73.1%，中位年龄1.2岁（IQR:0.1 ~ 7.0））被诊断为重度VAS（30天53例，30 ~ 1y 74例，1 ~ 12y 84例，12 ~ 18y 34例）。中位生存随访为23.3年（IQR: 10.3-31.2）（99.0%完成率）。确诊后35年的死亡率为16.2% （95% CI:9.7 - 22.2%）。在245例患者中，211例（86.1%）患者接受了干预，34例（13.9%）患者未接受干预。在这34名儿童中，7名儿童出现与时间相关的多普勒梯度回归到轻度或中度VAS， 17名儿童有稳定的重度VAS。这24名儿童在诊断后30年的死亡率为6.0%。随着时间的推移，最常见的干预措施（47.5%）是球囊瓣膜成形术，特别是在新生儿和婴儿中，其次是主动脉瓣置换术（37.5%），特别是在年龄较大的儿童中。结论本研究强调了不同年龄诊断为严重VAS的儿童的治疗途径和结果的巨大异质性。在24名儿童中观察到的严重VAS稳定或多普勒梯度序列峰值回归到无症状的轻度或中度VAS突出了更好地了解疾病进程决定因素的必要性。

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引用次数: 0

The rudimentary left ventricle does not impact on right ventricular size and function in hypoplastic left heart syndrome during serial follow up after Fontan completion 在Fontan完成后的连续随访中，未发育的左心室对左心发育不全综合征患者的右心室大小和功能没有影响

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-09 DOI: 10.1016/j.ijcchd.2025.100627

Abigail Burleigh , Dominik Daniel Gabbert , Yujiro Ide , Anselm Uebing , Inga Voges

Background

Previous studies in patients with hypoplastic left heart syndrome (HLHS) suggested that a larger left ventricle (LV) might negatively impact right ventricular (RV) function. This study aimed to analyse the impact of the presence of an LV remnant on RV size and function during serial follow up.

Methods

Serial cardiovascular magnetic resonance (CMR) examinations after completion of the total cavopulmonary connection were retrospectively analysed. Patients were divided into those with and those without a rudimentary LV. RV and LV end diastolic and end systolic volumes as well as stroke volume, ejection fraction (RVEF, LVEF) and end diastolic mass were measured.

Results

90 HLHS patients (female: 26) who had at least two CMR examinations were included. 51 patients had three examinations. 56 patients had an LV remnant, 34 did not. RV volumes and mass indexed to body surface area as well as RVEF did not differ significantly between both groups. LV volumes showed no association with RV volumes and RVEF.

Conclusion

Analysis of serial CMR examination suggests that the presence of an LV remnant does not have a major impact on RV size and function during longer-term follow-up. Future studies might focus on regional RV function.

背景先前对左心发育不全综合征（HLHS）患者的研究表明，较大的左心室（LV）可能会对右心室（RV）功能产生负面影响。本研究旨在分析左室残余的存在对左室大小和功能的影响。方法回顾性分析全腔肺连接完成后的连续心血管磁共振（CMR）检查结果。患者被分为有和没有初级左室的两组。测量左室和左室舒张末期和收缩末期容积、卒中容积、射血分数（RVEF、LVEF）和舒张末期质量。结果入选至少2次CMR检查的HLHS患者90例（女26例）。51例患者进行了三次检查。56例患者有左室残余，34例没有。RV体积和质量与体表面积指数以及RVEF在两组之间无显著差异。左室容积与右室容积和RVEF无相关性。结论连续CMR检查分析表明，在长期随访中，左室残余的存在对左室大小和功能没有重大影响。未来的研究可能会集中在区域RV功能上。

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引用次数: 0

Right ventricular pressure-volume loop analysis in congenital heart disease 先天性心脏病右心室压力-容积环分析

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-02 DOI: 10.1016/j.ijcchd.2025.100625

Renée S. Joosen , Michael G. Dickinson , Marielle C. van de Veerdonk , Rahi S. Alipour Symakani , Daphne Merkus , Michiel Voskuil , Gregor J. Krings , Johannes M.P.J. Breur

Background

Right ventricular (RV) function is an independent predictor of prognosis in patients with congenital heart disease (CHD). Early detection is therefore crucial but current methods to assess RV function are highly load-dependent. RV pressure-volume (PV) loop analysis is an invasive technique which is load-independent and considered the gold standard for evaluating RV adaptation in relation to its load. However, RV PV loop analysis can be time consuming and prone to inter-operator variability.

Methods

This article provides a comprehensive guide on performing RV PV loop analysis using the multi-beat method in pediatric and adult patients with complex CHD.

Results

RV PV loop analysis using the multi-beat method can be safely and reliably applied in children and adults with complex CHD.

Conclusion

RV PV loop analysis offers a valuable tool for advanced insight into RV adaptation and a deeper understanding of cardiac physiology in complex CHD patients with RV pressure and/or volume overload. By enhancing our understanding of RV adaptation, this approach holds great potential for improving patient management and optimizing treatment strategies.

背景：右心室（RV）功能是先天性心脏病（CHD）患者预后的独立预测因子。因此，早期检测至关重要，但目前评估右心室功能的方法高度依赖于负荷。RV压力-体积（PV）环分析是一种与负荷无关的侵入性技术，被认为是评估RV与负荷相关的适应性的金标准。然而，RV PV回路分析可能是耗时的，并且容易受到操作者之间的变化。方法采用多拍法对儿童和成人复杂冠心病患者进行RV - PV环路分析。结果采用多拍法进行rv PV环路分析可安全可靠地应用于儿童和成人复杂冠心病患者。结论右心室PV环分析为深入了解右心室适应和更深入地了解右心室压力和/或容量过载的复杂冠心病患者的心脏生理提供了有价值的工具。通过增强我们对RV适应的理解，这种方法在改善患者管理和优化治疗策略方面具有巨大的潜力。

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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 : 2025-09-28 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