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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 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。
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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 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 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 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 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。
{"title":"Right ventricular pressure-volume loop analysis in congenital heart disease","authors":"Renée S. Joosen ,&nbsp;Michael G. Dickinson ,&nbsp;Marielle C. van de Veerdonk ,&nbsp;Rahi S. Alipour Symakani ,&nbsp;Daphne Merkus ,&nbsp;Michiel Voskuil ,&nbsp;Gregor J. Krings ,&nbsp;Johannes M.P.J. Breur","doi":"10.1016/j.ijcchd.2025.100625","DOIUrl":"10.1016/j.ijcchd.2025.100625","url":null,"abstract":"<div><h3>Background</h3><div>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.</div></div><div><h3>Methods</h3><div>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.</div></div><div><h3>Results</h3><div>RV PV loop analysis using the multi-beat method can be safely and reliably applied in children and adults with complex CHD.</div></div><div><h3>Conclusion</h3><div>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.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100625"},"PeriodicalIF":1.2,"publicationDate":"2025-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145361709","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 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
The role of artificial intelligence and mobile health in diagnosis and management of pulmonary arterial hypertension 人工智能和移动医疗在肺动脉高压诊断和治疗中的作用
IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-09-23 DOI: 10.1016/j.ijcchd.2025.100622
Maria Luisa Benesch Vidal , Alexandra Arvanitaki , Gerhard-Paul Diller
Pulmonary arterial hypertension (PAH) is a rare, progressive disorder characterized by pulmonary vascular remodeling, increased pulmonary vascular resistance, and ultimately right ventricular failure. Despite therapeutic advances, delayed diagnosis and imprecise risk stratification remain key challenges. Artificial intelligence (AI) and machine learning (ML) offer opportunities across the care continuum. This includes early detection from electronic health records, electrocardiography and imaging, automated, standardized interpretation of echocardiography, computed tomography (CT), and cardiac magnetic resonance (CMR) potentially expediting referral and final diagnosis. Deep learning applied to echocardiography achieves expert-level PAH classification and provides automated right-heart quantification while CT/CMR-based models segment the heart and great vessels, quantify lung disease radiomics, and infer hemodynamics, supporting noninvasive triage and prognostication. In future, remote monitoring with wearables and telemedicine, coupled with AI analytics, promises to enable proactive management and potentially reduce hospitalizations. While early studies are promising, translation to practice requires rigorous external validation, prospective studies, bias auditing and seamless integration into guideline-aligned workflows. Overall, AI stands to augment expert clinical judgement by converting high-dimensional, multimodal data into actionable insights. With careful governance and evidence generation, AI has the potential to shorten time-to-diagnosis, refine risk stratification, and personalize therapy in PAH, ultimately improving outcomes for this high-risk population.
肺动脉高压(PAH)是一种罕见的进行性疾病,其特征是肺血管重构,肺血管阻力增加,最终导致右心室衰竭。尽管治疗取得了进步,但延迟诊断和不精确的风险分层仍然是主要挑战。人工智能(AI)和机器学习(ML)为整个护理连续体提供了机会。这包括从电子健康记录、心电图和成像、超声心动图的自动化、标准化解释、计算机断层扫描(CT)和心脏磁共振(CMR)中进行早期检测,从而可能加快转诊和最终诊断。应用于超声心动图的深度学习实现了专家级别的多环芳烃分类,并提供了自动的右心量化,而基于CT/ cmr的模型可以分割心脏和大血管,量化肺部疾病放射组学,并推断血流动力学,支持无创分诊和预测。未来,通过可穿戴设备和远程医疗进行远程监控,再加上人工智能分析,有望实现主动管理,并可能减少住院治疗。虽然早期的研究很有希望,但将其转化为实践需要严格的外部验证、前瞻性研究、偏见审计以及与指导方针一致的工作流程的无缝集成。总的来说,人工智能通过将高维、多模式的数据转化为可操作的见解来增强专家的临床判断。通过谨慎的管理和证据生成,人工智能有可能缩短PAH的诊断时间,完善风险分层,个性化治疗,最终改善这一高危人群的预后。
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引用次数: 0
Role of balloon pulmonary valvuloplasty in symptomatic infants with tetralogy of Fallot awaiting intracardiac repair 球囊肺动脉瓣成形术在等待心内修复的有症状的法洛四联症婴儿中的作用
IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-09-18 DOI: 10.1016/j.ijcchd.2025.100621
Sanjeev Hanumantacharya Naganur, Abhishek Mallick, Manoj Kumar Rohit, Parag Barwad, Vinothkumar S.P.

Background and aim

Mortality risk in tetralogy of Fallot (TOF) is highest in infancy, predominantly due to hypercyanotic spells. Timing of corrective surgery is centre/expertise dependent and surgical shunt procedures carry high mortality risk. We aimed to study the efficacy and feasibility of balloon pulmonary valvuloplasty (BPV) in TOF physiology children, as an alternative to surgical shunt, in those infants who were severely cyanosed (saturation <75 %) or having recurrent spells and who were still awaiting intracardiac repair (ICR). The primary objective was to assess the following parameters pre and post BPV: resting saturation, Z score of pulmonary annuli by 2D Echocardiography, right ventricular outflow tract (RVOT) gradient, hypercyanotic spell frequency. Secondarily we looked at the modified Nakata index (MNI), any procedural complications, requirement of early shunt or ICR (within study period).

Materials and methods

This was a single arm interventional study done over a period of one year. Values pre and post intervention were compared using Wilcoxon matched pair signed rank test and Chi-square test for categorical variables as appropriate. Independent groups were compared using Mann Whitney U test. The children were followed up for a period of 6 months post procedure.

Results

After enrolling 10 infants, data could be studied in 9 of them. 1 child was lost to follow up. Significant improvement was noted in saturation, size of PA annulus, RVOT gradient, MNI (p value < 0.05 each). 2 patients had access site thrombosis which resolved with heparin. 1 had transient complete heart block which resolved within 24 h but required temporary pacing. There was no mortality and none had more than mild pulmonary regurgitation. Out of 5 children who presented with recurrent spells, 2 were completely free of spells after BPV, 2 had spells with reduced frequency/duration, 1 continued to have spells needing surgical intervention. (p = 0.36). Apart from this child who needed surgery, one other patient remained severely cyanotic and had to undergo a shunt procedure.

Conclusion

To conclude, BPV seems like a safe and cost-effective alternative to high-risk surgical shunt palliation in symptomatic TOF infants, in resource constrained regions.
背景和目的法洛四联症(TOF)的死亡风险在婴儿期最高,主要是由于高紫绀期。矫正手术的时机取决于中心/专业知识,外科分流手术具有高死亡率风险。我们的目的是研究球囊肺瓣膜成形术(BPV)在TOF生理儿童中作为手术分流的替代方法的有效性和可行性,用于那些严重氰化(饱和度<; 75%)或反复发作且仍在等待心内修复(ICR)的婴儿。主要目的是评估BPV前后的以下参数:静息饱和度,二维超声心动图肺环Z评分,右心室流出道(RVOT)梯度,高紫绀型发作频率。其次,我们观察了改良的Nakata指数(MNI),任何程序性并发症,早期分流或ICR的要求(在研究期间)。材料和方法这是一项为期一年的单组干预性研究。干预前后的数值比较酌情采用Wilcoxon配对对有符号秩检验和卡方检验。独立组间比较采用Mann Whitney U检验。术后随访6个月。结果纳入10例婴儿,其中9例可进行数据研究。失访1例。饱和度、PA环大小、RVOT梯度、MNI均有显著改善(p值均为0.05)。2例患者出现通路血栓形成,经肝素治疗后痊愈。1例有短暂性完全性心脏传导阻滞,24小时内消退,但需要临时起搏。无死亡,无轻度肺反流。在5例反复发作的儿童中,2例在BPV后完全没有发作,2例发作频率/持续时间减少,1例仍然需要手术干预。(p = 0.36)。除了这个需要手术的孩子,另一个病人仍然严重紫绀,不得不接受分流手术。总之,在资源有限的地区,BPV似乎是有症状的TOF婴儿的一种安全且具有成本效益的替代高风险手术分流姑息。
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引用次数: 0
Clinical implications of progressive systemic ventricular dysfunction in adults with Fontan palliation 渐进性全身性脑室功能障碍在成人Fontan姑息治疗中的临床意义
IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-09-13 DOI: 10.1016/j.ijcchd.2025.100620
Meena Bai, Ahmed Bahnasy, Sara Aboelmaaty, Mohamed Ellabbad, Ahmed Ali, Alexander C. Egbe

Background

The purpose of this study was to determine the clinical implications of progressive systemic ventricular (SV) systolic dysfunction in adults with Fontan palliation.

Methods

Retrospective study of Fontan patients with ≥2 echocardiograms at Mayo Clinic. SV systolic function was assessed using echo-derived ejection fraction (Echo_EF) at baseline, and annually for 3 years. Temporal decline in SV systolic function was estimated as relative change (relative Δ_Echo_EF)/year. Exploratory analysis was performed to assess the effect of guideline-directed medical therapy (GDMT) on Echo_EF.

Results

Of 414 patients (age 27 ± 9 years; males 228 [55 %]), 287 (69 %) and 127 (31 %) had dominant morphologic left ventricle (LV) and right ventricle (RV), respectively. Assessment of Echo_EF was feasible in 1464 of 1603 echocardiograms (91 %). The baseline Echo_EF was 54 % (48–58), and the relative Δ_Echo_EF was −3.9 % (95 %CI -6.3 to −2.5 %)/year. The predictors of progressive SV systolic dysfunction were older age, morphologic RV, ≥moderate atrioventricular valve regurgitation, cardiac implantable electronic devices, and atrial fibrillation. Progressive SV systolic dysfunction was associated with an approximately 2-fold increase in death/transplant (hazard ratio 1.92, p = 0.009), independent of baseline Echo_EF and comorbidities. GDMT was associated with improvement in Echo_EF in patients with morphologic LV.

Conclusions

The current study underscores the importance of longitudinal echocardiographic monitoring of SV systolic function, and the potential clinical benefits of GDMT in patients with morphologic LV. Further studies are required to determine whether interventions such as valve surgery, rhythm control strategy, and physiologic pacing would prevent or reverse SV systolic dysfunction.
本研究的目的是确定成人Fontan姑息治疗中进行性系统性心室(SV)收缩功能障碍的临床意义。方法对梅奥诊所超声心动图≥2张的Fontan患者进行回顾性研究。在基线时使用回声衍生射血分数(Echo_EF)评估SV收缩功能,并在3年内每年评估一次。SV收缩功能的时间下降估计为相对变化(相对Δ_Echo_EF)/年。采用探索性分析方法评价指南导向药物治疗(GDMT)对Echo_EF的影响。结果414例患者(年龄27±9岁,男性228例[55%]),287例(69%)和127例(31%)分别表现为左心室(LV)和右心室(RV)优势形态。1603张超声心动图中有1464张(91%)可评价Echo_EF。基线Echo_EF为54%(48-58),相对Δ_Echo_EF为- 3.9% (95% CI -6.3 - 2.5%)/年。进行性SV收缩功能障碍的预测因子为年龄较大、形态RV、≥中度房室瓣膜返流、心脏植入式电子装置和心房颤动。进行性SV收缩功能障碍与死亡/移植增加约2倍相关(危险比1.92,p = 0.009),与基线Echo_EF和合并症无关。GDMT与形态学左室患者Echo_EF的改善相关。结论当前的研究强调了纵向超声心动图监测SV收缩功能的重要性,以及GDMT在形态学左室患者中的潜在临床益处。需要进一步的研究来确定诸如瓣膜手术、节律控制策略和生理性起搏等干预措施是否能预防或逆转SV收缩功能障碍。
{"title":"Clinical implications of progressive systemic ventricular dysfunction in adults with Fontan palliation","authors":"Meena Bai,&nbsp;Ahmed Bahnasy,&nbsp;Sara Aboelmaaty,&nbsp;Mohamed Ellabbad,&nbsp;Ahmed Ali,&nbsp;Alexander C. Egbe","doi":"10.1016/j.ijcchd.2025.100620","DOIUrl":"10.1016/j.ijcchd.2025.100620","url":null,"abstract":"<div><h3>Background</h3><div>The purpose of this study was to determine the clinical implications of progressive systemic ventricular (SV) systolic dysfunction in adults with Fontan palliation.</div></div><div><h3>Methods</h3><div>Retrospective study of Fontan patients with ≥2 echocardiograms at Mayo Clinic. SV systolic function was assessed using echo-derived ejection fraction (Echo_EF) at baseline, and annually for 3 years. Temporal decline in SV systolic function was estimated as relative change (relative Δ_Echo_EF)/year. Exploratory analysis was performed to assess the effect of guideline-directed medical therapy (GDMT) on Echo_EF.</div></div><div><h3>Results</h3><div>Of 414 patients (age 27 ± 9 years; males 228 [55 %]), 287 (69 %) and 127 (31 %) had dominant morphologic left ventricle (LV) and right ventricle (RV), respectively. Assessment of Echo_EF was feasible in 1464 of 1603 echocardiograms (91 %). The baseline Echo_EF was 54 % (48–58), and the relative Δ_Echo_EF was −3.9 % (95 %CI -6.3 to −2.5 %)/year. The predictors of progressive SV systolic dysfunction were older age, morphologic RV, ≥moderate atrioventricular valve regurgitation, cardiac implantable electronic devices, and atrial fibrillation. Progressive SV systolic dysfunction was associated with an approximately 2-fold increase in death/transplant (hazard ratio 1.92, p = 0.009), independent of baseline Echo_EF and comorbidities. GDMT was associated with improvement in Echo_EF in patients with morphologic LV.</div></div><div><h3>Conclusions</h3><div>The current study underscores the importance of longitudinal echocardiographic monitoring of SV systolic function, and the potential clinical benefits of GDMT in patients with morphologic LV. Further studies are required to determine whether interventions such as valve surgery, rhythm control strategy, and physiologic pacing would prevent or reverse SV systolic dysfunction.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100620"},"PeriodicalIF":1.2,"publicationDate":"2025-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145106097","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
Advances in the imaging of pulmonary hypertension 肺动脉高压的影像学研究进展
IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-09-10 DOI: 10.1016/j.ijcchd.2025.100619
Polona Kačar , Katja Prokšelj , Sarah Ghonim , Thomas Semple , Sonya V. Babu-Narayan , Heba Nashat , Stephen J. Wort , Michael A. Gatzoulis , Margarita Brida
Pulmonary hypertension (PH) is a complex and progressive disorder characterized by elevated pulmonary arterial pressures leading to right ventricular dysfunction and increased morbidity and mortality. Non-invasive imaging, including echocardiography, computed tomography (CT) and cardiovascular magnetic resonance (CMR), plays a crucial role in the diagnosis, risk stratification, and management of PH. The integration of these imaging modalities facilitates a multimodal approach to PH evaluation, enabling more precise diagnosis, improved phenotyping, and better-guided therapeutic decision-making. Echocardiography remains the first-line modality, offering valuable insights into pulmonary artery pressures, right ventricular size and function, and associated cardiac anomalies. Recent developments in speckle-tracking echocardiography and 3D imaging have enhanced its diagnostic and prognostic utility. CT imaging provides detailed evaluation of the pulmonary vasculature, parenchyma, and perfusion, which is essential in distinguishing PH subtypes. CMR is non-invasive, radiation free, and highly sensitive to changes in anatomy and function making it ideal for the long-term follow up of patients with PH. It offers in-depth evaluation of all cardiac chambers as well as pulmonary blood flow assessment and tissue characterisation. In this work we discuss current strengths, limitations, and future directions in these key imaging modalities used for the comprehensive assessment of PH.
肺动脉高压(PH)是一种复杂的进行性疾病,其特征是肺动脉压升高导致右心室功能障碍,发病率和死亡率增加。非侵入性成像,包括超声心动图、计算机断层扫描(CT)和心血管磁共振(CMR),在PH的诊断、风险分层和管理中起着至关重要的作用。这些成像模式的整合促进了PH评估的多模式方法,实现了更精确的诊断,改善了表型,并更好地指导了治疗决策。超声心动图仍然是一线方式,提供有价值的见解肺动脉压,右心室大小和功能,以及相关的心脏异常。斑点跟踪超声心动图和3D成像的最新发展增强了其诊断和预后的效用。CT成像提供了肺血管、实质和灌注的详细评估,这是区分PH亚型所必需的。CMR是非侵入性的,无辐射的,对解剖和功能的变化高度敏感,使其成为ph患者长期随访的理想选择。它提供对所有心腔的深入评估,以及肺血流评估和组织特征。在这项工作中,我们讨论了用于PH综合评估的这些关键成像模式的当前优势、局限性和未来方向。
{"title":"Advances in the imaging of pulmonary hypertension","authors":"Polona Kačar ,&nbsp;Katja Prokšelj ,&nbsp;Sarah Ghonim ,&nbsp;Thomas Semple ,&nbsp;Sonya V. Babu-Narayan ,&nbsp;Heba Nashat ,&nbsp;Stephen J. Wort ,&nbsp;Michael A. Gatzoulis ,&nbsp;Margarita Brida","doi":"10.1016/j.ijcchd.2025.100619","DOIUrl":"10.1016/j.ijcchd.2025.100619","url":null,"abstract":"<div><div>Pulmonary hypertension (PH) is a complex and progressive disorder characterized by elevated pulmonary arterial pressures leading to right ventricular dysfunction and increased morbidity and mortality. Non-invasive imaging, including echocardiography, computed tomography (CT) and cardiovascular magnetic resonance (CMR), plays a crucial role in the diagnosis, risk stratification, and management of PH. The integration of these imaging modalities facilitates a multimodal approach to PH evaluation, enabling more precise diagnosis, improved phenotyping, and better-guided therapeutic decision-making. Echocardiography remains the first-line modality, offering valuable insights into pulmonary artery pressures, right ventricular size and function, and associated cardiac anomalies. Recent developments in speckle-tracking echocardiography and 3D imaging have enhanced its diagnostic and prognostic utility. CT imaging provides detailed evaluation of the pulmonary vasculature, parenchyma, and perfusion, which is essential in distinguishing PH subtypes. CMR is non-invasive, radiation free, and highly sensitive to changes in anatomy and function making it ideal for the long-term follow up of patients with PH. It offers in-depth evaluation of all cardiac chambers as well as pulmonary blood flow assessment and tissue characterisation. In this work we discuss current strengths, limitations, and future directions in these key imaging modalities used for the comprehensive assessment of PH.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100619"},"PeriodicalIF":1.2,"publicationDate":"2025-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145361705","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
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International journal of cardiology. Congenital heart disease
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