International journal of cardiology. Congenital heart disease最新文献_第5页

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 : 2025-12-01 Epub 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

Long-term outcome of invasive electrophysiological studies and catheter ablation in patients with patent foramen ovale and atrial septal defect–A single-center retrospective cohort study 有创电生理研究和导管消融治疗卵圆孔未闭房间隔缺损的远期疗效——单中心回顾性队列研究

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-12-01 Epub Date: 2025-08-25 DOI: 10.1016/j.ijcchd.2025.100615

Pieter-Jan Lewandowski , Thomas Rosseel , Peter Haemers , Els Troost , Christophe Garweg , Werner Budts , Joris Ector , Pieter De Meester , Alexander Van De Bruaene , Bert Vandenberk

Introduction

Patients with atrial septal defect (ASD) and patent foramen ovale (PFO) are prone to atrial arrhythmias due to structural and surgical alterations. While radiofrequency catheter ablation (RFCA) is a common treatment, arrhythmia recurrence remains a clinical challenge. This study aimed to assess arrhythmia patterns, procedural outcomes, and long-term recurrence following electrophysiological interventions.

Methods

A retrospective cohort study was performed at a tertiary center including patients with ASD or PFO undergoing invasive electrophysiological procedures. Clinical and procedural data were extracted from medical records. Arrhythmias were classified based on electrocardiographic and electrophysiological findings. Kaplan-Meier analysis was used to assess event-free survival for arrhythmia recurrence.

Results

Among 87 patients (57.5 % female, median age 46.4 years), 82.8 % underwent RFCA as the first intervention. The most common arrhythmias were non-cavo-tricuspid isthmus (CTI) dependent intra-atrial reentrant tachycardia (35.4 %) and atrial fibrillation (18.6 %). Acute procedural success was achieved in 97.2 % of patients. Recurrence-free survival after the first RFCA was 83.6 % at 1 year, 64.2 % at 3 years, and 60.0 % at 5 years. After the last RFCA, recurrence-free survival improved to 88.3 % at 1 year and 74.3 % at 5 years.

Conclusion

In patients with ASD and PFO, RFCA is safe and acutely effective but followed by arrhythmia recurrence in a quarter of patients. The evolving arrhythmic substrate highlights the need for early intervention strategies and emerging technologies to optimize long-term outcomes in this patient population.

房间隔缺损（ASD）和卵圆孔未闭（PFO）患者由于房间隔结构和手术改变，易发生房性心律失常。虽然射频导管消融（RFCA）是一种常见的治疗方法，但心律失常复发仍然是一个临床挑战。本研究旨在评估电生理干预后的心律失常模式、手术结果和长期复发。方法在某三级中心对ASD或PFO患者进行有创电生理手术进行回顾性队列研究。从医疗记录中提取临床和手术资料。根据心电图和电生理结果对心律失常进行分类。Kaplan-Meier分析用于评估心律失常复发的无事件生存率。结果87例患者中（57.5%为女性，中位年龄46.4岁），82.8%的患者接受RFCA作为首次干预。最常见的心律失常是非腔室-三尖瓣峡（CTI）依赖性房内重入性心动过速（35.4%）和心房颤动（18.6%）。97.2%的患者手术成功。第一次RFCA术后1年无复发生存率为83.6%，3年为64.2%，5年为60.0%。在最后一次RFCA后，1年无复发生存率提高到88.3%，5年生存率提高到74.3%。结论在ASD合并PFO患者中，RFCA是安全且急性有效的，但有1 / 4的患者出现心律失常复发。不断发展的心律失常基底强调需要早期干预策略和新兴技术来优化这一患者群体的长期预后。

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引用次数: 0

The PAH patient's perspective PAH患者的观点

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-05-26 DOI: 10.1016/j.ijcchd.2025.100596

Daniel A. Jeffery , Wendy Gin-Sing , Radwa Bedair , Luke S. Howard

Pulmonary Hypertension is a serious condition which can occur in adults living with congenital heart disease. Due to advances in the care of patients with congenital heart disease, patients are living longer with the condition. Unfortunately, patients living with Pulmonary Arterial Hypertension associated with Congenital Heart Disease (PAH-CHD) face a number of different issues directly related to their condition, which range from physical limitations and uncertainty over their diagnosis, which in some cases cause significant depression and anxiety, to impact on their relationships with friends and family, financial challenges and family planning.

In this article, we interview two patients living with PAH-CHD and discuss the numerous issues that patients may face as a direct result of their condition.

肺动脉高压是一种严重的疾病，可能发生在患有先天性心脏病的成年人身上。由于对先天性心脏病患者护理的进步，患有这种疾病的患者寿命更长。不幸的是，与先天性心脏病（PAH-CHD）相关的肺动脉高压患者面临着许多与其病情直接相关的不同问题，包括身体限制和诊断的不确定性，在某些情况下会导致严重的抑郁和焦虑，影响他们与朋友和家人的关系，财务挑战和计划生育。在这篇文章中，我们采访了两名患有PAH-CHD的患者，并讨论了患者可能面临的许多问题，这些问题是他们的病情的直接结果。

引用次数: 0

Evaluation of prosthetic dysfunction in the diagnosis of endocarditis associated to prosthetic pulmonary valve and pulmonary conduit 假体功能障碍在假体肺瓣膜及肺导管相关心内膜炎诊断中的价值评价

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-05-13 DOI: 10.1016/j.ijcchd.2025.100591

Andrés Cano Pérez , Larraitz Orive Melero , Jose Félix Larrea Egurbide , Jagoba Larrazábal López , Luis Fernández González , Roberto Blanco Mata , Josune Arriola Meabe , Leire Artiñano Mendizábal , Ane Josune Goikoetxea Agirre , María José Blanco Vidal , Javier Ayala Curiel , Pedro María Montes Orbe

Introduction

The number of cases of infective endocarditis associated to prosthetic pulmonary valves and pulmonary conduits (PPVIE) is likely to increase in the future. Transthoracic echocardiography (TTE) presents challenges in visualizing lesions suggestive of endocarditis in these patients. However, TTE may provide additional findings, such as prosthetic dysfunction, which can guide the diagnosis of this condition. The main objective of this study is to analyze prosthetic pulmonary valve dysfunction as an echocardiographic manifestation of PPVIE.

Methods

All cases of PPVIE (definite and possible, according to the modified Duke criteria) at Cruces University Hospital (Baracaldo, Spain) between January 2014 and July 2024 were included. Prosthetic dysfunction was defined as a peak transvalvular gradient (PTG) ≥25 mmHg for homografts and ≥40 mmHg for prosthetic pulmonary valves and bovine pulmonary conduits (stenosis) and/or pulmonary regurgitation (PR) moderate or severe. Number of cases of prosthetic dysfunction between the PPVIE episode and prior to the episode were compared. We analyzed the mechanisms of prosthetic dysfunction in the PPVIE episode.

Results

14 cases of PPVIE were identified. In cases prior to the PPVIE episode, 42.9 % had prosthetic dysfunction, while in the PPVIE episode, 92.3 % had prosthetic dysfunction. Stenosis was a more frequent cause of valve dysfunction than PR.

Conclusions

Prosthetic dysfunction (due to stenosis or regurgitation) is a relevant finding in the diagnosis of PPVIE in cases where lesions suggestive of endocarditis are not visualised. Although not included in the Duke criteria, stenosis is a more frequent mechanism of dysfunction than PR.

感染性心内膜炎与假肺瓣膜和肺导管（PPVIE）相关的病例数在未来可能会增加。在这些患者中，经胸超声心动图（TTE）在显示提示心内膜炎的病变方面提出了挑战。然而，TTE可能提供额外的发现，如假体功能障碍，这可以指导这种情况的诊断。本研究的主要目的是分析人工肺瓣膜功能障碍作为PPVIE的超声心动图表现。方法收集2014年1月至2024年7月在西班牙克鲁塞大学医院（Baracaldo, Spain）就诊的所有PPVIE病例（确诊和可能符合Duke标准）。假体功能障碍定义为同种移植物的峰值经瓣梯度(PTG)≥25 mmHg，假体肺瓣膜和牛肺导管（狭窄）和/或肺反流(PR)≥40 mmHg，中度或重度。比较PPVIE发作前后假体功能障碍的病例数。我们分析了PPVIE发作中假体功能障碍的机制。结果共鉴定出PPVIE 14例。在PPVIE发作之前的病例中，42.9%的患者有假体功能障碍，而在PPVIE发作期间，92.3%的患者有假体功能障碍。瓣膜狭窄是瓣膜功能障碍更常见的原因。结论在未见心内膜炎病变的情况下，假体功能障碍（由狭窄或反流引起）是诊断PPVIE的一个相关发现。虽然没有包括在Duke标准中，但狭窄是比PR更常见的功能障碍机制。

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引用次数: 0

A detailed profiling study from the national Australian Congenital Heart Disease registry: rationale and design 来自澳大利亚国家先天性心脏病登记处的详细分析研究：基本原理和设计

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-05-30 DOI: 10.1016/j.ijcchd.2025.100595

Tanya Badal , Calum Nicholson , Julian Ayer , Michael Cheung , Leeanne Grigg , David S. Celermajer , Geoff Strange

Background

Previous research has outlined significant gaps in the care of children and adults with Congenital Heart Disease (CHD). The Australian Study of the Burden and Outcomes of CHD will assess medical, psychological, social and economic issues affecting CHD patients and carers and provide a detailed account of CHD patients’ healthcare utilisation.

Methods

A random sample of patients aged ≥5 years, equally split between genders, CHD complexity (mild, moderate, severe) and rural and remote locations (20–25%) was extracted from the National Australian CHD Registry (n = 68,214) from four large participating CHD centres (2 adult and 2 child). Patients and their carers were invited to participate in an in-person study visit for physical assessment and for a series of surveys questioning physical and mental health, neurodevelopment, and quality of life.

Results

Of 6,726 contacted patients, 1,911 (28%) consented, with 99.5% of surveys completed online. 1,658 consented participants completed at least one study activity (87% response rate). Proxies/carers completed surveys for 11% of participants (primarily those under age 8 or with intellectual disability). All study activities were completed by 750 respondents (45%). Respondents were older (median age 24 [IQR 12–41 years] vs 18 [IQR 11–35 years], p = 0.005) and had a higher proportion of severe CHD (28%) compared to non-respondents (23%). No significant differences in disease complexity were observed between respondents and non-respondents.

Conclusion

This multi-site study of over 1600 CHD patients aims to offer the first comprehensive profiling of the burdens and outcomes faced by Australia's growing CHD population.

背景先前的研究概述了先天性心脏病（CHD）儿童和成人护理方面的重大差距。澳大利亚冠心病负担和结果研究将评估影响冠心病患者和护理人员的医疗、心理、社会和经济问题，并提供冠心病患者医疗保健利用的详细说明。方法随机抽取年龄≥5岁、性别、冠心病复杂程度（轻度、中度、重度）、农村和偏远地区（20-25%）的患者样本，这些患者来自澳大利亚国家冠心病登记处（n = 68214） 4个大型参与冠心病中心（2个成人和2个儿童）。患者和他们的护理人员被邀请参加一次面对面的研究访问，以进行身体评估，并进行一系列关于身心健康、神经发育和生活质量的调查。结果在6726名接触的患者中，1911名（28%）同意，其中99.5%的调查是在线完成的。1,658名同意的参与者完成了至少一项研究活动（87%的响应率）。代理/护理人员为11%的参与者（主要是8岁以下或有智力残疾的参与者）完成了调查。所有研究活动由750名受访者（45%）完成。受访者年龄较大（中位年龄24岁[IQR 12-41岁]对18岁[IQR 11-35岁]，p = 0.005），严重冠心病的比例（28%）高于非受访者（23%）。调查对象和非调查对象在疾病复杂性方面没有显著差异。这项涉及1600多名冠心病患者的多中心研究旨在首次全面分析澳大利亚不断增长的冠心病患者所面临的负担和结果。

{"title":"A detailed profiling study from the national Australian Congenital Heart Disease registry: rationale and design","authors":"Tanya Badal , Calum Nicholson , Julian Ayer , Michael Cheung , Leeanne Grigg , David S. Celermajer , Geoff Strange","doi":"10.1016/j.ijcchd.2025.100595","DOIUrl":"10.1016/j.ijcchd.2025.100595","url":null,"abstract":"<div><h3>Background</h3><div>Previous research has outlined significant gaps in the care of children and adults with Congenital Heart Disease (CHD). The Australian Study of the Burden and Outcomes of CHD will assess medical, psychological, social and economic issues affecting CHD patients and carers and provide a detailed account of CHD patients’ healthcare utilisation.</div></div><div><h3>Methods</h3><div>A random sample of patients aged ≥5 years, equally split between genders, CHD complexity (mild, moderate, severe) and rural and remote locations (20–25%) was extracted from the National Australian CHD Registry (n = 68,214) from four large participating CHD centres (2 adult and 2 child). Patients and their carers were invited to participate in an in-person study visit for physical assessment and for a series of surveys questioning physical and mental health, neurodevelopment, and quality of life.</div></div><div><h3>Results</h3><div>Of 6,726 contacted patients, 1,911 (28%) consented, with 99.5% of surveys completed online. 1,658 consented participants completed at least one study activity (87% response rate). Proxies/carers completed surveys for 11% of participants (primarily those under age 8 or with intellectual disability). All study activities were completed by 750 respondents (45%). Respondents were older (median age 24 [IQR 12–41 years] vs 18 [IQR 11–35 years], p = 0.005) and had a higher proportion of severe CHD (28%) compared to non-respondents (23%). No significant differences in disease complexity were observed between respondents and non-respondents.</div></div><div><h3>Conclusion</h3><div>This multi-site study of over 1600 CHD patients aims to offer the first comprehensive profiling of the burdens and outcomes faced by Australia's growing CHD population.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100595"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144330248","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

Echocardiographic effects of sodium-glucose cotransporter 2 inhibitors in single ventricle circulatory failure 钠-葡萄糖共转运蛋白2抑制剂对单心室循环衰竭的超声心动图影响

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-06-21 DOI: 10.1016/j.ijcchd.2025.100603

Ralph M.L. Neijenhuis , Madelien V. Regeer , Niki L. Walker , Amanda Hunter , Philippine Kiès , Eduard R. Holman , J. Wouter Jukema , Monique R.M. Jongbloed , Gruschen R. Veldtman , Anastasia D. Egorova

Background

Single ventricle patients are at high risk of developing circulatory failure. There is limited evidence for pharmacological treatment. This study assessed the echocardiographic changes in ventricular function during sodium-glucose cotransporter 2 inhibitor (SGLT2i) therapy in patients with single ventricle failure (SVF).

Methods

SVF patients with a baseline transthoracic echocardiogram within six months before starting SGLT2i and at least one echocardiographic examination within twelve months follow-up were included from a real-world international registry of adult congenital heart disease patients on SGLT2i. Mixed models were used to evaluate longitudinal changes in ventricular function and differences between patients with SVF with ≥ moderately reduced systolic function (SVFrEF) and with ≤ mildly reduced function (SVFpEF).

Results

Thirteen patients were included. The median age was 21 [20–42] years, 8 (61.5 %) were female, 10 (76.9 %) had a Fontan circulation, 8 (61.5 %) had SVFrEF, and 5 (38.5 %) SVFpEF at the start of SGLT2i. The mean follow-up was 7.6 ± 3.3 months. End-systolic area decreased significantly in all patients (−1.6 cm² per month, p = 0.007) in the first 100 days. Fractional area change improved in the first 100 days in SVFrEF patients (3.5 %-point per month, p < 0.001), while SVFpEF patients remained stable. There was a significant improvement in the free wall strain in all patients (−0.3 %-point per month, p = 0.036) but not in global longitudinal strain (p = 0.087). Isovolumic acceleration also improved in the first 100 days (0.5 m/s² per month, p = 0.010).

Conclusions

Echocardiographic signals of improved ventricular function were observed in the first year of SGLT2i therapy in patients with SVF.

背景：单心室患者发生循环衰竭的风险很高。药物治疗的证据有限。本研究评估了单心室衰竭（SVF）患者在钠-葡萄糖共转运蛋白2抑制剂（SGLT2i）治疗期间心室功能的超声心动图变化。方法选取SGLT2i治疗前6个月内接受基线经胸超声心动图检查的ssvf患者，并在随访12个月内进行至少一次超声心动图检查。采用混合模型评价收缩功能中度降低（SVFrEF）和≤轻度降低（SVFpEF）的SVF患者心室功能的纵向变化和差异。结果纳入13例患者。中位年龄为21岁[20-42]岁，女性8例（61.5%），Fontan循环10例（76.9%），SGLT2i发病时SVFrEF 8例（61.5%），SVFpEF 5例（38.5%）。平均随访7.6±3.3个月。所有患者在前100天收缩末期面积均显著减少（- 1.6 cm2 /月，p = 0.007）。SVFrEF患者的部分面积变化在前100天得到改善(每月3.5%,p <；0.001)，而SVFpEF患者保持稳定。所有患者的游离壁应变均有显著改善（- 0.3% -点/月，p = 0.036），但整体纵向应变无显著改善（p = 0.087）。等体积加速度在前100天也有所改善（每月0.5 m/s2, p = 0.010）。结论在SGLT2i治疗SVF患者的第一年，可观察到心室功能改善的心电图信号。

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引用次数: 0

Kawasaki disease with persistent severe psoriatic-like skin manifestations and sudden cardiac death 川崎病伴有持续严重的银屑病样皮肤表现和心源性猝死

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-06-26 DOI: 10.1016/j.ijcchd.2025.100604

Aso F. Salih , Trifa M. Qadir , Nagib Dahdah

This case report describes a 1.3-year-old infant with an atypical presentation of Kawasaki Disease (KD) following exposure to COVID-19 within the family and positive SARS-CoV-2 IgG antibodies. The clinical course was marked by significant left ventricular (LV) dysfunction, progressive coronary artery involvement, and complicated by severe dermatologic manifestations including Steven-Johnson-like lesions and psoriatic-like eruptions. Despite initial improvement in cardiac function, the infant developed multiple coronary aneurysms and succumbed to sudden cardiac death. This case highlights the diagnostic and therapeutic challenges in managing atypical KD in the context of COVID-19. We describe the case and review the literature related to the associated severe skin lesions.

本病例报告描述了一名1.3岁婴儿在家庭成员暴露于COVID-19和SARS-CoV-2 IgG抗体阳性后出现不典型川崎病（KD）的症状。临床表现为明显的左心室功能障碍，进行性冠状动脉受累，并伴有严重的皮肤症状，包括史蒂文-约翰逊样病变和银屑病样皮疹。尽管最初心脏功能有所改善，但婴儿出现了多个冠状动脉瘤并死于心源性猝死。该病例强调了在COVID-19背景下管理非典型KD的诊断和治疗挑战。我们描述的情况下，回顾文献相关的严重皮肤病变。

引用次数: 0

Fatigue in adults with congenital heart disease aged over 40 years 40岁以上先天性心脏病患者的疲劳

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-06-16 DOI: 10.1016/j.ijcchd.2025.100601

Linda Ternrud , Bengt Johansson , David Sparv , Zacharias Mandalenakis , Christina Christersson , Liesbet Van Bulck , Philip Moons , Camilla Sandberg , Joanna Hlebowicz

Background

Fatigue is a symptom that has been described among adult patients with congenital heart disease (CHD), but the prevalence and impact of fatigue on the patient's daily life has been poorly studied. This study (i) examines the prevalence of fatigue in patients aged over 40 years with moderately complex or complex congenital heart disease compared to controls and (ii) explores the relationship between fatigue, heart disease complexity, clinical characteristics and self-reported New York Heart Association Functional Class (self-reported NYHA class).

Methods

The Multidimensional Fatigue Inventory (MFI-20) was applied in 166 patients with moderately complex CHD or complex CHD (44 % females, median age 55.3 years, IQR 47.6–64.8) along with 89 controls (43 % female, median age 54.0, IQR 46.0–65.9). MFI-20 measured general fatigue, physical fatigue, mental fatigue, reduced motivation and reduced activity.

Results

Physical fatigue (severe to very severe) was more common in complex CHD compared with moderately complex CHD (25 % vs. 52 %, p = 0.006). Complex CHD was associated with severe to very severe physical fatigue (odds ratio 3.1 (95 % CI 1.1–9.1). Patients with complex CHD had higher levels of self-reported NYHA class than patients with moderately complex CHD. All dimensions of fatigue were positively associated with self-reported NYHA class.

Conclusions

Patients over the age of 40 with complex CHD were three times more likely to report severe to very severe physical fatigue and reported higher levels of self-reported NYHA class than patients with moderately complex CHD. This highlights the importance of considering CHD complexity in clinical practice.

疲劳是先天性心脏病（CHD）成年患者的一种症状，但疲劳的患病率和对患者日常生活的影响研究甚少。本研究(i)与对照组相比，研究了40岁以上患有中度复杂或复杂先天性心脏病的患者的疲劳患病率；（ii）探讨了疲劳、心脏病复杂性、临床特征和自我报告的纽约心脏协会功能等级（NYHA等级）之间的关系。方法采用多维疲劳量表（MFI-20）对166例中、复杂冠心病患者（女性占44%，中位年龄55.3岁，IQR 47.6 ~ 64.8）和89例对照组（女性占43%，中位年龄54.0岁，IQR 46.0 ~ 65.9）进行分析。MFI-20测量一般疲劳、身体疲劳、精神疲劳、动力减少和活动减少。结果与中度复杂冠心病相比，重度至极重度躯体疲劳在复杂冠心病中更为常见（25% vs. 52%, p = 0.006）。复杂冠心病与严重到非常严重的身体疲劳相关（优势比3.1 (95% CI 1.1-9.1)）。复杂冠心病患者自我报告的NYHA等级水平高于中度复杂冠心病患者。疲劳的所有维度都与自我报告的NYHA等级呈正相关。结论：40岁以上的复杂冠心病患者报告重度至极重度身体疲劳的可能性是中度复杂冠心病患者的3倍，自述NYHA等级水平高于中度复杂冠心病患者。这突出了在临床实践中考虑冠心病复杂性的重要性。

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引用次数: 0

Trends in congenital heart disease mortality in the United States from 1968 to 2022 1968年至2022年美国先天性心脏病死亡率趋势

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-06-30 DOI: 10.1016/j.ijcchd.2025.100607

Zahra Imran , Taimor Mohammed Khan , Saad Ahmed Waqas , Raheel Ahmed , Abdul Mannan Khan Minhas

Purpose

Congenital heart disease (CHD) remains the leading cause of infant mortality due to birth defects in the United States. We analyzed long-term CHD mortality trends across age, sex, and racial groups from 1968 to 2022.

Methods

We extracted CHD mortality data from the CDC WONDER database (1968–2022), using ICD-8/9/10 codes. Age-adjusted mortality rates (AAMRs) and crude mortality rates (CMRs) were calculated per 100,000 population. Joinpoint regression was used to assess temporal trends in mortality, reporting annual percentage changes (APCs) and average APCs (AAPCs) with 95 % confidence intervals (CIs).

Results

From 1968 to 2019, 234,658 CHD-related deaths were recorded. Overall AAMR declined from 3.2 (1968) to 0.8 (2019) per 100,000 (AAPC: −2.7 %; 95 % CI: −2.9 to −2.5). Males consistently had higher AAMRs than females. Racial disparities persisted, with slower declines among Black or African American individuals. Infants under 1 year accounted for 56.0 % of CHD deaths and showed the steepest mortality decline (AAPC: −3.2 %). Mortality rates plateaued in recent years.

Conclusions

CHD mortality in the U.S. has declined markedly over the past five decades, though progress has slowed since 2009. Persistent disparities by race and sex emphasize the need for equitable access to specialized CHD care and ongoing public health efforts.

目的：在美国，继发性心脏病（CHD）仍然是婴儿因出生缺陷而死亡的主要原因。我们分析了1968年至2022年间不同年龄、性别和种族的冠心病死亡率的长期趋势。方法采用ICD-8/9/10编码，从CDC WONDER数据库中提取冠心病死亡率数据（1968-2022）。计算每10万人的年龄调整死亡率（AAMRs）和粗死亡率（CMRs）。采用联结点回归评估死亡率的时间趋势，报告年百分比变化（APCs）和平均APCs (AAPCs)，置信区间为95% （ci）。结果从1968年到2019年，共记录了234,658例冠心病相关死亡。总体AAMR从每10万人3.2例（1968年）下降到0.8例（2019年）(AAPC: - 2.7%；95% CI:−2.9 ~−2.5)。男性的aamr始终高于女性。种族差异仍然存在，黑人或非裔美国人的下降速度较慢。1岁以下婴儿占冠心病死亡人数的56.0%，死亡率下降幅度最大（AAPC: - 3.2%）。近年来死亡率趋于稳定。结论：在过去的50年里，美国的心脏病死亡率显著下降，尽管自2009年以来进展有所放缓。种族和性别的持续差异强调了公平获得专门的冠心病护理和持续的公共卫生努力的必要性。

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引用次数: 0

A novel approach to determining pulmonary valve replacement timing in repaired tetralogy of Fallot patients 一种确定修复法洛四联症患者肺瓣膜置换术时机的新方法

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-09-01 Epub Date: 2025-05-27 DOI: 10.1016/j.ijcchd.2025.100597

Laura Delaney , Andrew Browne , Gruschen R. Veldtman , Sihong Huang

Background

Here we propose a novel approach to determine a patient-specific optimal time for pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot (rTOF) based on a mathematical technique known as optimal stopping theory.

Methods

We outline, in a theoretical framework, how we would apply the proposed timing rule for PVR in patients with rTOF. We define each of the model input variables specific to PVR, and we describe how the model can be assessed for its suitability for use in clinical practice to guide timing of PVR in patients with rTOF.

Results

In this manuscript we demonstrate, for the first time, an application of a timing rule derived from a mathematical technique known as optimal stopping theory to determine the optimal time to perform PVR in patients with rTOF. Using the technique, we derive two patient-specific-thresholds on clinical status based on patient specific risk factors. PVR is recommended if at the time of assessment, a patient's clinical status lies between the thresholds. Otherwise, they are either too well or too unwell to undergo PVR.

Conclusions

Current clinical practice guidelines for timing of PVR in patients with rTOF remains flawed and has since been challenged. We believe the timing rule is a suitable approach to complement current medical guidelines on when to perform PVR in patients with rTOF on a patient-specific level. To determine its suitability for generalized application in clinical practice, we intent to validate it retrospectively using large clinical cohort(s) who have undergone PVR for rTOF.

在此，我们提出了一种新的方法来确定修复法洛四联症（rTOF）患者肺瓣膜置换术（PVR）的患者特异性最佳时间，该方法基于一种称为最佳停止理论的数学技术。方法在一个理论框架中，我们概述了我们如何在rTOF患者中应用所提出的PVR时间规则。我们定义了每个特定于PVR的模型输入变量，并描述了如何评估该模型在临床实践中用于指导rTOF患者PVR时机的适用性。结果在这篇论文中，我们首次证明了从称为最佳停止理论的数学技术中导出的时间规则的应用，以确定rTOF患者进行PVR的最佳时间。使用该技术，我们根据患者特定的风险因素得出两个患者特定的临床状态阈值。如果在评估时，患者的临床状态处于阈值之间，则建议进行PVR。否则，他们要么太好，要么太不舒服，不能接受PVR。结论：目前关于rTOF患者PVR时机的临床实践指南仍然存在缺陷，并且一直受到挑战。我们认为时间规则是一种合适的方法来补充当前的医学指南，即何时在患者特异性水平上对rTOF患者进行PVR。为了确定其在临床实践中广泛应用的适用性，我们打算使用大型临床队列进行回顾性验证，这些队列接受了PVR治疗rTOF。

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