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International journal of cardiology. Congenital heart disease最新文献

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Thromboprophylaxis and adult congenital heart disease: The latest on indications, risk scoring and therapy 血栓预防和成人先天性心脏病:最新的适应症,风险评分和治疗
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-29 DOI: 10.1016/j.ijcchd.2025.100570
Mariana Sousa Paiva , Jorge Ferreira , Rui Anjos , Michael A. Gatzoulis
Advances in medical care have significantly extended the lifespan of patients with congenital heart disease (CHD), allowing most to survive into adulthood. However, they continue to face significant cardiovascular morbidity, particularly atrial arrhythmias (AA), heart failure, and thromboembolic (TE) events. TE events in adult CHD patients arise from various factors, including AA, intracardiac repairs, cyanotic CHD, Fontan palliation, pregnancy, and mechanical heart valves (MHV). As randomized clinical trials are lacking, most current guidelines rely on observational data and expert opinions, leading to inherent variability. While vitamin K antagonists are the only option for patients with MHV and significant mitral stenosis, direct oral anticoagulants appear to be a reasonable choice for other indications. In the presence of AA, complex conditions alone may justify anticoagulation, whereas thromboembolic and haemorrhagic risks should be evaluated individually for simpler lesions. This review summarizes the available evidence and makes relevant recommendations regarding thromboprophylaxis in ACHD patients, focusing on indications, risk scores, and therapies.
医疗保健的进步大大延长了先天性心脏病(CHD)患者的寿命,使大多数人能够活到成年。然而,他们仍然面临显著的心血管疾病,特别是心房心律失常(AA)、心力衰竭和血栓栓塞(TE)事件。成年冠心病患者的TE事件可由多种因素引起,包括AA、心内修复、紫型冠心病、Fontan姑息治疗、妊娠和机械心脏瓣膜(MHV)。由于缺乏随机临床试验,目前大多数指南依赖于观察数据和专家意见,导致固有的可变性。虽然维生素K拮抗剂是MHV和明显二尖瓣狭窄患者的唯一选择,但直接口服抗凝剂似乎是其他适应症的合理选择。在存在AA的情况下,单独的复杂情况可以证明抗凝是合理的,而对于简单的病变,血栓栓塞和出血风险应该单独评估。这篇综述总结了现有的证据,并就ACHD患者的血栓预防提出了相关建议,重点是适应证、风险评分和治疗。
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引用次数: 0
Sedentary behavior and physical activity on exercise capacity in adult patients with congenital heart disease 久坐行为和体力活动对成年先天性心脏病患者运动能力的影响
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-22 DOI: 10.1016/j.ijcchd.2025.100569
Masahiro Matsui , Keisei Kosaki , Naoto Kawamatsu , Yoshihiro Nozaki , Tomoko Machino-Otsuka , Yoshio Nakata , Seiji Maeda , Tomoko Ishizu

Background

Reduced exercise capacity is associated with a poor prognosis in adult patients with congenital heart disease (CHD). Reducing sedentary behavior (SB) and increasing physical activity (PA) could be potential strategies that may contribute to enhanced fitness and prevention of acquired cardiovascular disease in adult patients with CHD. The present study aimed to examine the association of SB and PA with exercise capacity in adult patients with CHD.

Methods

Ninety-six adult patients with CHD (age: 18–74 years) underwent measurements of peak oxygen uptake (VO2), % predicted peak VO2, and time spent in SB, light physical activity (LPA), and moderate-to-vigorous physical activity (MVPA). Three regression models (single-activity, partition, and isotemporal substitution) were used to examine the associations of the time spent in SB, LPA, and MVPA with peak VO2 and % predicted peak VO2.

Results

In the single-activity and partition models, time spent in MVPA was consistently associated with peak VO2 and % predicted peak VO2. The isotemporal substitution model indicated that replacing 10-min of SB with the same duration of MVPA was associated with a higher peak VO2 (by 0.454 mL/min/kg [0.100 mL/min/kg, 0.807 mL/min/kg]) and % predicted peak VO2 (by 1.810 % [0.594 %, 3.026 %]).

Conclusion

These findings suggest that reducing SB time and increasing PA time are associated with improved exercise capacity in adult patients with CHD.
研究背景:成人先天性心脏病(CHD)患者运动能力降低与预后不良相关。减少久坐行为(SB)和增加身体活动(PA)可能是有助于增强成年冠心病患者健康和预防获得性心血管疾病的潜在策略。本研究旨在探讨SB和PA与成年冠心病患者运动能力的关系。方法96例成年冠心病患者(年龄:18-74岁)测量了峰值摄氧量(VO2)、预测峰值VO2百分比、SB运动时间、轻体力活动(LPA)和中高强度体力活动(MVPA)。使用三种回归模型(单活动、分区和等时间替代)来检查在SB、LPA和MVPA中花费的时间与峰值VO2和预测峰值VO2 %之间的关系。结果在单活动模型和分区模型中,在MVPA中花费的时间与VO2峰值和预测VO2峰值百分比一致。等时间替代模型表明,用相同时间的MVPA代替10分钟的SB可提高VO2峰值(提高0.454 mL/min/kg [0.100 mL/min/kg, 0.807 mL/min/kg])和%预测VO2峰值(提高1.810 %[0.594 %,3.026%])。结论减少SB时间和增加PA时间可提高成年冠心病患者的运动能力。
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引用次数: 0
Right heart reverse remodeling: “facta non verba” 右心脏反向重构:“事实非语言”
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-17 DOI: 10.1016/j.ijcchd.2025.100568
Tommaso Recchioni , Giovanna Manzi , Alexandra Mihai , Francesca Ileana Adamo , Annalisa Caputo , Domenico Filomena , Giorgia Serino , Silvia Papa , Nadia Cedrone , Carmine Dario Vizza , Roberto Badagliacca
Pulmonary arterial hypertension (PAH) is characterized by pulmonary vascular remodeling and arterial narrowing, leading to a progressive rise in right ventricular (RV) afterload and poor survival outcomes. PAH prognosis largely depends on RV remodeling and function: when the increased afterload exceeds the RV's adaptive capacity, ventricular-arterial uncoupling occurs, ultimately causing right heart failure and death. In this clinical setting the primary treatment goal is to achieve low mortality risk and right heart reverse remodeling (RHRR). Unfortunately, the definition of RHRR vary across studies and imaging modalities (echocardiography or Cardiac Magnetic Resonance). The likelihood of RHRR increases with a significant reduction in pulmonary vascular resistance (PVR) from baseline, ideally by at least 50 %. Evidence supports initial triple therapy, including parenteral prostanoids, as the most effective approach to reduce PVR enough to facilitate RHRR and thus achieve the low-risk status.
肺动脉高压(PAH)以肺血管重构和动脉狭窄为特征,导致右心室(RV)后负荷进行性升高和生存预后差。PAH的预后在很大程度上取决于右心室重塑和功能:当增加的后负荷超过右心室的适应能力时,发生心室-动脉解耦,最终导致右心衰和死亡。在这个临床环境中,主要的治疗目标是实现低死亡率和右心反向重构(RHRR)。不幸的是,RHRR的定义因研究和成像方式(超声心动图或心脏磁共振)而异。随着肺血管阻力(PVR)从基线显著降低,理想情况下至少降低50%,RHRR的可能性增加。有证据支持最初的三联疗法,包括肠外前列腺素,是降低PVR的最有效方法,足以促进RHRR,从而达到低风险状态。
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引用次数: 0
Family caregiver frailty in adult congenital heart disease is overlooked: A call to action 家庭照顾者脆弱在成人先天性心脏病被忽视:行动呼吁。
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-17 DOI: 10.1016/j.ijcchd.2025.100567
Arianna Magon , Serena Francesca Flocco , Laura Schianchi , Massimo Chessa , Daniele Ciofi , Salvatore Angileri , Maddalena De Maria , Gianluca Conte , Silvia Favilli , Cristina Arrigoni , Rosario Caruso
The frailty of family caregivers (FCs) of adults with congenital heart disease (CHD) is an underrecognized yet critical issue in healthcare. As individuals with CHD live longer, their FCs, often aging alongside them, face escalating physical, emotional, and psychological challenges. This brief report examines frailty in FCs as a multidimensional issue stemming from prolonged caregiving demands and cumulative stress. The evidence gathered in this regard identifies three key domains affecting FCs: psychological stress and childcare, physical health decline, and the challenges of caregiving during transitions to adulthood. These findings underscore the urgent need for family-centered care models that incorporate tailored frailty assessments and targeted interventions to support FCs throughout their caregiving journey. Addressing caregiver frailty could improve outcomes for both patients and caregivers, strengthen resilience, and optimize resource allocation. This perspective calls for immediate action in research and policy to prioritize caregiver well-being in transition care programs, enhancing the quality of life for both caregivers and patients.
先天性心脏病(CHD)成人患者的家庭照顾者(FCs)的虚弱是医疗保健中一个未得到充分认识但却至关重要的问题。随着先天性心脏病患者寿命的延长,他们的家庭照顾者通常也会随之老去,面临着身体、情感和心理方面不断升级的挑战。本简要报告探讨了功能障碍患者的虚弱问题,认为这是一个多层面的问题,源于长期的护理需求和累积的压力。在这方面收集到的证据确定了影响家庭佣工的三个关键领域:心理压力和育儿、身体健康状况下降以及在向成年过渡期间的护理挑战。这些研究结果突出表明,以家庭为中心的护理模式亟需纳入量身定制的虚弱评估和有针对性的干预措施,以便在整个护理过程中为家庭护理者提供支持。解决照顾者的虚弱问题可以改善患者和照顾者的预后、增强恢复能力并优化资源分配。这一观点呼吁在研究和政策方面立即采取行动,在过渡护理计划中优先考虑护理人员的福祉,提高护理人员和患者的生活质量。
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引用次数: 0
Pregnancy and pulmonary artery hypertension: Management challenges 妊娠和肺动脉高压:管理挑战
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-07 DOI: 10.1016/j.ijcchd.2025.100566
Gurleen Wander , Claudia Montanaro , Prithvi Dixit , Daryl Dob , Mark R. Johnson , Roshni R. Patel
Pulmonary hypertension (PH) is a term used to describe a complex heterogenous group of conditions defined by a mean pulmonary arterial pressure of more than 20 mmHg at rest on right-heart catheterization. PH in pregnancy is associated with high rates of maternal morbidity and mortality and poor fetal outcomes. Currently, pregnancy in these women is classified as modified WHO class IV (pregnancy contraindicated). More recent data suggest that the prognosis varies with the underlying aetiology as well as with PH severity. Consequently, management during pregnancy must be individualised, with patients cared for in a tertiary unit as part of an experienced multidisciplinary team. In this article, we will discuss the importance of preconception counselling, the impact of the haemodynamic changes induced by pregnancy, the maternal and fetal risks of pregnancy in women with PH, and how these can be minimised by close antenatal, intrapartum and post-partum care and the development of individualised pregnancy plans.
肺动脉高压(PH)是一个术语,用于描述一组复杂的异质性疾病,其定义为右心导管静息时平均肺动脉压超过20mmhg。妊娠期PH与高产妇发病率和死亡率以及不良胎儿结局有关。目前,这些妇女的妊娠被归类为修改后的世卫组织第四类(妊娠禁忌症)。最近的数据表明,预后随潜在的病因和PH严重程度而变化。因此,怀孕期间的管理必须个性化,患者在三级单位作为一个有经验的多学科团队的一部分进行护理。在本文中,我们将讨论孕前咨询的重要性,妊娠引起的血流动力学变化的影响,PH妇女妊娠的母婴风险,以及如何通过密切的产前、产时和产后护理以及个性化妊娠计划的制定来最大限度地减少这些风险。
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引用次数: 0
Physical exercise for pulmonary arterial hypertension diagnosis and therapy 体育锻炼对肺动脉高压的诊断和治疗
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-01-04 DOI: 10.1016/j.ijcchd.2025.100565
Myo Lwin , Abigail Masding , Colm McCabe
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引用次数: 0
Cardiac rehabilitation in ACHD: Further investment is now due ACHD的心脏康复:现在需要进一步的投资。
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-12-31 DOI: 10.1016/j.ijcchd.2024.100564
Ioannis Kasouridis , Heather Probert , Michael A. Gatzoulis
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引用次数: 0
Toronto ACHD program: A 65 year legacy 多伦多ACHD项目:65年的历史。
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-12-30 DOI: 10.1016/j.ijcchd.2024.100563
J.M. Colman , W.G. Williams , C.K. Silversides , L. Harris , L. Benson , J. Heggie , R. Alonso-Gonzalez , E. Oechslin
The Toronto Adult Congenital Heart Disease (ACHD) Program at the University Health Network, University of Toronto, began in 1959. It traces its origins to a Paul Wood protégé, Dr. John Evans, and to a long-standing and supportive relationship with Hospital for Sick Children (SickKids), located just across the street. Over the decades, the program has grown to become a major center for training and research in ACHD and one of the largest clinical programs for ACHD care globally. This paper recounts the 65-year history of the program, including some of its key individuals, challenges, milestones, innovations, discoveries, and future aspirations.
多伦多大学健康网络的多伦多成人先天性心脏病(ACHD)项目始于1959年。它的起源可以追溯到保罗·伍德(Paul Wood)一位名叫约翰·埃文斯(John Evans)的博士,并与街对面的病童医院(SickKids)建立了长期的支持关系。几十年来,该项目已发展成为ACHD培训和研究的主要中心,也是全球最大的ACHD护理临床项目之一。本文叙述了该项目65年的历史,包括一些关键人物、挑战、里程碑、创新、发现和未来的愿望。
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引用次数: 0
Ventricular arrhythmias and the role of antitachycardia pacing in patients with electrical heart disease and hypertrophic cardiomyopathy 心电性疾病和肥厚性心肌病患者室性心律失常和抗心动过速起搏的作用
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-12-28 DOI: 10.1016/j.ijcchd.2024.100562
Verena Kantenwein , Herribert Pavaci , Bernhard Haller , Marta Telishevska , Lena Friedrich , Maximilian Walgenbach , Carsten Lennerz , Christof Kolb

Background

Whether it is reasonable to program ATP in patients with electrical heart disease (EHD) or hypertrophic cardiomyopathy (HCM) is not thoroughly clarified. Aim of the study was to define the types of ventricular arrhythmias and evaluate the safety and efficacy of ATP activation in these patients.

Methods and results

A total of 154 patients (53.9 % male, 64.9 % secondary prevention) with EHD or HCM, who had an implanted cardioverter defibrillator (ICD) with ATP activated, were included in this retrospective analysis; comprising a median of 65.0 months of follow-up. In 39/154 (25.3 %) patients appropriate ICD therapy was delivered during the follow-up. Patients with HCM had a significantly higher incidence rate of monomorphic VTs than patients with EHD (0.21 versus 0.01 per month, 0 < 0.001). ATP terminated monomorphic VT with an efficacy of 88,2 % in 94.9 % of the occurring episodes. The incidence rate per month of torsade de pointes (TdP) tachycardia and VF was significantly higher in patients with EHD versus HCM (0.04 vs. 0.001, p=<0.001; 0.06 vs. 0.007, p=<0.001). The termination of TdP tachycardia and VF was associated with ATP in 14.0 % and 0 % (ATP efficacy of 28.3 % and 0 % respectively). The implantation for secondary prevention was associated with the occurrence of appropriate ICD therapy during the follow-up period (OR 3.94 [95%CI 1.53–10.14], p = 0.005).

Conclusion

Ventricular tachycardias in patients with HCM are primarily monomorphic and can be effectively terminated with ATP. In patients with EHD, TdP tachycardias and VF occur more frequently and are preferentially terminated by ICD shock.
背景:对于电性心脏病(EHD)或肥厚性心肌病(HCM)患者,对ATP进行编程是否合理尚不完全清楚。本研究的目的是确定室性心律失常的类型,并评估ATP激活在这些患者中的安全性和有效性。方法和结果回顾性分析154例EHD或HCM患者(53.9%为男性,64.9%为二级预防),植入ATP激活的心律转复除颤器(ICD);中位随访时间为65.0个月。39/154(25.3%)患者在随访期间给予适当的ICD治疗。HCM患者单型型室性血栓的发生率明显高于EHD患者(0.21 vs 0.01 /月,0 <;0.001)。在94.9%的发作中,ATP终止单型室速的有效率为88.2%。EHD患者每月TdP心动过速和VF的发生率明显高于HCM患者(0.04 vs. 0.001, p=<0.001;0.06 vs. 0.007, p=<0.001)。TdP心动过速和VF的终止与ATP的相关性分别为14.0%和0% (ATP的有效性分别为28.3%和0%)。二级预防植入与随访期间适当的ICD治疗发生率相关(OR 3.94 [95%CI 1.53-10.14], p = 0.005)。结论HCM患者室性心动过速以单形态性心动过速为主,ATP可有效终止。在EHD患者中,TdP心动过速和室颤更频繁发生,并优先通过ICD休克终止。
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引用次数: 0
Coarctation of the aorta and accelerated atherosclerosis: A contemporary review on the burden of atherosclerotic cardiovascular disease 主动脉缩窄和动脉粥样硬化加速:动脉粥样硬化性心血管疾病负担的当代回顾
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-12-25 DOI: 10.1016/j.ijcchd.2024.100561
Melissa G.Y. Lee , Phillip S. Naimo , Anoop N. Koshy , Edward Buratto , William M. Wilson , Leeanne E. Grigg , Subodh B. Joshi , Katherine M. English
Coarctation of the aorta (CoA) is one of the most common types of congenital heart disease. Unfortunately, there is a high prevalence of hypertension and late cardiovascular mortality in patients with CoA despite successful repair. The growing impact of acquired cardiovascular disease remains a significant concern as the adult congenital heart disease population continues to rapidly expand and age. This review aims to explore (1) the determinants of vascular health and atherosclerosis including endothelial dysfunction and vascular wall abnormalities, (2) the prevalence of atherosclerosis and associated sequelae in repaired CoA including coronary artery disease, coronary artery calcium, aortic calcium, stroke, and peripheral artery disease, and (3) the contributing factors specific to CoA. This review aims to guide optimization of long-term cardiovascular health to ultimately reduce mortality and morbidity in this young high-risk population.
主动脉缩窄(CoA)是一种最常见的先天性心脏病。不幸的是,尽管修复成功,CoA患者的高血压患病率和晚期心血管死亡率仍然很高。随着成人先天性心脏病人口持续迅速扩大和老龄化,获得性心血管疾病的影响越来越大,这仍然是一个值得关注的问题。本综述旨在探讨(1)血管健康和动脉粥样硬化的决定因素,包括内皮功能障碍和血管壁异常;(2)动脉粥样硬化的患病率和相关后遗症,包括冠状动脉疾病、冠状动脉钙化、主动脉钙化、中风和外周动脉疾病;(3)CoA特异性的影响因素。本综述旨在指导优化长期心血管健康,最终降低这一年轻高危人群的死亡率和发病率。
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引用次数: 0
期刊
International journal of cardiology. Congenital heart disease
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