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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 : 2025-03-01 Epub 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 : 2025-03-01 Epub 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
Atrial tachycardia in patients with repaired tetralogy of Fallot; its characteristics and catheter ablation outcome 修复法洛四联症患者心房性心动过速的研究其特点及导管消融效果
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-03-01 Epub Date: 2024-12-10 DOI: 10.1016/j.ijcchd.2024.100558
Qasim J. Naeemah , Miyako Igarashi , Noor K. Albakaa , Yuichi Hanaki , Noboru Ichihara , Chihiro Ota , Akira Kimata , Kojiro Ogawa , Naoto Kawamatsu , Tomoko Machino , Yuki Komatsu , Hiro Yamasaki , Akihiko Nogami , Masaki Ieda , Tomoko Ishizu

Background

Patients with repaired tetralogy of Fallot (TOF) now live longer. However, dysrhythmia becomes prevalent in adults with repaired TOF, especially atrial tachyarrhythmia.

Objective

To identify the characteristics of patients who develop atrial tachycardia (AT) and the mechanism of the clinical AT and the induced one.

Method

Seventy-seven patients with repaired TOF were enrolled. The patients were divided into two groups (AT and non-AT). Clinical and electrophysiologic data were studied.

Results

The mean age was 34 years. Twenty-three patients had AT (30 %). In AT group, the left ventricular ejection fraction was lower (58 ± 6 vs 62 ± 5; P = 0.011), the right and left atrial area (cm2) was larger (29 ± 13 vs 15 ± 5; P < 0.001, and 19 ± 3 vs 16 ± 4; P < 0.001, respectively), and the right ventricular S′ wave (cm/s) was smaller (8 ± 2 vs 10 ± 3; P = 0.029).
Patients with AT underwent catheter ablation, and 32 AT were ablated. The mechanism of AT was intra-atrial reentrant tachycardia in 14 AT (44 %), cavotricuspid isthmus-dependent in 12 AT (37 %), and focal activity in the remaining 6 AT (19 %). An important finding was that after the first AT was ablated, another AT was induced in 7 patients. The mechanism was focal in about half of them, in contrast to the first ablated AT, where the focal mechanism was the least common. After a median follow-up of 37 months, four patients had AT recurrence.

Conclusion

The patients with AT had biventricular dysfunction and bi-atrial dilatation. Aggressive induction and ablation of the induced AT may reduce the future AT recurrence.
背景:修复法洛四联症(TOF)的患者现在寿命更长。然而,心律失常在修复TOF的成年人中变得普遍,尤其是心房性心动过速。目的探讨心房心动过速(AT)患者的特点及临床和诱发性心房心动过速的发生机制。方法入选77例修复性TOF患者。患者分为AT组和非AT组。研究临床和电生理数据。结果患者平均年龄34岁。23例患者有AT(30%)。AT组左室射血分数较低(58±6 vs 62±5);P = 0.011),左右心房面积(cm2)较大(29±13 vs 15±5;P & lt;0.001, 19±3 vs 16±4;P & lt;右心室S波(cm/ S)较小(8±2 vs 10±3;p = 0.029)。AT患者行导管消融,32例AT消融。AT的发生机制为14例(44%)心房再入性心动过速,12例(37%)心房再入性心动过速,其余6例(19%)心房再入性心动过速。一个重要的发现是,在第一个AT消融后,7例患者又诱导了另一个AT。与第一次消融AT相比,其中大约一半的机制是焦点性的,焦点机制是最不常见的。中位随访37个月后,4例AT复发。结论AT患者存在双室功能障碍和双房扩张。积极诱导和消融诱发的AT可能减少未来AT的复发。
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引用次数: 0
Does stroke volume limit exercise capacity in TGA patients after the arterial switch operation? 动脉转换手术后,搏出量是否会限制 TGA 患者的运动能力?
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-03-01 Epub Date: 2025-02-15 DOI: 10.1016/j.ijcchd.2025.100576
Renée S. Joosen , Michiel Voskuil , Wieke G. de Pater , Sebastiaan W.H. van Wijk , Dominika Suchá , Abraham van Wijk , Heleen B.van der Zwaan , Gregor J. Krings , Tim Takken , Johannes M.P.J. Breur

Background

Patients with transposition of the great arteries (TGA) experience reduced exercise capacity after the arterial switch operation (ASO), possibly due to limited stroke volume. This study evaluates the role of stroke volume in reduced exercise capacity in these patients.

Methods

A retrospective analysis was conducted on TGA patients who underwent a transthoracic echocardiogram (TTE), cardiac magnetic resonance (CMR) and cardiopulmonary exercise test (CPET) within one year between September 2009 and February 2024 at the University Medical Center Utrecht. Excluding those with submaximal CPET results, the remaining patients were divided into <18 and ≥ 18 years old groups. Reduced exercise capacity was defined as a peak oxygen uptake (VO2peak) with a Z-score < -2. Left and right ventricular (LV and RV) data including volumes, function, strain and RV outflow tract obstructions were collected from TTE and CMR.

Results

A total of 126 patients (72 % male, mean age 19 ± 8 years) were included. Left ventricular function, RV volumes, function and strain were relatively preserved on CMR. Reduced VO2peak was seen in 55 % of patients ≥18 years, significantly more than those <18 years (23 %, p < 0.001). Reduced VO2peak was independently associated with time since ASO, body mass index, peak heat rate (HRpeak), and O2pulse. VO2peak showed weak to moderate correlations with time after ASO (R = −0.295,p < 0.001), body mass index (R = −0.468,p < 0.001) and HRpeak (R = 0.270,p = 0.002) and a strong correlation with O2pulse (R = 0.621,p < 0.001).

Conclusion

Exercise capacity in TGA patients after ASO might be limited by an impaired ability to increase stroke volume.
背景:大动脉转位(TGA)患者在动脉转换手术(ASO)后运动能力降低,可能是由于卒中容量有限。本研究评估卒中容量在这些患者运动能力降低中的作用。方法回顾性分析2009年9月至2024年2月在乌得勒支大学医学中心接受经胸超声心动图(TTE)、心脏磁共振(CMR)和心肺运动试验(CPET)检查的TGA患者。除CPET结果亚极大者外,其余患者分为18岁组和≥18岁组。运动能力降低定义为峰值摄氧量(vo2峰值),z分数为<;2。TTE和CMR收集左、右心室(LV和RV)数据,包括容积、功能、应变和RV流出道阻塞。结果共纳入126例患者,其中男性72%,平均年龄19±8岁。CMR上左室功能、左室容积、功能及应变均相对保存。在≥18岁的患者中,55%的患者出现vo2峰值降低,显著高于≥18岁的患者(23%,p <;0.001)。降低的vo2峰值与ASO后的时间、体重指数、峰值热率(HRpeak)和o2脉冲独立相关。vo2峰值与ASO后时间呈弱至中度相关性(R = - 0.295,p <;0.001),体重指数(R = - 0.468,p <;0.001)和HRpeak (R = 0.270,p = 0.002),与O2pulse (R = 0.621,p <;0.001)。结论ASO术后TGA患者的运动能力可能因增加卒中容量的能力受损而受到限制。
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引用次数: 0
Effect of pregnancy on bioprosthetic structural valve degeneration 妊娠对生物假体结构瓣膜变性的影响
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-03-01 Epub Date: 2024-12-20 DOI: 10.1016/j.ijcchd.2024.100559
Joshua J. Price , Kalani Ruiz , Jaewon Lim , Yuli Kim , Jonathan Buber

Background

Despite increasing frequency of pregnancies among patients with cardiac conditions, including the presence of prosthetic heart valves, data on the effects of physiological changes during pregnancy on the function of bioprosthetic valves remains scarce and shows conflicting results.

Objectives

This study was aimed to determine the effect of pregnancy on the rate of bioprosthetic structural valve degeneration.

Methods

We designed a retrospective matched cohort study of patients seen between June 2018 and February 2023. All pregnant patients with bioprosthetic valves were matched to non-pregnant controls with prior valve replacement based on bioprosthetic valve location, time since valve implantation, age of the patient at valve implantation and time between baseline and follow up echocardiograms. Echocardiograms of pregnant patients were evaluated for bioprosthetic structural valve degeneration grade based on a dedicated scale before and after pregnancy. Non-pregnant controls had echocardiogram scoring of structural valve degeneration over a similar time period.

Results

Thirty four pregnant patients with bioprosthetic valves in the pulmonary, aortic and mitral positions were matched with 71 non-pregnant controls with identical bioprosthetic valves locations. Over a median follow up period of 13.5 months that included the gestational period, 18 (53 %) pregnant patients had an increase in structural valve degeneration score as compared to 17 (26 %) of the non-pregnant patients in median follow up of 13.7 months (OR 3.87, p = 0.004). On multivariable analysis, pregnancy was the only variable associated with increased structural valve degeneration score.

Conclusions

Our results suggest that pregnancy is associated with increased bioprosthetic structural valve degeneration.
尽管有心脏疾病的患者怀孕的频率越来越高,包括人工心脏瓣膜的存在,但关于怀孕期间生理变化对生物人工心脏瓣膜功能影响的数据仍然很少,并且结果相互矛盾。目的探讨妊娠对人工瓣膜退行性变发生率的影响。方法我们设计了一项回顾性匹配队列研究,纳入了2018年6月至2023年2月期间就诊的患者。根据生物瓣膜置换术的位置、瓣膜置换术的时间、瓣膜置换术患者的年龄以及基线和随访超声心动图之间的时间,将所有接受生物瓣膜置换术的孕妇与未接受过瓣膜置换术的未怀孕对照组进行匹配。采用超声心动图对妊娠患者在妊娠前后的生物假体结构瓣膜退行性变分级进行评价。未怀孕的对照组在相似的时间内有结构性瓣膜变性的超声心动图评分。结果34例在肺动脉、主动脉和二尖瓣位置放置生物瓣膜的孕妇与71例具有相同生物瓣膜位置的非妊娠对照组匹配。在包括妊娠期在内的中位随访13.5个月期间,18例(53%)妊娠患者的结构性瓣膜退变评分增加,而在中位随访13.7个月期间,17例(26%)非妊娠患者的结构性瓣膜退变评分增加(OR 3.87, p = 0.004)。在多变量分析中,妊娠是唯一与结构性瓣膜变性评分增加相关的变量。结论妊娠与生物假体结构瓣膜变性增加有关。
{"title":"Effect of pregnancy on bioprosthetic structural valve degeneration","authors":"Joshua J. Price ,&nbsp;Kalani Ruiz ,&nbsp;Jaewon Lim ,&nbsp;Yuli Kim ,&nbsp;Jonathan Buber","doi":"10.1016/j.ijcchd.2024.100559","DOIUrl":"10.1016/j.ijcchd.2024.100559","url":null,"abstract":"<div><h3>Background</h3><div>Despite increasing frequency of pregnancies among patients with cardiac conditions, including the presence of prosthetic heart valves, data on the effects of physiological changes during pregnancy on the function of bioprosthetic valves remains scarce and shows conflicting results.</div></div><div><h3>Objectives</h3><div>This study was aimed to determine the effect of pregnancy on the rate of bioprosthetic structural valve degeneration.</div></div><div><h3>Methods</h3><div>We designed a retrospective matched cohort study of patients seen between June 2018 and February 2023. All pregnant patients with bioprosthetic valves were matched to non-pregnant controls with prior valve replacement based on bioprosthetic valve location, time since valve implantation, age of the patient at valve implantation and time between baseline and follow up echocardiograms. Echocardiograms of pregnant patients were evaluated for bioprosthetic structural valve degeneration grade based on a dedicated scale before and after pregnancy. Non-pregnant controls had echocardiogram scoring of structural valve degeneration over a similar time period.</div></div><div><h3>Results</h3><div>Thirty four pregnant patients with bioprosthetic valves in the pulmonary, aortic and mitral positions were matched with 71 non-pregnant controls with identical bioprosthetic valves locations. Over a median follow up period of 13.5 months that included the gestational period, 18 (53 %) pregnant patients had an increase in structural valve degeneration score as compared to 17 (26 %) of the non-pregnant patients in median follow up of 13.7 months (OR 3.87, p = 0.004). On multivariable analysis, pregnancy was the only variable associated with increased structural valve degeneration score.</div></div><div><h3>Conclusions</h3><div>Our results suggest that pregnancy is associated with increased bioprosthetic structural valve degeneration.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100559"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163934","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
My journey with pulmonary hypertension: From diagnosis to thriving 我的肺动脉高压之旅:从诊断到康复
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-03-01 Epub Date: 2025-01-31 DOI: 10.1016/j.ijcchd.2025.100572
Hall Skaara
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引用次数: 0
The root of the matter: Linking oral health to chronic diseases prevention 问题的根源:将口腔健康与慢性病预防联系起来
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-03-01 Epub Date: 2025-02-12 DOI: 10.1016/j.ijcchd.2025.100574
Francesca D'Aiuto , Jeanie Suvan , Nisachon Siripaiboonpong , Michael A. Gatzoulis , Francesco D'Aiuto
Oral health is increasingly recognized as a vital component of general health, influencing various systemic systems. Periodontal diseases, particularly periodontitis, a chronic inflammatory condition affecting the gums and supporting tissues of teeth, have far-reaching implications beyond the oral cavity. Treating periodontitis not only benefits oral health but also plays a crucial role in reducing the burden of these chronic conditions, improving patient outcomes and lowering healthcare costs. Regular screenings for oral health issues, especially in patients with conditions such as cardiovascular disease or diabetes, should become standard practice in medical settings. Additionally, oral health professionals must be empowered to identify early signs of systemic diseases, creating a bidirectional flow of referrals between dentists and physicians. Ultimately, prioritizing oral health not only enhances individual well-being but also serves the greater public health good.
口腔健康越来越被认为是整体健康的重要组成部分,影响着各个系统。牙周病,特别是牙周炎,一种影响牙龈和牙齿支撑组织的慢性炎症,对口腔以外的疾病有着深远的影响。治疗牙周炎不仅有利于口腔健康,而且在减轻这些慢性疾病的负担、改善患者预后和降低医疗保健成本方面起着至关重要的作用。定期对口腔健康问题进行筛查,特别是对患有心血管疾病或糖尿病等疾病的患者,应成为医疗机构的标准做法。此外,口腔卫生专业人员必须有权识别全身性疾病的早期迹象,在牙医和医生之间建立双向转诊。最终,优先考虑口腔健康不仅可以提高个人福祉,还可以为更大的公共卫生服务。
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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 : 2025-03-01 Epub Date: 2024-12-31 DOI: 10.1016/j.ijcchd.2024.100564
Ioannis Kasouridis , Heather Probert , Michael A. Gatzoulis
{"title":"Cardiac rehabilitation in ACHD: Further investment is now due","authors":"Ioannis Kasouridis ,&nbsp;Heather Probert ,&nbsp;Michael A. Gatzoulis","doi":"10.1016/j.ijcchd.2024.100564","DOIUrl":"10.1016/j.ijcchd.2024.100564","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100564"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11772920/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143070034","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Hypoplastic left heart syndrome (HLHS) becomes of age: Assessing the young adult with HLHS including the neoaorta/aortic arch 左心发育不全综合征(HLHS)的年龄变化:评估包括新主动脉/主动脉弓在内的HLHS的年轻人
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-03-01 Epub Date: 2024-11-29 DOI: 10.1016/j.ijcchd.2024.100555
Polona Kačar , Pietro Paolo Tamborrino , Giulia Iannaccone , Gianfranco Butera , Margarita Brida , Katja Prokšelj , Michael A. Gatzoulis , Claudia Montanaro
Hypoplastic left heart syndrome (HLHS) is one of the most complex congenital heart defects (CHD), characterized by a hypoplastic left ventricle (LV), dominant right ventricle (RV) and small left-sided heart structures. The introduction of the Norwood operation has significantly improved outcomes, with 5-year survival reported up to 65 %. Despite these advances, post-operative morbidity and mortality remain high, and the long-term complications in adult survivors represent a challenge. The number of HLHS patients with Fontan circulation is expected to double in the next 20 years, leading to a growing population requiring specialized care from adult congenital heart disease (ACHD) teams.
This article reviews current management strategies for HLHS, outlines potential long-term complications, and highlights existing knowledge gaps. Specific considerations in this population include the assessment of the neo-aorta and aortic arch, and systemic RV dysfunction in the setting of a Fontan circulation. The proposed surveillance strategy emphasizes the need for vigilant monitoring and timely intervention to treat the complications unique to this population, ensuring better outcomes for HLHS patients reaching adulthood.
左心发育不全综合征(HLHS)是最复杂的先天性心脏缺陷(CHD)之一,其特征是左心室发育不全,右心室占主导地位,左侧心脏结构较小。Norwood手术的引入显著改善了预后,据报道5年生存率高达65%。尽管取得了这些进展,但术后发病率和死亡率仍然很高,成年幸存者的长期并发症是一个挑战。患有Fontan循环的HLHS患者数量预计在未来20年内将翻一番,导致越来越多的人需要成人先天性心脏病(ACHD)团队的专业护理。本文回顾了当前的HLHS管理策略,概述了潜在的长期并发症,并强调了现有的知识差距。在这一人群中需要特别考虑的因素包括评估新主动脉和主动脉弓,以及在Fontan循环设置下的全体性RV功能障碍。拟议的监测策略强调需要警惕监测和及时干预,以治疗这一人群特有的并发症,确保HLHS患者成年后获得更好的结果。
{"title":"Hypoplastic left heart syndrome (HLHS) becomes of age: Assessing the young adult with HLHS including the neoaorta/aortic arch","authors":"Polona Kačar ,&nbsp;Pietro Paolo Tamborrino ,&nbsp;Giulia Iannaccone ,&nbsp;Gianfranco Butera ,&nbsp;Margarita Brida ,&nbsp;Katja Prokšelj ,&nbsp;Michael A. Gatzoulis ,&nbsp;Claudia Montanaro","doi":"10.1016/j.ijcchd.2024.100555","DOIUrl":"10.1016/j.ijcchd.2024.100555","url":null,"abstract":"<div><div>Hypoplastic left heart syndrome (HLHS) is one of the most complex congenital heart defects (CHD), characterized by a hypoplastic left ventricle (LV), dominant right ventricle (RV) and small left-sided heart structures. The introduction of the Norwood operation has significantly improved outcomes, with 5-year survival reported up to 65 %. Despite these advances, post-operative morbidity and mortality remain high, and the long-term complications in adult survivors represent a challenge. The number of HLHS patients with Fontan circulation is expected to double in the next 20 years, leading to a growing population requiring specialized care from adult congenital heart disease (ACHD) teams.</div><div>This article reviews current management strategies for HLHS, outlines potential long-term complications, and highlights existing knowledge gaps. Specific considerations in this population include the assessment of the neo-aorta and aortic arch, and systemic RV dysfunction in the setting of a Fontan circulation. The proposed surveillance strategy emphasizes the need for vigilant monitoring and timely intervention to treat the complications unique to this population, ensuring better outcomes for HLHS patients reaching adulthood.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100555"},"PeriodicalIF":0.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163419","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Ahmanson/UCLA adult congenital heart disease center, at UCLA, los angeles, USA: The history of a leading ACHD institution and its vision for the future 位于美国洛杉矶加州大学洛杉矶分校的Ahmanson/UCLA成人先天性心脏病中心:一家领先的先天性心脏病机构的历史及其对未来的展望
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-03-01 Epub Date: 2024-12-11 DOI: 10.1016/j.ijcchd.2024.100556
Jamil Aboulhosn
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引用次数: 0
期刊
International journal of cardiology. Congenital heart disease
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