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Percutaneous implantation of self-expanding pulmonary valves: results from real-life experience of the Venus-P valve Registry of the Italian Society of Pediatric Cardiology (SICPED). 经皮自扩张肺动脉瓣植入术:来自意大利儿科心脏病学会(SICPED)静脉- p瓣膜注册中心的实际经验。
IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-07-16 eCollection Date: 2025-09-01 DOI: 10.1016/j.ijcchd.2025.100609
M Pilati, F A D'Aiello, M Chessa, G Santoro, A Donti, E Mariucci, C Marrone, G Annoni, G Gaio, M Rebonato, E Piccinelli, M Cheli, R Formigari, G Butera

Background: Self-expandable valves may have a significant impact on the feasibility of transcatheter treatments of RVOT dysfunction. Venus P valve is the first autoexpadable Valve that received CE mark in 2022. Here, we are reporting on a multicenter data collection about patients undergoing Venus P valve implantation in Italy.

Materials: Seven italian centers were included in the study. Data were collected from patients treated with a venus P valve between July 2022 and May 2024. Indication for percutaneous valve implantation were in accordance with published guidelines.

Results: During the study period, a total of 65 patients were considered eligible to percutaneous implantation of a Venus P valve. Median age at procedure was 35 years (IQR 12-63 years) median weight 63 Kg (IQR 33-103 Kg). Indication for PPVI was severe regurgitation in all subjects but one where ventricular pressure was increased because of stenosis on a homograft conduit and pulmonary arteries which needed previous bilateral stenting. Venus P valve was implanted successfully in 61/65(94 %) patients. Four procedural complications occurred(6 %): 1 artero-venous fistula, 1 pulmonary bleeding, 1 hemothorax and a pulmonary oedema. During follow up (median 13 months,1-20months), all patients experienced a significant improvement of functional class. Neither deaths or re-intervention occurred. CT Scan showed hypoattenuating leaflet thickness (HALT) in 4 patients and one of them experienced endocarditis 12 months after the procedure.

Conclusions: Our study shows that Venus P valve implantation is safe and results are stable during a short-to-medium term follow-up. Endocarditis and HALT are inducing some concern and larger studies are needed.

背景:自膨胀瓣膜可能对经导管治疗RVOT功能障碍的可行性有重要影响。Venus P阀是第一个在2022年获得CE标志的自动膨胀阀。在这里,我们报告了意大利接受金星P瓣植入术患者的多中心数据收集。材料:七个意大利中心纳入研究。数据收集于2022年7月至2024年5月期间接受金星P瓣治疗的患者。经皮瓣膜植入术的适应症符合已出版的指南。结果:在研究期间,共有65例患者被认为符合经皮植入金星P瓣的条件。手术时中位年龄为35岁(IQR为12-63岁),中位体重为63公斤(IQR为33-103公斤)。PPVI的适应症是严重的反流,除了一例由于同种移植物导管和肺动脉狭窄而导致心室压力增加,这需要先前的双侧支架植入。65例患者中有61例(94%)成功植入金星P瓣。手术并发症4例(6%):动静脉瘘1例,肺出血1例,胸血1例,肺水肿1例。在随访期间(中位13个月,1-20个月),所有患者的功能分级均有显著改善。没有发生死亡或再干预。CT扫描显示4例患者小叶厚度减薄(HALT),其中1例患者术后12个月出现心内膜炎。结论:我们的研究表明,在中短期随访中,金星P瓣植入术是安全的,结果稳定。心内膜炎和HALT引起了一些关注,需要更大规模的研究。
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引用次数: 0
Living with congenital heart disease: a qualitative study to explore psychosocial impacts in adulthood. 患有先天性心脏病:一项探讨成年期社会心理影响的定性研究。
IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-07-12 eCollection Date: 2025-09-01 DOI: 10.1016/j.ijcchd.2025.100610
Larissa Lloyd, Sasha Ruban, Tanya Badal, Geoff Strange, David S Celermajer, Carissa Bonner

Background: The survival prospects of adults with congenital heart disease have improved considerably due to improved surgical interventions, resulting in a growing population. This study aims to explore the experiences of adults with congenital heart disease, to identify support needs.

Methods: Participants were recruited from the National Australian Congenital Heart Disease Registry using purposive sampling to ensure diversity in terms of gender, rurality and disease complexity. Semi-structured interviews were conducted with adults with congenital heart disease. The interviews were recorded digitally and transcribed verbatim. Framework Analysis was used to ensure rigour in identifying themes.

Results: 43 interviews were completed (23 males and 20 females; age 20 to 54 years). Three main themes and seven subthemes were identified to explain varying experiences of living with congenital heart disease: I. Lifestyle factors; (i) physical activity, (ii) weight management. II. Practical issues; (iii) employment, (iv) financial stress, (v) education. III. Psychosocial impact; (vi) mental health and resilience, and (vii) social engagement. Participants across a range of disease complexities reported more impact on their lives when they felt inadequately supported by their healthcare providers to address these challenges, such as insufficient education on exercise leading to difficulty performing clinically recommended physical activity.

Conclusions: The lived experiences of adults living with congenital heart disease highlight specific areas needing support, which could inform future improvements in their "whole of life" care. This could include referral to allied health professionals, education across lifestyle and practical domains, and appropriate peer support networks.

背景:由于手术干预的改善,成人先天性心脏病患者的生存前景大大改善,导致患者数量不断增加。本研究旨在探讨成人先天性心脏病患者的经历,以确定支持需求。方法:参与者从澳大利亚国家先天性心脏病登记处招募,采用有目的抽样,以确保性别、农村性和疾病复杂性方面的多样性。对患有先天性心脏病的成年人进行了半结构化访谈。采访以数字方式记录下来,并逐字抄录。采用框架分析来确保确定主题的严谨性。结果:共完成访谈43例(男23例,女20例,年龄20 ~ 54岁)。确定了三个主要主题和七个次要主题来解释患有先天性心脏病的不同经历:1 .生活方式因素;(i)身体活动;(ii)体重管理。2。实际问题;(iii)就业,(iv)经济压力,(v)教育。3。社会心理的影响;(六)心理健康和复原力;(七)社会参与。各种疾病复杂程度的参与者报告说,当他们感到没有得到医疗保健提供者的充分支持来应对这些挑战时,他们的生活受到更大的影响,例如运动教育不足导致难以进行临床推荐的体育活动。结论:成人先天性心脏病患者的生活经历突出了需要支持的特定领域,这可以为未来改善他们的“终身”护理提供信息。这可能包括转诊到联合卫生专业人员,跨生活方式和实践领域的教育,以及适当的同伴支持网络。
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引用次数: 0
Correlation between Olink and SomaScan proteomics platforms in adults with a Fontan circulation 成人Fontan循环患者中Olink和SomaScan蛋白组学平台的相关性
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-06-01 Epub Date: 2025-04-15 DOI: 10.1016/j.ijcchd.2025.100584
Ismael Z. Assi , Michael J. Landzberg , Kristian C. Becker , David Renaud , Fernando Baraona Reyes , David M. Leone , Mark Benson , Miriam Michel , Robert E. Gerszten , Alexander R. Opotowsky

Background

High-throughput proteomics platforms using aptamers (SomaScan) or proximity extension assay (Olink) provide novel opportunities for improving diagnostic and risk stratification tools in cardiovascular diseases, including understudied congenital heart diseases. The correlation between these proteomics approaches has not yet been studied among individuals with a Fontan circulation.

Objective

The correlation of plasma protein measurements between SomaScan and Olink platforms was evaluated in adults with a Fontan circulation.

Methods

We measured 491 proteins in plasma of 71 adults with a Fontan circulation using Olink and SomaScan. Missing Olink measurements (0.13%, 47/34,861) were imputed using non-parametric imputation. Spearman's rank correlation coefficient for absolute values of protein expression between platforms was calculated. Protein correlation frequencies were compared to 3 cohorts reported in the literature using Pearson's Chi-squared test of independence.

Results

Overall, protein correlations between Olink and SomaScan measurements were moderately strong for most proteins, (rho > 0.4 for 57.2%), but with substantial variability (median correlation = 0.457, IQR = 0.538). The distribution of protein correlations was qualitatively similar to published literature in non-Fontan cohorts. Both Olink and SomaScan identified proteins with sex-based differences; both identified differences in myostatin and leptin, but each identified additional nonoverlapping sexually dimorphic proteins (n = 14 Olink, n = 5 SomaScan).

Conclusions

In adults with a Fontan circulation, correlations between plasma proteins measured by Olink and SomaScan varied widely, approximately in line with prior reports in other populations. While these tools may be uniquely useful to generate hypotheses, specifically regarding potential molecular mechanisms, more definitive inference requires independent validation.
使用适体(SomaScan)或邻近扩展测定(Olink)的高通量蛋白质组学平台为改善心血管疾病(包括未充分研究的先天性心脏病)的诊断和风险分层工具提供了新的机会。这些蛋白质组学方法之间的相关性尚未在Fontan循环个体中进行研究。目的评价成人Fontan循环患者血浆蛋白水平与SomaScan和Olink平台的相关性。方法应用Olink和SomaScan检测了71例Fontan循环成人血浆中的491种蛋白。缺失的Olink测量值(0.13%,47/34,861)使用非参数估算。计算平台间蛋白表达绝对值的Spearman秩相关系数。使用Pearson卡方独立性检验将蛋白相关频率与文献中报道的3个队列进行比较。结果总的来说,对于大多数蛋白质,Olink和SomaScan测量之间的蛋白质相关性中等强,(rho >;0.4为57.2%),但存在显著的可变性(中位相关= 0.457,IQR = 0.538)。在非fontan队列中,蛋白质相关性的分布在质量上与已发表的文献相似。Olink和SomaScan都鉴定出了基于性别差异的蛋白质;两者都发现了肌肉生长抑制素和瘦素的差异,但都发现了额外的非重叠性二态蛋白(n = 14 Olink, n = 5 SomaScan)。结论:在Fontan循环的成年人中,Olink和SomaScan测定的血浆蛋白之间的相关性差异很大,与先前在其他人群中的报道大致一致。虽然这些工具可能是唯一有用的产生假设,特别是关于潜在的分子机制,更明确的推断需要独立的验证。
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引用次数: 0
Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies ahead 单心室生理学和Fontan循环中的内皮功能障碍-未来的前景
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-06-01 Epub Date: 2025-04-28 DOI: 10.1016/j.ijcchd.2025.100589
Raksheeth Agarwal , Louise E. Coats , Ali N. Zaidi
Endothelial dysfunction is characterized by a vasoconstricted, pro-coagulative, and pro-inflammatory phenotype and is known to play a role in several chronic non-communicable diseases. Several biophysical and biochemical markers have been developed to assess endothelial function clinically. Its relevance in individuals born with single-ventricle congenital heart disease (SV-CHD) is increasingly recognized. Endothelial dysfunction has been observed in all stages of palliation in SV-CHD patients. Several mechanisms possibly contribute, including genetic factors, hypoxia, loss of pulsatility of blood flow, neurohormonal and sympathetic overactivation, and oxidative stress. Clinically, it possibly contributes to impaired pulmonary flow, exercise limitation, thromboembolisms, liver dysfunction, and adverse pregnancy outcomes. Based on this information, several therapeutic targets have been proposed such as early surgical and exercise interventions, pulmonary vasodilators, and other common pharmacological agents. However, much remains unknown and future studies must unravel the relationship of endothelial dysfunction with this complex patient group, ultimately improving their clinical care.
内皮功能障碍的特点是血管收缩、促凝和促炎表型,已知在几种慢性非传染性疾病中发挥作用。一些生物物理和生化标志物已经被开发出来用于临床评估内皮功能。它与出生时患有单心室先天性心脏病(SV-CHD)的个体的相关性越来越被认识到。在SV-CHD患者的所有缓解阶段都观察到内皮功能障碍。可能有几种机制起作用,包括遗传因素、缺氧、血流脉搏丧失、神经激素和交感神经过度激活以及氧化应激。在临床上,它可能导致肺血流受损、运动受限、血栓栓塞、肝功能障碍和不良妊娠结局。基于这些信息,提出了几种治疗靶点,如早期手术和运动干预、肺血管扩张剂和其他常用药物。然而,仍有许多未知的,未来的研究必须揭示内皮功能障碍与这一复杂患者群体的关系,最终改善他们的临床护理。
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引用次数: 0
Feasibility and effectiveness of telemedicine for adult patients with congenital heart disease: A one-year single-center experience-based study 成人先天性心脏病远程医疗的可行性和有效性:一项为期一年的单中心经验研究
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-06-01 Epub Date: 2025-04-07 DOI: 10.1016/j.ijcchd.2025.100582
Nunzia Borrelli , Nicola Grimaldi , Flavia Fusco , Antonio Orlando , Michela Palma , Maria Cristina Boccia , Sabrina Bassolino , Anna Iervolino , Berardo Sarubbi

Introduction

The number of adults with congenital heart disease has significantly increased in recent years. While telemedicine has emerged as a promising approach to improve care delivery and patient outcomes, its use for adults with congenital cardiopathies has not been extensively explored. This study aims to evaluate the feasibility and clinical impact of a telemedicine programme for adults with congenital heart diseases.

Methods

This was a single-center, prospective, observational study, carried out between January 2022 and January 2023. A wristwatch, a paired mobile device, and a dedicated hospital workstation were used to telemonitor 25 adult patients with moderate-to-severe congenital heart condition, in II-IV New-York Heart Association class. We assessed changes in hospital admission days and related costs, patient satisfaction, and functional parameters, including 6-min walk test distance, vital signs, and echocardiographic ejection fraction.

Results

All 25 patients agreed to participate to the telemedicine program (mean age 38.35 ± 11.33 years, 52 % male). Twenty-three patients completed the program. No significant changes were observed in vital signs, functional class, ventricular ejection fraction, while walking distance significantly improved (404 ± 82 m versus 433 ± 142 m, p = 0.03). Notably, hospital admission days and related costs were significantly reduced during the TM program year compared to the prior year. Patient satisfaction was high. One patient experienced a prolonged hospitalization and death due to the natural progression of their condition.

Conclusion

High-risk patients with moderate-severe congenital heart disease can benefit from a device-implemented telemedicine program, which offers tailored, specialized care directly at home, reducing hospital admissions and ensuring stable clinical status.
近年来,患有先天性心脏病的成年人数量明显增加。虽然远程医疗已经成为一种有希望改善护理服务和患者预后的方法,但其在成人先天性心脏病患者中的应用尚未得到广泛探索。本研究旨在评估成人先天性心脏病远程医疗方案的可行性和临床影响。方法:这是一项单中心、前瞻性、观察性研究,于2022年1月至2023年1月进行。在纽约心脏协会II-IV级,使用腕表、配对移动设备和专用医院工作站对25例中度至重度先天性心脏病成年患者进行远程监测。我们评估了住院天数和相关费用、患者满意度和功能参数的变化,包括6分钟步行测试距离、生命体征和超声心动图射血分数。结果25例患者均同意参加远程医疗项目,平均年龄38.35±11.33岁,男性占52%。23名患者完成了该项目。生命体征、功能分级、心室射血分数无明显变化,步行距离明显改善(404±82 m vs 433±142 m, p = 0.03)。值得注意的是,与前一年相比,住院天数和相关费用在TM计划年度显着减少。患者满意度高。一名患者因病情的自然进展而长期住院并死亡。结论中重度先天性心脏病高危患者可从设备实施的远程医疗项目中获益,该项目可直接在家中提供量身定制的专科护理,减少住院率,确保临床状态稳定。
{"title":"Feasibility and effectiveness of telemedicine for adult patients with congenital heart disease: A one-year single-center experience-based study","authors":"Nunzia Borrelli ,&nbsp;Nicola Grimaldi ,&nbsp;Flavia Fusco ,&nbsp;Antonio Orlando ,&nbsp;Michela Palma ,&nbsp;Maria Cristina Boccia ,&nbsp;Sabrina Bassolino ,&nbsp;Anna Iervolino ,&nbsp;Berardo Sarubbi","doi":"10.1016/j.ijcchd.2025.100582","DOIUrl":"10.1016/j.ijcchd.2025.100582","url":null,"abstract":"<div><h3>Introduction</h3><div>The number of adults with congenital heart disease has significantly increased in recent years. While telemedicine has emerged as a promising approach to improve care delivery and patient outcomes, its use for adults with congenital cardiopathies has not been extensively explored. This study aims to evaluate the feasibility and clinical impact of a telemedicine programme for adults with congenital heart diseases.</div></div><div><h3>Methods</h3><div>This was a single-center, prospective, observational study, carried out between January 2022 and January 2023. A wristwatch, a paired mobile device, and a dedicated hospital workstation were used to telemonitor 25 adult patients with moderate-to-severe congenital heart condition, in II-IV New-York Heart Association class. We assessed changes in hospital admission days and related costs, patient satisfaction, and functional parameters, including 6-min walk test distance, vital signs, and echocardiographic ejection fraction.</div></div><div><h3>Results</h3><div>All 25 patients agreed to participate to the telemedicine program (mean age 38.35 ± 11.33 years, 52 % male). Twenty-three patients completed the program. No significant changes were observed in vital signs, functional class, ventricular ejection fraction, while walking distance significantly improved (404 ± 82 m versus 433 ± 142 m, p = 0.03). Notably, hospital admission days and related costs were significantly reduced during the TM program year compared to the prior year. Patient satisfaction was high. One patient experienced a prolonged hospitalization and death due to the natural progression of their condition.</div></div><div><h3>Conclusion</h3><div>High-risk patients with moderate-severe congenital heart disease can benefit from a device-implemented telemedicine program, which offers tailored, specialized care directly at home, reducing hospital admissions and ensuring stable clinical status.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100582"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143935175","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
Percutaneous repair of biatrial drainage of the right superior vena cava using a covered stent 有盖支架经皮修复右上腔静脉双房引流
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-06-01 Epub Date: 2025-04-26 DOI: 10.1016/j.ijcchd.2025.100587
Samuel L. Casella , Sangeeta Shah
Biatrial drainage of the right superior vena cava (RSVC) is a rare anomaly of the cavo-pulmonary venous confluence which may lead to chronic cyanosis, stroke and cerebral abscess. Historical corrections have been limited to open surgical repair. We describe an adult patient with biatrial drainage of the RSVC with chronic cyanosis who underwent successful percutaneous correction using a covered stent.
右上腔静脉双房引流是一种罕见的腔静脉-肺静脉汇合处异常,可导致慢性紫绀、中风和脑脓肿。历史上的纠正仅限于开放性手术修复。我们描述了一个成人患者双心房引流的RSVC慢性发绀谁接受成功的经皮矫正使用覆盖支架。
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引用次数: 0
Organization and structure of intensive care management of adult congenital heart disease in the United States 美国成人先天性心脏病重症监护管理的组织和结构
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-06-01 Epub Date: 2024-03-29 DOI: 10.1016/j.ijcchd.2024.100509
Alex J. Katz , Myke Federman , Jeremy P. Moore , Jeannette P. Lin , Kuan-Ting Chen , Nicholas J. Jackson , Jamil Aboulhosn

Background

The growing number of adults with congenital heart disease has led to the formation of dedicated centers to provide specialized care. There is a paucity of published data on the organization, staffing, and resources available when adult congenital heart disease (ACHD) patients are hospitalized in intensive care settings. This study aims to describe the delivery of intensive care for ACHD patients in the United States and identify key differences between accredited centers or those pending accreditation and non-accredited Adult Congenital Heart Association (ACHA) centers.

Methods

A web-based cross-sectional survey targeting centers with specialized ACHD programs was conducted.

Results

Data were obtained from 48 of 96 centers (50%). Twenty-seven (56%) were accredited by the ACHA and 8 (17%) were pending accreditation. Surgical ACHD patients were primarily co-managed by cardiologists and intensivists (n = 11, 23%) and treated within an adult cardiac surgical intensive care unit (n = 34, 72%). Compared to non-accredited centers, ACHA accredited centers or those pending accreditation were more likely to offer daily rounds with ACHD providers (30, 86% vs. 6, 46%, p=<0.01), were more likely to support an ACHD fellowship training program (16, 46% vs. 0, 0%, p=<0.01); and were more likely to have durable mechanical support, such as ventricular assist devices (35, 100% vs. 8, 62%, p < 0.01).

Conclusion

This survey reports current trends in organization, staffing, and resources available to ACHD patients requiring intensive care. ACHA accredited centers or those pending accreditation were more likely to have dedicated ACHD rounds, fellowship training programs, and access to durable mechanical support.
越来越多的成人先天性心脏病患者已经形成了专门的中心来提供专门的护理。关于成人先天性心脏病(ACHD)患者在重症监护机构住院治疗时的组织、人员配备和可用资源的公开数据很少。本研究旨在描述美国ACHD患者重症监护的交付情况,并确定认证中心或待认证中心与未认证的成人先天性心脏协会(ACHA)中心之间的主要差异。方法采用基于网络的横断面调查方法,针对有专门ACHD项目的中心进行调查。结果96个中心中48个(50%)获得数据。27家(56%)获得了ACHA的认证,8家(17%)正在等待认证。外科ACHD患者主要由心脏病专家和重症监护医师共同管理(n = 11,23%),并在成人心脏外科重症监护病房接受治疗(n = 34,72%)。与未认证的中心相比,获得ACHA认证的中心或待认证的中心更有可能提供ACHD提供者的每日查房(30.86%对6.46%,p=<0.01),更有可能支持ACHD奖学金培训计划(16.46%对0,0%,p=<0.01);并且更有可能使用耐用的机械支持,如心室辅助装置(35.100% vs. 8.62%, p <;0.01)。结论:本调查报告了目前需要重症监护的ACHD患者的组织、人员配备和可用资源的趋势。获得ACHA认证的中心或那些即将获得认证的中心更有可能有专门的ACHD轮次、奖学金培训计划和持久的机械支持。
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引用次数: 0
Thrombus formation after the Norwood procedure: Incidence, risk factors, and its impact on late outcomes 诺伍德手术后血栓形成:发生率、危险因素及其对晚期预后的影响
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-06-01 Epub Date: 2025-02-15 DOI: 10.1016/j.ijcchd.2025.100575
Alessandra Poppe , Muneaki Matsubara , Jonas Palm , Thibault Schaeffer , Takuya Osawa , Carolin Niedermaier , Paul Philipp Heinisch , Nicole Piber , Bettina Ruf , Alfred Hager , Peter Ewert , Jürgen Hörer , Masamichi Ono

Objective

Thrombus formation is a feared complication after congenital heart surgery. We aimed to clarify the clinical characteristics associated with thrombus formation after the Norwood procedure.

Methods

All neonates who underwent the Norwood procedure between 2001 and 2022 were reviewed. The incidence and location of thrombus were evaluated. Risk factors for thrombus formation and its impact on survival were analyzed.

Results

Among 360 patients who were included, thrombus formation was detected in 42 patients (11.7 %) during the postoperative in-hospital period, with a median of 12 (range: 5–30) postoperative days. The most common site of thrombus was the superior vena cava in 9 (2.5 %) patients, followed by the right atrium in 8 (2.2 %). Patients who received a right ventricle to pulmonary artery conduit had a higher incidence of thrombus than those who received a modified Blalock-Taussig-Thomas shunt (16.4 vs. 7.7 %, p = 0.011). Patients with thrombus formation had a longer stay in the intensive care unit (ICU), than those without (median 21 vs. 13 days, p = 0.018). Survival after ICU discharge was lower in patients with thrombus than those without (57, 54, and 54 % vs 73, 71, and 70 % at 2, 4, and 6 years, respectively; p = 0.032). Restrictive atrial septal defect was identified as an independent risk for thrombus (odds ratio: 2.61; p = 0.005).

Conclusions

Thrombus formation was observed in 12 % of the patients during the hospital stay after the Norwood procedure and was associated with prolonged recovery and high mortality. A restrictive atrial septal defect was identified as a risk factor for thrombus formation.
目的:血栓形成是先天性心脏手术后令人担忧的并发症。我们的目的是阐明诺伍德手术后与血栓形成相关的临床特征。方法回顾性分析2001 ~ 2022年接受诺伍德手术的新生儿。评估血栓的发生率和位置。分析血栓形成的危险因素及其对生存的影响。结果纳入的360例患者中,42例(11.7%)患者在术后住院期间检测到血栓形成,中位数为12(范围:5-30)天。血栓最常见的部位为上腔静脉(2.5%),其次为右心房(2.2%)。接受右心室至肺动脉导管的患者血栓发生率高于接受改良Blalock-Taussig-Thomas分流术的患者(16.4% vs. 7.7%, p = 0.011)。血栓形成的患者在重症监护病房(ICU)的住院时间比没有血栓形成的患者更长(中位21天vs. 13天,p = 0.018)。在ICU出院后,血栓患者的生存率低于无血栓患者(分别为57、54和54% vs 73、71和70%,分别为2年、4年和6年);p = 0.032)。限制性房间隔缺损被认为是血栓形成的独立危险因素(优势比:2.61;p = 0.005)。结论12%的患者在诺伍德手术后住院期间观察到血栓形成,并伴有较长时间的恢复和高死亡率。限制性房间隔缺损被认为是血栓形成的危险因素。
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引用次数: 0
Pulmonary hypertension aetiologies in different parts of the world 世界不同地区肺动脉高压的病因
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-06-01 Epub Date: 2025-04-15 DOI: 10.1016/j.ijcchd.2025.100586
Ghazwan Butrous
Pulmonary hypertension is a serious condition characterised by elevated blood pressure in the pulmonary arteries, caused by various aetiologies and via different pathological processes. Over the past seventy years, our understanding and management of this disorder have greatly improved, resulting in increased diagnosis and effective clinical management. Current epidemiological estimates are challenged by the increased awareness of this condition and the changing definitions and classification systems. The Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) has also shown temporal changes in the epidemiology of pulmonary hypertension over the last thirty years, contributing to regional variations in prevalence and incidence.
This review explores the complexities of global and regional variations in different types of pulmonary hypertension reported through many registries, databases and regional studies. Although these tools can help estimate prevalence and incidences, they may also underestimate the actual number of cases due to the continuously changing understanding of the condition and increase awareness globally. Therefore, continued research, international collaboration, and standardised data collection are essential for achieving a more accurate global view of pulmonary hypertension and developing effective management strategies for this serious condition that significantly impacts general health.
肺动脉高压是一种以肺动脉血压升高为特征的严重疾病,由多种病因和不同的病理过程引起。在过去的七十年里,我们对这种疾病的理解和管理有了很大的提高,导致诊断和有效的临床管理增加。目前的流行病学估计受到对该病认识提高以及定义和分类系统变化的挑战。全球疾病、损伤和风险因素负担研究(GBD)也显示了肺动脉高压流行病学在过去三十年中的时间变化,这导致了患病率和发病率的区域差异。本综述探讨了通过许多登记、数据库和区域研究报告的不同类型肺动脉高压的全球和区域差异的复杂性。虽然这些工具可以帮助估计患病率和发病率,但由于对该病的认识不断变化和全球认识的提高,它们也可能低估了实际病例数。因此,持续的研究、国际合作和标准化的数据收集对于实现更准确的肺动脉高压全球视图和制定有效的管理策略至关重要,这一严重疾病严重影响一般健康。
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引用次数: 0
Excellent medium to long term outcomes after cardiac surgery for moderate and complex congenital heart disease, regardless of geographic location 不论地理位置如何,中度和复杂先天性心脏病心脏手术后中长期预后良好
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-06-01 Epub Date: 2025-03-25 DOI: 10.1016/j.ijcchd.2025.100579
Larissa Lloyd , Calum Nicholson , Geoff Strange , Rachael Cordina , David S. Celermajer , Michael M.H. Cheung

Objective

To compare the outcomes for repaired tetralogy of Fallot and Fontan patients who must travel from regional Victoria and interstate, in order to receive specialist congenital heart disease (CHD) surgery and ongoing care, with those of local patients.

Methods

This retrospective study included 332 patients who underwent tetralogy of Fallot (ToF) repair and 159 patients who underwent a Fontan procedure at Royal Children's Hospital (RCH) Melbourne between 2003 and 2017. Data was obtained from the National CHD Registry, linked with National Death Index data, and follow-up data from the Australian and New Zealand Fontan Registry.

Results

Equivalent outcomes were observed between location groups in both cohorts for all of the main outcomes of interest. Repaired ToF subjects were aged 0.76 years (IQR 0.52–3.33) at operation and 10.2 years (IQR 5.46–14.9) at last follow-up, whilst Fontan subjects were aged 4.94 (IQR 4.27–5.66) years at operation and 14.2 years (IQR 11.3–16.4) at last follow-up. Mortality rates were extremely low and did not significantly differ between geographic groups, with 10-year survival in the repaired ToF cohort 98.0 % in the City group, 98.1 % in the Regional group, and 98.8 % in the Interstate group; and 97.8 %, 92.3 %, and 97.5 % in the Fontan cohort, respectively.

Conclusions

In the Australian setting and with adequate planning and local follow-up options, patients travelling from regional areas or interstate for their CHD operations have similar outcomes, out to 21 years, compared to patients living locally.
目的比较法洛四联症和方丹四联症患者与当地患者的预后,这些患者必须从维多利亚地区和州际公路出发,才能接受专科先天性心脏病(CHD)手术和持续护理。方法本回顾性研究包括2003年至2017年在墨尔本皇家儿童医院(RCH)接受法洛四联症(ToF)修复的332例患者和接受Fontan手术的159例患者。数据来自国家冠心病登记处,与国家死亡指数数据相关联,并来自澳大利亚和新西兰Fontan登记处的随访数据。结果在两个队列的位置组之间,所有感兴趣的主要结果都是相同的。修复ToF患者手术时年龄为0.76岁(IQR 0.52-3.33),末次随访时年龄为10.2岁(IQR 5.46-14.9),而Fontan患者手术时年龄为4.94岁(IQR 4.27-5.66),末次随访时年龄为14.2岁(IQR 11.3-16.4)。死亡率极低,地理组间无显著差异,城市组修复ToF组的10年生存率为98.0%,地区组为98.1%,州际组为98.8%;Fontan组分别为97.8%、92.3%和97.5%。结论:在澳大利亚,通过适当的计划和当地随访选择,从地区或州际旅行的冠心病患者与居住在当地的患者相比,在21年内的预后相似。
{"title":"Excellent medium to long term outcomes after cardiac surgery for moderate and complex congenital heart disease, regardless of geographic location","authors":"Larissa Lloyd ,&nbsp;Calum Nicholson ,&nbsp;Geoff Strange ,&nbsp;Rachael Cordina ,&nbsp;David S. Celermajer ,&nbsp;Michael M.H. Cheung","doi":"10.1016/j.ijcchd.2025.100579","DOIUrl":"10.1016/j.ijcchd.2025.100579","url":null,"abstract":"<div><h3>Objective</h3><div>To compare the outcomes for repaired tetralogy of Fallot and Fontan patients who must travel from regional Victoria and interstate, in order to receive specialist congenital heart disease (CHD) surgery and ongoing care, with those of local patients.</div></div><div><h3>Methods</h3><div>This retrospective study included 332 patients who underwent tetralogy of Fallot (ToF) repair and 159 patients who underwent a Fontan procedure at Royal Children's Hospital (RCH) Melbourne between 2003 and 2017. Data was obtained from the National CHD Registry, linked with National Death Index data, and follow-up data from the Australian and New Zealand Fontan Registry.</div></div><div><h3>Results</h3><div>Equivalent outcomes were observed between location groups in both cohorts for all of the main outcomes of interest. Repaired ToF subjects were aged 0.76 years (IQR 0.52–3.33) at operation and 10.2 years (IQR 5.46–14.9) at last follow-up, whilst Fontan subjects were aged 4.94 (IQR 4.27–5.66) years at operation and 14.2 years (IQR 11.3–16.4) at last follow-up. Mortality rates were extremely low and did not significantly differ between geographic groups, with 10-year survival in the repaired ToF cohort 98.0 % in the City group, 98.1 % in the Regional group, and 98.8 % in the Interstate group; and 97.8 %, 92.3 %, and 97.5 % in the Fontan cohort, respectively.</div></div><div><h3>Conclusions</h3><div>In the Australian setting and with adequate planning and local follow-up options, patients travelling from regional areas or interstate for their CHD operations have similar outcomes, out to 21 years, compared to patients living locally.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"20 ","pages":"Article 100579"},"PeriodicalIF":0.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143785302","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}
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期刊
International journal of cardiology. Congenital heart disease
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