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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 Pub 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
GORE® CARDIOFORM ASD occluder thrombus in a patient of rheumatoid arthritis occurred three days after deployment GORE®CARDIOFORM ASD闭塞血栓在类风湿关节炎患者部署后3天发生
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-06-16 DOI: 10.1016/j.ijcchd.2025.100600
Naomi Nakagawa, Yusuke Shigemitsu, Kengo Okamoto
A 67-year-old female patient with rheumatoid arthritis that was well suppressed by medications except for steroids had catheter closure of the secundum atrial septal defect (ASD). After the administration of aspirin and intravenous heparin, a 32-mm of GORE® CARDIOFORM ASD occluder was successfully deployed. On postprocedural day 3, transthoracic echocardiography showed mobile echogenic mass of 9 mm on the right side of the device. Intravenous heparin 10 IU/kg/hr was administrated continuously, and clopidogrel was added. On the following day, the mobile mass was not visible. Even though the occurrence rate of device thrombus is not high, device thrombosis can become a major complication of device closure of ASD because it may cause systemic and/or pulmonary embolism. Administration of dual antiplatelet therapy or other anticoagulant therapy should be considered if the patients have a higher risk of thrombosis.
67岁女性类风湿关节炎患者,除类固醇外药物抑制良好,导管关闭第二房间隔缺损(ASD)。在给予阿司匹林和静脉肝素后,一个32毫米的GORE®CARDIOFORM ASD闭塞器成功部署。术后第3天,经胸超声心动图显示装置右侧有9mm的可移动回声肿块。持续静脉滴注肝素10 IU/kg/hr,同时加用氯吡格雷。第二天,移动的团块就看不见了。尽管器械血栓的发生率不高,但器械血栓形成可成为ASD器械关闭的主要并发症,因为它可能导致全身和/或肺栓塞。如果患者有较高的血栓形成风险,应考虑双重抗血小板治疗或其他抗凝治疗。
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引用次数: 0
Clinical outcomes after implantation of bioprosthetic valves in adults with congenital heart disease 成人先天性心脏病生物瓣膜置入术的临床效果
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-06-03 DOI: 10.1016/j.ijcchd.2025.100598
Andrea R. Hsu , Snigdha Karnakoti , Ahmed T. Abdelhalim , Jason H. Anderson , C. Charles Jain , William R. Miranda , Elizabeth H. Stephens , Joseph A. Dearani , Hartzell V. Schaff , Heidi M. Connolly , Alexander C. Egbe

Background

Bioprosthetic valves are frequently implanted in adults with congenital heart disease (CHD), but comprehensive data on bioprosthetic valve longevity and outcomes across all valve positions are lacking. The purpose of this study was to determine the incidence and risk factors for bioprosthetic valve dysfunction (BVD) across all valve positions in adults with CHD.

Method

BVD was defined as Doppler mean gradient >40 mmHg and/or severe prosthetic/periprosthetic regurgitation for aortic or pulmonary bioprostheses, or Doppler mean gradient >10 mmHg and/or severe prosthetic/periprosthetic regurgitation for mitral or tricuspid bioprostheses.

Results

There were 1221 adults with CHD that underwent implantation of a bioprosthetic valve (age 39 ± 16 years, males 617 [51 %]). At the time of valve implantation, 755 (62 %), 325 (27 %), 176 (14 %), and 21 (1.7 %) received a bioprosthetic valve in the pulmonary, tricuspid, aortic, and mitral positions, respectively. The 10-year cumulative incidence of BVD was 28 %, 36 %, 43 %, and 68 % for pulmonary, tricuspid, aortic, and mitral bioprosthetic valves, respectively (p < 0.001). Using the pulmonary valve position as the reference group, valve position (tricuspid [HR 1.26, 95 % CI 1.09–1.58), aortic [HR 1.64, 95 % CI 1.22–1.97], mitral [HR 2.01, 95 % CI 0.83–4.05]) were associated with higher risk of BVD. Younger age was associated higher risk of BVD (HR 0.95, 95 % CI 0.86–0.98 per 5-year increment).

Conclusions

These data support the existing evidence that left-sided bioprosthetic valves demonstrate reduced durability in young patients and should be included in counseling and clinical decision-making.
成人先天性心脏病(CHD)患者经常植入生物瓣膜,但缺乏关于所有瓣膜位置的生物瓣膜寿命和预后的综合数据。本研究的目的是确定成人冠心病患者所有瓣膜位置生物瓣膜功能障碍(BVD)的发生率和危险因素。方法将dbvd定义为主动脉或肺生物假体的多普勒平均梯度为40 mmHg和/或严重假体/假体周围反流,二尖瓣或三尖瓣生物假体的多普勒平均梯度为10 mmHg和/或严重假体/假体周围反流。结果1221例成人冠心病患者行生物瓣膜植入术(年龄39±16岁,男性617例[51%])。在瓣膜植入时,分别有755例(62%)、325例(27%)、176例(14%)和21例(1.7%)在肺动脉、三尖瓣、主动脉和二尖瓣位置植入了生物瓣膜。肺动脉瓣、三尖瓣、主动脉瓣和二尖瓣生物瓣膜10年累计BVD发生率分别为28%、36%、43%和68% (p <;0.001)。以肺动脉瓣位置为参照组,瓣膜位置(三尖瓣[HR 1.26, 95% CI 1.09-1.58]、主动脉瓣[HR 1.64, 95% CI 1.22-1.97]、二尖瓣[HR 2.01, 95% CI 0.83-4.05])与BVD的高风险相关。年龄越小,BVD风险越高(每5年增加危险度0.95,95% CI 0.86-0.98)。结论这些数据支持了现有的证据,即左侧生物假体瓣膜在年轻患者中表现出耐久性降低,应纳入咨询和临床决策。
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引用次数: 0
Utilizing optical coherence tomography to explore pulmonary arteries and pulmonary vascular disease in Fontan patients 利用光学相干断层扫描探查方丹患者肺动脉和肺血管疾病
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-06-03 DOI: 10.1016/j.ijcchd.2025.100599
François Côté , Leonaes Brahel Tatchinda Kuete , Réda Ibrahim , Jean-Claude Tardif , Colombe Roy , François-Pierre Mongeon , Annie Dore , Blandine Mondésert , Paul Khairy , Marie-A. Chaix

Background

The Fontan procedure is the palliative intervention of choice for patients with single ventricle physiology. Pulmonary vascular disease (PVD) is a major contributor to Fontan circulatory failure. Our study utilized optical coherence tomography (OCT) imagery in pulmonary arteries to better characterize PVD in Fontan patients.

Methods

In a prospective pilot study, OCT images were acquired during cardiac catheterization in a distal left pulmonary artery (PA) branch (A10). The PA intima-media ratio and wall-vessel ratio (i.e., difference between the external elastic membrane area and the luminal area divided by the external elastic membrane area) were measured off-line.

Results

A total of 9 Fontan patients (5 women), median age of 23.0 (21.0,38.0) years, were included. All patients exhibited an intima-to-media ratio above 1.0. The ratio exceeded 2.8 in patients with high pulmonary vascular resistance. No difference is observed between patients for the wall-vessel ratio. No relation between time of completion of Fontan and intima-media ratio is identified.

Conclusion

OCT shows promise in evaluating PVD in patients with Fontan palliation. PA remodeling is characterized by intimal hyperplasia with medial regression. Further studies are required to determine the role of OCT in guiding treatment decisions and assessing therapeutic responses.
背景Fontan手术是单心室生理患者的姑息性干预选择。肺血管疾病(PVD)是方坦循环衰竭的主要原因。我们的研究利用光学相干断层扫描(OCT)成像的肺动脉更好地表征PVD在Fontan患者。方法在一项前瞻性先导研究中,在左肺动脉远端(PA)分支(A10)心导管插入术期间获得OCT图像。离线测量PA内膜-中膜比和血管壁比(即外弹性膜面积与管腔面积之差除以外弹性膜面积)。结果共纳入Fontan患者9例(女性5例),中位年龄23.0(21.0,38.0)岁。所有患者的内膜中膜比均大于1.0。在肺血管阻力高的患者中,该比值超过2.8。两组患者的血管壁比无差异。Fontan的完成时间与内膜-中膜比例没有关系。结论oct在评估Fontan姑息治疗患者PVD方面有较好的应用前景。PA重塑的特征是内膜增生伴内侧退行。需要进一步的研究来确定OCT在指导治疗决策和评估治疗反应中的作用。
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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 Pub 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 ,&nbsp;Calum Nicholson ,&nbsp;Julian Ayer ,&nbsp;Michael Cheung ,&nbsp;Leeanne Grigg ,&nbsp;David S. Celermajer ,&nbsp;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":"2025-05-30","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
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 Pub 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。
{"title":"A novel approach to determining pulmonary valve replacement timing in repaired tetralogy of Fallot patients","authors":"Laura Delaney ,&nbsp;Andrew Browne ,&nbsp;Gruschen R. Veldtman ,&nbsp;Sihong Huang","doi":"10.1016/j.ijcchd.2025.100597","DOIUrl":"10.1016/j.ijcchd.2025.100597","url":null,"abstract":"<div><h3>Background</h3><div>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.</div></div><div><h3>Methods</h3><div>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.</div></div><div><h3>Results</h3><div>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.</div></div><div><h3>Conclusions</h3><div>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.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100597"},"PeriodicalIF":1.2,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144810503","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
Pulmonary arterial hypertension treatment. A new era 肺动脉高压治疗。新时代
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-05-27 DOI: 10.1016/j.ijcchd.2025.100594
Sophia Anastasia Mouratoglou , Alexandra Arvanitaki , George Papadopoulos , Rogerio Souza , George Giannakoulas
Pulmonary arterial hypertension (PAH) is a progressive and life-threatening condition characterized by elevated pulmonary vascular resistance, ultimately leading to right heart failure and death. Advances in the understanding of PAH pathophysiology have driven the development of targeted therapies that improve functional capacity, quality of life and survival. The current treatment paradigm emphasizes early and aggressive therapy, with combination therapy now considered standard of care, offering superior outcomes compared to monotherapy. Herewith, we examine the underlying pathophysiological mechanisms of PAH and explore the modes of action and clinical efficacy of major therapeutic classes, providing a comprehensive overview of contemporary treatment strategies.
肺动脉高压(PAH)是一种进行性和危及生命的疾病,其特征是肺血管阻力升高,最终导致右心衰和死亡。对多环芳烃病理生理学的理解的进步推动了靶向治疗的发展,这些治疗可以改善功能能力、生活质量和生存率。目前的治疗模式强调早期和积极的治疗,与单一治疗相比,联合治疗现在被认为是标准治疗,提供了更好的结果。在此,我们研究了多环芳烃的潜在病理生理机制,并探讨了主要治疗类别的作用模式和临床疗效,提供了当代治疗策略的全面概述。
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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 Pub 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。
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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 Pub 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
Overcoming borders: International cooperation in re-use and re-interpretation of omics data in Fontan circulation 跨越国界:方坛流通中组学数据再利用与再解释的国际合作
IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2025-05-05 DOI: 10.1016/j.ijcchd.2025.100590
Miriam Michel , David Renaud , Benjamin Kelly , Ismael Z. Assi , Liming Pei , Alexander R. Opotowsky
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引用次数: 0
期刊
International journal of cardiology. Congenital heart disease
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