International journal of cardiology. Congenital heart disease最新文献

英文中文

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

International journal of cardiology. Congenital heart disease

Pub 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.

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引用次数: 0

The root of the matter: Linking oral health to chronic diseases prevention

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub 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.

引用次数: 0

My journey with pulmonary hypertension: From diagnosis to thriving

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-01-31 DOI: 10.1016/j.ijcchd.2025.100572

Hall Skaara

引用次数: 0

Pulmonary arterial hypertension in Latin America. The age and comorbidity paradox

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-01-31 DOI: 10.1016/j.ijcchd.2025.100573

Tomas Pulido , Sofia de la Cruz-Perez , Daniela Valencia , Rafael Conde , Adrian Lescano , Nayeli Zayas

Pulmonary arterial hypertension (PAH) has been classically described as a disease in young adults, predominantly females with no comorbidities. However, in recent registries, the epidemiology has changed to older patients with comorbidities such as obesity, diabetes, systemic hypertension, and coronary heart disease. Nevertheless, there is not enough inclusion of these patients in clinical trials. In contrast, in Latin America, registries have shown that PAH patients are younger and have fewer comorbidities, which raises the question of whether Latin American patients present a different phenotype or if we are lagging behind developed countries and whether we will experience a change in epidemiology in the next couple of years. We analyzed these trends in this review.

引用次数: 0

New therapies in pulmonary arterial hypertension: Recent insights

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-01-30 DOI: 10.1016/j.ijcchd.2025.100571

Giulia Guglielmi , Konstantinos Dimopoulos , S. John Wort

Pulmonary Arterial Hypertension (PAH) is a complex and progressive disease characterized by elevated pulmonary vascular resistance and right heart failure. Current therapies primarily focus on pulmonary vasodilation; however, novel approaches that target the underlying pathophysiological mechanisms—such as TGF-β signalling, epigenetic alterations, growth factors, inflammation, and extracellular matrix remodelling—are promising alternatives for improving treatment outcomes. This is a review of recent advances in the development of innovative therapeutic strategies for PAH.

The first section of this paper explores approaches targeting TGF-β signalling, both acting directly on receptors through drugs like Sotatercept and exogenous BMP9, and indirectly, inhibiting the degradation of key receptors, such as BMPR2. Subsequent sections describe treatments that target epigenetic regulators, e.g. poly (ADP-ribose) polymerase-1 (PARP-1) inhibitors and direct BRD4 antagonists, tyrosine kinase inhibitors (Seralutinib), and therapies aimed at inflammation, such as IL-6 inhibitors, CD-20 inhibitors, and monoclonal antibodies that prevent macrophage migration. Finally, strategies that target the serotonin pathway, and other metabolic and hormonal pathways are described.

This review includes both preclinical and clinical trial data that support efficacy, safety and the future potential of such therapies. Collectively, these therapeutic approaches can be valuable in treating PAH by targeting multiple aspects of its pathogenesis, potentially resulting in improved clinical outcomes for patients affected by this debilitating, life-limiting condition.

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引用次数: 0

Thromboprophylaxis and adult congenital heart disease: The latest on indications, risk scoring and therapy

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

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.

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引用次数: 0

Sedentary behavior and physical activity on exercise capacity in adult patients with congenital heart disease

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

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.

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引用次数: 0

Right heart reverse remodeling: “facta non verba”

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

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.

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引用次数: 0

Family caregiver frailty in adult congenital heart disease is overlooked: A call to action

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

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

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

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.

引用次数: 0

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International journal of cardiology. Congenital heart disease

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