International journal of cardiology. Congenital heart disease最新文献

英文中文

Exercise testing in clinical context: Reference ranges for interpreting anaerobic threshold as an outcome for congenital heart disease patients 临床背景下的运动测试：解释先天性心脏病患者无氧阈值的参考范围

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2024-08-27 DOI: 10.1016/j.ijcchd.2024.100540

Background

Change in the oxygen consumption (VO₂) at the ventilatory anaerobic threshold (VAT) is an important outcome in research studies of children with congenital heart disease (CHD). The range of values reported by different raters for any given VAT is needed to contextualize a change in VAT in intervention studies.

Methods

Sixty maximal cardiopulmonary exercise tests (CPET) for CHD patients 8–21 years old were independently reviewed by six exercise physiologists and four pediatric cardiologists. For each of the unique rater pairs for the 60 CPETs, the absolute difference in VAT was calculated and displayed on a histogram to demonstrate the distribution of inter-rater variability. This method was repeated for subgroups of test modality (cycle/treadmill), patient factors (diagnoses, exercise capacity), and rater factors (cardiologist/physiologist, years of experience).

Results

Rater agreement was good with an intraclass correlation coefficient of 0.79–0.91 but the distribution of differences was broad. The median difference was 2.7 % predicted peak VO₂ (60 mL/min, 1.0 mL/kg/min), the 75th percentile was 6.4 % (140 mL/min, 2.5 mL/kg/min), and the 95th percentile was 16.3 % (421 mL/min, 6.5 mL/kg/min). Distributions were similar for CPET modality and years of rater experience, but differed for other factors.

Conclusions

The baseline distribution of reported VAT is relatively broad, varied by units, and was not explained by differences in rater experience or test modality, but varies by patient factors. When evaluating clinical relevance, a change in the VO₂ at VAT in response to an intervention of <6.5 % predicted falls within the majority (75th percentile) of expected variability and should be interpreted with caution.

背景在先天性心脏病（CHD）儿童的研究中，通气无氧阈值（VAT）耗氧量（VO2）的变化是一项重要的研究结果。方法由六位运动生理学家和四位儿科心脏病专家对六十名 8-21 岁先天性心脏病患者的最大心肺运动测试（CPET）进行独立审查。对于 60 项 CPET 的每一对独特的评分者，计算出增值血管舒张功能的绝对差异，并显示在直方图上，以显示评分者之间变异性的分布。对测试模式（循环/跑步机）、患者因素（诊断、运动能力）和评分者因素（心脏病专家/生理学家、经验年限）等分组重复了这一方法。结果评分者之间的一致性很好，类内相关系数为 0.79-0.91，但差异分布很广。中位数差异为预测峰值 VO2 的 2.7%（60 毫升/分钟，1.0 毫升/千克/分钟），第 75 百分位数为 6.4%（140 毫升/分钟，2.5 毫升/千克/分钟），第 95 百分位数为 16.3%（421 毫升/分钟，6.5 毫升/千克/分钟）。结论报告的 VAT 基线分布相对宽泛，因单位而异，不能用评分者经验或测试模式的差异来解释，但因患者因素而异。在评估临床相关性时，预测值为 6.5 % 的 VO2 对干预措施的响应变化属于预期变异的大部分（第 75 百分位数），因此应谨慎解释。

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引用次数: 0

A national Australian Congenital Heart Disease registry; methods and initial results 澳大利亚全国先天性心脏病登记；方法和初步结果

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2024-08-24 DOI: 10.1016/j.ijcchd.2024.100538

Background

Although several National Data Registries for Congenital Heart Disease (CHD) exist, few are comprehensive and contemporary. A National Australian CHD Registry has been developed that aims to redress this by creating the first comprehensive data collection for CHD children and adults, initially across Australia.

Methods

We defined and collected a minimum dataset of demographics, diagnoses, and procedures from people with CHD presenting at participating quaternary CHD services Australia-wide. Data were collected from a range of clinical data sources. Diagnoses and procedures were standardised to the European Paediatric Congenital Code – Short List. Methodological limitations were carefully documented.

Results

From 8 participating institutions, an initial 359,084 patient records were assessed for eligibility and 68,234 unique individuals with structural CHD have been included in the current dataset. There were 20,395 (30 %) people with mild CHD, 25,157 (37 %) with moderate CHD, and 13,530 (20 %) with severe CHD (6 % unknown complexity). The most common diagnoses were Ventricular Septal Defect (16,781, 25 %), Atrial Septal Defect (6,607, 10 %), Aortic Valve Disorders (5516 8 %), Coarctation of the Aorta (5,321, 8 %), Tetralogy of Fallot (4,489, 7 %), Transposition of the Great Arteries (4,009, 6 %).

Conclusion

The data presented here represents the most comprehensive cohort collected for the Australian CHD population thus far and is comparable with the largest contemporary CHD registries around the world. This Registry represents a key resource for improved understanding of the CHD population and will drive better care and outcomes for people living with CHD.

背景虽然目前已有多个国家先天性心脏病（CHD）数据登记处，但很少有登记处是全面和现代的。澳大利亚国家先天性心脏病登记处（National Australian CHD Registry）已经建立，旨在通过首次在全澳范围内全面收集先天性心脏病儿童和成人的数据来解决这一问题。方法我们定义并收集了在全澳范围内参与的四级先天性心脏病服务机构就诊的先天性心脏病患者的最低人口统计学、诊断和手术数据集。数据收集自一系列临床数据源。诊断和手术均按照《欧洲儿科先天性疾病代码--简表》进行标准化。结果8家参与机构的359,084份病历通过了初步资格评估，68,234名结构性先天性心脏病患者被纳入当前数据集中。其中，20,395 人（30%）患有轻度先天性心脏病，25,157 人（37%）患有中度先天性心脏病，13,530 人（20%）患有重度先天性心脏病（6%复杂程度未知）。最常见的诊断是室间隔缺损（16781 人，25%）、心房隔缺损（6607 人，10%）、主动脉瓣疾病（5516 人，8%）、主动脉共动脉症（5321 人，8%）、法洛氏四联症（4489 人，7%）、大动脉转位（4009 人，6%）。结论本文提供的数据是迄今为止收集到的最全面的澳大利亚先天性心脏病人群队列数据，可与全球最大的当代先天性心脏病登记处的数据相媲美。该登记处是增进对冠心病人群了解的重要资源，将推动为冠心病患者提供更好的护理和治疗效果。

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引用次数: 0

Assessing subaortic right ventricle function after atrial switch repair through myocardial work analysis 通过心肌功分析评估心房转换修复术后主动脉瓣下右心室的功能

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2024-08-14 DOI: 10.1016/j.ijcchd.2024.100537

Background

The Mustard and Senning operations for dextro-transposition of the great arteries (D-TGA) establish a biventricular physiology with a subaortic right ventricle (sRV). While prolonged QRS has been associated with worse prognosis in these patients, current echocardiographic tools fall short in adequately assessing the (mal)performance and function decline of the sRV during follow-up. The present study is the first to characterize Myocardial Work (MW) indices of the sRV in D-TGA patients after Mustard/Senning repair.

Methods and results

All adult D-TGA patients at follow-up in the University Hospital of Leuven between 2018 and 2022 were screened for inclusion. We retrospectively collected the most recent electrocardiogram parameters, 2D echocardiographic images and serum biomarkers’ values. Offline calculations of MW indices were performed. We established correlations between all variables and categorized patients into QRS<120 ms and QRS≥120 ms for further analysis. A total of 51 D-TGA patients were included (13 Mustard, 33 male, 39 ± 6 years). QRS duration increased with age and was correlated with sRV dimensions and serum levels of troponins (R = 0.42, p < 0.01) and NT-proBNP (R = 0.31, p = 0.03). However, no significant correlation was found between QRS duration and intraventricular desynchrony or conventional functional echocardiographic parameters. QRS prolongation was associated with a deterioration in septal, but not lateral, MW parameters. Patients with QRS≥120 ms had significantly larger ventricles and higher levels of troponins and NT-proBNP.

Conclusion

QRS prolongation after Mustard/Senning repair is linked to ventricular dilatation and worse performance, particularly affecting the septal wall. Global and regional MW analysis may be useful to assess the subclinical deterioration of sRV function.

背景针对大动脉右侧横位（D-TGA）的芥末和森宁手术建立了具有主动脉瓣下右心室（sRV）的双心室生理结构。虽然 QRS 延长与这些患者的预后较差有关，但目前的超声心动图工具无法充分评估 sRV 在随访期间的（不良）表现和功能下降情况。本研究首次描述了Mustard/Senning修复术后D-TGA患者sRV的心肌工作（MW）指数。方法和结果筛选出2018年至2022年期间在鲁汶大学医院随访的所有成年D-TGA患者。我们回顾性地收集了最新的心电图参数、二维超声心动图图像和血清生物标志物值。我们对 MW 指数进行了离线计算。我们建立了所有变量之间的相关性，并将患者分为 QRS<120 ms 和 QRS≥120 ms 两类，以便进一步分析。共纳入 51 名 D-TGA 患者（13 名 Mustard，33 名男性，39 ± 6 岁）。QRS 持续时间随年龄增长而增加，并与 sRV 尺寸、血清肌钙蛋白水平（R = 0.42，p < 0.01）和 NT-proBNP（R = 0.31，p = 0.03）相关。然而，QRS持续时间与心室内非同步性或常规功能性超声心动图参数之间没有发现明显的相关性。QRS 延长与室间隔 MW 参数的恶化有关，但与侧壁 MW 参数无关。QRS≥120毫秒的患者心室明显增大，肌钙蛋白和NT-proBNP水平升高。整体和区域 MW 分析可能有助于评估 sRV 功能的亚临床恶化。

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引用次数: 0

Imaging in chronic thromboembolic pulmonary disease: Current practice and advances 慢性血栓栓塞性肺病的成像：当前实践与进展

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2024-08-10 DOI: 10.1016/j.ijcchd.2024.100536

Chronic thromboembolic pulmonary disease (CTEPD) with or without pulmonary hypertension (PH) occurs when thromboemboli in pulmonary arteries fail to resolve completely. Pulmonary artery obstructions due to chronic thrombi and secondary microvasculopathy can increase pulmonary arterial pressure and resistance leading to chronic thromboembolic PH (CTEPH). Mechanical interventions and/or PH medications can improve cardiopulmonary haemodynamic, alleviate symptoms, and decrease mortality risk. Imaging is pivotal throughout the CTEPD management journey, spanning diagnosis, treatment planning, and assessing treatment outcome. With just computed tomography (CT) pulmonary angiogram and right heart catheterisation, an experienced multidisciplinary team can determine surgical candidacy in most cases. Dual energy CT, lung subtraction iodine mapping CT, and dynamic contrast-enhanced magnetic resonance imaging (MRI) offer comparable sensitivities with ventilation-perfusion scintigraphy in diagnosing CTEPD. Pulmonary angiogram with digital subtraction angiography although considered the gold standard for assessing thrombi extent and vasculature morphology is now mostly used to assess targets for balloon pulmonary angioplasty. Advancements in CT modalities and innovative MRI metrics offer better insight into CTEPD management but are limited by the availability of technology and expertise. Learning from current artificial intelligence application in medical imaging, there is promise in tapping the wealth of data provided by CTEPD imaging through automating cardiopulmonary and vascular morphology analysis.

当肺动脉中的血栓栓塞未能完全消退时，就会发生伴有或不伴有肺动脉高压（PH）的慢性血栓栓塞性肺病（CTEPD）。慢性血栓和继发性微血管病变造成的肺动脉阻塞会增加肺动脉压力和阻力，导致慢性血栓栓塞性肺动脉高压（CTEPH）。机械干预和/或 PH 药物可改善心肺血流动力学、缓解症状并降低死亡风险。在 CTEPD 的整个治疗过程中，影像学在诊断、治疗计划和治疗效果评估方面都起着至关重要的作用。只需进行计算机断层扫描（CT）肺血管造影和右心导管检查，经验丰富的多学科团队就能确定大多数病例是否适合手术。双能量 CT、肺减影碘映射 CT 和动态对比增强磁共振成像（MRI）在诊断 CTEPD 方面的灵敏度与通气-灌注闪烁成像相当。虽然数字减影血管造影被认为是评估血栓范围和血管形态的金标准，但目前主要用于评估球囊肺血管成形术的目标。CT 模式和创新磁共振成像指标的进步为 CTEPD 的管理提供了更好的视角，但受限于技术和专业知识的可用性。借鉴当前人工智能在医学影像领域的应用，通过自动化心肺和血管形态分析，有望利用 CTEPD 成像提供的丰富数据。

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引用次数: 0

Sleep disordered breathing and adult congenital heart disease 睡眠呼吸紊乱与成人先天性心脏病

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2024-08-02 DOI: 10.1016/j.ijcchd.2024.100532

引用次数: 0

The latest definition and classification of pulmonary hypertension 肺动脉高压的最新定义和分类

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2024-07-31 DOI: 10.1016/j.ijcchd.2024.100534

Pulmonary hypertension (PH) is a serious potential complication of some congenital heart diseases (CHDs). PH encompasses a range of diseases which may be idiopathic or inherited, or secondary to cardiac, respiratory, systemic or thromboembolic conditions, amongst others. Our increasing understanding of the normal ranges of pulmonary haemodynamics, as well as evidence supporting the benefits of early treatment, has resulted in a number of recent revisions to the haemodynamic definition of PH. In this Review Article, we report on the recent updates to haemodynamic definitions and classification of PH, as reflected in the 2022 Pulmonary Hypertension Guidelines and particularly focus on the CHD related sub-type of PH, where the aetiology is often multi-factorial.

肺动脉高压（PH）是某些先天性心脏病（CHD）的严重潜在并发症。肺动脉高压包括一系列疾病，可能是特发性或遗传性的，也可能是继发于心脏、呼吸、全身或血栓栓塞等疾病。我们对肺血流动力学正常范围的认识不断加深，同时有证据表明早期治疗有益，因此最近对 PH 的血流动力学定义进行了多次修订。在这篇综述文章中，我们将报告 2022 年《肺动脉高压指南》中对 PH 的血流动力学定义和分类的最新更新，并特别关注 PH 的冠心病相关亚型，因为其病因通常是多因素的。

引用次数: 0

Subpulmonary ventricular function and inflammation are related to clinical heart failure in patients with a systemic right ventricle 肺下心室功能和炎症与系统性右心室患者的临床心力衰竭有关

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2024-07-31 DOI: 10.1016/j.ijcchd.2024.100535

Background

Timely diagnosis of heart failure (HF) in patients with a systemic right ventricle (sRV) is difficult but important since clinical deterioration is fast once HF develops. We aimed to compare echocardiography and biomarker profile between sRV patients with and without HF and patients with a systemic left ventricle diagnosed with HF (sLV-HF).

Methods and results

Eighty-seven sRV patients and 30 sLV-HF patients underwent echocardiographic evaluation and blood sampling. Compared to sRV patients without HF, sRV-HF patients had more remodeling of the subpulmonary LV (spLV) (internal diameter 3.9 cm [3.3–5.7] vs 3.4 cm [2.9–3.9], P = 0.03, posterior wall 0.93 cm [0.76–1.20] vs 0.71 cm [0.59–0.91], P = 0.006) and lower spLV systolic function: ejection fraction (59 % ± 14 vs 70 % ± 10, P = 0.011), mitral annular plane systolic excursion (1.7 cm ± 0.5 vs 2.1 cm ± 0.4, P = 0.003), fractional area change (47 % [38–58] vs 59 % [51–70], P = 0.002) and lateral strain rate (−1.2/s ± 0.46 vs −1.5/s ± 0.39, P = 0.016). Inflammatory biomarkers were higher in sRV-HF patients compared to those without HF: red cell distribution width (13.3 fL [12.8–14.1] vs 12.6 fL [12.3–13.1], P < 0.001), neutrophil lymphocyte ratio (NLR, 3.7 [2.2–4.9] vs 2.4 [1.9–3.0], P = 0.015), C-reactive protein (CRP, 2.5 mg/dL [1.0–4.2] vs 1.2 mg/dL [0.0–2.0], P = 0.005) and compared to sLV-HF patients (NLR (3.7 [2.2–4.9] vs 2.5 [1.7–3.3], P = 0.044) and CRP (2.5 mg/dL [1.0–4.2] vs 0.85 mg/dL [0.6–2.0], P = 0.006).

Conclusion

Biventricular echocardiographic evaluation with a focus on the subpulmonary LV together with assessing inflammatory status in sRV patients could help in an earlier detection of HF.

背景及时诊断全身性右心室（sRV）患者的心力衰竭（HF）很困难，但却很重要，因为一旦发生 HF，临床症状会迅速恶化。我们旨在比较伴有和不伴有心力衰竭的 sRV 患者与确诊为心力衰竭的全身性左心室患者（sLV-HF）之间的超声心动图和生物标志物谱。与无 HF 的 sRV 患者相比，sRV-HF 患者的肺下左心室（spLV）重塑程度更高（内径 3.9 cm [3.3-5.7] vs 3.4 cm [2.9-3.9]，P = 0.03，后壁 0.93 cm [0.76-1.20] vs 0.71 cm [0.59-0.91]，P = 0.006]）和较低的二尖瓣收缩功能：射血分数（59 % ± 14 vs 70 % ± 10，P = 0.011）、二尖瓣瓣环平面收缩期偏移（1.7 cm ± 0.5 vs 2.1 cm ± 0.4，P = 0.003）、分数面积变化（47 % [38-58] vs 59 % [51-70]，P = 0.002）和侧向应变率（-1.2/s ± 0.46 vs -1.5/s ± 0.39，P = 0.016）。与非 HF 患者相比，sRV-HF 患者的炎症生物标志物更高：红细胞分布宽度（13.3 fL [12.8-14.1] vs 12.6 fL [12.3-13.1]，P < 0.001）、中性粒细胞淋巴细胞比值（NLR，3.7 [2.2-4.9] vs 2.4 [1.9-3.0]，P = 0.015）、C反应蛋白（CRP，2.5 mg/dL [1.0-4.2] vs 1.2 mg/dL [0.0-2.0]，P = 0.005），与 sLV-HF 患者相比（NLR（3.7 [2.2-4.9] vs 2.5 [1.7-3.3]，P = 0.044）和 CRP（2.5 mg/dL [1.0-4.2] vs 0.85 mg/dL [0.6-2.0]，P = 0.006）。

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引用次数: 0

Outcomes, mortality risk factors, and functional status post-Norwood: A single-center study 结果、死亡率风险因素和诺伍德后的功能状态：单中心研究

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2024-07-26 DOI: 10.1016/j.ijcchd.2024.100533

Background

The Norwood operation (NO) for infants with univentricular physiology has high interstage mortality. This study evaluated outcomes and risk factors for mortality following NO.

Methods

Retrospective single-center study of patients undergoing NO from 2010 to 2020. Analysis used appropriate statistics.

Results

Of 269 patients undergoing NO, 213 (79.2 %) survived to discharge. Non-survivors had longer bypass times, delayed sternal closure, required nitric oxide, higher vasoactive scores, required post-operative catheterization, Extracorporeal Life Support (ECLS), and longer ventilation (p < 0.05). Logistic regression showed moderate-severe atrioventricular valve regurgitation on intraoperative TEE (OR 2.6), requiring nitric oxide (OR 2.63), delayed sternal closure (OR 2.94), post-operative catheterization (OR 10.48), and ECLS (OR 14.54) increased mortality odds (p < 0.05). Multivariable analysis confirmed catheterization (aOR 10.48) and ECLS (aOR 14.54) as significant predictors. Of survivors, 26 (12.3 %) developed new morbidity, 9 (4.2 %) had unfavorable outcomes. Functional status improved from 6.0 to 8.04, mainly in feeding and respiratory domains (p < 0.0001).

Conclusions

Norwood survival was 79.2 %. Requiring post-operative catheterization and ECLS significantly increased mortality risk. Multicenter evaluation of these modifiable risk factors is needed to improve outcomes in this high-risk population.

背景诺伍德手术（Norwood operation，NO）用于治疗单心室生理的婴儿，其阶段间死亡率很高。本研究评估了诺伍德手术后的结果和死亡风险因素。方法对 2010 年至 2020 年接受诺伍德手术的患者进行单中心回顾性研究。结果在269名接受无创手术的患者中，213人（79.2%）存活至出院。非存活患者的旁路时间更长、胸骨闭合延迟、需要一氧化氮、血管活性评分更高、需要术后导管插入、体外生命支持（ECLS）和通气时间更长（p <0.05）。逻辑回归显示，术中 TEE 显示的中重度房室瓣反流（OR 2.6）、需要一氧化氮（OR 2.63）、延迟胸骨闭合（OR 2.94）、术后导管插入（OR 10.48）和体外生命支持（ECLS）（OR 14.54）增加了死亡率几率（p < 0.05）。多变量分析证实导管插入术（aOR 10.48）和 ECLS（aOR 14.54）是重要的预测因素。在幸存者中，26 人（12.3%）出现了新的病症，9 人（4.2%）出现了不良后果。功能状态从 6.0 改善到 8.04，主要是在进食和呼吸领域（p < 0.0001）。结论诺伍德的存活率为 79.2%，术后需要导管插入术和 ECLS 会显著增加死亡风险。需要对这些可改变的风险因素进行多中心评估，以改善这一高风险人群的预后。

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引用次数: 0

A rare presentation of pulmonary hypertension 肺动脉高压的罕见表现

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2024-07-25 DOI: 10.1016/j.ijcchd.2024.100531

引用次数: 0

Congenital heart defects and consanguinity: An analysis of the Sidra cardiac registry data in Qatar 先天性心脏缺陷与血缘关系：卡塔尔 Sidra 心脏登记数据分析

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2024-07-20 DOI: 10.1016/j.ijcchd.2024.100529

引用次数: 0

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