International journal of cardiology. Congenital heart disease最新文献

Percutaneous closure of perimembranous and infundibular ventricular septal defects: single-center experience in 203 patients with medium- and long-term follow-up 经皮膜周和小室间隔缺损闭合：203例中长期随访患者的单中心经验

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-12-01 DOI: 10.1016/j.ijcchd.2025.100641

Nataliia Yashchuk , Igor Ditkivskyy , Denys Voloshyn , Bogdan Cherpak , Yuliia Yermolovych

引用次数: 0

Prognostic implications of heart failure rehospitalization in adults with CHD & clinical benefits of medical therapy 成人冠心病患者心力衰竭再住院的预后意义及药物治疗的临床益处

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-12-01 DOI: 10.1016/j.ijcchd.2025.100640

Emili P. Rosado Rodriguez, Cynthia S. Crowson, Alexander C. Egbe

Background

The risk factors for heart failure (HF) rehospitalization, and the effect of guideline directed medical therapy (GDMT) are poorly understood in adults with congenital heart disease (ACHD). We aimed to describe the outcomes of HF hospitalization and to assess the clinical benefits of GDMT for HF.

Methods

We conducted a retrospective cohort study of ACHD hospitalized for HF at Mayo Clinic from 2003 to 2023. Cox proportional hazard models were used to determine the predictors of HF rehospitalization and all-cause mortality, and to assess the association between death and GDMT, as measured by the Heart Failure Collaborative score.

Results

Of the 324 patients, 164 were rehospitalized for HF and 149 died. The 10-year cumulative incidence of rehospitalization was 75.8 % (95 % CI 70.1 %–81.5 %), and the 10-year survival rate from mortality was 47.3 % (95 % CI 40.9 %–54.7 %). Chronic kidney disease (HR 1.46, 95 % CI 1.06–2.01, p = 0.021) and Fontan physiology (HR 1.59, 95 % CI 1.02–2.49, p = 0.043) were associated with HF rehospitalization. HF rehospitalization within 1 year was associated with nearly a 3-fold increased risk of mortality (HR 2.88, 95 % CI 2.00–4.15, p < 0.001). The HFC score was associated with a lower risk of all-cause mortality (HR 0.77, 95 % CI 0.62–0.95, p = 0.016) in the subgroup of patients with reduced ejection fraction.

Conclusions

Among adults with CHD hospitalized for HF, half of the patients were rehospitalized for HF. Rehospitalization within 1 year of the index HF hospitalization was associated with mortality. The use of GDMT for HF was associated with improved survival in patients with reduced ejection fraction.

成人先天性心脏病（ACHD）患者心衰（HF）再住院的危险因素以及指导药物治疗（GDMT）的效果尚不清楚。我们的目的是描述HF住院的结果，并评估GDMT治疗HF的临床益处。方法对2003 - 2023年梅奥诊所因心衰住院的ACHD患者进行回顾性队列研究。使用Cox比例风险模型来确定HF再住院和全因死亡率的预测因素，并评估死亡与GDMT之间的关系，通过心力衰竭协作评分来衡量。结果324例患者中，164例因心衰再次住院，149例死亡。10年累计再住院发生率为75.8% (95% CI 70.1% - 81.5%)， 10年死亡生存率为47.3% （95% CI 40.9% - 54.7%）。慢性肾脏疾病（HR 1.46, 95% CI 1.06-2.01, p = 0.021）和丰坦生理（HR 1.59, 95% CI 1.02-2.49, p = 0.043）与HF再住院相关。心力衰竭患者1年内再住院与死亡风险增加近3倍相关（HR 2.88, 95% CI 2.00-4.15, p < 0.001）。在射血分数降低的亚组中，HFC评分与较低的全因死亡风险相关（HR 0.77, 95% CI 0.62-0.95, p = 0.016）。结论在因心衰住院的成人冠心病患者中，有一半的患者再次因心衰住院。心力衰竭住院后1年内再次住院与死亡率相关。使用GDMT治疗心衰与射血分数降低患者的生存率提高相关。

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引用次数: 0

Correlates of left ventricular hypertrophy in adults with coarctation of aorta and role in risk stratification 成人左室肥厚与主动脉缩窄的相关性及其在危险分层中的作用

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-11-22 DOI: 10.1016/j.ijcchd.2025.100642

Zeyad Kholeif, Ahmed E. Ali, Karim Ali, Marwan Ahmed, Ahmed Bahnasy, Meena Bai, Sara Aboelmaaty, Mohamed Ellabbad, Alexander C. Egbe

Background

Coarctation of aorta (COA) results in chronic left ventricular (LV) pressure overload and LV hypertrophy, which is associated with cardiovascular mortality. Therapies that decrease LV pressure overload result in regression of LV hypertrophy and improved survival. The goal of COA intervention is to relieve LV pressure overload, and the guidelines recommend the use of several COA severity indices as indications for intervention. These indices are Doppler mean gradient, systolic blood pressure (SBP), upper-to-lower-extremity SBP (ULE-SBP), aortic isthmus ratio, presence of collateral vessels, and exercise-induced hypertension (EIH). Although these indices are physiologically intuitive, it is unclear how many of these indices are required in order to recommend intervention, and the hierarchical importance of the different indices in patients that have some but not all the indices.

Methods

In this cross-sectional study, multivariate linear regression analysis was used to assess the correlation between LV mass index (LVMI) and indices of COA severity.

Results

Of the COA indices analyzed in 811 adult with repaired COA patients, aortic isthmus ratio had the strongest correlation with LVMI (β coefficients ±standard error −31.6 ± 13.8, p < 0.001), as compared to Doppler peak gradient (0.79 ± 0.22, p = 0.002) and SBP (0.53 ± 0.21, p = 0.03).

Conclusion

Aortic isthmus ratio had the strongest correlation with LVMI, which is a reflection of chronic LV pressure overload. An aortic isthmus ratio ≤0.7 was the optimal cut-off point to detect LV hypertrophy with good sensitivity and specificity. These results can potentially improve and simplify patient selection for COA intervention.

背景：主动脉缩窄（COA）导致慢性左室（LV）压力过载和左室肥厚，这与心血管疾病死亡率相关。减少左室压力过载的治疗可导致左室肥大的消退和生存率的提高。COA干预的目标是缓解左室压力过载，指南建议使用几个COA严重程度指标作为干预的指征。这些指标是多普勒平均梯度、收缩压（SBP）、上下肢收缩压（ULE-SBP）、主动脉峡比、侧支血管的存在和运动性高血压（EIH）。虽然这些指标在生理上是直观的，但目前尚不清楚需要多少这些指标才能推荐干预措施，以及不同指标在具有某些但不是全部指标的患者中的等级重要性。方法采用多变量线性回归分析，评价左室质量指数（LVMI）与COA严重程度指标的相关性。结果811例成人COA修复患者的COA指标中，主动脉峡部比值与LVMI （β系数±标准误差- 31.6±13.8,p < 0.001）、多普勒峰梯度（0.79±0.22,p = 0.002）和收缩压（0.53±0.21,p = 0.03）的相关性最强。结论主动脉峡比与LVMI相关性最强，是慢性左室压过负荷的反映。主动脉峡部比值≤0.7是检测左室肥厚的最佳分界点，具有良好的敏感性和特异性。这些结果可以潜在地改善和简化COA干预的患者选择。

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引用次数: 0

Congenital heart disease in men of reproductive age: Nationwide cohort studies of prevalence, male fertility, and birth outcomes in offspring 育龄男性先天性心脏病：患病率、男性生育能力和后代出生结局的全国队列研究

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-11-06 DOI: 10.1016/j.ijcchd.2025.100637

Marit Sandberg , Nina Øyen , Tatiana Fomina , Ferenc Macsali , Gottfried Greve , Elisabeth Leirgul

Background

The potential for men with congenital heart disease (CHD) to father children and the impact of paternal CHD on offspring birth outcomes are not well understood.

Methods

Using Norwegian nationwide registries from 1994 to 2014, we identified two study populations: 1 829 877 men aged 18–50 years and 1 207 410 newborns. First, we reported the time trends of CHD in men and paternal CHD in newborns. Second, we compared the rate of becoming fathers in men with and without CHD. Third, we compared birth outcomes in offspring with and without paternal CHD.

Results

Between 1994 and 2014, the prevalence of CHD in men of reproductive age increased from 22.7 to 32.2 per 10 000, while the prevalence of paternal CHD in newborns increased from 21.6 to 26.9 per 10 000. Men with mild CHD had a similar rate of becoming fathers compared to men without CHD (rate ratio 0.97, 95 % confidence interval 0.90–1.05), but men with moderate/severe CHD had a lower rate (rate ratio 0.78, 95 % confidence interval 0.70–87). Newborns with paternal CHD had no increased risk of preterm birth or being small for gestational age, compared to newborns without paternal CHD.

Conclusion

The prevalence of CHD in men and paternal CHD in newborns was increasing. Moderate/severe CHD in men was associated with a lower rate of becoming fathers compared to the general male population. Paternal CHD was not associated with an increased risk of newborns being preterm or small for gestational age.

研究背景先天性心脏病（CHD）男性是否有可能成为孩子的父亲，以及父亲的CHD对后代出生结局的影响尚不清楚。方法利用1994 - 2014年挪威全国登记资料，确定了两个研究人群：18-50岁男性1 829 877人，新生儿1 207 410人。首先，我们报道了男性冠心病和新生儿父亲冠心病的时间趋势。其次，我们比较了有和没有冠心病的男性成为父亲的比率。第三，我们比较了有和没有父亲冠心病的后代的出生结果。结果1994 - 2014年育龄男性冠心病患病率从22.7 / 1万上升到32.2 / 1万，新生儿父亲冠心病患病率从21.6 / 1万上升到26.9 / 1万。与非冠心病男性相比，轻度冠心病男性成为父亲的比率相似（比率比0.97,95%可信区间0.90-1.05），但中度/重度冠心病男性的比率较低（比率比0.78,95%可信区间0.70-87）。与没有父亲冠心病的新生儿相比，父亲冠心病的新生儿早产或胎龄小的风险没有增加。结论男性冠心病患病率和新生儿父系冠心病患病率呈上升趋势。与一般男性人群相比，中度/重度冠心病男性成为父亲的几率较低。父亲患冠心病与新生儿早产或小于胎龄的风险增加无关。

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引用次数: 0

Machine learning-based predictive model for atrial arrhythmia following transcatheter atrial septal defect closure 基于机器学习的经导管房间隔缺损闭合后房性心律失常预测模型

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-11-05 DOI: 10.1016/j.ijcchd.2025.100639

Xander Jacquemyn , Alexander Van De Bruaene , Joris Ector , Peter Haemers , Pieter De Meester , Cedric Manlhiot , Werner Budts , Bert Vandenberk

Background

Atrial septal defects (ASDs) are frequently closed percutaneously. Despite successful closure, many patients still develop atrial arrhythmias. There is inconsistent data on the risk factors associated with these atrial arrhythmias. As such, we aimed to develop a machine learning (ML) model predicting atrial arrhythmias following transcatheter ASD closure in adolescents and adults.

Methods

Patients with secundum-type ASDs undergoing transcatheter closure between 2008 and 2024 at a single center were retrospectively analyzed. Patients with prior atrial arrhythmias were excluded. A deep neural network, adapted via transfer learning from a large external ECG dataset, was used to extract predictive features from preprocedural 12-lead ECGs. These features were combined with clinical, demographic, biochemical and hemodynamic variables in ensemble survival models. Model performance was assessed using the integrated Brier scores and the area under the receiver operating curve (AUC).

Results

A total of 148 adult patients (median 44.4 years [30.6–57.8], 105 females [70.9 %]) were eligible for included. There were a total of 1055 person-years of follow-up (median follow-up 7.3 [3.1–11.3]), during which 28 patients (18.9 %) developed atrial arrhythmias. The final ensemble ML model incorporating ECG-derived features demonstrated strong predictive performance (integrated Brier score 0.044, mean AUC 0.823). Subgroup and sensitivity analyses confirmed the robustness of the model across various patient profiles.

Conclusions

We developed a novel ML-based risk model using a transfer learning approach to predict atrial arrhythmias after transcatheter ASD closure. Further research and external validation are needed to refine the proposed risk stratification prior to clinical implementation.

背景：室间隔缺损（asd）通常是经皮缝合的。尽管成功关闭，许多患者仍然发生心房心律失常。关于与这些心房心律失常相关的危险因素的数据不一致。因此，我们旨在开发一种机器学习（ML）模型，预测青少年和成人经导管ASD关闭后的心房心律失常。方法回顾性分析2008 ~ 2024年在单中心行经导管闭合术的二次型asd患者。排除既往有心房心律失常的患者。通过从大型外部心电数据集进行迁移学习，采用深度神经网络从术前12导联心电图中提取预测特征。在整体生存模型中，这些特征与临床、人口统计学、生化和血流动力学变量相结合。采用综合Brier评分和受试者工作曲线下面积（AUC）评估模型性能。结果共纳入148例成人患者，中位年龄44.4岁[30.6 ~ 57.8岁]，女性105例[70.9%]。共随访1055人-年（中位随访7.3[3.1-11.3]），其中28例（18.9%）发生心房心律失常。结合心电图衍生特征的最终集成ML模型表现出较强的预测性能（综合Brier评分0.044，平均AUC 0.823）。亚组分析和敏感性分析证实了该模型在不同患者资料中的稳健性。我们开发了一种新的基于ml的风险模型，使用迁移学习方法来预测经导管ASD关闭后的心房心律失常。在临床实施之前，需要进一步的研究和外部验证来完善建议的风险分层。

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引用次数: 0

Clinical characteristics and outcomes of cardiovascular surgery and transcatheter procedures in adults with Down syndrome and congenital heart disease 成人唐氏综合征和先天性心脏病的心血管手术和经导管手术的临床特点和结果

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-11-05 DOI: 10.1016/j.ijcchd.2025.100638

Takumi Osawa , Tomoko Machino-Ohtsuka , Keitaro Kume , Takahiro Yano , Koshiro Kanaoka , Yoko Sumita , Yusuke Yano , Ruriko Numata , Naoto Kawamatsu , Hideyuki Kato , Yuji Hiramatsu , Tomoko Ishizu

Background

Advances in medical care have increased the number of adults with Down syndrome and congenital heart disease (CHD), including those with residual or unrepaired lesions. However, clinical outcomes following invasive cardiovascular procedures in this population remain poorly understood.

Methods

We extracted data on patients with Down syndrome aged ≥16 years and diagnosed with CHD from the Japanese Registry of All Cardiac and Vascular Diseases Diagnosis Procedure Combination (JROAD-DPC), covering the period from April 1, 2013, to March 31, 2023. Patients who underwent cardiovascular surgery or transcatheter procedures were included in the analysis.

Results

The study included 115 patients (median age: 21 [17–33] years; 47.8 % female), of whom 72 underwent surgery and 43 underwent transcatheter intervention. Procedures were most common among those aged 16–29 years. The main CHD subtypes were tetralogy of Fallot, atrial septal defects, and atrioventricular septal defects. Catheter-based interventions were associated with shorter hospital stay and no in-hospital mortality. In contrast, all three deaths (4.2 %) occurred in the surgical group, with pulmonary hypertension medications used in two of these patients. Among surgical patients receiving pulmonary hypertension medications, the in-hospital mortality rate reached 25 %. In multivariate analysis, emergency admission and the use of pulmonary hypertension medications were independently associated with longer hospital stays after surgery.

Conclusions

In adults with Down syndrome and CHD, invasive cardiovascular procedures had relatively acceptable in-hospital outcomes. Careful patient selection and perioperative management are crucial when planning cardiac surgery, especially in patients taking medication for pulmonary hypertension.

医疗保健的进步增加了患有唐氏综合征和先天性心脏病（CHD）的成年人的数量，包括那些有残留或未修复病变的人。然而，在这一人群中，有创心血管手术后的临床结果仍然知之甚少。方法我们从日本所有心血管疾病诊断程序组合登记处（JROAD-DPC）中提取年龄≥16岁且诊断为冠心病的唐氏综合征患者的数据，涵盖时间为2013年4月1日至2023年3月31日。接受心血管手术或经导管手术的患者被纳入分析。结果纳入115例患者，中位年龄21岁[17-33]，女性47.8%，其中手术72例，经导管介入43例。手术在16-29岁的人群中最为常见。冠心病主要亚型为法洛四联症、房间隔缺损和房室间隔缺损。基于导管的干预措施与更短的住院时间和无院内死亡率相关。相比之下，所有3例死亡（4.2%）发生在手术组，其中2例患者使用了肺动脉高压药物。在接受肺动脉高压药物治疗的外科患者中，住院死亡率达到25%。在多变量分析中，急诊入院和肺动脉高压药物的使用与术后住院时间的延长独立相关。结论：在患有唐氏综合征和冠心病的成年人中，有创心血管手术具有相对可接受的住院结果。仔细的病人选择和围手术期管理是至关重要的，当计划心脏手术，特别是在病人服用药物肺动脉高压。

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引用次数: 0

Cardiac autonomic function in patients with single ventricle physiology after Fontan palliation: A literature review Fontan姑息治疗后单心室患者的心脏自主神经功能：文献综述

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-30 DOI: 10.1016/j.ijcchd.2025.100636

Elizaveta Polyakova , Marieke Nederend , Philippine Kies , Anastasia D. Egorova , Monique R.M. Jongbloed

Background

The Fontan operation has significantly improved survival in patients with single ventricle physiology. This comes at a price of highly prevalent long-term complications. Autonomic dysfunction has been documented in patients after Fontan palliation. Although autonomic dysfunction is associated with a range of adverse outcomes, the exact clinical implications in patients with a Fontan circulation remain unclear.

Aims

The aims of this review are to address (1) the extent and characteristics of autonomic dysfunction in Fontan patients; (2) its association with clinical short- and long-term outcomes, and (3) identify key gaps in the literature.

Methods

A literature search was performed in PubMed using a dedicated query for single ventricle disease and cardiac autonomic (dys)function.

Results

Data from the literature consistently indicated overt autonomic dysfunction in patients after Fontan operation versus controls, marked by reduced heart rate variability and impaired parasympathetic tone. However, autonomic dysfunction showed different associations with clinical outcomes in Fontan patients. Several studies reported an association with diminished exercise capacity, arrhythmias, organ function markers, while other studies found no clear predictive value. Gaps in literature include data on adult patients, the influence of anatomical substrate, sex and lifestyle, as well as specific “driving” factors for the development of cardiac autonomic dysfunction.

Conclusion

Autonomic dysfunction in Fontan patients is a persistent and potentially progressive condition, characterized by reduced heart rate variability and reduced parasympathetic tone. Long-term studies are needed to clarify its clinical significance and address gaps in literature relating to contributing factors like sex, anatomy, and underlying mechanisms.

背景Fontan手术可显著提高单心室生理患者的生存率。这是以非常普遍的长期并发症为代价的。在Fontan姑息治疗后的患者中有植物神经功能障碍的记录。尽管自主神经功能障碍与一系列不良后果相关，但Fontan循环患者的确切临床意义尚不清楚。本综述的目的是解决(1)Fontan患者自主神经功能障碍的程度和特征；(2)其与临床短期和长期预后的关系；(3)确定文献中的关键空白。方法在PubMed上使用单心室疾病和心脏自主神经（dys）功能的专用查询进行文献检索。结果来自文献的数据一致表明Fontan手术后患者与对照组相比存在明显的自主神经功能障碍，其特征是心率变异性降低和副交感神经张力受损。然而，自主神经功能障碍与Fontan患者的临床结果有不同的相关性。一些研究报告了与运动能力下降、心律失常、器官功能指标的关联，而其他研究没有发现明确的预测价值。文献中的空白包括成年患者的数据，解剖基质、性别和生活方式的影响，以及心脏自主神经功能障碍发展的具体“驱动”因素。结论Fontan患者的自主神经功能障碍是一种持续的、潜在的进行性疾病，其特征是心率变异性降低和副交感神经张力降低。需要长期的研究来阐明其临床意义，并解决与性别、解剖学和潜在机制等因素相关的文献空白。

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引用次数: 0

How to set up a pregnancy heart team 如何成立孕心小组

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-28 DOI: 10.1016/j.ijcchd.2025.100635

K. Rijs , C.D. van der Marel , A.L.M.J. van der Knijff-van Dortmont , I.J.J. Dons-Sinke , V.F.R. Adriaens , J.M.J. Cornette , A.G.M.G.J. Mulders , J.J. Duvekot , M.M.P. Driessen , A.E. van den Bosch , J.A.A.E. Cuypers , R.M. Kauling , J.W. Roos-Hesselink

Cardiovascular disease (CVD) is the leading cause of maternal morbidity and mortality in developed countries, complicating 4–11 % of pregnancies. Women with CVD are at increased risk of maternal complications, including heart failure and arrhythmias, as well as adverse fetal outcomes such as preterm birth and perinatal mortality. Traditionally, care for pregnant women with CVD has been fragmented across specialties, leading to discontinuity and missed opportunities for optimization. To address this, recent European Society of Cardiology (ESC) guidelines strongly recommend the implementation of a pregnancy heart team: a dedicated multidisciplinary team providing integrated care for this high-risk population.

We describe the implementation and organization of a pregnancy heart team in our academic tertiary hospital. The core team consists of a cardiologist specialized in cardio-obstetrics, a maternal-fetal medicine specialist, and an obstetric anesthesiologist, with additional experts consulted as required. Patients are discussed at predefined time points: preconception, during pregnancy, and postpartum. Standardized risk assessment using the modified World Health Organization (mWHO) classification guides counseling, management, and delivery planning. Shared decision-making is central, balancing maternal and fetal risks with patient preferences. The team establishes individualized peripartum care plans covering timing, mode, and location of delivery, anesthesia strategies, anticoagulation management, and postpartum monitoring.

This structured, multidisciplinary model ensures continuity of care and offers a practical framework to improve outcomes in women with CVD during pregnancy.

心血管疾病（CVD）是发达国家孕产妇发病和死亡的主要原因，使4 - 11%的妊娠并发症。患有心血管疾病的妇女发生产妇并发症的风险增加，包括心力衰竭和心律失常，以及不良胎儿结局，如早产和围产期死亡。传统上，对患有心血管疾病的孕妇的护理分散在各个专科，导致不连续性和错失优化机会。为了解决这个问题，最近的欧洲心脏病学会（ESC）指南强烈建议实施妊娠心脏小组：一个专门的多学科小组，为这一高危人群提供综合护理。介绍了我院三级专科医院妊娠心脏科的实施与组织。核心小组由一名专门从事心脏产科的心脏病专家、一名母胎医学专家和一名产科麻醉师组成，并根据需要咨询其他专家。患者在预定的时间点进行讨论：孕前，怀孕期间和产后。使用经修订的世界卫生组织（mWHO）分类指南进行标准化风险评估，包括咨询、管理和交付计划。共同决策是核心，平衡产妇和胎儿的风险与患者的偏好。团队建立个性化的围产期护理计划，包括分娩的时间、方式和地点、麻醉策略、抗凝管理和产后监测。这种结构化的多学科模式确保了护理的连续性，并为改善妊娠期心血管疾病妇女的预后提供了实用的框架。

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引用次数: 0

Burden of diffuse myocardial fibrosis assessed by cardiac magnetic resonance in repaired tetralogy of Fallot: A systematic review and meta-analysis 通过心脏磁共振评估修复法洛四联症患者弥漫性心肌纤维化负担：一项系统回顾和荟萃分析

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-24 DOI: 10.1016/j.ijcchd.2025.100631

José Carlos P. Secco , Eduardo Schaustz , Adriana M. Innocenzi , Fernanda.P. Fernandes , Emiliano Medei , Andréa Silvestre-Sousa , Gabriel C. Camargo , Ronir R. Luiz , Jessica P.L. Moreira , Carlos A.S. Magliano , Renata Moll-Bernardes , Mariana Póvoa-Corrêa

Background

The assessment of myocardial fibrosis by cardiac magnetic resonance (CMR) in patients with repaired tetralogy of Fallot (rTOF) is not routinely performed, primarily due to technical challenges in measuring right ventricular native T1 and extracellular volume (ECV). Additionally, although native T1 can be assessed without contrast, offering broader applicability, its role in evaluating myocardial fibrosis in rTOF patients remains unclear.

Objectives

The aim of this systematic review and meta-analysis was to evaluate interstitial fibrosis in both the right and left ventricles using native T1 and ECV quantification in patients with rTOF.

Methods

This systematic review was conducted according to the 2020 PRISMA guidelines and was registered in PROSPERO. The PubMed, Scopus, and Embase databases were searched to identify studies in which patients with rTOF underwent CMR with native T1 and ECV assessment. Random-effect models were used to estimate pooled effect sizes.

Results

The sample comprised 14 studies that included 893 patients with rTOF and 284 controls. LV native T1 values did not differ significantly between groups, except in the studies with a 3.0 T scanner (d = 3.52; p < 0.01). RV values were significantly elevated in patients with rTOF (d = 1.36, p < 0.01). The left ventricle and right ventricle ECVs were significantly higher in these patients than in controls (d = 0.95 and 1.11, respectively; both p < 0.01).

Conclusion

Despite successful surgical repair, patients with rTOF frequently exhibit evidence of myocardial fibrosis, a marker of adverse remodeling that may progress long after anatomical correction.

通过心脏磁共振（CMR）评估修复法洛四联症（rTOF）患者的心肌纤维化并没有常规进行，主要是由于测量右心室原生T1和细胞外体积（ECV）的技术挑战。此外，虽然原生T1可以不进行对比评估，具有更广泛的适用性，但其在评估rTOF患者心肌纤维化中的作用尚不清楚。本系统综述和荟萃分析的目的是利用原生T1和ECV量化评估rTOF患者的左右心室间质纤维化。方法本系统评价按照2020年PRISMA指南进行，并在PROSPERO注册。检索PubMed、Scopus和Embase数据库，以确定rTOF患者接受CMR并进行原生T1和ECV评估的研究。随机效应模型用于估计合并效应大小。结果共纳入14项研究，包括893例rTOF患者和284例对照组。除了使用3.0 T扫描仪的研究外，各组间LV原生T1值无显著差异（d = 3.52; p < 0.01）。rTOF患者的RV值显著升高（d = 1.36, p < 0.01）。这些患者的左心室和右心室ecv明显高于对照组（d分别= 0.95和1.11，p均为0.01）。结论：尽管手术修复成功，但rTOF患者经常表现出心肌纤维化的迹象，这是一种不良重构的标志，可能在解剖矫正后很长一段时间内持续发展。

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引用次数: 0

Congenital uterine anomalies and congenital heart disease: An underappreciated association? 先天性子宫异常与先天性心脏病：一种未被充分认识的关联？

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-10-24 DOI: 10.1016/j.ijcchd.2025.100634

Harrison J. VanDolah , Kathy Mostajeran , Luis Goncalves , Wayne J. Franklin , Melissa Parks , Tabitha G. Moe

Background

Congenital uterine anomalies (CUAs), also known as Müllerian duct anomalies, can have an impact on gynecological care, pre-pregnancy counseling, and obstetrical outcomes. Patients with congenital heart disease (CHD), particularly of higher complexity, often have extracardiac manifestations. The relationship between CUAs and CHD is not well-described in the currently available literature.

Methods

A single center retrospective cohort study was performed of all patients born female with ICD-10 codes corresponding to congenital heart disease and a completed pelvic MRI or ultrasound. CT imaging was not included given the known shortcomings of CUA detection and classification with CT. CHD diagnoses were confirmed with echocardiography and were classified into “simple”, “moderate”, or “complex” per the 2018 ACHD guidelines. CUAs were categorized based on the 2021 American Society of Reproductive Medicine Guidelines for classification of Müllerian anomalies.

Results

197 patients met inclusion criteria. 33 (16.7 %) were found to have a CUA. 17 (20 %) patients with moderate or complex CHD had a CUA. Notable associations include VACTERL and partial anomalous pulmonary venous return. More complex CHD was associated with more complex CUA.

Conclusions

Within the limitations of a single center retrospective study, our data suggests there is an underappreciated association between CUA and CHD that requires further prospective multicenter study to assess prevalence data. There may ultimately be a role for targeted screening for CUA in patients with CHD, particularly those with genetic syndromes or moderate or complex CHD who are considering pregnancy or gynecologic intervention.

背景先天性子宫异常（CUAs），也被称为胆管异常，可对妇科护理、孕前咨询和产科结果产生影响。先天性心脏病（CHD）患者，特别是复杂性较高的患者，通常有心外表现。在目前的文献中，CUAs与冠心病之间的关系并没有得到很好的描述。方法采用单中心回顾性队列研究方法，对所有出生时患有先天性心脏病ICD-10编码并完成盆腔MRI或超声检查的女性患者进行研究。鉴于已知CT检测和分类CUA的缺点，未包括CT成像。通过超声心动图确诊冠心病，并根据2018年冠心病指南将其分为“简单”、“中度”和“复杂”。根据2021年美国生殖医学学会关于勒氏管异常分类的指南对cua进行分类。结果197例患者符合纳入标准。33例（16.7%）发现有CUA。17例（20%）中度或复杂冠心病患者发生CUA。值得注意的关联包括VACTERL和部分肺静脉异常回流。更复杂的冠心病与更复杂的CUA相关。结论：在单中心回顾性研究的限制下，我们的数据表明CUA和冠心病之间存在未被充分认识的关联，需要进一步的前瞻性多中心研究来评估患病率数据。对冠心病患者，特别是那些有遗传综合征或中度或复杂冠心病且考虑妊娠或妇科干预的患者，可能最终有针对性地筛查CUA。

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