International journal of cardiology. Congenital heart disease最新文献_第5页

Incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliation 2期缓解后肺动静脉畸形发生的发生率和危险因素

IF 1.2 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-07-18 DOI: 10.1016/j.ijcchd.2025.100611

Lea Behrend , Thibault Schaeffer , Muneaki Matsubara , Jonas Palm , Teresa Lemmen , Nicole Piber , Paul Philipp Heinisch , Stanimir Georgiev , Alfred Hager , Peter Ewert , Jürgen Hörer , Masamichi Ono

Objective

This study evaluated the current incidence of pulmonary arteriovenous malformations (PAVMs) following stage 2 palliation (S2P).

Methods

Patients who underwent S2P, either through a bidirectional cavopulmonary shunt (BCPS) or the Kawashima procedure (KP) between 1992 and 2022, were reviewed. The cumulative incidence of PAVMs was compared between BCPS and KP. Risk factors for the development of PAVMs were identified.

Results

Among 682 patients who underwent S2P, 661 (96.9 %) underwent BCPS and 21 (3.1 %) KP. Median age at S2P was 5.1 (interquartile ranges (IQR): 3.6–9.6) months. During the median interstage follow-up of 1.6 (IQR: 1.6–2.2) years, PAVMs developed in 11 (1.6 %) patients (1.1 % (n = 7) after BCPS and 19.0 % (n = 4) after KP). Cumulative incidence of PAVMs was higher in patients after KP than those after BCPS (p < 0.001). PAVMs were observed in the right lung in 9 patients and both lungs in two. One patient with biliary atresia died of progressive PAVMs and liver cirrhosis after KP, and the remaining 10 patients underwent Fontan completion with a median interval of 1.9 (IQR: 1.5–2.4) years. PAVMs improved in all patients (9 resolutions and 1 improved). Independent risk factors for the development of PAVMs were KP (hazard ratio (HR): 16.364, p < 0.001) in all patients, anomalous pulmonary venous connection (HR: 6.772, p = 0.023) in BCPS patients, and hypoplastic left heart syndrome (HR: 18.819, p = 0.018) in KP patients.

Conclusions

The incidence of PAVMs after S2P is very low after BCPS but still relevant after KP. Resolution or improvement of PAVMs is probable after Fontan completion.

目的评价2期姑息（S2P）后肺动静脉畸形（pavm）的发生率。方法回顾了1992年至2022年间通过双向腔隙肺分流术（BCPS）或Kawashima手术（KP）接受S2P的患者。比较BCPS组和KP组的累计pavm发生率。确定了发生pavm的危险因素。结果682例S2P患者中，661例（96.9%）行BCPS， 21例（3.1%）行KP。S2P时的中位年龄为5.1个月（四分位间距（IQR）： 3.6-9.6个月）。在1.6年（IQR: 1.6 - 2.2）的中位期间随访期间，11例（1.6%）患者出现了pavm，其中BCPS组为1.1% (n = 7)， KP组为19.0% （n = 4）。KP患者的累积pavm发生率高于BCPS患者（p < 0.001）。9例患者右肺出现pavm， 2例患者双肺出现pavm。1例胆道闭锁患者在KP后死于进行性pavm和肝硬化，其余10例患者完成Fontan治疗，中位间隔为1.9 （IQR: 1.5-2.4）年。所有患者的pavm均有所改善（9例缓解，1例改善）。所有患者发生pavm的独立危险因素为KP（风险比（HR）： 16.364, p < 0.001）， BCPS患者肺静脉连接异常（HR: 6.772, p = 0.023）， KP患者左心发育不全综合征（HR: 18.819, p = 0.018）。结论S2P术后pams发生率极低，而KP术后仍有相关性。在丰滩完工后，可能会解决或改善pavm。

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引用次数: 0

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

International journal of cardiology. Congenital heart disease

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

International journal of cardiology. Congenital heart disease

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

Prognostic value of right ventricular-pulmonary arterial coupling in adults with repaired coarctation of aorta 成人主动脉缩窄修复后右心室-肺动脉耦合的预后价值

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-07-03 DOI: 10.1016/j.ijcchd.2025.100608

Alexander C. Egbe, William R. Miranda, Charles Jain, Heidi M. Connolly, Luke J. Burchill, Barry A. Borlaug

Background

Right ventricular (RV) systolic dysfunction and pulmonary hypertension is present in 20 % of adults with repaired coarctation of aorta (COA). However, the prognostic value of RV to pulmonary artery (RV-PA) coupling in this population is unknown. The purpose of this study was to assess the relationship between RV-PA coupling and clinical outcomes (heart failure hospitalization and all-cause mortality) in this population.

Methods

Retrospective cohort study of adults with repaired COA and normal RV systolic function defined as RV free wall strain ≥ -24 %. RV-PA coupling was assessed using tricuspid annular plane systolic excursion/RV systolic pressure (TAPSE/RVSP) ratio.

Results

Of 509 patients (median age 32 [20–45] years; men 290 [57 %]), the average TAPSE and RVSP were 22 ± 5 mm and 33 ± 9 mmHg, respectively, and TAPSE/RVSP ratio was 0.78 (0.56–0.96) mm/mmHg. Of 509 patients, 51 (10 %) died and 43 (8 %) were hospitalized for heart failure during a median follow-up of 8.5 (4.9–10.4) years. TAPSE/RVSP was associated with all-cause mortality (hazard ratio 0.82, 95 % confidence interval 0.78–0.86, per 0.1 mm/mmHg), and heart failure hospitalization (hazard ratio 0.86, 95 % confidence interval 0.79–0.93, per 0.1 mm/mmHg) after adjustment for anatomic lesions, cardiovascular interventions, comorbidities, and echocardiographic indices. The inclusion of TAPSE/RVSP ratio in the risk models improved prognostic power of the models to predict all-cause mortality (C-statistics difference 0.046, p < 0.001), and heart failure hospitalization (C-statistics difference 0.031, p = 0.007).

Conclusions

TAPSE/RVSP ratio was associated with outcomes in COA patients with normal RV systolic function, suggesting that abnormal RV-PA coupling may be present prior to the onset of overt RV systolic function in this population.

背景：20%的成人修复主动脉缩窄（COA）患者存在右心室（RV）收缩功能障碍和肺动脉高压。然而，RV与肺动脉（RV- pa）耦合在该人群中的预后价值尚不清楚。本研究的目的是评估该人群中RV-PA偶联与临床结果（心力衰竭住院和全因死亡率）之间的关系。方法回顾性队列研究成人COA修复，右心室收缩功能正常，右心室游离壁应变≥- 24%。采用三尖瓣环形平面收缩偏移/RV收缩压（TAPSE/RVSP）比评估RV- pa耦合。结果509例患者中位年龄32岁[20-45]；男性290例（57%），平均TAPSE和RVSP分别为22±5 mm和33±9 mmHg， TAPSE/RVSP比值为0.78 (0.56 ~ 0.96)mm/mmHg。509例患者中，51例（10%）死亡，43例（8%）因心力衰竭住院，中位随访时间为8.5年（4.9-10.4年）。在校正解剖病变、心血管干预、合共病和超声心动图指标后，TAPSE/RVSP与全因死亡率（风险比0.82,95%置信区间0.78-0.86，每0.1 mm/mmHg）和心力衰竭住院（风险比0.86,95%置信区间0.79-0.93，每0.1 mm/mmHg）相关。在风险模型中纳入TAPSE/RVSP比值提高了模型预测全因死亡率的预后能力(c -统计差异0.046,p <；心力衰竭住院（c -统计差异0.031,p = 0.007）。结论stapse /RVSP比值与右心室收缩功能正常的COA患者的预后相关，提示该人群在右心室明显收缩功能出现之前可能存在异常的RV- pa耦合。

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引用次数: 0

Trends in congenital heart disease mortality in the United States from 1968 to 2022 1968年至2022年美国先天性心脏病死亡率趋势

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-06-30 DOI: 10.1016/j.ijcchd.2025.100607

Zahra Imran , Taimor Mohammed Khan , Saad Ahmed Waqas , Raheel Ahmed , Abdul Mannan Khan Minhas

Purpose

Congenital heart disease (CHD) remains the leading cause of infant mortality due to birth defects in the United States. We analyzed long-term CHD mortality trends across age, sex, and racial groups from 1968 to 2022.

Methods

We extracted CHD mortality data from the CDC WONDER database (1968–2022), using ICD-8/9/10 codes. Age-adjusted mortality rates (AAMRs) and crude mortality rates (CMRs) were calculated per 100,000 population. Joinpoint regression was used to assess temporal trends in mortality, reporting annual percentage changes (APCs) and average APCs (AAPCs) with 95 % confidence intervals (CIs).

Results

From 1968 to 2019, 234,658 CHD-related deaths were recorded. Overall AAMR declined from 3.2 (1968) to 0.8 (2019) per 100,000 (AAPC: −2.7 %; 95 % CI: −2.9 to −2.5). Males consistently had higher AAMRs than females. Racial disparities persisted, with slower declines among Black or African American individuals. Infants under 1 year accounted for 56.0 % of CHD deaths and showed the steepest mortality decline (AAPC: −3.2 %). Mortality rates plateaued in recent years.

Conclusions

CHD mortality in the U.S. has declined markedly over the past five decades, though progress has slowed since 2009. Persistent disparities by race and sex emphasize the need for equitable access to specialized CHD care and ongoing public health efforts.

目的：在美国，继发性心脏病（CHD）仍然是婴儿因出生缺陷而死亡的主要原因。我们分析了1968年至2022年间不同年龄、性别和种族的冠心病死亡率的长期趋势。方法采用ICD-8/9/10编码，从CDC WONDER数据库中提取冠心病死亡率数据（1968-2022）。计算每10万人的年龄调整死亡率（AAMRs）和粗死亡率（CMRs）。采用联结点回归评估死亡率的时间趋势，报告年百分比变化（APCs）和平均APCs (AAPCs)，置信区间为95% （ci）。结果从1968年到2019年，共记录了234,658例冠心病相关死亡。总体AAMR从每10万人3.2例（1968年）下降到0.8例（2019年）(AAPC: - 2.7%；95% CI:−2.9 ~−2.5)。男性的aamr始终高于女性。种族差异仍然存在，黑人或非裔美国人的下降速度较慢。1岁以下婴儿占冠心病死亡人数的56.0%，死亡率下降幅度最大（AAPC: - 3.2%）。近年来死亡率趋于稳定。结论：在过去的50年里，美国的心脏病死亡率显著下降，尽管自2009年以来进展有所放缓。种族和性别的持续差异强调了公平获得专门的冠心病护理和持续的公共卫生努力的必要性。

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引用次数: 0

Late diagnosis of severe aortic coarctation in a 65-year-old endurance athlete: Insights into exercise physiology and collateral compensation 65岁耐力运动员重度主动脉缩窄的晚期诊断：运动生理学和侧支代偿的见解

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-06-30 DOI: 10.1016/j.ijcchd.2025.100606

Arundhati Sharma , Anubhuti Sharma , Kalyan Chaliki , Marco Antonio Fernandes , Francois Marcotte , Satyajit Reddy

Background

Coarctation of the aorta (CoA) is a relatively rare congenital narrowing of the distal aortic arch to proximal descending thoracic aorta, usually diagnosed in infancy or early childhood with upper extremity hypertension as a typical finding. Late detection in asymptomatic, physically active older adults is rare and can be clinically significant.

Case presentation

We present the case of a 65-year-old lifelong endurance athlete found to have transient severe left ventricular (LV) systolic dysfunction after completing a marathon. Stress testing revealed a hypertensive response, prompting cardiac MRI, which detected a new severe juxta-ductal CoA with collateralization. The patient underwent successful endovascular stenting with improved blood pressure response and stabilization of LV function.

Conclusion

This case underscores the importance of thorough cardiovascular assessment in older athletes and highlights the impact of lifelong exercise in compensating for congenital defects like CoA.

背景主动脉缩窄（CoA）是一种相对罕见的先天性主动脉弓远端至近段胸降主动脉狭窄，通常在婴儿期或幼儿期诊断，典型表现为上肢高血压。在无症状、体力活动的老年人中，晚期发现是罕见的，可能具有临床意义。病例介绍：我们报告了一位65岁的终身耐力运动员，在完成马拉松后发现有短暂的严重左心室收缩功能障碍。压力测试显示高血压反应，促使心脏MRI检查发现新的严重导管旁CoA伴侧支。患者成功接受血管内支架植入术，血压反应改善，左室功能稳定。结论本病例强调了对老年运动员进行全面心血管评估的重要性，并强调了终身运动对补偿先天性缺陷（如CoA）的影响。

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引用次数: 0

Clinical outcomes of children operated for congenital heart diseases in Rwanda: a 14-year retrospective analysis 卢旺达先天性心脏病患儿手术治疗的临床结果：14年回顾性分析

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-06-28 DOI: 10.1016/j.ijcchd.2025.100605

Providence Akingeneye , David J. Bradley , Joseph Mucumbitsi , Naphtal Nyilimanzi , Yves Mutabandama , Emmanuel K. Rusingiza

Background

Congenital heart disease surgery and catheterization for Rwandan children has historically been accomplished with the assistance of visiting external teams and referrals abroad. However, follow-up information on patient-specific clinical outcomes is lacking.

Methods

This is a retrospective cross-sectional study of clinical outcomes among Rwandan children under 15 years of age at the time of heart surgery operated between 2006 and 2020. Data from two Rwandan teaching hospitals were collected from paper and digital patient charts.

Results

Among the 406 children included in our study, 59.1 % were female; 30.5 % and 28.1 % of the children were underweight and stunted, respectively. Tetralogy of Fallot was the most common cyanotic CHD. Of procedures, VSD repair was the most performed operation and PDA closure represented the majority of therapeutic catheterizations. Postoperatively complications such as pneumonia occurred in 6 %, residual shunt in 17 % and residual mild or greater stenosis in 41 %. The overall mortality was 3.7 %, with sepsis as the leading cause of death. Intensive care unit stay longer than seven days, symptomatic heart failure before surgery, and being underweight were significantly associated with complications. Cardiopulmonary bypass over 60 min was associated with mortality among operated children.

Conclusion

Procedural treatment of CHD in Rwandan children was predominantly accomplished by visiting missions during the study period. Of patients with sufficient clinical follow up data, survival was 96 % and complications occurred in 35 %. Nutritional compromise is a correlate of poor outcome. A centralized cardiac registry might mitigate the observed loss of clinical follow up data in 14 % of children.

历史上，卢旺达儿童的先天性心脏病手术和导管术是在访问外部团队和国外转诊的帮助下完成的。然而，缺乏患者特异性临床结果的随访信息。方法：这是一项回顾性横断面研究，对2006年至2020年期间卢旺达15岁以下儿童进行心脏手术的临床结果进行研究。来自两家卢旺达教学医院的数据从纸质和数字患者图表中收集。结果纳入研究的406例患儿中，女性占59.1%；30.5%的儿童体重不足，28.1%的儿童发育不良。法洛四联症是最常见的紫绀型冠心病。在手术中，室间隔缺损修复是最常见的手术，PDA关闭是大多数治疗性导管。术后并发症如肺炎的发生率为6%，残留分流的发生率为17%，残留轻度或较大狭窄的发生率为41%。总死亡率为3.7%，败血症是主要死亡原因。重症监护病房住院时间超过7天、术前症状性心力衰竭和体重过轻与并发症显著相关。超过60分钟的体外循环与手术儿童的死亡率相关。结论在研究期间，卢旺达儿童冠心病的程序性治疗主要通过访问特派团完成。在临床随访资料充足的患者中，生存率为96%，并发症发生率为35%。营养不良与不良结果有关。集中的心脏登记可能会减轻14%儿童临床随访数据的缺失。

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引用次数: 0

Kawasaki disease with persistent severe psoriatic-like skin manifestations and sudden cardiac death 川崎病伴有持续严重的银屑病样皮肤表现和心源性猝死

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-06-26 DOI: 10.1016/j.ijcchd.2025.100604

Aso F. Salih , Trifa M. Qadir , Nagib Dahdah

This case report describes a 1.3-year-old infant with an atypical presentation of Kawasaki Disease (KD) following exposure to COVID-19 within the family and positive SARS-CoV-2 IgG antibodies. The clinical course was marked by significant left ventricular (LV) dysfunction, progressive coronary artery involvement, and complicated by severe dermatologic manifestations including Steven-Johnson-like lesions and psoriatic-like eruptions. Despite initial improvement in cardiac function, the infant developed multiple coronary aneurysms and succumbed to sudden cardiac death. This case highlights the diagnostic and therapeutic challenges in managing atypical KD in the context of COVID-19. We describe the case and review the literature related to the associated severe skin lesions.

本病例报告描述了一名1.3岁婴儿在家庭成员暴露于COVID-19和SARS-CoV-2 IgG抗体阳性后出现不典型川崎病（KD）的症状。临床表现为明显的左心室功能障碍，进行性冠状动脉受累，并伴有严重的皮肤症状，包括史蒂文-约翰逊样病变和银屑病样皮疹。尽管最初心脏功能有所改善，但婴儿出现了多个冠状动脉瘤并死于心源性猝死。该病例强调了在COVID-19背景下管理非典型KD的诊断和治疗挑战。我们描述的情况下，回顾文献相关的严重皮肤病变。

引用次数: 0

Echocardiographic effects of sodium-glucose cotransporter 2 inhibitors in single ventricle circulatory failure 钠-葡萄糖共转运蛋白2抑制剂对单心室循环衰竭的超声心动图影响

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-06-21 DOI: 10.1016/j.ijcchd.2025.100603

Ralph M.L. Neijenhuis , Madelien V. Regeer , Niki L. Walker , Amanda Hunter , Philippine Kiès , Eduard R. Holman , J. Wouter Jukema , Monique R.M. Jongbloed , Gruschen R. Veldtman , Anastasia D. Egorova

Background

Single ventricle patients are at high risk of developing circulatory failure. There is limited evidence for pharmacological treatment. This study assessed the echocardiographic changes in ventricular function during sodium-glucose cotransporter 2 inhibitor (SGLT2i) therapy in patients with single ventricle failure (SVF).

Methods

SVF patients with a baseline transthoracic echocardiogram within six months before starting SGLT2i and at least one echocardiographic examination within twelve months follow-up were included from a real-world international registry of adult congenital heart disease patients on SGLT2i. Mixed models were used to evaluate longitudinal changes in ventricular function and differences between patients with SVF with ≥ moderately reduced systolic function (SVFrEF) and with ≤ mildly reduced function (SVFpEF).

Results

Thirteen patients were included. The median age was 21 [20–42] years, 8 (61.5 %) were female, 10 (76.9 %) had a Fontan circulation, 8 (61.5 %) had SVFrEF, and 5 (38.5 %) SVFpEF at the start of SGLT2i. The mean follow-up was 7.6 ± 3.3 months. End-systolic area decreased significantly in all patients (−1.6 cm² per month, p = 0.007) in the first 100 days. Fractional area change improved in the first 100 days in SVFrEF patients (3.5 %-point per month, p < 0.001), while SVFpEF patients remained stable. There was a significant improvement in the free wall strain in all patients (−0.3 %-point per month, p = 0.036) but not in global longitudinal strain (p = 0.087). Isovolumic acceleration also improved in the first 100 days (0.5 m/s² per month, p = 0.010).

Conclusions

Echocardiographic signals of improved ventricular function were observed in the first year of SGLT2i therapy in patients with SVF.

背景：单心室患者发生循环衰竭的风险很高。药物治疗的证据有限。本研究评估了单心室衰竭（SVF）患者在钠-葡萄糖共转运蛋白2抑制剂（SGLT2i）治疗期间心室功能的超声心动图变化。方法选取SGLT2i治疗前6个月内接受基线经胸超声心动图检查的ssvf患者，并在随访12个月内进行至少一次超声心动图检查。采用混合模型评价收缩功能中度降低（SVFrEF）和≤轻度降低（SVFpEF）的SVF患者心室功能的纵向变化和差异。结果纳入13例患者。中位年龄为21岁[20-42]岁，女性8例（61.5%），Fontan循环10例（76.9%），SGLT2i发病时SVFrEF 8例（61.5%），SVFpEF 5例（38.5%）。平均随访7.6±3.3个月。所有患者在前100天收缩末期面积均显著减少（- 1.6 cm2 /月，p = 0.007）。SVFrEF患者的部分面积变化在前100天得到改善(每月3.5%,p <；0.001)，而SVFpEF患者保持稳定。所有患者的游离壁应变均有显著改善（- 0.3% -点/月，p = 0.036），但整体纵向应变无显著改善（p = 0.087）。等体积加速度在前100天也有所改善（每月0.5 m/s2, p = 0.010）。结论在SGLT2i治疗SVF患者的第一年，可观察到心室功能改善的心电图信号。

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引用次数: 0

Trends in mortality from pulmonary hypertension amongst population with congenital heart disease in the United States from 1999 to 2020: A CDC WONDER analysis 1999年至2020年美国先天性心脏病患者肺动脉高压死亡率趋势：CDC WONDER分析

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology. Congenital heart disease

Pub Date : 2025-06-21 DOI: 10.1016/j.ijcchd.2025.100602

Allahdad Khan , Tehreem Asghar , Kumail Mustafa Ali , Muhammad Saeed , Saniya Ishtiaq , Fatima Ashfaq , Jamil Nasrallah , Laiba Yumn , Usman Ali Akbar , Peter Collins , Raheel Ahmed

Background

Congenital heart disease (CHD) is an umbrella term describing a variety of structural cardiac malformations at birth. CHD affects approximately 1 % of live births, generating a large adult population with these abnormalities. Pulmonary hypertension (PH) in patients with adult CHD is heterogeneous, based on the type of defect and associated conditions, but is a known cause of adverse outcome.

Method

We retrieved death certificate data from the CDC-WONDER database using ICD codes (I27.0, I27.2, I27.8, and I27.9., and Q20-26). Crude mortality rates (CMRs) and age-adjusted mortality rates (AAMRs) per 100,000 persons were calculated. Temporal trends were examined using the annual percent change (APC) and average annual percent change (AAPC) determined by Joinpoint regression.

Result

From 1999 to 2020, an overall declining pattern was observed in the mortality rate. Men and women with congenital heart disease and pulmonary hypertension in the U.S. experienced a statistically significant decline in mortality rates. In terms of races, among White individuals, the decline was the most pronounced. All four U.S. census regions experienced statistically significant declines in mortality due to pulmonary hypertension among individuals with congenital heart disease. The rate of decline was steeper in rural areas compared to urban ones.

Conclusion

This study highlights that previously implemented targeted interventions significantly contributed to the reduction of mortality amongst patiemnts with congenital heart disease and pulmonary hypertension in the U.S. Still improvements are required in certain areas, including female gender, Hispanic or Latino, and the Northeast and West regions.

先天性心脏病（CHD）是描述出生时各种结构性心脏畸形的总称。冠心病影响约1%的活产婴儿，导致大量成年人患有这些异常。成人冠心病患者的肺动脉高压（PH）是异质性的，基于缺陷的类型和相关条件，但它是已知的不良结果的原因。方法使用ICD代码（I27.0、I27.2、I27.8和I27.9）从CDC-WONDER数据库中检索死亡证明数据。Q20-26)。计算了每10万人的粗死亡率（cmr）和年龄调整死亡率（AAMRs）。采用年变化百分数（APC）和平均年变化百分数（AAPC）进行时间趋势分析。结果1999 - 2020年，死亡率总体呈下降趋势。在美国，患有先天性心脏病和肺动脉高压的男性和女性的死亡率在统计上有显著下降。就种族而言，在白人中，这种下降最为明显。在美国所有四个人口普查地区，先天性心脏病患者因肺动脉高压导致的死亡率在统计上都有显著下降。与城市地区相比，农村地区的下降速度更快。结论：本研究强调，先前实施的有针对性的干预措施显著有助于降低美国先天性心脏病和肺动脉高压患者的死亡率，但在某些地区仍需改进，包括女性、西班牙裔或拉丁裔以及东北和西部地区。

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