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Review of cardiovascular complications in children with sickle cell disease 儿童镰状细胞病心血管并发症综述

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2025-05-01 DOI: 10.1016/j.ppedcard.2025.101836

Saswat Kumar Jha , Linhan Jasmine Ha , Maxon Bassett , Avery Silar , Brianna L. Gilner , Induja Gajendran , Preethi Marri

Background

Cardiovascular complications in pediatric patients with sickle cell disease may be life-threatening.

Aim of review

The purpose of this article is to review cardiovascular problems associated with sickle cell disease in children and adolescents, including pathophysiology, clinical presentation, diagnosis, and treatment.

Key scientific concepts of review

Sickle cell disease is a hemoglobinopathy that causes red blood cell sickling and vaso-occlusive crises. Chronic anemia may cause high-output cardiac failure. Other cardiovascular abnormalities include pulmonary hypertension, increased tricuspid regurgitation velocity, increased right ventricular pressure, cardiomyopathy with left ventricular hypertrophy and dysfunction, and increased risk of myocardial infarction, dysrhythmia, and sudden cardiac death. Patients may have chronic hemolytic anemia, red blood cell sickling, vaso-occlusive crises, and cardiovascular symptoms including chest pain, palpitations, shortness of breath, and fatigue. Cardiovascular complications also may include stroke, acute renal failure, and priapism. Diagnosis includes family history and diagnostic tests such as complete blood count, electrocardiography, echocardiography, chest radiography, and pulmonary function tests. Treatment of sickle cell disease may include hydroxyurea and treatment of complications, including pain from vaso-occlusive crises and cardiovascular disease. Early detection and intervention are important to limit sequelae of cardiovascular complications, and regular cardiovascular screening in pediatric patients with sickle cell disease is recommended.

儿童镰状细胞病患者的心血管并发症可能危及生命。本文的目的是回顾与儿童和青少年镰状细胞病相关的心血管问题，包括病理生理、临床表现、诊断和治疗。评述的关键科学概念镰状细胞病是一种导致红细胞镰状和血管闭塞危象的血红蛋白病。慢性贫血可引起高输出量心力衰竭。其他心血管异常包括肺动脉高压、三尖瓣反流速度增加、右心室压力增加、心肌病伴左心室肥厚和功能障碍、心肌梗死、心律失常和心源性猝死的风险增加。患者可能有慢性溶血性贫血、红细胞镰状坏死、血管闭塞危象和心血管症状，包括胸痛、心悸、呼吸短促和疲劳。心血管并发症还可能包括中风、急性肾功能衰竭和勃起功能亢进。诊断包括家族史和诊断检查，如全血细胞计数、心电图、超声心动图、胸片和肺功能检查。镰状细胞病的治疗可能包括羟基脲和并发症的治疗，包括血管闭塞危象和心血管疾病引起的疼痛。早期发现和干预对于限制心血管并发症的后遗症非常重要，建议镰状细胞病患儿定期进行心血管筛查。

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引用次数: 0

Aortic arch dimensions in neonates with Down syndrome 唐氏综合征新生儿主动脉弓的尺寸

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2025-04-30 DOI: 10.1016/j.ppedcard.2025.101835

Abhishek Chakraborty , Mohanageetha Ardhanari , Marjorie Gayanilo , Hanah Walker , Lindsey Bird , Nao Sasaki , Sethuraman Swaminathan

Background

Congenital heart disease (CHD) is a major cause of morbidity and mortality in patients with Down syndrome.

Objectives

The objective of this study is to identify the prevalence of abnormal aortic arch dimensions in patients with Down syndrome.

Methods

This was a retrospective chart review of patients with Down syndrome who were born at Jackson Health System over a 14-year period. The initial neonatal echocardiograms were reviewed for presence of CHD along with the dimensions of aortic annulus, distal transverse arch, and aortic isthmus, and then compared with age and sex matched cohorts.

Results

A total of 138 patients were included in this study. The overall prevalence of congenital heart disease in our cohort was 70.7 % (n = 97). Among those identified, the most common CHDs were ventricular septal defect (VSD) (23.2 %), atrial septal defect (ASD) (24.6 %), and atrioventricular septal defect (AVSD) (34.8 %) Fourteen patients (10.1 %) had aortic isthmus hypoplasia and 9 (6.5 %) had hypoplasia of the distal transverse arch.

Conclusion

Mild aortic arch hypoplasia without coarctation is found in 13.8 % of patients with Down syndrome. Specific nomograms may be necessary for measuring aortic arch dimensions in patients with Trisomy 21.

背景先天性心脏病（CHD）是唐氏综合征患者发病和死亡的主要原因。目的本研究的目的是确定唐氏综合征患者主动脉弓尺寸异常的患病率。方法回顾性分析在杰克逊卫生系统出生的唐氏综合症患者14年的数据。回顾新生儿初始超声心动图是否存在冠心病，以及主动脉环、远端横弓和主动脉峡的尺寸，然后与年龄和性别匹配的队列进行比较。结果本研究共纳入138例患者。我们队列中先天性心脏病的总患病率为70.7% （n = 97）。其中，最常见的冠心病是室间隔缺损（VSD）（23.2%）、房间隔缺损（ASD）（24.6%）和房室间隔缺损（AVSD）（34.8%）。14例（10.1%）存在主动脉峡部发育不全，9例（6.5%）存在远端横弓发育不全。结论13.8%的唐氏综合征患者存在轻度主动脉弓发育不全且无缩窄。在21三体患者中，测量主动脉弓的尺寸可能需要特殊的形态图。

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引用次数: 0

Risk of neurodevelopmental delay in children with congenital heart defects: Hospital based prospective cross-sectional study 先天性心脏缺陷儿童神经发育迟缓的风险：基于医院的前瞻性横断面研究

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2025-04-23 DOI: 10.1016/j.ppedcard.2025.101832

Tesfaye Taye Gelaw, Shitahun Fentie Tilahun

Background

The effect of congenital heart defects (CHDs) on adverse neurodevelopment has been reduced with modern medical services in high-income countries. This is in contrast to low-and-middle-income countries where the benefits of modern medical advantages are practically non-existent.

Objective

Determine the risk of developmental delay in children with CHD and examine associated factors.

Method

A hospital-based prospective cross-sectional study was conducted on 288 children from 2 to 60 months of age with CHDs. Data were collected prospectively from August 15, 2024 – January 15, 2025. Development assessment was conducted using the “CDC's Milestone Moments booklet.” Logistic regression was performed to see the association of independent variables with the dependent variable – development delay. A P-value of ≤0.05 was considered significant. SPSS version 27 was used for analysis.

Result

Of the studied children with CHDs, 51 % were females. The mean (SD) age was 18 months (16). Delayed development was seen in 46 % of the studied children. The motor domain is the most affected development domain (42 %). Rural residency {AOR = 2.21, 95 % CI = (1.08, 4.53)}, suspected genetic/syndromic comorbidity {AOR = 18.92, 95 % CI = (7.91, 45.24)}, severe stunting {AOR = 5.91, 95 % CI = (2.67, 13.11)} and dextro-transposition of great arteries (d-TGA) {AOR = 58.56, 95 % CI = (4.97, 689.6)} were statistically significant associated factors.

Conclusion

The risk of neurodevelopmental delay is a common problem in children with CHD. Suspected genetic/syndromic comorbidities, severe stunting, rural residency, and d-TGA are statistically significantly associated factors with neurodevelopmental delay in a developing nation.

高收入国家的现代医疗服务已经降低了先天性心脏缺陷（CHDs）对神经发育不良的影响。这与低收入和中等收入国家形成鲜明对比，在这些国家，现代医疗优势的好处实际上是不存在的。目的探讨冠心病患儿发育迟缓的风险及相关因素。方法对288例2 ~ 60月龄冠心病患儿进行前瞻性横断面研究。数据收集时间为2024年8月15日至2025年1月15日。开发评估是使用“CDC的里程碑时刻小册子”进行的。采用Logistic回归分析自变量与因变量发育滞后之间的关系。p值≤0.05被认为是显著的。采用SPSS第27版进行分析。结果在所研究的冠心病患儿中，51%为女性。平均（SD）年龄为18个月（16）。46%的被研究儿童发育迟缓。运动领域是最受影响的发展领域（42%）。农村居住{AOR = 2.21, 95% CI =(1.08, 4.53)}、疑似遗传/综合征合并症{AOR = 18.92, 95% CI =(7.91, 45.24)}、严重发育迟缓{AOR = 5.91, 95% CI =(2.67, 13.11)}和大动脉右转（d-TGA） {AOR = 58.56, 95% CI =(4.97, 689.6)}是具有统计学意义的相关因素。结论神经发育迟缓是冠心病患儿的常见问题。在发展中国家，疑似遗传/综合征合并症、严重发育迟缓、农村居住和d-TGA是与神经发育迟缓有统计学意义的相关因素。

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引用次数: 0

Performance of first-trimester fetal nuchal translucency, ductus venosus, tricuspid regurgitation in diagnosing congenital heart defects: A systematic review and meta-analysis 早期妊娠胎儿颈透明、静脉导管、三尖瓣反流诊断先天性心脏缺陷的表现：一项系统回顾和荟萃分析

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2025-04-23 DOI: 10.1016/j.ppedcard.2025.101833

Behnaz Moradi , Payam Jannatdoust , Paria Shafiekhani , Mohamadreza Tahamtan , Gita Manzari Tavakoli , Parya Valizadeh , Pouya Ebrahimi , Niloofarsadaat Eshaghhosseiny , Farzan Moodi , Hamed Ghorani , Parisa Kohnepoushi , Mahshad Razaghi , Morteza Sanei Taheri , Delaram J. Ghadimi

Background

Congenital heart disease (CHD) is the main cause of perinatal morbidity and mortality. Nuchal Translucency (NT), Ductus Venosus (DV), and Tricuspid Regurgitation (TR) have shown potential in CHD detection.

Aim of review

We evaluated the pooled diagnostic test accuracy of these markers during the first-trimester screening.

Key scientific concepts of review

PubMed, Scopus, Web of Science, and Embase were searched. A bivariate random effects model created Summary Receiver Operating Characteristic (SROC) curves and the pooled sensitivities and specificities. Forty-two studies were included. For major CHDs, the pooled sensitivities and specificities were 43.1 % (95 % CI: 35.0 %–51.6 %) and 95.5 % (95 % CI: 93.5 %–96.9 %) for A/R DV a wave, 57.8 % (95 % CI: 43.3 %–71.0 %) and 88.8 % (95 % CI: 77.7 %–94.7 %) for abnormal DV-PIV, 37.0 % (95 % CI: 26.6 %–48.6 %) and 97.7 % (95 % CI: 94.6 %–99.1 %) for TR, 41.4 % (95 % CI: 23.2 %–62.2 %) and 93.7 % (95 % CI: 92.7 %–94.6 %) for NT > 95th percentile, and 26.6 % (95 % CI: 11.0 %–51.7 %) and 98.3 % (95 % CI: 97.5 %–98.9 %) for NT > 99th percentile. For the combined models in detecting major CHDs, the highest specificity of 97.8 % (95 % CI: 93.9 %–99.2 %) belonged to NT > 95th percentile and A/R DV a wave. The most sensitive tests were the combination of NT > 95th percentile or A/R DV a wave or TR 61.4 % (95 % CI: 49.7 %–71.9 %). Combining increased NT with the presence of A/R a-wave can help diagnose CHD, while normal NT, A/R DV a wave, and TR indicate lower CHD risk.

背景先天性心脏病（CHD）是围产期发病和死亡的主要原因。颈透明（NT）、静脉导管（DV）和三尖瓣返流（TR）显示出冠心病检测的潜力。回顾目的：我们评估了这些标志物在妊娠早期筛查时的综合诊断测试的准确性。检索了reviewPubMed、Scopus、Web of Science和Embase的关键科学概念。双变量随机效应模型建立了总接受者工作特征（SROC）曲线和合并的敏感性和特异性。纳入了42项研究。主要的冠心病,合用的敏感性和特异性分别为43.1% (95% CI: 35.0% - -51.6%)和95.5% (95% CI: 93.5% - -96.9%) / R DV波,57.8% (95% CI: 43.3% - -71.0%)和88.8% (95% CI: 77.7% - -94.7%) DV-PIV异常,37.0% (95% CI: 26.6% - -48.6%)和97.7% (95% CI: 94.6% - -99.1%) TR, 41.4% (95% CI: 23.2% - -62.2%)和93.7% (95% CI: 92.7% - -94.6%) NT祝辞第95个百分位,26.6% (95% CI: 11.0% - -51.7%)和98.3% (95% CI: 97.5% - -98.9%) NT祝辞第99个百分位。在检测主要冠心病的联合模型中，NT >的特异性最高，为97.8% (95% CI: 93.9% ~ 99.2%)；95百分位和A/R DV A波。最敏感的试验是NT >联合试验；第95百分位或A/R DV A波或TR 61.4% （95% CI: 49.7% - 71.9%）。NT升高合并A/R A波有助于诊断冠心病，而NT、A/R DV A波和TR正常则提示冠心病风险较低。

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引用次数: 0

Differences in school preparedness for sudden cardiac arrest based on automated external defibrillator availability and staff training between schools of different enrollment size 基于自动体外除颤器可用性和不同招生规模学校员工培训的学校心脏骤停准备的差异

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2025-04-17 DOI: 10.1016/j.ppedcard.2025.101831

Ryan Kimball , Isaac J. Jacobs , Matthew W. Sorensen , Jeffrey A. Robinson

Background

A school has a 1–2 % chance of having a sudden cardiac arrest (SCA) on its campus in any given year. Schools can be prepared by having automated external defibrillators (AEDs) readily accessible with staff trained in their use. The overall survival rate of SCA is less than 10 %. However, those who suffer SCA at a school that has an AED on-site have more than a 60 % chance of surviving to discharge. Currently, AEDs are not required in schools across all 50 states in the US.

Objective

Investigate the availability of AEDs in high schools across a state prior to any formal mandate and explore discrepancies among schools of varying enrollment sizes.

Methods

A 16-question survey about AED availability, accessibility, and percentage of staff trained for its use was distributed to every high school in Nebraska in July 2022.

Results

Schools with smaller enrollments were more likely to have AEDs accessible to the public (p = 0.047), available at all events (p = 0.0002), and over 50 % of staff trained for its use (p = 0.00019) than schools with larger enrollments. There was no correlation between number of AEDs and enrollment (R² = 0.172).

Conclusions

Most high schools have automated external defibrillators (AEDs), even without statewide mandates; however, continuing efforts are needed to ensure that AEDs at all schools are accessible to the public and that staff are appropriately trained. This study highlights the need for increased advocacy and targeted resource allocation, particularly for larger schools, to ensure appropriate/adequate AED distribution and emergency preparedness across all high schools.

在任何一年中，一所学校都有1 - 2%的机会在校园内发生心脏骤停（SCA）。学校可以准备好配备自动体外除颤器（aed），并配备经过使用培训的工作人员。SCA的总生存率低于10%。然而，那些在有AED的学校里患SCA的人有超过60%的机会活到出院。目前，美国所有50个州的学校都不要求使用除颤器。目的调查在任何正式授权之前，在一个州的高中使用aed的情况，并探讨不同招生规模的学校之间的差异。方法于2022年7月向内布拉斯加州的每所高中分发了一份关于AED的可获得性、可获得性和培训人员使用AED的百分比的16个问题的调查。结果招生人数较少的学校更有可能向公众提供aed (p = 0.047)，在任何情况下都可以使用（p = 0.0002），超过50%的员工接受过使用aed的培训（p = 0.00019）。aed数量与入组人数无相关性（R2 = 0.172）。大多数高中都有自动体外除颤器（aed），即使没有全州范围的强制规定；然而，需要继续努力确保公众可以在所有学校使用除颤器，并确保工作人员得到适当的培训。这项研究强调需要加强宣传和有针对性的资源分配，特别是对规模较大的学校，以确保在所有高中分发适当/充分的AED和应急准备。

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引用次数: 0

Transcatheter occlusion of the neoaorta to augment mechanical circulatory support after stage 1 single ventricle palliation 经导管阻断新主动脉以增强1期单心室缓和后的机械循环支持

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2025-04-01 DOI: 10.1016/j.ppedcard.2025.101830

Chelsea Miller, Michael J. Shorofsky, Michael Hainstock

Aortic insufficiency can be a complication of continuous mechanical circulatory support that leads to inefficient circulation and decreased coronary perfusion. In adults, this has been addressed through both surgical and transcatheter approaches. However, this has rarely been reported in children with congenital heart disease. We present the case of a neonate who developed severe neoaortic insufficiency after stage one single ventricle palliation while on central veno-arterial extracorporeal membrane oxygenation (VA-ECMO) that was managed with transcatheter neoaortic occlusion. We used an Amplatzer Vascular Plug II 10 mm device (Abbott Cardiovascular) to successfully improve neoaortic insufficiency and overall hemodynamics. This procedure was technically feasible and may be considered in rare cases to address aortic insufficiency while on mechanical circulatory support.

主动脉功能不全可能是持续机械循环支持的并发症，导致循环效率低下和冠状动脉灌注减少。在成人中，这已经通过手术和经导管入路来解决。然而，这在患有先天性心脏病的儿童中很少有报道。我们提出的情况下，一个新生儿谁发展严重的新主动脉功能不全后一期单心室姑息，而中央静脉-动脉体外膜氧合（VA-ECMO）是经导管处理的新主动脉闭塞。我们使用Amplatzer血管塞II 10毫米装置（雅培心血管公司）成功改善新主动脉功能不全和整体血流动力学。该手术在技术上是可行的，在极少数情况下可以考虑在机械循环支持下解决主动脉功能不全。

引用次数: 0

Hypothermia vs normothermia in neonatal arterial switch operation – Clinical outcomes and myocardial preservation 新生儿动脉开关手术中的低温与常温-临床结果和心肌保存

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2025-03-20 DOI: 10.1016/j.ppedcard.2025.101829

Uri Pollak , Asaf Mandel , Yshia Langer , Salmas Watad , Hiba Abuelhija , Yshai Salem , Hai Zemmour , Eitan Keizman , David Mishaly , Alain E. Serraf

Background

Cardiopulmonary bypass (CPB) management during pediatric cardiac surgery represents a critical aspect of optimizing patient outcomes. Historically, the arterial switch operation (ASO) for transposition of the great arteries (TGA) was performed using hypothermic CPB, but growing evidence suggests potential benefits of normothermic approaches.

Objectives

This study aimed to compare clinical outcomes and myocardial preservation between hypothermic (HT-CPB) and normothermic (NT-CPB) temperature management strategies during neonatal ASO, with special emphasis on cardiomyocyte injury assessed through tissue-specific cell-free DNA (cfDNA).

Methods

This retrospective observational study analyzed data from 27 neonates (12 HT-CPB, 15 NT-CPB) who underwent ASO between July 2017 and December 2018. Clinical parameters were compared between groups, including CPB duration, cross-clamp time, postoperative cardiac biomarkers, and cfDNA trajectories as markers of cellular damage.

Results

The NT-CPB group demonstrated significantly shorter CPB (71.93 ± 28.49 vs. 116.00 ± 20.15 min, p < 0.001) and cross-clamp times (54.73 ± 32.24 vs. 96.92 ± 21.66 min, p < 0.001). Maximal lactate (51.00 ± 18.11 vs. 65.33 ± 12.43 mg/dL, p = 0.028) and Troponin-I levels (10.57 ± 2.83 vs. 15.37 ± 1.55 ng/dL, p < 0.001) were significantly lower in the NT-CPB group in oppose to higher incidence of AKI. While total cfDNA levels were comparable between groups at most time points, cardiac cfDNA was significantly lower in the NT-CPB group at 24 h post-surgery (p = 0.002).

Conclusion

Normothermia during neonatal ASO was associated with shorter operative times, lower metabolic stress, and reduced myocardial injury compared to hypothermia. These findings support the non-inferiority of normothermia as a viable and potentially beneficial temperature management strategy for neonatal ASO, offering comparable safety with improved hemodynamic and myocardial preservation outcomes.

背景：小儿心脏手术期间的体外循环（CPB）管理是优化患者预后的关键方面。历史上，大动脉转位（TGA）的动脉转换手术（ASO）是使用低温CPB进行的，但越来越多的证据表明常温入路的潜在益处。本研究旨在比较新生儿ASO期间低温（HT-CPB）和常温（NT-CPB）温度管理策略的临床结果和心肌保存情况，特别强调通过组织特异性无细胞DNA （cfDNA）评估心肌细胞损伤。方法回顾性观察分析2017年7月至2018年12月27例接受ASO手术的新生儿（12例HT-CPB， 15例NT-CPB）的数据。比较两组间的临床参数，包括CPB持续时间、交叉钳夹时间、术后心脏生物标志物和作为细胞损伤标志物的cfDNA轨迹。结果NT-CPB组CPB时间明显缩短(71.93±28.49 min vs 116.00±20.15 min, p <；0.001)和交叉夹夹时间(54.73±32.24 vs. 96.92±21.66 min, p <；0.001)。最大乳酸（51.00±18.11 vs. 65.33±12.43 mg/dL, p = 0.028）和肌钙蛋白- i水平(10.57±2.83 vs. 15.37±1.55 ng/dL, p <；0.001)，与较高的AKI发生率相反，NT-CPB组的AKI发生率显著降低。虽然在大多数时间点，两组之间的总cfDNA水平具有可比性，但NT-CPB组在术后24小时的心脏cfDNA水平显著降低（p = 0.002）。结论与低体温相比，常温可缩短新生儿ASO手术时间，降低代谢应激，减少心肌损伤。这些研究结果支持常温无劣效性作为新生儿ASO可行且潜在有益的温度管理策略，具有相当的安全性和改善的血流动力学和心肌保存结果。

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引用次数: 0

Cor triatrium dexter as a rare cause of central cyanosis: A case report 右心房三室心是一种罕见的中枢性紫绀的病因：1例报告

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2025-03-19 DOI: 10.1016/j.ppedcard.2025.101826

Hend A. Khalil , Rami A. Sabri , Alaa Abdorahman , Mohamed-Magdy Abuelkheir , Hany M. Abo-Haded

Cor triatrium dexter (CTD) is considered a rare congenital heart disease and accounts for 0.1 % of cardiac malformations. This condition occurred due to failure of regression of the right venous valve at the opening of the sinoatrial orifice in the right atrium, dividing the right atrium into 2 parts and forming a tri-atrial heart.

Case presentation: A 7-year-old child presented with fatigue and exertional dyspnea. Examination revealed oxygen saturation of 85 % and an ejection systolic murmur over the pulmonary area. Echocardiography showed moderate valvular pulmonary stenosis and a suspected membrane in the right atrium, while transesophageal echocardiography revealed a high secundum atrial septal defect (ASD) with bidirectional flow and a non-obstructive membrane in the right atrium, CTD. The patient underwent successful surgical closure of the ASD and resection of the CTD, resulting in improved oxygen saturation (98 %) and significant clinical improvement.

Conclusion, Cor triatriatum dexter is a rare congenital cardiac anomaly that can be missed on transthoracic echocardiography despite being a potential cause of central cyanosis.

心房右三房（CTD）是一种罕见的先天性心脏病，占心脏畸形的0.1%。这种情况的发生是由于右心房窦房孔开口处的右静脉瓣膜退行失败，将右心房分成两部分，形成三房心。病例介绍：一名7岁儿童表现为疲劳和用力性呼吸困难。检查显示血氧饱和度85%，肺区有射血性收缩期杂音。超声心动图示中度瓣膜性肺动脉狭窄，右心房疑有膜，经食管超声心动图示高窦性房间隔缺损（ASD）伴双向血流，右心房非阻塞性膜（CTD）。患者成功完成了ASD的手术闭合和CTD的切除，改善了血氧饱和度（98%）和显著的临床改善。结论：右三心房是一种罕见的先天性心脏异常，尽管它可能是中枢性紫绀的潜在原因，但在经胸超声心动图上却可能被遗漏。

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引用次数: 0

Investigating cardiopulmonary complications in cirrhotic pediatrics with contrast echocardiography 对比超声心动图研究肝硬化儿科的心肺并发症

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2025-03-18 DOI: 10.1016/j.ppedcard.2025.101828

Pejman Rohani , Elahe Motamedi , Arian Kariman , Koroush Vahidshahi , Maryam Fathi Khorasani , Mohammad Hassan Sohouli

Background

Liver cirrhosis is a rare but serious form of liver dysfunction that can lead to severe complications including cardiopulmonary involvement in children.

Objectives

In this study, our aim is to investigate the cardiopulmonary complications in children with cirrhosis by using contrast echocardiography.

Method

In this observational cross-sectional study, all pediatrics diagnosed with cirrhosis were enrolled from 2020 to 2024. Demographic data, weight, height, BMI, z-score, consanguinity, patient's history, cause of cirrhosis, family history of cirrhosis, laboratory data, abdominal sonography, liver biopsies, and saline contrast echocardiography for arteriovenous malformation (AVM) were evaluated and recorded.

Result

A total of twenty-two children were observed, with 59 % of them being boys. The average age of all the children was 8.43 ± 4.6 years. During the study, it was found that 27.3 % of the patients had mild left ventricular diastolic dysfunction, 13.6 % had mild AVM, and 4.5 % had moderate AVM in the contrast echocardiography. No relationship was found between cardiopulmonary complications with age, sex, and laboratory data. However, there was a significant relationship between moderate AVM diagnosed in contrast echocardiography and nodular formation, as well as a significant relationship between left ventricular enlargement (LVE) and nodular formation. One patient had nodular formation in the liver biopsy concurrent with LVE and moderate AVM. Mild AVM was seen in a patient with severe vascular change and giant cell transformation. Additionally, there was a relationship between contrast echocardiography and BUN, creatinine, and vitamin D levels (all P-values <0.05).

Conclusion

The results of contrast echocardiography are correlated with some complications of cirrhosis and can be used as a method to evaluate cardiopulmonary complications in children with cirrhosis.

背景：肝硬化是一种罕见但严重的肝功能障碍，可导致严重的并发症，包括儿童心肺受累。目的通过对比超声心动图分析肝硬化患儿的心肺并发症。方法在这项观察性横断面研究中，纳入了2020年至2024年诊断为肝硬化的所有儿科患者。评估并记录患者的人口统计学资料、体重、身高、BMI、z-score、血亲关系、患者病史、肝硬化原因、肝硬化家族史、实验室资料、腹部超声检查、肝活检、生理盐水超声心动图检查动静脉畸形（AVM）。结果共观察到22例患儿，其中男生占59%。所有患儿平均年龄为8.43±4.6岁。在研究过程中，超声造影发现27.3%的患者有轻度左室舒张功能不全，13.6%的患者有轻度AVM， 4.5%的患者有中度AVM。没有发现心肺并发症与年龄、性别和实验室数据之间的关系。然而，超声造影诊断的中度AVM与结节形成有显著相关性，左心室增大（LVE）与结节形成也有显著相关性。1例患者在肝活检中出现结节形成，同时伴有LVE和中度AVM。轻度AVM见于严重血管改变和巨细胞转化的患者。此外，超声心动图造影与BUN、肌酐和维生素D水平之间存在相关性（p值均为0.05）。结论超声心动图造影结果与肝硬化部分并发症有一定的相关性，可作为评价肝硬化患儿心肺并发症的一种方法。

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引用次数: 0

Fontan assist devices; A systematic review of twenty years of experimental and in vivo trials 方坦辅助装置；二十年实验和体内试验的系统回顾

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2025-03-17 DOI: 10.1016/j.ppedcard.2025.101827

Antoine Fakhry AbdelMassih , Zahraa Allami , Moyasar H. AlTatari , Rahaf Z. AbuGhosh , Alma AlFakhori , Omar J. AlMistarihi , Laila AlKhouli

Background

The Fontan completion represents the final phase of univentricular repair, which drains the inferior vena cava against gravity into a higher-pressure pulmonary arterial circulation. The passive and anti-gravity characteristics of this circulation make encountering a failing Fontan not uncommon. Twenty years ago, experiments exploring the potential of vascular pumps to bolster the passive Fontan have started, and trials are still ongoing.

Aim of review

Through this review, our aim is to summarize the outcomes of these trials.

Key scientific concepts and findings of review

A total of ten trials have been included, encompassing 23 different settings. Three distinct designs were identified, of which two are classified as pump designs: the connecting chamber and the intravascular pump designs. Only one trial investigated the potential use of a compression device. The most frequently encountered design in vivo was the intravascular pump, accounting for 67 % of the cases. There were no reports of significant hemolysis or thrombosis. However, an increasing flow rate exceeding 5 L/min was associated with negative outcomes in the connecting chamber design due to rising upstream pressure in the SVC. In contrast, intravascular pumps did not exhibit this limitation.

Fontan完成代表了单心室修复的最后阶段，它将下腔静脉引流到高压肺动脉循环中。这种环流的被动和反重力特性使得遇到失败的丰滩并不罕见。20年前，探索血管泵增强被动Fontan的潜力的实验已经开始，试验仍在进行中。综述的目的通过这篇综述，我们的目的是总结这些试验的结果。综述的主要科学概念和发现共纳入10项试验，涵盖23种不同的环境。确定了三种不同的设计，其中两种被归类为泵设计：连接腔和血管内泵设计。只有一项试验调查了压缩装置的潜在用途。体内最常见的设计是血管内泵，占67%的病例。没有明显的溶血或血栓的报道。然而，当流量超过5升/分钟时，由于SVC上游压力的上升，连接室的设计结果会出现负面影响。相比之下，血管内泵没有表现出这种局限性。

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