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World Journal for Pediatric and Congenital Heart Surgery最新文献

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Konno-Rastan Operation in a Patient With Dextrocardia and Situs Inversus. 在一名右心室突出和心功能不全患者身上实施的 Konno-Rastan 手术
IF 0.9 Q3 Medicine Pub Date : 2023-07-01 Epub Date: 2023-06-06 DOI: 10.1177/21501351231174824
Alwaleed Al-Dairy, Bairak Salameh, Hadeel Badran, Hasan Hasan, Lujain Alkyakhii

The Konno-Rastan operation is performed for relief of complex left ventricular outflow obstruction with a small aortic annulus. When associated with situs inversus and dextrocardia, important aspects should be kept in mind due to the mirror-image anatomy. In this report, we present a case of a 10-year-old child with the diagnosis of recurrent diffuse subaortic stenosis and situs inversus and dextrocardia who underwent the Konno-Rastan operation successfully and was asymptomatic with normal physical activity after a follow-up period of one year.

Konno-Rastan 手术用于缓解主动脉瓣环较小的复杂左心室流出道梗阻。当伴有坐位倒置和右心室突出时,由于其镜像解剖,应注意一些重要方面。在本报告中,我们介绍了一例诊断为复发性弥漫性主动脉瓣下狭窄、坐位反转和右心室缺血的 10 岁患儿,该患儿成功接受了 Konno-Rastan 手术,随访一年后无症状,可正常活动。
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引用次数: 0
Prevalence and Pattern of Congenital Heart Diseases in School Children in Dongguan, China. 中国东莞学龄儿童先天性心脏病的患病率和模式。
IF 0.9 Q3 Medicine Pub Date : 2023-07-01 Epub Date: 2023-03-17 DOI: 10.1177/21501351231163133
GuanYang Kang, JianMin Xiao, Jieying Wang, HuiQing Zhang

Background: To determine the prevalence and pattern of congenital heart disease (CHD) among elementary school children in Dongguan, China.

Methods: Between November 2011 and November 2012, 540,574 school children from 449 elementary schools were screened for CHD by trained doctors in Dongguan. The school children who were suspected to have CHD were referred to a pediatric cardiologist and/or an echocardiographer for a complete evaluation.

Results: The total prevalence of CHD was 2.14 per 1,000 school children (1,157/540,574). The most common form of CHD was isolated ventricular septal defect (37.77%; 437/1,157), followed by isolated atrial septal defect (20.22%; 234/1,157) and patent ductus arteriosus (9.94%; 115/1,157). With respect to sex, CHD was equally distributed between males and females.

Conclusion: Our data show that the prevalence of CHD in Dongguan is not as high as expected and that isolated ventricular septal defect is the predominant pathology. Echocardiography plays a crucial role in the diagnosis of CHD.

背景:目的:了解中国东莞市小学生先天性心脏病(CHD)的患病率和发病模式:调查中国东莞市小学生先天性心脏病(CHD)的患病率和模式:方法:2011 年 11 月至 2012 年 11 月期间,东莞市经过培训的医生对 449 所小学的 540,574 名学龄儿童进行了先天性心脏病筛查。疑似患有先天性心脏病的学龄儿童将被转诊至儿科心脏病专家和/或超声心动图专家处进行全面评估:结果:每 1,000 名学龄儿童中有 2.14 人患有先天性心脏病(1,157/540,574)。最常见的先天性心脏病是孤立性室间隔缺损(37.77%;437/1,157),其次是孤立性房间隔缺损(20.22%;234/1,157)和动脉导管未闭(9.94%;115/1,157)。在性别方面,CHD 在男性和女性之间的分布相当:我们的数据显示,东莞的先天性心脏病发病率并不像预期的那么高,孤立性室间隔缺损是主要的病因。超声心动图在诊断先天性心脏病中起着至关重要的作用。
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引用次数: 0
Spontaneous Hemopericardium Complicated by Tamponade in a Child With Moderate Hemophilia A: Case-Based Review. 中度血友病 A 患儿自发性血心包并发心肌填塞:基于病例的综述》(Spontaneous Hemopericardium Complicated by Tamponade in a Child with Moderate Hemophilia A: Case-Based Review.
IF 0.9 Q3 Medicine Pub Date : 2023-07-01 Epub Date: 2023-04-26 DOI: 10.1177/21501351231168868
Seyfeddine Zayani, Farah Thabet, Majdi Ben Massoud, Chokri Chouchane, Habib Gamra, Slaheddine Chouchane

Hemophilia A is the most common severe innate bleeding disorder. It is an X-linked recessive inherited bleeding disorder characterized by a qualitative and/or quantitative deficiency of factor VIII. The clinical manifestation of this disease is hemorrhaging that can affect every organ, in particular joints (hemarthrosis) and muscles (hematoma). Some serious but rare hemorrhages can be life-threatening, in particular hemorrhage of the central nervous system and hemopericardium. We report a rare case of spontaneous hemopericardium complicated by tamponade in a child with moderate hemophilia A treated with Factor VIII replacement infusion and pericardial drainage, with a favorable outcome. To our knowledge, this is the second case described in the literature of spontaneous hemopericardium occurring in a child with hemophilia A. Our case suggests that a dose of 50 IU/kg/8 h of factor VIII maintained for up to one day after removal of the pericardial drain seems to be sufficient to ensure correct hemostasis, though further evidence is needed to confirm this impression.

血友病 A 是最常见的严重先天性出血性疾病。它是一种 X 连锁隐性遗传出血性疾病,其特征是第八因子定性和/或定量缺乏。该病的临床表现为出血,可累及各个器官,尤其是关节(血肿)和肌肉(血肿)。一些严重但罕见的出血可危及生命,尤其是中枢神经系统出血和血心包。我们报告了一例罕见的中度 A 型血友病患儿自发性血心包并发心包填塞的病例,患儿接受了因子 VIII 替代输注和心包引流治疗,结果良好。据我们所知,这是文献中描述的第二例发生在 A 型血友病患儿身上的自发性血心包积血病例。我们的病例表明,在拔除心包引流管后维持 50 IU/kg/8 h 的 VIII 因子剂量长达一天似乎就足以确保正确止血,但这一印象还需要进一步的证据来证实。
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引用次数: 0
Perfusion Strategy to Prevent Right Ventricular Decompression on Cardiopulmonary Bypass During Extracardiac Fontan for Right Ventricle-Dependent Coronary Circulation. 心外方坦术治疗右心室依赖性冠状动脉循环时防止心肺旁路右心室减压的灌注策略。
IF 0.9 Q3 Medicine Pub Date : 2023-07-01 Epub Date: 2023-04-02 DOI: 10.1177/21501351231162894
Reena Khantwal Joshi, Neeraj Aggarwal, Mridul Agarwal, Siddartha C Rudrappa, Raja Joshi

Early and long-term outcomes in patients with pulmonary atresia-intact ventricular septum undergoing staged univentricular palliations have been known to be adversely affected by the presence of right ventricle-dependent coronary circulation. We describe a surgical technique to circumvent the coronary insufficiency caused by acute decompression of the right heart.

众所周知,肺动脉瓣闭锁-室间隔缺损患者接受分期单心室姑息术的早期和长期疗效会受到右心室依赖性冠状循环的不利影响。我们介绍了一种规避右心急性减压引起的冠状动脉供血不足的手术技术。
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引用次数: 0
Modification of the Warden Procedure for Surgical Repair of Partial Anomalous Pulmonary Venous Connection. 修改部分异常肺静脉连接手术修复的 Warden 程序。
IF 0.9 Q3 Medicine Pub Date : 2023-07-01 Epub Date: 2023-05-15 DOI: 10.1177/21501351231168830
Mark H D Danton, Emeka B Kesieme

Warden procedure is a popular technique for repairing partial anomalous pulmonary venous connection. We describe a modification of this technique for surgical repair of this condition by raising both a superior vena cava (SVC) flap and right atrial appendage flap to create a tension-free SVC-RA continuity (neo-SVC). The anomalous pulmonary veins are routed via the remanent of proximal SVC and baffled to the left atrium across a surgically created or enlarged atrial septal defect using autologous pericardium.

Warden手术是修复部分异常肺静脉连接的常用技术。我们描述了对这种技术进行手术修复的一种改良方法,即同时隆起上腔静脉(SVC)瓣和右心房阑尾瓣,以形成无张力的SVC-RA连接(新SVC)。异常的肺静脉经由近端 SVC 的残余部分,并利用自体心包将其挡板穿过手术形成或扩大的房间隔缺损到达左心房。
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引用次数: 0
Identifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology. 识别全身静脉引流异常:全身静脉异常;心房形态。
IF 0.9 Q3 Medicine Pub Date : 2023-07-01 Epub Date: 2023-03-15 DOI: 10.1177/21501351231158539
Madan M Maddali, Hamood N Al Kindi, Pranav Subbaraya Kandachar, Abdulla Al Farqani, Khalid S Al Alawi, Faiza Al Kindi, Salim Nasser Al-Maskari, Diane E Spicer, Robert Henry Anderson

So as to produce totally anomalous systemic venous connection, all of the systemic venous tributaries, along with the coronary sinus, should be connected with the morphologically left atrium. Previous descriptions of this rare constellation of anomalous connections of the systemic venous tributaries of the heart have been compromised by the inclusion of individuals having isomeric atrial appendages. In these settings, most frequently, the totally, or almost totally, anomalous systemic venous connections are associated with a sinus venosus defect. It is the anomalous pulmonary venous connections that then create a venovenous bridge, which permits the systemic venous tributaries to drain into the morphologically left atrium, even though they may be predominantly connected to the right atrium. More rarely, it is feasible for the primary atrial septum to develop so as to leave the systemic venous sinus in direct connection with the body of the morphologically left, rather than the morphologically right, atrium. We report a series of patients potentially falling into the category of anomalous systemic venous connections. The findings show a spectrum from partially to totally anomalous connections, with some better interpreted on the basis of anomalous drainage. Included in our cases, nonetheless, is an autopsied example of totally anomalous systemic venous connection produced by an abnormal location of the primary atrial septum. We discuss the potential morphogenesis for this finding. We emphasize the distinction that needs to be made between anomalous systemic venous connections and anomalous systemic venous drainage.

为了产生完全异常的全身静脉连接,所有的全身静脉支流以及冠状窦都应该与形态上的左心房相连。以前对这种罕见的心脏全身静脉支流异常连接的描述,由于包含了具有异构心房附属物的个体而受到影响。在这些情况下,最常见的是全身静脉连接完全异常或几乎完全异常与窦静脉缺损有关。异常的肺静脉连接会形成静脉桥,使全身静脉支流排入形态学上的左心房,尽管它们可能主要连接右心房。更罕见的情况是,原发性房间隔的发育使全身静脉窦与形态学上的左心房而非右心房直接相连。我们报告了一系列可能属于全身静脉连接异常的患者。研究结果显示了从部分异常连接到完全异常连接的不同范围,其中一些可以根据异常引流进行更好的解释。然而,在我们的病例中,有一例因原发性房间隔位置异常而导致全身静脉连接完全异常的尸检病例。我们讨论了这一发现的潜在形态发生机制。我们强调需要区分异常的全身静脉连接和异常的全身静脉引流。
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引用次数: 0
Novel Diabolo Configuration of a Large Covered Stent to Treat Cyanosis Related to Pulmonary Arteriovenous Malformations. 治疗肺动静脉畸形相关性青紫症的新型双螺旋结构大型有盖支架。
IF 0.9 Q3 Medicine Pub Date : 2023-07-01 Epub Date: 2023-03-27 DOI: 10.1177/21501351231162958
Sarosh P Batlivala, Amr Matoq, Shabana Shahanavaz

Microvascular pulmonary arteriovenous malformations (pAVMs) can lead to profound hypoxemia. "Hepatic factor" is postulated to play a role in their development. Certain patients with congenital heart disease are at particular risk to develop pAVMs, including those with heterotaxy syndromes and complex Fontan palliation. Ideally, an underlying cause is identified and corrected, although pAVMs may persist despite those interventions. We report a patient with heterotaxy syndrome s/p Fontan who had pAVMs that persisted despite Fontan revision with equal hepatic flow to both lungs. We employed a novel method to produce a diabolo configuration of a large covered stent to restrict lung flow while maintaining the potential for future dilation.

微血管肺动静脉畸形(pAVMs)可导致深度低氧血症。据推测,"肝脏因素 "在微血管肺动静脉畸形的形成过程中扮演了重要角色。某些患有先天性心脏病的患者尤其容易患上肺动静脉畸形,包括那些患有异位综合征和复杂的丰坦姑息术的患者。理想的情况是找到并纠正潜在的病因,但尽管采取了这些干预措施,pAVM 仍可能持续存在。我们报告了一名患有异位综合征和/或Fontan的患者,尽管进行了Fontan翻修,双肺的肝血流量相等,但pAVM仍持续存在。我们采用了一种新颖的方法来制作一个双螺旋结构的大型覆盖支架,以限制肺部血流,同时保持未来扩张的可能性。
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引用次数: 0
Severe Aortic Root Dilatation in a Patient With Larsen Syndrome. 一名拉森综合征患者的主动脉根部严重扩张
IF 0.9 Q3 Medicine Pub Date : 2023-07-01 Epub Date: 2023-03-08 DOI: 10.1177/21501351231155868
Ruth Eletta, Abinash Pandey, Tarathya Dharmasaputra, Marcial Moreno, Yonna Oparaugo, Tk Susheel Kumar, Susan Beker

We present a five-year-old female diagnosed with Larsen syndrome at birth with severe aortic root dilatation, failure to thrive, and developmental delay. This report highlights the specific physical findings, imaging, genetics, and surgical treatment, along with a brief overview of Larsen syndrome.

我们为您介绍一名五岁女性患者,她在出生时被诊断患有拉森综合征,伴有严重的主动脉根扩张、发育不良和发育迟缓。本报告重点介绍了具体的体格检查结果、影像学、遗传学和手术治疗,以及拉森综合征的简要概述。
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引用次数: 0
Invited Commentary: Achieving Post-Bypass Hemostasis in Young Children: At What Cost? 特邀评论:实现幼童搭桥后止血:代价是什么?
IF 0.9 Q3 Medicine Pub Date : 2023-07-01 DOI: 10.1177/21501351231174826
Susan Nicholson

Young children requiring bypass often develop coagulopathy resulting in major postoperative blood loss. Increased post-bypass bleeding and donor exposures are independently associated with adverse outcomes. When transfusion of hemostatic blood products fails to reduce bleeding to an acceptable level, rescue therapies including prothrombin complex concentrates (PCCs), and/or recombinant activated factor VII are being given "off-label" with increasing frequency. A number of studies attempting to determine the safety and efficacy of PCCs in neonates and young children are being published. These studies are most commonly retrospective, observational, performed in a single center with varying doses, indications for, and timing of administration in a small number of patients with varying results. The results of these individual studies are questionable and are not to be generalized to other center's patients. Because factor VIII inhibitor bypassing activity (FEIBA) contains the activated form of factor VII and factor X there are concerns regarding the potential for thrombotic events in a population with a known risk of postoperative thromboembolism. Currently, there is no validated assay with which to measure the efficacy of FEIBA in vivo to determine dose titration. Well-designed multicenter randomized control trials are needed to determine the optimal dose and risk-benefit of PCCs after pediatric cardiac surgery. Until such data are available the decision to give a procoagulant to neonates and young children after bypass needs to be made when the consequences of blood loss and replacement pose more risk than the risk of thrombotic complications from the drug.

需要搭桥的幼儿往往会出现凝血障碍,导致术后大量失血。搭桥术后出血量和供体暴露量的增加与不良预后密切相关。当输注止血血制品无法将出血量降低到可接受的水平时,包括凝血酶原复合物浓缩物(PCC)和/或重组活化因子 VII 在内的抢救性疗法越来越多地在 "标签外 "使用。目前已发表了大量研究报告,试图确定凝血酶原复合物浓缩物对新生儿和幼儿的安全性和有效性。这些研究多为回顾性、观察性研究,在单一中心进行,对少数患者的用药剂量、适应症和用药时间各不相同,结果也不尽相同。这些个别研究的结果值得商榷,不能推广到其他中心的患者身上。由于因子 VIII 抑制剂旁路活性(FEIBA)含有活化形式的因子 VII 和因子 X,因此人们担心在已知有术后血栓栓塞风险的人群中发生血栓事件的可能性。目前,还没有有效的检测方法来测量 FEIBA 的体内疗效,以确定剂量滴定。需要进行精心设计的多中心随机对照试验,以确定小儿心脏手术后 PCCs 的最佳剂量和风险效益。在获得此类数据之前,需要在失血和换血的后果比药物引起血栓并发症的风险更大的情况下,决定是否在搭桥术后给新生儿和幼儿使用促凝血剂。
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引用次数: 0
Preoperative Computed Tomographic Angiography Can Predict Need for Branch Pulmonary Artery Intervention in Patients With Ductal-Dependent Pulmonary Blood Flow. 术前计算机断层血管造影可以预测导管依赖性肺血流患者是否需要肺动脉分支介入治疗。
IF 0.9 Q3 Medicine Pub Date : 2023-05-01 DOI: 10.1177/21501351221151050
Kirsten Graff, Anthony M Hlavacek, Nicholas Aizcorbe, Scott M Bradley, Shahryar M Chowdhury

Background: Neonates with ductal-dependent pulmonary blood flow (DD-PBF) are at risk for pulmonary artery (PA) stenosis. The objective of this study was to identify preoperative cardiovascular computed tomography angiography (CTA) measures that are associated with the need for branch PA intervention.

Methods: We identified neonates with DD-PBF who underwent preoperative CTA at our center and were followed for 24 months. The primary outcome was requiring intervention for branch PA stenosis at the initial or subsequent procedure. Patients were divided into three groups: 1) No PA intervention, 2) Initial PA intervention, and 3) Remote PA intervention. Measurements of the branch PAs and patent ductus arteriosus (PDA) were made prospectively.

Results: Forty patients were included, 7 (18%) did not receive a PA intervention, 23 (58%) were in the initial PA intervention group, and 10 (25%) were in the remote PA intervention group. The distance from PA bifurcation to the largest diameter of the PA that receives the PDA showed a difference between the no-intervention group versus the initial and remote intervention groups (0.8 mm [IQR 0.7, 2.0], 8.2 mm [IQR 1.9, 13.7], 8.5 mm [IQR 6.5, 11.1], respectively, P  =  .02). The receiver operating characteristic curve showed a distance >2.2 mm had a sensitivity  =  91% and specificity  =  86% in predicting the need for PA intervention.

Conclusion: The distance from the PA bifurcation to the largest diameter of the branch PA that accepts the PDA on preoperative CTA is highly predictive of the need for initial or remote PA intervention in this group. Preoperative CTA should be considered for risk stratification in neonates undergoing intervention for DD-PBF.

背景:导管依赖性肺血流(DD-PBF)的新生儿有肺动脉(PA)狭窄的危险。本研究的目的是确定术前心血管计算机断层血管造影(CTA)措施与需要分支PA干预相关。方法:我们确定了在我中心术前接受CTA的DD-PBF新生儿,并随访了24个月。主要结果是在初始或后续手术中需要对支动脉狭窄进行干预。患者分为3组:1)无PA干预,2)初始PA干预,3)远程PA干预。前瞻性测量动脉导管未闭分支PAs和动脉导管未闭(PDA)。结果:纳入40例患者,7例(18%)未接受PA干预,23例(58%)为初始PA干预组,10例(25%)为远程PA干预组。无干预组与初始干预组和远程干预组相比,前列腺癌分叉至接受前列腺癌最大直径的距离分别为0.8 mm [IQR 0.7, 2.0]、8.2 mm [IQR 1.9, 13.7]、8.5 mm [IQR 6.5, 11.1], P = 0.02)。受试者工作特征曲线显示,距离>2.2 mm预测PA干预的敏感性为91%,特异性为86%。结论:在本组患者中,术前CTA显示的PA分叉到接受PDA的PA分支最大直径的距离可高度预测是否需要进行初始或远程PA干预。对于接受DD-PBF干预的新生儿,术前CTA应考虑风险分层。
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引用次数: 0
期刊
World Journal for Pediatric and Congenital Heart Surgery
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