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Commentary: “Pediatric cardiomyopathy illustrates the importance of reinterpreting the significance of genetic variants” 评论:"小儿心肌病说明重新解释遗传变异意义的重要性
IF 0.6 Q4 PEDIATRICS Pub Date : 2024-07-18 DOI: 10.1016/j.ppedcard.2024.101742
Robert Weintraub , David J. Amor
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引用次数: 0
Autologous pericardium: A bright future in pediatric aortic valve surgery? 自体心包:小儿主动脉瓣手术的光明前景?
IF 0.6 Q4 PEDIATRICS Pub Date : 2024-07-18 DOI: 10.1016/j.ppedcard.2024.101743
Dong Zhang , Xiang-Yang Wei , Ming-Jun Gao , Xiao-Lin Wang , Yu-Sheng Shu

Background: At present, the majority of aortic valve diseases have been traditionally treated by prosthetic valve replacement, whether using a mechanical or a bio-prosthetic valve. Surgical management of aortic valve disease in pediatric patients is still a great challenge, especially the surgical timing, selection of surgical approach, and optimal surgical materials. Aim of Review: In this paper, the main perspective is to propose the use of an aortic stent coating with endothelial cells and smooth muscle cells on both sides as aortic valve in the Ozaki procedure. Owing to its good biocompatibility, stability, thin but high tensile strength, anti-calcification, anticoagulant properties, and rapid endothelialization in vivo, long-term results from that instead of autologous pericardium in the Ozaki procedure may be superior to Ross in pediatric patients. Key Scientific Concepts of Review: The key scientific concepts are as follows: (1) a one-step, rapid, and robust concept for the preparation of aortic stent coating with endothelial cells and smooth muscle cells, and (2) Ozaki procedure.

背景:目前,大多数主动脉瓣疾病的传统治疗方法是人工瓣膜置换术,无论是使用机械瓣膜还是生物人工瓣膜。小儿主动脉瓣疾病的手术治疗仍然是一个巨大的挑战,尤其是手术时机、手术方式的选择和最佳手术材料。综述目的:本文的主要观点是提出在 Ozaki 手术中使用两侧涂有内皮细胞和平滑肌细胞的主动脉支架作为主动脉瓣。由于其良好的生物相容性、稳定性、薄而高的抗张强度、抗钙化、抗凝特性以及在体内快速内皮化,在小儿患者的 Ozaki 手术中替代自体心包的长期效果可能优于 Ross。评论的关键科学概念:关键科学概念如下:(1) 用内皮细胞和平滑肌细胞制备主动脉支架涂层的一步到位、快速而稳健的概念,以及 (2) Ozaki 程序。
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引用次数: 0
Ventricular assist device implant and management in a pediatric patient with dextrocardia and autism spectrum disorder 一名患有右心室缺血和自闭症谱系障碍的儿科患者的心室辅助装置植入和管理
IF 0.6 Q4 PEDIATRICS Pub Date : 2024-07-15 DOI: 10.1016/j.ppedcard.2024.101741
Nanda Nayak , Erin Munn , David Bearl

The utilization of ventricular assist devices (VAD) in the treatment of patients with congenital heart disease has expanded significantly in the pediatric population, largely due to the improved outcomes and availability of paracorporeal devices. Here, we describe a unique case of a non-verbal 5-year-old male with a complicated medical history of autism spectrum disorder and dextrocardia with complex two-ventricle congenital heart disease who was successfully bridged to transplant through the use of a VAD. Patient-tailored management was necessary in the successful use of the VAD with the goal of reducing stress and anxiety during his hospital stay.

在儿童先天性心脏病患者的治疗中,心室辅助装置(VAD)的使用范围已大幅扩大,这主要归功于治疗效果的改善和体外辅助装置的可用性。在这里,我们描述了一例独特的病例:一名无语言能力的 5 岁男性患有自闭症谱系障碍和右心室缺血,并伴有复杂的双心室先天性心脏病。在成功使用 VAD 的过程中,需要对患者进行量身定制的管理,目的是减轻其住院期间的压力和焦虑。
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引用次数: 0
Parental smoking and pulmonary arterial hypertension in infants: A cohort study 父母吸烟与婴儿肺动脉高压:一项队列研究
IF 0.6 Q4 PEDIATRICS Pub Date : 2024-07-10 DOI: 10.1016/j.ppedcard.2024.101740
Mohammad Reza Alipour, Hamidreza Kooshamoghaddam, Mohammadtaghi Sarebanhassanabadi

Background

Secondhand smoke exposes children to numerous toxic chemicals. Passive cigarette smoke causes a number of diseases, such as respiratory disease. Pulmonary arterial hypertension (PAH) is a progressive disease with a dismal prognosis. Some research studies have identified hypoxia and genetic variables as the etiological factors for PAH. As a result, it appears that environmental factors, such as tobacco smoke, disrupt the function of vascular endothelial cells.

Objectives

1) Evaluating the correlation between parental smoking and PAH in infants. 2) Examining the correlation between the number of cigarettes smoked per day by parents and systolic pulmonary arterial pressure (SPAP) during a year.

Method

In a cohort study, 140 neonates were classified into smokers and non-smokers. Birth weight and SPAP in neonates were measured in the smoker parental group. Following the measurement of the variables, the second group consisted of neonates with non-smoking parents whose birth weight and SPAP were matched one-to-one with the first group. After one year, we measured SPAP in two groups.

Result

At birth, all neonates had normal SPAP. The mean infant SPAP was different between smoker and non-smoker parents; this difference was statistically significant (P-value <0.0001). Over 45 % of infants whose parents smoked developed PAH. There was an association between the number of cigarettes that parents smoke daily and infant SPAP levels (P-value <0.0001). There was no association between PAH and the gender of the infants (p-value = 0.497).

Conclusion

This research revealed a significant association between parental smoking and PAH in infants. Also, the infant SPAP was significantly linked to the number of cigarettes that the parents smoked per day.

背景二手烟使儿童接触到大量有毒化学物质。被动吸烟会导致多种疾病,如呼吸系统疾病。肺动脉高压(PAH)是一种进展性疾病,预后不良。一些研究发现,缺氧和遗传变量是 PAH 的致病因素。因此,烟草烟雾等环境因素似乎会破坏血管内皮细胞的功能。目标1)评估父母吸烟与婴儿 PAH 之间的相关性。方法在一项队列研究中,将 140 名新生儿分为吸烟者和非吸烟者。测量吸烟父母组新生儿的出生体重和肺动脉收缩压。测量变量后,第二组由父母不吸烟的新生儿组成,其出生体重和SPAP与第一组一一对应。一年后,我们测量了两组新生儿的SPAP。吸烟和不吸烟父母的婴儿平均 SPAP 值不同,差异具有统计学意义(P 值为 0.0001)。父母吸烟的婴儿中有超过 45% 患有 PAH。父母每天吸烟的数量与婴儿 SPAP 水平之间存在关联(P 值为 0.0001)。结论这项研究揭示了父母吸烟与婴儿 PAH 之间的显著关系。此外,婴儿的SPAP与父母每天吸烟的数量也有明显关系。
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引用次数: 0
Salmonella endocarditis in an immunocompetent 8-year-old with a mechanical aortic valve: A case report and literature review 一名患有机械主动脉瓣、免疫功能正常的 8 岁儿童患上沙门氏菌心内膜炎:病例报告和文献综述
IF 0.6 Q4 PEDIATRICS Pub Date : 2024-06-20 DOI: 10.1016/j.ppedcard.2024.101739
Tamo Sultan , Claus Moser , Morten Holdgaard Smerup , Lars Idorn

Salmonella infections are prevalent globally and cause invasive infections in adults and children alike. Here, we present a rare case report and literature review based on a complicated presentation of S. enteritidis endocarditis in an immunocompetent 8-year-old child with a mechanical aortic valve. The child initially presented with gastrointestinal symptoms, leading to an 11-day hospitalization for gastroenteritis and bacteremia caused by Salmonella. After a 1.5-week hospitalization, receiving intravenous Ceftriaxone and oral Ciprofloxacin, the child was discharged and underwent another week of treatment. Two weeks post-discharge, recurrent fever led to rehospitalization. During this admission, the diagnosis of infective endocarditis was made, revealing complications including pseudoaneurysms and aortic root abscesses. This diagnosis necessitated cardiac surgery and an extended course of antibiotic treatment for an additional seven weeks. The child made a full recovery and was well at follow up. This is the twelfth reported case of Salmonella endocarditis in a child. The overall mortality rate of Salmonella Endocarditis in children is approximately 50 %, and it predominantly affects males. Though rare, Salmonella endocarditis can lead to severe cardiovascular complications. Therefore, it is important to maintain a high index of suspicion for endocarditis when risk factors are present and promptly initiate treatment.

沙门氏菌感染在全球普遍存在,可导致成人和儿童的侵入性感染。在此,我们根据一名患有机械主动脉瓣、免疫功能正常的 8 岁儿童肠炎沙门氏菌心内膜炎的复杂病例,提交了一份罕见病例报告和文献综述。患儿最初出现胃肠道症状,因沙门氏菌引起的胃肠炎和菌血症而住院11天。住院 1.5 周后,患儿接受了静脉注射头孢曲松和口服环丙沙星治疗,随后出院并接受了一周的治疗。出院两周后,由于反复发烧,患儿再次入院治疗。入院期间,诊断为感染性心内膜炎,并出现假性动脉瘤和主动脉根部脓肿等并发症。根据诊断结果,有必要进行心脏手术,并延长抗生素治疗疗程七周。患儿完全康复,随访时情况良好。这是报告的第 12 例儿童沙门氏菌心内膜炎病例。沙门氏菌心内膜炎在儿童中的总死亡率约为 50%,主要影响男性。沙门氏菌心内膜炎虽然罕见,但可导致严重的心血管并发症。因此,当存在危险因素时,必须高度怀疑心内膜炎并及时开始治疗。
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引用次数: 0
Impact of long lasting pulmonary arterial hypertension on the surgery of congenital heart defect 长期肺动脉高压对先天性心脏缺损手术的影响
IF 0.6 Q4 PEDIATRICS Pub Date : 2024-06-13 DOI: 10.1016/j.ppedcard.2024.101737
Fouad Nya , Sara Ahchouch , Houda Mokhlis , Rachida Amri , El arbi Bouaiti , Mehdi Bamous

Background

Little is known about the management of this high-risk subgroup of patients because only a few small retrospective studies were available in the literature.

Objectives

This study was designed to identify the determinants of post operative mortality and to assess during 10 years follow-up functional and hemodynamic variables for children presenting at surgery with pulmonary vascular disease associated with congenital heart defect.

Methods

This is a prospective study that enrolled children with pulmonary arterial hypertension associated with congenital heart disease and presenting beyond the optimal time frame for surgery. Inclusion criteria were late presentation for surgery and severe pulmonary arterial hypertension, defined as mean pulmonary arterial pressure > 50 mmhg. An univariable analysis, receiver operating characteristic curves, and Kaplan-Meier curves were used for survival analysis.

Results

75 patients underwent repair of their cardiac lesions. The operative mortality rate was 16%(12 patients); 43 patients (57 %) had right heart catheterization, and all of them were positive for acute vasodilatatory challenge (NO, oxygen). In the postoperative period, 32 % of patients had pulmonary hypertensive crisis, and 72 % had right heart failure. During follow-up, pulmonary arterial hypertension subclass 4 was present in 5% of our patients.

Conclusion

Our study indicated that for operable patients but beyond the optimal time frame the surgical mortality remained high despite using iNO. However, for those who survived they achieved good results in functional exercise, their PAP dropped to normal level and small proportion had persistent pulmonary arterial hypertension encouraging surgical closure of the defect.

本研究旨在确定术后死亡率的决定因素,并评估因先天性心脏缺损导致肺血管疾病而接受手术的患儿在 10 年随访期间的功能和血流动力学变量。纳入标准为手术时间较晚和严重肺动脉高压,即平均肺动脉压为 50 mmhg。采用单变量分析、接收器操作特征曲线和 Kaplan-Meier 曲线进行生存分析。手术死亡率为 16%(12 例患者);43 例患者(57%)接受了右心导管检查,所有患者的急性血管扩张试验(NO、氧)均呈阳性。术后,32%的患者出现肺动脉高压危象,72%出现右心衰竭。结论:我们的研究表明,尽管使用了 iNO,但对于可手术但超过最佳时间的患者来说,手术死亡率仍然很高。然而,对于那些存活下来的患者,他们在功能锻炼方面取得了良好的效果,PAP 下降到了正常水平,小部分患者出现了持续性肺动脉高压,这促使他们通过手术关闭缺损。
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引用次数: 0
Assessment of current pediatric electrocardiogram (ECG) interpretation practices 评估当前的儿科心电图(ECG)判读方法
IF 0.6 Q4 PEDIATRICS Pub Date : 2024-06-11 DOI: 10.1016/j.ppedcard.2024.101738
Tracy Marrs Conner , Sriketan Tamirisa , Lisa M. Roelle , Nathan Miller , Anthony Pompa , William B. Orr , Jenifer N. Avari Silva

Background

Despite improvements in digital electrocardiograms (ECGs), current standard of care requires physician confirmation. Mismatched expectations between ordering providers and ECG readers, often pediatric cardiologists and electrophysiologists (EPs), are common, especially since there are no standardized practices for pediatric ECG reading.

Objectives

The aim of this study was to understand current practices in pediatric ECG reading.

Methods

An electronic survey was sent to members of the Pediatric and Congenital Electrophysiology Society (PACES). Participation was optional; results were recorded from 12/19/22–1/9/23. Only complete and non-duplicate entries were included.

Results

A total of 127 responses were received, 93 were analyzed. Most responses were from centers in North America (n = 65, 70 %), including the US (n = 58, 62 %), Canada (n = 6, 6 %), and Mexico (n = 1, 1 %). The remaining were from Europe (n = 18, 19 %), Asia (n = 7, 8 %), Australia (n = 2, 2 %), and South America (n = 1, 1 %). Most (n = 46, 49 %) were from small centers (0–25 ECGs read per day), 27 respondents (29 %) were from medium centers (26–50 ECGs read per day), 20 respondents (22 %) were from large centers (>50 ECGs read per day). The majority (n = 65, 70 %) reported >3 readers/day for inpatient and emergency department ECGs. 49 % (n = 46) of centers read ECGs >2 times/day on weekdays with more variable practice on weekends. For critical/time sensitive findings, most centers (n = 90, 97 %) used verbal communication +/− the EMR. There was consensus (≥50 % agreement) that the following findings are critical/time sensitive: QTc >500 ms, T-wave alternans, narrow complex tachycardia, wide complex tachycardia, pre-excited atrial fibrillation, focal ischemic changes, second degree heart block type II, complete heart block, and pacemaker malfunction.

Conclusion

Reading practices are variable. Critical/time sensitive findings are most often communicated verbally, however, there is no agreed upon standard. There was consensus in critical/time-sensitive findings. Improved understanding of common practices and resource allocation may lead to increased consistency in pediatric ECG reading.

背景尽管数字心电图(ECG)有所改进,但目前的护理标准仍需要医生确认。本研究旨在了解儿科心电图阅读的现行做法。方法向儿科和先天性电生理学会(PACES)成员发送电子调查问卷。参与方式为选择性参与;结果记录时间为 12/19/22-1/9/23。结果共收到 127 份回复,对其中 93 份进行了分析。大多数回复来自北美的中心(n = 65,70%),包括美国(n = 58,62%)、加拿大(n = 6,6%)和墨西哥(n = 1,1%)。其余来自欧洲(18 人,占 19%)、亚洲(7 人,占 8%)、澳大利亚(2 人,占 2%)和南美洲(1 人,占 1%)。大多数受访者(n = 46,49 %)来自小型中心(每天读取 0-25 张心电图),27 名受访者(29 %)来自中型中心(每天读取 26-50 张心电图),20 名受访者(22 %)来自大型中心(每天读取 50 张心电图)。大多数受访者(65 人,占 70%)称,住院和急诊心电图每天需要 3 名读图员。49% 的中心(n = 46)在工作日每天读取 2 次心电图,而周末的读取次数更多。对于关键/时间敏感的结果,大多数中心(n = 90,97 %)使用口头交流 +/- EMR。大家一致认为(≥50%)以下结果为关键/时间敏感结果:QTc>500毫秒、T波交替、窄复律心动过速、宽复律心动过速、预激性心房颤动、局灶性缺血性改变、二度心脏传导阻滞II型、完全性心脏传导阻滞和起搏器故障。关键/时间敏感的检查结果最常以口头方式传达,但没有统一的标准。在关键/时间敏感结果方面存在共识。加强对常见做法的了解和资源分配可提高儿科心电图阅读的一致性。
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引用次数: 0
Successful use of MITRIS RESILIA mitral valve in pediatric patients MITRIS RESILIA二尖瓣在儿科患者中的成功应用
IF 0.9 Q4 PEDIATRICS Pub Date : 2024-06-08 DOI: 10.1016/j.ppedcard.2024.101736
Donald Mattia , Mohamad Alaeddine , Daniel Velez

Here, we demonstrate the capability of implanting the MITRIS RESILIA mitral valve in pediatric patients with severe mitral valve disease requiring mitral valve replacement. The MITRIS RESILIA valve is a bioprosthetic valve with a unique design allowing for improved physiological flow dynamics (minimizing left ventricular outflow obstruction) and potential for future valve-in-valve intervention. We retrospectively analyzed two cases: a 9-year-old with rheumatic heart disease and a 12-year-old with juvenile idiopathic arthritis-related mitral valve deterioration. Both patients underwent successful mitral valve replacement with the MITRIS RESILIA valve, demonstrating significant postoperative improvement in valve function and symptom relief. Follow-up echocardiograms 1 year later showed stable valve function with no significant regurgitation or stenosis, indicating satisfactory short-term outcomes.

在这里,我们展示了在需要进行二尖瓣置换术的严重二尖瓣疾病儿科患者中植入 MITRIS RESILIA 二尖瓣的能力。MITRIS RESILIA瓣膜是一种生物人工瓣膜,其独特的设计可改善生理血流动力学(最大限度地减少左心室流出道阻塞),并具有未来瓣中瓣介入治疗的潜力。我们对两个病例进行了回顾性分析:一个是患有风湿性心脏病的 9 岁儿童,另一个是患有幼年特发性关节炎相关二尖瓣恶化的 12 岁儿童。这两名患者都成功接受了 MITRIS RESILIA 瓣膜二尖瓣置换术,术后瓣膜功能明显改善,症状缓解。一年后的随访超声心动图显示瓣膜功能稳定,无明显反流或狭窄,表明短期疗效令人满意。
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引用次数: 0
Reintervention after ascending–descending aortic bypass for coarctation of the aorta in adulthood 成年后主动脉粥样硬化升主动脉-降主动脉搭桥术后的再介入治疗
IF 0.9 Q4 PEDIATRICS Pub Date : 2024-06-08 DOI: 10.1016/j.ppedcard.2024.101735
Toshi Maeda, Kosuke Yoshizawa, Otohime Mori

Prior to the establishment of a subclavian flap or end–to–end anastomosis, an ascending–descending aortic bypass was conducted to address coarctation of the aorta. Although good results for ascending-descending bypass have been reported in adulthood, reports about its long-term complications after repair in childhood are scarce. We performed reintervention in two adult patients with aneurysmal formation who underwent ascending–descending aortic bypass in childhood. Anatomical repair was performed through median sternotomy with deep hypothermic circulatory arrest. Case 1 is doing well 13 years postoperatively, whereas Case 2 died 4 months postoperatively due to hepatic failure. Close observation is imperative, and early reintervention is necessary when aneurysmal formation is observed.

在建立锁骨下皮瓣或端对端吻合术之前,人们采用升-降主动脉搭桥术来治疗主动脉共动脉瘤。虽然升主动脉-降主动脉搭桥术在成年后取得了良好的效果,但有关其在儿童期修复后的长期并发症的报道却很少。我们对两名在儿童时期接受过升-降主动脉搭桥术的动脉瘤形成成人患者进行了再介入治疗。解剖修复是通过胸骨正中切开术和深低温停循环进行的。病例 1 术后 13 年情况良好,而病例 2 术后 4 个月因肝功能衰竭死亡。密切观察是当务之急,一旦发现动脉瘤形成,必须及早进行再干预。
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引用次数: 0
Transcatheter closure of congenital extrahepatic portosystemic shunt to right atrium causing severe pulmonary hypertension in a child: A case report 经导管关闭先天性肝外门静脉分流至右心房导致重度肺动脉高压的患儿:病例报告
IF 0.9 Q4 PEDIATRICS Pub Date : 2024-05-30 DOI: 10.1016/j.ppedcard.2024.101729
Samir Shakya , Dinesh Chataut , Sharma Paudel , Ratna Mani Gajurel

Congenital extrahepatic portosystemic shunt is an abnormal connection between portal vein (prior to its branching) and one of the systemic veins. It is a rare cause of pulmonary hypertension. We report a 2-year-old child who presented with severe pulmonary artery hypertension and a unique form of congenital extrahepatic portosystemic shunt draining directly into right atrium. The shunt was successfully closed percutaneously, and pulmonary artery pressure fell from severe pulmonary artery hypertension to moderate pulmonary artery hypertension on follow up.

先天性肝外门静脉分流是指门静脉(在其分支之前)与全身静脉之一之间的异常连接。它是肺动脉高压的罕见病因。我们报告了一名两岁患儿的病例,该患儿患有严重的肺动脉高压和一种直接排入右心房的独特形式的先天性肝外门静脉分流。经皮成功关闭了分流道,随访时肺动脉压力从重度肺动脉高压降至中度肺动脉高压。
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引用次数: 0
期刊
PROGRESS IN PEDIATRIC CARDIOLOGY
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