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Successful surgical management of cardiac tamponade following direct intrahepatic portosystemic shunt in a child with Budd-Chiari syndrome: A potentially life-threatening situation. 成功通过手术治疗了一名患有巴德-卡氏综合征的儿童肝内门体系统直接分流术后的心脏填塞:可能危及生命的情况。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI: 10.4103/apc.apc_107_23
Anand Kumar Mishra, Meenakshi Mandal, Irshad R Mohamed, Sadhna Lal, Harish Bhujade

Direct intrahepatic portosystemic shunt is a technique of portocaval shunting performed through the percutaneous route in patients with Budd-Chiari syndrome. Stent migration into right-sided heart chambers or perforations causing hemopericardium and cardiac tamponade is rare and underreported. In a child with a coexisting decompensated liver disease, surgical intervention and cardiopulmonary bypass can be challenging. However, surgical management in these life-threatening situations is better than redo interventions.

肝内门体系统直接分流术是一种通过经皮途径为巴德-卡氏综合征患者进行门腔分流的技术。支架移位至右侧心腔或穿孔导致血心包积血和心脏填塞的情况非常罕见,且报告不足。对于同时患有失代偿性肝病的患儿,外科干预和心肺旁路手术可能具有挑战性。然而,在这些危及生命的情况下,手术治疗比重新介入治疗效果更好。
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引用次数: 0
Intermittent flow reversal in the aortic arch. 主动脉弓间歇性血流逆转。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI: 10.4103/apc.apc_101_23
Shyam S Kothari, Nihar Lalitkumar Pathak, Nayan Banerji, Bhavik Champaneri
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引用次数: 0
The paucity of high-level evidence for therapy in pediatric cardiology. 儿科心脏病学治疗缺乏高水平的证据。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI: 10.4103/apc.apc_120_23
Emily Littman, Diana Hsiao, Kanekal S Gautham

Introduction: Clinical practice should be based on the highest quality of evidence available. Therefore, we aimed to classify publications in the field of pediatric cardiology in the year 2021 based on the level of scientific evidence.

Materials and methods: A PubMed search was performed to identify pediatric cardiology articles published in the calendar year 2021. The abstract or manuscript of each study was reviewed. Each study was categorized as high, medium, or low level of evidence based on the study design. Disease investigated, treatment studied, and country of publication were recorded. Randomized control trials (RCTs) in similar fields of neonatology and adult cardiology were identified for comparison. Descriptive statistics were performed on the level of evidence, type of disease, country of publication, and therapeutic intervention.

Results: In 2021, 731 studies were identified. A decrease in prevalence for the level of evidence as a function of low, medium, and high was found (50.1%, 44.2%, and 5.8%, respectively). A low level of evidence studies was the majority for all types of cardiac disease identified, including acquired heart disease, arrhythmias, congenital heart disease, and heart failure, and for treatment modalities, including circulatory support, defibrillator, percutaneous intervention, medicine, and surgery. In a subgroup analysis, most high-level evidence studies were from the USA (31%), followed by China (26.2%) and India (14.3%). Comparing RCTs, 21 RCTs were identified in pediatric cardiology compared to 178 in neonatology and 413 in adult ischemic heart disease.

Conclusions: There is a great need for the conduct of studies that offer a high level of evidence in the discipline of pediatric cardiology.

导言:临床实践应以现有的最高质量的证据为基础。因此,我们旨在根据科学证据的水平对 2021 年发表在儿科心脏病学领域的文章进行分类:我们在 PubMed 上进行了搜索,以确定 2021 年发表的儿科心脏病学文章。对每项研究的摘要或手稿进行审查。根据研究设计,将每项研究分为高、中、低证据级别。研究的疾病、治疗方法和发表国家均有记录。此外,还确定了新生儿科和成人心脏科类似领域的随机对照试验(RCT),以便进行比较。对证据水平、疾病类型、发表国和治疗干预进行了描述性统计:结果:2021 年,共确定了 731 项研究。发现证据级别为低、中、高的流行率有所下降(分别为 50.1%、44.2% 和 5.8%)。低证据水平的研究在所有类型的心脏病(包括后天性心脏病、心律失常、先天性心脏病和心力衰竭)和治疗方式(包括循环支持、除颤器、经皮介入、药物和手术)中占大多数。在分组分析中,大多数高级别证据研究来自美国(31%),其次是中国(26.2%)和印度(14.3%)。比较研究性试验,发现儿科心脏病学有21项研究性试验,而新生儿科有178项,成人缺血性心脏病有413项:结论:在儿科心脏病学领域,亟需开展能提供高水平证据的研究。
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引用次数: 0
Clinical parameters to predict adverse outcomes in patients with shunt-dependent physiology with a Blalock-Taussig-Thomas shunt. 预测布洛克-陶西格-托马斯分流术分流依赖生理患者不良后果的临床参数。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI: 10.4103/apc.apc_135_23
Saloni P Shah, Rohit S Loomba

In patients with shunt-dependent physiology, early risk factor identification can facilitate the prevention of adverse outcomes. This study aims to determine a scoring system to estimate the risk for adverse outcomes after Blalock-Taussig-Thomas shunt placement. Of the 39 neonates with Blalock-Taussig-Thomas shunt placement, 10 experienced the composite outcome. The resulting risk score from clinical and hemodynamic variables attributed 1 point for each of the following: central venous pressure >7.8, serum lactate >1.8 mmol/L, renal oxygen extraction ratio >32, and vasoactive-inotrope score >8.7. A score of 0 was associated with a 0% risk of the composite outcome, a score of 1 or 2 with a 15% risk, and a score of 3 or 4 with a 60% risk. A combination of increased central venous pressure, increased serum lactate, increased renal oxygen extraction ratio, and increased vasoactive-inotrope score are highly accurately associated with the risk of cardiopulmonary arrest, need for extracorporeal membrane oxygenation, or inpatient mortality after a Blalock-Taussig-Thomas shunt in patients with shunt-dependent physiology.

对于具有分流依赖性生理学的患者,早期识别风险因素有助于预防不良后果的发生。本研究旨在确定一个评分系统,以估计布洛克-陶西格-托马斯分流术后不良后果的风险。在 39 名接受布洛克-陶西格-托马斯分流术的新生儿中,有 10 名出现了综合结果。根据临床和血液动力学变量得出的风险评分为:中心静脉压>7.8、血清乳酸>1.8 mmol/L、肾氧萃取比>32、血管活性-肌动素评分>8.7,每项均得 1 分。评分为 0 时,出现综合结果的风险为 0%;评分为 1 或 2 时,风险为 15%;评分为 3 或 4 时,风险为 60%。中心静脉压升高、血清乳酸升高、肾氧萃取率升高和血管活性肌醇评分升高的组合与分流依赖性生理学患者在布洛克-陶西格-托马斯分流术后发生心肺骤停、需要体外膜氧合或住院死亡率的风险高度精确相关。
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引用次数: 0
Lack of "ventricular interdependence" in constrictive pericarditis and atrial septal defect. 缩窄性心包炎和房间隔缺损缺乏 "心室相互依存"。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI: 10.4103/apc.apc_148_23
Maulik Kalyani, Bhavik Champaneri, Shyam S Kothari

Ventricular interdependence, i.e., reciprocal variations in the left and right ventricle pressures with respiration, is a hallmark of the hemodynamic diagnosis of constrictive pericarditis (CP). Similarly, respiratory variations in the mitral and tricuspid valve Doppler inflow velocities on echocardiogram are very helpful in the diagnosis of CP. We document the absence of such variations in a patient with CP and associated atrial septal defect. It is important to be mindful of this intuitively obvious fact; otherwise, the diagnosis of CP might be missed.

心室相互依存,即左右心室压力随呼吸而相互变化,是缩窄性心包炎(CP)血液动力学诊断的标志。同样,超声心动图上二尖瓣和三尖瓣多普勒血流速度的呼吸变化也非常有助于 CP 的诊断。根据我们的记录,一名患有心绞痛并伴有房间隔缺损的患者没有出现这种变化。必须注意这一直观明显的事实,否则可能会漏诊 CP。
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引用次数: 0
Survival and outcomes of isolated neonatal ventricular septal defects: A population-based study from a middle-income country. 孤立性新生儿室间隔缺损的存活率和预后:一项基于中等收入国家人口的研究。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI: 10.4103/apc.apc_130_23
Mohd Nizam Mat Bah, Mohd Hanafi Sapian, Mohd Hazman Mohd Anuar, Emieliyuza Yusnita Alias

Background and aims: Limited data on the survival and outcomes of ventricular septal defect (VSD) in middle-income countries are available. Hence, this study aims to determine the survival and factors associated with mortality among neonatal VSD.

Materials and methods: This is a retrospective, population based study of neonates with isolated VSD born between 2009 and 2019. Kaplan-Meier analysis was used to estimate the overall survival. Cox regression analysis was used to determine factors associated with mortality.

Results: There were 726 patients studied, with 82 (11%) of them having trisomy 21. The median age of diagnosis and follow-up was 5 days (interquartile range [IQR]: 2-10 days) and 2.3 years (IQR: 0.6-4.8 years), respectively. Of 726, 399 (55%) were perimembranous, 218 (30%) muscular, and 109 (15%) outlet VSD. VSD was small in 309 (42%), moderate in 337 (46%), and large in 80 (11%). Of 726 patients, 189 (26%) had congestive heart failure (CHF) and 52 (7.2%) developed pulmonary hypertension (PHT). Interestingly, one-third of CHF and PHT resolved over time during follow-up. Only 1 (0.1%) patient had infective endocarditis, 38 (5.2%) developed aortic regurgitation, and none had Eisenmenger syndrome. Overall, 149 (20%) needed surgery, 399 (55%) spontaneously closed, and 178 (25%) remained small. The mortality rate was 3.9% (28), 16 (57%) preoperatively, and 11 (39%) due to pneumonia. Trisomy 21, PHT, and birth weight <2.5 kg were independent factors for mortality with an adjusted hazard ratio of 6.0 (95% confidence interval [CI]: 2.1-16.9), 3.2 (95% CI: 1.2-8.4), and 3.6 (95% CI: 1.7-7.8), respectively. The overall survival at 1, 5, and 10 years was 96% (95% CI: 95-98), 95% (95% CI: 94-97), and 95% (95% CI: 94-97), respectively.

Conclusions: Despite limited pediatric and congenital cardiac services in middle-income countries, the overall survival of neonatal VSD is good, with poor outcomes in small infants, PHT, and trisomy 21.

背景和目的:有关中等收入国家室间隔缺损(VSD)存活率和预后的数据有限。因此,本研究旨在确定新生儿 VSD 的存活率以及与死亡率相关的因素:这是一项基于人群的回顾性研究,研究对象为2009年至2019年间出生的患有孤立性VSD的新生儿。采用 Kaplan-Meier 分析法估算总体存活率。Cox回归分析用于确定与死亡率相关的因素:共有726名患者接受了研究,其中82人(11%)患有21三体综合征。诊断和随访的中位年龄分别为 5 天(四分位间距 [IQR]:2-10 天)和 2.3 年(IQR:0.6-4.8 年)。在 726 例患者中,399 例(55%)为包膜型 VSD,218 例(30%)为肌型 VSD,109 例(15%)为出口型 VSD。309例(42%)VSD为小,337例(46%)为中,80例(11%)为大。在 726 名患者中,189 人(26%)患有充血性心力衰竭(CHF),52 人(7.2%)出现肺动脉高压(PHT)。有趣的是,三分之一的充血性心力衰竭和肺动脉高压在随访期间逐渐缓解。只有 1 名(0.1%)患者患有感染性心内膜炎,38 名(5.2%)患者出现主动脉瓣反流,没有人患有艾森曼格综合征。总体而言,149 例(20%)需要手术,399 例(55%)自然闭合,178 例(25%)仍然很小。死亡率为3.9%(28例),其中16例(57%)在术前死亡,11例(39%)因肺炎死亡。21 三体综合征、PHT 和出生体重 结论:尽管中等收入国家的儿科和先天性心脏病服务有限,但新生儿 VSD 的总体存活率较高,小婴儿、PHT 和 21 三体综合征的预后较差。
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引用次数: 0
Double left atrial appendage: A diagnostic dilemma. 双左心房阑尾:诊断难题。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI: 10.4103/apc.apc_136_23
Nilanjan Dutta, Debasis Das, Unmesh Chakraborty, Shubhadeep Das, Manish Kumar Sharma, Shivani Gajpal, Amitabha Chattopadhyay, Sanjiban Ghosh, Jayita Nandy Das

We report a unique intraoperative finding of an additional double left atrial appendage (LAA) during an arterial switch operation with ventricular septal defect closure in a 4-month-old girl. Immediately after the procedure, a prolapsing mass within the left atrium (LA) on the transesophageal echocardiogram raised concerns of a possible thrombus. The LAA was clearly visible with a pressure monitoring line which was put intraoperatively. To investigate further, cardiopulmonary bypass was resumed, and the heart was arrested and explored. There was an appendage-like structure, separate from the one that had the pressure monitoring line, which was inverted inside. It was pulled out from outside clearly establishing a double LAA. This report illustrates an example of a diagnostic dilemma caused by a double atrial appendage which was invaginated into LA masquerading as a mass or thrombus.

我们报告了在为一名 4 个月大的女孩进行动脉转流手术并关闭室间隔缺损时,术中发现双左心房阑尾(LAA)的独特情况。手术结束后,经食道超声心动图显示左心房(LA)内有一个脱垂的肿块,这引起了她对可能存在血栓的担忧。术中放置的压力监测线清晰可见 LAA。为了进一步检查,恢复了心肺旁路,对心脏进行了停跳和探查。有一个类似阑尾的结构,与压力监测管线分开,倒置在里面。从外部将其拉出后,可以清楚地看到双 LAA。本报告举例说明了双心房阑尾伪装成肿块或血栓侵入 LA 所造成的诊断困境。
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引用次数: 0
Failure to wean off after a routine total anomalous pulmonary venous connection operation: An uncommon association. 常规全异常肺静脉连接手术后断奶失败:一种不常见的关联。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI: 10.4103/apc.apc_165_23
Riddhi Dhanak, Vikram Halder, Ritesh Shah, Amit Mishra, Shyam S Kothari

A 6-month-old infant was operated on for supracardiac total anomalous pulmonary venous connection (TAPVC) with usual anatomy. The vertical vein was ligated. Weaning from bypass was attempted twice but was unsuccessful. Coronary sinus atresia was suspected and identified on the opening of the right atrium. A smooth postoperative course occurred after unroofing the coronary sinus. Coronary sinus atresia should be remembered as an uncommon association with TAPVC.

一名 6 个月大的婴儿因心上全肺静脉连接异常(TAPVC)而接受手术,解剖结构正常。垂直静脉被结扎。曾两次尝试断开旁路,但均未成功。怀疑冠状窦闭锁,并在右心房开口处发现了冠状窦。解开冠状窦后,术后恢复顺利。冠状窦闭锁与TAPVC的关系并不常见,应引起重视。
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引用次数: 0
Are children with mitral valve prolapse more anxious? 患有二尖瓣脱垂的儿童更焦虑吗?
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI: 10.4103/apc.apc_126_23
Meryem Beyazal, Utku Arman Orun, Ilker Ufuk Sayici

Background: Mitral valve prolapse (MVP) is a common valvular disease in children. Symptoms in children with MVP are not explained by the severity of mitral regurgitation alone. Hence, we sought to correlate symptom status with the incidence of anxiety disorder in this population.

Materials and methods: Groups were as follows: (a) MVP; n = 115; mean age: 13.5 ± 3.06 years and (b) control; n = 53; mean age: 15.1 ± 13.2. The Screen for Child Anxiety-Related Emotional Disorders (SCAREDs) scale was filled by all participants. The SCARED scores of 25 and above determined a warning for anxiety disorders.

Results: The mean SCARED scale values of MVP and control groups were 29.2 ± 13.08 and 24.9 ± 14.17, respectively (P = 0.065). Although the SCARED scale score was higher among the MVP patients, no significant difference was found between the groups in terms of the mean SCARED score, or the number of participants with a score of 25 or more. While girls with MVP had higher anxiety scores compared to boys with the disorder, there was no difference with respect to SCARED scores in children with MVP and the general population when analyzed separately by gender. Moreover, the SCARED scale score was significantly higher in symptomatic MVP patients than in asymptomatic cases.

Conclusion: Children with MVP did not have higher anxiety scores compared to those without the syndrome. However, MVP patients with higher anxiety scores may benefit from a psychiatric assessment since higher scores correlate with symptoms.

背景:二尖瓣脱垂(MVP)是儿童常见的瓣膜疾病。二尖瓣反流的严重程度并不能单独解释二尖瓣脱垂儿童的症状。因此,我们试图将这一人群的症状状况与焦虑症的发病率联系起来:分组如下(a) MVP;n = 115;平均年龄:13.5 ± 3.06 岁;(b) 对照组;n = 53;平均年龄:15.1 ± 13.2。所有参与者都填写了儿童焦虑相关情绪障碍筛查量表(SCAREDs)。结果显示,SCARED 评分达到或超过 25 分即为焦虑症预警:MVP 组和对照组的 SCARED 量表平均值分别为(29.2 ± 13.08)和(24.9 ± 14.17)(P = 0.065)。虽然 MVP 患者的 SCARED 量表得分较高,但就 SCARED 平均值或得分在 25 分或以上的参与者人数而言,两组之间没有发现明显差异。与患有该疾病的男孩相比,患有 MVP 的女孩的焦虑得分更高,但按性别单独分析,患有 MVP 的儿童与普通人群的 SCARED 评分没有差异。此外,有症状的 MVP 患者的 SCARED 量表得分明显高于无症状的病例:结论:MVP 儿童的焦虑评分并不比无此综合征的儿童高。然而,焦虑评分较高的 MVP 患者可能会从精神评估中获益,因为较高的评分与症状相关。
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引用次数: 0
SIGIRR gene variants in term newborns with congenital heart defects and necrotizing enterocolitis. 患有先天性心脏缺陷和坏死性小肠结肠炎的足月新生儿中的 SIGIRR 基因变异。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI: 10.4103/apc.apc_30_23
Ekaterina Konstantinovna Zaikova, Aleksandra Vladimirovna Kaplina, Natalia Aleksandrovna Petrova, Tatiana Mikhailovna Pervunina, Anna Aleksandrovna Kostareva, Olga Viktorovna Kalinina

Background: Necrotizing enterocolitis (NEC) is a common gastrointestinal emergency among neonates which is characterized by acute intestinal inflammation and necrosis. The main risk factors for NEC are prematurity, low birth weight, and some preexisting health conditions such as congenital heart defects (CHDs). Investigation of the potential genetic predisposition to NEC is a promising approach that might provide new insights into its pathogenesis. One of the most important proteins that play a significant role in the pathogenesis of NEC is Toll-like receptor 4 (TLR4) which recognizes lipopolysaccharide found in Gram-negative bacteria. In intestinal epithelial cells, a protein encoded by the SIGIRR gene is a major inhibitor of TLR4 signaling. A few SIGIRR variants, including rare p.Y168X and p.S80Y, have already been identified in preterm infants with NEC, but their pathogenic significance remains unclear. This study aimed to investigate the spectrum of SIGIRR genetic variants in term newborns with CHD and to assess their potential association with NEC.

Methods and results: A total of 93 term newborns with critical CHD were enrolled in this study, 33 of them developed NEC. SIGIRR genetic variants were determined by Sanger sequencing of all exons. In total, eight SIGIRR genetic variants were identified, two of which were found only in newborns with NEC (P = 0.12). The rare missense p.S80Y (rs117739035) variant in exon 4 was found in two infants with NEC stage IIA. Two infants with NEC stage III and stage IB carried a novel duplication c. 102_121dup (rs552367848) variant in exon 10 that has not been previously associated with any clinical phenotype.

Conclusions: The presence of both variants only in neonates who developed NEC, together with earlier published data, may suggest their potential contribution to the risk of developing NEC in term infants with CHD and allow planning larger cohort studies to clarify their relevance.

背景:坏死性小肠结肠炎(NEC坏死性小肠结肠炎(NEC)是新生儿常见的胃肠道急症,以急性肠道炎症和坏死为特征。发生 NEC 的主要风险因素是早产、出生体重过低以及一些原有的健康状况,如先天性心脏缺陷 (CHD)。对 NEC 潜在遗传易感性的研究是一种很有前景的方法,可能会为了解其发病机制提供新的视角。在 NEC 发病机制中起重要作用的最重要蛋白质之一是 Toll 样受体 4(TLR4),它能识别革兰氏阴性细菌中的脂多糖。在肠上皮细胞中,SIGIRR 基因编码的蛋白质是 TLR4 信号传导的主要抑制剂。在患有 NEC 的早产儿中已经发现了一些 SIGIRR 变异,包括罕见的 p.Y168X 和 p.S80Y,但其致病意义仍不清楚。本研究旨在调查患有先天性心脏病的足月新生儿的 SIGIRR 基因变异谱,并评估它们与 NEC 的潜在关联:本研究共纳入了 93 例患有严重先天性心脏病的足月新生儿,其中 33 例发生了 NEC。通过对所有外显子进行 Sanger 测序,确定了 SIGIRR 基因变异。总共发现了 8 个 SIGIRR 基因变异,其中 2 个变异仅在 NEC 新生儿中发现(P = 0.12)。在两个 NEC IIA 期的婴儿中发现了外显子 4 中的罕见错义 p.S80Y (rs117739035)变异。102_121dup (rs552367848)变异,该变异以前从未与任何临床表型相关联:结论:这两个变异仅存在于发生 NEC 的新生儿中,加上之前发表的数据,可能表明它们对患有先天性心脏病的足月儿发生 NEC 的风险有潜在影响,因此可以计划进行更大规模的队列研究,以明确它们的相关性。
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引用次数: 0
期刊
Annals of Pediatric Cardiology
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