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Comparison of risk stratification scoring system as a predictor of mortality and morbidity in congenital heart disease patients requiring surgery. 比较风险分层评分系统作为需要手术的先天性心脏病患者死亡率和发病率的预测指标。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI: 10.4103/apc.apc_142_23
Dicky Fakhri, Ni Made Ayu Sintya Damayanti, Muhammad Nurhanif

Backgrounds: Risk stratification systems have been important in reducing morbidity and mortality among congenital heart disease (CHD) patients requiring cardiac surgery. Multiple risk stratification scoring systems have been developed, including Aristotle Basic Complexity Score (ABC), Aristotle Comprehensive Complexity Score (ACC), Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery (STS-EACTS), and Risk Adjustment in Congenital Heart Surgery (RACHS-1). This study aims to access the superior risk stratification scoring system model in predicting mortality and morbidity.

Methods: The authors used Embase, PubMed, Scopus, and ProQuest as the primary databases for searching and included studies from hand searching. The area under the receiver operating characteristic curve was compared.

Results: A total of 11 articles were included in this review. The AUC of ABC for predicting mortality ranges from 0.59 to 0.71, and morbidity ranges from 0.673 to 0.743. The AUC of ACC score for predicting mortality ranges from 0.704 to 0.87, and a study revealed the AUC of morbidity is 0.730. The AUC of RACHS-1 for predicting mortality ranges from 0.68 to 0.782. The AUC of STS-EACTS for predicting mortality ranges from 0.739 to 0.8 and 0.732 for predicting morbidity.

Conclusion: ABC, ACC, RACHS-1, and STS-EACTS have acceptable to excellent discriminatory ability in predicting mortality and morbidity among CHD patients requiring cardiac surgery.

背景:风险分层系统对降低需要进行心脏手术的先天性心脏病(CHD)患者的发病率和死亡率非常重要。目前已开发出多种风险分层评分系统,包括亚里士多德基本复杂性评分(ABC)、亚里士多德综合复杂性评分(ACC)、胸外科医师协会和欧洲心胸外科协会(STS-EACTS)以及先天性心脏病手术风险调整(RACHS-1)。本研究旨在了解风险分层评分系统模型在预测死亡率和发病率方面的优势:作者使用 Embase、PubMed、Scopus 和 ProQuest 作为主要检索数据库,并纳入了手工检索的研究。比较了接收者操作特征曲线下的面积:本综述共纳入 11 篇文章。ABC 预测死亡率的 AUC 为 0.59 至 0.71,预测发病率的 AUC 为 0.673 至 0.743。ACC 评分预测死亡率的 AUC 为 0.704 至 0.87,一项研究显示发病率的 AUC 为 0.730。RACHS-1 预测死亡率的 AUC 为 0.68 至 0.782。STS-EACTS预测死亡率的AUC为0.739至0.8,预测发病率的AUC为0.732:结论:ABC、ACC、RACHS-1 和 STS-EACTS 在预测需要进行心脏手术的冠心病患者的死亡率和发病率方面具有可接受到卓越的鉴别能力。
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引用次数: 0
Percutaneous hepatic vein recanalization in pediatric Budd-Chiari syndrome: Comment. 小儿 Budd-Chiari 综合征的经皮肝静脉再通术:评论。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI: 10.4103/apc.apc_167_23
Hinpetch Daungsupawong, Viroj Wiwanitkit
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引用次数: 0
Left pulmonary artery reconstruction using cryopreserved pulmonary homograft. 使用低温保存的肺动脉同种移植体重建左肺动脉。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-07-01 Epub Date: 2024-01-05 DOI: 10.4103/apc.apc_89_23
Sachin Talwar, Pratik Kumar Jha, Arindam Choudhury, Amitabh Satsangi, Shiv Kumar Choudhary

In patients with univentricular heart, the Fontan procedure is the final palliation. This is usually staged. A systemic-to-pulmonary artery shunt is performed in the presence of episodes of cyanotic spells in the neonatal period or in some patients in infancy; a bidirectional superior cardiopulmonary anastomosis is preferred early in life. This is followed by the final Fontan palliation on an elective basis later. For an effective bidirectional superior cavopulmonary anastomosis and Fontan palliation, good-sized confluent pulmonary arteries (PAs) are mandatory in addition to favorable hemodynamic data. Patients with discontinuous PAs that are small in size present a surgical challenge at initial palliation as the one described in this report.

对于单心室心脏患者,丰坦手术是最后的缓解方法。这通常是分阶段进行的。在新生儿期或某些婴儿期患者出现发绀时,可进行全身-肺动脉分流术;在生命早期,最好进行双向上心肺吻合术。双向上腔心肺吻合术首选在生命早期进行,之后再选择性地进行最终的丰坦姑息术。要进行有效的双向上腔肺吻合术和Fontan姑息术,除了要有良好的血液动力学数据外,还必须有良好大小的汇合肺动脉(PA)。不连续的肺动脉(PA)尺寸较小的患者在最初的姑息治疗中面临手术挑战,就像本报告中描述的患者一样。
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引用次数: 0
Pleuropericardial cyst presenting as recurrent pneumonia: A diagnostic dilemma. 表现为复发性肺炎的胸膜心包囊肿:诊断难题。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-07-01 Epub Date: 2024-01-05 DOI: 10.4103/apc.apc_67_23
Bandya Sahoo, Anshika Garg, Asish Ranjan Mohakud, Reshmi Mishra
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引用次数: 0
Successful hybrid management of hypertensive patent ductus arteriosus with unilateral absence of the right pulmonary artery - A case report. 高血压性动脉导管未闭伴单侧右肺动脉缺失的成功混合治疗 - 病例报告。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-07-01 Epub Date: 2024-01-05 DOI: 10.4103/apc.apc_54_23
Jayitri Mazumder, Debasree Gangopadhyay, Mahua Roy, Anoop Singh, Somrita Laha, Joyeeta Dutta

Unilateral absence of pulmonary artery (UAPA) can be either isolated or associated with other congenital cardiac defects. It is a rare congenital lesion with a diverse clinical presentation. We present a case of absent right pulmonary artery and large patent ductus arteriosus (PDA) with severe unilateral pulmonary hypertension. We describe our strategy of staged ductus arteriosus closure in this patient and successful outcome on long term follow up.

单侧肺动脉缺失(UAPA)既可单独存在,也可与其他先天性心脏缺陷伴发。这是一种罕见的先天性病变,临床表现多种多样。我们介绍了一例右肺动脉缺失和大动脉导管未闭(PDA)并伴有严重单侧肺动脉高压的病例。我们介绍了为该患者分阶段关闭动脉导管的策略以及长期随访的成功结果。
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引用次数: 0
In silico prediction, molecular modeling, and dynamics studies on the targeted next-generation sequencing identified genes underlying congenital heart disease in Down syndrome patients. 通过对定向新一代测序进行硅学预测、分子建模和动力学研究,确定了唐氏综合征患者先天性心脏病的潜在基因。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-07-01 Epub Date: 2024-01-05 DOI: 10.4103/apc.apc_63_23
Fiona Hannah Carlus, L Balasubramaniam Sujatha, Anbazhagan Ganesh Kumar, Lakshmanan Loganathan, Karthikeyan Muthusamy, Silas Justin Carlus

Background: Individuals with Down syndrome (DS) have a 40%-60% chance of being born with congenital heart disease (CHD). This indicates that CHD in individuals with DS is not solely caused by trisomy 21, and there may be other genetic factors contributing to the development of CHD in these children. A study has identified variants in the specific genes that contribute to the pathogenesis of CHD in children with DS, isolated DS, and the CHD group. Computational studies on these identified variants, which, together with trisomy 21, determine the risk for CHD in DS cases, were limited. Here, we aimed to identify the impact of the identified variants that contribute to the pathogenesis of CHD in children with DS through in silico prediction, molecular modeling, and dynamics studies.

Methodology and results: The target single-nucleotide polymorphisms included in the study were examined for pathogenicity, residue conservation, and protein structural changes. The structural predictions were done using I-TASSER, Robetta, SWISS-MODEL, and Phyre2 tools. Further, the predicted models were validated through the PROCHECK server and molecular dynamics simulation using GROMACS software. The conservation analysis conducted on the identified variant highlights its significance in relation to the genetic disorders. Furthermore, a dynamics simulation study revealed the impact of the variant on protein structural stability (≤3 Å), providing valuable insights into its pathogenicity. We have also observed that the structure of the centrosomal protein of 290 kDa gene is relatively unstable, which may be attributed to its exclusive inclusion of helices within its secondary structural components.

Conclusions: This computational study explores, for the first time, the association between genes and CHD-DS, evaluating the identified specific frameshift variants. The observed pathogenic mutations in CHD-DS patients require further experimental validation and may contribute to the development of prospective drug design research. The insights gained from the structural and functional implications of these variants could potentially serve as a cornerstone in the development of effective treatments for this debilitating condition.

背景:唐氏综合征(DS)患儿出生后患先天性心脏病(CHD)的几率为 40%-60%。这表明,唐氏综合征患者的先天性心脏病并不完全是由 21 三体综合征引起的,可能还有其他遗传因素导致这些儿童患上先天性心脏病。一项研究发现了导致 DS 患儿、孤立 DS 患儿和 CHD 组 CHD 发病的特定基因变异。这些已确定的变异与 21 三体综合征共同决定了 DS 患儿发生先天性心脏病的风险,但有关这些变异的计算研究却很有限。在此,我们旨在通过硅预测、分子建模和动力学研究,确定已发现的变异对DS患儿CHD发病机制的影响:对研究中的目标单核苷酸多态性进行了致病性、残基保护和蛋白质结构变化方面的检查。使用 I-TASSER、Robetta、SWISS-MODEL 和 Phyre2 工具进行了结构预测。此外,还通过 PROCHECK 服务器和使用 GROMACS 软件进行的分子动力学模拟对预测模型进行了验证。对鉴定出的变异体进行的保护分析凸显了其与遗传性疾病相关的重要性。此外,动力学模拟研究还揭示了该变体对蛋白质结构稳定性(≤3 Å)的影响,为了解其致病性提供了有价值的见解。我们还观察到,290 kDa 基因的中心体蛋白结构相对不稳定,这可能是由于其二级结构成分中只包含螺旋:这项计算研究首次探讨了基因与 CHD-DS 之间的关联,评估了已确定的特定移帧变异。在 CHD-DS 患者中观察到的致病突变需要进一步的实验验证,并可能有助于前瞻性药物设计研究的发展。从这些变异的结构和功能影响中获得的见解有可能成为开发治疗这种衰弱病症的有效方法的基石。
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引用次数: 0
Novel use of the double kissing crush technique to stent complex pulmonary artery stenosis in a child with Alagille syndrome. 在一名患有阿拉吉尔综合征的儿童身上,新颖地使用双吻挤压技术对复杂的肺动脉狭窄进行支架置入。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-07-01 Epub Date: 2024-01-05 DOI: 10.4103/apc.apc_60_23
Danielle D Strah, Riley D Hellinger, Kwan S Lee, Michael D Seckeler

Coronary bifurcation lesions and treatment with two-stent techniques have been developed, including the double kissing (DK) crush technique. The use of this technique in children or noncoronary vessels, including pulmonary arteries, has not been described. We present a 12-year-old girl with Alagille syndrome, a ventricular septal defect (VSD), and complex bilateral pulmonary artery (PA) stenoses who is status post six catheterizations for PA angioplasty and stenting to improve her marked right ventricular hypertension. With collaboration between the congenital and structural teams, she successfully underwent the DK crush technique for a complex lesion in her PA. This improved pulmonary flow and allowed for successful surgical VSD closure.

冠状动脉分叉病变和双支架治疗技术已经发展起来,包括双吻(DK)粉碎技术。该技术在儿童或包括肺动脉在内的非冠状动脉血管中的应用尚未见报道。我们介绍了一名患有阿拉吉尔综合征、室间隔缺损(VSD)和复杂的双侧肺动脉(PA)狭窄的 12 岁女孩,为了改善她明显的右室高血压,她接受了六次导管插入术,进行了肺动脉成形术和支架植入术。在先天性和结构性团队的通力合作下,她成功地接受了 DK 压碎技术治疗 PA 的复杂病变。这改善了肺血流,并使手术成功关闭了 VSD。
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引用次数: 0
Retrospective study of preoperative sildenafil in prolonged pleural effusion after Fontan. 术前西地那非治疗方坦术后长期胸腔积液的回顾性研究。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-07-01 Epub Date: 2024-01-05 DOI: 10.4103/apc.apc_76_23
Dicky Fakhri, Pribadi Wiranda Busro, Budi Rahmat, Salomo Purba, Oktavia Lilyasari, Toshihide Asou

Background: By rerouting the systemic venous return into the pulmonary circulation, the Fontan operation is perhaps the most effective palliative procedure in patients with complex congenital heart disease. The Fontan, however, still comes with several significant complications and morbidities, one of them being postoperative prolonged pleural effusion. In our center from 2017 to 2021, 10% of all our patients who underwent Fontan operation developed prolonged pleural effusion. With this study, we aimed to analyze the preoperative use of sildenafil for 6 months and its role in prolonged pleural effusion after the Fontan operation.

Materials and methods: In total, 158 patients were included in the analysis. The cohort was divided into patients who developed prolonged pleural effusion after Fontan surgery (n = 17) and those who did not (n = 141). We analyzed several risk factors and the history of sildenafil use in each group in the cohort. We found that patients who used sildenafil for at least 6 months prior to Fontan surgery versus those who did not use (n = 112 vs. n = 46) were found to be at lower risk of developing prolonged pleural effusion (odds ratio [OR]: 0.32 [confidence interval 95%: 0.11-0.88], P = 0.027).

Conclusion: Preoperative administration of sildenafil for at least 6 months before Fontan might reduce the risk of postoperative prolonged pleural effusion.

背景:通过将全身静脉回流改道进入肺循环,丰坦手术可能是复杂先天性心脏病患者最有效的姑息性手术。然而,丰坦手术仍伴随着一些重大并发症和发病率,其中之一就是术后长期胸腔积液。从2017年到2021年,在我们中心接受丰坦手术的所有患者中,有10%出现了胸腔积液时间延长的情况。通过这项研究,我们旨在分析术前使用西地那非6个月的情况及其在丰坦手术后胸腔积液时间延长中的作用:共有 158 名患者纳入分析。该组患者分为在丰坦手术后出现长期胸腔积液的患者(17 人)和未出现长期胸腔积液的患者(141 人)。我们分析了队列中每组患者的几个风险因素和西地那非使用史。我们发现,在丰坦手术前使用西地那非至少6个月的患者与未使用西地那非的患者(n = 112 vs. n = 46)相比,发生长期胸腔积液的风险较低(几率比[OR]:0.32[置信区间95%:0.11-0.88],P = 0.027):结论:在Fontan手术前服用西地那非至少6个月可降低术后长期胸腔积液的风险。
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引用次数: 0
Spectrum and outcome of the use of noninvasive ventilation in a pediatric cardiac intensive care unit: A single-center experience. 儿科心脏重症监护室使用无创通气的范围和结果:单中心经验。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-07-01 Epub Date: 2024-01-05 DOI: 10.4103/apc.apc_57_23
Shubhadeep Das, Minal Desai, Debasis Das, Nilanjan Dutta, Shivani Gajpal, Manish Sharma

Background and aims: The pediatric cardiac intensive care unit (PCICU) frequently uses noninvasive ventilation (NIV). There are several reasons for its use, including prophylactic use right after the patient has been extubated. It is also used when patients are experiencing acute respiratory failure due to either cardiac or noncardiac reasons but are still able to maintain their airways. The objective of this study was to understand the spectrum of use of NIV following congenital cardiac surgery and analyze the outcome.

Methods and results: A retrospective observational study was conducted in a 14-bed PCICU, reviewing data from August 2019 to August 2022. Among 1750 congenital cardiac surgeries, 523 patients (29.9%) received NIV. The median age of the population was 2.5 months. Factors such as higher Risk-Adjusted Classification for Congenital Heart Surgery-1 category, longer intraoperative cardiopulmonary bypass time, and aortic cross-clamp time were associated with increased NIV use. Preoperative ventilator needs, infections, genetic syndromes, diaphragmatic paralysis, high vasoactive inotrope score (VIS) in the first 24 h, neonatal age, and weight <5 kg were independently associated with increased NIV need. The NIV group had a longer intensive care unit (ICU) stay compared to non-NIV patients. The success rate of NIV was 84%, with 440 successful cases and 83 failures. The mortality rate in the success and failure groups was not significantly different (5.27% vs. 6.0%).

Conclusions: NIV is widely used in PCICU, but it is associated with longer ICU stays. It proves beneficial after congenital cardiac surgery, especially for patients with specific risk factors. However, NIV may not directly impact mortality rates, suggesting that other factors contribute to patient survival.

背景和目的:儿科心脏重症监护病房(PCICU)经常使用无创通气(NIV)。使用无创通气有多种原因,包括患者拔管后的预防性使用。当患者因心脏或非心脏原因出现急性呼吸衰竭,但仍能维持呼吸道通畅时,也会使用 NIV。本研究旨在了解先天性心脏病手术后使用 NIV 的范围并分析其结果:在一家拥有 14 张病床的 PCICU 开展了一项回顾性观察研究,回顾了 2019 年 8 月至 2022 年 8 月期间的数据。在1750例先天性心脏手术中,有523名患者(29.9%)接受了NIV治疗。患者的中位年龄为 2.5 个月。先天性心脏病手术风险调整分类-1类别较高、术中心肺旁路时间较长、主动脉交叉钳夹时间较长等因素与NIV使用率增加有关。术前呼吸机需求、感染、遗传综合征、膈肌麻痹、头 24 小时内血管活性肌力剂评分(VIS)高、新生儿年龄和体重结论:NIV 被广泛应用于 PCICU,但会延长重症监护室的住院时间。事实证明,在先天性心脏病手术后使用 NIV 是有益的,尤其是对有特殊风险因素的患者。不过,NIV 可能不会直接影响死亡率,这表明其他因素也会影响患者的存活率。
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引用次数: 0
Three-dimensional/four-dimensional spatiotemporal image correlation morphology of the ductus arteriosus in fetuses with pulmonary atresia undergoing neonatal ductal stenting. 接受新生儿动脉导管支架术的肺动脉闭锁胎儿动脉导管的三维/四维时空图像相关形态。
IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2023-07-01 Epub Date: 2024-01-05 DOI: 10.4103/apc.apc_95_23
Sonia Karapurkar, Aishwarya Gurav, Abish Sudhakar, Navaneetha Sasikumar, Raman Krishna Kumar, Balu Vaidyanathan

Background: The value of prenatal identification of morphology of ductus arteriosus in fetuses with congenital heart defects (CHD) with pulmonary atresia and duct-dependent pulmonary circulation (DDPC) in planning neonatal ductal stenting procedure is untested. The aim of the study is to analyze the utility of three-dimensional/four-dimensional (3D/4D) spatiotemporal image correlation (STIC) fetal echocardiography in delineating the morphology of ductus arteriosus in fetuses with DDPC undergoing neonatal ductal stenting.

Methods: In this retrospective study (2017-22), prenatal imaging of pulmonary artery (PA) anatomy, aortic arch sidedness, and morphology of ductus arteriosus (ductal origin was classified as vertical/horizontal and ductal course as tortuous/straight) was done using 3D/4D STIC imaging and volume datasets. Prenatal findings were correlated with angiographic findings during stenting and the degree of agreement was calculated.

Results: We included 27 fetuses with a prenatal diagnosis of CHD with DDPC who underwent neonatal ductal stenting. The accuracy of prenatal assessment of PA anatomy, branch PA stenosis, and arch sidedness was 100%, 92.6%, and 88.9%, respectively. The accuracy of prenatal assessment of ductal origin and course, compared with angiography, was 85.2% and 88.9%, respectively. Prenatal imaging had a diagnostic accuracy of 100% for vertical straight and horizontal tortuous ducts, 84.6% for vertical tortuous, and 67% for horizontal straight ducts. Duct stenting was successful in 25 (92.6%) babies; two died after the procedure from stent occlusion.

Conclusion: Fetal echocardiography using 3D/4D STIC imaging enables accurate delineation of the morphology of ductus arteriosus in fetuses with DDPC, thereby aiding parental counseling and planning neonatal ductal stenting.

背景:先天性心脏缺陷(CHD)合并肺动脉闭锁和管道依赖性肺循环(DDPC)的胎儿在产前识别动脉导管形态对计划新生儿动脉导管支架植入术的价值尚未得到验证。该研究旨在分析三维/四维(3D/4D)时空图像相关(STIC)胎儿超声心动图在划分接受新生儿动脉导管支架术的DDPC胎儿动脉导管形态中的实用性:在这项回顾性研究(2017-22 年)中,利用三维/四维 STIC 成像和容积数据集对肺动脉(PA)解剖、主动脉弓两侧性和动脉导管形态(导管起源分为垂直/水平,导管走向分为迂曲/笔直)进行了产前成像。将产前检查结果与支架植入术中的血管造影结果进行关联,并计算两者的吻合程度:结果:我们共纳入了27名产前诊断为DDPC合并先天性心脏病的胎儿,他们都接受了新生儿导管支架植入术。产前评估PA解剖结构、PA分支狭窄和弓侧性的准确率分别为100%、92.6%和88.9%。与血管造影相比,产前评估导管起源和走向的准确率分别为 85.2% 和 88.9%。产前成像对垂直直管和水平迂曲导管的诊断准确率为 100%,对垂直迂曲导管的诊断准确率为 84.6%,对水平直管的诊断准确率为 67%。25名婴儿(92.6%)成功进行了导管支架植入术;两名婴儿在术后因支架闭塞而死亡:结论:使用三维/四维 STIC 成像进行胎儿超声心动图检查可准确划分 DDPC 胎儿动脉导管的形态,从而帮助父母进行咨询和计划新生儿动脉导管支架植入术。
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引用次数: 0
期刊
Annals of Pediatric Cardiology
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