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A neonate with a spongy failing heart - What could it be? 新生儿心脏呈海绵状衰竭--会是什么原因?
IF 0.9 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-01-01 Epub Date: 2024-05-24 DOI: 10.4103/apc.apc_193_23
Arumugom Archana, Chandra Kumar Natarajan, Vaanathi Hementha Kumar, Gnanasambandam Subramaniyam, Bala Ramachandran, Komarakshi Balakrishnan, K G Suresh Rao, Abhishek Berwal, Vishwanath Nandyala, Swati Iyer

A neonate born of third-degree consanguineous marriage presented on day 12 of life with congestive cardiac failure. A male sibling died at 3 months of age, cause of which was not known. He was treated with decongestive measures and multiple inotropes. 2D Echocardiogram revealed severe Left ventricular dysfunction with prominent trabeculations and deep recesses in the left ventricle suggestive of Left ventricular non-compaction. He was also found to have horse-shoe kidney. Considering the presence of cardiac left ventricular non compaction, horse-shoe kidney and family history of neonatal death and pregnancy loss clinical exome sequencing was done. It detected a homozygous missense variant in exon 6 of the AGK gene suggestive of Senger's syndrome. Baby was on regular follow-up and was thriving well on diuretics, sacubitril-valsartan and weekly levosimendan infusions. At 8 months of age, cardiac transplantation was successfully done and baby has been doing well post-transplantation. LVNC in children is rare with an estimated incidence of 0.11 per 100,000, the highest incidence being during infancy. Senger's syndrome is autosomal recessive in inheritance. Senger's syndrome associated with Left ventricular non compaction has been reported only once in literature so far. Renal manifestations in the form of horse shoe kidney like in our index baby has not been reported previously with Senger's syndrome.

一名三代近亲结婚的新生儿在出生后第 12 天出现充血性心力衰竭。他的一个男性兄弟姐妹在 3 个月大时死亡,死因不明。他接受了减充血措施和多种肌力药物治疗。二维超声心动图显示左心室严重功能障碍,左心室有突出的小梁和深凹陷,提示左心室不充盈。他还被发现患有马蹄肾。考虑到存在心脏左室不充盈、马蹄肾以及新生儿死亡和妊娠失败的家族史,医生对他进行了临床外显子组测序。结果在 AGK 基因的第 6 号外显子中检测到一个同源错义变异,提示患有森格尔综合征。婴儿接受了定期随访,在服用利尿剂、沙库比特利-缬沙坦和每周输注左西孟旦后恢复良好。婴儿 8 个月大时,成功进行了心脏移植手术,移植后一直表现良好。儿童 LVNC 很少见,估计发病率为十万分之 0.11,婴儿期发病率最高。森格综合征为常染色体隐性遗传。迄今为止,与左心室非压缩症相关的森格综合征仅在文献中报道过一次。像我们的患儿一样出现马蹄肾的肾脏表现在以前还没有关于森格综合征的报道。
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引用次数: 0
Open-heart surgery in preterm infants: A single-center experience. 早产儿的开胸手术:单中心经验。
IF 0.9 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-01-01 Epub Date: 2024-05-24 DOI: 10.4103/apc.apc_3_24
Danish Memon, Praveen Reddy Bayya, Perraju Bendapudi, Jessin Puliparambil Jayashankar, Brijesh Parayaru Kottayil, Balaji Srimurugan, Raman Krishna Kumar

Background: Open-heart surgery is challenging in preterm neonates and infants, and its feasibility in low-resource settings has not been defined. We describe our institutional experience with open-heart surgeries performed on consecutive preterm infants.

Materials methods and results: This was a single-center retrospective cohort from a tertiary hospital in Southern India and included consecutive preterm neonates (<37 weeks) admitted for open-heart surgery. This report is limited to babies who were <3 months at the surgery. The salient features of the 15 preterm included twin gestation: 7 (46.7%); median gestational age at birth: 35 weeks (28-36 weeks); median corrected gestational age at surgery: 37 weeks (33-40 weeks); birth weight: 1.75 kg (1.0-2.6 kg); weight at surgery: 1.8 kg (1.2-2.9 kg); and small for gestational age: 12 (80%). The heart defects included transposition of the great arteries (7), total anomalous pulmonary venous return (3), large ventricular septal defect (VSD) (1), and VSD with coarctation of the aorta (4). Eleven (73%) were mechanically ventilated preoperatively and five had preoperative sepsis. The mean cardiopulmonary bypass time was 169.7 ± 61.5 min, and cross-clamp time was 99.7 ± 43.8 min. There was no inhospital mortality; one baby expired during follow-up at 1 month. Postoperative mechanical ventilation duration was 126.50 h (84.25-231.50 h), and intensive care unit stay was 13.5 days (9-20.8). The total hospital stay was 39 days (11-95 days). Two children (13.3%) had postoperative sepsis.

Conclusion: Through collaborative multidisciplinary management, excellent outcomes are feasible in low-resource environments for selected preterm neonates undergoing corrective open-heart operations.

背景:对于早产新生儿和婴儿来说,开胸手术具有挑战性,而且其在低资源环境中的可行性尚未确定。我们介绍了本院对连续早产儿进行开胸手术的经验:这是一项来自印度南部一家三级医院的单中心回顾性队列研究,包括连续早产新生儿(结论:通过多学科协作管理,早产儿和低资源环境中的新生儿均可接受开胸手术:通过多学科协作管理,在资源匮乏的环境中,对选定的早产新生儿进行开胸矫治手术可取得良好的效果。
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引用次数: 0
Percutaneous closure of multiple atrial septal defects in a child utilizing three Occlutech Figulla septal occluders: A bailout. 利用三个 Occlutech Figulla 房间隔封堵器经皮封堵一名儿童的多个房间隔缺损:保驾护航。
IF 0.9 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-01-01 Epub Date: 2024-05-24 DOI: 10.4103/apc.apc_202_23
Ziad Bulbul, Haytham Bou Hussein, Fadi Sawaya, Fadi Bitar

We describe a unique report of percutaneous closure of multiple secundum atrial septal defects in a child utilizing three Occlutech Figulla septal occluders deployed sequentially. The procedure was performed under live three-dimensional transesophageal echocardiography guidance.

我们描述了一份独特的报告,该报告利用三个顺序展开的 Occlutech Figulla 房间隔封堵器,经皮封堵了一名儿童的多个房间隔缺损。手术在实时三维经食道超声心动图引导下进行。
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引用次数: 0
Permanent longitudinal strain damage of cardiotoxic drugs in childhood cancer: What is the safe level? 儿童癌症患者心脏毒性药物的永久性纵向应变损伤:安全水平是多少?
IF 0.9 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-01-01 Epub Date: 2024-05-24 DOI: 10.4103/apc.apc_146_23
Hamid Mohammadi, Hossein Hosseini, Mohammadreza Bordbar, Nima Mehdizadegan, Hamid Amoozgar, Mohammad Reza Edraki, Amir Naghshzan, Nima Naderi, Elham Abedi, Kambiz Keshavarz

Objective: Anthracycline administration in children is associated with cardiac dysfunction. Speckle-tracking echocardiography (STE) can detect subclinical cardiac damage that may go undetected by conventional two-dimensional (2D) echocardiography. This study aims to investigate medium-term anthracycline cardiotoxicity using STE and determine a safer administrable level of anthracyclines (ACs).

Methods: This observational case-control study enrolled 37 healthy controls and 78 pediatric cancer survivors who received chemotherapy. The patients were divided into two groups: cardiotoxic received (CR) and cardiotoxic free (CF). Data on segmental longitudinal strain (LS), global LS (GLS), and 2D echocardiographic parameters were collected after a drug-free period of at least one year.

Results: A total of 115 children with a mean age of 108 ± 55 months, of whom 66% were males, were included in the study. Both the groups of cancer survivors exhibited significantly reduced GLS compared to healthy controls (CR vs. controls, P = 0.001; CF vs. controls, P = 0.013), but no significant difference in left ventricular ejection fraction (LVEF) was observed (P = 0.75). Overall, cancer survivors treated with ACs demonstrated a significant reduction in strain in 10 left ventricular segments, particularly in the basal segments (P < 0.05). Among CR patients, those with impaired GLS (n = 43, GLS worse than -21.9) had significantly higher mean age and cumulative anthracycline dose compared to CR patients with normal GLS (age, P = 0.024; anthracycline dosage, P = 0.036). Using an anthracycline cutoff of 223 mg/m2 resulted in a higher detection rate (49% vs. 25%) and fewer missed cases (51% vs. 74%) compared to the 360 mg/m2 anthracycline cutoff.

Conclusion: Childhood cancer survivors demonstrate significantly reduced GLS while preserving a normal LVEF, which does not differ significantly from reference values of healthy children. The reduction in strain appears to be associated with higher anthracycline doses and older age. Lowering the anthracycline threshold to 223 mg/m2 may improve the predictability of a decline in cardiac function using strain imaging at medium-term follow-up.

目的儿童服用蒽环类药物会导致心脏功能障碍。斑点追踪超声心动图(STE)可检测出传统二维(2D)超声心动图可能检测不到的亚临床心脏损伤。本研究旨在利用 STE 调查蒽环类药物的中期心脏毒性,并确定更安全的蒽环类药物(ACs)用药剂量:这项观察性病例对照研究招募了 37 名健康对照者和 78 名接受过化疗的儿童癌症幸存者。患者被分为两组:接受心脏毒性治疗组(CR)和无心脏毒性治疗组(CF)。在至少一年的无药期后,收集有关节段纵向应变(LS)、整体LS(GLS)和二维超声心动图参数的数据:研究共纳入 115 名儿童,平均年龄为 108 ± 55 个月,其中 66% 为男性。与健康对照组相比,两组癌症幸存者的GLS均明显降低(CR与对照组相比,P = 0.001;CF与对照组相比,P = 0.013),但左心室射血分数(LVEF)无明显差异(P = 0.75)。总体而言,接受 ACs 治疗的癌症幸存者 10 个左心室节段的应变明显减少,尤其是基底节段(P < 0.05)。在 CR 患者中,与 GLS 正常的 CR 患者相比,GLS 受损的患者(n = 43,GLS 差于-21.9)的平均年龄和蒽环类药物累积剂量明显更高(年龄,P = 0.024;蒽环类药物剂量,P = 0.036)。与 360 毫克/平方米的蒽环类药物临界值相比,使用 223 毫克/平方米的蒽环类药物临界值可提高检出率(49% 对 25%),减少漏检病例(51% 对 74%):结论:儿童癌症幸存者在保持正常 LVEF 的同时,GLS 明显降低,与健康儿童的参考值差异不大。应变的减少似乎与蒽环类药物剂量增加和年龄增大有关。将蒽环类药物的阈值降低到223毫克/平方米可能会提高中期随访时使用应变成像预测心功能下降的能力。
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引用次数: 0
Delayed retrieval of embolized ductal stent: A surgical challenge. 栓塞导管支架的延迟取出:手术挑战。
IF 0.9 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-01-01 Epub Date: 2024-05-24 DOI: 10.4103/apc.apc_177_23
Biswa Ranjan Panda, Jayashree Mishra

Retrieval of embolized ductal stents from the pulmonary or systemic circulation can be challenging. Most children benefit from surgical shunts in such scenarios. Although early retrieval is advised, stents lodged in the peripheral pulmonary tree can be inaccessible, making the removal complicated. In such patients, stents can be "parked" in the segmental pulmonary arterial branches for retrieval later. In the low-pressure single ventricle pulmonary circulation, partially expanded embolized stents, if left in situ, can precipitate pulmonary arterial thrombosis. This subset of patients may benefit from meticulous anticoagulation and antiplatelet agents. In our case report, we describe the successful extraction of an embolized ductal stent without damage to the right lower lobe pulmonary artery (PA). In the follow-up evaluation, the growth of the right PA is good.

从肺循环或全身循环中取出栓塞的导管支架可能具有挑战性。在这种情况下,大多数患儿都能从手术分流中获益。虽然建议尽早取出支架,但由于无法进入外周肺动脉树,因此取出支架的过程会变得复杂。在这种情况下,可将支架 "停放 "在肺动脉节段分支中,以便日后取出。在低压单心室肺循环中,部分膨胀的栓塞支架如果留在原位,可能会诱发肺动脉血栓形成。这部分患者可能会从严格的抗凝和抗血小板药物中获益。在我们的病例报告中,我们成功取出了一个栓塞的导管支架,且未对右下叶肺动脉(PA)造成损伤。在随访评估中,右肺动脉生长良好。
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引用次数: 0
Alternative technique for femoral access in neonates undergoing cardiac catheterization. 新生儿接受心导管检查时股骨入路的替代技术。
IF 0.9 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-01-01 Epub Date: 2024-05-24 DOI: 10.4103/apc.apc_181_23
Nicola McCay, Keviin Walsh

We report an alternative technique for femoral access in neonates <3.5 kg undergoing cardiac catheterization. By modifying a 0.014" Balance middleweight Elite wire and using a 24-gauge Galt introducer needle, we have noted increased ease and success in obtaining vascular access in this challenging cohort.

我们报告了一种新生儿股骨入路的替代技术
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引用次数: 0
Different mechanisms for persistent and residual left-to-right shunt after transcatheter sinus venosus defect closure and their management. 经导管窦静脉缺损闭合术后持续和残留左向右分流的不同机制及其处理方法。
IF 0.9 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-01-01 Epub Date: 2024-05-24 DOI: 10.4103/apc.apc_190_23
Pramod Sagar, Kothandam Sivakumar

Transcatheter closure of superior vena cava (SVC) form of sinus venosus defects (SVDs) using covered stents is emerging as an alternative to surgery in the current decade. A covered stent placed in the cavoatrial junction creates a roof for the right upper pulmonary vein (RUPV) that stops the left-to-right shunt and redirects the vein to the left atrium. While surgical literature has clearly documented the incidence of stenosis of SVC and RUPV, sinus nodal dysfunction, and persistent residual shunts following surgical correction, it is imperative to have similar data after this new transcatheter intervention on the incidence of complications and follow-up outcomes. Since patients with pretricuspid shunts are often clinically asymptomatic, correction is primarily performed to prevent a persistent right heart volume overload and allow remodeling of the heart chambers. Any residual left-to-right shunt after a correction will result in persistent right heart dilatation. Residual flows can result from various mechanisms, including lack of apposition of the covered stent to the free edge of the SVD, fabric breach, and persistent anomalous drainage of additional right-sided pulmonary veins that drain very high in the SVC or can be due to a coexistent defect in the oval fossa. This review analyzes the different mechanisms, explains the transesophageal and angiographic images for each one, and offers solutions tailored for various reasons. Different mechanisms warrant different treatment principles. A solution for residual shunt from one mechanism may not be appropriate for residual flow through another mechanism. A thorough understanding would aid the operator in effective interventions for these SVDs.

使用有盖支架经导管闭合上腔静脉(SVC)形式的窦静脉缺损(SVDs)是近十年来出现的一种手术替代方法。放置在腔心交界处的有盖支架为右上肺静脉(RUPV)创造了一个屋顶,阻止左向右分流并将静脉重新导向左心房。手术文献清楚地记录了手术矫正后 SVC 和 RUPV 狭窄、窦房结功能障碍和持续残余分流的发生率,而在这种新的经导管介入治疗后,必须获得有关并发症发生率和随访结果的类似数据。由于前三尖瓣分流患者在临床上通常没有症状,因此进行矫正主要是为了防止右心容积持续超负荷,并使心腔得以重塑。任何矫正后残留的左向右分流都会导致右心持续扩张。残留血流可由多种机制造成,包括覆盖支架与 SVD 游离边缘的贴合不足、织物破损、SVC 极高处额外右侧肺静脉的持续异常引流或卵圆窝的并存缺损。这篇综述分析了不同的机制,解释了每种机制的经食道和血管造影图像,并针对各种原因提供了相应的解决方案。不同的机制需要不同的治疗原则。针对一种机制的残余分流的解决方案可能不适合通过另一种机制的残余血流。透彻了解这些机制将有助于操作员对这些 SVD 进行有效干预。
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引用次数: 0
Innominate vein occlusion by the fabric of covered stent during transcatheter closure of sinus venosus defects - Causes, management, and outcome. 经导管闭合窦静脉缺损过程中覆盖支架织物造成的腹腔静脉闭塞--原因、处理和结果。
IF 0.9 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-01-01 Epub Date: 2024-05-24 DOI: 10.4103/apc.apc_186_23
Kothandam Sivakumar, Pramod Sagar, Puthiyedath Thejaswi, Rajeshkumar Ramaswamy, Ramyashri Chandrasekaran

Transcatheter sinus venosus defect (SVD) closure with covered stents is emerging as an alternative to surgery. An adequate anchor zone in the superior vena cava is mandatory for the stability of the covered stent to prevent caudal embolization. There is a potential risk of innominate vein occlusion by the fabric of the covered stent in patients with a very short superior caval vein. Three among a total of 105 patients who underwent SVD closure at our institution developed innominate vein occlusion. Predisposing anatomical factors, identification and management of occluded innominate vein, and follow-up outcomes are discussed.

使用有盖支架进行经导管窦静脉缺损(SVD)闭合术正在成为手术的一种替代方法。上腔静脉内必须有足够的锚区,以保证覆盖支架的稳定性,防止尾端栓塞。对于上腔静脉很短的患者,覆盖支架的织物有可能造成腹腔静脉闭塞。在我院接受 SVD 闭合术的 105 位患者中,有 3 位出现了腹股沟静脉闭塞。本文讨论了导致腹股沟静脉闭塞的解剖因素、闭塞腹股沟静脉的识别和处理以及随访结果。
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引用次数: 0
Systemic venous anomalies in a child with a vein of Galen. 一名患有盖伦静脉的儿童全身静脉异常。
IF 0.9 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-01-01 Epub Date: 2024-05-24 DOI: 10.4103/apc.apc_170_23
Shamsa Al Kaabi, Madan Mohan Maddali, Salim Nasser Al Maskari, Halima Adam Al-Hashm, Abdullah Mohammed Al Farqani, Robert Henry Anderson, Justin T Tretter

We describe our findings in a child with a vein of Galen malformation, in whom the right superior caval and the azygos veins drained into the roof of the morphologically left atrium. A persistent left superior caval vein drained into the morphologically right atrium through the coronary sinus. The additional presence of dual brachiocephalic veins permitted the deployment of a multifunctional ventricular septal defect occluder device to occlude the right superior caval vein, correcting the right-to-left shunt. This also prevented azygos venous drainage into the left atrium.

我们描述了对一名盖伦静脉畸形患儿的研究结果,该患儿的右腔上静脉和颧静脉排入形态学上的左心房顶部。持续存在的左腔上静脉通过冠状窦排入形态上的右心房。由于存在双股肱静脉,因此可以使用多功能室间隔缺损封堵器封堵右腔上静脉,纠正右向左分流。这也防止了颧静脉排入左心房。
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引用次数: 0
Isolation of the left innominate artery: When to operate? 隔离左腹内动脉:何时手术?
IF 0.9 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2024-01-01 Epub Date: 2024-05-24 DOI: 10.4103/apc.apc_191_23
Samuel J H Parsons, Harry Nuttall, Alexander Jones

A right aortic arch with an isolated left innominate artery from the pulmonary artery is an exceedingly rare congenital cardiac malformation. We describe the management and complex surgical timing considerations in two such cases, successfully operated on day 4 and 7 months of age, including the use of cranial ultrasound as a helpful tool to guide decision-making. We also describe the first reported association of this defect with a 4q25 deletion encompassing the LEF1 gene.

右主动脉弓伴有从肺动脉分离出来的左心房动脉是一种极为罕见的先天性心脏畸形。我们描述了两例在 4 个月大和 7 个月大时成功手术的病例的处理方法和复杂的手术时机考虑,包括使用头颅超声作为指导决策的有用工具。我们还首次报道了这种缺陷与包含 LEF1 基因的 4q25 缺失的关联。
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引用次数: 0
期刊
Annals of Pediatric Cardiology
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