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The Surgical Significance of Phenotypic Variability in the Setting of Tetralogy of Fallot. 法洛氏四联症表型变异的外科意义。
Pub Date : 2025-01-01 Epub Date: 2024-09-26 DOI: 10.1177/21501351241274731
Ujjwal Kumar Chowdhury, Robert H Anderson, Diane E Spicer, Niraj N Pandey, Saurabh K Gupta, Niwin George, Maroof A Khan, Chaitanya Chittimuri

The phenotypic feature of tetralogy of Fallot is anterocephalad deviation of the muscular outlet septum, or its fibrous remnant, relative to the septoparietal trabeculation, coupled with hypertrophy of septoparietal trabeculations. Although this feature permits recognition of the entity, no two cases are identical. Once diagnosed, treatment is surgical. The results of surgical treatment have improved remarkably over recent decades. The results are now sufficiently excellent, including those in the developing world, that attention is now directed toward avoidance of morbidity, while still seeking, of course to minimize any fatalities due to surgical intervention. It is perhaps surprising that attention thus far has not been directed on the potential significance of phenotypic variation relative to either mortality or morbidity subsequent to surgical correction. The only study we have found specifically addressing this variability focused on the extent of aortic override, and associated malformations, but made no mention of variability in the right ventricular margins of the interventricular communication, nor the substrates for subpulmonary obstruction. In this review, therefore, we assessed the potential significance of known morphological variability to the outcomes of surgical intervention in over 1,000 individuals undergoing correction by the same surgeon in a center of excellence in a developing country. We sought to assess whether the variations were associated with an increased risk of postoperative death, or problems of rhythm. In our hands, double outlet ventriculoarterial connection was associated with increased risk of death, while the presence of a juxta-arterial defect with perimembranous extension was associated with rhythm problems.

法洛氏四联症的表型特征是肌肉性出口隔膜或其纤维残余与室间隔小梁相对的前脑偏位,同时伴有室间隔小梁肥大。虽然这一特征允许识别该实体,但没有两个病例是完全相同的。一旦确诊,治疗方法就是手术。近几十年来,手术治疗的效果显著提高。现在,包括发展中国家在内的许多国家都取得了很好的疗效,因此,人们开始关注如何避免发病,当然,同时也在努力将因手术治疗而导致的死亡人数降至最低。也许令人惊讶的是,迄今为止,人们还没有注意到表型变异对手术矫正后死亡率或发病率的潜在影响。我们发现的唯一一项专门针对这种变异性的研究侧重于主动脉覆膜的程度和相关的畸形,但没有提及室间隔右室边缘的变异性,也没有提及肺下梗阻的基础。因此,在这篇综述中,我们评估了已知形态学变异对手术干预结果的潜在影响,这些变异发生在发展中国家的一个卓越中心,由同一外科医生对 1000 多人进行了矫正。我们试图评估这些变异是否与术后死亡风险增加或节律问题有关。在我们手中,双出口脑室动脉连接与死亡风险增加有关,而存在脑膜周围延伸的并动脉缺损与心律问题有关。
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引用次数: 0
Invited Commentary: Morphological Variations in Tetralogy of Fallot and Their Impact in the Surgical Approach and Postoperative Evolution. 法洛氏四联症的形态变异及其对手术方法和术后演变的影响。
Pub Date : 2025-01-01 Epub Date: 2024-11-05 DOI: 10.1177/21501351241292620
Vera D Aiello
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引用次数: 0
Randomized Trial of Pulsatile and Nonpulsatile Flow in Cyanotic and Acyanotic Congenital Heart Surgery. 紫绀型和无绀型先心病手术中搏动性和非搏动性血流的随机试验。
Pub Date : 2024-12-22 DOI: 10.1177/21501351241288835
Krishna Patel, Tracie K Lin, Joseph B Clark, Gary D Ceneviva, Jason R Imundo, Debra Spear, Allen R Kunselman, Neal J Thomas, John L Myers, Akif Ündar

Background: The study objective was to determine the impact of cardiopulmonary bypass perfusion modalities on cerebral hemodynamics and clinical outcomes in congenital cardiac surgery patients stratified by acyanotic versus cyanotic heart disease.

Methods: A total of 159 pediatric (age <18 years) cardiac surgery patients were prospectively randomized to pulsatile or nonpulsatile cardiopulmonary bypass and stratified by type of congenital heart disease: acyanotic versus cyanotic. Intraoperative cerebral gaseous microemboli counts and middle cerebral artery pulsatility index were assessed. Organ injury was quantified by Pediatric Logistic Organ Dysfunction-2 (PELOD-2) score at 24, 48, and 72 h postoperatively. Additional outcomes included Pediatric Risk of Mortality-3 score, vasoactive-inotropic score, duration of mechanical ventilation, intensive care and hospital length of stay, and 180-day mortality. Heterogenous variance linear models (ie, ANOVA and mixed models) and χ2 tests were used to compare groups for continuous and categorical variables, respectively.

Results: Within congenital heart disease subgroups, patients randomized to nonpulsatile versus pulsatile bypass had similar preoperative and operative characteristics. While the intraoperative pulsatility index was higher in the pulsatile subset of both acyanotic and cyanotic groups (P < .05), regional cerebral oxygen saturation, mean arterial pressure, and gaseous microemboli counts were similar. Postoperative PELOD-2 scores decreased at similar rates in the acyanotic and cyanotic subgroups regardless of the perfusion modality utilized. There were also no significant between-group differences in the additional postoperative outcomes by perfusion modality in either acyanotic or cyanotic groups.

Conclusions: Despite patients undergoing pulsatile cardiopulmonary bypass experiencing a more physiologic pulsatility index in both acyanotic and cyanotic groups, no significant differences in cerebral hemodynamics or clinical outcomes were appreciated.

背景:研究目的是确定体外循环灌注方式对先天性心脏手术患者脑血流动力学和临床结果的影响,这些患者分为无青绀型和紫绀型心脏病。方法:采用159项2岁儿童试验,分别对连续变量和分类变量进行组间比较。结果:在先天性心脏病亚组中,随机分配到非搏动和搏动搭桥的患者具有相似的术前和手术特征。结论:尽管接受搏动式体外循环的患者在无氰和青紫两组中都经历了更高的生理性搏动指数,但在脑血流动力学和临床结果方面没有显著差异。
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引用次数: 0
Peritoneal Dialysis Catheter Placement on the Right Side of the Falciform Ligament to Prevent Obstruction by the Omentum. 在镰状韧带右侧放置腹膜透析导管以防止大网膜阻塞。
Pub Date : 2024-12-19 DOI: 10.1177/21501351241299762
F Doig, H Kheslat, Y d'Udekem, S P Namachivayam, A J Iyengar

We describe a simple and reproducible technique for neonatal peritoneal dialysis catheter insertion following cardiac surgery which prevents the catheter from becoming blocked by the omentum.

我们描述了一种简单和可重复的技术,用于新生儿腹膜透析导管插入心脏手术后,防止导管被网膜阻塞。
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引用次数: 0
Prevalence of Congenital Heart Disease in Children and Adolescents Under 18 in Africa: A Systematic Review and Meta-Analysis. 非洲 18 岁以下儿童和青少年先天性心脏病患病率:系统回顾与元分析》。
Pub Date : 2024-12-19 DOI: 10.1177/21501351241299405
Kwadwo A Danso, Grace Appah, Rosemary S Akuaku, Yaa S Karikari, Annette K Ansong, Frank Edwin, Nana-Akyaa Yao

Background: There are limited population-based studies on congenital heart disease (CHD) in the pediatric population in Africa. Technological advancements in diagnostic tools have resulted in multiple echocardiographic studies in hospital settings. We aimed to determine the prevalence of CHD in both settings (population-based and hospital based) followed by comparing the two estimates for a difference.

Methods: We systematically searched PubMed, Google Scholar, African Journals Online, and African Index Medicus for eligible studies from 1992 through 2022. We performed a meta-analysis using the random-effects model.

Results: We selected 42 studies; 10 population studies with 1,011,163 participants, and 32 hospital-based studies with 605,268 patients for the analyses. The population and hospital-based prevalence were 5.12 versus 12.63 per 1,000 population of children (P = .007). Ventricular septal defect was the most common type of CHD in both settings (0.61 vs 1.88 per 1,000), followed by atrial septal defect (0.26 vs 0.68 per 1,000). Tetralogy of Fallot was the most common cyanotic heart lesion in both settings (0.08 vs 0.52 per 1,000).

Conclusions: The population-based prevalence of CHD was significantly lower than the hospital-based prevalence (5.12 vs 12.63 per 1,000 population of children). Juxtaposing these two prevalence estimates against each other can be a reasonable alternative to quantifying the contemporary burden of CHD in the pediatric population of Africa. Moving forward, efforts should bolster awareness of CHD in Africa, and further advocacy for children with CHD should be a priority on the continent.

背景:非洲儿童先天性心脏病(CHD)的基于人群的研究有限。诊断工具的技术进步导致了医院设置的多重超声心动图研究。我们的目的是确定两种情况下(以人群为基础和以医院为基础)冠心病的患病率,然后比较两种估计的差异。方法:我们系统地检索PubMed、谷歌Scholar、African Journals Online和African Index Medicus从1992年到2022年的符合条件的研究。我们使用随机效应模型进行了荟萃分析。结果:我们选择了42项研究;10项人口研究涉及1,011,163名参与者,32项基于医院的研究涉及605,268名患者进行分析。人口患病率和医院患病率分别为5.12 / 1000和12.63 / 1000 (P = 0.007)。室间隔缺损是两种情况下最常见的冠心病类型(0.61 vs 1.88 / 1000),其次是房间隔缺损(0.26 vs 0.68 / 1000)。法洛四联症是两种情况下最常见的紫绀心脏病变(0.08 vs 0.52 / 1000)。结论:以人群为基础的冠心病患病率明显低于以医院为基础的患病率(5.12 / 1000儿童vs 12.63 / 1000儿童)。将这两种患病率估计值相互对照,可以作为一种合理的替代方法,量化非洲儿科人群中冠心病的当代负担。展望未来,应努力提高非洲对冠心病的认识,并应将进一步宣传冠心病儿童作为非洲大陆的优先事项。
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引用次数: 0
Classical Ozaki Procedure in a Seven-Month-Old Infant. 7个月大婴儿的经典尾崎手术。
Pub Date : 2024-12-08 DOI: 10.1177/21501351241297712
Aleksei Ye Chernogrivov, Aleksei I Kim, Frank Cetta, Tatyana V Rogova, Inessa Ye Nefedova, Igor E Chernogrivov, Aleksandr A Esayan, Vera I Dontsova, Alina S Sarkisyan

We present a case study of neotricuspidization of the aortic valve with neocusp suturing based on templates similar to those used in the Ozaki procedure but adapted for infants. This is an alternative for patients when valve reconstruction is impossible or risky. A good postoperative hemodynamic outcome was obtained, but it is necessary to evaluate this approach on the basis of long-term results.

我们提出了一个案例研究的主动脉瓣新三瓣缝合与新缝合的模板类似于Ozaki程序中使用的,但适用于婴儿。对于瓣膜重建不可能或有风险的患者,这是一种替代方法。获得了良好的术后血流动力学结果,但有必要在长期结果的基础上评估这种方法。
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引用次数: 0
Novel Use of the Right Atrial Appendage as a Neopulmonary Valve in a Child With Infective Endocarditis. 在感染性心内膜炎患儿中应用右心耳作为新肺瓣膜的新方法。
Pub Date : 2024-12-08 DOI: 10.1177/21501351241297713
Bharat Siddharth, Pavan Kumar Dammalapati, Vasudev V, Sameh Said

Infective endocarditis (IE) is a serious complication following any cardiac surgery. Pseudomonas as the causative organism is very rare. The authors report a case of IE post cardiac surgery for ventricular septal defect closure which was found to be caused by Pseudomonas involving the pulmonary valve. It was successfully managed by resection of a considerable amount of vegetation that had damaged the pulmonary valve. A previously placed patch was removed and pulmonary valve reconstruction using the right atrial appendage was accomplished.

感染性心内膜炎(IE)是任何心脏手术后的严重并发症。假单胞菌作为病原菌是非常罕见的。作者报告一例室间隔缺损关闭心脏手术后IE被发现是由假单胞菌累及肺动脉瓣引起的。通过切除大量损坏肺动脉瓣的植被,成功地处理了这一问题。移除先前放置的补片,使用右心房附件完成肺动脉瓣重建。
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引用次数: 0
Adjudicating Registry Data: Benefits and Pitfalls-The ACTION Experience. 裁定注册数据:好处和缺点- ACTION经验。
Pub Date : 2024-12-08 DOI: 10.1177/21501351241293714
David M Kwiatkowski, Scott Auerbach, Robert A Niebler

The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry collects data and adjudicates adverse events on pediatric patients receiving mechanical circulatory support at participating centers. To date, the registry includes over 1500 patients from 48 centers. Data collected has been used for research publications, quality improvement projects, and the regulatory support of several ventricular assist devices. While this dataset is large and adjudicated, the registry has real limitations that may limit the interpretation of findings. A full understanding of potential biases and limitations allows researchers and clinicians to make meaningful conclusions from the patient population. This report reviews the strengths and limitations of the ACTION registry to ensure optimal utility among pediatric patients receiving mechanical circulatory support. It also suggests potential areas for improvement to facilitate best care of this vulnerable population.

高级心脏治疗改善结果网络(ACTION)注册收集数据并判定参与中心接受机械循环支持的儿科患者的不良事件。迄今为止,该登记处包括来自48个中心的1500多名患者。收集的数据已用于研究出版物、质量改进项目和几种心室辅助装置的监管支持。虽然这个数据集很大,而且经过审核,但该注册表确实存在局限性,可能会限制对研究结果的解释。充分了解潜在的偏见和局限性,使研究人员和临床医生能够从患者群体中得出有意义的结论。本报告回顾了ACTION注册的优势和局限性,以确保在接受机械循环支持的儿科患者中获得最佳效用。它还提出了潜在的改进领域,以促进对这一弱势群体的最佳照顾。
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引用次数: 0
Aspirin Nonresponsiveness in Congenital Heart Disease-Prevalence, Risk Factors, and Management. 先天性心脏病中阿司匹林无反应性的患病率、危险因素和管理。
Pub Date : 2024-12-05 DOI: 10.1177/21501351241293721
Catherine E Stauber, Andrew Well, Catherine Dawson-Gore, Michelle Mizrahi, Charles D Fraser, Carlos M Mery, Daniel Stromberg

Background: Aspirin is frequently utilized for antiplatelet therapy in children with congenital heart disease (CHD). Patients who are unresponsive to aspirin, as measured by aspirin reaction units (ARU), are at higher risk for thrombotic events. It is undetermined if dose modification of aspirin results in adequate responsiveness in these patients. This study evaluates the prevalence and risk factors for aspirin nonresponsiveness and the results of dose escalation in this population.

Methods: This is a retrospective review of patients cared for in the cardiac care unit at a quaternary care academic congenital heart center who received aspirin and had responsiveness evaluated between January 2018 and January 2023. Patient demographics and clinical characteristics were extracted from the medical record. Descriptive, parametric, and nonparametric univariate analysis were employed.

Results: A total of 142 patients (69 [49%] female, 45 [32%]Non-Hispanic White, and 63 [44%] Hispanic]) were identified. Median age at first aspirin responsiveness assessment was 54 [interquartile range, IQR: 23.3-411.5] days with a median weight of 5.2 [IQR: 3.64-9.29] kg. Of these, 32/142 (22.5%) were nonresponsive on their initial testing. Of these patients, 23/32 (72%) had follow-up testing with 19/23 (83%) subsequently becoming therapeutic. This was achieved with an increased dose in 12/19 (63%) patients and increased duration of therapy in 7/19 (37%) patients. Seventeen of 142 (12%) patients experienced a thrombotic event, 13/17 (77%) of which were therapeutic on initial responsiveness assessment.

Conclusions: It is common for CHD patients to be aspirin nonresponsive with initial weight-based dosing. If aspirin is used in this population, it is necessary to evaluate ARUs on all patients as underdosing is not uncommon with current weight-based dosing methods.

背景:阿司匹林常用于先天性心脏病(CHD)患儿的抗血小板治疗。用阿司匹林反应单位(ARU)来衡量,对阿司匹林无反应的患者发生血栓事件的风险更高。目前还不确定阿司匹林的剂量调整是否能使这些患者产生足够的反应性。本研究评估了该人群中阿司匹林无反应的患病率和危险因素以及剂量增加的结果。方法:本研究对2018年1月至2023年1月期间在一家第四保健学术先天性心脏病中心心脏护理单元接受阿司匹林治疗并评估反应性的患者进行回顾性分析。从病历中提取患者人口统计学和临床特征。采用描述性、参数性和非参数性单变量分析。结果:共发现142例患者(女性69例[49%],非西班牙裔白人45例[32%],西班牙裔63例[44%])。首次进行阿司匹林反应性评估时的中位年龄为54[四分位间距,IQR: 23.3-411.5]天,中位体重为5.2 [IQR: 3.64-9.29] kg。其中,32/142(22.5%)在最初的测试中没有反应。在这些患者中,23/32(72%)进行了随访检测,19/23(83%)随后开始治疗。这是通过增加12/19(63%)患者的剂量和增加7/19(37%)患者的治疗时间来实现的。142例患者中有17例(12%)发生血栓形成事件,其中13/17例(77%)在初始反应性评估中是治疗性的。结论:冠心病患者在初始体重剂量下对阿司匹林无反应是很常见的。如果在这一人群中使用阿司匹林,则有必要评估所有患者的ARUs,因为使用目前基于体重的给药方法,剂量不足并不罕见。
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引用次数: 0
Subxiphoid Insertion of a Pediatric Implantable Pulse Generator in a Neonate With Congenital Complete Heart Block. 小儿植入式脉冲发生器在先天性完全性心脏传导阻滞新生儿中的剑突下插入。
Pub Date : 2024-12-05 DOI: 10.1177/21501351241297711
Benjamin P Kirk, Benjamin Hale, Mohsen Karimi

Permanent pacemaker placement is often indicated in newborns with congenital complete heart block (CCHB). The disproportionate size between commercially available devices and the neonate can lead to delayed intervention and high postoperative complication rates. Recently, Medtronic developed a Pediatric Implantable Pulse Generator (IPG); a very small single chamber pacemaker requiring a bipolar lead. This case report describes a minimally invasive approach for the implantation of this Pediatric IPG shortly after birth in a 2.32 kg neonate with CCHB.

永久性起搏器安置通常适用于先天性完全性心脏传导阻滞(CCHB)的新生儿。市售设备和新生儿之间不成比例的尺寸可能导致延迟干预和高术后并发症发生率。最近,美敦力开发了一种儿童植入式脉冲发生器(IPG);需要双极导联的非常小的单室起搏器。本病例报告描述了一种微创方法,用于在2.32公斤新生儿出生后不久植入小儿IPG。
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引用次数: 0
期刊
World journal for pediatric & congenital heart surgery
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