World journal for pediatric & congenital heart surgery最新文献

{"title":"They Deserve to Live: Impact of the Berlin Heart EXCOR on Small Children's Heart Transplant Waitlist in Low-Resource Settings.","authors":"Luiz Fernando Caneo, Aida Luiza Ribeiro Turquetto, Fábio Augusto Rodrigues Gonçalves, Evelinda Marramon Trindade, Holger Buchholz, Daniel Garros, Leonardo Augusto Miana, Maria Raquel Brigoni Massoti, Carla Tanamati, Juliano Gomes Penha, Marcelo Biscegli Jatene, Fabio Biscegli Jatene","doi":"10.1177/21501351241282283","DOIUrl":"10.1177/21501351241282283","url":null,"abstract":"<p><p><b>Background:</b> In newly emerging economy countries, the shortage of pediatric donor hearts and poor healthcare infrastructure poses a significant challenge. Although mechanical circulatory support (MCS) has been proven effective in enhancing heart transplant waiting list outcomes, economic barriers hinder its widespread adoption. <b>Methods:</b> A single-center retrospective study reviewed children under 10 kg on the heart transplant (HTx) list from 2012 to 2023. Elective or priority status was assigned based on their clinical condition at the time of transplant. In cases of clinical decompensation, centrifugal pump and extracorporeal membrane oxygenation, transitioned to the Berlin Heart EXCOR (BHE) pediatric ventricular assist device (VAD) was employed. Pre- and post-HTx outcomes were analyzed with descriptive statistics, Cox regression, and competing survival risks. <b>Results:</b> Out of 81 infants on the HTx list, 61.7% (50/81) were in critical condition. The median wait time was 224 days, and 34% (28/81) died while waiting. Out of 37 transplanted patients, 6 (16%) had graft dysfunction, and 10 (27%) had acute renal injury. Survival to discharge was 84% (31/37). Patients who received the BHE exhibited higher chances of receiving a transplant (hazard ratio: 2.3; 95% confidence interval: 1.2-4.6; <i>P</i> = .01). Priority status or MCS use did not significantly impact mortality post-transplant. <b>Conclusion:</b> Advanced MCS technologies can potentially reduce the mortality risk on the pediatric HTx waitlist. The findings highlight the significant waiting time for HTx and the critical role of the BHE in improving outcomes in children, particularly those under 10 kg. The results advocate for the adoption of ventricular assist devices as a viable interim solution to bridge critically ill children to HTx, ultimately enhancing their chances of survival despite limited donor heart availability.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"313-322"},"PeriodicalIF":0.0,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142515735","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Aspirin Nonresponsiveness in Congenital Heart Disease-Prevalence, Risk Factors, and Management.","authors":"Catherine E Stauber, Andrew Well, Catherine Dawson-Gore, Michelle Mizrahi, Charles D Fraser, Carlos M Mery, Daniel Stromberg","doi":"10.1177/21501351241293721","DOIUrl":"10.1177/21501351241293721","url":null,"abstract":"<p><p>BackgroundAspirin 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.MethodsThis 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.ResultsA 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.ConclusionsIt 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.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"388-394"},"PeriodicalIF":0.0,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12012269/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142788356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Use of Cervical Approach for Insertion of the Impella 5.5 Ventricular Assist Device (Abiomed) in Pediatric Patients: Cohort Study.","authors":"Mohammed Absi, Andrew J Gienapp, Cihangir Buyukgoz, Shyam Sathanandam, Umar Boston","doi":"10.1177/21501351241285451","DOIUrl":"10.1177/21501351241285451","url":null,"abstract":"<p><p>BackgroundImpella 5.5 ventricular assist device (VAD) insertion is typically done via the axillary artery or directly through the aorta; however, an axillary artery must be ≥6 mm in diameter, which excludes many pediatric patients who do not meet this criterion. The innominate artery is a larger vessel that can better accommodate the Impella VAD in pediatric patients. Outcomes of this technique were compared with other pediatric patients undergoing the standard axillary artery cannulation.MethodsThe Heart Center at Le Bonheur Children's Hospital recently adopted the trans-innominate cervical approach for insertion of the Impella 5.5 VAD. We performed a retrospective cohort study comparing these cases to data collected from cases using an axillary approach found in the Advanced Cardiac Therapies Improving Outcomes Network database. Comparisons between preoperative characteristics, postoperative course, adverse events, anticoagulation, and clinical outcomes for these two groups were analyzed.ResultsThirty-nine patients were implanted via the axillary approach; seven patients were implanted via the transinnominate cervical approach at our institution. Patients inserted via the trans-innominate approach had fewer days on the device (median, 8 [range, 3-24]), postimplant intensive care unit days (16 [3-24]), and hospital length of stay (14 [3-28]) compared with axillary insertion patients (12.5 [6.75-29.2], 58.5 [12.5-43.5], and 32 [19.7-83.5], respectively). Trans-innominate patients had fewer adverse events than axillary patients.ConclusionsTrans-innominate cervical approach for insertion of the Impella 5.5 VAD is safe with low rates of adverse events in pediatric patients. As miniaturization of VAD therapy continues to evolve, this technique may provide greater application of these devices for even smaller children.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"323-328"},"PeriodicalIF":0.0,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019440","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Total Anomalous Pulmonary Venous Return, Marfan Syndrome, and Severe Combined Immunodeficiency: A Lethal Combination?","authors":"Spencer J Hogue, Muhammed Faateh, Awais Ashfaq","doi":"10.1177/21501351241311262","DOIUrl":"10.1177/21501351241311262","url":null,"abstract":"<p><p>The authors present an exceptionally rare case of a newborn in whom total anomalous pulmonary venous return (TAPVR) had been diagnosed prenatally and in whom genetic testing after neonatal cardiac repair confirmed a missense variant of the <i>FBN1</i> gene consistent with Marfan syndrome as well as a <i>PRKDC</i> gene mutation associated with severe combined immunodeficiency. To the authors' knowledge, this is the first reported case with this unique combination. Neonatal TAPVR repair was undertaken with a good postoperative outcome and survival until the last follow-up.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"421-423"},"PeriodicalIF":0.0,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143054532","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"In Memoriam: Albert Starr (1926-2024).","authors":"Marshall L Jacobs, Carl L Backer","doi":"10.1177/21501351251322901","DOIUrl":"https://doi.org/10.1177/21501351251322901","url":null,"abstract":"","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":"16 3","pages":"303-305"},"PeriodicalIF":0.0,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144029590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Subxiphoid Insertion of a Pediatric Implantable Pulse Generator in a Neonate With Congenital Complete Heart Block.","authors":"Benjamin P Kirk, Benjamin Hale, Mohsen Karimi","doi":"10.1177/21501351241297711","DOIUrl":"10.1177/21501351241297711","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"411-413"},"PeriodicalIF":0.0,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142788357","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neoaortic Dilation and Polyvalvar Dysfunction: The Coexistence of Marfan Syndrome with Hypoplastic Left Heart Syndrome.","authors":"Sruti Rao, Rukmini Komarlu, Hani K Najm, Brittany Hansen, Rene E Rodriguez, Kenneth G Zahka","doi":"10.1177/21501351241313314","DOIUrl":"10.1177/21501351241313314","url":null,"abstract":"<p><p>We present a unique case of progressive neoaortic root and neoascending aortic dilatation, with poly-valvar regurgitation in a patient with Marfan syndrome and hypoplastic left heart syndrome palliated to Fontan procedure. This progressive dilation and valvar dysfunction necessitated a modified Bentall procedure and tricuspid valve repair. Our case highlights the impact of both a primary and secondary aortopathy complicating the Fontan procedure and the need for mechanical aortic valve replacement.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"424-426"},"PeriodicalIF":0.0,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143257709","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Assessment of the Utility of Pulsatile Over Nonpulsatile Flow for Neonatal and Pediatric Cardiopulmonary Bypass Procedures Using Multidisciplinary Translational Research at a Tertiary Care Center.","authors":"Christopher Collin Hayes, Akif Ündar","doi":"10.1177/21501351241269912","DOIUrl":"10.1177/21501351241269912","url":null,"abstract":"","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"354-356"},"PeriodicalIF":0.0,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12012267/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142484830","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Adjudicating Registry Data: Benefits and Pitfalls-The ACTION Experience.","authors":"David M Kwiatkowski, Scott Auerbach, Robert A Niebler","doi":"10.1177/21501351241293714","DOIUrl":"10.1177/21501351241293714","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"309-312"},"PeriodicalIF":0.0,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142796753","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevalence of Congenital Heart Disease in Children and Adolescents Under 18 in Africa: A Systematic Review and Meta-Analysis.","authors":"Kwadwo A Danso, Grace Appah, Rosemary S Akuaku, Yaa S Karikari, Annette K Ansong, Frank Edwin, Nana-Akyaa Yao","doi":"10.1177/21501351241299405","DOIUrl":"10.1177/21501351241299405","url":null,"abstract":"<p><p>BackgroundThere 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.MethodsWe 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.ResultsWe 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 (<i>P</i> = .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).ConclusionsThe 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.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"357-367"},"PeriodicalIF":0.0,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142866255","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}