Lily Suh, Jason R Buckley, Jessica E Hook, Dennis R Delany, Minoo N Kavarana, Shahryar M Chowdhury, Laura E Hollinger, John M Costello
{"title":"Risk Factors and Outcomes of Perioperative Extracorporeal Membrane Oxygenation in Neonates and Infants Undergoing Truncus Arteriosus Repair.","authors":"Lily Suh, Jason R Buckley, Jessica E Hook, Dennis R Delany, Minoo N Kavarana, Shahryar M Chowdhury, Laura E Hollinger, John M Costello","doi":"10.1177/21501351241279121","DOIUrl":"10.1177/21501351241279121","url":null,"abstract":"<p><p><b>Background:</b> Despite surgical advances, neonatal truncus arteriosus repair remains high risk and approximately 10% of patients receive perioperative extracorporeal membrane oxygenation (ECMO). We aimed to assess factors and outcomes associated with the use of perioperative ECMO in infants undergoing truncus arteriosus repair. <b>Methods:</b> We conducted a retrospective cohort study of patients who underwent truncus arteriosus repair between 2004 and 2019, using administrative data from the Pediatric Health Information System database. <b>Results:</b> We identified 1,645 neonates and infants who underwent truncus arteriosus repair at 49 centers, of which 141 (8.6%) received ECMO. Prematurity (adjusted odds ratio [aOR], 2.06; 95% CI, 1.38-3.06; <i>P</i> < .001), truncal valve intervention (aOR, 4.69; 95% CI, 2.56-8.59; <i>P</i> < .001), and interrupted aortic arch repair (aOR, 1.80; 95% CI, 0.96-3.38; <i>P</i> = .07) were associated with perioperative ECMO. Hospital mortality occurred in 87 of 141 (62%) patients who received ECMO compared with 77/1504 (5.1%) who did not require ECMO(aOR, 13.39; 95% CI, 8.70-20.61; <i>P</i> < .001). In the 1,481 patients who survived to hospital discharge, ECMO was associated with higher rates of postoperative length of stay >30 days (63% [34/54] vs 28% [400/1427]; aOR 2.65; 95% CI, 1.24-5.64, <i>P</i> = .012) and hospital readmission within 90 days (61% [33/54 [ vs 33% [474/1427] [; aOR, 2.66; 95% CI, 1.47-4.82; <i>P</i> = .001). <b>Conclusions:</b> Prematurity, truncal valve intervention, and interrupted aortic arch repair are important risk factors that could help predict the use of perioperative ECMO. Extracorporeal membrane oxygenation utilization is strongly associated with greater odds of hospital mortality, prolonged postoperative length of stay, and higher rates of hospital readmission in surviving patients.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"246-253"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142515734","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":"Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries: The Natural History and Late Presentation.","authors":"Darshan Reddy, Robert Kleinloog, Robin Kinsley","doi":"10.1177/21501351241311882","DOIUrl":"10.1177/21501351241311882","url":null,"abstract":"<p><p>Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries represents a heterogenous and complex congenital heart defect. Treatment pathways vary widely depending on center expertise. The recommended approach in infancy is to achieve uniform pulmonary artery and capillary bed development, and a biventricular repair when feasible. The natural history of this condition is particularly relevant in low-middle income countries where early intervention is not possible due to poor access to cardiac surgical care. This group of patients may present in late childhood or as adults with symptoms of chronic cyanosis that may be associated with cardiac failure. The surgical approach to this group may range from complete correction to pulmonary artery rehabilitation or unifocalization, or consideration for heart-lung transplantation.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"203-207"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143191333","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":"Milky White Supernatant in the Cardiopulmonary Bypass Circuit in Severe Hypertriglyceridemia During Pediatric Cardiac Surgery: A Case Report.","authors":"Reddy Sandhya Poornima, Unnikrishnan Kp, Aspari Mahammad Azeez","doi":"10.1177/21501351241293656","DOIUrl":"10.1177/21501351241293656","url":null,"abstract":"<p><p>We present a case of severe hypertriglyceridemia (HTG) in a 21-month old female undergoing cardiac surgery for a ventricular septal defect and subaortic membrane excision. During the operation, a milky white supernatant was observed in the cardiopulmonary bypass circuit, prompting immediate lipid profile testing that revealed elevated triglycerides. The management involved maintaining anticoagulation with heparin dose-response testing and ensuring oxygenator function by measuring blood gas analysis, lactate, and arterial line pressure. The child recovered without complications. This case highlights the critical need for prompt identification and management of HTG in pediatric cardiac surgery to mitigate risks and ensure successful outcomes.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"281-283"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142923503","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}
Christo I Tchervenkov, Richard Tang, Giles J Peek, Mark S Bleiweis, Jeffrey P Jacobs
{"title":"Pulmonary Atresia and Ventricular Septal Defect: Definitions, Nomenclature, and Classification.","authors":"Christo I Tchervenkov, Richard Tang, Giles J Peek, Mark S Bleiweis, Jeffrey P Jacobs","doi":"10.1177/21501351241311878","DOIUrl":"10.1177/21501351241311878","url":null,"abstract":"<p><p>This manuscript will provide information about pulmonary atresia + ventricular septal defect, including definitions, nomenclature, and classification, based on the 2021 <i>International Paediatric and Congenital Cardiac Code</i> (IPCCC) and the <i>Eleventh Revision of the International Classification of Diseases</i> (<i>ICD-11</i>). The 2021 IPCCC and <i>ICD-11</i> provide the following definition for Tetralogy of Fallot with pulmonary atresia: Tetralogy of Fallot with pulmonary atresia is defined as \"A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which there is no direct communication between the right ventricle and the pulmonary arterial tree.\" The 2021 IPCCC and <i>ICD-11</i> provide the following definition for Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral arteries: Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral arteries is defined as \"A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which there is no direct communication between the right ventricle and the pulmonary arterial tree and there are collateral blood vessels between the systemic and pulmonary arteries.\" \"Tetralogy of Fallot + pulmonary atresia\" is a specific type of \"pulmonary atresia + ventricular septal defect\" where the intracardiac malformation is more accurately defined. The presence or absence of major aortopulmonary collateral arteries (MAPCA[s]) does not change these definitions. Therefore, \"tetralogy of Fallot + pulmonary atresia + MAPCA(s)\" is a specific type of \"pulmonary atresia + ventricular septal defect + MAPCA(s)\" where the intracardiac malformation is more accurately defined. In the universe of patients with pulmonary atresia + ventricular septal defect, the anatomy and morphology of the pulmonary circulation to a large extent determines the surgical approach and overall outcome, with the intracardiac anatomy playing a secondary role. Based on the characterization of the pulmonary circulation, patients with pulmonary atresia + ventricular septal defect can be classified into three groups: In type A, pulmonary blood flow is provided by native pulmonary arteries (NPAs). In type B, pulmonary blood flow is provided by both NPA and MAPCA(s). In type C, pulmonary blood flow is provided only by MAPCA(s).</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"173-176"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143257711","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}
Jeffrey P Jacobs, James D St Louis, Darshan Reddy, Attilio A Lotto, Osami Honjo, Sivakumar Sivalingam, Robert D B Jaquiss, Ignacio Juaneda, Kirsten Finucane, James K Kirklin, Christo I Tchervenkov, Carl L Backer
{"title":"Introduction to Proceedings of the World University for Pediatric and Congenital Heart Surgery Webinars About Pulmonary Atresia and Ventricular Septal Defect.","authors":"Jeffrey P Jacobs, James D St Louis, Darshan Reddy, Attilio A Lotto, Osami Honjo, Sivakumar Sivalingam, Robert D B Jaquiss, Ignacio Juaneda, Kirsten Finucane, James K Kirklin, Christo I Tchervenkov, Carl L Backer","doi":"10.1177/21501351241312679","DOIUrl":"10.1177/21501351241312679","url":null,"abstract":"","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"171-172"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143191243","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}
Arvind Bishnoi, Richard D Mainwaring, Anna Seehofnerova, Frank L Hanley
{"title":"Intraconal Left Main Coronary Artery Originating From the Left Sinus of Valsalva.","authors":"Arvind Bishnoi, Richard D Mainwaring, Anna Seehofnerova, Frank L Hanley","doi":"10.1177/21501351241293152","DOIUrl":"10.1177/21501351241293152","url":null,"abstract":"<p><p>Anomalous left main coronary artery (LMCA) with an intraconal course is a relatively rare form of anomalous coronary artery. Intraconal LMCA typically originates from the right sinus of Valsalva. However, this report describes the very unusual circumstance of an intraconal LMCA originating from the left sinus.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"279-281"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142635457","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}
Pezad Doctor, Irtiza S Islam, Catherine M Ikemba, Munes Fares, Jay Moore, Gregory Sturgeon, Robert Db Jaquiss, Nicholas D Andersen
{"title":"Three-Dimensional Imaging Defines Straddling Mitral Valve Anatomy to Allow Successful Biventricular Repair.","authors":"Pezad Doctor, Irtiza S Islam, Catherine M Ikemba, Munes Fares, Jay Moore, Gregory Sturgeon, Robert Db Jaquiss, Nicholas D Andersen","doi":"10.1177/21501351241297709","DOIUrl":"10.1177/21501351241297709","url":null,"abstract":"<p><p>Biventricular repair of a straddling mitral valve (MV) can involve relocating ectopic papillary muscles and chordae. However, this increases operative complexity and risks MV incompetence. We describe a nine-month-old with D-transposition of the great arteries and straddling MV. Three-dimensional imaging identified ventricular septum malposition and defined a simple repair strategy via ventricular septal defect patch closure rightward of the ectopic papillary muscle along with the arterial switch operation. This case highlights the role of 3D imaging in planning safe biventricular repair of straddling MV in an infant.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"283-286"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142690216","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":"Pulmonary Atresia With Ventricular Septal Defect Without Major Aorto-Pulmonary Collateral Arteries: Echocardiography and the Role of Computed Tomography and Magnetic Resonance Imaging.","authors":"Sarah Chambers Gurson","doi":"10.1177/21501351241289128","DOIUrl":"10.1177/21501351241289128","url":null,"abstract":"<p><p>Pulmonary atresia with ventricular septal defect (PA-VSD) is usually diagnosed by transthoracic or fetal echocardiography, with the prenatal diagnosis being feasible and accurate if fetal cardiology services are available. The limitations of transthoracic echocardiography (TTE) in the evaluation of PA-VSD include the complete evaluation of the pulmonary arteries and patent ductus arteriosus, quantitative evaluation of the right ventricle size and function, and delineation of associated cardiac anomalies such as coronary artery anomalies, anomalies of systemic or pulmonary venous return, and complex arch anomalies. Echocardiography also has limitations in evaluating hemodynamics such as flow volumes, shunts, and regurgitant fraction. Despite these limitations, TTE remains the most widely available and the most cost-effective cardiac imaging modality for patients with PA-VSD and its accuracy in evaluating the sources of pulmonary blood flow and in selecting patients for systemic-to-pulmonary artery shunt palliation is well established. Cardiac computed tomography (CT) can answer many of the questions not answered by TTE, including demonstrating the PA anatomy and defining coronary artery, systemic and pulmonary venous, and aortic arch anatomies. The short acquisition time allows for the study to be performed without sedation/anesthesia in most patients. Cardiac CT is also useful in defining postoperative anatomy when there are non-magnetic resonance imaging (MRI) compatible devices or even MRI-compatible devices that cause a significant MRI artifact. Cardiac MRI/magnetic resonance angiography has emerged as an ideal modality to evaluate patients with PA-VSD as it allows for anatomic, functional, and hemodynamic assessment without exposure to ionizing radiation or iodinated contrast material. Both cardiac CT and cardiac MRI can be used to generate 3D imaging of the heart.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"183-190"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142974121","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}
Andres M Palacio, William G Williams, David J Barron, Madison B Argo, Anusha Jegatheeswaran, Marshall L Jacobs, Igor Bondarenko, Karl F Welke, James K Kirklin, Tara Karamlou, Bahaaldin Alsoufi, Brian W McCrindle
{"title":"Management of Tricuspid Atresia With Normally Related Great Arteries and Left-Sided Obstruction.","authors":"Andres M Palacio, William G Williams, David J Barron, Madison B Argo, Anusha Jegatheeswaran, Marshall L Jacobs, Igor Bondarenko, Karl F Welke, James K Kirklin, Tara Karamlou, Bahaaldin Alsoufi, Brian W McCrindle","doi":"10.1177/21501351241286441","DOIUrl":"10.1177/21501351241286441","url":null,"abstract":"<p><p>BackgroundTricuspid atresia (TA) is the second most common form of functionally univentricular heart. For patients with TA and normally related great arteries (Type I), left ventricular outflow tract obstruction (LVOTO) is rare.MethodsFrom the Congenital Heart Surgeons' Society multi-institutional cohort of 445 patients with Type I TA enrolled from 1999 to 2024 from 42 sites, 14 infants (3%) had interventions for associated LVOTO, either at presentation or after their first TA-related intervention.ResultsOf seven infants initially undergoing Norwood/Damus-Kaye-Stansel (DKS), six survived to Stage II, of whom five survived with one developing pulmonary hypertension and four achieving Fontan. An additional seven infants who were first managed with pulmonary artery band placement subsequently had bidirectional superior cavopulmonary anastomosis (BCPA) and a DKS procedure; there were six survivors, all achieving Fontan. All ten survivors who underwent the Fontan procedure had normal left ventricular and mitral valve function at the latest follow-up. The overall Kaplan-Meier survival estimate at 20 years for these 14 patients was 79% (70% CI, 66%-88%), and the median follow-up was 8.3 years (0.24-21.5).ConclusionsWhile infants with TA and transposition of the great arteries are more likely to have LVOTO, this can also occur in the setting of normally related great arteries. Infants with Type I TA and LVOTO can be managed in the neonatal period with the Norwood procedure ensuring complete arch relief with acceptable outcomes. If LVOTO becomes evident after initial pulmonary artery band placement, a subsequent DKS procedure facilitates satisfactory success to Fontan.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"254-261"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11951366/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142669881","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}
Salman A Shah, Mohammad Umair Khan, Nabil Awan, Faiz Rasool, Tehmina Kazmi, Amina Zafar Qureshi, Saaniya Hasan, Talal Ahmad Shah, M Asim Khan, Uzma Kazmi, Najam Hyder, Masood Sadiq
{"title":"Surgical Ligation of Isolated Type I Aortopulmonary Window Without Cardiopulmonary Bypass is a Safe and Cost-Effective Surgical Technique.","authors":"Salman A Shah, Mohammad Umair Khan, Nabil Awan, Faiz Rasool, Tehmina Kazmi, Amina Zafar Qureshi, Saaniya Hasan, Talal Ahmad Shah, M Asim Khan, Uzma Kazmi, Najam Hyder, Masood Sadiq","doi":"10.1177/21501351251315142","DOIUrl":"https://doi.org/10.1177/21501351251315142","url":null,"abstract":"<p><strong>Introduction: </strong>Aortopulmonary window (APW) is an extremely rare congenital anomaly of the heart where there is a direct communication between the ascending aorta and main pulmonary artery. Surgical repair of APW on cardiopulmonary bypass (CPB) is considered the standard of surgical care. In this study, we selected patients with a type I APW to undergo direct surgical ligation to determine if this technique is safe and can produce equivalent results to standard open-heart repair.</p><p><strong>Method: </strong>Twelve infants with simple or isolated type I APW underwent direct surgical ligation by a single surgeon at Children's Hospital, Lahore, Pakistan, from December 2020 to January 2024. Diagnosis was based on two-dimensional transthoracic echocardiography, and classification was based on the Society of Thoracic Surgeons (STS) nomenclature. A sternotomy approach was employed, and CPB was on stand-by for all cases. The aortic and pulmonary ends of the APW were ligated with close attention to anatomic details.</p><p><strong>Results: </strong>All patients underwent successful APW ligation with no intraoperative complications. Follow-up, ranging from 6 months to 3 years, revealed no mortality, residual defects, aortic or pulmonary valve insufficiency, branch pulmonary artery stenosis, or coronary compromise. A notable cost benefit of $2,000 per patient was observed.</p><p><strong>Conclusion: </strong>In this case series, direct surgical ligation of an APW was found to be a safe and cost-effective alternative to standard surgical repair on CPB. Further research with a larger sample size with longer follow-up is required to better determine this alternative's efficacy.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"21501351251315142"},"PeriodicalIF":0.0,"publicationDate":"2025-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143517722","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}