Alessia Di Nardo B Eng , Elizabeth D Persaud HBSc , Rachel D Vanderlaan MD, PhD
{"title":"Postrepair Pulmonary Vein Stenosis: Addressing Anatomic and Patient Risk Factors to Improve Outcomes","authors":"Alessia Di Nardo B Eng , Elizabeth D Persaud HBSc , Rachel D Vanderlaan MD, PhD","doi":"10.1053/j.pcsu.2025.02.008","DOIUrl":"10.1053/j.pcsu.2025.02.008","url":null,"abstract":"<div><div>Surgical results for correction of total anomalous pulmonary venous connection (TAPVC) have improved in the current era. Postrepair pulmonary vein stenosis (PR-PVS) remains a major complication following TAPVC repair, often necessitating multiple reinterventions, and impacting patient outcomes. Considerations for patient and anatomical risk factors, surgical techniques for primary TAPVC repair, as well PR-PVS postoperative surveillance and management will be reviewed. We will also touch on emerging areas of research related to PR-PVS pathogenesis and incorporation of patient-specific simulation for surgical planning in TAPVC.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 107-116"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948349","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}
Emile A. Bacha MD, Stephanie N. Nguyen MD, Andrew B. Goldstone MD, PhD, David Kalfa MD, PhD
{"title":"A Perspective on Neonatal Management of Hypoplastic Left Heart Syndrome","authors":"Emile A. Bacha MD, Stephanie N. Nguyen MD, Andrew B. Goldstone MD, PhD, David Kalfa MD, PhD","doi":"10.1053/j.pcsu.2024.12.002","DOIUrl":"10.1053/j.pcsu.2024.12.002","url":null,"abstract":"<div><div>Hypoplastic left heart syndrome (HLHS)—a universally fatal condition only 40 years ago—remains one of the most challenging congenital heart defects. The advancements and insights gained from historical experiences with the Norwood operation have transformed the prognosis for HLHS patients and profoundly shaped contemporary outcomes. This progress has spurred continuous refinement of surgical techniques, transcatheter interventions, and perioperative care, leading to notable improvements in both survival rates and quality of life for HLHS patients. Herein, we focus on key historical lessons and their implications for future practices for neonatal management of HLHS.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 3-12"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948358","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}
Justus G. Reitz MD , Sofia Hanabergh MS , Eiri Kisamori MD , Yves d'Udekem PhD, MD
{"title":"Mitral Valve Repair Techniques in Neonates and Infants","authors":"Justus G. Reitz MD , Sofia Hanabergh MS , Eiri Kisamori MD , Yves d'Udekem PhD, MD","doi":"10.1053/j.pcsu.2025.01.003","DOIUrl":"10.1053/j.pcsu.2025.01.003","url":null,"abstract":"<div><div>Mitral valve repair is often preferred over replacement due to the preservation of native valve function, allowing for somatic growth and resulting in fewer complications and reoperations. Although there are no randomized trials comparing repair and replacement specifically in this age group, retrospective studies still indicate better long-term survival and lower reoperation rates with repair. While neonates and infants experience higher mortality rates compared to older children, freedom from re-intervention is durable in the survivors and matches that of older children. Especially compression suture annuloplasty and chordal replacement using Gore-Tex sutures have shown favorable outcomes, but the rarity of the condition makes it challenging to train new surgeons. Utilizing detailed video documentation could help bridge this educational gap. While advancements in materials for valve repair in neonates and infants have been slow, emerging options like new materials that grow with the patient offer potential for future innovation. In complex cases such as Shone's syndrome, new techniques, including an-bloc replacement of the aorta and mitral valve, may improve outcomes.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 46-50"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948032","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}
Ayman Almousa MD , Andrew Behrmann , Paighton Miller MD , Shamik Bhattacharya PhD , Dilip Nath MD , Jacob R. Miller MD , Pirooz Eghtesady MD, PhD
{"title":"Use of Right Atrial Appendage Tissue for Pulmonary Valve Reconstruction in Tetralogy of Fallot","authors":"Ayman Almousa MD , Andrew Behrmann , Paighton Miller MD , Shamik Bhattacharya PhD , Dilip Nath MD , Jacob R. Miller MD , Pirooz Eghtesady MD, PhD","doi":"10.1053/j.pcsu.2025.02.010","DOIUrl":"10.1053/j.pcsu.2025.02.010","url":null,"abstract":"<div><div>We explore the innovative use of atrial appendage tissue for creating pulmonary valves in children, focusing on its application in Tetralogy of Fallot repair. We discuss the background, preclinical studies, surgical approach, and early clinical outcomes, demonstrating the potential of this technique in reducing pulmonary insufficiency and improving surgical outcomes.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 60-67"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948024","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":"Don't Give up on the Left Ventricle! Surgical Strategies for Recruitment of the Borderline Left Heart","authors":"Gianna Dafflisio BS, Sitaram M. Emani MD","doi":"10.1053/j.pcsu.2025.02.009","DOIUrl":"10.1053/j.pcsu.2025.02.009","url":null,"abstract":"<div><div>Patients with hypoplastic left heart syndrome often undergo a definitive decision in infancy regarding lifetime circulatory management. However, the decision between single ventricle palliation or primary biventricular repair is less obvious for patients with ‘borderline left heart’ structures. An alternative strategy for the patient with borderline left heart is staged left ventricular recruitment (SLVR). SLVR refers to the strategy in which initial stage I palliation is pursued, and interstage procedures are then performed to promote flow through the left heart and induce growth of left heart structures. If ventricular response to SLVR is robust, eventual biventricular conversion can be pursued. At our institution, 53 patients have undergone (or are undergoing) SLVR for hypoplastic left heart syndrome, with 29 patients successfully progressing to biventricular or 1.5 ventricle repair. This paper outlines the rationale and management strategy of SLVR from the past two decades of our center's experience.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 13-20"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948357","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":"What is the Optimal Surgical Technique for Anomalous Aortic Origin of a Coronary Artery?","authors":"Carlos M. Mery MD, MPH , Ziv Beckerman MD","doi":"10.1053/j.pcsu.2025.02.006","DOIUrl":"10.1053/j.pcsu.2025.02.006","url":null,"abstract":"<div><div>Anomalous aortic origin of a coronary artery (AAOCA) is associated with an increased risk of sudden cardiac death. Multiple surgical techniques have been developed to treat this anomaly. The optimal surgical technique to treat AAOCA is unclear but varies depending on the specific patient anatomy. We discuss 3 different techniques: unroofing of an intramural segment, coronary transection and reimplantation, and creation of a neo-ostium. Unroofing is optimal when the anomalous coronary has a long intramural segment that travels above the aortic valve. Creation of a neo-ostium by partially unroofing the coronary at the appropriate sinus should be considered if there is a long intramural segment that travels below the aortic valve. Coronary reimplantation is optimal if the intramural segment travels below the aortic valve but is not long enough for creation of a neo-ostium, or if there is a short or nonexistent intramural segment. Surgical technique should be individualized based on patient anatomy, and all potential anatomic culprits addressed.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 94-100"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948347","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}
Luciana da Fonseca da Silva MD , Victor Morell MD , Mario Castro Medina MD , Jose Pedro da Silva MD
{"title":"Staged Treatment for Critically Ill Neonates With Ebstein Anomaly: Cone Repair After Starnes Procedure","authors":"Luciana da Fonseca da Silva MD , Victor Morell MD , Mario Castro Medina MD , Jose Pedro da Silva MD","doi":"10.1053/j.pcsu.2025.02.007","DOIUrl":"10.1053/j.pcsu.2025.02.007","url":null,"abstract":"<div><div>Following the Starnes procedure, a progressive reduction in the RV cavity occurs, suggesting that the RV was unsuitable for a biventricular repair. For that reason, those patients traditionally followed the single ventricle pathway. In 2019, we performed the initial tricuspid valve (TV) repair cases after Starnes. Since then, we have routinely applied the staged treatment with Cone repair after Starnes achieving 2-ventricle or one-and-a-half ventricle repair in 17 patients. A Glenn procedure associated with pulmonary valve (PV) commissurotomy has been offered as an intermediate stage in patients with associated pulmonary atresia (PAt). Aiming to facilitate the following surgical steps and allow RV growth, we have described some maneuvers for these patients during the Starnes and the Glenn procedure. In this paper, we describe the up-to-date staged repair for critical neonatal Ebstein, emphasizing surgical details of the Starnes procedure, the intermediate interventions, and the Cone repair after Starnes.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 38-45"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948031","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":"Ross Procedure in Adolescence: Replacement is Better Than Repair, Mostly","authors":"Justin Robinson MD , David S. Winlaw MBBS, MD","doi":"10.1053/j.pcsu.2025.03.005","DOIUrl":"10.1053/j.pcsu.2025.03.005","url":null,"abstract":"<div><div>Surgical management of isolated aortic valve pathology in adolescents primarily involves aortic valve repair and the Ross procedure, each with distinct advantages and limitations influenced by anatomical considerations. The optimal surgical strategy in this population remains a subject of ongoing debate, as it must balance the need for future growth potential with the goal of maintaining near-normal hemodynamics while minimizing reinterventions and maximizing survival. Ultimately, the choice of procedure should be tailored to the patient's clinical and anatomical characteristics. Here, we critically examine the existing data on aortic valve repair, with a particular emphasis on the role and outcomes of the Ross procedure in adolescents.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 68-78"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948344","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":"Donation After Circulatory Determination of Death in Congenital Heart Disease: Should We Embrace?","authors":"Reshma M. Biniwale MD","doi":"10.1053/j.pcsu.2025.02.005","DOIUrl":"10.1053/j.pcsu.2025.02.005","url":null,"abstract":"<div><div>The lack of donors that are age and size appropriate has compromised pediatric heart transplantation. There may be poor outcomes following mechanical circulatory support as a bridge to heart transplant in certain younger patient cohorts, which combined with the scarcity of donors, increases the morbidity and mortality in children waiting for a heart transplant. Transplantation of hearts donated after circulatory determination of death (DCDD) has been utilized to increase the pool of pediatric donor allografts. The original approach included direct procurement and perfusion in the recipient (DPP), and recently ex-situ normothermic machine perfusion (NMP). However, NMP is expensive, and this methodology does not allow for a functional assessment in a loaded state of the donor organ prior to transplantation. NMP is also not available to the smallest pediatric patients. An alternative method, normothermic regional perfusion (NRP), can overcome the limitations of NMP. NRP is a thoraco-abdominal dynamic in-situ organ assessment method and is instituted following declaration after determination of circulatory death. In-situ tissue perfusion is established to the transplantable organs using a bypass circuit based on strict, prespecified guidelines that do not violate the “dead donor rule.”</div><div>The first pediatric DCDD heart transplant using NRP was performed in 2019, and since then, several centers have reported immense success. Ethical concerns for cerebral reperfusion have not been substantiated by clinical data. Utilization of DCDD donors can vastly expand the donor pool in congenital heart disease (CHD).</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 124-129"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948351","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}