William B. Swicord MD, David C. Cleveland MD, John D. Cleveland MD
{"title":"Pediatric Cardiac Xenotransplantation as a Bridge to Allotransplantation: State of the Field and Preclinical Insights From a Pediatric Model","authors":"William B. Swicord MD, David C. Cleveland MD, John D. Cleveland MD","doi":"10.1053/j.pcsu.2026.02.008","DOIUrl":"10.1053/j.pcsu.2026.02.008","url":null,"abstract":"<div><div>Infants with complex congenital heart disease represent a vulnerable subset of pediatric patients who experience unacceptably high mortality while awaiting cardiac allotransplantation. The limited availability of size-matched donor organs and the unsuitability of existing mechanical circulatory support in this patient population compels the development of alternative bridging strategies. Cardiac xenotransplantation using genetically modified porcine donors represents a potential solution to this complex clinical issue. In this review, we describe the scientific and clinical landscape of pediatric cardiac xenotransplantation, summarize the key findings from a preclinical baboon model of orthotopic cardiac xenotransplantation as a bridge to allotransplantation, and discuss future directions required to bring this concept to clinical implementation.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 109-114"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807574","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 Major Aorto-Pulmonary Collateral Arteries (MAPCAs): Timing and Type of Intervention","authors":"David J. Barron FRCS (CT)","doi":"10.1053/j.pcsu.2025.11.002","DOIUrl":"10.1053/j.pcsu.2025.11.002","url":null,"abstract":"<div><div>Management of this complex condition has evolved considerably over the past 30 years, transforming the prognosis for this rare and heterogeneous condition. Central to this evolution has been the concept of achieving \"unifocalisation\" together with the combined approaches of MAPCA recruitment and vascular rehabilitation. Improvements in imaging modalities and applications have transformed the anatomical assessment and understanding of the pulmonary vasculature, which in turn, has facilitated refined surgical techniques and approaches to achieve these improved outcomes. Early assessment enables clinicians to create a precise \"road-map\" of the pulmonary vasculature, and allow for meticulous planning of the surgical procedures. This article breaks down the management into 6 key decisions that need to be taken in order to achieve the best outcomes across all the variants of the condition.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 69-77"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807573","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}
Charles D. Fraser Jr MD , Davi F. Tenorio MD , Lee M. Fuentes MD , Kaoutar Farahi MD , Hani K. Najm MD
{"title":"Pulmonary Atresia With Intact Ventricular Septum: Expert Review of the Surgical Management","authors":"Charles D. Fraser Jr MD , Davi F. Tenorio MD , Lee M. Fuentes MD , Kaoutar Farahi MD , Hani K. Najm MD","doi":"10.1053/j.pcsu.2026.02.009","DOIUrl":"10.1053/j.pcsu.2026.02.009","url":null,"abstract":"<div><div>Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare but highly heterogeneous congenital heart lesion defined by absence of continuity between the right ventricle (RV) and pulmonary arteries, a morphologically variable tricuspid valve (TV), RV hypoplasia and frequent right ventricle to coronary connections. Management has evolved from uniform surgical palliation toward nuanced, morphology-driven individualized strategies. Current approaches include transcatheter perforation, surgical valvotomy, staged ventricular recruitment, and single-ventricle or 1.5-ventricle palliation, with transplantation reserved for select patients. Recent publication reported a successful procedure to palliate coronary RV dependency with an aortic to TV conduit. Long-term outcomes hinge on RV growth potential, coronary anatomy, and institutional expertise.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 60-68"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807572","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":"Bilateral Bronchus Suis Tracheoplasty","authors":"Michael E. Mitchell MD , Margaret Ann Butler BS","doi":"10.1053/j.pcsu.2026.02.005","DOIUrl":"10.1053/j.pcsu.2026.02.005","url":null,"abstract":"<div><div>Complex tracheal anomalies are life threatening without intervention and often require surgery in the neonatal period to establish a stable airway. Variations of slide tracheoplasty using all autologous tissue are the gold standard in tracheal reconstruction. We have encountered 9 patients whose complex tracheal anomalies required significant variations to slide tracheoplasty. Six of these patients presented with bronchus suis with extreme long segment tracheobronchial stenosis which were effectively repaired using a side-to-side bronchus suis tracheobronchoplasty. This technique has the advantage of providing an all-autologous reconstruction with significantly less tension. Recently, we have encountered 3 patients with even more radical anatomies consisting of long segment tracheobronchial stenosis in the context of bilateral bronchus suis or Christmas-tree trachea. We employed bilateral side-to-side bronchus suis tracheoplasty in one and anterior-posterior bilateral bronchus suis tracheoplasty in the remaining 2 cases. These techniques have proven extremely effective in stabilizing the airway while preserving growth potential. Early management of these patients remains challenging, and complications are exponentially magnified by the interplay between complex cardiac physiology and complex tracheal reconstruction.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 78-84"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807576","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":"How I Do It: Modified Senning Double Switch For Criss-Cross Heart With AV/VA Discordance","authors":"Kenichi Okamura MD, Minoo N. Kavarana MD","doi":"10.1053/j.pcsu.2026.02.004","DOIUrl":"10.1053/j.pcsu.2026.02.004","url":null,"abstract":"<div><div>The criss-cross heart with double outlet right ventricle (DORV) and combined atrioventricular (AV) and ventriculoarterial (VA) discordance presents one of the most formidable challenges in congenital cardiac surgery. The unique ventricular topology and complex inflow-outflow relationships often preclude standard approaches to biventricular repair, and single-ventricle palliation is frequently selected. However, in appropriately selected patients, anatomical repair through a double switch procedure may offer superior long-term outcomes by preserving systemic ventricular function. This report describes a surgical strategy for addressing a criss-cross heart with DORV and AV/VA discordance using a modified Senning procedure as part of a double switch operation. Preoperative imaging—including transthoracic echocardiography, computed tomography angiography, and cardiac catheterization—is critical for assessing operability. Particular attention must be paid to pulmonary vascular resistance, Qp/Qs, morphologic left ventricular end-diastolic pressure, and coronary anatomy. The surgical technique involves ventricular septal defect baffle closure to direct left ventricular output to the pulmonary artery, an arterial switch operation, and a modified Senning atrial switch. The Senning component is performed using a pedicled autologous pericardial baffle augmented with a large bovine pericardial patch to prevent pulmonary venous pathway narrowing, a frequent concern in patients with criss-cross anatomy. This approach allows for biventricular repair even in cases with severe atrial and ventricular malalignment. The technique aims to achieve unobstructed systemic and pulmonary venous pathways, minimize arrhythmia risk, and establish long-term left ventricular systemic circulation. Careful patient selection and meticulous surgical execution are essential for optimizing outcomes in this complex subgroup of congenital heart disease.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 24-31"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807513","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":"Balanced Atrio Ventricular Canal Defect: Distilling the Data","authors":"Meena Nathan MD, MPH , Alyssa Tutunjian MD, MS, MPH , Gerald Marx MD","doi":"10.1053/j.pcsu.2026.03.001","DOIUrl":"10.1053/j.pcsu.2026.03.001","url":null,"abstract":"<div><div>Complete atrioventricular canal defect (CAVC) is the seventh most common congenital heart defect, affecting 1 in 1712 live births. Embryologically CAVC defect arises from failure of normal fusion of the endocardial cushions resulting in a spectrum of abnormalities that involve the atrial septum, atrioventricular (AV) valve, subvalvar apparatus, and the ventricular septum. This article will discuss historic timelines, evolution of management strategies, and outcomes following current surgical approaches in well-balanced CAVC. Recent advances in genetics and imaging techniques will also be discussed.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 42-53"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807575","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}
Heidi Van MD, Christopher J. Petit MD, Oliver M. Barry MD, MHS
{"title":"Transcatheter Interventional Options in Pulmonary Vein Stenosis: When to Stent?","authors":"Heidi Van MD, Christopher J. Petit MD, Oliver M. Barry MD, MHS","doi":"10.1053/j.pcsu.2026.02.007","DOIUrl":"10.1053/j.pcsu.2026.02.007","url":null,"abstract":"<div><div>Pulmonary vein stenosis (PVS) is a rare and aggressive condition in infants and children, characterized by progressive neointimal proliferation, multivessel involvement, and high early mortality. Despite historically poor outcomes, a growing body of evidence supports the use of catheter-based interventions as a cornerstone of modern multimodal treatment strategies. Balloon angioplasty, and bare-metal or drug-eluting stents are used to restore vessel patency, often as part of serial procedures. However, recurrent stenosis remains common, necessitating frequent reintervention. Reintervention is associated with improved survival, particularly when paired with early diagnosis, aggressive surveillance, and systemic medical therapies. When transcatheter interventions are applied as part of a multidisciplinary approach, they contribute to meaningful improvements in survival and quality of life. Ongoing innovation in device technology and biologic modulation will be critical in further advancing outcomes for this high-risk population.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 9-16"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807511","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":"Rewriting the 10 Commandments: Fontan Candidacy in the Modern Era","authors":"Anne C. Taylor MD , Matthew L. Stone MD, PhD","doi":"10.1053/j.pcsu.2026.01.002","DOIUrl":"10.1053/j.pcsu.2026.01.002","url":null,"abstract":"<div><div>Single ventricle congenital heart disease (SVCHD) remains a high-risk pathology for transplantation-free survival and quality of life. While medical and surgical advancements have significantly improved attrition through palliation and ultimately survival following the Fontan operation, Fontan candidacy evaluation remains a critical component to insuring optimal outcomes for patients with SVCHD. While the initial 10 Commandments proposed by Choussat and colleagues provided pioneering insights and supported early success of the Fontan operation, advancements in care have shifted the focus of Fontan candidacy determination to be patient- and center-specific, seeking to maintain both Fontan and transplantation candidacy across all risk categories. Herein, we present a focused review of the current state of Fontan candidacy determination while also offering a multidisciplinary perspective within this evolving and nuanced aspect of care for patients with SVCHD.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 17-23"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807512","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}
Muhammed Ikbal Aydin MD, MEBCTS , Mahmut Ozturk MD , Can Yerebakan MD, PhD , Yves d’Udekem MD, PhD
{"title":"Pushing the Limits of Balance in Atrioventricular Septal Defect and Complex Techniques for Valve Re-repair in Atrioventricular Septal Defect","authors":"Muhammed Ikbal Aydin MD, MEBCTS , Mahmut Ozturk MD , Can Yerebakan MD, PhD , Yves d’Udekem MD, PhD","doi":"10.1053/j.pcsu.2026.01.001","DOIUrl":"10.1053/j.pcsu.2026.01.001","url":null,"abstract":"<div><div>Repair of atrioventricular septal defect (AVSD) remains one of the most technically demanding procedures in congenital cardiac surgery. In patients with balanced anatomy, biventricular repair provides excellent long-term survival; however, borderline and unbalanced AVSD continue to pose major challenges, particularly in children with Down syndrome. Contemporary practice emphasizes that balance is not fixed but can be influenced by surgical planning, ventricular recruitment, and meticulous management of the left atrioventricular valve (LAVV). LAVV regurgitation remains the principal cause of early failure and late reoperation after AVSD repair, and its impact is especially pronounced in the setting of repeat operations, where annular dilation, leaflet deficiency, and prior surgical manipulation complicate repair. Institutional experience demonstrates that immediate moderate or greater LAVV regurgitation after repair strongly predicts both mortality and reoperation, highlighting the importance of rigorous intraoperative echocardiographic assessment and close collaboration between surgeons and cardiologists. Mild to moderate regurgitation represents a surgical “trap,” as it frequently worsens during early follow-up. Longitudinal data show that nearly half of patients with postoperative moderate regurgitation deteriorate to severe, while almost one third improve spontaneously. Successful redo repair requires mechanism-driven strategies, including cleft closure or patch plasty, annuloplasty, papillary muscle splitting, and leaflet augmentation, tailored to residual pathology. Although advances in imaging, surgical techniques, and experience have improved outcomes, recurrent LAVV regurgitation continues to limit survival and freedom from reoperation. Early recognition and aggressive management remain essential, and the surgical threshold for reintervention should be low to optimize long-term results in this complex population.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 54-59"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807571","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":"Airway Malacia: Stent Innovations and Emerging Ex Vivo Testing Platforms","authors":"Abhijit Mondal PhD , Aditya K. Kaza MD, MBA","doi":"10.1053/j.pcsu.2026.02.001","DOIUrl":"10.1053/j.pcsu.2026.02.001","url":null,"abstract":"<div><div>Tracheobronchomalacia is classified as a benign condition characterized by airway collapse resulting from weakening of the airway walls. Current treatment options range from simpler measures, such as the use of a continuous positive airway pressure machine, to more complex surgeries. Airway stents provide a middle ground in terms of treatment invasiveness and post-procedure recovery. Unfortunately, long-term implantation of stents is not clinically viable due to complications of excessive granulation tissue formation and high impedance to mucociliary clearance. Existing stent systems are not optimally designed for benign airway collapse and do not directly address these complications. Optimally designed helical stents, fabricated from both metallic and non-metallic materials, demonstrate potential to address these complications and offer a system suitable for long-term implantation. While a variety of strategies are being investigated to develop viable stent systems, there is a lack of standardized and precise stent-testing platforms. Recent progress in <em>ex vivo</em> tissue-based platforms shows promise of making meaningful progress in airway stent development.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 85-89"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807577","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}