Sairam Palaparthi MD , Nicholas A. Oh MD , Benjamin Zendejas-Mummert MD , Christopher W. Baird MD
{"title":"Surgical Approaches for Vascular-Related Aerodigestive Compression","authors":"Sairam Palaparthi MD , Nicholas A. Oh MD , Benjamin Zendejas-Mummert MD , Christopher W. Baird MD","doi":"10.1053/j.pcsu.2026.02.002","DOIUrl":"10.1053/j.pcsu.2026.02.002","url":null,"abstract":"<div><div>Aerodigestive compression syndromes of vascular origin can be broadly classified based on congenital and acquired causes. Congenital causes include various types of arch anomalies (vascular rings including double arches, right aortic arch with aberrant left subclavian artery, circumflex aorta, etc.), arch branching anomalies (left aortic arch with aberrant right subclavian artery, innominate artery-related compression), aneurysms of either the aorta or main pulmonary artery or branch pulmonary arteries, and any combinations of these. Acquired causes of aerodigestive compression are mostly postsurgical, which is a distinct and challenging entity usually arising from arch reconstructive procedures, root amalgamation procedures, procedures involving the LeCompte maneuver, and procedures involving PDA stenting. When there are associated thoracic cage and vertebral anomalies, an additional layer of complexity is added to an already challenging problem. At Boston Children’s Hospital, these patients are evaluated with a multidisciplinary team using diagnostic studies, including echocardiography, esophagram, computed tomographic angiography, and three-phase dynamic bronchoscopy for a thorough understanding of anatomic and physiologic complexities. Surgical correction to relieve compression requires an individualized plan for each patient, and 3D models are routinely employed. Posterior thoracotomy, sternotomy, and video-assisted thorocoscopy (VATs) are approaches to correct these problems with additional concomitant usage of aortopexy, tracheopexy, and localized or complete esophageal mobilization. Intraoperative bronchoscopy is widely used both for surgical planning and for confirmation of relief of compression.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 90-101"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807569","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}
Sahithi Bhavana MBBS , Rachel Johnstone MM , Wasim Khan MD , Robert D. Stewart MD , Tara Karamlou MD, MSc
{"title":"Ross Procedure After Arterial Switch Operation?","authors":"Sahithi Bhavana MBBS , Rachel Johnstone MM , Wasim Khan MD , Robert D. Stewart MD , Tara Karamlou MD, MSc","doi":"10.1053/j.pcsu.2026.02.003","DOIUrl":"10.1053/j.pcsu.2026.02.003","url":null,"abstract":"<div><div>Arterial switch operation (ASO) has been the standard anatomic repair for D-transposition of the great arteries (d-TGA) since Jatene et al. first described it in 1975. While survival is excellent, some patients develop late complications requiring reoperation, including neo-aortic regurgitation (neo-AR) and root dilatation (ARD). Mechanisms of neo-AR are multifactorial, and management depends on whether the pathology is leaflet- or root-driven. When neo-AR is secondary to ARD, options include valve-sparing root replacement (VSRR) or composite root replacement (Bentall procedure) using either a mechanical or a bioprosthetic valve. In cases of isolated neo-AR without significant ARD, valve repair with annular stabilization can be considered. Alternatively, such cases may be managed with the Ross procedure, first described as ‘Switch-Back Ross’ by Hazekamp et al., although this approach remains less commonly employed. The specific surgical choice should integrate anatomy, physiology, and patient-specific factors within a shared decision-making framework. This review focuses on the Ross procedure after ASO, with emphasis on operative pearls, expected complications, and durability considerations. We illustrate these principles through the case of a 19-year-old man, late after ASO, who presented with severe neo-AR and mild ARD. He underwent a ‘supported’ Ross with external annuloplasty and recovered uneventfully. This case underscores the feasibility of the Ross procedure when valve repair is nondurable and emphasizes annular stabilization as key to long-term success, contributing to the limited literature on this approach in post-ASO patients.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 32-41"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807514","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}
John A. Kucera MD , Seth E.M. Wolf MD , Lesley Skalla PhD , Joseph W. Turek MD, PhD, MBA , Douglas M. Overbey MD, MPH
{"title":"Comprehensive Options for Pediatric Donation After Circulatory Death Donors","authors":"John A. Kucera MD , Seth E.M. Wolf MD , Lesley Skalla PhD , Joseph W. Turek MD, PhD, MBA , Douglas M. Overbey MD, MPH","doi":"10.1053/j.pcsu.2025.11.001","DOIUrl":"10.1053/j.pcsu.2025.11.001","url":null,"abstract":"<div><div>Cardiac transplant represents the only definitive strategy for children with end-stage heart failure. Pediatric patients face significant wait-list times and resultant mortality due to a worsening shortage of hearts available for transplant. Donation after circulatory death (DCD) represents an opportunity to increase the number of hearts available to pediatric recipients by 30%. DCD donation has been historically limited in application due to logistical challenges and ethical concerns. A systematic review was carried out using the Cochrane Handbook. Eligible studies were identified using MEDLINE (via OVID), Embase (Elsevier), Cochrane Library/Cochrane Central Register of Controlled Trials (CENTRAL; Wiley), and Web of Science Core Collection (Clarivate). This search was conducted by a professional medical librarian in consultation with the author team and validated against a set of pre-selected articles. This resulted in 438 articles, which were screened based on title and abstract by 2 independent authors. Full-text review was subsequently performed, resulting in 21 articles. Agreement for inclusion included pediatric DCD, published in English, and complete manuscript availability. Four principal techniques were identified for pediatric DCD: rapid procurement and static cold storage, organ care systems, normothermic regional perfusion, and on-table reanimation. These all portend unique advantages and disadvantages and have largely evolved due to limitations of prior techniques. Providing a comprehensive approach to pediatric cardiac DCD is the most effective method to ensure organ stewardship, minimize wait list mortality, and mitigate ethical concerns associated with pediatric DCD.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 102-108"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807570","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 Vein Stenosis Medical Therapy: Current and on the Horizon","authors":"Mudit Gupta MD, PhD, David B. Frank MD, PhD","doi":"10.1053/j.pcsu.2026.02.006","DOIUrl":"10.1053/j.pcsu.2026.02.006","url":null,"abstract":"<div><div>Pulmonary vein stenosis (PVS) is a severe vascular obstructive lesion historically associated with poor survival. As outcomes have improved in the current era, PVS has become a chronic disease requiring frequent surgeries, catheter-based interventions, and experimental therapies to prevent progression and recurrence. Despite this, PVS survival remains low and has pushed experimental research into new therapies into the forefront. In this paper, we briefly examine the pathophysiology and etiology of PVS and provide a more comprehensive review of computational and animal modeling of PVS and therapies derived from these studies, both current and on the horizon.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"29 ","pages":"Pages 3-8"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147807578","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}
Christopher Z. Lam MD , Shi-Joon Yoo MD, PhD , Osami Honjo MD, PhD , David J. Barron MBBS, MD
{"title":"Borderline Left Ventricle: Criteria for Surgical Decision Making With an Emphasis on Cardiac Magnetic Resonance Imaging","authors":"Christopher Z. Lam MD , Shi-Joon Yoo MD, PhD , Osami Honjo MD, PhD , David J. Barron MBBS, MD","doi":"10.1053/j.pcsu.2025.02.003","DOIUrl":"10.1053/j.pcsu.2025.02.003","url":null,"abstract":"<div><div>The borderline left ventricle (LV) encompasses a heterogenous group of cardiac defects that result in underdevelopment of the left heart<strong>.</strong> Data supporting decision making is difficult to interpret because borderline LV hypoplasia is a relatively rare disease comprising of a heterogenous morphologic spectrum with data originating from single-institution retrospective studies that have all used varying inclusion criteria and imaging modalities/analysis methods, whilst further confounded by heterogenous institutional practice patterns and era effects. Long-term data is lacking. This invited expert review offers a perspective on how to interpret and use some of the preoperative imaging parameters commonly proposed to decide between primary biventricular repair (BVR) versus LV recruitment. The need to integrate functional cardiovascular magnetic resonance imaging (CMR) parameters is emphasized. Current approaches and a broad framework with imaging criteria are presented.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 21-28"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948029","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}
Bari Murtuza MD , Juan Lehoux MD , Victor O. Morell MD
{"title":"Single and Double Root Translocation for Transposition and Malposition of the Great Arteries","authors":"Bari Murtuza MD , Juan Lehoux MD , Victor O. Morell MD","doi":"10.1053/j.pcsu.2025.01.001","DOIUrl":"10.1053/j.pcsu.2025.01.001","url":null,"abstract":"<div><div>Aortic root translocation and double root translocation have emerged as potentially valuable surgical options for complex transposition of the great arteries or double outlet right ventricle with VSD and LV outflow tract obstruction (LVOTO). These complex approaches offer excellent laminar LV outflow tracts with almost no late LVOT reinterventions. Whilst the advantages for the LVOT are clear, there is an early cost in terms of prolonged operative ischemic times compared with the Rastelli procedure or even arterial switch with LVOTO resection. This appears to translate into a degree of perioperative morbidity. Further, concerning double root translocation, a clear benefit above a single root approach with no right ventricle-pulmonary artery conduit, should be demonstrable. Adoption of root translocation should not come at the expense of a higher incidence of neo-aortic incompetence or mitral regurgitation as has been reported in some series. We suggest that the best outcomes are likely to be achieved where the strategy is tailored to individual patient anatomy and pathophysiology, particularly taking into account: the relative size of the native pulmonary-to-aortic valve; complexity of LVOTO; and presence of abnormal coronary artery patterns or associated lesions. Additional long-term data for these relatively recent techniques are still awaited, although there are some early suggestions that for the most complex reconstructions, there may be some late occurrence of heart failure. Judicious intra-operative assessment is often the final arbiter for the best approach in a given patient.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 51-59"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948033","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}
Elisabeth Martin MD, MPH, Michael Ma MD, Frank L Hanley MD
{"title":"State of the Art: Complex Peripheral Pulmonary Artery Reconstruction Techniques","authors":"Elisabeth Martin MD, MPH, Michael Ma MD, Frank L Hanley MD","doi":"10.1053/j.pcsu.2025.03.002","DOIUrl":"10.1053/j.pcsu.2025.03.002","url":null,"abstract":"<div><div>Over the last several years, we developed and improved our surgical approach to patients requiring pulmonary artery reconstruction in the setting of TOF-PA-MAPCA and other forms of peripheral pulmonary artery stenosis (PPAS). PPAS is a relatively rare form of congenital heart disease and typically associated with genetic syndromes, such as Williams or Alagille syndromes. We no longer do a staged approach and use a midline incision in all cases.</div><div>We have now operated on almost 1000 patients with TOF-PA-MAPCA or extensive bilateral PPAS. Early mortality ranges from 2.1% (3/145 PPAS) to 4.9% (38/780 TOF-PA-MAPCA, total cohort). In our TOF-PA-MAPCA cohort, independent factors for early mortality were Alagille syndrome (HR 2.8, 95% CI: 1.4-5.7; <em>P</em> < 0.004), any preoperative respiratory support (HR 2.0, 95% CI: 1.2-3.3; <em>P</em> < 0.008), and previous palliative surgery at our center (HR 3.5, 95% CI: 2.3-5.4; <em>P</em> < 0.001) on multivariable Cox regression analysis. Complete intracardiac repair was achieved in 90% (704/780) of this cohort.</div><div>This document reports our surgical approach to pulmonary artery reconstruction with emphasis on certain key concepts. Our surgical strategy is applicable to essentially every patient with TOF-PA-MAPCA or PPAS.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 101-106"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948348","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}
Christopher A. Caldarone MD , Peter C. Laussen MBBS, FANZCA, FCICM
{"title":"Learning from Small-Volume Congenital Heart Programs","authors":"Christopher A. Caldarone MD , Peter C. Laussen MBBS, FANZCA, FCICM","doi":"10.1053/j.pcsu.2024.12.001","DOIUrl":"10.1053/j.pcsu.2024.12.001","url":null,"abstract":"<div><div>The relationship between surgical volume and clinical outcomes in congenital heart surgery has been frequently studied and interpreted to indicate that higher volume programs are associated with superior outcomes after congenital heart surgery. Volume-based assumptions have been used to support the notion that volume-specific stratification of case mix would improve overall outcomes in the United States with specific attention to programs with annualized volumes of 75-200 STS index cases/year. Although not intended, some have perceived these recommendations to indicate that programs performing 75-200 annualized index cases/year offer surgical outcomes of lesser quality than higher volume programs. Nevertheless, some programs performing 75-200 annualized index cases/year consistently perform exceptionally well. Because the inherent advantages - and challenges - associated with being a program in this size range have not been frequently examined, this manuscript focuses on describing these attributes. We hypothesize that better understanding of the determinants of high (or low) performance in this size range could have significant impact on delivery of care in the United States.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 130-137"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948352","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":"Improving Standards in Congenital Heart Surgery Using Outcomes Data and Risk Stratification","authors":"S. Ram Kumar MD, PhD, FACS","doi":"10.1053/j.pcsu.2025.03.003","DOIUrl":"10.1053/j.pcsu.2025.03.003","url":null,"abstract":"<div><div>The complex heterogeneity inherent to reparative procedures for congenital heart defects and their relatively small volumes makes comprehensive analyses of outcomes particularly challenging. That said, an incisive understanding of the impact of patient- and disease-specific factors is crucial to improving overall outcomes. Datasets that reflect ‘real world’ contemporary practice of congenital cardiac care and provide collective outcomes data across age groups and institutions in various parts of the world play a vital role in filling this gap. Risk-adjusted benchmark outcomes data offer opportunities to improve the quality and value of care provided at the level of an individual patient, an institution or program, and the overall specialty. On-going enhancements to data collected and their analyses will ensure that the care we provide continues to evolve. Future efforts should be aimed at integrating multiple datasets to ensure access to longitudinal follow-up and effective analysis of long-term outcomes.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 138-142"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948353","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}