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}
{"title":"Advice to Young Athletes With Anomalous Aortic Origin of a Coronary Artery With and Without Surgery","authors":"Silvana Molossi MD, PhD , Shagun Sachdeva MD","doi":"10.1053/j.pcsu.2025.01.002","DOIUrl":"10.1053/j.pcsu.2025.01.002","url":null,"abstract":"<div><div>Congenital coronary artery anomalies remain a leading cause of sudden cardiac death in the young. Within these, anomalous aortic origin of a coronary artery represents the largest group, with anomalies of the left coronary artery carrying higher risk for sudden cardiac arrest and clinical manifestations of myocardial ischemia. In contrast, anomalies of origin of the right coronary artery are more common and generally have a more benign clinical course, though rarely also associated with sudden events and myocardial ischemia. Risk stratification to guide management decisions remains to be well defined, though substantial advances have occurred in the last few years, with assessment of myocardial perfusion under provocative stress being an integral part of the evaluation in the young. Discussion and counseling on exercise behavior is essential to foster healthy lifestyle for these patients, acknowledging shared decision-making should be practiced in lieu of many unanswered questions as to outcomes long-term.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 83-93"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948346","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":"Is the STAT Classification the Best That We Can Do?","authors":"R.D.B. Jaquiss MD","doi":"10.1053/j.pcsu.2025.02.002","DOIUrl":"10.1053/j.pcsu.2025.02.002","url":null,"abstract":"<div><div>Case-mix description and adjustment is foundational for quality assessment and improvement. The current STAT system has evolved and improved over the past 2 decades. While imperfect, in its present form it has been and will be immensely useful as a tool to help guide and assess progress. Herein is presented an abbreviated review of the origin and evolution of the current system. Pitfalls in its use and inherent weaknesses are discussed, and the need for continued revisions and updates is acknowledged. A summary judgement of “good enough for now” is suggested.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 143-147"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948354","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":"Surgical Management of Congenital Aortic and Truncal Valve Disease: A Comprehensive Review","authors":"Supreet P. Marathe MCh , Christopher W. Baird MD","doi":"10.1053/j.pcsu.2025.03.001","DOIUrl":"10.1053/j.pcsu.2025.03.001","url":null,"abstract":"<div><div>Congenital aortic and truncal valve diseases pose significant challenges due to their complex anatomy, diverse clinical presentations, and impact on long-term outcomes. Surgical management requires a tailored approach, balancing the need for immediate functional improvement with strategies to minimize reintervention, especially in growing children. This article outlines different surgical options to treat this pathology. Despite multiple choices, surgical decision-making remains complex, influenced by patient age, valve morphology, and comorbidities. Long-term outcomes depend on achieving a durable repair while preserving ventricular function and minimizing complications, emphasizing the need for multidisciplinary expertise in managing these challenging conditions.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 29-37"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948030","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}
Vincent Chauvette MD, Elbert E. Williams MD, Lilyanne Chen MD, Ismail El-Hamamsy MD, PhD
{"title":"How-I-Do-It: The Ross Procedure in Adolescents With Bicuspid Aortic Valve, Aortic Regurgitation and a Dilated Annulus","authors":"Vincent Chauvette MD, Elbert E. Williams MD, Lilyanne Chen MD, Ismail El-Hamamsy MD, PhD","doi":"10.1053/j.pcsu.2025.02.001","DOIUrl":"10.1053/j.pcsu.2025.02.001","url":null,"abstract":"<div><div>While the Ross procedure provides optimal clinical outcomes for young patients with aortic valve disease, the presence of preoperative aortic regurgitation (AR) with a dilated aortic annulus have long been recognized as independent predictors of early autograft failure. While this had led many to abandon the Ross procedure in this setting, we sought to specifically address the clinical and anatomic features that are unique to patients with severe AR, namely a dilated aortic annulus, aortic/pulmonary annular mismatch, ascending aortic dilatation and persistent hypertension early after surgery. Using a tailored surgical approach along with strict blood pressure control postoperatively, we believe that many of the concerns with the Ross procedure in this setting can be successfully mitigated. Importantly, these adjuncts do not compromise the dynamism of the autograft root, an important feature for the long-term benefits of the Ross procedure, especially in young and adolescent populations. In this article, we describe in detail our Ross technique in the setting of young patients with bicuspid AR and a dilated aortic annulus.</div></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"28 ","pages":"Pages 79-82"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143948345","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}