{"title":"Mechanical Support for The Failing Single Ventricle at Pre-Fontan Stage: Current State of The Field and Future Directions","authors":"Kriti Puri MBBS , Iki Adachi MD","doi":"10.1053/j.pcsu.2021.04.004","DOIUrl":"10.1053/j.pcsu.2021.04.004","url":null,"abstract":"<div><p><span>With the substantial growth of pediatric </span>ventricular assist device<span><span> (VAD) support, there has been an expansion of the target population towards more complex patients, including congenital heart disease<span> (CHD) with single ventricle (SV) physiology. The outcomes of Stage I and Stage II SV-CHD patients on VAD support from the Pedimacs database are poor, with less than 50% survival on VAD by the 3-month mark in both. The primary objective of this article is to describe the current state of VAD support for the failing Stage I and II SV-CHD circulation, to provide insight into potential areas of outcome improvement. We reviewed the published literature in the form of database and registry reports as well as single-center studies to discuss the outcomes of Stage I and Stage II SV-CHD patients on VAD support. Registry-based studies suggest that VAD support for the failing Stage I and Stage II SV-CHD circulations is challenging. However, the more promising outcomes in several single-institutional reports for both Stage I and Stage II SV-VAD indicate that the grim picture from the databases does not reflect the best outcomes that are possible to be achieved, potentially at experienced centers with higher volumes. Areas of future study and potential improvement including timely initiation of VAD support in the cohort of patients expected to not be a candidate for standard SV </span></span>palliations, pump selection and the benefits of continuous-flow devices, and the decision-making for setting up the optimum circulation for VAD support, be it Fontan completion if feasible or takedown to shunt physiology.</span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"24 ","pages":"Pages 10-18"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39085013","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}
Zachary Spigel MD , Ziyad M. Binsalamah MD, MSc, FRCSC , Christopher A. Caldarone MD, FRCSC
{"title":"Corrigendum to “Congenitally Corrected Transposition of the Great Arteries: Anatomic, Physiologic Repair, and Palliation” [Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2019;22:32–42]","authors":"Zachary Spigel MD , Ziyad M. Binsalamah MD, MSc, FRCSC , Christopher A. Caldarone MD, FRCSC","doi":"10.1053/j.pcsu.2021.05.001","DOIUrl":"10.1053/j.pcsu.2021.05.001","url":null,"abstract":"","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"24 ","pages":"Pages 95-96"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1092912621000259/pdfft?md5=230b46299d8388d7ab70d7495b1c9263&pid=1-s2.0-S1092912621000259-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39017501","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}
Vikram Sood MD , Glenn E. Green MD , Andrea Les PhD , Richard G Ohye MD
{"title":"Advanced Therapies for Severe Tracheobronchomalacia: A Review of the Use of 3D-Printed, Patient-Specific, Externally Implanted, Bioresorbable Airway Splints","authors":"Vikram Sood MD , Glenn E. Green MD , Andrea Les PhD , Richard G Ohye MD","doi":"10.1053/j.pcsu.2021.02.001","DOIUrl":"10.1053/j.pcsu.2021.02.001","url":null,"abstract":"<div><p><span>Tracheobronchomalacia is a condition of dynamic collapse of the trachea and mainstem bronchi. The </span>clinical significance<span><span> of tracheobronchomalacia depends on its severity. Mild cases may be medically managed with limited symptomology, while severe cases require advanced therapies, lengthy hospital stays, and carry significant morbidity and mortality. Current therapies for severe tracheobronchomalacia include tracheostomy with prolonged </span>mechanical ventilation<span>, aortopexy<span>, tracheobronchopexy, and intraluminal metallic, silicone, or bioresorbable stents. Three-dimensional (3D)-printed, patient-specific, bioresorbable airway splinting is a novel treatment<span> option that is undergoing investigation in a cohort of critically ill children with severe tracheobronchomalacia. At the time of our last review of our data, 29 splints had been implanted in 15 children with intrathoracic tracheobronchomalacia. The median follow-up was 8.5 months. There were 12 long-term survivors, and all but one lived at home. This article discusses the details of our institution's development and use of 3D-printed, patient-specific, bioresorbable splints for treatment of severe tracheobronchomalacia in the pediatric population.</span></span></span></span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"24 ","pages":"Pages 37-43"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39005268","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":"Left Ventricular Recruitment in Patients With Hypoplastic Left Heart Syndrome","authors":"Peter Chiu MD, Sitaram Emani MD","doi":"10.1053/j.pcsu.2021.03.001","DOIUrl":"10.1053/j.pcsu.2021.03.001","url":null,"abstract":"<div><p><span><span>Hypoplastic left heart complex with “borderline left ventricle” and intact ventricular septum is a clinical conundrum for the congenital heart surgeon. The choice between neonatal biventricular repair and </span>single ventricle </span>palliation<span><span><span> can be challenging, and the current tools to aid in the decision-making process fail to account for the morbidity associated with intermediate and late diastolic dysfunction. Staged ventricular recruitment, consisting of valvular repair techniques, resection of </span>endocardial fibroelastosis, atrial septal restriction, and augmentation of </span>pulmonary blood flow, has been shown to improve left ventricular size and function culminating in eventual biventricular circulation. Despite staged ventricular recruitment, some patients cannot undergo biventricular conversion. Strategies to address these complex patients, including the “reverse” double switch, are the next frontier in biventricular repair.</span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"24 ","pages":"Pages 30-36"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39005267","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}
S. Lucy Roche MB, ChB, MRCPCH , David S Crossland MB, ChB, MRCPCH , Iki Adachi MD , Christopher Broda MD , Katrijn Jansen MD , Edward Hickey FRCSC
{"title":"Mechanical Circulatory Support for the Failing Sub-Aortic Right Ventricle in Adults","authors":"S. Lucy Roche MB, ChB, MRCPCH , David S Crossland MB, ChB, MRCPCH , Iki Adachi MD , Christopher Broda MD , Katrijn Jansen MD , Edward Hickey FRCSC","doi":"10.1053/j.pcsu.2021.04.003","DOIUrl":"10.1053/j.pcsu.2021.04.003","url":null,"abstract":"<div><p><span>Patients with ccTGA<span> or d-TGA managed via atrial switch (Mustard or Senning operations) have biventricular circulations with a sub-aortic right ventricle (2V-RV). Other than in a tiny percentage of ccTGA patients, premature heart failure (HF) is common, driven by chronic RV dilatation and dysfunction and/or </span></span>tricuspid regurgitation<span>. These patients are different from the general HF population in that they are younger, more heterogeneous, are predisposed to pulmonary hypertension and present unique and complex surgical challenges. Despite their young age, they experience disproportionately poor access to advanced therapies and are often disqualified for transplant by pulmonary hypertension, HLA sensitization, program risk-tolerance and psychosocial issues. Mechanical support of the subaortic RV with ventricular assist device<span> (subaortic RVAD, also known as SVAD), although technically challenging, can be an effective alternative to palliative care<span> and offers high likelihood of bridging patients to heart transplant candidacy. In addition, temporary trans-catheter SVAD Impella support has been advantageous for stabilization of decompensated 2V-RV patients or as bridge to durable SVAD support. Improved awareness of and access to specialist ACHD-HF teams offering mechanical support (and transplantation) for 2V-RV patients is increasingly urgent for this aging population, and will improve options and outcomes for these patients as HF emerges.</span></span></span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"24 ","pages":"Pages 2-9"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39005264","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}
Federica Caldaroni MD , Christian P. Brizard MD , Yves d'Udekem MD, PhD
{"title":"Replacement of the Mitral Valve Under One Year of Age: Size Matters","authors":"Federica Caldaroni MD , Christian P. Brizard MD , Yves d'Udekem MD, PhD","doi":"10.1053/j.pcsu.2021.03.006","DOIUrl":"10.1053/j.pcsu.2021.03.006","url":null,"abstract":"<div><p><span>Surgical management of mitral valve disease<span> in neonates and infants is challenging. When repair is no longer feasible, replacement may become inevitable, but should only be considered as an option of last resort due to the remarkably high rate of associated morbidity and mortality. Mechanical valves are the preferred choice in large annuli, while stented conduits seem promising in smaller ones. In patients<span> with a preoperative mitral valve annulus equal or larger than 15-16 mm, an intra-annular placement of the smallest mechanical valve available should be attempted. In patients with smaller annuli, the placement of a stented </span></span></span>valved conduit seems to display a lower mortality risk. Supra-annular implantation of prostheses should be reserved for exceptional cases and to those familiar with this technique because of the high rate of associated complications.</p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"24 ","pages":"Pages 57-61"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39005270","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}
Luke M. Wiggins MD , S. Ram Kumar MD, PhD , Vaughn A. Starnes MD
{"title":"The Ross Procedure in Children: The Gold Standard?","authors":"Luke M. Wiggins MD , S. Ram Kumar MD, PhD , Vaughn A. Starnes MD","doi":"10.1053/j.pcsu.2021.03.002","DOIUrl":"10.1053/j.pcsu.2021.03.002","url":null,"abstract":"<div><p><span><span><span>The management of aortic valve disease in the </span>pediatric population is complex and requires an individualized approach and opportune application of techniques focused on each individual patient's specific </span>anatomy<span>, pathology, and clinical presentation. Though some patients may require variations in the approach to management, the ultimate goal should be to perform a Ross procedure when </span></span>aortic valve replacement is indicated.</p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"24 ","pages":"Pages 62-66"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39005271","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":"Stem Cell Therapy in Single-Ventricle Physiology: Recent Progress and Future Directions","authors":"Mohamed Abdullah MD, PhD , Samantha Kegel BS , Muthukumar Gunasekaran PhD , Progyaparamita Saha PhD , Xuebin Fu PhD , Rachana Mishra PhD , Sudhish Sharma PhD , Sunjay Kaushal MD, PhD","doi":"10.1053/j.pcsu.2021.03.003","DOIUrl":"10.1053/j.pcsu.2021.03.003","url":null,"abstract":"<div><p><span><span><span>Current surgical and medical treatment options for </span>single ventricle<span> physiology conditions remain palliative. On the long term, despite treatment, the systemic ventricle has a significant risk of developing failure. There are unmet needs to develop novel treatment modalities to help ameliorate the ventricular dysfunction. Advances in the field of </span></span>stem cell therapy have been promising for the treatment of heart failure. Numerous stem cell populations have been identified. Preclinical studies in small and large animal models provide evidence for effectiveness of this treatment modality and reveal several mechanisms of action by which stem cells exert their effect. Many </span>clinical trials<span> have been designed to further investigate the therapeutic potential that stem cell therapy may hold for pediatric populations with single ventricle physiology. In this review, we discuss the stem cell types used in these populations, some preclinical studies, and the clinical trials of stem cell therapy in single ventricle patients.</span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"24 ","pages":"Pages 67-76"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39017498","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}
David G. Lehenbauer MD, James S. Tweddell MD, David S. Winlaw MBBS, MD, FRACS
{"title":"Debate - Replacement of the Mitral Valve Under One Year of Age: Mechanical Valves Should Be Used","authors":"David G. Lehenbauer MD, James S. Tweddell MD, David S. Winlaw MBBS, MD, FRACS","doi":"10.1053/j.pcsu.2021.03.005","DOIUrl":"10.1053/j.pcsu.2021.03.005","url":null,"abstract":"<div><p>This article reviews the literature, focusing on publications from the third millennium and the results of mitral valve replacement in children younger than 1 year of age. Special consideration has been given to neonatal and infant valve replacement to provide insights into valve choice and technique. Mitral valve replacement is an important topic because it carries the highest mortality and poorer long-term prognosis than any other valve replacement in children.</p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"24 ","pages":"Pages 44-56"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39005269","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 There Any Clinical Utility to Genetic Testing for Patients With Congenital Heart Disease?","authors":"Richard W. Kim MD","doi":"10.1053/j.pcsu.2021.04.002","DOIUrl":"10.1053/j.pcsu.2021.04.002","url":null,"abstract":"<div><p>Genetic diagnosis is becoming increasingly sophisticated, with the ability to identify even fine differences in patients with a wide variety of congenital heart lesions. Although we have an incomplete understanding of the clinical consequences of most genetic findings, some categories of mutations can have important implications for disease recurrence and prognosis. Consideration of the biology underlying a genetic deficiency, when known, can be useful in the clinical management of some patients.</p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"24 ","pages":"Pages 26-29"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39005265","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}