Athar M. Qureshi , Christopher A. Caldarone , Travis J. Wilder
{"title":"Transcatheter Approaches to Palliation for Tetralogy of Fallot","authors":"Athar M. Qureshi , Christopher A. Caldarone , Travis J. Wilder","doi":"10.1053/j.pcsu.2022.05.001","DOIUrl":"10.1053/j.pcsu.2022.05.001","url":null,"abstract":"<div><p>To this day, controversy still exists regarding the optimal method to treat symptomatic neonates and infants with Tetralogy of Fallot (TOF). Symptomatic (severely cyanotic or ductal dependent) infants with TOF can undergo either a staged repair approach (consisting of initial palliation followed by complete repair) or primary repair. Traditionally, initial palliative procedures have been surgical, for example placement of a Blalock-Taussig-Thomas (BTT) shunt. Recent advances in technology have facilitated the introduction of catheter-based procedures as palliative techniques, for example, patent ductus arteriosus (PDA) stenting and right ventricular outflow tract (RVOT) stenting as more durable solutions than balloon pulmonary valvuloplasty (BPV). In this article, we discuss the rationale for these procedures, technical aspects of these procedures and outcomes data compared to traditional surgical procedures. Recent data have suggested that RVOT and PDA stenting procedures offer many advantages over traditional surgical palliative procedures as palliative methods in this patient population. This comes at a cost of increased reintervention burden, which may be considered part of the overall treatment strategy in smaller neonates and can be minimized with a focus on technical aspects and overall treatment strategies. Advanced surgical techniques are required at the eventual complete repair to negotiate removal of stent material and pulmonary artery reconstruction in some instances. Further adoption of catheter based palliative procedures for infants with symptomatic TOF has the potential to tip the outcomes towards favoring a staged approach, particularly in high-risk infants.</p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"25 ","pages":"Pages 48-57"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40522542","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":"Comparing Long-Term Sequelae of the Systemic Right Ventricle: An Overview of Single Versus Biventricular Arrangements","authors":"Stephanie Fuller","doi":"10.1053/j.pcsu.2022.05.002","DOIUrl":"10.1053/j.pcsu.2022.05.002","url":null,"abstract":"<div><p><span><span><span>Patients with systemic right ventricles (RV) are at risk for heart failure and </span>sudden cardiac death. Contributing factors to RV dysfunction include increased afterload from the </span>systemic circulation<span>, coronary insufficiency, progressive </span></span>tricuspid valve regurgitation<span>, the presence of residual lesions after palliation<span> and arrhythmias. While all patients with a systemic right ventricle (SRV) are vulnerable to heart failure, there are distinct differences between patients with congenital dextro-transpostion of the great arteries<span> (d-TGA) repaired by atrial switch, unrepaired congenitally corrected transposition of the great arteries (cc-TGA) and single systemic right ventricles palliated with a Fontan operation<span>. Herein, we explore both the similarities and differences in progression of heart failure by phenotype as well as both the advancements and limitations in treatment options by each type of SRV.</span></span></span></span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"25 ","pages":"Pages 2-10"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40505545","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}
Ismail Bouhout , David Kalfa , Amee Shah , Andrew B. Goldstone , Jamie Harrington , Emile Bacha
{"title":"Surgical Management of Complex Aortic Valve Disease in Young Adults: Repair, Replacement, and Future Alternatives","authors":"Ismail Bouhout , David Kalfa , Amee Shah , Andrew B. Goldstone , Jamie Harrington , Emile Bacha","doi":"10.1053/j.pcsu.2022.04.002","DOIUrl":"10.1053/j.pcsu.2022.04.002","url":null,"abstract":"<div><p><span>The ideal aortic valve<span> substitute in young adults remains unknown. Prosthetic valves<span> are associated with a suboptimal survival and carry a significant risk of valve-related complications in young patients, mainly reinterventions with tissue valves and, thromboembolic events and major bleeding with mechanical prostheses. The </span></span></span>Ross procedure<span><span> is the only substitute that restores a survival curve similar to that of a matched general population, and permits a normal life without functional limitations. Though the risk of reintervention is the Achilles’ heel of this procedure, it is very low in patients<span> with aortic stenosis and can be mitigated in patients with </span></span>aortic regurgitation<span> by tailored surgical techniques. Finally, the Ozaki procedure and the transcatheter aortic valve implantation are seen by many as future alternatives but lack evidence and long-term follow-up in this specific patient population.</span></span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"25 ","pages":"Pages 28-37"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40505546","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":"Integrating Novel Physiologic Data into Decision-Making in Congenital Heart Surgery","authors":"Osami Honjo","doi":"10.1053/j.pcsu.2022.02.001","DOIUrl":"10.1053/j.pcsu.2022.02.001","url":null,"abstract":"<div><p>Optimal decision-making to determine the type and timing of surgical intervention for various congenital heart disease (CHD) requires adequate understanding and interpretation of anatomic and physiologic data obtained from various imaging modalities. Cardiac magnetic resonance (CMR) has revolutionized the way we evaluate the anatomy and physiology of CHD. In addition to 2- and 3-dimensional anatomic data and volumetry, phase-contrast CMR allows quantitative measurements of cardiac output, pulmonary blood flow, pulmonary-to-systemic flow ratio, the amount of intracardiac shunt, valve regurgitation, and aortopulmonary collateral flows. This review article describes the utilization of CMR-derived flow data in surgical decision-making in three distinct subgroups: (1) patients with borderline left ventricle (LV) with emphasis on the ascending aortic flow and other physiologic parameters, (2) single ventricle patients who undergo bidirectional cavopulmonary shunt with emphasis on the impact of superior vena cava blood flow on postoperative physiology, and (3) patients with pulmonary atresia and major aortopulmonary collateral arteries with emphasis on the impact of total pulmonary blood flow and systemic-to-pulmonary flow ratio on clinical outcomes.</p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"25 ","pages":"Pages 19-27"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40505543","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":"Management Options for Congenitally Corrected Transposition: Which, When, and for Whom?","authors":"Jacob R Miller, Vinod Sebastian, Pirooz Eghtesady","doi":"10.1053/j.pcsu.2022.04.001","DOIUrl":"10.1053/j.pcsu.2022.04.001","url":null,"abstract":"<div><p>Management strategies for congenitally corrected transposition of the great arteries (ccTGA) historically consisted of a physiologic repair, resulting in the morphologic right ventricle (mRV) supporting systemic circulation. This strategy persisted despite the development of heart failure by middle age because of the reasonable short-term outcomes, and the natural history of some patients with favorable anatomy (felt to demonstrate the mRV's ability to function in the long-term), and due to the less-than-optimal outcomes associated with anatomical repair. As outcomes with anatomical repair improved, and the long-term risk of systemic mRV dysfunction became apparent, more have begun to realize its advantages. In addition to the decision on whether or not to pursue anatomical repair, and the optimal timing, studies demonstrating the nuance to morphologic left ventricle retraining have demonstrated its feasibility. Further considerations in ccTGA have begun to be better understood, including: the management of a poorly functioning mRV, systemic tricuspid valve regurgitation, the utility of morphologic left ventricle outflow tract obstruction (native or surgically created) and pacing strategies. While some considerations are apparent: biventricular pacing is superior to univentricular, tricuspid regurgitation must be managed early with either progression towards anatomical repair (pulmonary artery banding if needed for retraining) or tricuspid replacement (not repair) based on the patient's age; others remain to be completely elucidated. Overall, the heterogeneity of ccTGA, as well as the unique presentation with each patient regarding ventricular and valvular function and center-to-center variability in management strategies has made the interpretation of published data difficult. That said, more recent long-term outcomes favor anatomical repair in most situations.</p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"25 ","pages":"Pages 38-47"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40505547","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}
{"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}
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}
{"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}
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}