Michael Ma MD , Elisabeth Martin MD , Claudia Algaze MD , Ronnie Thomas Collins MD , Doff McElhinney MD , Richard Mainwaring MD , Frank Hanley MD
{"title":"Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable?","authors":"Michael Ma MD , Elisabeth Martin MD , Claudia Algaze MD , Ronnie Thomas Collins MD , Doff McElhinney MD , Richard Mainwaring MD , Frank Hanley MD","doi":"10.1053/j.pcsu.2022.12.003","DOIUrl":"10.1053/j.pcsu.2022.12.003","url":null,"abstract":"<div><p><span>Williams syndrome, and various </span>elastin<span><span> protein mediated arteriopathies<span>, presents a clinical challenge to pediatric cardiovascular specialists. In the severest phenotypes, multilevel obstruction to the systemic and pulmonic arterial systems result in biventricular dysfunction which can be imminently life-threatening. As a longstanding, quaternary referral center for complex pulmonary arteriopathies and pediatric </span></span>connective tissue disease, Stanford Medicine Children's Health has developed a sizeable experience managing these patients. This manuscript is a summary of our current strategies, with a focus on our surgical techniques, peri-procedural considerations on timing and staging of various interventions, and long-term results.</span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"26 ","pages":"Pages 2-8"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9355294","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}
Igor E. Konstantinov MD, PhD, FRACS , Christian P. Brizard MD, MS , Edward Buratto MD, PhD, FRACS
{"title":"Congenital Aortic Valve Repair When the Options aren’t Good: Truncus Arteriosus and Transposition of the Great Arteries","authors":"Igor E. Konstantinov MD, PhD, FRACS , Christian P. Brizard MD, MS , Edward Buratto MD, PhD, FRACS","doi":"10.1053/j.pcsu.2022.12.004","DOIUrl":"10.1053/j.pcsu.2022.12.004","url":null,"abstract":"<div><p><span>Patients with truncus arteriosus and transposition of great arteries are prone to neo-aortic valve insufficiency. Although presenting at opposite ends of the age spectrum, both conditions tend to be commonly associated with neo </span>aortic root<span> dilatation. In patients with truncus arteriosus there is an additional complexity of quadricuspid valve morphology, which make up the majority of valves requiring repair. A unified approach to all these patients would include reduction and stabilization of the annulus and sinotubular junction, as well as achieving equal and symmetrical coaptation of the valve leaflets. By systematically employing these techniques, valve replacement should be avoidable in most children.</span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"26 ","pages":"Pages 56-62"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9355293","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":"Editor's Introduction","authors":"Glen S. Van Arsdell MD","doi":"10.1053/j.pcsu.2023.01.002","DOIUrl":"10.1053/j.pcsu.2023.01.002","url":null,"abstract":"","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"26 ","pages":"Page 1"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10860135","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 M. Overman MD , Francis X. Moga MD , Elizabeth H. Stephens MD, PhD , Joseph A. Dearani MD , Robroy H. MacIver MD, MPH
{"title":"Infant Mitral Valve Replacement: Current State of the Art","authors":"David M. Overman MD , Francis X. Moga MD , Elizabeth H. Stephens MD, PhD , Joseph A. Dearani MD , Robroy H. MacIver MD, MPH","doi":"10.1053/j.pcsu.2023.01.001","DOIUrl":"10.1053/j.pcsu.2023.01.001","url":null,"abstract":"<div><p><span>Mitral valve replacement (MVR) in the very young is an imposing clinical challenge. Early and late mortality risk is substantial, severe adverse events are common, and redo mitral valve replacement is inevitable. Therapeutic options are limited. In the older infant with an annulus of 17mm or larger, mechanical MVR is associated with low risk of mortality and predictable durability. For the very young with annular </span>hypoplasia<span>, bovine jugular vein conduit MVR appears to offer equivalent or better early outcomes with the possibility of subsequent valve expansion, potentially prolonging the interval to redo MVR. Experience with cylinder MVR and other forms of surgeon-manufactured MVR is quite limited, and there is currently no information on late outcomes or durability.</span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"26 ","pages":"Pages 75-80"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9339881","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":"Hybrid First-stage Palliation and Other Strategies to Achieve Biventricular Repair in High-Risk Neonates With Complex Heart Anomalies and Aortic Arch Obstruction","authors":"Bahaaldin Alsoufi MD","doi":"10.1053/j.pcsu.2022.12.009","DOIUrl":"10.1053/j.pcsu.2022.12.009","url":null,"abstract":"<div><p>Medical and surgical advances have allowed single-stage total repair in neonates born with complex congenital heart anomalies<span><span> and aortic arch obstruction. Nonetheless, total repair might be too complex or high risk in certain neonates with demographic, clinical or morphologic risk factors. Alternative management strategies might offer these neonates better outcomes with superior anatomic repair, shorter hospitalization, reduced morbidity, and improved survival. Alternative initial surgical strategies might include </span>aortic arch repair<span> and pulmonary artery band with or without cardiopulmonary bypass<span><span>, extracardiac repair only and pulmonary artery band, Norwood operation, and hybrid first-stage </span>palliation; all deferring complex biventricular intra-cardiac repair to later stage. The strategy choice should be personalized to each patient, taking into consideration the morphologic and clinical state, and the existent goals of care.</span></span></span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"26 ","pages":"Pages 40-49"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9339883","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":"Strategies for Complex Reoperative Aortic Arch Reconstruction in Patients With Congenital Heart Disease","authors":"William C. Frankel MD , Eric E. Roselli MD","doi":"10.1053/j.pcsu.2022.12.006","DOIUrl":"10.1053/j.pcsu.2022.12.006","url":null,"abstract":"<div><p><span><span>Aortic dilation is common in patients<span><span> with congenital heart disease including those with a </span>bicuspid aortic valve, </span></span>connective tissue disease<span><span>, coarctation of the aorta<span>, and conotruncal defects. In addition, neo-aortic dilation has been described in patients after aortic reconstruction including the </span></span>Norwood procedure, the </span></span>arterial switch operation<span><span>, and the Ross procedure<span><span>. Although aortic catastrophe is rare in patients with congenital heart disease, common pathologic endpoints in these patients likely manifest with similar aortic tissue behavior. A lifelong care model with similar indications for surveillance and prophylactic repair to other more common </span>aortopathies is therefore warranted. Still, reoperative </span></span>aortic arch reconstruction<span> in these patients is often a complex and high-risk endeavor, and in all cases, a tailored and adaptable plan ensuring adequate myocardial and cerebral protection with appropriate rescue measures is paramount. A surgical team taking on these challenging cases should possess an armamentarium of open, hybrid, and endovascular techniques which can be individualized to a patient's unique anatomy<span>, surgical history, and concomitant lesions as well as the team's measured outcomes and experience.</span></span></span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"26 ","pages":"Pages 81-88"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9339882","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 to Start a Lymphatic Program","authors":"Mohamed Ali H. Ghandour MD, Sanjay P. Sinha MD","doi":"10.1053/j.pcsu.2022.12.008","DOIUrl":"10.1053/j.pcsu.2022.12.008","url":null,"abstract":"<div><p>Many patients are born with lymphatic abnormalities or are susceptible to pathology following cardiac surgery. It therefore becomes important to evaluate and treat lymphatic disorders in centers performing congenital heart surgery. Programs can make strides towards starting a lymphatic branch if appropriately equipped with proper staff, tools, and other capabilities to perform lymphatic system access, imaging, and intervention. In reality, many of these components already exist in most centers, and a successful and comprehensive program can be established by enlisting these already established services.</p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"26 ","pages":"Pages 18-25"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9355292","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 El-Hamamsy MD, PhD, Charles Laurin MD, Elbert E. Williams MD
{"title":"The Ross Procedure in Adolescence and Beyond: Are There Still Contraindications?","authors":"Ismail El-Hamamsy MD, PhD, Charles Laurin MD, Elbert E. Williams MD","doi":"10.1053/j.pcsu.2022.12.010","DOIUrl":"10.1053/j.pcsu.2022.12.010","url":null,"abstract":"<div><p><span>The Ross procedure<span> is an excellent operation to treat children and adults with aortic valve disease. Compared to prosthetic </span></span>aortic valve replacement<span><span>, it provides important clinical benefits in terms of survival, hemodynamics, freedom from valve-related complications, and durability, especially in women of childbearing age. However, the Ross procedure is a longer and technically more challenging operation. As a result, the choice of procedure should be driven by patient </span>anatomy<span> and clinical characteristics. This highlights the importance of concentrating care in Ross reference centers where surgical expertise and experience are present to ensure patient safety and long-term effectiveness of the operation. This manuscript reviews the major and relative contraindications to the Ross procedure.</span></span></p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"26 ","pages":"Pages 50-55"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9340318","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":"Editor's Introduction to the 2022 issue of Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual","authors":"Christopher A. Caldarone","doi":"10.1053/j.pcsu.2022.05.003","DOIUrl":"10.1053/j.pcsu.2022.05.003","url":null,"abstract":"","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"25 ","pages":"Page 1"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40505542","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":"Considerations for Biventricular Conversion of Fontan Circulation","authors":"S. Ram Kumar , Jon Detterich","doi":"10.1053/j.pcsu.2022.04.003","DOIUrl":"10.1053/j.pcsu.2022.04.003","url":null,"abstract":"<div><p><span>Despite significant improvements in the management of Fontan circulation in patients<span> with single ventricle physiology, long-term outcomes continue to be suboptimal. Conversion to biventricular circulation is increasingly gaining popularity, particularly in the subset of patients who are not ideal Fontan candidates. Meticulous image-guided planning, extensive preoperative discussions, and a team-based approach are required for successful execution of complex biventricular conversion. A segmental approach to the </span></span>anatomy of the heart defect allows methodical planning of the technique of biventricular conversion. Ventricular size and function continue to be the Achilles heel of successful biventricular repair. Long-term studies comparing outcomes in patients converted to biventricular circulation to those in patients with Fontan physiology are required to appropriately tailor management approaches to an individual patient.</p></div>","PeriodicalId":38774,"journal":{"name":"Pediatric Cardiac Surgery Annual","volume":"25 ","pages":"Pages 11-18"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40505544","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}