Pediatric Cardiology最新文献

{"title":"Pathophysiology and Treatment of Intraseptal-Course Left Coronary Anomaly: Surgery for All?","authors":"Paolo Angelini, Carlo Uribe, Antonio F Corno","doi":"10.1007/s00246-023-03328-1","DOIUrl":"10.1007/s00246-023-03328-1","url":null,"abstract":"<p><p>Intraseptal-course, ectopic coronary anomalies are not well characterized as to anatomy, function, prognosis, and treatment. Recently, a revolutionary but unsupported new theory is claiming that most patients with a Left Anomalous Coronary Artery originating from the Opposite Sinus with anomalous Intra-Septal course (L-ACAOS-IS)-even small children-have significant stenoses and require open-heart surgery to prevent acute myocardial infarction and death. This surprising view has spurred ongoing discussions among adult and pediatric cardiologists and cardiac surgeons, compelling us (the conservative party in the discussion) to offer an in-depth and comprehensive review of this anomaly, based on objective but opposite data. We and other adult cardiologists have followed numerous L-ACAOS-IS patients for many years and have observed none of the claimed catastrophes. Rather, we have consistently found that L-ACAOS-IS generally has a benign clinical prognosis. We present the general principle of coronary artery dysfunction in anatomical congenital anomalies (that only significant luminal coronary stenosis can have clinical repercussions). We then review anatomical and functional details of L-ACAOS-IS related to prognosis and treatment indications, which could explain many of the clinical presentations recently mentioned. Finally, we encourage our more liberal colleagues to recognize that, compared with normal coronary arteries, those with anomalies of origin and course are associated with frequent coronary spasm. In particular, we underscore that some of the ischemic manifestations and other results might actually be caused by pressure wire-induced artifacts (rigid wires tend to cause coronary spasm when advanced into tortuous coronary arteries).</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"4-13"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11753303/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71522322","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correction: Isometric Exercise Does Not Affect the Diameter of the Ascending Aorta in Youth with Bicuspid Aortic Valves.","authors":"Jacob Hartz, Laura Mansfield, Sarah de Ferranti, David W Brown, Jonathan Rhodes","doi":"10.1007/s00246-024-03665-9","DOIUrl":"10.1007/s00246-024-03665-9","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"252"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142472000","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Valved Conduits for Right Ventricular Outflow Tract Reconstruction: A Review of Current Technologies and Future Directions.","authors":"Sameer K Singh, Erfan Faridmoayer, Nicolo Vitale, Evan Woodard, Yingfei Xue, Alexey Abramov, Robert J Levy, Giovanni Ferrari","doi":"10.1007/s00246-023-03346-z","DOIUrl":"10.1007/s00246-023-03346-z","url":null,"abstract":"<p><p>The need for right ventricular outflow tract reconstruction is common and growing in congenital heart surgery given expanding indications for the repair of congenital as well as acquired heart disease. Various valved conduit options currently exist including homografts, xenograft pulmonary valved conduits (Contegra™), and porcine valved conduits. The major limitation for all conduits is implant durability, which requires reoperation. Currently, cryopreserved homografts are often used given their superiority shown in long-term data. Significant limitations remain in the cost and availability of the graft, particularly for smaller sizes. Contegra conduits are available in a variety of sizes. Nonetheless, the data regarding long-term durability are less robust and studies comparing durability with homografts have been conflicting. Additionally, there is concern for increased rates of late endocarditis in this conduit. Porcine valved conduits offer a reliable option but are limited by structural valve degeneration associated with all types of bioprosthetic heart valve replacements. New developments in the field of tissue engineering have produced promising bio-restorative valved conduits that may overcome many of the limitations of previous conduit technologies. These remain in the early stages of clinical testing. This review summarizes the clinical data surrounding the conduits used most commonly in clinical practice today and explores emerging technologies that may bring us closer to developing the ideal conduit.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"14-26"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11285445/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138470650","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Aspirin in Children; are There Not Two Sides of the Same Coin?","authors":"Dietmar Schranz","doi":"10.1007/s00246-024-03676-6","DOIUrl":"10.1007/s00246-024-03676-6","url":null,"abstract":"<p><p>Dose-dependent in vitro effects of aspirin on platelet inhibition and predictors of non-responsiveness have led to the recommendation of significantly higher doses of aspirin (5 mg/kg/day) in newborns and infants. The results are inconsistent with the pharmacodynamic effects of clopidogrel in newborns, where approximately 30% (0.2 mg/kg/day) of the adult dose (75 mg/day) showed equally effective antiaggregative effects. Consequently, the optimal aspirin dosage remains to be determined. The administration to newborns with congenital heart defects needs to address treatment goals, while accounting for the intricate interactions between platelets and endothelium, as well as the unique aspects of surgical and interventional procedures.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"235-236"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11746964/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142392284","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Dichorionic Diamniotic Twin Pairs with Complex Congenital Heart Disease.","authors":"Utkarsh Kohli, Melissa L Perrotta, Saif Aljemmali, Cyndi Sosnowski, Yvonne M Cardenas, Kavita Sharma","doi":"10.1007/s00246-023-03339-y","DOIUrl":"10.1007/s00246-023-03339-y","url":null,"abstract":"<p><p>Complex congenital heart disease (CHD) in each of dichorionic diamniotic (DiDi) twin pairs is extremely rare and has not been well characterized. Four DiDi twin pairs were included in this multi-institutional case series. The congenital cardiac abnormalities noted included tetralogy of Fallot (ToF) with pulmonary atresia and collaterals (n = 1), ToF with absent pulmonary valve (n = 1), ToF (n = 2), discontinuous right pulmonary artery (RPA) (n = 1), tricuspid atresia (TA) with normally related great arteries and pulmonary valve stenosis or atresia (n = 2) and coarctation of aorta (CoA) with bicuspid aortic valve (BAV) and borderline left-sided structures (n = 1). Genetic testing was obtained on seven of the eight twins but did not reveal any causal abnormality. A comprehensive review of literature yielded another 8 DiDi twin pairs with complex CHD. The CHD noted in these twin pairs included ToF (n = 2), CoA (n = 4), corrected transposition of great arteries (ccTGA) (n = 2), truncus arteriosus (n = 2), complete common atrioventricular canal (CCAVC) (n = 2), hypoplastic left heart syndrome (HLHS) (n = 2), Shone's complex (n = 1), and hypoplastic right heart syndrome (HRHS) (n = 1). Limited genetic testing was obtained on 4 of these twins and revealed trisomy 21 in a twin pair. Conotruncal abnormalities (42%), CoA (21%), and abnormalities of the right ventricle, the right ventricular outflow tract and pulmonary arteries (17%) are more prevalent in DiDi twins with complex CHD. Clustering of these abnormalities suggests a possible genetic basis; however, genetic testing was obtained on eleven of the twins, and except for trisomy 21 in a twin pair both of whom had CCAVC, did not reveal any causal abnormality. A major direct genetic contribution is therefore unlikely and like other CHD, the underlying etiopathological basis is likely multifactorial.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"213-221"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"107591959","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"15-Year Analysis of Surgical Approaches and Outcomes for Coarctation in 132 Neonates and Infants.","authors":"Yuriy Stukov, Jeffrey P Jacobs, Omar M Sharaf, Giles J Peek, Andrew D Pitkin, Susana C Cruz Beltrán, Dalia Lopez-Colon, Connie S Nixon, Mark S Bleiweis","doi":"10.1007/s00246-023-03360-1","DOIUrl":"10.1007/s00246-023-03360-1","url":null,"abstract":"<p><p>A variety of surgical approaches exist to treat aortic coarctation in neonates and infants. Our institutional approach is designed to match the surgical approach to the individual anatomy of the patient. The objective of this study is to evaluate operative characteristics and outcomes of all neonates and infants who underwent surgical repair of coarctation of the aorta or hypoplastic aortic arch at University of Florida from 2006 to 2021, inclusive, either in isolation or with concomitant repair of atrial septal defect (ASD) and/or ventricular septal defect (VSD). A retrospective review was performed of 132 patients aged 0-1 year who underwent surgical repair of aortic coarctation or hypoplastic aortic arch between 2006 and 2021, inclusive, either in isolation or with concomitant repair of ASD and/or VSD. Patients were divided into two groups based on the surgical approach: Group 1 = Median Sternotomy and Group 2 = Left Lateral Thoracotomy. Continuous variables are presented as median (minimum-maximum); categorical variables are presented as N (%). The most common operative technique in Group 1 was end-to-side reconstruction with ligation of the aortic isthmus. The most common operative technique in Group 2 was extended end-to-end repair. Operative Mortality was one patient (1/132 = 0.76%). Transcatheter intervention for recurrent coarctation was performed in seven patients (7/132 = 5.3%). Surgical re-intervention for recurrent coarctation was performed in three patients (3/132 = 2.3%). From these data, one can conclude that a strategy of matching the surgical approach to the anatomy of neonates and infants who underwent surgical repair of aortic coarctation or hypoplastic aortic arch, either in isolation or with concomitant repair of ASD and/or VSD, is associated with less than 1% Operative Mortality and less than 3% recurrent coarctation requiring reoperation.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"173-180"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140336482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Commentary on \"Biomarkers and Related Factors for the Diagnosis, Risk of Coronary Artery Lesions, and Resistance to Intravenous Immunoglobulin in Kawasaki Disease: An Umbrella Review of Meta-Analyses\".","authors":"Rachana Mehta, Ashok Kumar Balaraman, Muhammed Shabil, Sanjit Sah","doi":"10.1007/s00246-024-03692-6","DOIUrl":"10.1007/s00246-024-03692-6","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"242-243"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142558453","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correction: Pulmonary Valve Aneurysm Associated with a Patent Ductus Arteriosus.","authors":"W Wyatt Lindsey, Catherine E Tomasulo, Alan E Benheim","doi":"10.1007/s00246-024-03715-2","DOIUrl":"10.1007/s00246-024-03715-2","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"255"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142716836","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Minimum Supervision Levels Required for Pediatric Cardiology Fellowship Graduation by Pediatric Cardiology Fellowship Program Directors.","authors":"Ruchika Karnik, Bradley Robinson, Julie Glickstein, Alan Schwartz, Richard Mink, Ashley E Neal, Lowell H Frank, Colin J McMahon, Michael E Kim, David A Turner, Shubhika Srivastava","doi":"10.1007/s00246-023-03371-y","DOIUrl":"10.1007/s00246-023-03371-y","url":null,"abstract":"<p><p>Entrustable professional activities (EPAs) are \"observable essential tasks expected to be performed by a physician for safe patient care in practice.\" Six Pediatric Cardiology (PC) EPAs and their level of supervision (LOS) scales were developed by medical educators in PC using a modified Delphi process and reviewed by the Subspecialty Pediatrics Investigator Network (SPIN). However, their general use in assessment for PC fellows for graduation requirements has yet to be studied. The objective of this study was to determine the minimum LOS required for PC fellows to graduate and compare it with the minimum LOS expected for safe and effective practice for the six PC EPAs, from the perspective of the PC Fellowship Program Directors(FPD). All Fellowship Program Directors(FPD) of ACGME-accredited PC fellowships were surveyed through SPIN between April 2017 and August 2017. For each of the PC EPAs, the FPDs were asked to indicate the minimum LOS expected for graduation and whether they would allow a fellow to graduate if this level was not achieved and the minimum LOS expected for a practicing pediatric cardiologist to provide safe and effective patient care. The minimum LOS was defined as the LOS for which no more than 20% of FPDs would want a lower level. The survey response rate was 80% (47/59). The majority of the FPDs did not require a minimum LOS of five corresponding to unsupervised practice in any of the six PC EPAs at graduation. For EPAs related to imaging, arrhythmia management, and management of cardiac problems, the minimum LOS for graduation was 3, corresponding to being \"trusted to perform a task with indirect supervision for most simple and a few complex cases.\" For the EPAs related to interventional cardiology, heart failure pulmonary hypertension, and cardiac intensive care, the minimum LOS for graduation was 2, corresponding to being \"trusted to perform a task only with direct supervision and coaching.\" The minimum LOS considered necessary for safe and effective practice for all but one EPA was 3. For the EPA related to the management of cardiac problems, the minimum LOS for safe practice was 4, corresponding to being \"trusted to execute tasks independently except for few complex and critical cases.\" Most PC FPDs reported they would not require fellows to achieve the highest entrustment level for any of the six PC EPAs for graduation. It is crucial that educational programs evolve to address these essential activities during training better and that stakeholders ensure that graduating PC fellows have adequate resources and infrastructure to continue professional development as early career pediatric cardiologists.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"181-188"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138801414","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hemodynamics in Adults with Systemic Right Ventricles: Differences Between Congenitally Corrected and Complete Transposition of the Great Arteries.","authors":"William R Miranda, C Charles Jain, Alexander C Egbe, Yogesh N Reddy, Joseph A Dearani, Donald J Hagler, Heidi M Connolly","doi":"10.1007/s00246-023-03381-w","DOIUrl":"10.1007/s00246-023-03381-w","url":null,"abstract":"<p><p>Despite their anatomical differences, congenitally corrected (ccTGA) and complete transposition of the great arteries (d-TGA) post-atrial switch are frequently studied together and managed similarly from a medical standpoint due to the shared systemic right ventricle (sRV). The aim was to assess differences in their underlying hemodynamics. The study is a retrospective review of 138 adults with ccTGA or d-TGA post-atrial switch undergoing cardiac catheterization at Mayo Clinic, MN between 2000 and 2021. ccTGA was categorized into isolated or complex ccTGA depending on concomitant ventricular septal defect and/or left ventricular outflow obstruction. There were 53 patients with d-TGA (91% post-Mustard procedure), 51 with complex and 34 with isolated ccTGA. Isolated ccTGA patients were older (51.8 ± 13.1 years) than those with d-TGA (37.5 ± 8.3 years) or complex ccTGA (40.8 ± 13.4 years). There were no differences in sRV or left ventricular size and function across groups. The ccTGA group more commonly had ≥ moderate tricuspid regurgitation than those with d-TGA; ≥ moderate mitral and ≥ moderate pulmonary regurgitation were most prevalent in complex ccTGA. There were no differences in sRV end-diastolic pressure (sRVEDP) or PAWP between groups. However, the ratio of PAWP:sRVEDP was higher in those with d-TGA compared to those with ccTGA. Cardiac index was higher in the d-TGA group than both groups of ccTGA patients with the latter showing higher indices of ventricular afterload. In conclusion, despite sharing a sRV, adults with d-TGA and ccTGA have substantial differences in hemodynamics and structural/valvular abnormalities. Further investigation regarding disease-specific responses to heart failure therapy in those with d-TGA and ccTGA is warranted.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"189-197"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139478248","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}