Ujjwal Kumar Chowdhury, Robert H Anderson, Niraj Nirmal Pandey, Niwin George, Lakshmi Kumari Sankhyan, Maroof A Khan, Shikha Goja, Sivasubramanian Ramakrishnan, Saurabh Kumar Gupta
{"title":"Long-Term Surgical Outcomes of Patients With Isomeric Right and Left Atrial Appendages.","authors":"Ujjwal Kumar Chowdhury, Robert H Anderson, Niraj Nirmal Pandey, Niwin George, Lakshmi Kumari Sankhyan, Maroof A Khan, Shikha Goja, Sivasubramanian Ramakrishnan, Saurabh Kumar Gupta","doi":"10.1177/21501351221151049","DOIUrl":"https://doi.org/10.1177/21501351221151049","url":null,"abstract":"<p><p><b>Objectives:</b> To compare the long-term outcomes of biventricular, univentricular, and so-called one-and-one-half ventricular repairs in patients with left and right isomerism. <b>Methods:</b> Surgical correction was undertaken, between 2000 and 2021, in 198 patients with right, and 233 with left isomerism. <b>Results:</b> The median age at operation was 24 days (interquartile range [IQR]: 18-45) and 60 days (IQR: 29-360) for those with right and left isomerism, respectively. Multidetector computed-tomographic angiocardiography demonstrated more than half of those with right isomerism had superior caval venous abnormalities, and one-third had a functionally univentricular heart. Almost four-fifths of those with left isomerism had an interrupted inferior caval vein, and one-third had complete atrioventricular septal defect. Biventricular repair was achieved in two-thirds of those with left isomerism, but under one-quarter with right isomerism (<i>P</i> < .001). Hazard regression for mortality revealed odds for prematurity at 5.5, pulmonary atresia at 2.81, atrioventricular septal defect with a common valvar orifice at 2.28, parachute mitral valve at 3.73, interrupted inferior caval vein at 0.53, and functionally univentricular heart with a totally anomalous pulmonary venous connection at 3.77. At a median follow-up of 124 months, the probability of survival was 87% for those with left, and 77% for those with right isomerism (<i>P</i> = .006). <b>Conclusions:</b> Multimodality imaging characterizes and delineates the relevant anatomical details, facilitating surgical management of individuals with isomeric atrial appendages. Continuing higher mortality despite surgical intervention in those with right isomerism points to the need for the reassessment of strategies for management.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":"14 3","pages":"291-299"},"PeriodicalIF":0.9,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9408187","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}
Lisa Wise-Faberowski, Jin Long, Michael Ma, Helen R Nadel, Jennifer Shek, Jeffrey A Feinstein, Elisabeth Martin, Frank L Hanley, Doff B McElhinney
{"title":"Serial Lung Perfusion Scintigraphy After Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals.","authors":"Lisa Wise-Faberowski, Jin Long, Michael Ma, Helen R Nadel, Jennifer Shek, Jeffrey A Feinstein, Elisabeth Martin, Frank L Hanley, Doff B McElhinney","doi":"10.1177/21501351231162959","DOIUrl":"https://doi.org/10.1177/21501351231162959","url":null,"abstract":"<p><strong>Background: </strong>In patients with tetralogy of Fallot and major aortopulmonary collaterals (MAPCAs), pulmonary blood supply is highly variable. Our approach to this condition emphasizes complete unifocalization of the pulmonary circulation, incorporating all lung segments and addressing stenoses out to the segmental level. Post-repair, we recommend serial lung perfusion scintigraphy (LPS) to assess short-term changes in pulmonary blood flow distribution.</p><p><strong>Methods: </strong>We reviewed post-discharge and follow-up LPS performed through three years post-repair and analyzed serial changes in perfusion, risk factors for change, and the relationship between LPS parameters and pulmonary artery reintervention.</p><p><strong>Results: </strong>Of 543 patients who had postoperative LPS results in our system, 317 (58%) had only a predischarge LPS available for review, while 226 had 1 (20%) or more (22%) follow-up scans within three years. Overall, pulmonary flow distribution prior to discharge was balanced, and there was minimal change over time; however, there was considerable patient-to-patient variation in both metrics. On multivariable mixed modeling, time after repair (<i>P</i> = .025), initial anatomy consisting of a ductus arteriosus to one lung (<i>P</i> < .001), and age at repair (<i>P</i> = .014) were associated with changes on serial LPS. Patients who had follow-up LPS were more likely to undergo pulmonary artery reintervention, but within that cohort, LPS parameters were not associated with reintervention risk.</p><p><strong>Conclusion: </strong>Serial LPS during the first year after MAPCAs repair is a noninvasive method of screening for significant post-repair pulmonary artery stenosis that occurs in a small but important minority of patients. In patients who received follow-up LPS beyond the perioperative period, there was minimal change over time in the population overall, but large changes in some patients and considerable variability. There was no statistical association between LPS findings and pulmonary artery reintervention.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":"14 3","pages":"261-272"},"PeriodicalIF":0.9,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9761368","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}
Ilya A Soynov, Yuriy Y Kulyabin, Alexey N Arkhipov, Sergey M Ivanzov, Meline G Galstyan, Alexey V Voitov
{"title":"Surgical Repair of Anomalous Origin of the Left Subclavian Artery From the Pulmonary Artery Using a Supraclavicular Approach.","authors":"Ilya A Soynov, Yuriy Y Kulyabin, Alexey N Arkhipov, Sergey M Ivanzov, Meline G Galstyan, Alexey V Voitov","doi":"10.1177/21501351221149901","DOIUrl":"https://doi.org/10.1177/21501351221149901","url":null,"abstract":"<p><p>Anomalous origin of the left subclavian artery from the pulmonary artery is a rare congenital cardiac malformation. We describe a case of anomalous origin of the left subclavian artery from the pulmonary artery in a patient who presented with symptoms of vertebrobasilar insufficiency and underwent reimplantation of the left subclavian artery into the left common carotid artery from the supraclavicular approach.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":"14 3","pages":"392-394"},"PeriodicalIF":0.9,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9407783","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}
William N Evans, Ruben J Acherman, Alvaro Galindo, Abraham Rothman, Michael L Ciccolo, Juan Lehoux, Humberto Restrepo
{"title":"Hepatic Fibrosis Risk Factors in Extracardiac-Fontan Patients: Observations From a Single Center.","authors":"William N Evans, Ruben J Acherman, Alvaro Galindo, Abraham Rothman, Michael L Ciccolo, Juan Lehoux, Humberto Restrepo","doi":"10.1177/21501351231154216","DOIUrl":"https://doi.org/10.1177/21501351231154216","url":null,"abstract":"<p><p><b>Objective:</b> We reviewed our experience with transvenous liver biopsy-derived hepatic fibrosis scores and possible associated risk factors in those postextracardiac Fontan patients. <b>Methods:</b> We identified extracardiac-Fontan patients with postoperative durations <20 years who underwent cardiac catheterizations with transvenous hepatic biopsies between April 2012 and July 2022. If a patient underwent two liver biopsies, we averaged the two total fibrosis scores and concurrent time, pressure, and oxygen saturation data. We grouped patients by the following factors: (1) <i>sex</i>, (2) venovenous collaterals, and (3) type of functionally univentricular heart. We identified potential hepatic fibrosis risk factors as the following: female, presence of venovenous collaterals, and a functional univentricle of right-ventricular type. For statistical analysis, we used Kruskal-Wallis nonparametric testing. <b>Results:</b> We identified 127 patients who underwent 165 transvenous biopsies, with 38 patients undergoing 2 biopsies. We found that females with two additional risk factors had the highest median total fibrosis scores, 4 (1-8); males with <2 risk factors had the lowest median total fibrosis scores, 2 (0-5); and females with <2 additional risk factors and males with two risk factors were in the middle, median total fibrosis score 3 (0-6), <i>P</i> =.002; and there were no statistical differences for the other demographic or hemodynamic variables. <b>Conclusions:</b> For extracardiac-Fontan patients with similar demographic and hemodynamic variables, identifiable risk factors are associated with the degree of hepatic fibrosis.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":"14 3","pages":"345-349"},"PeriodicalIF":0.9,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9408308","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":"Invited Commentary: The Fate of MAPCAs.","authors":"Frank Cetta","doi":"10.1177/21501351231162913","DOIUrl":"https://doi.org/10.1177/21501351231162913","url":null,"abstract":"Pulmonary valve atresia with ventricular septal defect (PA/VSD) rep-resents the most extreme form of tetralogy of Fallot. In contradistinc-tion to patients with tetralogy of Fallot, there is no continuity between the right ventricle and the pulmonary artery tree. Therefore, pulmonary blood supply in these patients is dependent on extracardiac sources. These sources may be the ductus arteriosus, collateral arteries from the thoracic aorta","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":"14 3","pages":"273-274"},"PeriodicalIF":0.9,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9409750","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}
Martin O Schmiady, Robert Cesnjevar, Yakup Yakupoglu, Ojuola B Adjibodou, Andreas Spirig, Dominique Bettex, Lukas Meier, Thierry Carrel
{"title":"Anomalous Origin of Left Circumflex Coronary Artery From Right Pulmonary Artery: An Unusual Cause of Sudden Cardiac Arrest.","authors":"Martin O Schmiady, Robert Cesnjevar, Yakup Yakupoglu, Ojuola B Adjibodou, Andreas Spirig, Dominique Bettex, Lukas Meier, Thierry Carrel","doi":"10.1177/21501351231151664","DOIUrl":"https://doi.org/10.1177/21501351231151664","url":null,"abstract":"<p><p>Anomalous origin of the left circumflex coronary artery from the right pulmonary artery is an extremely rare coronary anomaly out of the group of anomalous coronary arteries arising from the pulmonary artery. We present the case of a 27-year-old male, in whom the diagnosis of an anomalous left circumflex coronary artery from the pulmonary artery was made after sudden cardiac arrest. The diagnosis was confirmed by multimodal imaging and the patient underwent successful surgical correction. Abnormal origins of a coronary artery may become symptomatic later in life and may occur as an isolated cardiac malformation. Due to a potentially unfavorable clinical course, surgical correction should be considered as soon as a diagnosis is made.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":"14 3","pages":"399-402"},"PeriodicalIF":0.9,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9761315","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 A Grishin, M D Svechkov, S E Shorokhov, A A Avramenko
{"title":"False Aneurysm of the Proximal Sano Shunt in a Patient With Hypoplastic Left Heart Syndrome After Bidirectional Superior Cavopulmonary Connection.","authors":"S A Grishin, M D Svechkov, S E Shorokhov, A A Avramenko","doi":"10.1177/21501351221151056","DOIUrl":"10.1177/21501351221151056","url":null,"abstract":"<p><p>The case describes the surgical treatment of a patient with a false aneurysm of the proximal anastomosis of the Sano shunt in a patient with hypoplastic left heart syndrome midterm after bidirectional cavo-pulmonary anastomosis. The diagnostic algorithm included a physical examination, echocardiography, and multispiral computed tomography. Surgical treatment included closure of the right ventricular defect and removal of blood clots from the cavity of the false aneurysm.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":"14 3","pages":"395-396"},"PeriodicalIF":0.9,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9777603","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":"Successful Management of a Very Unusual Coronary Pattern in Transposition of the Great Arteries.","authors":"Anand K Mishra, Javid Raja, Vidur Bansal, Nitish Kumar, Manoj Kumar Rohit, Anmol Bhatia","doi":"10.1177/21501351231154201","DOIUrl":"https://doi.org/10.1177/21501351231154201","url":null,"abstract":"<p><p>Coronary artery anatomy is the key to a successful arterial switch operation in transposition of the great arteries. We came across an unusual coronary pattern in a child with transposition in which the three major coronary arteries were seen arising from all three aortic sinuses. This coronary pattern is the first of its kind in transposition, and this case report emphasizes the difficulty in translocating such a rare coronary pattern while performing an arterial switch operation.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":"14 3","pages":"380-382"},"PeriodicalIF":0.9,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9407788","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":"Adolescents and Adults With Congenital Heart Disease: Why Are They Lost to Follow-Up?","authors":"Tianwei Liu, Alun C Jackson, Samuel Menahem","doi":"10.1177/21501351221149897","DOIUrl":"https://doi.org/10.1177/21501351221149897","url":null,"abstract":"<p><p>Almost 90% of infants with congenital heart disease (CHD) now reach adulthood but require long-term surveillance to recognize and manage residual and/or evolving lesions. Yet many are lost to follow-up. A scoping review identified four specific domains that pose barriers to consistent follow-up. There are multiple issues associated with transition from pediatric to adult care which included-the lack of a seamless transfer, the establishment of a new trusting relationship, promoting the right balance of patient autonomy and addressing knowledge gaps. Additional issues related to logistic problems of time, distance, cost, and the availability of specialized care, are further compounded by the psychosocial factors and the heterogeneity of the cardiac abnormality affecting our patients. Further study of all these issues is warranted to improve ongoing engagement.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":"14 3","pages":"357-363"},"PeriodicalIF":0.9,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9408650","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}
Pranav Subbaraya Kandachar, Eapen Thomas, Areej Al Maskary, Andrew Campbell
{"title":"Can Isolated Discordant Atrioventricular Connections Survive Without Interatrial or Interventricular Shunt?","authors":"Pranav Subbaraya Kandachar, Eapen Thomas, Areej Al Maskary, Andrew Campbell","doi":"10.1177/21501351221145169","DOIUrl":"https://doi.org/10.1177/21501351221145169","url":null,"abstract":"<p><p>Clinical presentation of isolated discordant atrioventricular connections is akin to transposition of the great arteries. In the absence of a significant intracardiac shunt, profound cyanosis is expected at birth. We report one such 5-month-old infant who had only mild cyanosis. The left-sided tricuspid valve straddled the interventricular septum with a closed interventricular communication, a type of \"Double Outlet Left Atrium with three atrioventricular valves,\" which provided the necessary \"left to right\" shunt while severe regurgitation through the straddling part and a patent ductus arteriosus provided the effective pulmonary blood flow.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":"14 3","pages":"387-389"},"PeriodicalIF":0.9,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9414669","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}