Dextro-transposition of the great arteries with an intact ventricular septum in older children

Dextro-transposition of the great arteries with an intact ventricular septum in older children | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Dextro-transposition of the great arteries with an intact ventricular septum in older children Fanyan Luo, Haisong Bu This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-3710169/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Complete transposition of the great arteries (TGA) is a common life-threatening complex cyanotic congenital heart disease in infants, resulting in the operation is usually performed about one week after birth. However, little is known about the surgical strategy and experience of TGA with an intact ventricular septum in older patients. Herein, we present an abandoned 7-year-old boy with severe cyanosis with clubbed fingers and toes and then diagnosed as dextro-TGA with an intact ventricular septum, atrial septal defect, patent ductus arteriosus (PDA), and pulmonary hypertension. The patient was performed a two-staged procedure, the aortopulmonary shunt and pulmonary artery banding were performed at the first stage, and then the Switch operation, defect repair, and PDA ligation were successfully performed. The patient was discharged on the 15th day after the operation, and the arterial oxygen saturation returned to normal level (99%). The illustrative report highlights the essence of improving the raising awareness and accurate treatment strategy of dextro-TGA, especially for the remote rural areas of eastern countries where the level of health care and services is relatively backward. transposition of the great arteries intact ventricular septum older children surgery health care and services Figures Figure 1 Figure 2 Figure 3 Figure 4 Case report Complete transposition of the great arteries (TGA), a common life-threatening complex cyanotic congenital heart disease in infants, is a kind of arteries malformation with the aorta arising anterior to the pulmonary artery and from the right ventricle, while the pulmonary artery lies posterior to the aorta and arises from the left ventricle( 1 , 2 ). TGA can be divided into dextro-TGA (D-TGA) and levo-TGA (L-TGA) (Fig. 1 ) ( 1 , 3 ). The most common type of TGA is called D-TGA, which is characterized by the right ventricle on the right side of the left ventricle and the aorta on the front and right side of the pulmonary artery. The lesser-known form of L-TGA, also known as corrected TGA, is characterized by the left ventricle located on the right side of the right ventricle. The main pulmonary artery and aorta are anatomically correct. However, due to ventricular inversion, the aorta merges with the right ventricle, and the main pulmonary artery merges with the left ventricle ( 1 ). Cyanosis usually occurs within 30 days after birth, so patent ductus arteriosus and ventricular septal defect are needed to mix oxygen-rich and oxygen-deficient blood( 4 ). The initial treatment of TGA patients is to ensure adequate oxygenation( 5 ). Once the hemodynamics of the patients are stable, corrective surgery can be performed( 6 ). The surgical repair of TGA is usually carried out about one week after birth, which can be divided into two types: one-stage radical operation and staged radical operation according to the number of operations, such as Arterial switch operation (ASO), Rastelli procedure, the Mustard and Senning procedure, Nakaidoh procedure and Réparation à l'Etage ventriculaire (REV)( 7 – 10 ). Compared with other congenital heart diseases, TGA has the characteristics of critical condition, complex operation process, long treatment cycle, and high operation cost, which has a great impact on the family and society, so it has great clinical significance. Presentation An abandoned 7-year-old boy from a remote village in southern China, who experienced severe cyanosis and tachypnea, was referred to our institution for cardiac defect repair. Physical examination revealed a thin body, poor nutritional development, hexadactyly on the right hand, a decreased arterial oxygen saturation level (50% without oxygen at room temperature), and severe cyanosis with clubbed fingers and toes. The electrocardiogram showed no obvious abnormality. Chest radiography showed enlarged heart shadow, apical protuberance, increased pulmonary vascularity, and an “egg on a string” appearance (Fig. 2 A). Echocardiography revealed D-TGA, atrial septal defect (ASD), patent ductus arteriosus (PDA), pulmonary hypertension (Fig. 2 B), and the ventricular septum protruded to the left. Cardiac computed tomography was performed and confirmed D-TGA, the right ventricle on the right side of the left ventricle and the aorta on the front and right side of the pulmonary artery (Fig. 3 A), PDA (Fig. 3 C), aorta connected right ventricle, pulmonary artery connected left ventricle, both the anatomical relationship of large arteries was reversed (Fig. 3 B-F), and normal origins of the coronary arteries (Fig. 3 B and D). Diagnostic cardiac catheterization further confirmed pulmonary hypertension and left ventricular hypotension (Data not shown). There has been no relevant report at home and abroad, which has brought us great trouble in the reference of experience and the choice of surgical scheme. Under these circumstances that the patient presents low left ventricular pressure is low, a staged repair may be performed for ventricular training to induce left ventricular hypertrophy for adequate postoperative systemic circulatory support. Following communication with the patient's family, the aortopulmonary shunt + pulmonary artery banding + PDA ligation then were performed to exercise the function of the left ventricle and ensure adequate oxygenation. Repeat postoperative evaluation was then performed before the arterial switch to measure and assess left ventricular wall thickness, ventricular volumes, ejection fraction, and left ventricular muscle mass, etc. After more than two months of left ventricular training, the patient revealed normal left ventricular wall thickness and muscle mass for age. Then the Switch operation, defect repair, and PDA ligation were successfully performed. Cardiac computed tomography was revealed the pulmonary artery on the front and right side of the aorta (Fig. 4 A), the pulmonary artery and aorta are anatomically correct (Fig. 4 B-F). The patient was discharged on the 15th day after the operation, and the arterial oxygen saturation returned to normal level (99%). There was no recurrence or other serious complications in the follow-up, but long-term follow-up is necessary. Discussion TGA is a common life-threatening complex cyanotic congenital heart disease in infants and young children, and its incidence rate is about 5%-7% of congenital heart disease( 11 ). The clinical manifestations of D-TGA depend entirely on the degree of blood mixing between systemic circulation and pulmonary circulation. Most patients have symptoms and signs in the neonatal period, such as the following typical clinical manifestations: cyanosis, tachypnea, and murmur( 12 , 13 ). Because of the loss of the physiological principle of circulatory interaction between systemic circulation and pulmonary circulation, it is necessary to mix oxygen-enriched and hypoxia blood to maintain the life of the infant. Mixing can occur through the atrial septal or ventricular septal defect, patent ductus arteriosus, or bronchopulmonary collateral circulation( 4 ). Otherwise, the disease will deteriorate rapidly, resulting in acidosis and progressive aggravation, even death. However, L-TGA patients usually have no obvious signs in the neonatal period, until the right ventricle cannot meet the increased systemic afterload in later years. These patients will have symptoms and signs of heart failure( 13 ). Thus, accurate diagnosis and early treatment are very important. Echocardiography is the main non-invasive diagnostic tool in early screening, which can reveal the abnormal origin of the aorta and pulmonary trunk and any related intracardiac defects( 14 ). At the same time, some other auxiliary examinations are also necessary, such as electrocardiography, chest radiography, computed tomographic angiography (CTA), and even cardiac catheterization. Recently, CTA has become a reference standard for the identification and qualitative analysis of macrovascular abnormalities. It can not only accurately diagnose the origin and course of arteries but also provide a three-dimensional evaluation of the relationship between vessels and adjacent structures, and provide cross-sections of cardiac structures from different angles( 14 , 15 ). Therefore, it can be considered as the preferred imaging method for the noninvasive depiction of macrovascular anatomy, and it is a valuable diagnostic tool in surgical intervention and postoperative follow-up. Cardiac catheterization angiography is an invasive method, but it is rarely used to diagnose TGA. However, it is the gold standard to elucidate the origin of the coronary artery( 14 ), and it also provides the diagnostic basis for collateral circulation, intracardiac defect, and vascular anatomy, and provides a critical important guidance for balloon atrial septostomy (BAS)( 16 – 19 ). The initial treatment of patients with D-TGA is centered on ensuring adequate oxygenation. The common management strategy is to use prostaglandin E1 to keep the ductus arteriosus unobstructed and perform BAS( 5 , 6 ). Once the patient's hemodynamics is stable, corrective surgery can be performed. Surgical repair of D-TGA is usually performed around the first week after birth. At present, D-TGA has two common surgical methods: ASO, which is the standard operation for patients without pulmonary artery stenosis ( 20 ). If there are other intracardiac defects, they will also be repaired during this period. Rastelli procedure, which is suitable for patients with D-TGA, large ventricular septal defect, and pulmonary artery stenosis( 21 ). Other corrective procedures include the Mustard and Senning procedure, Nakaidoh procedure and REV, but these are less commonly performed( 7 – 10 ). Although the prognosis of D-TGA patients after surgical correction is good and the survival rate is more than 90%( 1 , 10 ), there may be some complications. These include aortic root dilatation, pulmonary artery stenosis, arrhythmia, obstruction or leakage of the baffle (Rastelli procedure), and aortic regurgitation( 22 ). Recent studies have shown that adolescents who need special education services after experiencing ASO may suffer from attention deficit hyperactivity disorder( 23 ). Therefore, it is very important to give children more care and education, even special education services, and regular postoperative reviews for the improvement of children's quality of life. The management of TGA can be also challenging. Although the correction of D-TGA is mainly performed by pediatric cardiothoracic surgeons, multidisciplinary efforts are needed, such as the coordination of cardiopulmonary bypass physicians, BAS and angiography detection in the interventional radiology department, routine preventive care and neurodevelopmental screening in pediatrics and neurology department, drug regulation and pain management in the pharmacy department, and routine preoperative, intraoperative and postoperative care in anesthesiology department and nursing department Regulation management and so on. Patient and family education are also very important during hospitalization and after discharge. The best results will be achieved when patients' families and medical staff jointly evaluate and monitor patients and deal with problems in a coordinated way. We successfully reported for the first time the efficacy and safety of staged repair of an older TGA with an intact ventricular septum. Considering the large population of China, the number of TGA cases reported in China is far less than it should be. We believe that the following main factors contribute to this phenomenon. First, there is a lack of a stable and advanced medical system, especially in rural areas of China. Because of the lack of an excellent medical system, the opportunity for surgical repair for TGA is limited. Second, financial constraints hinder the referral of patients with TGA to better hospitals. There are still many patients with TGA or complex congenital heart disease who are not diagnosed and treated in time in the early stage. Finally, due to the lack of understanding and publicity of TGA, especially in remote areas of our country, people ignore and misunderstand TGA, and miss the best opportunity for treatment. Taken together, this illustrative report highlights the essence of improving the raising awareness and accurate treatment strategy of the disease, especially for the remote rural areas of eastern countries where the level of health care and services is relatively backward. Declarations Competing interests The authors declare no conflicts of interest. Ethics approval and consent to participate Written informed consent was obtained from the patient to the use of his all the related images and information for scientific purposes. The study was approved by the Ethics Committee of Xiangya Hospital of Central South University, Changsha, China. Consent to publish Written informed consent to participate in the research was obtained from the patient’s parents in the case of participants under the age of 18 to the use of his/her all the related images and information for scientific purposes. A copy of the written consent is available for review by the Editor of this journal. Competing interests The authors declare that they have no competing interests. Funding This work was supported by the Natural Science Foundation of Hunan Province (2022JJ40765) and the Natural Science Foundation of Changsha City, China (kq2202366). The funders had no role in study design, data collection, and analysis, publication decision, or manuscript preparation. Author Contribution HB and FY conceived and designed the study and drafted the manuscript. HB and FY collected the data. FY was involved in data cleaning and verification. HB and FY analyzed the data and critically revised manuscript. All authors were involved in the final draft of the manuscript. Acknowledgements We thank the patient and his parents for participating in this study. Availability of data and materials The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request. 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JAMA 196(11):991–992 Hazekamp MG, Nevvazhay T, Sojak V (2018) Nikaidoh vs Reparation a l'Etage Ventriculaire vs Rastelli. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 21:58–63 Hazekamp MG, Gomez AA, Koolbergen DR, Hraska V, Metras DR, Mattila IP et al (2010) Surgery for transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction: European Congenital Heart Surgeons Association multicentre study. Eur J Cardiothorac Surg 38(6):699–706 Muter A, Evans HM, Gauvreau K, Colan S, Newburger J, Del Nido PJ et al (2020) Technical Performance Score's Association With Arterial Switch Operation Outcomes. Ann Thorac Surg. Tobler D, Williams WG, Jegatheeswaran A, Van Arsdell GS, McCrindle BW, Greutmann M et al (2010) Cardiac outcomes in young adult survivors of the arterial switch operation for transposition of the great arteries. J Am Coll Cardiol 56(1):58–64 Bravo-Valenzuela NJ, Peixoto AB, Araujo Junior E (2020) Prenatal diagnosis of transposition of the great arteries: an updated review. Ultrasonography 39(4):331–339 Oster ME, Aucott SW, Glidewell J, Hackell J, Kochilas L, Martin GR et al (2016) Lessons Learned From Newborn Screening for Critical Congenital Heart Defects. Pediatrics. ;137(5) Van Praagh R, Geva T, Kreutzer J (1989) Ventricular septal defects: how shall we describe, name and classify them? J Am Coll Cardiol 14(5):1298–1299 Bu H, Zhao T (2020) Image Diagnosis: An Anomalous Origin of Left Coronary Artery from the Pulmonary Artery. Arq Bras Cardiol 114(4 Suppl 1):4–7 Vizzuso A, Righi R, Zerbini M, Gamanji S, Cucchi P, Gallo F et al (2018) An unusual presentation of anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome in a 70-year-old man: a case report. J Med Case Rep 12(1):308 Bu H, Zhao T (2019) Pulmonary Sequestration With Abdominal Aorta Feeding Vessel in an Infant. Circ J 83(7):1612 Bu H, Gong X, Zhao T (2020) Image diagnosis: Eisenmenger's syndrome in patients with simple congenital heart disease. BMC Cardiovasc Disord 20(1):194 Bu H, Gong X (2019) Image Diagnosis- Interrupted Aortic Arch in a Child With Differential Cyanosis. Circ J 83(8):1766 Gopalakrishnan A, Krishnamoorthy KM, Sivasubramonian S (2019) Balloon atrial septostomy at the bedside versus the catheterisation laboratory. Cardiol Young 29(3):454 Jatene AD, Fontes VF, Paulista PP, Souza LC, Neger F, Galantier M et al (1976) Anatomic correction of transposition of the great vessels. J Thorac Cardiovasc Surg 72(3):364–370 Rastelli GC, Wallace RB, Ongley PA (1969) Complete repair of transposition of the great arteries with pulmonary stenosis. A review and report of a case corrected by using a new surgical technique. Circulation 39(1):83–95 Schwartz ML, Gauvreau K, del Nido P, Mayer JE, Colan SD (2004) Long-term predictors of aortic root dilation and aortic regurgitation after arterial switch operation. Circulation 110(11 Suppl 1):II128–II132 Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, Gaynor JW et al (2012) Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation 126(9):1143–1172 Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-3710169","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":256831078,"identity":"5457c1fa-3b02-4ccd-a397-8a8aeefde5b3","order_by":0,"name":"Fanyan Luo","email":"","orcid":"","institution":"Central South University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Fanyan","middleName":"","lastName":"Luo","suffix":""},{"id":256831079,"identity":"01159fb9-671f-45a2-b1ce-b298746740d4","order_by":1,"name":"Haisong Bu","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA4UlEQVRIiWNgGAWjYBACAwYeBoYEEIu9ASzA2EC8Fp4DpGgBA4kEIrWYS+Qe/PDgj02efOTjx595GGxkNxxgfvYAnxbLGXnJEoltacWGt9PMpHkY0ow3HGAzN8DrsBs5BhKJDYcTN87OYWPmYTicuOEAD5sEAS3GPxL+ALXMPMMMdNh/orSYSSSwHU6cL8HDAHTYAcJaLHvemFkA/ZK4gSfNTHKOQbLxzMNsZni1mLPnGN/88ccmcX774ccf3lTYyfYdb36GVwvChQfAJBAzE6UeCOQbiFU5CkbBKBgFIw4AAHEuSVyd75ZQAAAAAElFTkSuQmCC","orcid":"","institution":"Central South University","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Haisong","middleName":"","lastName":"Bu","suffix":""}],"badges":[],"createdAt":"2023-12-05 13:44:20","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-3710169/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-3710169/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":47804029,"identity":"5e170d8c-dfa1-407b-97d8-9af2c5e7979e","added_by":"auto","created_at":"2023-12-07 15:51:00","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":4690052,"visible":true,"origin":"","legend":"\u003cp\u003eSchematic diagram shows the spatial relationships between the great arteries in patients with TGA.\u003c/p\u003e\n\u003cp\u003eTGA: transposition of the great arteries; D: dextro; L: levo; AO: aorta; PA: pulmonary artery.\u003c/p\u003e","description":"","filename":"Figure1.png","url":"https://assets-eu.researchsquare.com/files/rs-3710169/v1/6792ed43eb15d063904b7348.png"},{"id":47804030,"identity":"c3072bd7-35ad-4d52-87d9-2237d1a9be33","added_by":"auto","created_at":"2023-12-07 15:51:00","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":2627383,"visible":true,"origin":"","legend":"\u003cp\u003eChest radiography showed enlarged heart shadow, apical protuberance, and an “egg on a string” appearance (A). Echocardiography revealed dextro-TGA (B).\u003c/p\u003e\n\u003cp\u003eLA: left atrium; AV: aortic valve; LV: left ventricle; RV: right ventricle, mPA: main pulmonary artery.\u003c/p\u003e","description":"","filename":"Figure2.png","url":"https://assets-eu.researchsquare.com/files/rs-3710169/v1/869a13011a5956049e4ab3f0.png"},{"id":47804028,"identity":"686367d1-b95b-469c-9e5a-bf5ed87b562f","added_by":"auto","created_at":"2023-12-07 15:51:00","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":9180012,"visible":true,"origin":"","legend":"\u003cp\u003ePreoperative cardiac computed tomography was confirmed dextro-TGA (A), the anatomical relationship of large arteries was reversed (B-F), and PDA (C).\u003c/p\u003e\n\u003cp\u003eTGA: transposition of the great arteries; AO: aorta; PA: pulmonary artery; LV: left ventricle; RV: right ventricle; PDA: patent ductus arteriosus.\u003c/p\u003e","description":"","filename":"Figure3.png","url":"https://assets-eu.researchsquare.com/files/rs-3710169/v1/29930e8db7731962c23657cf.png"},{"id":47804403,"identity":"f5321934-e74c-4ec5-8c8c-62ff2d8411be","added_by":"auto","created_at":"2023-12-07 15:59:00","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":9363982,"visible":true,"origin":"","legend":"\u003cp\u003ePostoperativecardiac computed tomography was revealed the pulmonary artery on the front and right side of the aorta (A), the pulmonary artery and aorta were anatomically correct (B-F).\u003c/p\u003e\n\u003cp\u003eAO: aorta; PA: pulmonary artery; LV: left ventricle; RV: right ventricle.\u003c/p\u003e","description":"","filename":"Figure4.png","url":"https://assets-eu.researchsquare.com/files/rs-3710169/v1/5e8ad92d875b4a6b4420d7c9.png"},{"id":47937380,"identity":"05c01c96-1247-441f-a0d6-df9a61350457","added_by":"auto","created_at":"2023-12-10 17:22:31","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":3083791,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-3710169/v1/37d52047-a708-4145-b7f4-e396dcf05896.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Dextro-transposition of the great arteries with an intact ventricular septum in older children","fulltext":[{"header":"Case report","content":"\u003cp\u003eComplete transposition of the great arteries (TGA), a common life-threatening complex cyanotic congenital heart disease in infants, is a kind of arteries malformation with the aorta arising anterior to the pulmonary artery and from the right ventricle, while the pulmonary artery lies posterior to the aorta and arises from the left ventricle(\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). TGA can be divided into dextro-TGA (D-TGA) and levo-TGA (L-TGA) (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e) (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). The most common type of TGA is called D-TGA, which is characterized by the right ventricle on the right side of the left ventricle and the aorta on the front and right side of the pulmonary artery. The lesser-known form of L-TGA, also known as corrected TGA, is characterized by the left ventricle located on the right side of the right ventricle. The main pulmonary artery and aorta are anatomically correct. However, due to ventricular inversion, the aorta merges with the right ventricle, and the main pulmonary artery merges with the left ventricle (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). Cyanosis usually occurs within 30 days after birth, so patent ductus arteriosus and ventricular septal defect are needed to mix oxygen-rich and oxygen-deficient blood(\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e). The initial treatment of TGA patients is to ensure adequate oxygenation(\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e). Once the hemodynamics of the patients are stable, corrective surgery can be performed(\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e). The surgical repair of TGA is usually carried out about one week after birth, which can be divided into two types: one-stage radical operation and staged radical operation according to the number of operations, such as Arterial switch operation (ASO), Rastelli procedure, the Mustard and Senning procedure, Nakaidoh procedure and R\u0026eacute;paration \u0026agrave; l'Etage ventriculaire (REV)(\u003cspan additionalcitationids=\"CR8 CR9\" citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e). Compared with other congenital heart diseases, TGA has the characteristics of critical condition, complex operation process, long treatment cycle, and high operation cost, which has a great impact on the family and society, so it has great clinical significance.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Presentation","content":"\u003cp\u003eAn abandoned 7-year-old boy from a remote village in southern China, who experienced severe cyanosis and tachypnea, was referred to our institution for cardiac defect repair. Physical examination revealed a thin body, poor nutritional development, hexadactyly on the right hand, a decreased arterial oxygen saturation level (50% without oxygen at room temperature), and severe cyanosis with clubbed fingers and toes. The electrocardiogram showed no obvious abnormality. Chest radiography showed enlarged heart shadow, apical protuberance, increased pulmonary vascularity, and an \u0026ldquo;egg on a string\u0026rdquo; appearance (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003eA). Echocardiography revealed D-TGA, atrial septal defect (ASD), patent ductus arteriosus (PDA), pulmonary hypertension (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003eB), and the ventricular septum protruded to the left. Cardiac computed tomography was performed and confirmed D-TGA, the right ventricle on the right side of the left ventricle and the aorta on the front and right side of the pulmonary artery (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eA), PDA (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eC), aorta connected right ventricle, pulmonary artery connected left ventricle, both the anatomical relationship of large arteries was reversed (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eB-F), and normal origins of the coronary arteries (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eB and D). Diagnostic cardiac catheterization further confirmed pulmonary hypertension and left ventricular hypotension (Data not shown).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eThere has been no relevant report at home and abroad, which has brought us great trouble in the reference of experience and the choice of surgical scheme. Under these circumstances that the patient presents low left ventricular pressure is low, a staged repair may be performed for ventricular training to induce left ventricular hypertrophy for adequate postoperative systemic circulatory support. Following communication with the patient's family, the aortopulmonary shunt\u0026thinsp;+\u0026thinsp;pulmonary artery banding\u0026thinsp;+\u0026thinsp;PDA ligation then were performed to exercise the function of the left ventricle and ensure adequate oxygenation. Repeat postoperative evaluation was then performed before the arterial switch to measure and assess left ventricular wall thickness, ventricular volumes, ejection fraction, and left ventricular muscle mass, etc. After more than two months of left ventricular training, the patient revealed normal left ventricular wall thickness and muscle mass for age. Then the Switch operation, defect repair, and PDA ligation were successfully performed. Cardiac computed tomography was revealed the pulmonary artery on the front and right side of the aorta (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003eA), the pulmonary artery and aorta are anatomically correct (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003eB-F). The patient was discharged on the 15th day after the operation, and the arterial oxygen saturation returned to normal level (99%). There was no recurrence or other serious complications in the follow-up, but long-term follow-up is necessary.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eTGA is a common life-threatening complex cyanotic congenital heart disease in infants and young children, and its incidence rate is about 5%-7% of congenital heart disease(\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e). The clinical manifestations of D-TGA depend entirely on the degree of blood mixing between systemic circulation and pulmonary circulation. Most patients have symptoms and signs in the neonatal period, such as the following typical clinical manifestations: cyanosis, tachypnea, and murmur(\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e, \u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e). Because of the loss of the physiological principle of circulatory interaction between systemic circulation and pulmonary circulation, it is necessary to mix oxygen-enriched and hypoxia blood to maintain the life of the infant. Mixing can occur through the atrial septal or ventricular septal defect, patent ductus arteriosus, or bronchopulmonary collateral circulation(\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e). Otherwise, the disease will deteriorate rapidly, resulting in acidosis and progressive aggravation, even death. However, L-TGA patients usually have no obvious signs in the neonatal period, until the right ventricle cannot meet the increased systemic afterload in later years. These patients will have symptoms and signs of heart failure(\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e). Thus, accurate diagnosis and early treatment are very important.\u003c/p\u003e \u003cp\u003eEchocardiography is the main non-invasive diagnostic tool in early screening, which can reveal the abnormal origin of the aorta and pulmonary trunk and any related intracardiac defects(\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e). At the same time, some other auxiliary examinations are also necessary, such as electrocardiography, chest radiography, computed tomographic angiography (CTA), and even cardiac catheterization. Recently, CTA has become a reference standard for the identification and qualitative analysis of macrovascular abnormalities. It can not only accurately diagnose the origin and course of arteries but also provide a three-dimensional evaluation of the relationship between vessels and adjacent structures, and provide cross-sections of cardiac structures from different angles(\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e, \u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e). Therefore, it can be considered as the preferred imaging method for the noninvasive depiction of macrovascular anatomy, and it is a valuable diagnostic tool in surgical intervention and postoperative follow-up. Cardiac catheterization angiography is an invasive method, but it is rarely used to diagnose TGA. However, it is the gold standard to elucidate the origin of the coronary artery(\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e), and it also provides the diagnostic basis for collateral circulation, intracardiac defect, and vascular anatomy, and provides a critical important guidance for balloon atrial septostomy (BAS)(\u003cspan additionalcitationids=\"CR17 CR18\" citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eThe initial treatment of patients with D-TGA is centered on ensuring adequate oxygenation. The common management strategy is to use prostaglandin E1 to keep the ductus arteriosus unobstructed and perform BAS(\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e). Once the patient's hemodynamics is stable, corrective surgery can be performed. Surgical repair of D-TGA is usually performed around the first week after birth. At present, D-TGA has two common surgical methods: ASO, which is the standard operation for patients without pulmonary artery stenosis (\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e). If there are other intracardiac defects, they will also be repaired during this period. Rastelli procedure, which is suitable for patients with D-TGA, large ventricular septal defect, and pulmonary artery stenosis(\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e). Other corrective procedures include the Mustard and Senning procedure, Nakaidoh procedure and REV, but these are less commonly performed(\u003cspan additionalcitationids=\"CR8 CR9\" citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eAlthough the prognosis of D-TGA patients after surgical correction is good and the survival rate is more than 90%(\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e), there may be some complications. These include aortic root dilatation, pulmonary artery stenosis, arrhythmia, obstruction or leakage of the baffle (Rastelli procedure), and aortic regurgitation(\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e). Recent studies have shown that adolescents who need special education services after experiencing ASO may suffer from attention deficit hyperactivity disorder(\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e). Therefore, it is very important to give children more care and education, even special education services, and regular postoperative reviews for the improvement of children's quality of life.\u003c/p\u003e \u003cp\u003eThe management of TGA can be also challenging. Although the correction of D-TGA is mainly performed by pediatric cardiothoracic surgeons, multidisciplinary efforts are needed, such as the coordination of cardiopulmonary bypass physicians, BAS and angiography detection in the interventional radiology department, routine preventive care and neurodevelopmental screening in pediatrics and neurology department, drug regulation and pain management in the pharmacy department, and routine preoperative, intraoperative and postoperative care in anesthesiology department and nursing department Regulation management and so on. Patient and family education are also very important during hospitalization and after discharge. The best results will be achieved when patients' families and medical staff jointly evaluate and monitor patients and deal with problems in a coordinated way.\u003c/p\u003e \u003cp\u003eWe successfully reported for the first time the efficacy and safety of staged repair of an older TGA with an intact ventricular septum. Considering the large population of China, the number of TGA cases reported in China is far less than it should be. We believe that the following main factors contribute to this phenomenon. First, there is a lack of a stable and advanced medical system, especially in rural areas of China. Because of the lack of an excellent medical system, the opportunity for surgical repair for TGA is limited. Second, financial constraints hinder the referral of patients with TGA to better hospitals. There are still many patients with TGA or complex congenital heart disease who are not diagnosed and treated in time in the early stage. Finally, due to the lack of understanding and publicity of TGA, especially in remote areas of our country, people ignore and misunderstand TGA, and miss the best opportunity for treatment. Taken together, this illustrative report highlights the essence of improving the raising awareness and accurate treatment strategy of the disease, especially for the remote rural areas of eastern countries where the level of health care and services is relatively backward.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare no conflicts of interest.\u003c/p\u003e\n\u003ch2\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/h2\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient to the use of his all the related images and information for scientific purposes. The study was approved by the Ethics Committee of Xiangya Hospital of Central South University, Changsha, China.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent to publish\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent to participate in the research was obtained from the patient\u0026rsquo;s parents in the case of participants under the age of 18 to the use of his/her all the related images and information for scientific purposes. A copy of the written consent is available for review by the Editor of this journal.\u003c/p\u003e\n\u003ch2\u003eCompeting interests\u003c/h2\u003e\n\u003cp\u003eThe authors declare that they have no competing interests.\u003c/p\u003e\n\u003ch2\u003eFunding\u003c/h2\u003e\n\u003cp\u003eThis work was supported by the Natural Science Foundation of Hunan Province (2022JJ40765) and the Natural Science Foundation of Changsha City, China (kq2202366). The funders had no role in study design, data collection, and analysis, publication decision, or manuscript preparation.\u003c/p\u003e\n\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\n\u003cp\u003eHB and FY conceived and designed the study and drafted the manuscript. HB and FY collected the data. FY was involved in data cleaning and verification. HB and FY analyzed the data and critically revised manuscript. All authors were involved in the final draft of the manuscript.\u003c/p\u003e\n\u003ch2\u003eAcknowledgements\u003c/h2\u003e\n\u003cp\u003eWe thank the patient and his parents for participating in this study.\u003c/p\u003e\n\u003ch2\u003eAvailability of data and materials\u003c/h2\u003e\n\u003cp\u003eThe datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eSzymanski MW, Moore SM, Kritzmire SM, Goyal A Transposition Of The Great Arteries. StatPearls. Treasure Island (FL)2020\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eLachaud M, Dionne A, Brassard M, Charron MA, Birca A, Dehaes M et al (2021) Cardiac hemodynamics in fetuses with transposition of the great arteries and intact ventricular septum from diagnosis to end of pregnancy: longitudinal follow-up. 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Circulation 126(9):1143\u0026ndash;1172\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"transposition of the great arteries, intact ventricular septum, older children, surgery, health care and services","lastPublishedDoi":"10.21203/rs.3.rs-3710169/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-3710169/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eComplete transposition of the great arteries (TGA) is a common life-threatening complex cyanotic congenital heart disease in infants, resulting in the operation is usually performed about one week after birth. However, little is known about the surgical strategy and experience of TGA with an intact ventricular septum in older patients. Herein, we present an abandoned 7-year-old boy with severe cyanosis with clubbed fingers and toes and then diagnosed as dextro-TGA with an intact ventricular septum, atrial septal defect, patent ductus arteriosus (PDA), and pulmonary hypertension. The patient was performed a two-staged procedure, the aortopulmonary shunt and pulmonary artery banding were performed at the first stage, and then the Switch operation, defect repair, and PDA ligation were successfully performed. The patient was discharged on the 15th day after the operation, and the arterial oxygen saturation returned to normal level (99%). The illustrative report highlights the essence of improving the raising awareness and accurate treatment strategy of dextro-TGA, especially for the remote rural areas of eastern countries where the level of health care and services is relatively backward.\u003c/p\u003e","manuscriptTitle":"Dextro-transposition of the great arteries with an intact ventricular septum in older children","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2023-12-07 15:50:55","doi":"10.21203/rs.3.rs-3710169/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"12f89ec0-81a7-4413-a454-2687c56625e1","owner":[],"postedDate":"December 7th, 2023","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2023-12-10T17:14:24+00:00","versionOfRecord":[],"versionCreatedAt":"2023-12-07 15:50:55","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-3710169","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-3710169","identity":"rs-3710169","version":["v1"]},"buildId":"WrCJVZZCHTDjtuVLN7oU0","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}