Central Shunt as Palliation in a 30-Year-Old with Unrepaired Tetralogy of Fallot and Pulmonary Atresia: A Case Report

Central Shunt as Palliation in a 30-Year-Old with Unrepaired Tetralogy of Fallot and Pulmonary Atresia: A Case Report | 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 Central Shunt as Palliation in a 30-Year-Old with Unrepaired Tetralogy of Fallot and Pulmonary Atresia: A Case Report Manoj Kumar Daga, Gaur Hari Das, Aniket Sinha, Pravir Kumar Das, and 1 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-8011932/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 Late presentation of complex congenital heart disease is uncommon in adults, particularly in regions with established paediatric cardiac services. We report a case of a 30-year-old male with unrepaired tetralogy of Fallot (TOF) and pulmonary atresia (PA) who presented with severe shortness of breath. Imaging revealed TOF with atresia of the proximal main pulmonary artery and multiple aortopulmonary collateral arteries (MAPCAs). Given the severely hypoplastic pulmonary arteries, unfavourable anatomy, and prohibitive risk for complete repair, a central aortopulmonary shunt was performed as a palliative measure. Postoperatively, oxygen saturation improved from 65% to 90%, and the patient reported significant symptomatic relief. This case highlights the role of central shunt as a viable palliative option in adult patients with late-presenting complex congenital heart disease where complete repair is not feasible. Cardiothoracic Surgery tetralogy of Fallot pulmonary atresia central shunt adult congenital heart disease palliation Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Introduction Tetralogy of Fallot (TOF) with pulmonary atresia (PA) represents one of the most severe forms of Cono-truncal anomalies. Surgical correction is typically performed in childhood, with palliation by systemic-to-pulmonary artery shunts or primary repair depending on pulmonary artery morphology. Survival to adulthood without surgical intervention is exceedingly rare, particularly in cases of pulmonary atresia, where pulmonary blood flow depends on patent ductus arteriosus or major aortopulmonary collateral arteries (MAPCAs). Adults with unrepaired TOF and PA present a unique clinical and surgical challenge. Prolonged cyanosis results in secondary erythrocytosis, coagulopathy, and multiorgan effects, while distorted anatomy and hypertrophied collaterals complicate operative strategies. The primary objective of palliative shunts in this setting is to provide a reliable source of pulmonary blood flow to alleviate hypoxia. We report the case of a 30-year-old man with unrepaired TOF and PA, in whom a central shunt was chosen as a palliative intervention due to anatomical limitations precluding complete repair. Case Presentation A 30-year-old male presented with worsening exertional dyspnea, cyanosis, and reduced exercise tolerance. He reported lifelong bluish discoloration, clubbing and progressive limitation of activity. He had no prior cardiac interventions. Clinical findings On examination, the patient was thin built, with marked central cyanosis and grade IV digital clubbing (Fig. 1). Oxygen saturation on room air was 65%. Haemoglobin was elevated to 25.4 g/dL with haematocrit of 74%. Cardiac auscultation revealed a single second heart sound and continuous murmurs along the back, suggestive of MAPCAs. Investigations Electrocardiogram demonstrated right ventricular hypertrophy. Chest radiograph revealed almost normal pulmonary vascular markings with rib notching suggestive of established collateral circulation (Fig. 2). Two-dimensional echocardiography showed features of TOF with pulmonary atresia with hypoplastic confluent branch pulmonary arteries (RPA – Small 9mm, LPA – Small 9mm, MPA – 11mm) with a large mal-aligned sub-aortic ventricular septal defect and absent PDA. CT pulmonary angiography revealed TOF, Atresia of the proximal main pulmonary artery, thin distal main pulmonary artery and right and left branch pulmonary arteries with confluent branching. Multiple MAPCAs were present. The right ventricle was hypertrophied, and ascending aorta was dilated − 43mm (Fig. 3). Surgical decision-making A multidisciplinary heart team reviewed the case. Complete repair was deemed technically unfeasible given severely hypoplastic pulmonary arteries and extensive MAPCAs. The decision was made to perform a central aortopulmonary shunt (ascending aorta to main pulmonary artery) using a prosthetic graft, aiming for symptomatic relief and improved oxygenation. Operative details The patient underwent median sternotomy. Extensive collateral circulation was encountered, requiring meticulous dissection and haemostasis. A 6 mm polytetrafluoroethylene (PTFE) graft was anastomosed between the ascending aorta and the confluence of the right and left pulmonary arteries (Fig. 4). Cardiopulmonary bypass was not required. Anomalous origin of the Left anterior descending artery from the Right coronary ostium was noted (Fig. 5). Postoperative course The patient was extubated within 8 hours. Postoperative oxygen saturation improved to 90%. Minimal inotropes were needed which were gradually weaned off. Heparin infusion was ongoing till day 3 post surgery. Patient was discharged on day 5 post surgery on aspirin prophylaxis. The patient has shown symptomatic improvement and is presently classified as New York Heart Association (NYHA) functional class II. Discussion Survival into adulthood with unrepaired TOF and pulmonary atresia is rare, with only isolated case reports in the literature ( 2 , 3 ). Most patients succumb to hypoxemia or complications of long-standing cyanosis in childhood or early adulthood. Those who survive have well developed MAPCAs that provide pulmonary blood flow ( 4 ). Adult patients with unrepaired TOF and PA pose formidable challenges: Anatomical complexity – hypoplastic or absent central pulmonary arteries, extensive MAPCAs, and right ventricular hypertrophy. Physiological consequences – longstanding cyanosis results in secondary erythrocytosis, coagulopathy, hyper-viscosity, and multiorgan dysfunction ( 5 ). Surgical risks – fragile collaterals increase bleeding risk; pulmonary arteries may be too small for effective repair. Role of central shunt The central aortopulmonary shunt, first described by Waterston and later modified with prosthetic grafts, remains a valuable palliative option in select patients ( 1 ). In adults, it provides several benefits: Augments pulmonary blood flow when MAPCAs are insufficient ( 6 ). Improves systemic oxygen saturation. Relieves symptoms and enhances quality of life. May serve as a bridge to future staged interventions if pulmonary arteries show growth. In the present case, complete repair was not feasible. This would have required unifocalization—a complex and high-risk operation to detach all the MAPCAs from the aorta and re-attach them to the central PAs—which was precluded by the diminutive size of the central PAs. A central shunt offered a safe, technically straightforward, and effective palliative solution. Learning points Adult survival in TOF + PA is extremely rare and usually associated with MAPCAs. Preoperative imaging is critical to evaluate pulmonary artery anatomy and collateral circulation. Central shunt remains a useful option for palliation in adults where complete repair is not feasible. Such cases underscore disparities in access to early diagnosis and surgery for congenital heart disease. Conclusion This case illustrates that a central aortopulmonary shunt can be a safe and effective palliative strategy in adults with unrepaired TOF and PA when complete repair is not possible. It provides meaningful improvement in oxygenation and functional status, highlighting the importance of individualized surgical planning in late-presenting congenital heart disease. Declarations Disclaimer Permission was granted by the patient for publishing of the given case report. References Sharma S, Kaur B, Baldinger SH Central Aortopulmonary Shunt. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 [cited 2025 Oct 2]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK614160/ Ciltea R, Filipoiu F, Bordejevic DA, Grigorescu B, Tudoran C, Săftoiu A et al (2022) The case of a 44-year-old survivor of unrepaired tetralogy of Fallot, right aortic arch and abdominal aortopulmonary collateral vessels. Med (Kaunas) 58(8):1011 Alkashkari W, Alharbi A, Alzahrani H, Alsufiani H, Alghamdi S (2020) An adult patient with a tetralogy of Fallot case. Cureus 12(8):e9661 Bull K, Somerville J, Ty E, Spiegelhalter D (1995) Presentation and attrition in complex pulmonary atresia. J Am Coll Cardiol 25(2):491–499 Eren NK, Dönmez K, Güler A, Ay Y, Yıldız T, Erdem A (2008) A late presentation of tetralogy of Fallot with pulmonary atresia and coronary-pulmonary artery collateral and a congenital aortic stenosis. Congenit Heart Dis 3(5):360–363 Duncan BW, Mee RB, Mesia CI, Qureshi A, Rosenthal GL, Seshadri SG et al (2003) Staged repair of tetralogy of Fallot with pulmonary atresia. J Thorac Cardiovasc Surg 126(3):694–702 Additional Declarations The authors declare no competing interests. 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. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. 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-8011932","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":538729616,"identity":"cb3b4ca3-920f-489f-832d-4002b8f9bc2a","order_by":0,"name":"Manoj Kumar Daga","email":"","orcid":"","institution":"B.M. Birla Heart Research Center","correspondingAuthor":false,"prefix":"","firstName":"Manoj","middleName":"Kumar","lastName":"Daga","suffix":""},{"id":538729617,"identity":"671c078b-d848-4638-8163-8ba40f9c8423","order_by":1,"name":"Gaur Hari Das","email":"","orcid":"","institution":"B.M. 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2","display":"","copyAsset":false,"role":"figure","size":125937,"visible":true,"origin":"","legend":"\u003cp\u003eAlmost normal pulmonary vascular markings with rib notching suggestive of collateral circulation\u003c/p\u003e","description":"","filename":"2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8011932/v1/c9b6a62627e0152eeb7b530b.jpg"},{"id":95119555,"identity":"3cb2978c-a952-4f73-b7f1-e0d6e92da5dc","added_by":"auto","created_at":"2025-11-04 13:42:27","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":97764,"visible":true,"origin":"","legend":"\u003cp\u003eCT – Pulmonary Angiography showing TOF with PA along with MAPCAs.\u003c/p\u003e","description":"","filename":"3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8011932/v1/7f76fae1e3c75b058e866a33.jpg"},{"id":95225465,"identity":"90337083-015c-408b-b5aa-b24c73b952e3","added_by":"auto","created_at":"2025-11-05 16:25:05","extension":"jpg","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":100870,"visible":true,"origin":"","legend":"\u003cp\u003eCT – Pulmonary Angiography showing TOF with PA along with MAPCAs.\u003c/p\u003e","description":"","filename":"4.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8011932/v1/b12c74087955233812146eb1.jpg"},{"id":95119560,"identity":"624ed552-a284-40f7-a0ed-be133fe525da","added_by":"auto","created_at":"2025-11-04 13:42:27","extension":"jpg","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":100239,"visible":true,"origin":"","legend":"\u003cp\u003eCT – Pulmonary Angiography showing TOF with PA along with MAPCAs.\u003c/p\u003e","description":"","filename":"5.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8011932/v1/375734c9c12f261e4fbd916e.jpg"},{"id":95224423,"identity":"1d238876-2ff1-4bd1-8433-6c1de9f0cb1e","added_by":"auto","created_at":"2025-11-05 16:23:44","extension":"jpg","order_by":6,"title":"Figure 6","display":"","copyAsset":false,"role":"figure","size":74970,"visible":true,"origin":"","legend":"\u003cp\u003eCentral Shunt from Ascending Aorta to Main Pulmonary Artery\u003c/p\u003e","description":"","filename":"6.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8011932/v1/f37ba08aefd9078c693db32b.jpg"},{"id":95119558,"identity":"6357ddf0-2482-4e9b-9c3b-55e4a74a0aeb","added_by":"auto","created_at":"2025-11-04 13:42:27","extension":"jpg","order_by":7,"title":"Figure 7","display":"","copyAsset":false,"role":"figure","size":106036,"visible":true,"origin":"","legend":"\u003cp\u003eAnomalous origin of left anterior descending artery from right coronary ostium.\u003c/p\u003e","description":"","filename":"7.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8011932/v1/6210129972c7d4bc73cff90b.jpg"},{"id":95312129,"identity":"2f33d3b3-3ef8-43d8-b374-f14f5fc3983e","added_by":"auto","created_at":"2025-11-06 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Surgical correction is typically performed in childhood, with palliation by systemic-to-pulmonary artery shunts or primary repair depending on pulmonary artery morphology. Survival to adulthood without surgical intervention is exceedingly rare, particularly in cases of pulmonary atresia, where pulmonary blood flow depends on patent ductus arteriosus or major aortopulmonary collateral arteries (MAPCAs).\u003c/p\u003e\u003cp\u003eAdults with unrepaired TOF and PA present a unique clinical and surgical challenge. Prolonged cyanosis results in secondary erythrocytosis, coagulopathy, and multiorgan effects, while distorted anatomy and hypertrophied collaterals complicate operative strategies. The primary objective of palliative shunts in this setting is to provide a reliable source of pulmonary blood flow to alleviate hypoxia.\u003c/p\u003e\u003cp\u003eWe report the case of a 30-year-old man with unrepaired TOF and PA, in whom a central shunt was chosen as a palliative intervention due to anatomical limitations precluding complete repair.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eA 30-year-old male presented with worsening exertional dyspnea, cyanosis, and reduced exercise tolerance. He reported lifelong bluish discoloration, clubbing and progressive limitation of activity. He had no prior cardiac interventions.\u003c/p\u003e\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e\u003ch2\u003eClinical findings\u003c/h2\u003e\u003cp\u003eOn examination, the patient was thin built, with marked central cyanosis and grade IV digital clubbing (Fig.\u0026nbsp;1). Oxygen saturation on room air was 65%. Haemoglobin was elevated to 25.4 g/dL with haematocrit of 74%. Cardiac auscultation revealed a single second heart sound and continuous murmurs along the back, suggestive of MAPCAs.\u003c/p\u003e\u003c/div\u003e\n\u003ch3\u003eInvestigations\u003c/h3\u003e\n\u003cp\u003eElectrocardiogram demonstrated right ventricular hypertrophy. Chest radiograph revealed almost normal pulmonary vascular markings with rib notching suggestive of established collateral circulation (Fig.\u0026nbsp;2).\u003c/p\u003e\u003cp\u003eTwo-dimensional echocardiography showed features of TOF with pulmonary atresia with hypoplastic confluent branch pulmonary arteries (RPA \u0026ndash; Small 9mm, LPA \u0026ndash; Small 9mm, MPA \u0026ndash; 11mm) with a large mal-aligned sub-aortic ventricular septal defect and absent PDA.\u003c/p\u003e\u003cp\u003eCT pulmonary angiography revealed TOF, Atresia of the proximal main pulmonary artery, thin distal main pulmonary artery and right and left branch pulmonary arteries with confluent branching. Multiple MAPCAs were present. The right ventricle was hypertrophied, and ascending aorta was dilated \u0026minus;\u0026thinsp;43mm (Fig.\u0026nbsp;3).\u003c/p\u003e\n\u003ch3\u003eSurgical decision-making\u003c/h3\u003e\n\u003cp\u003eA multidisciplinary heart team reviewed the case. Complete repair was deemed technically unfeasible given severely hypoplastic pulmonary arteries and extensive MAPCAs. The decision was made to perform a central aortopulmonary shunt (ascending aorta to main pulmonary artery) using a prosthetic graft, aiming for symptomatic relief and improved oxygenation.\u003c/p\u003e\n\u003ch3\u003eOperative details\u003c/h3\u003e\n\u003cp\u003eThe patient underwent median sternotomy. Extensive collateral circulation was encountered, requiring meticulous dissection and haemostasis. A 6 mm polytetrafluoroethylene (PTFE) graft was anastomosed between the ascending aorta and the confluence of the right and left pulmonary arteries (Fig.\u0026nbsp;4). Cardiopulmonary bypass was not required. Anomalous origin of the Left anterior descending artery from the Right coronary ostium was noted (Fig.\u0026nbsp;5).\u003c/p\u003e\n\u003ch3\u003ePostoperative course\u003c/h3\u003e\n\u003cp\u003eThe patient was extubated within 8 hours. Postoperative oxygen saturation improved to 90%. Minimal inotropes were needed which were gradually weaned off. Heparin infusion was ongoing till day 3 post surgery. Patient was discharged on day 5 post surgery on aspirin prophylaxis. The patient has shown symptomatic improvement and is presently classified as New York Heart Association (NYHA) functional class II.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eSurvival into adulthood with unrepaired TOF and pulmonary atresia is rare, with only isolated case reports in the literature (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). Most patients succumb to hypoxemia or complications of long-standing cyanosis in childhood or early adulthood. Those who survive have well developed MAPCAs that provide pulmonary blood flow (\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eAdult patients with unrepaired TOF and PA pose formidable challenges: Anatomical complexity \u0026ndash; hypoplastic or absent central pulmonary arteries, extensive MAPCAs, and right ventricular hypertrophy. Physiological consequences \u0026ndash; longstanding cyanosis results in secondary erythrocytosis, coagulopathy, hyper-viscosity, and multiorgan dysfunction (\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e). Surgical risks \u0026ndash; fragile collaterals increase bleeding risk; pulmonary arteries may be too small for effective repair.\u003c/p\u003e\n\u003ch3\u003eRole of central shunt\u003c/h3\u003e\n\u003cp\u003eThe central aortopulmonary shunt, first described by Waterston and later modified with prosthetic grafts, remains a valuable palliative option in select patients (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). In adults, it provides several benefits:\u003c/p\u003e\u003cp\u003eAugments pulmonary blood flow when MAPCAs are insufficient (\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eImproves systemic oxygen saturation.\u003c/p\u003e\u003cp\u003eRelieves symptoms and enhances quality of life.\u003c/p\u003e\u003cp\u003eMay serve as a bridge to future staged interventions if pulmonary arteries show growth.\u003c/p\u003e\u003cp\u003eIn the present case, complete repair was not feasible. This would have required unifocalization\u0026mdash;a complex and high-risk operation to detach all the MAPCAs from the aorta and re-attach them to the central PAs\u0026mdash;which was precluded by the diminutive size of the central PAs. A central shunt offered a safe, technically straightforward, and effective palliative solution.\u003c/p\u003e\u003cp\u003e\u003cb\u003eLearning points\u003c/b\u003e\u003c/p\u003e\u003cp\u003e\u003col\u003e\u003cspan\u003e\u003cli\u003e\u003cp\u003eAdult survival in TOF\u0026thinsp;+\u0026thinsp;PA is extremely rare and usually associated with MAPCAs.\u003c/p\u003e\u003c/li\u003e\u003c/span\u003e\u003cspan\u003e\u003cli\u003e\u003cp\u003ePreoperative imaging is critical to evaluate pulmonary artery anatomy and collateral circulation.\u003c/p\u003e\u003c/li\u003e\u003c/span\u003e\u003cspan\u003e\u003cli\u003e\u003cp\u003eCentral shunt remains a useful option for palliation in adults where complete repair is not feasible.\u003c/p\u003e\u003c/li\u003e\u003c/span\u003e\u003c/ol\u003e\u003c/p\u003e\u003cp\u003eSuch cases underscore disparities in access to early diagnosis and surgery for congenital heart disease.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eThis case illustrates that a central aortopulmonary shunt can be a safe and effective palliative strategy in adults with unrepaired TOF and PA when complete repair is not possible. It provides meaningful improvement in oxygenation and functional status, highlighting the importance of individualized surgical planning in late-presenting congenital heart disease.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cdiv id=\"Sec11\" class=\"Section2\"\u003e\u003ch2\u003eDisclaimer\u003c/h2\u003e\u003cp\u003ePermission was granted by the patient for publishing of the given case report.\u003c/p\u003e\u003c/div\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eSharma S, Kaur B, Baldinger SH Central Aortopulmonary Shunt. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 [cited 2025 Oct 2]. Available from: \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://www.ncbi.nlm.nih.gov/books/NBK614160/\u003c/span\u003e\u003cspan address=\"https://www.ncbi.nlm.nih.gov/books/NBK614160/\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eCiltea R, Filipoiu F, Bordejevic DA, Grigorescu B, Tudoran C, Săftoiu A et al (2022) The case of a 44-year-old survivor of unrepaired tetralogy of Fallot, right aortic arch and abdominal aortopulmonary collateral vessels. Med (Kaunas) 58(8):1011\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eAlkashkari W, Alharbi A, Alzahrani H, Alsufiani H, Alghamdi S (2020) An adult patient with a tetralogy of Fallot case. Cureus 12(8):e9661\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eBull K, Somerville J, Ty E, Spiegelhalter D (1995) Presentation and attrition in complex pulmonary atresia. J Am Coll Cardiol 25(2):491\u0026ndash;499\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eEren NK, D\u0026ouml;nmez K, G\u0026uuml;ler A, Ay Y, Yıldız T, Erdem A (2008) A late presentation of tetralogy of Fallot with pulmonary atresia and coronary-pulmonary artery collateral and a congenital aortic stenosis. Congenit Heart Dis 3(5):360\u0026ndash;363\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eDuncan BW, Mee RB, Mesia CI, Qureshi A, Rosenthal GL, Seshadri SG et al (2003) Staged repair of tetralogy of Fallot with pulmonary atresia. J Thorac Cardiovasc Surg 126(3):694\u0026ndash;702\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"hideJournal":true,"highlight":"","institution":"B.M. Birla Heart Research Center","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":"tetralogy of Fallot, pulmonary atresia, central shunt, adult congenital heart disease, palliation","lastPublishedDoi":"10.21203/rs.3.rs-8011932/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-8011932/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eLate presentation of complex congenital heart disease is uncommon in adults, particularly in regions with established paediatric cardiac services. We report a case of a 30-year-old male with unrepaired tetralogy of Fallot (TOF) and pulmonary atresia (PA) who presented with severe shortness of breath. Imaging revealed TOF with atresia of the proximal main pulmonary artery and multiple aortopulmonary collateral arteries (MAPCAs). Given the severely hypoplastic pulmonary arteries, unfavourable anatomy, and prohibitive risk for complete repair, a central aortopulmonary shunt was performed as a palliative measure. Postoperatively, oxygen saturation improved from 65% to 90%, and the patient reported significant symptomatic relief. This case highlights the role of central shunt as a viable palliative option in adult patients with late-presenting complex congenital heart disease where complete repair is not feasible.\u003c/p\u003e","manuscriptTitle":"Central Shunt as Palliation in a 30-Year-Old with Unrepaired Tetralogy of Fallot and Pulmonary Atresia: A Case Report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-11-04 13:42:22","doi":"10.21203/rs.3.rs-8011932/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":"cf367afb-3f47-46b2-b554-122f090d05f0","owner":[],"postedDate":"November 4th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[{"id":57295000,"name":"Cardiothoracic Surgery"}],"tags":[],"updatedAt":"2025-11-04T13:42:23+00:00","versionOfRecord":[],"versionCreatedAt":"2025-11-04 13:42:22","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-8011932","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-8011932","identity":"rs-8011932","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}