A Pediatric Case of Cardiogenic Shock Bridged With ECMO Due to a Malignant Left Main Coronary Artery Anomaly

A Pediatric Case of Cardiogenic Shock Bridged With ECMO Due to a Malignant Left Main Coronary Artery Anomaly | 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 A Pediatric Case of Cardiogenic Shock Bridged With ECMO Due to a Malignant Left Main Coronary Artery Anomaly EMRE KIYMIK, MURAT ERDAL, NAZAN ULGEN TEKEREK, ALPER KOKER, FIRAT KARDELEN, and 3 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-8967276/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 Background Malignant left main coronary artery (LMCA) anomalies are rare but potentially fatal congenital defects that may cause myocardial ischemia or sudden cardiac death during exertion, even in otherwise healthy children. Early recognition and timely intervention are crucial to prevent catastrophic outcomes. Case Presentation: We report a 10-year-old boy who presented with acute chest pain and dyspnea during exercise, followed by pulmonary edema and refractory cardiogenic shock. Electrocardiography showed persistent ST-segment elevation, and coronary CT angiography revealed a malignant LMCA with an interarterial and intramural course between the aorta and pulmonary artery. Despite intensive medical therapy, the patient required emergent veno-arterial extracorporeal membrane oxygenation (VA-ECMO) support. After stabilization, surgical re-anastomosis of the LMCA to its anatomical position was successfully performed. Outcome: The patient recovered rapidly, with normalization of cardiac enzymes and resolution of electrocardiographic abnormalities. At follow-up, he remained clinically stable after elective coronary stenting. Conclusion This case highlights the importance of maintaining a high index of suspicion for malignant LMCA anomalies in children presenting with exertional chest pain. Prompt hemodynamic support with ECMO and timely surgical correction can be lifesaving in these rare but high-risk conditions. congenital coronary anomaly LMCA pediatric cardiogenic shock ECMO surgical re-anastomosis Figures Figure 1 Figure 2 INTRODUCTION Chest pain during childhood constitutes a significant proportion of presentations to pediatric emergency departments and cardiology outpatient clinics. However, existing literature indicates that the majority of these cases are benign in nature and arise from non-cardiac etiologies. Non-cardiac causes are most commonly psychogenic, musculoskeletal, respiratory, or gastrointestinal in origin [ 1 ]. Although cardiac chest pain is uncommon in children, certain clinical features are considered “red flags” that call for further cardiologic evaluation. Chest pain associated with exertion, a history of syncope, or radiation of pain to the arm should raise concern for an underlying cardiovascular disorder. In such cases, electrocardiography and echocardiography are the first-line investigations, and advanced imaging may be required when initial findings are inconclusive. Even though rare, congenital coronary artery anomalies — where the coronary arteries arise from an abnormal origin or follow an atypical course — should always be kept in mind when evaluating children with these warning symptoms [ 1 , 2 ]. This report describes a pediatric patient with a malignant course of the left main coronary artery (LMCA) anomaly who developed pulmonary edema and refractory cardiogenic shock following exertion, despite intensive medical management. CASE PRESENTATION A previously healthy 10-year-old boy presented to an outside facility with acute chest pain and shortness of breath that developed while he was exercising. During observation at the local hospital, the patient developed pulmonary edema and required endotracheal intubation. Conventional coronary angiography revealed a left main coronary artery (LMCA) with an intramural course and approximately 95% stenosis in the proximal segment (Fig. 1 ). Following these findings, he was transferred to a tertiary intensive care unit for advanced management. At presentation, the electrocardiogram (ECG) showed persistent ST-segment elevation. Transthoracic echocardiography (ECHO) revealed a left ventricular ejection fraction (LVEF) of 50–52%. Coronary computed tomography (CT) angiography demonstrated that the left main coronary artery (LMCA) originated abnormally high from the aortic root and followed an interarterial course between the aorta and pulmonary artery, consistent with a malignant anatomical pattern (Fig. 2 ). Laboratory evaluation revealed a lactate level of 6 mmol/L, troponin I > 10,000 ng/L (reference range: 10–14 ng/L), and pro-BNP of 6,443 ng/L (reference range: 0–317 ng/L). These clinical and biochemical findings were consistent with cardiogenic shock. Intensive medical therapy was initiated, including inotropic support with adrenaline (maximum dose 0.6 µg/kg/min) and noradrenaline (maximum dose 0.5 µg/kg/min), in addition to milrinone (maximum dose 0.375 µg/kg/min). Continuous diuretic infusion was also started to reduce volume overload. However, due to the lack of adequate hemodynamic response, emergent central veno-arterial extracorporeal membrane oxygenation (VA-ECMO) support was initiated. Following the initiation of ECMO, hemodynamic stability was achieved, accompanied by a marked decline in lactate levels and cardiac biomarkers. Serial echocardiograms showed that the ejection fraction ranged between 44% and 52%. On the sixth day of hospitalization, the patient underwent surgery performed by the cardiovascular team. The intramural segment of the left main coronary artery (LMCA) was surgically dissected and released, then re-anastomosed to its appropriate anatomical position. After aortic repair and closure, the patient was successfully weaned from cardiopulmonary bypass (CPB), and ECMO support was discontinued. In the postoperative period, the patient was extubated the following day. Control electrocardiography showed resolution of ST-segment elevations with restoration of sinus rhythm. On the eighth day of hospitalization, chest drains and central lines were removed, oral feeding was initiated, and mobilization was achieved. The patient remained clinically stable and was discharged on day 13. During follow-up, a residual narrowing of the LMCA was detected, and elective open surgical coronary stenting was performed at another center. The procedure was successful, and the patient demonstrated good clinical recovery. DISCUSSION Normally, the coronary arteries arise from their respective sinuses of Valsalva — the left coronary artery (LCA) from the left coronary sinus and the right coronary artery (RCA) from the right coronary sinus — supplying oxygenated blood to the myocardium. Developmental deviations during embryogenesis may result in congenital coronary artery anomalies (CCAAs), which can occur either in isolation or in association with other cardiac defects. The clinical spectrum of these anomalies ranges widely, from completely asymptomatic and benign variants to malignant anatomical patterns that predispose to myocardial ischemia and sudden cardiac death during exertion [ 3 ]. Coronary arteries that follow an intramural or interarterial course are generally classified as malignant variants. This anatomical configuration can significantly restrict coronary blood flow during exertion, leading to myocardial ischemia, and in some cases, sudden cardiac death may occur as the first and only symptom. This underscores both the clinical importance of malignant variants and the diagnostic challenges they present [ 2 ]. In our case, the pediatric patient presented with pulmonary edema and cardiogenic shock that developed after chest pain and dyspnea during exercise. Persistent ST-segment elevation, markedly elevated troponin levels, and coronary CT angiography findings confirmed a malignant left main coronary artery (LMCA) anomaly. This clinical picture was consistent with acute myocardial ischemia secondary to impaired coronary perfusion caused by the interarterial and intramural course of the anomalous LMCA. Given the refractory cardiogenic shock despite maximal medical therapy, veno-arterial extracorporeal membrane oxygenation (VA-ECMO) was initiated as an emergency intervention, providing temporary circulatory support and preserving organ perfusion. The successful use of ECMO in similar cases of hemodynamic instability secondary to coronary artery anomalies in children has also been reported in the literature [ 4 ]. In a young adult patient described in the literature, coronary CT angiography performed after exertional angina revealed that the left coronary artery originated anomalously from the right coronary sinus (L-ACAOS) and followed an interarterial course between the pulmonary trunk and ascending aorta. An intramural LMCA was identified, and surgical “unroofing” of the anomalous segment was successfully performed. Similarly, in our case, the diagnosis was established by coronary CT angiography; however, VA-ECMO support was required due to the development of cardiogenic shock. In contrast to the reported case, anatomical re-anastomosis of the LMCA to its normal position was performed in our patient. The rapid clinical improvement and normalization of cardiac biomarkers after surgery highlight the favorable physiological impact of the successful anatomical correction [ 5 ]. This case strongly illustrates that malignant LMCA anomalies, though rare, are an important cause of sudden cardiac events in children, and that early diagnosis, prompt hemodynamic support with ECMO, and timely surgical correction can be lifesaving. CONCLUSION Congenital coronary artery anomalies are abnormalities involving the origin, course, or structure of the coronary vessels, with an estimated incidence ranging from 0.2% to 5.6% [ 6 ]. Among these, malignant intramural left main coronary artery (LMCA) anomalies represent a particularly rare and high-risk form of congenital heart disease that can lead to sudden cardiac death during childhood. Children presenting with chest pain and red flag symptoms should be evaluated carefully for potential cardiac causes. This case represents one of the few pediatric examples in which a malignant LMCA anomaly was stabilized with VA-ECMO support and successfully managed through surgical re-anastomosis. Early diagnosis, a multidisciplinary approach, and timely initiation of mechanical circulatory support play a lifesaving role in managing such high-risk anomalies. Declarations FUNDING This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. DECLARATİON OF COMPETİNG INTERESTS Nothing to declare. DATA AVAILABILITY STATEMENT The data supporting the findings of this study are available from the corresponding author upon reasonable request. ETHICAL APPROVAL Ethical approval was not required for this single case report. Written informed consent for publication of this case and accompanying images was obtained from the patient’s parents. INFORMED CONSENT Written informed consent was obtained from the patient’s parents for publication of this case report and the accompanying images. All identifying information has been removed to protect patient anonymity. ACKNOWLEDGMENTS The authors thank all pediatric intensive care nurses who voluntarily participated in this study. Author Contribution E.K. conceived and designed the case report, collected the clinical data, and prepared the first draft of the manuscript.M.E., N.U.T., and A.K. contributed to patient management, data interpretation, and critical revision of the manuscript.F.K. reviewed the cardiac findings and provided expert input regarding the coronary anomaly and diagnostic imaging.S.O. contributed to the surgical management and interpretation of intraoperative data.A.Ko. and O.D. supervised the case, provided intellectual guidance, and critically reviewed the final version of the manuscript.All authors read and approved the final manuscript. References Fogliazza F, Cifaldi M, Antoniol G, Canducci N, Esposito S (2024) Approaches to pediatric chest pain: a narrative review. J Clin Med 13(22):6659. 10.3390/jcm13226659 PMID: 39597803; PMCID: PMC11594360 Campos CV, Croti UA, Aiello VD (2025) Totally anomalous origin of the coronary arteries from the pulmonary trunk: a case report. Cardiol Young 35(10):2184–2186. 10.1017/S1047951125109566 Feng J, Zhao J, Li J, Sun Z, Li Q (2023) Classification, diagnosis and clinical strategy of congenital coronary artery disease in children. Front Pediatr 11:1132522. 10.3389/fped.2023.1132522 PMCID: PMC10036911; PMID: 36969282 Şerifoğlu Ş, Atik Ugan S, Onan İS, Guzeltas A (2025) A rare coronary artery anomaly: abnormal single coronary artery from pulmonary artery. Cardiol Young 35(7):1472–1475. 10.1017/S1047951125101406 Firdaus AJ, Handari SD, Prasetya I, Tjahjono CT (2025) Successful unroofing of anomalous aortic origin of the left coronary artery with intramural course in a patient with near syncope. Heart Sci J . ;6(3):Article 17. 10.21776/ub.hsj.2025.006.03.17 Silva A, Baptista MJ, Araújo E (2018) Congenital coronary artery anomalies. Rev Port Cardiol (Engl Ed) 37(4):341–350. 10.1016/j.repce.2017.09.015 Additional Declarations No competing interests reported. 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2","display":"","copyAsset":false,"role":"figure","size":401480,"visible":true,"origin":"","legend":"\u003cp\u003eCoronary CT angiography showing interarterial and intramural LMCA course between the aorta and pulmonary artery.\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-8967276/v1/554b5cb0e17735231e283c49.png"},{"id":104782118,"identity":"210e9121-c9ac-4c07-8cce-d9a1f1cd957f","added_by":"auto","created_at":"2026-03-17 07:56:51","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1334465,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-8967276/v1/0dd22c47-43a9-465f-a0cd-f088cb547364.pdf"},{"id":104176289,"identity":"a1729bc4-5131-4c99-ad7e-8dfb8b8f16b1","added_by":"auto","created_at":"2026-03-08 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However, existing literature indicates that the majority of these cases are benign in nature and arise from non-cardiac etiologies. Non-cardiac causes are most commonly psychogenic, musculoskeletal, respiratory, or gastrointestinal in origin [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eAlthough cardiac chest pain is uncommon in children, certain clinical features are considered \u0026ldquo;red flags\u0026rdquo; that call for further cardiologic evaluation. Chest pain associated with exertion, a history of syncope, or radiation of pain to the arm should raise concern for an underlying cardiovascular disorder. In such cases, electrocardiography and echocardiography are the first-line investigations, and advanced imaging may be required when initial findings are inconclusive. Even though rare, congenital coronary artery anomalies \u0026mdash; where the coronary arteries arise from an abnormal origin or follow an atypical course \u0026mdash; should always be kept in mind when evaluating children with these warning symptoms [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThis report describes a pediatric patient with a malignant course of the left main coronary artery (LMCA) anomaly who developed pulmonary edema and refractory cardiogenic shock following exertion, despite intensive medical management.\u003c/p\u003e"},{"header":"CASE PRESENTATION","content":"\u003cp\u003eA previously healthy 10-year-old boy presented to an outside facility with acute chest pain and shortness of breath that developed while he was exercising. During observation at the local hospital, the patient developed pulmonary edema and required endotracheal intubation. Conventional coronary angiography revealed a left main coronary artery (LMCA) with an intramural course and approximately 95% stenosis in the proximal segment (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). Following these findings, he was transferred to a tertiary intensive care unit for advanced management.\u003c/p\u003e \u003cp\u003eAt presentation, the electrocardiogram (ECG) showed persistent ST-segment elevation. Transthoracic echocardiography (ECHO) revealed a left ventricular ejection fraction (LVEF) of 50\u0026ndash;52%. Coronary computed tomography (CT) angiography demonstrated that the left main coronary artery (LMCA) originated abnormally high from the aortic root and followed an interarterial course between the aorta and pulmonary artery, consistent with a malignant anatomical pattern (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eLaboratory evaluation revealed a lactate level of 6 mmol/L, troponin I\u0026thinsp;\u0026gt;\u0026thinsp;10,000 ng/L (reference range: 10\u0026ndash;14 ng/L), and pro-BNP of 6,443 ng/L (reference range: 0\u0026ndash;317 ng/L). These clinical and biochemical findings were consistent with cardiogenic shock. Intensive medical therapy was initiated, including inotropic support with adrenaline (maximum dose 0.6 \u0026micro;g/kg/min) and noradrenaline (maximum dose 0.5 \u0026micro;g/kg/min), in addition to milrinone (maximum dose 0.375 \u0026micro;g/kg/min). Continuous diuretic infusion was also started to reduce volume overload. However, due to the lack of adequate hemodynamic response, emergent central veno-arterial extracorporeal membrane oxygenation (VA-ECMO) support was initiated.\u003c/p\u003e \u003cp\u003eFollowing the initiation of ECMO, hemodynamic stability was achieved, accompanied by a marked decline in lactate levels and cardiac biomarkers. Serial echocardiograms showed that the ejection fraction ranged between 44% and 52%. On the sixth day of hospitalization, the patient underwent surgery performed by the cardiovascular team. The intramural segment of the left main coronary artery (LMCA) was surgically dissected and released, then re-anastomosed to its appropriate anatomical position. After aortic repair and closure, the patient was successfully weaned from cardiopulmonary bypass (CPB), and ECMO support was discontinued.\u003c/p\u003e \u003cp\u003eIn the postoperative period, the patient was extubated the following day. Control electrocardiography showed resolution of ST-segment elevations with restoration of sinus rhythm. On the eighth day of hospitalization, chest drains and central lines were removed, oral feeding was initiated, and mobilization was achieved. The patient remained clinically stable and was discharged on day 13. During follow-up, a residual narrowing of the LMCA was detected, and elective open surgical coronary stenting was performed at another center. The procedure was successful, and the patient demonstrated good clinical recovery.\u003c/p\u003e"},{"header":"DISCUSSION","content":"\u003cp\u003eNormally, the coronary arteries arise from their respective sinuses of Valsalva \u0026mdash; the left coronary artery (LCA) from the left coronary sinus and the right coronary artery (RCA) from the right coronary sinus \u0026mdash; supplying oxygenated blood to the myocardium. Developmental deviations during embryogenesis may result in congenital coronary artery anomalies (CCAAs), which can occur either in isolation or in association with other cardiac defects. The clinical spectrum of these anomalies ranges widely, from completely asymptomatic and benign variants to malignant anatomical patterns that predispose to myocardial ischemia and sudden cardiac death during exertion [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eCoronary arteries that follow an intramural or interarterial course are generally classified as malignant variants. This anatomical configuration can significantly restrict coronary blood flow during exertion, leading to myocardial ischemia, and in some cases, sudden cardiac death may occur as the first and only symptom. This underscores both the clinical importance of malignant variants and the diagnostic challenges they present [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eIn our case, the pediatric patient presented with pulmonary edema and cardiogenic shock that developed after chest pain and dyspnea during exercise. Persistent ST-segment elevation, markedly elevated troponin levels, and coronary CT angiography findings confirmed a malignant left main coronary artery (LMCA) anomaly. This clinical picture was consistent with acute myocardial ischemia secondary to impaired coronary perfusion caused by the interarterial and intramural course of the anomalous LMCA. Given the refractory cardiogenic shock despite maximal medical therapy, veno-arterial extracorporeal membrane oxygenation (VA-ECMO) was initiated as an emergency intervention, providing temporary circulatory support and preserving organ perfusion. The successful use of ECMO in similar cases of hemodynamic instability secondary to coronary artery anomalies in children has also been reported in the literature [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eIn a young adult patient described in the literature, coronary CT angiography performed after exertional angina revealed that the left coronary artery originated anomalously from the right coronary sinus (L-ACAOS) and followed an interarterial course between the pulmonary trunk and ascending aorta. An intramural LMCA was identified, and surgical \u0026ldquo;unroofing\u0026rdquo; of the anomalous segment was successfully performed. Similarly, in our case, the diagnosis was established by coronary CT angiography; however, VA-ECMO support was required due to the development of cardiogenic shock. In contrast to the reported case, anatomical re-anastomosis of the LMCA to its normal position was performed in our patient. The rapid clinical improvement and normalization of cardiac biomarkers after surgery highlight the favorable physiological impact of the successful anatomical correction [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThis case strongly illustrates that malignant LMCA anomalies, though rare, are an important cause of sudden cardiac events in children, and that early diagnosis, prompt hemodynamic support with ECMO, and timely surgical correction can be lifesaving.\u003c/p\u003e"},{"header":"CONCLUSION","content":"\u003cp\u003eCongenital coronary artery anomalies are abnormalities involving the origin, course, or structure of the coronary vessels, with an estimated incidence ranging from 0.2% to 5.6% [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. Among these, malignant intramural left main coronary artery (LMCA) anomalies represent a particularly rare and high-risk form of congenital heart disease that can lead to sudden cardiac death during childhood. Children presenting with chest pain and red flag symptoms should be evaluated carefully for potential cardiac causes. This case represents one of the few pediatric examples in which a malignant LMCA anomaly was stabilized with VA-ECMO support and successfully managed through surgical re-anastomosis. Early diagnosis, a multidisciplinary approach, and timely initiation of mechanical circulatory support play a lifesaving role in managing such high-risk anomalies.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003eFUNDING\u003c/p\u003e\n\u003cp\u003eThis research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.\u003c/p\u003e\n\u003cp\u003eDECLARATİON OF COMPETİNG INTERESTS\u003c/p\u003e\n\u003cp\u003eNothing to declare.\u003c/p\u003e\n\u003cp\u003eDATA AVAILABILITY STATEMENT\u003c/p\u003e\n\u003cp\u003eThe data supporting the findings of this study are available from the corresponding author upon reasonable request.\u003c/p\u003e\n\u003cp\u003eETHICAL APPROVAL\u003c/p\u003e\n\u003cp\u003eEthical approval was not required for this single case report. Written informed consent for publication of this case and accompanying images was obtained from the patient\u0026rsquo;s parents.\u003c/p\u003e\n\u003cp\u003eINFORMED CONSENT\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient\u0026rsquo;s parents for publication of this case report and the accompanying images. All identifying information has been removed to protect patient anonymity.\u003c/p\u003e\n\u003cp\u003eACKNOWLEDGMENTS\u003c/p\u003e\n\u003cp\u003eThe authors thank all pediatric intensive care nurses who voluntarily participated in this study.\u003c/p\u003e\n\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\n\u003cp\u003eE.K. conceived and designed the case report, collected the clinical data, and prepared the first draft of the manuscript.M.E., N.U.T., and A.K. contributed to patient management, data interpretation, and critical revision of the manuscript.F.K. reviewed the cardiac findings and provided expert input regarding the coronary anomaly and diagnostic imaging.S.O. contributed to the surgical management and interpretation of intraoperative data.A.Ko. and O.D. supervised the case, provided intellectual guidance, and critically reviewed the final version of the manuscript.All authors read and approved the final manuscript.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eFogliazza F, Cifaldi M, Antoniol G, Canducci N, Esposito S (2024) Approaches to pediatric chest pain: a narrative review. J Clin Med 13(22):6659. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.3390/jcm13226659\u003c/span\u003e\u003cspan address=\"10.3390/jcm13226659\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003ePMID: 39597803; PMCID: PMC11594360\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eCampos CV, Croti UA, Aiello VD (2025) Totally anomalous origin of the coronary arteries from the pulmonary trunk: a case report. Cardiol Young 35(10):2184\u0026ndash;2186. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1017/S1047951125109566\u003c/span\u003e\u003cspan address=\"10.1017/S1047951125109566\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eFeng J, Zhao J, Li J, Sun Z, Li Q (2023) Classification, diagnosis and clinical strategy of congenital coronary artery disease in children. Front Pediatr 11:1132522. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.3389/fped.2023.1132522\u003c/span\u003e\u003cspan address=\"10.3389/fped.2023.1132522\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003ePMCID: PMC10036911; PMID: 36969282\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eŞerifoğlu Ş, Atik Ugan S, Onan İS, Guzeltas A (2025) A rare coronary artery anomaly: abnormal single coronary artery from pulmonary artery. Cardiol Young 35(7):1472\u0026ndash;1475. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1017/S1047951125101406\u003c/span\u003e\u003cspan address=\"10.1017/S1047951125101406\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eFirdaus AJ, Handari SD, Prasetya I, Tjahjono CT (2025) Successful unroofing of anomalous aortic origin of the left coronary artery with intramural course in a patient with near syncope. \u003cem\u003eHeart Sci J\u003c/em\u003e. ;6(3):Article 17. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.21776/ub.hsj.2025.006.03.17\u003c/span\u003e\u003cspan address=\"10.21776/ub.hsj.2025.006.03.17\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eSilva A, Baptista MJ, Ara\u0026uacute;jo E (2018) Congenital coronary artery anomalies. Rev Port Cardiol (Engl Ed) 37(4):341\u0026ndash;350. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/j.repce.2017.09.015\u003c/span\u003e\u003cspan address=\"10.1016/j.repce.2017.09.015\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\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":"congenital coronary anomaly, LMCA, pediatric cardiogenic shock, ECMO, surgical re-anastomosis","lastPublishedDoi":"10.21203/rs.3.rs-8967276/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-8967276/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e \u003cp\u003eMalignant left main coronary artery (LMCA) anomalies are rare but potentially fatal congenital defects that may cause myocardial ischemia or sudden cardiac death during exertion, even in otherwise healthy children. Early recognition and timely intervention are crucial to prevent catastrophic outcomes.\u003c/p\u003e\u003ch2\u003eCase Presentation:\u003c/h2\u003e \u003cp\u003eWe report a 10-year-old boy who presented with acute chest pain and dyspnea during exercise, followed by pulmonary edema and refractory cardiogenic shock. Electrocardiography showed persistent ST-segment elevation, and coronary CT angiography revealed a malignant LMCA with an interarterial and intramural course between the aorta and pulmonary artery. Despite intensive medical therapy, the patient required emergent veno-arterial extracorporeal membrane oxygenation (VA-ECMO) support. After stabilization, surgical re-anastomosis of the LMCA to its anatomical position was successfully performed.\u003c/p\u003e\u003ch2\u003eOutcome:\u003c/h2\u003e \u003cp\u003eThe patient recovered rapidly, with normalization of cardiac enzymes and resolution of electrocardiographic abnormalities. At follow-up, he remained clinically stable after elective coronary stenting.\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e \u003cp\u003eThis case highlights the importance of maintaining a high index of suspicion for malignant LMCA anomalies in children presenting with exertional chest pain. Prompt hemodynamic support with ECMO and timely surgical correction can be lifesaving in these rare but high-risk conditions.\u003c/p\u003e","manuscriptTitle":"A Pediatric Case of Cardiogenic Shock Bridged With ECMO Due to a Malignant Left Main Coronary Artery Anomaly","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2026-03-08 16:36:59","doi":"10.21203/rs.3.rs-8967276/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":"a83a265a-8bd6-40b9-8613-19e1fcacbe80","owner":[],"postedDate":"March 8th, 2026","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2026-03-14T10:54:55+00:00","versionOfRecord":[],"versionCreatedAt":"2026-03-08 16:36:59","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-8967276","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-8967276","identity":"rs-8967276","version":["v1"]},"buildId":"XKTyCvWXoU3ODBz1xrDgd","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}