Multimodality Imaging-Guided Diagnosis and Management of a Rare Coronary Artery Fistula with Coexistent Left Ventricular Noncompaction Cardiomyopathy

Multimodality Imaging-Guided Diagnosis and Management of a Rare Coronary Artery Fistula with Coexistent Left Ventricular Noncompaction Cardiomyopathy | 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 Multimodality Imaging-Guided Diagnosis and Management of a Rare Coronary Artery Fistula with Coexistent Left Ventricular Noncompaction Cardiomyopathy Camilo Fernández Bravo, Rachel Fernández Bravo This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-6565350/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 Coronary artery fistulae (CAF) are rare congenital anomalies involving abnormal communications between coronary arteries and cardiac chambers or vessels, with an estimated prevalence of 0.002% in the general population. Left ventricular noncompaction cardiomyopathy (LVNC) is a rare cardiomyopathy characterized by excessive trabeculations and deep recesses, associated with heart failure, arrhythmias, and thromboembolism. The coexistence of CAF and LVNC is exceptionally rare and poses significant diagnostic and therapeutic challenges. We report a 42year-old male presenting with severe heart failure due to a large CAF from the left anterior descending artery (LAD) to the right ventricle, coexisting with LVNC. Multimodality imaging, including transthoracic echocardiography (TTE), cardiac magnetic resonance imaging (CMR), coronary computed tomography angiography (CCTA), and invasive angiography, was instrumental in diagnosis and treatment planning. Genetic testing revealed a novel sarcomeric mutation, providing insights into the etiology of LVNC. The patient underwent successful transcatheter closure of the CAF using an Amplatzer Vascular Plug II, with significant improvement in symptoms and left ventricular function at 12-month follow-up. This case highlights the critical role of advanced imaging and genetic evaluation in managing complex cardiovascular anomalies and underscores the efficacy of transcatheter interventions in selected patients. Cardiac & Cardiovascular Systems Coronary artery fistula left ventricular noncompaction cardiomyopathy multimodality imaging transcatheter closure heart failure genetic testing Full Text 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. 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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-6565350","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":450236220,"identity":"71242af4-0fa0-4ed9-816b-d2ed3f279d07","order_by":0,"name":"Camilo Fernández Bravo","email":"data:image/png;base64,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","orcid":"https://orcid.org/0009-0004-6848-7115","institution":"UCM Carlos J Finlay","correspondingAuthor":true,"prefix":"","firstName":"Camilo","middleName":"Fernández","lastName":"Bravo","suffix":""},{"id":450236221,"identity":"852adb94-eb9b-4e1d-ae34-d0c41e18a707","order_by":1,"name":"Rachel Fernández Bravo","email":"data:image/png;base64,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","orcid":"","institution":"Programa Mais Medicos Brasil","correspondingAuthor":true,"prefix":"","firstName":"Rachel","middleName":"Fernández","lastName":"Bravo","suffix":""}],"badges":[],"createdAt":"2025-04-30 13:25:40","currentVersionCode":1,"declarations":{"humanSubjects":true,"vertebrateSubjects":false,"conflictsOfInterestStatement":false,"humanSubjectEthicalGuidelines":true,"humanSubjectConsent":true,"humanSubjectClinicalTrial":false,"humanSubjectCaseReport":false,"vertebrateSubjectEthicalGuidelines":false},"doi":"10.21203/rs.3.rs-6565350/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6565350/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":81794096,"identity":"dd27b0d8-a6a0-4903-b6a5-9e146c0952d4","added_by":"auto","created_at":"2025-05-02 02:49:30","extension":"pdf","order_by":1,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":432180,"visible":true,"origin":"","legend":"","description":"","filename":"CaseReport.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6565350/v1_covered_fad3c6bb-3706-4f0f-8cb4-4ba3fe2a7cab.pdf"}],"financialInterests":"The authors declare no competing interests.","formattedTitle":"\u003cp\u003eMultimodality Imaging-Guided Diagnosis and Management of a Rare Coronary Artery Fistula with Coexistent Left Ventricular Noncompaction Cardiomyopathy\u003c/p\u003e","fulltext":[],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":false,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"hideJournal":true,"highlight":"","institution":"UCM Carlos J Fiblay ","isAcceptedByJournal":false,"isAuthorSuppliedPdf":true,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":true,"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":"Coronary artery fistula, left ventricular noncompaction cardiomyopathy, multimodality imaging, transcatheter closure, heart failure, genetic testing","lastPublishedDoi":"10.21203/rs.3.rs-6565350/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6565350/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cem\u003eCoronary artery fistulae (CAF) are rare congenital anomalies involving abnormal communications between coronary arteries and cardiac chambers or vessels, with an estimated prevalence of 0.002% in the general population. Left ventricular noncompaction cardiomyopathy (LVNC) is a rare cardiomyopathy characterized by excessive trabeculations and deep recesses, associated with heart failure, arrhythmias, and thromboembolism. The coexistence of CAF and LVNC is exceptionally rare and poses significant diagnostic and therapeutic challenges. We report a 42year-old male presenting with severe heart failure due to a large CAF from the left anterior descending artery (LAD) to the right ventricle, coexisting with LVNC. Multimodality imaging, including transthoracic echocardiography (TTE), cardiac magnetic resonance imaging (CMR), coronary computed tomography angiography (CCTA), and invasive angiography, was instrumental in diagnosis and treatment planning. Genetic testing revealed a novel sarcomeric mutation, providing insights into the etiology of LVNC. 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This case highlights the critical role of advanced imaging and genetic evaluation in managing complex cardiovascular anomalies and underscores the efficacy of transcatheter interventions in selected patients.\u003c/em\u003e\u003c/p\u003e","manuscriptTitle":"Multimodality Imaging-Guided Diagnosis and Management of a Rare Coronary Artery Fistula with Coexistent Left Ventricular Noncompaction Cardiomyopathy","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-05-02 02:41:23","doi":"10.21203/rs.3.rs-6565350/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":"d544c258-aa52-4ad4-a93a-c346ff323be5","owner":[],"postedDate":"May 2nd, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[{"id":47911176,"name":"Cardiac \u0026 Cardiovascular Systems"}],"tags":[],"updatedAt":"2025-05-02T02:41:23+00:00","versionOfRecord":[],"versionCreatedAt":"2025-05-02 02:41:23","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-6565350","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6565350","identity":"rs-6565350","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}