A Rare Case of Ventricular Tachycardia Caused by an Intramyocardial Fibroma with Successful Surgical Resection in an Adult

A Rare Case of Ventricular Tachycardia Caused by an Intramyocardial Fibroma with Successful Surgical Resection in an Adult | 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 Rare Case of Ventricular Tachycardia Caused by an Intramyocardial Fibroma with Successful Surgical Resection in an Adult M. Scott Binder, Matthew Roby, Zach Chancellor, Leora Yarboro, and 3 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4266625/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 05 Sep, 2024 Read the published version in Journal of Cardiothoracic Surgery → Version 1 posted 18 You are reading this latest preprint version Abstract Background Cardiac fibromas are extremely rare in adults. The preferred treatment is surgical resection, but antiarrhythmic medications or heart transplantation have also been used previously. The cardiac imaging, particularly MRI, can be useful to help delineate between primary cardiac tumors, and surgical factors such as the extent/size of the fibroma, involvement of the coronary arteries or mitral apparatus and amount of residual myocardium influence whether surgical resection is feasible. Case Presentation A 42-year-old male presented with a wide-complex tachycardia, unresponsive to amiodarone. An echocardiogram was performed which showed a possible posterior wall mass. A cardiac MRI showed a well circumscribed lateral wall intracardiac fibroma, measuring 5.2x5.1x3.8cm with preserved function. Surgical resection was successful, and he was discharged without a defibrillator. Conclusions Cardiac fibromas are encapsulated tumors which do not infiltrate myocardium and should be surgically resected if possible. Cardiac Fibroma Cardiothoracic Surgery Multimodality Imaging Ventricular Tachycardia Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Background Cardiac fibromas in adults are extremely rare, and there are no consensus guidelines regarding management. Case reports have recommended surgical resection when feasible, but other cases have used antiarrhythmic medications or heart transplant as alternative treatment options. There is very little information regarding the optimal surgical approach and when or if patients are surgical candidates for resection. Operative treatment recommendations have typically been based upon the experience of the surgeon performing the procedure. Case Presentation A 42-year-old male was transferred from an outside hospital for a wide complex tachycardia and concern for left ventricular mass. His ECG is shown (Fig. 1 ) and was concerning for a rapid VT (around 220 beats per minute), given the width of the QRS complex and positive concordance throughout the precordial leads. He was started on Amiodarone and underwent synchronized cardioversion, but by report his rhythm degenerated into VF and he underwent CPR for 1 minute before his rhythm stabilized. He was switched to procainamide and admitted to the hospital. He underwent a TTE which illustrated a large basal to mid posterior wall mass (Fig. 2 ). He had a past medical history of potential Wolff-Parkinson-White (WPW) syndrome (following a wide complex arrhythmia at age 17, declined electrophysiologic study at that time). He also had a history of hypertension, hyperlipidemia, and type 1 diabetes on insulin. The differential diagnosis was broad, and including benign (cardiac fibroma, rhabdomyoma, myxoma, hemangioma, or lipoma) or malignant/metastatic etiologies for his mass which could result in his VT. Additionally, an atrioventricular re-entrant tachycardia was considered, given his potential history of WPW, but his baseline ECG lacked delta waves and no pre-excitation was visualized on telemetry (Fig. 1 ). Outflow tract or bundle branch re-entrant tachycardias were also considered, but less likely given the morphology of the VT. A cardiac MRI was obtained for further tissue characterization of the mass. It revealed a large, well circumscribed mass in the basal to mid lateral wall, which measured 5.2x5.1x3.8cm (Fig. 3). Left and right ventricular function was preserved. There was poor uptake on first pass perfusion, and tissue mapping showed low T1 and T2 values for the mass, relative to native myocardium (Fig. 4 ). An FDG-PET was obtained to evaluate for metabolic activity of the mass and any concurrent masses or metastases. It showed hypointense uptake of the mass relative to native myocardium (Fig. 5 ) and no significant extra-cardiac uptake. A CCT was also obtained which showed no significant coronary involvement and no coronary calcification or stenosis. Given the hypointense uptake of the mass, low T1 and T2 values and association with VT, a working diagnosis of an intramyocardial fibroma was made. A repeat MRI was obtained to ensure the mass did not infiltrate the atrioventricular groove or interfere with the mitral valve apparatus or papillary muscles. The MRI did not illustrate any involvement of the atrioventricular grove or mitral apparatus and did not appear to enter the cavity of the left ventricle. Given the localization of the mass without involvement of surrounding critical structures, surgical resection was recommended. He underwent successful surgical resection of the mass with primary closure of the left ventricle over felt (Fig. 6 ), and pathology was consistent with an intramyocardial fibroma. He had no recurrent sustained ventricular arrhythmias while hospitalized and was discharged on amiodarone and had a 30-day monitor placed to monitor for recurrences. Discussion Cardiac fibromas and rhabdomyomas are the most common benign tumors in children, but newly diagnosed fibromas in adulthood are extremely rare ( 1 ). Most patients with fibromas are asymptomatic and discovered incidentally, but there are associations with ventricular arrhythmias, heart failure or sudden cardiac death ( 1 ). Notably, unlike rhabdomyomas, fibromas do not regress over time ( 2 ). The mass may grow until the heart reaches mature size, usually around 17–20 years of age, and then remain stable, but the relative size of the fibroma to cardiac mass decreases ( 3 ). Younger patients have a smaller heart, so tend to present with heart failure due to larger tumor to heart ratios, whereas older patients present with ventricular arrhythmias most commonly ( 3 ). Given its rarity, there are no standardized guidelines for management of fibromas, but options include surgical resection, medical management (typically with beta blockers and amiodarone) or heart transplantation with or without the need for single ventricle palliation as a bridge have also been reported ( 3 – 5 ). Defibrillator placement is associated with a high rate of complications and lead fractures (if an epicardial lead is required) so should be utilized only as a last resort ( 3 ). If patients are surgical candidates, this is generally the preferred approach, and a median survival of 27 years following successful resection has been reported in retrospective studies ( 3 , 6 ). Younger age at diagnosis has been found to be a poor prognostic indicator, likely due to the larger relative tumor size compared to cardiac mass ( 7 ). Additionally, tumor location in the interventricular septum is associated with significantly increased mortality, thought to be due to involvement of the conduction system and predilection for increased arrhythmias ( 7 ). It is likely that his wide complex tachycardia at the age of 17 was misdiagnosed as WPW syndrome as well and was the first manifestation of his cardiac mass, although the details of this arrhythmia and strips are not available from that time. The patient is doing well at one month follow-up and has not had any recurrences of his ventricular arrhythmias. His cardiac monitor did not show any atrial or ventricular arrhythmias over 30 days. His ventricular function remains preserved following surgery and he has been weaned off amiodarone in the outpatient setting. He has no activity restrictions following his procedure. There are no plans for ICD placement currently. Conclusion An intramyocardial fibroma is an extremely rare cause of VT in adults, and several treatment options exist. For patients who are surgical candidates, resection is recommended to help prevent growth and recurrence of arrhythmias. Abbreviations CCT Cardiac computed tomography cMRI Cardiac magnetic resonance imaging CPR cardiopulmonary resuscitation FDG-PET fluorodeoxyglucose- positron emitted tomography TTE transthoracic echocardiogram VF ventricular fibrillation VT Ventricular Tachycardia Declarations Disclosures : The authors have nothing to disclose Funding: There was no funding utilized for this research project Ethics Approval and Consent to Participate Patient consent was obtained for performance of this research. IRB approval is waived at our institution for case reports. Consent for Publication Consent for publication was obtained from the patient in this case. Availability of Data or Materials Not applicable Competing Interests The authors declare that they have no competing interests Funding The authors declare that no funding was used in this research. Authors Contributions MB prepared the manuscript and background information. MR assisted with the MRI images. ZC, LY, SA, and JK assisted with surgical images and management perspective. DS helped with manuscript preparation. All authors read and approved the final manuscript. Acknowledgements We would like to acknowledge the patient for his contribution to furthering medical literature and understanding. We also would like to thank our institutions for their support with this research. References Gatti M, D’Angelo T, Muscogiuri G et al. Cardiovascular magnetic resonance of cardiac tumors and masses. World J Cardiol . 2021; 13(11): 628-649. Burke A, Virmani R. Pediatric Heart Tumors. Cardiovasc Pathol. 2008; 17(4): 193-198. Zheng X, Song B. Left ventricle primary cardiac fibroma in an adult: A case report. Oncol Lett. 2018; 16(4): 5463-5465. Jones J, Ramcharan T, Chaudhari M et al. Ventricular fibromas in children, arrhythmia risk, and outcomes: A multicenter study. Heart Rhythm . 2018; 15(10): 1507-1512. Waller B, Bradley S, Crumbley A, Wiles H, McQuinn T, Bennett A. Cardiac fibroma in an infant: single ventricle palliation as a bridge to heart transplantation. Ann Thorac Surg . 2003; 75(4): 1306-1308. Elbardissi A, Dearani J, Daly R et al. Survival after resection of primary cardiac tumors: a 48-year experience. Circulation . 2008; 118(14 Suppl): S7-S15. Torimitsu S, Nemoto T, Wakayama M et al. Literature survey on epidemiology and pathology of cardiac fibroma. Eur J Med Res . 2012; 17(1): 5. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 05 Sep, 2024 Read the published version in Journal of Cardiothoracic Surgery → Version 1 posted Editorial decision: Revision requested 25 May, 2024 Reviews received at journal 23 May, 2024 Reviews received at journal 14 May, 2024 Reviews received at journal 13 May, 2024 Reviews received at journal 09 May, 2024 Reviewers agreed at journal 09 May, 2024 Reviews received at journal 07 May, 2024 Reviewers agreed at journal 06 May, 2024 Reviewers agreed at journal 05 May, 2024 Reviewers agreed at journal 05 May, 2024 Reviews received at journal 05 May, 2024 Reviewers agreed at journal 05 May, 2024 Reviewers agreed at journal 04 May, 2024 Reviewers agreed at journal 02 May, 2024 Reviewers invited by journal 02 May, 2024 Editor assigned by journal 16 Apr, 2024 Submission checks completed at journal 16 Apr, 2024 First submitted to journal 14 Apr, 2024 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-4266625","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":292109060,"identity":"88f45d95-c0a0-457f-8d4a-8f7c32997b4a","order_by":0,"name":"M. Scott Binder","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABCElEQVRIie2RMUvEMBTHXxHs8qRri9BvIDwIVA+kn6WlcFPRgHA4SVx0OXTtxxAE5xwZuuTuHDve5Czc4iBonqu589xE8htCXsiP/3sJQCDwJ0HQsA+Q8L6SqVv3lAbaQckUK8RK9LMCrJDm4utmpLa2dXQ/nxk5OT0XvXl5XdFJfhzPro2UJSSH/bNPKYazynSL8ejJjkXnGhOjaa1MRw1kd630K0jm4MZQMYDgWeqHIVIGXZ9ksfIqS8vKB4kuXu+o6JYVTZTit5RYb5wFFw2ltr1gRZCtWWkwm6L3xYrl3KxxUlJy2z9Gb5dXOfXGnbyXeYLxyutsxEXg1g/18tuUQCAQ+Kd8AknOYToBKIWFAAAAAElFTkSuQmCC","orcid":"","institution":"Virginia Tech Carilion Roanoke Memorial Hospital","correspondingAuthor":true,"prefix":"","firstName":"M.","middleName":"Scott","lastName":"Binder","suffix":""},{"id":292109061,"identity":"ebbaa372-e7a9-47de-8e38-183db541824d","order_by":1,"name":"Matthew Roby","email":"","orcid":"","institution":"Virginia Tech Carilion Roanoke Memorial Hospital","correspondingAuthor":false,"prefix":"","firstName":"Matthew","middleName":"","lastName":"Roby","suffix":""},{"id":292109062,"identity":"a7aaf8b5-6c90-43be-8864-5787da44591c","order_by":2,"name":"Zach Chancellor","email":"","orcid":"","institution":"University of Virginia","correspondingAuthor":false,"prefix":"","firstName":"Zach","middleName":"","lastName":"Chancellor","suffix":""},{"id":292109063,"identity":"89b56509-3e72-4901-a2f0-8eac2d5d4556","order_by":3,"name":"Leora Yarboro","email":"","orcid":"","institution":"University of Virginia","correspondingAuthor":false,"prefix":"","firstName":"Leora","middleName":"","lastName":"Yarboro","suffix":""},{"id":292109064,"identity":"1ca361b4-6305-4a6d-9bfd-011ac87aa67c","order_by":4,"name":"Scott Arnold","email":"","orcid":"","institution":"Virginia Tech Carilion Roanoke Memorial Hospital","correspondingAuthor":false,"prefix":"","firstName":"Scott","middleName":"","lastName":"Arnold","suffix":""},{"id":292109065,"identity":"1ccf9008-06aa-4295-9b39-4f50375ec221","order_by":5,"name":"John Kern","email":"","orcid":"","institution":"University of Virginia","correspondingAuthor":false,"prefix":"","firstName":"John","middleName":"","lastName":"Kern","suffix":""},{"id":292109066,"identity":"f220a98d-03da-4d32-9066-6b9d2e9f4b84","order_by":6,"name":"David Sane","email":"","orcid":"","institution":"Virginia Tech Carilion Roanoke Memorial Hospital","correspondingAuthor":false,"prefix":"","firstName":"David","middleName":"","lastName":"Sane","suffix":""}],"badges":[],"createdAt":"2024-04-15 01:35:02","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4266625/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4266625/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s13019-024-02986-3","type":"published","date":"2024-09-05T16:05:07+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":55252273,"identity":"77aa1d4a-2fb6-46dd-926c-1cb7059271e1","added_by":"auto","created_at":"2024-04-24 17:47:53","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":628804,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eA. ECG in Wide Complex Tachycardia\u003c/strong\u003e. Panel A illustrates his wide complex tachycardia at a rate of around 220 beats per minute. Positive inferior leads (II, III, aVF) indicate a basal/mid ventricular or outflow tract origin, and positive concordance throughout the precordial leads suggests a posterior ventricular origin and argues against a supraventricular tachycardia with aberrancy.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eB. Baseline ECG\u003c/strong\u003e. Panel B illustrates his baseline ECG, which demonstrates nonspecific T wave flattening, a normal axis and notable lack of delta waves indicative of pre-excitation.\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-4266625/v1/28b488a85c5d934a2a2dd81b.png"},{"id":55252271,"identity":"c5a6145c-0619-4b4b-bc7c-236c5d8baa05","added_by":"auto","created_at":"2024-04-24 17:47:52","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":236581,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eTransthoracic Echocardiogram: \u003c/strong\u003eA transthoracic echocardiogram illustrates a large mass in the posterior wall of the left ventricle (LV) with normal right ventricular (RV) size.\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-4266625/v1/c9f133b82e19cc6b75b097be.png"},{"id":55252272,"identity":"e5210f43-bbd7-4be6-96e6-d6f967fd2718","added_by":"auto","created_at":"2024-04-24 17:47:53","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":244465,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eCardiac MRI: \u003c/strong\u003eA short axis basal section (A) and apical 3 chamber (B) on cardiac MRI illustrates the large inferolateral/posterior wall mass which is well circumscribed and iso-intense to native myocardium.\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-4266625/v1/6568ed8d1bfd398f8e101cbe.png"},{"id":55252637,"identity":"c0e13f47-1003-4c33-a02c-a72d907276f6","added_by":"auto","created_at":"2024-04-24 17:55:54","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":422077,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eT1 and T2 Maps: \u003c/strong\u003eShown are the T1 (left) and T2 (right) maps of short axis sections on the cardiac MRI. The mass is hypointense on T1 and T2 weighted imaging relative to native myocardium.\u003c/p\u003e","description":"","filename":"4.png","url":"https://assets-eu.researchsquare.com/files/rs-4266625/v1/f298185fa09bdd197e77fa3f.png"},{"id":55252276,"identity":"b6ed3815-dac6-4756-adde-b1408bcfe825","added_by":"auto","created_at":"2024-04-24 17:47:53","extension":"png","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":415919,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eFDG-PET Scan: \u003c/strong\u003eAn FDG-PET axial slice at the level of the left ventricle, which illustrates hypointense uptake of the mass relative to native myocardium. There is no significant uptake of FDG elsewhere in the heart.\u003c/p\u003e","description":"","filename":"5.png","url":"https://assets-eu.researchsquare.com/files/rs-4266625/v1/73cfd61333570d73d8d3d9e8.png"},{"id":55252275,"identity":"2548dedc-5123-44bb-8bf6-63f88fe3c55c","added_by":"auto","created_at":"2024-04-24 17:47:53","extension":"png","order_by":6,"title":"Figure 6","display":"","copyAsset":false,"role":"figure","size":791625,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eGross Specimens: \u003c/strong\u003ePanel A illustrates the fibroma removal from the lateral wall of the left ventricle. Panel B shows the final specimen after removal, around 10cm in length. Panel C is the residual ventricular myocardium, without entry into the lumen, which was sutured closed over felt (panel D).\u003c/p\u003e","description":"","filename":"6.png","url":"https://assets-eu.researchsquare.com/files/rs-4266625/v1/2cd7d073acbd9ef2635ebd8d.png"},{"id":64185701,"identity":"75fad6a5-e8cb-4fd4-b433-c29d258da636","added_by":"auto","created_at":"2024-09-09 16:21:06","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":3541750,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4266625/v1/d1e11d21-f781-4c55-a218-6430055ac5f0.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"A Rare Case of Ventricular Tachycardia Caused by an Intramyocardial Fibroma with Successful Surgical Resection in an Adult","fulltext":[{"header":"Background","content":"\u003cp\u003eCardiac fibromas in adults are extremely rare, and there are no consensus guidelines regarding management. Case reports have recommended surgical resection when feasible, but other cases have used antiarrhythmic medications or heart transplant as alternative treatment options. There is very little information regarding the optimal surgical approach and when or if patients are surgical candidates for resection. Operative treatment recommendations have typically been based upon the experience of the surgeon performing the procedure.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eA 42-year-old male was transferred from an outside hospital for a wide complex tachycardia and concern for left ventricular mass. His ECG is shown (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e) and was concerning for a rapid VT (around 220 beats per minute), given the width of the QRS complex and positive concordance throughout the precordial leads. He was started on Amiodarone and underwent synchronized cardioversion, but by report his rhythm degenerated into VF and he underwent CPR for 1 minute before his rhythm stabilized. He was switched to procainamide and admitted to the hospital. He underwent a TTE which illustrated a large basal to mid posterior wall mass (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). He had a past medical history of potential Wolff-Parkinson-White (WPW) syndrome (following a wide complex arrhythmia at age 17, declined electrophysiologic study at that time). He also had a history of hypertension, hyperlipidemia, and type 1 diabetes on insulin.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eThe differential diagnosis was broad, and including benign (cardiac fibroma, rhabdomyoma, myxoma, hemangioma, or lipoma) or malignant/metastatic etiologies for his mass which could result in his VT. Additionally, an atrioventricular re-entrant tachycardia was considered, given his potential history of WPW, but his baseline ECG lacked delta waves and no pre-excitation was visualized on telemetry (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). Outflow tract or bundle branch re-entrant tachycardias were also considered, but less likely given the morphology of the VT.\u003c/p\u003e \u003cp\u003eA cardiac MRI was obtained for further tissue characterization of the mass. It revealed a large, well circumscribed mass in the basal to mid lateral wall, which measured 5.2x5.1x3.8cm (Fig.\u0026nbsp;3). Left and right ventricular function was preserved. There was poor uptake on first pass perfusion, and tissue mapping showed low T1 and T2 values for the mass, relative to native myocardium (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e4\u003c/span\u003e). An FDG-PET was obtained to evaluate for metabolic activity of the mass and any concurrent masses or metastases. It showed hypointense uptake of the mass relative to native myocardium (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e5\u003c/span\u003e) and no significant extra-cardiac uptake. A CCT was also obtained which showed no significant coronary involvement and no coronary calcification or stenosis. Given the hypointense uptake of the mass, low T1 and T2 values and association with VT, a working diagnosis of an intramyocardial fibroma was made.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eA repeat MRI was obtained to ensure the mass did not infiltrate the atrioventricular groove or interfere with the mitral valve apparatus or papillary muscles. The MRI did not illustrate any involvement of the atrioventricular grove or mitral apparatus and did not appear to enter the cavity of the left ventricle. Given the localization of the mass without involvement of surrounding critical structures, surgical resection was recommended. He underwent successful surgical resection of the mass with primary closure of the left ventricle over felt (Fig.\u0026nbsp;\u003cspan refid=\"Fig5\" class=\"InternalRef\"\u003e6\u003c/span\u003e), and pathology was consistent with an intramyocardial fibroma. He had no recurrent sustained ventricular arrhythmias while hospitalized and was discharged on amiodarone and had a 30-day monitor placed to monitor for recurrences.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eCardiac fibromas and rhabdomyomas are the most common benign tumors in children, but newly diagnosed fibromas in adulthood are extremely rare (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). Most patients with fibromas are asymptomatic and discovered incidentally, but there are associations with ventricular arrhythmias, heart failure or sudden cardiac death (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). Notably, unlike rhabdomyomas, fibromas do not regress over time (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). The mass may grow until the heart reaches mature size, usually around 17\u0026ndash;20 years of age, and then remain stable, but the relative size of the fibroma to cardiac mass decreases (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). Younger patients have a smaller heart, so tend to present with heart failure due to larger tumor to heart ratios, whereas older patients present with ventricular arrhythmias most commonly (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). Given its rarity, there are no standardized guidelines for management of fibromas, but options include surgical resection, medical management (typically with beta blockers and amiodarone) or heart transplantation with or without the need for single ventricle palliation as a bridge have also been reported (\u003cspan additionalcitationids=\"CR4\" citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e). Defibrillator placement is associated with a high rate of complications and lead fractures (if an epicardial lead is required) so should be utilized only as a last resort (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). If patients are surgical candidates, this is generally the preferred approach, and a median survival of 27 years following successful resection has been reported in retrospective studies (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e). Younger age at diagnosis has been found to be a poor prognostic indicator, likely due to the larger relative tumor size compared to cardiac mass (\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e). Additionally, tumor location in the interventricular septum is associated with significantly increased mortality, thought to be due to involvement of the conduction system and predilection for increased arrhythmias (\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e). It is likely that his wide complex tachycardia at the age of 17 was misdiagnosed as WPW syndrome as well and was the first manifestation of his cardiac mass, although the details of this arrhythmia and strips are not available from that time.\u003c/p\u003e \u003cp\u003eThe patient is doing well at one month follow-up and has not had any recurrences of his ventricular arrhythmias. His cardiac monitor did not show any atrial or ventricular arrhythmias over 30 days. His ventricular function remains preserved following surgery and he has been weaned off amiodarone in the outpatient setting. He has no activity restrictions following his procedure. There are no plans for ICD placement currently.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eAn intramyocardial fibroma is an extremely rare cause of VT in adults, and several treatment options exist. For patients who are surgical candidates, resection is recommended to help prevent growth and recurrence of arrhythmias.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eCCT\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eCardiac computed tomography\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003ecMRI\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eCardiac magnetic resonance imaging\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eCPR\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003ecardiopulmonary resuscitation\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eFDG-PET\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003efluorodeoxyglucose- positron emitted tomography\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eTTE\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003etransthoracic echocardiogram\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eVF\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eventricular fibrillation\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eVT\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eVentricular Tachycardia\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eDisclosures\u003c/strong\u003e: The authors have nothing to disclose\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding:\u003c/strong\u003e There was no funding utilized for this research project\u003cstrong\u003e\u003cbr\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eEthics Approval and Consent to Participate\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003ePatient consent was obtained for performance of this research. IRB approval is waived at our institution for case reports.\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eConsent for Publication\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eConsent for publication was obtained from the patient in this case.\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eAvailability of Data or Materials\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eCompeting Interests\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no competing interests\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eFunding\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that no funding was used in this research.\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eAuthors Contributions\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eMB prepared the manuscript and background information. MR assisted with the MRI images. ZC, LY, SA, and JK assisted with surgical images and management perspective. DS helped with manuscript preparation. All authors read and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eAcknowledgements\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eWe would like to acknowledge the patient for his contribution to furthering medical literature and understanding. We also would like to thank our institutions for their support with this research.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n \u003cli\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003eGatti M, D\u0026rsquo;Angelo T, Muscogiuri G et al. Cardiovascular magnetic resonance of cardiac tumors and masses. \u003cem\u003eWorld J Cardiol\u003c/em\u003e. 2021; 13(11): 628-649.\u003c/li\u003e\n \u003cli\u003eBurke A, Virmani R. Pediatric Heart Tumors. \u003cem\u003eCardiovasc Pathol.\u003c/em\u003e 2008; 17(4): 193-198.\u003c/li\u003e\n \u003cli\u003eZheng X, Song B. Left ventricle primary cardiac fibroma in an adult: A case report. \u003cem\u003eOncol Lett.\u003c/em\u003e 2018; 16(4): 5463-5465.\u003c/li\u003e\n \u003cli\u003eJones J, Ramcharan T, Chaudhari M et al. Ventricular fibromas in children, arrhythmia risk, and outcomes: A multicenter study. \u003cem\u003eHeart Rhythm\u003c/em\u003e. 2018; 15(10): 1507-1512.\u003c/li\u003e\n \u003cli\u003eWaller B, Bradley S, Crumbley A, Wiles H, McQuinn T, Bennett A. Cardiac fibroma in an infant: single ventricle palliation as a bridge to heart transplantation. \u003cem\u003eAnn Thorac Surg\u003c/em\u003e. 2003; 75(4): 1306-1308.\u003c/li\u003e\n \u003cli\u003eElbardissi A, Dearani J, Daly R et al. Survival after resection of primary cardiac tumors: a 48-year experience. \u003cem\u003eCirculation\u003c/em\u003e. 2008; 118(14 Suppl): S7-S15.\u003c/li\u003e\n \u003cli\u003eTorimitsu S, Nemoto T, Wakayama M et al. Literature survey on epidemiology and pathology of cardiac fibroma. \u003cem\u003eEur J Med Res\u003c/em\u003e. 2012; 17(1): 5.\u003cstrong\u003e\u003c/strong\u003e\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"journal-of-cardiothoracic-surgery","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"jcts","sideBox":"Learn more about [Journal of Cardiothoracic Surgery](http://cardiothoracicsurgery.biomedcentral.com)","snPcode":"13019","submissionUrl":"https://submission.nature.com/new-submission/13019/3","title":"Journal of Cardiothoracic Surgery","twitterHandle":"@BioMedCentral","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Cardiac Fibroma, Cardiothoracic Surgery, Multimodality Imaging, Ventricular Tachycardia","lastPublishedDoi":"10.21203/rs.3.rs-4266625/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4266625/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e \u003cp\u003eCardiac fibromas are extremely rare in adults. The preferred treatment is surgical resection, but antiarrhythmic medications or heart transplantation have also been used previously. The cardiac imaging, particularly MRI, can be useful to help delineate between primary cardiac tumors, and surgical factors such as the extent/size of the fibroma, involvement of the coronary arteries or mitral apparatus and amount of residual myocardium influence whether surgical resection is feasible.\u003c/p\u003e\u003ch2\u003eCase Presentation\u003c/h2\u003e \u003cp\u003eA 42-year-old male presented with a wide-complex tachycardia, unresponsive to amiodarone. An echocardiogram was performed which showed a possible posterior wall mass. A cardiac MRI showed a well circumscribed lateral wall intracardiac fibroma, measuring 5.2x5.1x3.8cm with preserved function. Surgical resection was successful, and he was discharged without a defibrillator.\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e \u003cp\u003eCardiac fibromas are encapsulated tumors which do not infiltrate myocardium and should be surgically resected if possible.\u003c/p\u003e","manuscriptTitle":"A Rare Case of Ventricular Tachycardia Caused by an Intramyocardial Fibroma with Successful Surgical Resection in an Adult","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-04-24 17:47:48","doi":"10.21203/rs.3.rs-4266625/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2024-05-25T15:43:07+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-05-23T17:41:50+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-05-14T08:14:26+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-05-13T16:21:32+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-05-09T12:06:17+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"258152399092665964128870720694328992046","date":"2024-05-09T06:48:09+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-05-08T01:54:10+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"233527936601981678479308905788905259803","date":"2024-05-07T00:06:30+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"204939005665728400707547089011795109033","date":"2024-05-06T00:15:55+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"20584164601732295838435821327621510288","date":"2024-05-05T21:21:57+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-05-05T13:31:28+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"109563704647112679961089695719848876915","date":"2024-05-05T12:02:10+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"171612010550517348071941823840530104576","date":"2024-05-05T03:57:26+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"82913304743977280961419888810069792875","date":"2024-05-02T21:55:12+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-05-02T15:33:36+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-04-17T02:04:27+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2024-04-17T02:04:27+00:00","index":"","fulltext":""},{"type":"submitted","content":"Journal of Cardiothoracic Surgery","date":"2024-04-15T01:33:41+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"journal-of-cardiothoracic-surgery","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"jcts","sideBox":"Learn more about [Journal of Cardiothoracic Surgery](http://cardiothoracicsurgery.biomedcentral.com)","snPcode":"13019","submissionUrl":"https://submission.nature.com/new-submission/13019/3","title":"Journal of Cardiothoracic Surgery","twitterHandle":"@BioMedCentral","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"c0d7ad29-a0e4-4e97-adc9-a00ced81b066","owner":[],"postedDate":"April 24th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2024-09-09T16:10:38+00:00","versionOfRecord":{"articleIdentity":"rs-4266625","link":"https://doi.org/10.1186/s13019-024-02986-3","journal":{"identity":"journal-of-cardiothoracic-surgery","isVorOnly":false,"title":"Journal of Cardiothoracic Surgery"},"publishedOn":"2024-09-05 16:05:07","publishedOnDateReadable":"September 5th, 2024"},"versionCreatedAt":"2024-04-24 17:47:48","video":"","vorDoi":"10.1186/s13019-024-02986-3","vorDoiUrl":"https://doi.org/10.1186/s13019-024-02986-3","workflowStages":[]},"version":"v1","identity":"rs-4266625","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4266625","identity":"rs-4266625","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}