Double chambered right ventricle in adulthood: A case report and review of the literature

Double chambered right ventricle in adulthood: A case report and review of the literature | 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 Double chambered right ventricle in adulthood: A case report and review of the literature Taha El Khazzar, Kaoutar Benjaout, Mouhcine Madani This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-5595617/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: The double chambered right ventricle (DCRV) is a rare congenital malformation, in which a dislocated hypertrophic muscular band causes the subdivision of the right ventricle in two chambers, proximal or high-pressure chamber and distal or low-pressure chamber. It is recommended to use echocardiography and cardiac catheterization for diagnosis. This case report seeks to provide additional data for comparison with existing literature, thereby contributing to a more comprehensive understanding of this condition. Case presentation: We are reporting the case of DCRV in an adult female, who was evaluated for shortness of breath. Diagnosis was confirmed by transthoracic echocardiography, with a maximal gradient of 97mmHg. The patient underwent a successful surgery. Conclusions: Most cases of classic DCRV are diagnosed in infant, and are associated with ventricular septal defect (VSD). Surgery is the most effective treatment by removing the muscular band. Double chambered right ventricle congenital heart disease cardiac surgery case report Figures Figure 1 Background The double chambered right ventricle (DCRV) is a rare congenital malformation. According to the literature, there are two etiological hypotheses: the presence of a hypertrophied muscular band in an abnormal location or sub-infundibular hypertrophy of abnormal muscular tissue with varying degrees of obstruction. 1 This results in a proximal chamber with high pressure and a distal chamber with low pressure. In adults, it is associated with a ventricular septal defect (VSD) in 3–10% of cases. 2 , 3 Surgery is the only treatment is also recommended in the early stages, even for asymptomatic patients, to prevent prolonged exposure of the right ventricle to high pressure. Case presentation A 27-year-old female patient with no notable medical history presented to the emergency department with progressively worsening NYHA class II dyspnea. The clinical examination revealed no anomalies apart from a left parasternal systolic murmur, and the electrocardiogram showed right ventricular hypertrophy (RVH). Laboratory investigations revealed no abnormalities, transthoracic echocardiography revealed a right ventricle at the upper limit of size, with hypertrophied walls, a very tight mid-ventricular stenosis with a maximal gradient of 97 mmHg, and an infundibular ventricular septal defect measuring 5mm shunting from left to right. The surgical intervention was performed through a vertical median sternotomy under cardiopulmonary bypass with aortic clamping. The cardiac incision consisted of a vertical infundibulotomy, allowing exposure to the mid-ventricular stenosis. The transverse muscular bands were resected, and the infundibular stenosis was relieved. The infundibulotomy was subsequently closed using an autologous pericardial patch [Figure 1 ]. The postoperative course was favorable, with the resolution of symptoms and a good result on follow-up echocardiography. Discussion Peacock described the first examples of the muscular division of the right ventricle in 1867. Later, Keith (1909) and Brock (1957) described cases of the extension of muscular bands towards the apex of the ventricle and muscular diaphragms surrounding the base of the infundibulum, respectively. 4 Subsequently, Tsifuts et al. and Lucas et al. considered that the displacement of the septal origin of the moderator band produced an abnormal muscular band, leading to DCRV. The right ventricle consists of an inlet chamber, apical trabercular, and outlet. 5 The apex of the right ventricle is characterized by a rich set of trabecular muscles, forming three bands, with the most important being the moderator band that connects the free wall to the interventricular septum. 6 , 7 One of the etiological mechanisms is the mispositioning of the septal insertion of the moderator band at birth, especially in association with a VSD. 8 According to Thomas M et al., DCRV constitutes the only sub-infundibular form of congenital heart disease causing obstruction of the right ventricular outflow tract. 9 Other forms may cause turbulence in this tract, thus promoting the proliferation of septo-marginal trabeculations and the mispositioned moderator band's hypertrophy. Two classifications have been proposed: Folger described two abnormal positions of the muscular band, high (or horizontal) and low (or oblique). 11 Galiuto distinguished two types of intracavitary obstruction: type 1 is characterized by the presence of an abnormal muscular band, while in type 2, the obstruction is caused by the hypertrophy of septal and parietal muscles, often associated with a VSD. 12 Other associated anomalies have been reported, such as pulmonary stenosis, atrial septal defect (ASD), aortic regurgitation, tricuspid regurgitation, sinus of Valsalva aneurysm, Tetralogy of Fallot, complete or corrected transposition of the great vessels, and Ebstein's anomaly. 13 In adults, the most common symptoms are exertional dyspnea and exercise intolerance, likely related to low cardiac output or right ventricular dysfunction. Some patients have presented syncope or near-fainting due to tight infundibular stenosis causing disrupted blood flow. 14 Cases of fever and lethargy associated with endocarditis have also been reported. 15 Many studies have established the rapid progression of obstruction in this condition in the pediatric population. 3 , 16 , 17 Oliver et al. reported that DCRV can progress more rapidly in adults and established a significant relationship between age and pressure gradient on Doppler. 18 The diagnosis of DCRV is primarily made in childhood. In adulthood, diagnosis is challenging and sometimes missed. 2 , 19 , 20 Cardiac catheterization was the diagnostic method of choice for this malformation. 21 , 22 More recently, it has been recommended in cases of suspected significant intraventricular gradient. 23 Lascano et al. reported discrepancies in the results obtained by transthoracic echocardiography (TTE) compared to transesophageal echocardiography (TEE) in the diagnosis. TTE has limitations due to the retrosternal location and irregular shape of the right ventricle, making it difficult to visualize, especially in adults. 24 Our patient's diagnosis was delayed due to the operator-dependent nature of transthoracic echocardiography (TTE) and its associated limitations. TEE allows better visualization of the right ventricle to distinguish the abnormal muscular band and measure the pressure gradient between the two chambers. 2 Cardiac magnetic resonance imaging (MRI), thanks to acoustic windows and the choice of acquisition planes, is increasingly used in diagnosing congenital heart diseases in adults. 25 It allows precise characterization of DCRV in patients who are difficult to evaluate by echocardiography. 26 Surgical treatment is indicated when the gradient between the two chambers exceeds 40 mmHg (even in the absence of symptoms) or in cases of association with a VSD with heart failure symptoms. Resection can be performed through a transatrial, transventricular, or combined approach (transatrial and transpulmonary). 27 Ventriculotomy is often necessary in adults as they present at advanced stages of obstruction due to the rapid progression of this condition. It allows for complete resection of the obstruction. 28 The progression of pressure overload in the proximal right ventricular chamber and structural abnormalities related to hypertrophy of the moderator band promote the development of ventricular arrhythmias. 29 Therefore, avoiding ventriculotomy as a means of preventing this complication is not sufficient because extensive resection is required to relieve the obstruction. 27 Conclusions Double-chambered right ventricle (DCRV) is a rare congenital condition often missed in adults due to subtle presentation and imaging limitations. Advanced diagnostics like TEE and cardiac MRI are crucial for accurate identification, and timely surgical intervention is essential to prevent complications and ensure complete obstruction relief. Abbreviations DCRV Double chambered right ventricle VSD Ventricular septal defect RHV Right ventricular hypertrophy ASD Atrial septal defect TTE Transthoracic echocardiography TEE Transesophageal echocardiography MRI Magnetic resonance imaging Declarations Ethics approval and consent to participate Not applicable Consent for publication Yes. Written and informed consent to publish information is obtained from the patient Funding None Author Contribution The first ET and second BK authors were responsible for manuscript’s writing, literature review, and editing. MM was responsible for literature review and editing. The final paper has been reviewed and approved by all authors. References Aleksandra Nikolic et al., « An (In)Significant Ventricular Septal Defect and/or Double-Chambered Right Ventricle: Are There Any Differences in Diagnosis and Prognosis in Adult Patients? », Cardiology 134, n o 3 (2016): 375–80, https://doi.org/10.1159/000444743 . Martin E. Lascano et al., « Difficulty in Diagnosing Double-Chambered Right Ventricle in Adults », The American Journal of Cardiology 88, n o 7 (octobre 2001): 816–19, https://doi.org/10.1016/S0002-9149(01)01862-8 . Giacomo Pongiglione et al., « Mechanism of Acquired Right Ventricular Outflow Tract Obstruction in Patients with Ventricular Septal Defect: An Angiocardiographic Study », The American Journal of Cardiology 50, n o 4 (octobre 1982): 776–80, https://doi.org/10.1016/0002-9149(82)91233-4 . Angelo Restivo et al., « Divided Right Ventricle: A Review of Its Anatomical Varieties », Pediatric Cardiology 5, n o 3 (juillet 1984): 197–204, https://doi.org/10.1007/BF02427045 . Marios Loukas et al., « Double-Chambered Right Ventricle: A Review », Cardiovascular Pathology 22, n o 6 (novembre 2013): 417–23, https://doi.org/10.1016/j.carpath.2013.03.004 . S Y Ho, « Anatomy, Echocardiography, and Normal Right Ventricular Dimensions », Heart 92, n o suppl_1 (1 avril 2006): i2–13, https://doi.org/10.1136/hrt.2005.077875 . Marios Loukas et al., « Anatomical Observations of the Moderator Band », Clinical Anatomy 23, n o 4 (mai 2010): 443–50, https://doi.org/10.1002/ca.20968 . Pierre C. Wong et al., « Pulmonary Valve-Moderator Band Distance and Association with Development of Double-Chambered Right Ventricle », The American Journal of Cardiology 68, n o 17 (décembre 1991): 1681–86, https://doi.org/10.1016/0002-9149(91)90329-J . Thomas M. Bashore, « Adult Congenital Heart Disease: Right Ventricular Outflow Tract Lesions », Circulation 115, n o 14 (10 avril 2007): 1933–47, https://doi.org/10.1161/CIRCULATIONAHA.105.592345 . Sherif Moustafa et al., « Double Chambered Right Ventricle with Ventricular Septal Defect in Adults: Case Series and Review of the Literature », Journal of Cardiovascular Ultrasound 23, n o 1 (2015): 48, https://doi.org/10.4250/jcu.2015.23.1.48 . Gordon M. Folger, « The Right Ventricular Pouch: A Proposed Explanation for the Electro-Vectorcardiographic Pattern of Double Chambered Right Ventricle », Angiology 37, n o 7 (juillet 1986): 483–86, https://doi.org/10.1177/000331978603700701 . Leonarda Galiuto, Patrick W. O’Leary, et James B. Seward, « Double-Chambered Right Ventricle: Echocardiographic Features », Journal of the American Society of Echocardiography 9, n o 3 (mai 1996): 300–305, https://doi.org/10.1016/S0894-7317(96)90144-3 . Ergün Çil et al., « Double-Chambered Right Ventricle: Experience with 52 Cases », International Journal of Cardiology 50, n o 1 (juin 1995): 19–29, https://doi.org/10.1016/0167-5273(95)02343-U . Doff B McElhinney, Kanu M Chatterjee, et V.Mohan Reddy, « Double-Chambered Right Ventricle Presenting in Adulthood », The Annals of Thoracic Surgery 70, n o 1 (juillet 2000): 124–27, https://doi.org/10.1016/S0003-4975(00)01320-5 . Daniel Kveselis et al., « Long-Term Prognosis after Repair of Double-Chamber Right Ventricle with Ventricular Septal Defect », The American Journal of Cardiology 54, n o 10 (décembre 1984): 1292–95, https://doi.org/10.1016/S0002-9149(84)80084-3 . Russell V. Lucas et al., « Anomalous Muscle Bundle of the Right Ventricle: Hemodynamic Consequences and Surgical Considerations », Circulation 25, n o 3 (mars 1962): 443–55, https://doi.org/10.1161/01.CIR.25.3.443 . Alexis F. Hartmann, David Goldring, et Erik Carlsson, « Development of Right Ventricular Obstruction by Aberrant Muscular Bands », Circulation 30, n o 5 (novembre 1964): 679–85, https://doi.org/10.1161/01.CIR.30.5.679 . José María Oliver et al., « Rapid Progression of Midventricular Obstruction in Adults with Double-Chambered Right Ventricle », The Journal of Thoracic and Cardiovascular Surgery 126, n o 3 (septembre 2003): 711–17, https://doi.org/10.1016/S0022-5223(03)00044-8 . O. Galal et al., « Double-Chambered Right Ventricle in 73 Patients: Spectrum of the Disease and Surgical Results of Transatrial Repair », The Canadian Journal of Cardiology 16, n o 2 (février 2000): 167–74. D Matina et al., « Subxiphoid Two-Dimensional Echocardiographic Diagnosis of Double-Chambered Right Ventricle. », Circulation 67, n o 4 (avril 1983): 885–88, https://doi.org/10.1161/01.CIR.67.4.885 . M D Li, J C Coles, et A C McDonald, « Anomalous Muscle Bundle of the Right Ventricle. Its Recognition and Surgical Treatment. », Heart 40, n o 9 (1 septembre 1978): 1040–45, https://doi.org/10.1136/hrt.40.9.1040 . Ke Fellows, Ec Martin, et A Rosenthal, « Angiocardiography of Obstructing Muscular Bands of the Right Ventricle », American Journal of Roentgenology 128, n o 2 (1 février 1977): 249–56, https://doi.org/10.2214/ajr.128.2.249 . B.A. Animasahun, E.N. Ekure, et O.F. Njokanma, « Double-chambered right ventricle: an uncommon congenital heart disease. Case report and literature review », CardioVascular Journal of Africa 22, n o 5 (10 octobre 2011): 274–77, https://doi.org/10.5830/CVJA-2010-068 . Rei-Yeuh Chang et al., « Transesophageal Echocardiographic Image of Double-Chambered Right Ventricle », Journal of the American Society of Echocardiography 9, n o 3 (mai 1996): 347–52, https://doi.org/10.1016/S0894-7317(96)90151-0 . P. J. Kilner et al., « Recommendations for Cardiovascular Magnetic Resonance in Adults with Congenital Heart Disease from the Respective Working Groups of the European Society of Cardiology », European Heart Journal 31, n o 7 (1 avril 2010): 794–805, https://doi.org/10.1093/eurheartj/ehp586 . Tareq Ibrahim et al., « Assessment of Double Chamber Right Ventricle by Magnetic Resonance Imaging », Circulation 105, n o 22 (4 juin 2002): 2692–93, https://doi.org/10.1161/01.CIR.0000013206.40857.B6 . Mitsugi Nagashima et al., « Double-Chambered Right Ventricle in Adulthood », Asian Cardiovascular and Thoracic Annals 13, n o 2 (juin 2005): 127–30, https://doi.org/10.1177/021849230501300206 . Yoshikazu Hachiro et al., « Repair of Double-Chambered Right Ventricle: Surgical Results and Long-Term Follow-Up », The Annals of Thoracic Surgery 72, n o 5 (novembre 2001): 1520–22, https://doi.org/10.1016/S0003-4975(01)02982-4 . Miguel Álvarez et al., « Sustained Monomorphic Ventricular Tachycardia Associated with Unrepaired Double-Chambered Right Ventricle », EP Europace 8, n o 10 (1 octobre 2006): 901–3, https://doi.org/10.1093/europace/eul084 . Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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-5595617","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":393843655,"identity":"275cd218-492c-4e13-9c2f-51338686b44c","order_by":0,"name":"Taha El Khazzar","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABDUlEQVRIiWNgGAWjYFACHhBhYWAA4dnARPBpACuQgGlJI13LYcJa7NnPHvzAUCFhbM5+/PGHn3vOJ86fBhKpsWPQbT+A3RaevGQJhjMSZpY9CQmGPc9uJ264DRI5lsxgdiYBh8NyDCQY2yRsDA4kHEjgOQDUIg0UYWA7wGB2AIcW/jfGPxj/AbWcf9hw8M+Bc4nzZ+cY/2D4B9Ry/gF2LRI5ZhKMDRJmBjeSGZt5DhxIbLgNEmkDarmBw5Ybb8wsEo5JGBvceMbMLHMg2RjolzSLxL5kHrMb2G1h788xvvGhxsZww/n0xx/fHLCTnT879/CND9/s5MzOY7cFDDClEgjGzigYBaNgFIwCfAAAWPBgS7OhpeIAAAAASUVORK5CYII=","orcid":"","institution":"Cardiovascular surgery department,University Hospital Mohamed VI of Tangier","correspondingAuthor":true,"prefix":"","firstName":"Taha","middleName":"El","lastName":"Khazzar","suffix":""},{"id":393843656,"identity":"556f27c1-fc2d-4054-8ad0-f4f7ce801acf","order_by":1,"name":"Kaoutar Benjaout","email":"","orcid":"","institution":"Cardiovascular surgery department,University Hospital Mohamed VI of Tangier","correspondingAuthor":false,"prefix":"","firstName":"Kaoutar","middleName":"","lastName":"Benjaout","suffix":""},{"id":393843657,"identity":"2915e93d-1fac-477c-b3aa-3ae328c4c11f","order_by":2,"name":"Mouhcine Madani","email":"","orcid":"","institution":"Cardiovascular surgery department,University Hospital Mohamed VI of Tangier","correspondingAuthor":false,"prefix":"","firstName":"Mouhcine","middleName":"","lastName":"Madani","suffix":""}],"badges":[],"createdAt":"2024-12-06 18:38:17","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-5595617/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-5595617/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":72376626,"identity":"f910b220-40b5-47c6-8584-1e4b46f2f6c5","added_by":"auto","created_at":"2024-12-26 08:24:47","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":1114328,"visible":true,"origin":"","legend":"\u003cp\u003eDifferent stages of the surgical intervention under cardiopulmonary bypass after aortic clamping and myocardial protection.\u003cstrong\u003e A\u003c/strong\u003e: Transverse infundibulotomy, showing muscular hypertrophy and associated fibrous stenosis (white membranes); \u003cstrong\u003eB\u003c/strong\u003e: Resection of the fibromuscular stenosis; \u003cstrong\u003eC\u003c/strong\u003eand \u003cstrong\u003eD\u003c/strong\u003e: Closure of the infundibulotomy using an autologous pericardial patch.\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-5595617/v1/d21f511bd027d745ca376139.png"},{"id":72377915,"identity":"d868ab42-230d-4789-b1fe-5dda2be299ef","added_by":"auto","created_at":"2024-12-26 08:40:51","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1688350,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-5595617/v1/e85a7559-d285-478f-ab50-6686a77d4f47.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Double chambered right ventricle in adulthood: A case report and review of the literature","fulltext":[{"header":"Background","content":"\u003cp\u003eThe double chambered right ventricle (DCRV) is a rare congenital malformation. According to the literature, there are two etiological hypotheses: the presence of a hypertrophied muscular band in an abnormal location or sub-infundibular hypertrophy of abnormal muscular tissue with varying degrees of obstruction.\u003csup\u003e\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e\u003c/sup\u003e This results in a proximal chamber with high pressure and a distal chamber with low pressure. In adults, it is associated with a ventricular septal defect (VSD) in 3\u0026ndash;10% of cases.\u003csup\u003e\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e,\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e\u003c/sup\u003e Surgery is the only treatment is also recommended in the early stages, even for asymptomatic patients, to prevent prolonged exposure of the right ventricle to high pressure.\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cp\u003eA 27-year-old female patient with no notable medical history presented to the emergency department with progressively worsening NYHA class II dyspnea.\u003c/p\u003e \u003cp\u003eThe clinical examination revealed no anomalies apart from a left parasternal systolic murmur, and the electrocardiogram showed right ventricular hypertrophy (RVH).\u003c/p\u003e \u003cp\u003eLaboratory investigations revealed no abnormalities, transthoracic echocardiography revealed a right ventricle at the upper limit of size, with hypertrophied walls, a very tight mid-ventricular stenosis with a maximal gradient of 97 mmHg, and an infundibular ventricular septal defect measuring 5mm shunting from left to right.\u003c/p\u003e \u003cp\u003eThe surgical intervention was performed through a vertical median sternotomy under cardiopulmonary bypass with aortic clamping. The cardiac incision consisted of a vertical infundibulotomy, allowing exposure to the mid-ventricular stenosis. The transverse muscular bands were resected, and the infundibular stenosis was relieved. The infundibulotomy was subsequently closed using an autologous pericardial patch [Figure \u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe postoperative course was favorable, with the resolution of symptoms and a good result on follow-up echocardiography.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003ePeacock described the first examples of the muscular division of the right ventricle in 1867. Later, Keith (1909) and Brock (1957) described cases of the extension of muscular bands towards the apex of the ventricle and muscular diaphragms surrounding the base of the infundibulum, respectively.\u003csup\u003e\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e\u003c/sup\u003e Subsequently, Tsifuts et al. and Lucas et al. considered that the displacement of the septal origin of the moderator band produced an abnormal muscular band, leading to DCRV.\u003c/p\u003e \u003cp\u003eThe right ventricle consists of an inlet chamber, apical trabercular, and outlet.\u003csup\u003e\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e\u003c/sup\u003e The apex of the right ventricle is characterized by a rich set of trabecular muscles, forming three bands, with the most important being the moderator band that connects the free wall to the interventricular septum. \u003csup\u003e\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e,\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e\u003c/sup\u003e\u003c/p\u003e \u003cp\u003eOne of the etiological mechanisms is the mispositioning of the septal insertion of the moderator band at birth, especially in association with a VSD.\u003csup\u003e\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e\u003c/sup\u003e According to Thomas M et al., DCRV constitutes the only sub-infundibular form of congenital heart disease causing obstruction of the right ventricular outflow tract.\u003csup\u003e\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e\u003c/sup\u003e Other forms may cause turbulence in this tract, thus promoting the proliferation of septo-marginal trabeculations and the mispositioned moderator band's hypertrophy. Two classifications have been proposed: Folger described two abnormal positions of the muscular band, high (or horizontal) and low (or oblique).\u003csup\u003e\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e\u003c/sup\u003e\u003c/p\u003e \u003cp\u003eGaliuto distinguished two types of intracavitary obstruction: type 1 is characterized by the presence of an abnormal muscular band, while in type 2, the obstruction is caused by the hypertrophy of septal and parietal muscles, often associated with a VSD.\u003csup\u003e\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e\u003c/sup\u003e Other associated anomalies have been reported, such as pulmonary stenosis, atrial septal defect (ASD), aortic regurgitation, tricuspid regurgitation, sinus of Valsalva aneurysm, Tetralogy of Fallot, complete or corrected transposition of the great vessels, and Ebstein's anomaly.\u003csup\u003e\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e\u003c/sup\u003e\u003c/p\u003e \u003cp\u003eIn adults, the most common symptoms are exertional dyspnea and exercise intolerance, likely related to low cardiac output or right ventricular dysfunction. Some patients have presented syncope or near-fainting due to tight infundibular stenosis causing disrupted blood flow.\u003csup\u003e\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e\u003c/sup\u003e Cases of fever and lethargy associated with endocarditis have also been reported.\u003csup\u003e\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e\u003c/sup\u003e\u003c/p\u003e \u003cp\u003eMany studies have established the rapid progression of obstruction in this condition in the pediatric population.\u003csup\u003e\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e,\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e,\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e\u003c/sup\u003eOliver et al. reported that DCRV can progress more rapidly in adults and established a significant relationship between age and pressure gradient on Doppler.\u003csup\u003e\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e\u003c/sup\u003e\u003c/p\u003e \u003cp\u003eThe diagnosis of DCRV is primarily made in childhood. In adulthood, diagnosis is challenging and sometimes missed.\u003csup\u003e\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e,\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e,\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e\u003c/sup\u003e Cardiac catheterization was the diagnostic method of choice for this malformation.\u003csup\u003e\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e,\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e\u003c/sup\u003e More recently, it has been recommended in cases of suspected significant intraventricular gradient.\u003csup\u003e\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e\u003c/sup\u003e\u003c/p\u003e \u003cp\u003eLascano et al. reported discrepancies in the results obtained by transthoracic echocardiography (TTE) compared to transesophageal echocardiography (TEE) in the diagnosis. TTE has limitations due to the retrosternal location and irregular shape of the right ventricle, making it difficult to visualize, especially in adults.\u003csup\u003e\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e\u003c/sup\u003e Our patient's diagnosis was delayed due to the operator-dependent nature of transthoracic echocardiography (TTE) and its associated limitations.\u003c/p\u003e \u003cp\u003eTEE allows better visualization of the right ventricle to distinguish the abnormal muscular band and measure the pressure gradient between the two chambers.\u003csup\u003e\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e\u003c/sup\u003e\u003c/p\u003e \u003cp\u003eCardiac magnetic resonance imaging (MRI), thanks to acoustic windows and the choice of acquisition planes, is increasingly used in diagnosing congenital heart diseases in adults.\u003csup\u003e\u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e\u003c/sup\u003e It allows precise characterization of DCRV in patients who are difficult to evaluate by echocardiography.\u003csup\u003e\u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e\u003c/sup\u003e\u003c/p\u003e \u003cp\u003eSurgical treatment is indicated when the gradient between the two chambers exceeds 40 mmHg (even in the absence of symptoms) or in cases of association with a VSD with heart failure symptoms. Resection can be performed through a transatrial, transventricular, or combined approach (transatrial and transpulmonary).\u003csup\u003e\u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e\u003c/sup\u003e Ventriculotomy is often necessary in adults as they present at advanced stages of obstruction due to the rapid progression of this condition. It allows for complete resection of the obstruction.\u003csup\u003e\u003cspan citationid=\"CR28\" class=\"CitationRef\"\u003e28\u003c/span\u003e\u003c/sup\u003e The progression of pressure overload in the proximal right ventricular chamber and structural abnormalities related to hypertrophy of the moderator band promote the development of ventricular arrhythmias.\u003csup\u003e\u003cspan citationid=\"CR29\" class=\"CitationRef\"\u003e29\u003c/span\u003e\u003c/sup\u003e\u003c/p\u003e \u003cp\u003eTherefore, avoiding ventriculotomy as a means of preventing this complication is not sufficient because extensive resection is required to relieve the obstruction.\u003csup\u003e\u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e\u003c/sup\u003e\u003c/p\u003e"},{"header":"Conclusions","content":"\u003cp\u003eDouble-chambered right ventricle (DCRV) is a rare congenital condition often missed in adults due to subtle presentation and imaging limitations. Advanced diagnostics like TEE and cardiac MRI are crucial for accurate identification, and timely surgical intervention is essential to prevent complications and ensure complete obstruction relief.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eDCRV\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eDouble chambered right ventricle\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eVSD\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eVentricular septal defect\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eRHV\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eRight ventricular hypertrophy\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eASD\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eAtrial septal defect\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 echocardiography\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eTEE\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eTransesophageal echocardiography\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eMRI\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eMagnetic resonance imaging\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003c/div\u003e"},{"header":"Declarations","content":"\u003ch2\u003eEthics approval and consent to participate\u003c/h2\u003e \u003cp\u003eNot applicable\u003c/p\u003e \u003ch2\u003eConsent for publication\u003c/strong\u003e \u003cp\u003eYes. Written and informed consent to publish information is obtained from the patient\u003c/p\u003e\u003ch2\u003eFunding\u003c/h2\u003e \u003cp\u003eNone\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eThe first ET and second BK authors were responsible for manuscript\u0026rsquo;s writing, literature review, and editing. MM was responsible for literature review and editing. The final paper has been reviewed and approved by all authors.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eAleksandra Nikolic et al., \u0026laquo; An (In)Significant Ventricular Septal Defect and/or Double-Chambered Right Ventricle: Are There Any Differences in Diagnosis and Prognosis in Adult Patients? \u0026raquo;, \u003cem\u003eCardiology\u003c/em\u003e 134, n\u003csup\u003eo\u003c/sup\u003e 3 (2016): 375\u0026ndash;80, \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1159/000444743\u003c/span\u003e\u003cspan address=\"10.1159/000444743\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eMartin E. 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It is recommended to use echocardiography and cardiac catheterization for diagnosis. This case report seeks to provide additional data for comparison with existing literature, thereby contributing to a more comprehensive understanding of this condition.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCase presentation:\u003c/strong\u003e We are reporting the case of DCRV in an adult female, who was evaluated for shortness of breath. Diagnosis was confirmed by transthoracic echocardiography, with a maximal gradient of 97mmHg. The patient underwent a successful surgery.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusions:\u003c/strong\u003e Most cases of classic DCRV are diagnosed in infant, and are associated with ventricular septal defect (VSD). Surgery is the most effective treatment by removing the muscular band.\u003c/p\u003e","manuscriptTitle":"Double chambered right ventricle in adulthood: A case report and review of the literature","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-12-26 08:24:42","doi":"10.21203/rs.3.rs-5595617/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":"d4ff655a-2238-45cc-840a-6a6487f7e922","owner":[],"postedDate":"December 26th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-12-26T08:24:45+00:00","versionOfRecord":[],"versionCreatedAt":"2024-12-26 08:24:42","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-5595617","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-5595617","identity":"rs-5595617","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}