{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,28]],"date-time":"2026-06-28T18:47:26Z","timestamp":1782672446172,"version":"3.54.5"},"reference-count":28,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,3,27]],"date-time":"2024-03-27T00:00:00Z","timestamp":1711497600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Cardiovasc. Med."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Mitral annular disjunction (MAD) refers to the arrhythmic mitral valve prolapse (MVP) syndrome associated with ventricular arrhythmias and sudden cardiac death. Although the pathophysiology of this disease is still under investigation, specific imaging criteria that establish the diagnosis have been recognized. In this article, we demonstrate most of these criteria using three-dimensional transthoracic echocardiography (3D-TTE) and provide added value in the management of MAD syndrome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case presentation<\/jats:title><jats:p>A 50-year-old male patient with recent syncope and a history of mitral regurgitation (MR) and MAD was admitted to our clinic for further investigation. According to our protocol, the patient underwent a complete 3D-TTE, laboratory blood exams, and 24\u2005h ambulatory electrocardiogram (ECG). Our investigation confirmed the presence of MAD syndrome with bileaflet prolapse, severe MR, and non-sustained ventricular tachycardia, necessitating an implantable cardioverter defibrillator (ICD) and surgical mitral valve repair. The 3D-TTE analysis of the mitral valve demonstrated mitral annular systolic expansion and systolic flattening of the saddle-shaped annulus and quantified the extent of the disjunction arc. Additionally, four-dimensional (4D) strain analysis of the left ventricle revealed the presence of fibrosis of the posteromedial papillary muscle and basal inferolateral wall, which are variables that are required for the diagnosis and therapeutic management of MAD syndrome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>3D-TTE and 4D strain offer valuable insights for diagnosing and managing patients with MAD syndrome. This method seems to correlate well with the other imaging modalities and could be included in the management protocol of MAD syndrome.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fcvm.2024.1366444","type":"journal-article","created":{"date-parts":[[2024,3,27]],"date-time":"2024-03-27T05:03:46Z","timestamp":1711515826000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["The added value of three-dimensional transthoracic echocardiography in mitral annular disjunction: a case report"],"prefix":"10.3389","volume":"11","author":[{"given":"Konstantinos","family":"Papadopoulos","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ignatios","family":"Ikonomidis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mani A.","family":"Vannan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2024,3,27]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"976066","DOI":"10.3389\/fcvm.2022.976066","article-title":"Mitral annular disjunction: a case series and review of the literature","volume":"9","author":"Wu","year":"2022","journal-title":"Front Cardiovasc Med"},{"key":"B2","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1056\/NEJM199907013410101","article-title":"Prevalence and clinical outcome of mitral-valve prolapse","volume":"341","author":"Freed","year":"1999","journal-title":"N Engl J Med"},{"key":"B3","doi-asserted-by":"publisher","first-page":"2194","DOI":"10.1093\/eurheartj\/ehz314","article-title":"Causes and mechanisms of isolated mitral regurgitation in the community: clinical context and outcome","volume":"40","author":"Dziadzko","year":"2019","journal-title":"Eur Heart J"},{"key":"B4","doi-asserted-by":"publisher","first-page":"952","DOI":"10.1161\/CIRCULATIONAHA.118.034075","article-title":"Mitral valve prolapse, ventricular arrhythmias, and sudden death","volume":"140","author":"Basso","year":"2019","journal-title":"Circulation"},{"key":"B5","doi-asserted-by":"publisher","first-page":"2904","DOI":"10.1016\/j.jacc.2018.09.048","article-title":"Arrhythmic mitral valve prolapse: JACC review topic of the week","volume":"72","author":"Miller","year":"2018","journal-title":"J Am Coll Cardiol"},{"key":"B6","doi-asserted-by":"publisher","first-page":"1424","DOI":"10.1016\/j.jcmg.2016.11.022","article-title":"Functional implication of mitral annular disjunction in mitral valve prolapse: a quantitative dynamic 3D echocardiographic study","volume":"10","author":"Lee","year":"2017","journal-title":"JACC Cardiovasc Imaging"},{"key":"B7","doi-asserted-by":"publisher","first-page":"176","DOI":"10.1016\/j.echo.2021.09.002","article-title":"Morphology of mitral annular disjunction in mitral valve prolapse","volume":"35","author":"Faletra","year":"2022","journal-title":"J Am Soc Echocardiogr"},{"key":"B8","doi-asserted-by":"publisher","first-page":"411","DOI":"10.1136\/heartjnl-2020-317451","article-title":"Parameters associated with ventricular arrhythmias in mitral valve prolapse with significant regurgitation","volume":"107","author":"van Wijngaarden","year":"2021","journal-title":"Heart"},{"key":"B9","doi-asserted-by":"publisher","first-page":"183","DOI":"10.1093\/ehjci\/jez210","article-title":"Left ventricular mechanical dispersion in a general population: data from the Akershus cardiac examination 1950 study","volume":"21","author":"Aagaard","year":"2020","journal-title":"Eur Heart J Cardiovasc Imaging"},{"key":"B10","doi-asserted-by":"publisher","first-page":"1600","DOI":"10.1016\/j.jacc.2018.07.070","article-title":"The mitral annulus disjunction arrhythmic syndrome","volume":"72","author":"Dejgaard","year":"2018","journal-title":"J Am Coll Cardiol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"1610","DOI":"10.1016\/j.jacc.2018.07.069","article-title":"Mitral annulus disjunction: emerging role of myocardial mechanical stretch in arrhythmogenesis","volume":"72","author":"Basso","year":"2018","journal-title":"J Am Coll Cardiol"},{"key":"B12","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1136\/heartjnl-2020-317330","article-title":"Multimodality imaging assessment of mitral annular disjunction in mitral valve prolapse","volume":"107","author":"Mantegazza","year":"2021","journal-title":"Heart"},{"key":"B13","doi-asserted-by":"publisher","first-page":"S183","DOI":"10.1161\/CIRCULATIONAHA.111.084483","article-title":"Three-dimensional echocardiographic analysis of mitral annular dynamics: implication for annuloplasty selection","volume":"126","author":"Levack","year":"2012","journal-title":"Circulation"},{"key":"B14","doi-asserted-by":"publisher","first-page":"614","DOI":"10.1093\/ehjci\/jeab022","article-title":"Prevalence and extent of mitral annular disjunction in structurally normal hearts: comprehensive 3D analysis using cardiac computed tomography","volume":"22","author":"Toh","year":"2021","journal-title":"Eur Heart J Cardiovasc Imaging"},{"key":"B15","doi-asserted-by":"publisher","first-page":"1981","DOI":"10.1093\/europace\/euac125","article-title":"EHRA expert consensus statement on arrhythmic mitral valve prolapse and mitral annular disjunction complex in collaboration with the ESC council on valvular heart disease and the European association of cardiovascular imaging endorsed cby the heart rhythm society, by the Asia pacific heart rhythm society, and by the Latin American heart rhythm society","volume":"24","author":"Sabbag","year":"2022","journal-title":"Europace"},{"key":"B16","doi-asserted-by":"publisher","first-page":"475","DOI":"10.1093\/ehjci\/jew284","article-title":"3D echocardiographic reference ranges for normal left ventricular volumes and strain: results from the EACVI NORRE study","volume":"18","author":"Bernard","year":"2017","journal-title":"Eur Heart J Cardiovasc Imaging"},{"key":"B17","doi-asserted-by":"publisher","first-page":"2073","DOI":"10.1016\/j.jcmg.2021.04.029","article-title":"The mitral annular disjunction of mitral valve prolapse: presentation and outcome","volume":"14","author":"Essayagh","year":"2021","journal-title":"JACC Cardiovasc Imaging"},{"key":"B18","doi-asserted-by":"publisher","first-page":"1251","DOI":"10.1016\/j.jaccas.2021.06.038","article-title":"Varied extent of mitral annular disjunction among cases with different phenotypes of mitral valve prolapse","volume":"3","author":"Toh","year":"2021","journal-title":"JACC Case Rep"},{"key":"B19","doi-asserted-by":"publisher","first-page":"21","DOI":"10.1186\/s44156-023-00032-x","article-title":"Mitral annular disjunction in surgical mitral valve prolapse: prevalence, characteristics and outcomes","volume":"10","author":"Gray","year":"2023","journal-title":"Echo Res Pract"},{"key":"B20","doi-asserted-by":"publisher","first-page":"405","DOI":"10.1016\/j.echo.2011.01.012","article-title":"Quantitative analysis of mitral valve apparatus in mitral valve prolapse before and after annuloplasty: a three-dimensional intraoperative transesophageal study","volume":"24","author":"Maffessanti","year":"2011","journal-title":"J Am Soc Echocardiogr"},{"key":"B21","doi-asserted-by":"publisher","first-page":"e005030","DOI":"10.1161\/CIRCIMAGING.116.005030","article-title":"Morphofunctional abnormalities of mitral annulus and arrhythmic mitral valve prolapse","volume":"9","author":"Perazzolo Marra","year":"2016","journal-title":"Circ Cardiovasc Imaging"},{"key":"B22","doi-asserted-by":"publisher","first-page":"1245","DOI":"10.1016\/j.echo.2020.04.030","article-title":"Utility of three-dimensional transesophageal echocardiography for mitral annular sizing in transcatheter mitral valve replacement procedures: a cardiac computed tomographic comparative study","volume":"33","author":"Coisne","year":"2020","journal-title":"J Am Soc Echocardiogr"},{"key":"B23","doi-asserted-by":"publisher","first-page":"1512","DOI":"10.1111\/echo.15481","article-title":"Level of agreement between three-dimensional transthoracic and transesophageal echocardiography for mitral annulus evaluation: a feasibility and comparison study","volume":"39","author":"Papadopoulos","year":"2022","journal-title":"Echocardiography"},{"key":"B24","doi-asserted-by":"publisher","first-page":"32","DOI":"10.1186\/s12947-016-0073-4","article-title":"Three-dimensional dynamic morphology of the mitral valve in different forms of mitral valve prolapse\u2014potential implications for annuloplasty ring selection","volume":"14","author":"Apor","year":"2016","journal-title":"Cardiovasc Ultrasound"},{"key":"B25","doi-asserted-by":"publisher","first-page":"165","DOI":"10.1016\/j.echo.2021.09.004","article-title":"Mitral annular disjunction of degenerative mitral regurgitation: three-dimensional evaluation and implications for mitral repair","volume":"35","author":"Essayagh","year":"2022","journal-title":"J Am Soc Echocardiogr"},{"key":"B26","doi-asserted-by":"publisher","first-page":"858","DOI":"10.1016\/j.echo.2014.05.010","article-title":"Left ventricular myocardial strain by three-dimensional speckle-tracking echocardiography in healthy subjects: reference values and analysis of their physiologic and technical determinants","volume":"27","author":"Muraru","year":"2014","journal-title":"J Am Soc Echocardiogr"},{"key":"B27","doi-asserted-by":"publisher","first-page":"80","DOI":"10.1016\/j.jcmg.2007.12.007","article-title":"Myocardial strain measurement with 2-dimensional speckle-tracking echocardiography: definition of normal range","volume":"2","author":"Marwick","year":"2009","journal-title":"JACC Cardiovasc Imaging"},{"key":"B28","doi-asserted-by":"publisher","first-page":"jead119.143","DOI":"10.1093\/ehjci\/jead119.143","article-title":"Fully automated 3D echocardiographic algorithms: accurate and time saving\u2014the answer for 3D in routine clinical practice?","volume":"24","author":"Miranda Castilho","year":"2023","journal-title":"Eur Heart J Cardiovasc Imaging"}],"container-title":["Frontiers in Cardiovascular Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2024.1366444\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,3,27]],"date-time":"2024-03-27T05:03:48Z","timestamp":1711515828000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2024.1366444\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3,27]]},"references-count":28,"alternative-id":["10.3389\/fcvm.2024.1366444"],"URL":"https:\/\/doi.org\/10.3389\/fcvm.2024.1366444","relation":{},"ISSN":["2297-055X"],"issn-type":[{"value":"2297-055X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,3,27]]},"article-number":"1366444"}}