© 2014, Wiley Periodicals, Inc. DOI: 10.1111/echo.12573

Echocardiography

Spontaneous Bileaflet Chordal Rupture Secondary to Myxomatous Degeneration of the Mitral Valve Lutfi Ocal, M.D.,* Macit Kalcik, M.D.,* Cuneyt Toprak, M.D.,* Alev Kılıcgedik, M.D.,* Atilla Koyuncu, M.D.,* € khan Kahveci, M.D.,* and Mehmet Ozkan, M.D.*,† Go *Department of Cardiology, Kosuyolu Kartal Heart Training and Research Hospital, Istanbul, Turkey; and †Department of Cardiology, Faculty of Medicine, Kars Kafkas University, Kars, Turkey

(Echocardiography 2014;31:E195–E196) Case Presentation: A 60-year-old man presented with 1 month history of progressive dyspnea. His blood pressure, pulse rate, and respiratory rate were 120/ 70 mmHg, 92 beats/min, and 17 breaths/min, respectively. Clinical examination revealed a 4/6 pansystolic murmur that was loudest at the apex. Electrocardiogram (Burdick 8500 ECG, Cardiac Science Corporation, Bothell, WA, USA) showed normal sinus rhythm and laboratory findings were normal. The chest radiography showed cardiomegaly. Two-dimensional transthoracic and transesophageal echocardiography (TEE) with the transducer array at 60 degrees were performed and confirmed the presence of bileaflet chordal rupture, corresponding A2 and P2 scallops (Fig. 1A, movie clip S1) and severe posteriorly

eccentric mitral regurgitation was detected by color Doppler imaging (movie clip S2). Real time three-dimensional TEE clearly delineated flail A2 and P2 scallops secondary to rupture of primary chordae (Fig. 1B, movie clip S3). The patient underwent mitral valve surgery. Myxomatous degeneration and ruptured chordae of both anterior and posterior mitral leaflets were seen macroscopically during surgery. As mitral valve repair was unsatisfactory, mitral valve replacement with a mechanical prostheses was performed. Primary myxomatous degeneration was confirmed histopathologically as degeneration of collagen of the pars fibrosa and deposition of acid mucopolysaccharides in the pars spongiosa of the valve in the absence of severe calcification or fibrosis, with no evidence of rheumatic valvular disease.

Figure 1. Two-dimensional transesophageal echocardiography (TEE) showed the presence of bileaflet chordal rupture, corresponding A2 and P2 scallops A. and real time three-dimensional TEE delineated flail A2 and P2 scallops secondary to rupture of primary chordae B. Ao = aorta; LAA = left atrial appendage; LA = left atrium; LV = left ventricle; A2 and P2 are the corresponding scallops of anterior and posterior mitral leaflet, respectively, Arrows show the ruptured chordate of flailed leaflets. € Address for correspondence and reprint requests: Lutfi Ocal, M.D., Denizer Cad. Cevizli Kvsß. No:2 Cevizli Kartal, Istanbul, Turkey. Fax: (90)216 4596321; E-mail: lutfi[email protected]

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The incidence of posterior chordal rupture is higher than that of anterior mitral leaflet.1 However, spontaneous rupture of both anterior and posterior chordae is very rare. Two-dimensional and real time three-dimensional TEE have incremental value in delineation of mitral chordal rupture.2,3 References 1. Luo XL, Qiao SB, Yuan JS, et al: Clinical and echocardiographic characteristics of mitral chordal rupture from 292 hospitalized patients. Zhonghua Xin Xue Guan Bing Za Zhi 2009;37:253–256. 2. Jayaprakash M, Saritha Kumari K, Ming Chon H, et al: Comparison of real-time two-dimensional with live/real time three dimensional transesophageal echocardiography in the evaluation of mitral valve prolapse and chordae rupture. Echocardiography 2008;25:1131–1137. 3. Sudhakar S, Khairnar P, Nanda NC: Live/real time threedimensional transesophageal echocardiography. Echocardiography 2012;29:103–111.

Supporting Information Additional Supporting Information may be found in the online version of this article:

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Movie Clip 1. Two-dimensional transesophageal echocardiography with the transducer array at 60 degrees showed the presence of bileaflet chordal rupture, corresponding A2 and P2 scallops. LA = left atrium; LV = left ventricle; A2 and P2 are the corresponding scallops of anterior and posterior mitral leaflet, respectively, and arrows show the ruptured chordate of flailed leaflets. Movie Clip 2. Two-dimensional transesophageal echocardiography with the transducer array at 60 degrees color flow imaging showed severe posteriorly eccentric mitral regurgitation due to bileaflet chordal rupture. LA = left atrium; LV = left ventricle. Movie Clip 3. Real time three-dimensional transesophageal echocardiography delineated flail A2 and P2 scallops secondary to rupture of primary chordae. AML = anterior mitral leaflet; Ao = aorta; LAA = left atrial appendage; PML = posterior mitral leaflet; the arrow shows the ruptured chordate of flailed leaflets.

Spontaneous bileaflet chordal rupture secondary to myxomatous degeneration of the mitral valve.

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