Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2021 Aug 19;3(10):1303-1309.
doi: 10.1016/j.jaccas.2021.06.013. eCollection 2021 Aug 18.

Mitral Arcade With Anomalous Papillary Muscles: Rare Cause of Mitral Stenosis in a Young Adult

Caroline Ong  1   2 , Rachel-Maria Brown  1   2 , Adam Gershon  1 , Biana Trost  1   2 , Hossein Hosseini  3 , Luigi Pirelli  2   4
Affiliations
Case Reports

Mitral Arcade With Anomalous Papillary Muscles: Rare Cause of Mitral Stenosis in a Young Adult

Caroline Ong et al. JACC Case Rep. .

Abstract

We present the case of a symptomatic young woman with mitral stenosis and regurgitation due to a congenital mitral arcade. Multimodality imaging with echocardiography and computed tomography were used for diagnosis and surgical planning. The patient underwent successful bioprosthetic valve replacement. (Level of Difficulty: Intermediate.).

Keywords: COVID-19, coronavirus disease-2019; CT, computed tomography; ECG, electrocardiogram; MVA, mitral valve area; PASP, pulmonary artery systolic pressure; PHT, pressure half-time; RBBB, right bundle branch block; TEE, transesophageal echocardiogram; TTE, transthoracic echocardiogram; computed tomography; congenital heart defect; echocardiography; imaging; mitral valve.

PubMed Disclaimer

Conflict of interest statement

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Figures

None
Graphical abstract
Figure 1
Figure 1
Admission Electrocardiogram
Figure 2
Figure 2
Chest Radiograph and Computed Tomographic View
Figure 3
Figure 3
Transmitral Gradient and M-Mode of Mitral Valve (A) Mean transmitral gradient 17 mm Hg at a heart rate of 86 beats/min. (B) M-mode of leaflet tips.
Figure 4
Figure 4
Sequential Transthoracic Echocardiogram Parasternal Short Axis and 3-Dimensionial Transesophageal Echocardiogram Reconstruction of Mitral Valve “Fish-mouth” appearance of the mitral orifice leading to short thickened chordae tendinae and superiorly displaced anterolateral papillary muscle. Mitral valve area estimated by pressure half-time 1.1 cm2 and measured 1.0 cm2 by 3-dimensional multiplanar reconstruction. Estimated regurgitant orifice area 20 cm by proximal isovelocity surface area method, regurgitant volume 39 mL, regurgitant fraction 43%.
Figure 5
Figure 5
Cardiac Computed Tomographic Short Axis Cardiac computed tomographic view short axis showing fibrous band of tissue (arrow) between the papillary muscles.
Figure 6
Figure 6
Correlation of Surgical and Computed Tomographic Reconstruction (A) Surgical view of the heads of the hypertrophied papillary muscles after resection of chords to the anterior leaflet, (B) 3-dimensional cardiac computed tomographic reconstruction.
Figure 7
Figure 7
Gross Appearance of Mitral Arcade Mitral valve after partial resection of the anterior leaflet showing thick, fibrotic, and cranially displaced papillary muscles with diminutive, thickened chordae.
Figure 8
Figure 8
Histology of Resected Mitral Valve Mitral valve with focal myxoid change and fibrosis (hematoxylin and eosin, A: magnification ×100, B: magnification ×200).
Figure 9
Figure 9
Histology of Biopsied Lung Lung with intra-alveolar hemosiderin-laden macrophages (hematoxylin and eosin, magnification ×200).
See this image and copyright information in PMC

References

    1. Hakim F.A., Krishnaswamy C., Mookadam F. Mitral arcade in adults: a systematic overview. Echocardiography. 2013;30:354–359. - PubMed
    1. Layman T.E., Edwards J.E. Anomalous mitral arcade: a type of congenital mitral insufficiency. Circulation. 1967;35:389–395. - PubMed
    1. Dokollari A., Cameli M., Bisleri G. Mitral arcades unexpectedly encountered during cardiac surgery. J Cardiothorac Vasc Anesth. 2021;35:914–916. - PubMed
    1. Collins-Nakai R.L., Rosenthal A., Castaneda A.R., Bernhard W.F., Nadas A.S. Congenital mitral stenosis: a review of 20 years' experience. Circulation. 1977;56:1039–1047. - PubMed
    1. Woolley K., Stark P. Pulmonary parenchymal manifestations of mitral valve disease. Radiographics. 1999;19:965–972. - PubMed

Publication types

LinkOut - more resources