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Mitral Valve Congenital Abnormalities and Stenosis

  • Hani Mahmoud-ElsayedEmail author
Chapter

Abstract

Three-dimensional echocardiography (3DE) has tremendously improved our ability to diagnose and assess the severity of mitral valve congenital abnormalities such as congenital mitral cleft, congenital double orifice mitral valve and parachute mitral valve.

Rheumatic mitral valve stenosis is still a high-prevalent heart valve disease in many countries and degenerative, calcific, mitral stenosis is an emerging heart valve disease in the elderly. 3DE has become the reference imaging modality to assess the mitral valve stenosis qualitatively in terms of leaflet visualization, quantitation of the reduced diastolic opening, commissural calcification, leaflet mobility/pliability and assessment of the subvalvular apparatus. Moreover, 3DE provides accurate quantitative analysis of mitral stenosis severity and of mitral valve scoring system in order to assess the suitability for percutaneous mitral balloon valvuloplasty.

Finally, transesophageal 3DE is used to guide and assess the results of percutaneous mitral balloon valvuloplasty in the catheterization laboratory.

Keywords

Congenital mitral valve abnormalities Cleft mitral valve Double orifice mitral valve Parachute mitral valve Rheumatic mitral stenosis Commissural fusion Commissural calcification Degenerative calcific mitral stenosis Percutaneous mitral balloon valvuloplasty Septal puncture RATLe-90 maneuver 

Supplementary material

Video 8.1

Transesophageal 3DE zoom acquisition of the mitral valve which was cropped to show the mitral valve from both the left atrial (left panel) and the left ventricular (right panel) perspectives. The posterior mitral leaflet is divided into four distinct scallops (P1, P2, P3 and P4) by three cleft-like indentations (WMV 896 kb)

Video 8.2a

(Left) Transversal cut plane of a transthoracic 3DE full volume acquisition showing the mitral valve from the ventricular perspective and the accessory mitral valve tissue attached to the boby of anterior mitral valve leaflet (AVI 16104 kb)

Video 8.2b

(Right) Longitudinal cut plane of a transthoracic 3DE full volume acquisition showing the motion of the accessory mitral valve tissue in the left ventricular outflow tract (AVI 13851 kb)

Video 8.3a

(Left) Transthoracic 3DE visualization of the mitral valve from the left ventricular perspective showing a cleft of the anterior mitral leaflet in a patient with ostium primum atrial septal defect (AVI 2463 kb)

Video 8.3b

(Right) Transthoracic 3DE visualization of the mitral valve from the left atrial perspective showing a cleft of the anterior mitral leaflet in a patient with ostium primum atrial septal defect (AVI 2838 kb)

Video 8.4

Transesophageal 3DE visualization of the mitral valve from the atrial perspective showing a cleft which splits the posterior leaflet in two parts (AVI 10059 kb)

Video 8.5a

(Left) Transthoracic 3DE visualization of the mitral valve from the ventricular perspective clearly showing the two orifices with a central bridge of abnormal tissue connecting the anterior with the posterior leaflet (AVI 7887 kb)

Video 8.5b

(Right) Transthoracic 3DE visualization of the mitral valve from the atrial perspective clearly showing the two orifices with a central bridge of abnormal tissue connecting the anterior with the posterior leaflet (AVI 8514 kb)

Video 8.6a

(Left) Transthoracic 3DE visualization of a parachute mitral valve. A longitudinal cut plane shows a fused papillary muscle. All chordae tendineae are short and thickened and are inserted into this single papillary muscle (AVI 4089 kb)

Video 8.6b

(Right) En face view of a parachute mitral valve from the ventricular perspective (showing thickened valve leaflets with reduced diastolic excursion (AVI 5145 kb)

Video 8.7a

(Left) En face view of the stenotic mitral valve orifice as it appears from the left ventricular perspective. The doming of the anterior mitral leaflet is visualized (AVI 16291 kb)

Video 8.7b

(Right) Longitudinal cut plane of a full-volume 3DE transthoracic data set showing the various lesions of the mitral valve apparatus occurring in rheumatic mitral stenosis (reduced excursion of thickened leaflets, shortened and thickened chordae tendineae; enlarged left atrium) (AVI 20406 kb)

Video 8.8a

(Left) Transesophageal 3DE visualization of the stenotic mitral valve orifice as it appears from the left ventricular perspective. There is extensive fusion of the commissures (AVI 15048 kb)

Video 8.8b

(Right) Longitudinal cut plane obtained from a transesophageal full-volume 3DE data set showing the thickened leaflets and the anatomical relationships of the stenotic valve with the surrounding cardiac structures (AVI 12855 kb)

Video 8.9a

(Left) Thickness of the leaflets of a stenotic mitral valve as they are visualized with conventional two-dimensional echocardiography (AVI 14358 kb)

Video 8.9b

(Right) Thickness of the leaflets of a stenotic mitral valve as they are visualized with volume rendered three-dimensional echocardiography (AVI 10842 kb)

Video 8.10

Transesophageal 3DE data set of a stenotic rheumatic mitral valve displayed in an en face view from the ventricular perspective using volume rendering and rotate display modalities to visualize both the posteromedial and the anterolateral commissure (AVI 4529 kb)

Video 8.11

Transesophageal 3DE showing a stenotic rheumatic mitral valve in an en face view from the ventricular perspective. The commissures are partially fused with the medial commissure being less involved than the lateral one (AVI 31806 kb)

Video 8.12

Transesophageal 3DE showing a stenotic rheumatic mitral valve with complete fusion of bot commissures in an en face view from ventricular perspective (AVI 15093 kb)

Video 8.13

Transthoracic 3DE obtained from the parasternal approach in a patient with severe mitral stenosis. The longitudinal cut plane shows the extent of the involvement of the subvalvular apparatus with thickened, fused and shortened chordae tendineae (AVI 17173 kb)

Video 8.14

En face view of a stenotic mitral valve obtained from a full volume transthoracic 3DE data set showing that the orifice is clearly delimited by leaflet margins that are at different depth in the data set making unreliable a planimetry of the residual orifice area on the volume rendered images since planimetry will occur on a flat plane that cannot account for depth differences (AVI 21806 kb)

Video 8.15a

(Upper) Transesophageal two-dimensional long axis view showing the extensive calcification of the annulus involving the basal two thirds of the mitral leaflets in a patients with degenerative mitral stenosis (AVI 2185 kb)

Video 8.15b

(Left) Transesophageal 3DE en face view of the mitral valve from the atrial perspective showing the massive calcifications of the annulus narrowing the mitral valve orifice in a patients with degenerative mitral stenosis (AVI 2940 kb)

Video 8.15c

(Right) Transesophageal 3DE en face view of the mitral valve from the ventricular perspective showing the normal opening of both the commissures in a patients with degenerative mitral stenosis (AVI 2855 kb)

Video 8.16

RATLe-90 maneuver; starting by two-dimensional, mid-esophageal 90° bicaval view, activate the 3D-zoom mode. The zoom box should be optimized to include the openings of the superior vena cava (SVC), inferior vena cava (IVC), and aortic root (Ao), with enough depth to include the whole interatrial septum (IAS) from both atrial perspectives excluding extra atrial tissues. Then, acquiring this volume will give us a truncated volume with the SVC (arrow head) pointing to the right of the screen, IVC pointing to the left, left atrial perspective of the IAS is up, and right atrial perspective is down. Then we will rotate anticlockwise this volume for 90° around the z-axis to have the SVC pointing superiorly (arrow head) and the IVC pointing inferiorly. Then we will Tilt-Left this volume for 90° around the y-axis to have the anatomically oriented en face view of the IAS from the right atrial (RA) perspective. Then, reducing the gain will remove the blood signals and will allow clear identification of the right atrial structures, for example, SVC opening, IVC opening, Eustachian valve (EV), coronary sinus (CS) opening, and aortic root (Ao). Further gain reduction will cause dropout artifact in the thin area of the fossa ovalis (arrow head) that will help determining its location to guide septal puncture (MOV 4950 kb)

Video 8.17

Transesophageal 3DE. Reducing the gain will remove the noise and will allow clear identification of the right atrial structures, for example, superior (SVC) and inferior (IVC) vena cava opening, Eustachian valve (EV), coronary sinus (CS) opening, and aortic root (Ao). Further gain reduction will cause dropout artifact in the thin area of the fossa ovalis (arrow head) that will help determining its location to guide septal puncture (MOV 4273 kb)

Video 8.18

Transesophageal 3DE anatomically oriented view of the interatrial septum (IAS) from the right atrial perspective during guidance of transseptal puncture. By tilting the data set to the left along the Y axis, the catheter could be clearly appreciated from the left atrial side (WMV 2247 kb)

Video 8.19

Transesophageal 3DE (zoom mode) anatomically oriented view of the mitral valve from the atrial perspective showing the positioning of the balloon which crosses crossing the stenosed valve and it is inflated to dilate the valve (WMV 2122 kb)

Video 8.20

Live/real-time transesophageal 3DE during balloon inflation. The simultaneous visualization of two orthogonal two-dimensional views (four-chamber, upper left panel, and bicommissural, lower left panel) together with the volume rendered en face view from the left atrium allows the full control of balloon positioning during inflation (AVI 16345 kb)

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.CardioVascular Imaging/Cardiology DepartmentQueen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation TrustBirminghamUK

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