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Image focus
IMAGE FOCUS
doi:10.1093/ehjci/jeu125 Online publish-ahead-of-print 1 July 2014
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Congenital isolated cleft of the tricuspid valve Pierre-Emmanuel Se´gue´la*, Franc¸ois Roubertie, Nadir Tafer, Jean-Benoit Thambo, and Xavier Iriart Pediatric Cardiology Unit, Hoˆpital Haut Le´ve`que, Bordeaux University Hospital, Avenue de Magellan, 33604 Pessac cedex, France
* Corresponding author. Tel: +33 557656110; Fax: +33 557656828, E-mail: [email protected]
Supplementary data are available at European Heart Journal – Cardiovascular Imaging online. Conflict of interest: none declared. Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected].
Downloaded from http://ehjcimaging.oxfordjournals.org/ at University of Lethbridge on November 14, 2015
An asymptomatic 8-year-old boy was addressed to our echolab for the exploration of a systolic cardiac murmur. ECG demonstrated a right heart volume overload and a right bundle branch block. Chest X-ray showed a slight enlargement of right-sided heart chambers. TTE and TEE showed a prolapse of the anterior leaflet of the tricuspid valve (TV; Panels A and B; see Supplementary data online, Video S1). Because of a lack of coaptation, this prolapse was responsible for a severe tricuspid insufficiency (Panels C and D). The width of the vena contracta was estimated at 7.5 mm and the tricuspid annulus was enlarged (2.95 Z-score). Threedimensional (3D) TEE allowed a better anatomical description by clearly showing a cleft of the anterior leaflet that was bisected in two equal parts (Panel E and see Supplementary data online, Video S2). Only the superior part of the leaflet had an excessive systolic motion, beyond the plane of the tricuspid annulus, because it was not supported by any tendinous chords (Panel F and see Supplementary data online, Video S3). As the other leaflets, the inferior part of the anterior leaflet had a normal subvalvular apparatus. Surgical repair, with direct suturing of the cleft edges and tricuspid annuloplasty, was successfully performed. Whereas cleft of the mitral valve is a wellknown congenital heart defect, cleft of the TV is a rare echocardiographic finding that has been reported only a few times, mostly in isolation. Although pathogenesis is still unknown, an abnormality in the development of the endocardial cushions is suspected. This case shows the incremental value of 3D TEE to accurately depict the TV morphology. Panel A. 2D TTE, in the apical four-chamber view, showing the prolapse of the anterior leaflet of the TV (white arrow). Panel B. Mid-oesophageal, 2D TEE, systolic image in the apical four-chamber view, showing the excessive motion of the anterior leaflet of the TV, beyond the plane of the tricuspid annulus. Panel C. Mid-oesophageal, 2D TEE, in the Doppler colour mode, showing the tricuspid insufficiency. Panel D. Mid-oesophageal, 3D TEE, systolic image in the en face view of the TV (from the apex of the RV), showing the lack of coaptation of the TV (yellow arrow) and the prolapsed superior hemi-leaflet (star). Panel E. Mid-oesophageal, 3D TEE, diastolic image in the en face view of the TV, showing the cleft of the anterior TV leaflet (white arrow). Panel F. Mid-oesophageal, 3D TEE, systolic image in the surgical view of the TV (from the RA), showing the superior part of the anterior TV leaflet bulging into the RA (star). AL, anterior leaflet; IL, inferior leaflet; RA, right atrium; RV: right ventricle; SL, septal leaflet; TEE, transoesophageal echocardiography; TTE, transthoracic echocardiography; TV, tricuspid valve; VS, ventricular septum.
Image focus
IMAGE FOCUS
doi:10.1093/ehjci/jeu125 Online publish-ahead-of-print 1 July 2014
.............................................................................................................................................................................
Congenital isolated cleft of the tricuspid valve Pierre-Emmanuel Se´gue´la*, Franc¸ois Roubertie, Nadir Tafer, Jean-Benoit Thambo, and Xavier Iriart Pediatric Cardiology Unit, Hoˆpital Haut Le´ve`que, Bordeaux University Hospital, Avenue de Magellan, 33604 Pessac cedex, France
* Corresponding author. Tel: +33 557656110; Fax: +33 557656828, E-mail: [email protected]
Supplementary data are available at European Heart Journal – Cardiovascular Imaging online. Conflict of interest: none declared. Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected].
Downloaded from http://ehjcimaging.oxfordjournals.org/ at University of Lethbridge on November 14, 2015
An asymptomatic 8-year-old boy was addressed to our echolab for the exploration of a systolic cardiac murmur. ECG demonstrated a right heart volume overload and a right bundle branch block. Chest X-ray showed a slight enlargement of right-sided heart chambers. TTE and TEE showed a prolapse of the anterior leaflet of the tricuspid valve (TV; Panels A and B; see Supplementary data online, Video S1). Because of a lack of coaptation, this prolapse was responsible for a severe tricuspid insufficiency (Panels C and D). The width of the vena contracta was estimated at 7.5 mm and the tricuspid annulus was enlarged (2.95 Z-score). Threedimensional (3D) TEE allowed a better anatomical description by clearly showing a cleft of the anterior leaflet that was bisected in two equal parts (Panel E and see Supplementary data online, Video S2). Only the superior part of the leaflet had an excessive systolic motion, beyond the plane of the tricuspid annulus, because it was not supported by any tendinous chords (Panel F and see Supplementary data online, Video S3). As the other leaflets, the inferior part of the anterior leaflet had a normal subvalvular apparatus. Surgical repair, with direct suturing of the cleft edges and tricuspid annuloplasty, was successfully performed. Whereas cleft of the mitral valve is a wellknown congenital heart defect, cleft of the TV is a rare echocardiographic finding that has been reported only a few times, mostly in isolation. Although pathogenesis is still unknown, an abnormality in the development of the endocardial cushions is suspected. This case shows the incremental value of 3D TEE to accurately depict the TV morphology. Panel A. 2D TTE, in the apical four-chamber view, showing the prolapse of the anterior leaflet of the TV (white arrow). Panel B. Mid-oesophageal, 2D TEE, systolic image in the apical four-chamber view, showing the excessive motion of the anterior leaflet of the TV, beyond the plane of the tricuspid annulus. Panel C. Mid-oesophageal, 2D TEE, in the Doppler colour mode, showing the tricuspid insufficiency. Panel D. Mid-oesophageal, 3D TEE, systolic image in the en face view of the TV (from the apex of the RV), showing the lack of coaptation of the TV (yellow arrow) and the prolapsed superior hemi-leaflet (star). Panel E. Mid-oesophageal, 3D TEE, diastolic image in the en face view of the TV, showing the cleft of the anterior TV leaflet (white arrow). Panel F. Mid-oesophageal, 3D TEE, systolic image in the surgical view of the TV (from the RA), showing the superior part of the anterior TV leaflet bulging into the RA (star). AL, anterior leaflet; IL, inferior leaflet; RA, right atrium; RV: right ventricle; SL, septal leaflet; TEE, transoesophageal echocardiography; TTE, transthoracic echocardiography; TV, tricuspid valve; VS, ventricular septum.
