1IASO General Hospital, Athens, Greece
2Imperial College of Science, Technology and Medicine, National Heart and Lung Institute at Heart Science Centre, Harefield, Middlesex UB9 6JH, UK
* Corresponding author. Tel: +44 1895 453893; fax: +44 1895 828902. E-mail address: m.yacoub{at}imperial.ac.uk
There is still no agreement about the need for, timing, and type of repair of the Syndrome of VSD and AR (syndrome of Laubry and Pezzi). The syndrome is commonly thought of as due to a simple prolapse of the aortic leaflet into a high VSD. We believe that the syndrome is caused by congenital discontinuity of the media of the aortic sinus from the crest of the interventricular septum. This results in aneurismal dilatation and bulging of the right coronary sinus into the RVOT, VSD, and prolapse of the aortic cusp. Images in this report illustrate all the anatomical components both before and after repair using a simple technique described previously by us.
Panel A. The discontinuity between the aortic media and the crest of the septum, the longitudinal dilatation of the sinus of Valsava, the prolapse of the cusp, and lack of coaptation of the aortic cusps. This was associated with moderate aortic regurgitation with a jet directed towards the anterior mitral leaflet (Panel C). During systole, the VSD shunt is apparent (Panel E), while in diastole the dilated sinus and prolapsing cusp obstruct the VSD (Panel C). Following simple transaortic repair, re-attachment of the crest of the septum to the aortic media results in closure of the VSD, correction of the dilated sinus, elevation of the aortic cusp with increased coaptation (Panel B), and marked improvement in AR, which become trivial (Panel D). There were no shunts detected during systole (Panel F) or diastole (Panel D).
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