caa a22 4500 388115440 CHVBK 20180307125405.0 cr unu---uuuuu 161130e199905 xx s 000 0 eng 10.1017/S1047951100004972 doi S1047951100004972 pii (NATIONALLICENCE)cambridge-10.1017/S1047951100004972 Asymmetrically short tendinous cords causing congenital tricuspid regurgitation: improved understanding of tricuspid valvar dysplasia in the era of color flow echocardiography [Elektronische Daten] Abstract Background Tricuspid regurgitation as a manifestation of an isolated congenital anomaly of the tricuspid valve is rare. Cross-sectional and color Doppler echocardiography allow improved evaluation of tricuspid valvar function. As a result, the heterogeneous category of congenital tricuspid valvar dysplasia may be better understood from a functional point of view. We are reporting a distinct entity in which tricuspid valvar regurgitation results from failure of coaptation due to short tendinous cords tethering the septal leaflet. Patients and Results Three children with significant primary tricuspid regurgitation were evaluated, treated, and followed. On echocardiographic evaluation, a central regurgitant jet of moderate or severe degree was directed toward the atrial septum through poorly coapting tricuspid valvar leaflets, which did not approximate due to tethering of the septal leaflet by abnormally short cords. In one patient, the tricuspid valve was otherwise normal; in the other two the leaflets and cords were also thickened. Two patients underwent surgery at 9 and 11 years of age. The cords tethering the septal leaflet were augmented by interposing appropriate lengths of expanded polytetrafluoroethylene suture and performing commissural annuloplasty. Both patients are asymptomatic 33 and 42 months postoperatively, with mild residual tricuspid regurgitation that has not changed since surgery. The other patient, an 8 month-old infant, has not yet undergone surgery. Conclusions Asymmetric tendinous cords of the tricuspid valve causing tethering of the septal leaflet is a distinct cause of tricuspid regurgitation that can be recognized with echocardiography. Although rare, the importance of recognizing this lesion lies in its being readily amenable to surgical repair. Copyright © Cambridge University Press 1999 Atrioventricular valve nationallicence valvar function nationallicence valvar repair nationallicence Doppler echocardiography nationallicence McElhinney Doff B. Divisions of Pediatric Cardiology and Cardiothoracic Surgery, University of California, San Francisco, San Francisco, CA, USA Silverman Norman H. Divisions of Pediatric Cardiology and Cardiothoracic Surgery, University of California, San Francisco, San Francisco, CA, USA Brook Michael M. Divisions of Pediatric Cardiology and Cardiothoracic Surgery, University of California, San Francisco, San Francisco, CA, USA Hanley Frank L. Divisions of Pediatric Cardiology and Cardiothoracic Surgery, University of California, San Francisco, San Francisco, CA, USA Stanger Paul Divisions of Pediatric Cardiology and Cardiothoracic Surgery, University of California, San Francisco, San Francisco, CA, USA Cardiology in the Young Cambridge University Press 9/3(1999-05), 300-304 1047-9511 9:3<300 1999 9 CTY https://doi.org/10.1017/S1047951100004972 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1017/S1047951100004972 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- McElhinney Doff B. Divisions of Pediatric Cardiology and Cardiothoracic Surgery, University of California, San Francisco, San Francisco, CA, USA NATIONALLICENCE 700 1- Silverman Norman H. Divisions of Pediatric Cardiology and Cardiothoracic Surgery, University of California, San Francisco, San Francisco, CA, USA NATIONALLICENCE 700 1- Brook Michael M. Divisions of Pediatric Cardiology and Cardiothoracic Surgery, University of California, San Francisco, San Francisco, CA, USA NATIONALLICENCE 700 1- Hanley Frank L. Divisions of Pediatric Cardiology and Cardiothoracic Surgery, University of California, San Francisco, San Francisco, CA, USA NATIONALLICENCE 700 1- Stanger Paul Divisions of Pediatric Cardiology and Cardiothoracic Surgery, University of California, San Francisco, San Francisco, CA, USA NATIONALLICENCE 773 0- Cardiology in the Young Cambridge University Press 9/3(1999-05), 300-304 1047-9511 9:3<300 1999 9 CTY CC0 http://creativecommons.org/publicdomain/zero/1.0 nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-cambridge