Aberrant tendinous chords with tethering of the tricuspid leaflets: a congenital anomaly causing severe tricuspid regurgitation

Abstract

Objective: To define the entity of tricuspid regurgitation caused by tethering of the tricuspid valve leaflets by aberrant tendinous chords. Design: Retrospective study. Setting: Tertiary care centre (university teaching hospital). Patients: 10 patients with unexplained severe tricuspid regurgitation. Methods: The last 13 500 echocardiographic studies from our facility were reviewed to identify patients with severe unexplained tricuspid regurgitation. Tethering was defined by the presence of aberrant tendinous chords to the tricuspid valve leaflets limiting the mobility of the tricuspid leaflet and resulting in incomplete coaptation and apical displacement of the regurgitant jet origin. Aberrant tendinous chords were defined as those inserting at the clear zone of the tricuspid leaflet and not originating from the papillary muscle. Patients fulfilling the diagnostic criteria for Ebstein's anomaly were excluded. Results: 10 patients with aberrant tendinous chords tethering one or more tricuspid valve leaflets were identified. There were short non-aberrant tendinous chords in seven patients, five of whom also had right ventricular or tricuspid annulus dilatation. Conclusions: Tethering of the tricuspid valve leaflets by aberrant tendinous chords can be the sole mechanism of congenital tricuspid regurgitation. It is often associated with short non-aberrant tendinous chords, which may develop secondary to right ventricular or tricuspid annulus dilatation. Awareness of tethering as a cause of tricuspid regurgitation may be important in planning reconstructive surgery.

Comparison of the offset distance of the tricuspid septal leaflet in neonates with Ebstein’s anomaly and neonates with pulmonary atresia with intact ventricular septum

“…A prominent apical displacement of the septal leaflet of the tricuspid valve from the atrioventricular junction is an intrinsic morphologic feature of Ebstein's anomaly. An indexed offset distance of the tricuspid septal leaflet ⩾8 mm/m 2 has been used or suggested as a diagnostic criterion of Ebstein's anomaly in multiple studies and reviews [10][11][12][13][14][15][16][17] after it was first reported by Shiina et al in 1984, 18 although an overlap of the minimal septal offset distance between the cases with and without Ebstein's anomaly was reported in the same year by other authors. 19 To the best of our knowledge, this criterion has not yet been reviewed comprehensively in neonatal populations.…”

Congenital tricuspid regurgitation: review and a proposed new classification

“…The chest radiograph shows right atrial and right ventricular enlargement and normal pulmonary vascularity. 1,11,17,[29][30][31][32][33][34][35][36][37][38][39][40] An electrocardiograph usually shows right axis deviation, right atrial enlargement, right bundle branch block, and first degree atrioventricular block, 1,27,29 and sometimes prolonged corrected QT interval. 29 There may be sinus tachycardia, supraventricular extrasystoles, and atrial fibrillation.…”

“…29 There may be sinus tachycardia, supraventricular extrasystoles, and atrial fibrillation. 11,17,[29][30][31][32][33][34][35][36][37][38][39][40] Cardiac catheterisation reveals elevated right atrial pressure, a pronounced ''V'' wave of tricuspid regurgitation, normal right ventricular pressure, and sometimes a right-to-left shunt at the atrial level. 1,[10][11][12][13][14][15][33][34][35][36][37][38] However, cardiac catheterisation is not required to make a diagnosis in the present day.…”

Artificial chordae for pediatric mitral and tricuspid valve repair☆

“…A few cases have been reported, where artificial chords were inserted because of leaflet tethering due to short chordae or a total absence of chordae [10][11][12][13][14].…”