An accessory chord in a wrong place

Abstract

A 70-year-old male patient was assessed in the outpatient cardiology clinic with a 1 month history of progressively worsening dyspnoea. The electrocardiogram showed atrial fibrillation of uncertain onset. A transthoracic echocardiogram was performed, showing preserved left ventricular ejection fraction and mild mitral insufficiency. A rhythm control strategy was adopted and a transoesophageal echocardiogram was performed to exclude intracardiac thrombus. The transoesophageal echocardiogram did not reveal any evidence of a thrombus in the left atrium or in the atrial appendage, but revealed rheumatic mitral valve disease, without significant stenosis and with mild regurgitation ( Figure 1 ). The aortic valve appeared thickened and with reduced leaflet motion, also suggesting rheumatic involvement. Figure 1 Two-dimensional transoesophageal echocardiogram showing a rheumatic mitral valve with mild regurgitation. In the two-dimensional echocardiogram, we noticed a dot-shaped image at 45° that caught our attention ( Figure 2 ), so we performed a three-dimensional echocardiogram in which we visualized at 0° a linear, fibrous image that ran from the P2 scallop of the posterior mitral leaflet to the roof of the left atrium, causing tenting of the posterior mitral leaflet, and suggesting as an initial diagnostic possibility an accessory left atrial chord ( Figure 3 ). Figure 2 Two-dimensional transoesophageal echocardiogram at 45° showing a punctiform image (arrow). Figure 3 Three-dimensional transoesophageal echocardiogram at 0° showing a lineal, fibrous image that run from the P2 scallop of the posterior mitral leaflet to the roof of the left atrium (arrows), causing tenting of the posterior mitral leaflet (*). Over the years, accessory left atrial chords have been described as an extremely rare cause of severe mitral regurgitation and valvular cardiomyopathy due to mitral valve prolapse, especially in young patients. 1 , 2 The identification and recognition of these structures is important since valve repair (atrial chord and prolapsing tissue resection) is usually feasible and effective in these cases, avoiding prosthetic replacement. 2 Our case has some peculiarities. As far as we know, coexistence with rheumatic valvular involvement has not been previously described. We hypothesize that rheumatic involvement of the mitral valve, with the characteristic thickening of valvular leaflets and restricted mobility of the posterior mitral leaflet, prevented the accessory chord from producing higher tenting of the posterior mitral leaflet, avoiding the appearance of severe insufficiency. There are also some cases described in the literature with mild mitral regurgitation, but most of them refer to a mobile cord, not tense as in our case, which could support our hypothesis. 3 Another possible explanation for the mild mitral insufficiency could be the insertion point of the chord (closer to the base of the leaflet or closer to the tip). Supplementary material Supplementary material is available at European Heart Journal - Case Reports online. Slide sets: A fully edited slide set detailing this case and suitable for local presentation is available online as Supplementary data. Consent: The author/s confirm that written consent for submission and publication of this case report including image(s) and associated text has been obtained from the patient in line with COPE guidance. Conflict of interest: none declared. Supplementary Material ytz229_Supplementary_Data Click here for additional data file.