Abnormal Tricuspid Chordae Tendineae in a Patient with Congenital Pulmonary Stenosis

A 24-year-old male visited the cardiology clinic for a prerhinoplasty cardiac assessment. He had a history of open pulmonary valvotomy due to severe stenosis during infancy but reported no current cardiac issues. Electrocardiography showed nonspecific changes. Transthoracic echocardiography revealed normal left ventricular size with moderate systolic dysfunction, global hypokinesia, severe right ventricular (RV) enlargement with systolic dysfunction, severe right atrial enlargement, severe tricuspid regurgitation (TR), and severe pulmonary regurgitation. In addition, echocardiography illustrated an RV papillary muscle (RVPM) directly attached to the anterior leaflet of the tricuspid valve (TV) [Figure 1a-c and Video 1]. Cardiac magnetic resonance imaging was unable to provide a definitive judgment regarding this finding [Figure 1d-f and Video 2]. Ultimately, the patient underwent bioprosthetic pulmonary valve replacement and TV repair. The surgeon found a dilated TV annulus, normal tricuspid leaflets (TLs), a normal arrangement of RVPMs, normal attachment of RVPMs to TLs through chordae tendineae, and thickened TV chordae tendineae.Figure 1: Transthoracic echocardiography in the right ventricular (RV) inflow view (a) and modified apical four-chamber view (b and c) revealed a highly probable direct attachment of a papillary muscle to the anterior leaflet of the tricuspid valve. The steady-state free precision at the systolic time in the axial level (d) and RV inflow–outflow tract view (e) demonstrated an elongated anterior papillary muscle of the right ventricle that was thickened at the anatomic site of chordae tendineae (arrow). (f) Phase-sensitive inversion recovery sequence in the axial level showed late gadolinium enhancement (arrow), indicating fibrotic changes at the anatomic site of chordae tendineae. LV: Left ventricular, RA: Right atrial, RV: Right ventricular {"href":"Single Video Player","role":"media-player-id","content-type":"play-in-place","position":"float","orientation":"portrait","label":"Video 1.","caption":"Transthoracic echocardiography demonstrated left and RV dysfunction and severe tricuspid and pulmonary regurgitation. In addition, a highly probable direct attachment of a papillary muscle to the anterior leaflet of the TV was revealed.","object-id":[{"pub-id-type":"doi","id":""},{"pub-id-type":"other","content-type":"media-stream-id","id":"1_usbx2j7l"},{"pub-id-type":"other","content-type":"media-source","id":"Kaltura"}]} {"href":"Single Video Player","role":"media-player-id","content-type":"play-in-place","position":"float","orientation":"portrait","label":"Video 2.","caption":"The steady-state free precision sequence at the four-chamber and RV inflow–outflow view presented an elongated anterior papillary muscle of the right ventricle that was thickened at the anatomic site of the chordae tendineae.","object-id":[{"pub-id-type":"doi","id":""},{"pub-id-type":"other","content-type":"media-stream-id","id":"1_m9n97ysx"},{"pub-id-type":"other","content-type":"media-source","id":"Kaltura"}]} Typically, RVPMs attach to the TLs through chordae tendineae. However, sometimes, the RVPMs connect directly to TLs or through a membrane, as observed in nonprimates from an evolutionary standpoint.[1,2] In addition, such a connection between the RVPM and the TL has been noted in intermediate-grade tricuspid dysplasia.[2] In our patient, distinguishing between thickened chordae tendineae and a direct attachment of an RVPM to the TL was not possible through imaging studies. It appears that abnormal chordae tendineae were not the only cause of TR. Other factors, such as RV remodeling and annular dilation, contributed to the presence of severe pulmonary regurgitation, RV enlargement, and dysfunction. Nonsurgical TV interventions have recently garnered significant attention, making it more crucial than ever to pinpoint the exact cause of TR.[3] Among congenital causes of TR, the abnormal attachment of the RVPMs to the TLs should not be overlooked, and differential diagnoses such as thickened chordae tendineae should be considered. Authors’ contributions SAF, AH, KA, HG: Concept, Design, Definition of intellectual content, Literature search, Clinical studies, Data acquisition, Data analysis, Manuscript preparation, Manuscript editing and manuscript review. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.