Double Accessory Left Atrial Chordae Tendineae Resulting in Mitral Regurgitation

Combination of an extremely rare and a common congenital valvular anomalies: Accessory mitral valve chordae from left atrium and bicuspid aortic valve

“…As in our case, medical follow-up is appropriate in cases that accessory chordae does not cause severe valve pathology, 3 while surgery is the treatment strategy in cases that accessory chordae causes severe valve regurgitation. [4][5][6][7][8]…”

Left atrial mitral valve chordae: An important congenital abnormality mimicking endocarditis on transthoracic echocardiography—Case report

“…case reports worldwide and the clinical significance remains uncertain, ranging from incidental findings with mild-moderate mitral valve pathology managed conservatively [1][2][3][4] to severe mitral regurgitation requiring surgical intervention. [5][6][7][8][9][10][11][12][13] There has been an isolated report The origin of left atrial mitral valve chordae is proposed to be a result of a developmental defect during embryogenesis between the 14th and 17th weeks of gestation as the papillary muscles and chordae develop. 4 Left atrial mitral valve chordae have been identified in children as young as 8-years-old, 12 while one case was only diagnosed at the age of 85-years-old.…”

“…Left atrial mitral valve chordae is a rare congenital abnormality of which the prevalence is largely unknown. There have been few case reports worldwide and the clinical significance remains uncertain, ranging from incidental findings with mild‐moderate mitral valve pathology managed conservatively 1–4 to severe mitral regurgitation requiring surgical intervention 5–13 . There has been an isolated report of left atrial mitral valve chordae causing complex endocarditis 14 .…”

“…6 Other suggested mechanisms include the chord holding the free edge of the leaflet in a scallop during diastole with the atrial kicking motion resulting in the development of mitral regurgitation 7 and traumatic injury to the free edge of the leaflet resulting in tethering and restricted mobility. 10 Most cases in the literature describe involvement of the A2 leaflet of the mitral valve, 3,4,8,[10][11][12][13] with one case involving the A3 leaflet 7 and one case involving P2 leaflet. 6 We describe the first case which involves the junction of A1 and A2.…”

“…It has been hypothesised that symptoms may be delayed by progressive growth and dilatation of the left atrium, resulting in gradual traction of the aberrant chord eventually resulting in leaflet prolapse. 6 Other suggested mechanisms include the chord holding the free edge of the leaflet in a scallop during diastole with the atrial kicking motion resulting in the development of mitral regurgitation 7 and traumatic injury to the free edge of the leaflet resulting in tethering and restricted mobility. 10 Most cases in the literature describe involvement of the A2 leaflet of the mitral valve, 3,4,8,[10][11][12][13] with one case involving the A3 leaflet 7 and one case involving P2 leaflet.…”