A late presentation of congenital cardiac anomaly: Accessory chordae from the left atrium causing severe mitral regurgitation

Abstract

Mitral regurgitation secondary to accessory mitral valve (MV) chordae of the left atrium is an extremely rare congenital disease. A 85-year-old female (NYHA I-II) was hospitalized for investigations. An echocardiogram showed calcification of the MV with mild stenosis and moderate regurgitation. Transesophageal three-dimensional echocardiogram revealed a band-like structure extending from the distal third of the anterior wall of the left atrium to the MV. This accessory chordae determined severe systolic regurgitation and mild mitral stenosis. The patient was referred for consideration of cardiac surgery but was refused for comorbidities and anatomy. Usually aberrant chordae determinant valvulopathies are detected and treated at a much younger age. The delay of the symptoms could be explained in our case with the progressive growth and dilatation of the left atrium causing traction of the aberrant chord resulting in an increase in the leaflet prolapse and regurgitation.

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

“…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. 6 At 59-years-old, our patient was asymptomatic from his valvular pathology and his finding was incidental. 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.…”

“…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 6 . At 59‐years‐old, our patient was asymptomatic from his valvular pathology and his finding was incidental.…”

“…At 59‐years‐old, our patient was asymptomatic from his valvular pathology and his finding was incidental. 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 .…”

“…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.…”

A Left Atrial Mitral Valve Chord

“…The prevalence in the population is largely unknown, and the clinical relevance is unclear. A number of case reports highlight anomalous left atrial chords as a cause of significant mitral regurgitation for which surgical treatment can be indicated ( 1 ). In addition, there is 1 report of a left atrial chord being involved in a complex endocarditis case ( 2 ).…”