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

Echocardiography. 2018 May;35(5):750-752. doi: 10.1111/echo.13869. Epub 2018 Mar 22.

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.

Keywords: anterior mitral leaflet; congenital heart defects; echocardiography; mitral insufficiency; mitral stenosis.

Publication types

  • Case Reports
  • Video-Audio Media

MeSH terms

  • Aged, 80 and over
  • Chordae Tendineae / abnormalities
  • Chordae Tendineae / diagnostic imaging*
  • Delayed Diagnosis*
  • Echocardiography, Doppler, Color / methods*
  • Echocardiography, Transesophageal*
  • Female
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / diagnostic imaging
  • Humans
  • Mitral Valve / abnormalities*
  • Mitral Valve / diagnostic imaging
  • Mitral Valve Insufficiency / diagnostic imaging
  • Mitral Valve Insufficiency / etiology*
  • Severity of Illness Index