Abnormal position of the cleft of the anterior leaflet of the mitral valve in atrio-ventricular canal defects

by Mohammed Ayman Abdelhay, MD, PhD; Hoda Shehata, MD
Cardiology Department, Alexandria School of Medicine, Alexandria, Egypt.

Clinical Case

  • 25-year old female
  • atrio-ventricular canal defect repair at the age of 20 years
  • dyspnea on exertion
  • referred for routine echocardiographic follow-up

Two-dimensional transthoracic echocardiography. Apical 4-chamber view with color Doppler showing thickened mitral leaflets (left panel) and two regurgitant jets (right panel). The largest regurgitant jet originate at the mitral leaflet coaptation point. A smaller jet seems to originate from the base of the anterior leaflet

Two-dimensional echocardiography. Parasternal short-axis view showing a cleft of the anterior leaflet of the mitral valve

Two-dimensional transesophageal echocardiography. Four-chamber view with color Doppler confirming the presence of the cleft (left panel) and showing a better separation of the two mitral regurgitation jets. The interatrial septum was found intact.

Three-dimensional transesophageal echocardiography. Single-beat zoomed acquisition of the mitral valve shown from the left atrial perspective (“surgical view”). The mitral valve is clearly abnormal and the differential diagnosis is among:

  1. Cleft of the anterior mitral leaflet (black arrow)
  2. Cleft of the posterior mitral leaflet (blue arrow)
  3. Three-leaflet mitral valve

In patients with atrio-ventricular canal defects there is a counterclockwise rotation of the papillary muscles determining a different position of the cleft compared with patients with isolated mitral cleft (drawing on the right).  In patients with isolated cleft of the anterior mitral leaflet, the usual site of the cleft  is towards left ventricular outflow tract, at 11 o’clock (top left panel). Whereas, in patients with atrio-ventricular canal defects, the cleft is located towards the interventricular septum (bottom left panel).

Three-dimensional transesophageal echocardiography. Still-frame of the mitral valve shown from the left atrial perspective (“surgical view”) to show:

  1. The cleft (black arrow)
  2. The posterior-medial commissure (yellow arrow)
  3. The anterior-lateral commissure (blue arrow)

Key messages

  • Clefts of the anterior leaflets of the mitral valve are congenital malformations that are well-suited to be evaluated by three-dimensional echocardiography
  • Three-dimensional transesophageal echocardiography allows the “en face” view of the left atrio-ventricular valve from the atrial perspective that was pivotal to identify the position and the extent of the cleft in the left atrio-ventricular valve
  • Three-dimensional echocardiography improves our understanding of postoperative sequelae following repair of atrio-ventricular septal defects and allows to plan further surgical or interventional procedures

References:

  1. Smallhorn IF, Sutherland GR, Anderson R, et al: Cross-sectional echocardiographic assessment of conditions with atrioventricular valve leaets attached to the atrial septum at the same level. Br Heart J 1982;48:331–341
  2. Simpson J, Lopez L, Acar P, et al. Three-dimensional Echocardiography in Congenital Heart Disease: An Expert Consensus Document from the European Association of Cardiovascular Imaging and the American Society of Echocardiography. J Am Soc Echocardiogr 2017; 30: 1-27

Mohamad Ayman Abdel-Moniem Abdel-Hay, MD, PhD

Mohamad Ayman Abdel-Moniem Abdel-Hay is Professor of Cardiology- Faculty of Medicine, Alexandria University since September 2005. Currently, he is the Chairman of the cardiology department and the Director of the Adult Echocardiography Laboratory – Faculty of Medicine, Alexandria University. Egypt.
He graduated from Alexandria University school of Medicine, in 1981 (grade “Excellent with Honors”). He got his Master Degree in Internal Medicine, Alexandria University, in 1985 (grade Excellent) and got his Doctor Degree in Cardiology, Alexandria Universty in 1991.
Memberships: Egyptian Society of Cardiology; Board of Directors of Egyptian Echocardiography Working Group; and European Society of Cardiology.