Double-Orifice Mitral Valve

Double-Orifice Mitral Valve with Intact Atrioventricular Septum: An
Echocardiographic Study With Anatomic and Functional Considerations
Bibhuti B. Das, MD, Linda B. Pauliks, MD, Ole A. Knudson, Jr, RDCS, Scott Kirby, RDCS,
Kak-Chen Chan, MD, Lilliam Valdes-Cruz, MD, and Raul O. Cayre, MD,
Denver, Colorado; Boston, Massachusetts; and Corrientes, Argentina

We identified 18 patients with double-orifice mitral
valve (DOMV) and intact atrioventricular (AV) septum
out of 40,179 echocardiographic studies performed
between 1997 and 2002 at Children’s Hospital,
Denver, CO. In this study we describe (1) the
anatomic characteristics of the DOMV in the absence
of AV septal defect, (2) the function of the mitral
valve by spectral and color Doppler flow mapping,
and (3) associated lesions. The topographic location
of the orifices in the leaflets suggests possible embryologic
mechanisms of DOMV. In this series,
DOMV was most commonly associated with leftsided
obstructive lesions (in 39% of patients). Spectral
and color Doppler interrogation demonstrated a
normal flow profile in most cases; only 2 patients
had significant mitral regurgitation or stenosis.
Therefore, due to the uncertain natural history of
this lesion and the potential need for endocarditis
prophylaxis, careful imaging of the mitral valve is
recommended, particularly in the presence of leftsided
obstructive lesions.

(J Am Soc Echocardiogr
2005;18:231-6.)