Double-orifice mitral valve with intact atrioventricular septum: An echocardiographic study with anatomic and functional considerations

doi:10.1016/j.echo.2004.12.001

Journal of the American Society of Echocardiography

Volume 18, Issue 3, March 2005, Pages 231-236

https://doi.org/10.1016/j.echo.2004.12.001 Get rights and content

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 left-sided 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 left-sided obstructive lesions.

Section snippets

Study design

A review of the electronic database of the Cardiac Imaging Laboratory at Children’s Hospital, Denver revealed 18 cases coded as DOMV with intact AV septum out of 40,179 echocardiographic studies performed between 1997 and 2002. All 18 patients had analog videotape recordings, and in 17 also had digital image clips available for off-line analysis. The echocardiograms were reviewed by at least 2 independent investigators to confirm an intact AV septum and a DOMV. These cases constitute the

Results

Table 1 summarizes the demographic data for the study group. The patients ranged in age from 1 month to 25 years, and the female:male ratio was 2:1.

Discussion

This study presents anatomic and functional data on cases of DOMV with an intact AV septum. In our cases, DOMV was most commonly associated with left-sided obstructive lesions (in 39% of cases) and with VSDs (in 17% of cases). Although the individual orifices can be small with tethered chordal attachments causing significant mitral obstruction, in our series only 2 patients (11%) had significant mitral regurgitation or stenosis. Consequently, DOMV may be easily missed in patients with

References (18)

C.S. Wakai et al.
Pathologic study of persistent common atrioventricular canal
Am Heart J
(1958)
A. Bano-Rodrigo et al.
Double-orifice mitral valvea study of 27 postmortem cases with developmental, diagnostic, and surgical considerations
Am J Cardiol
(1988)
E. Trowitzsch et al.
Two-dimensional echocardiographic findings in double-orifice mitral valve
J Am Coll Cardiol
(1985)
D.W. Rowe et al.
Two-dimensional echocardiography in double-orifice mitral valve
J Am Coll Cardiol
(1984)
N. Ciampani et al.
The tensor apparatus in double-orifice mitral valveinterpretation of echocardiographic findings
J Am Soc Echocardiogr
(1997)
M.A. Quinones et al.
Recommendation for quantification of Doppler echocardiographya report from the Doppler Quantification Task Force of the Nomenclature and Standards Committee of the American Society of Echocardiography
J Am Soc Echocardiogr
(2002)
I.L. Hartyanszky et al.
Ebstein’s anomaly and intermediate-form atrioventricular septal defect with double-orifice mitral valve
J Thorac Cardiovasc Surg
(1992)
C. Warnes et al.
Double mitral orifice in atrioventricular defects
Br Heart J
(1983)
F.E. Sherman
An atlas of congenital heart disease
(1963)

There are more references available in the full text version of this article.

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Citation Excerpt :
DOLAVV is characterized by 2 valve orifices, usually with a separate subvalvular apparatus for each orifice. The presence of tendinous attachments to both openings distinguishes DOLAVV from acquired lesions such as perforation or partial fusion of valve leaflets caused by inflammatory lesions, perforated aneurysm of a leaflet, traumatic ruptures, and complications of interventional procedures (eg, balloon valvotomy) [12]. The morphogenetic mechanism of DOLAVV is explained by an abnormal union into a single structure between the dorsal endocardial and left lateral endocardial cushions during the early developmental stage [13].
The surgical management of double orifice left atrioventricular valve (DOLAVV) in atrioventricular septal defects (AVSD) can be challenging and has important surgical implications.
Between 1961 and 2009, 44 patients with DOLAVV associated with AVSD were reviewed; this constituted 6.7% of cases of AVSD. The median age at operation was 6.6 years (range 2 months–70 years). DOLAVV was associated with partial, intermediate, and complete AVSD in 28 patients (64%), 1 patient (2%), and 15 patients (34%), respectively.
Forty-one patients (93%) had partial or complete closure of the zone of apposition (ZOA), and in 3 patients (7%), the ZOA was left open. The accessory orifice was found to be regurgitant in 4 patients, and in all 4 patients it was closed. Four patients had partial annuloplasties. Early mortality consisted of 1 death (2.2%). This was a patient in whom the tissue bridge was divided and severe regurgitation resulted. Median follow-up was 10.3 years (maximum, 36 years). Three adult patients required mitral valve replacement for severe mitral regurgitation (MR) at 3, 11, and 20 years, respectively. Two of these patients experienced progression of MR resulting from an unsutured ZOA. There were 3 late deaths (6%). One death occurred after mitral valve replacement and the others died of noncardiac causes. No patient had hemodynamically significant mitral stenosis.
Repair of DOLAVV in AVSD can be performed with low risk, excellent late survival, and freedom from reoperation. The ZOA is typically closed unless the valve area appears small. In the majority of patients, the accessory orifice is competent and can be left alone. Late repeated repair may be difficult because of leaflet dysplasia.
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In previous studies, double orifice mitral valve (DOMV) was found to be a rare congenital anomaly, especially in isolated DOMV stenosis. The aim of this study was to demonstrate the usefulness of transthoracic echocardiography, which includes two-dimensional and Doppler echocardiography, for diagnosing and evaluating stenotic DOMV pre- and post-balloon mitral valvuloplasty (BMV). This is believed to be the first study ever reported in Taiwan.
We retrospectively reviewed the echocardiographic findings of eight symptomatic patients with stenotic DOMV who successfully underwent BMV between September 2000 and March 2005. We analyzed their echocardiographic characteristics of valve morphology, size, location and transmitral pressure gradient before BMV and valve restenosis after BMV.
All patients had incomplete bridge type with larger posteromedial orifice. No associated lesions were noted in any of the patients. After medical treatment and evaluation, they all underwent BMV successfully. Follow-up echocardiography showed no mitral valve restenosis.
Two-dimensional echocardiography is a dependable method for diagnosing DOMV and evaluating stenotic DOMV post-BMV.
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Original articlesDouble-orifice mitral valve with intact atrioventricular septum: An echocardiographic study with anatomic and functional considerations

Section snippets

Study design

Results

Discussion

Am Heart J

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

J Am Soc Echocardiogr

J Am Soc Echocardiogr

J Thorac Cardiovasc Surg

Double mitral orifice in atrioventricular defects

Br Heart J

An atlas of congenital heart disease

Original articles
Double-orifice mitral valve with intact atrioventricular septum: An echocardiographic study with anatomic and functional considerations