Original articlesDouble-orifice mitral valve with intact atrioventricular septum: An echocardiographic study with anatomic and functional considerations
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)
- et al.
Pathologic study of persistent common atrioventricular canal
Am Heart J
(1958) - et al.
Double-orifice mitral valvea study of 27 postmortem cases with developmental, diagnostic, and surgical considerations
Am J Cardiol
(1988) - et al.
Two-dimensional echocardiographic findings in double-orifice mitral valve
J Am Coll Cardiol
(1985) - et al.
Two-dimensional echocardiography in double-orifice mitral valve
J Am Coll Cardiol
(1984) - et al.
The tensor apparatus in double-orifice mitral valveinterpretation of echocardiographic findings
J Am Soc Echocardiogr
(1997) - 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) - et al.
Ebstein’s anomaly and intermediate-form atrioventricular septal defect with double-orifice mitral valve
J Thorac Cardiovasc Surg
(1992) - et al.
Double mitral orifice in atrioventricular defects
Br Heart J
(1983) An atlas of congenital heart disease
(1963)
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