Skip Navigation

European Journal of Echocardiography 2003 4(4):334-335; doi:10.1016/S1525-2167(03)00016-7
© 2003 by European Society of Cardiology
This Article
Right arrow Abstract Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Heyse, A.M
Right arrow Articles by Versée, L
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Heyse, A.M
Right arrow Articles by Versée, L
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Copyright © 2003, The European Society of Cardiology

Mitral Insufficiency with Congenital Double-Orifice Mitral Valve in an Elderly Patient

A.M Heyse, D Vanhercke*, J Nimmegeers, L Missault, R Claeys, G Hollanders and L Versée

Department of Cardiovascular Diseases, AZ St-Lucas, Ghent, Belgium

Received 13 September 2002; received in revised form 6 March 2003; accepted after revision 6 March 2003.

* Address correspondence to: D. Vanhercke, Department of Cardiovascular Diseases, Campus H Familie, AZ St-Lucas, Groenebriel 1, Ghent 9000, Belgium. Tel: +32-9-2246401; Fax: +32-9-2246409. echohf{at}azstlucas.be


   Abstract
Top
 Abstract
Introduction
 Case Report
 Discussion
 References
 
We describe the case of a 79-year-old woman with mitral insufficiency and a double-orifice mitral valve (DOMV), discovered by echocardiography. Transthoracic echocardiography showed two insufficiency jets. Transesophageal echocardiography revealed a DOMV. Each orifice was provided with a subvalvular apparatus. No associated congenital abnormalities were present. Our case demonstrates that even in elderly patients with a double regurgitant jet, DOMV should be suspected and assessed by transesophageal echocardiography.

Keywords: double-orifice mitral valve; mitral insufficiency; transesophageal echocardiography; congenital malformation


   Introduction
Top
 Abstract
 Introduction
Case Report
 Discussion
 References
 
Double-orifice mitral valve (DOMV) is a rare congenital malformation. More than 200 cases have been reported. We present the case of a 79-year-old woman with mitral regurgitation and DOMV, detected during echocardiographic examination.


   Case Report
Top
 Abstract
 Introduction
 Case Report
Discussion
 References
 
A 79-year-old woman with diabetes, hypertension and paroxysmal atrial fibrillation was admitted to our hospital. A few weeks before admission, she started complaining of shortness of breath during exercise, ultimately leading to severe dyspnea. Clinical examination revealed a systolic murmur grade 2 and bilateral rales. The blood pressure on admission was 170/90 mmHg. Twelve-lead electrocardiography showed sinus rhythm with aspecific ST-T segment abnormalities. On chest X-ray, signs of vascular congestion and fluid overload were seen. Blood tests were normal. Echocardiography (ATL 3000, HDI) showed left ventricular hypertrophy, normal left ventricular systolic function, severe mitral insufficiency, a restrictive diastolic filling pattern and moderate pulmonary hypertension of 50 mmHg. There were two mitral insufficiency jets (Fig. 1). Transesophageal echocardiography (TEE) confirmed these findings and in addition revealed the presence of a DOMV (Fig. 2). Both orifices were provided with a subvalvular apparatus. There were no signs of endocarditis. A therapy with loop diuretics was initiated and blood pressure was lowered. One week later, cardiac catheterization was performed. Coronary angiography was normal, there was only minor mitral insufficiency and borderline pulmonary hypertension without need for surgery.


Figure 1
View larger version (106K):
[in this window]
[in a new window]
[Download PowerPoint slide]
  		Figure 1 Transesophageal echocardiogram with color Doppler showing the mitral valve. Mitral insufficiency and two separate regurgitant jets can be seen. LA, left atrium; LV, left ventricle; Or 1, orifice 1; Or 2, orifice 2.

 


Figure 2
View larger version (97K):
[in this window]
[in a new window]
[Download PowerPoint slide]
  		Figure 2 Transesophageal echocardiogram showing the mitral valve with two separate orifices, each provided with a subvalvular apparatus. LA, left atrium; LV, left ventricle; Or 1, orifice 1; Or 2, orifice 2.

 
In this patient with hypertensive heart disease with marked diastolic dysfunction and mitral insufficiency leading to volume overload, lowering of the blood pressure and the filling pressures with diuretics dramatically decreased the mitral regurgitation. The patient became asymptomatic and could be dismissed from the hospital.


   Discussion
Top
 Abstract
 Introduction
 Case Report
 Discussion
References
 
In DOMV an accessory bridge of fibrous or mitral valve tissue partially or completely divides the mitral valve into two orifices. DOMV rarely occurs as an isolated anomaly but is most commonly associated with a variety of other cardiac anomalies of which atrioventricular septal defects are most frequent[1]. Although most cases are detected in childhood, DOMV is described in adulthood or even in the elderly population as in our case[2–7]. The clinical implication of DOMV depends on the severity of the insufficiency and the presence of stenosis or associated malformations.

Two-dimensional echocardiography is undoubtedly the best detection method, the parasternal short-axis view being most useful to show DOMV[8]. However, especially in the adult patient group, TEE with color flow mapping is probably superior to precordial scanning in assessing DOMV[4]. Recently, three-dimensional echocardiography was found to be helpful in defining spatial location of the orifices in DOMV[9].

On transthoracic echocardiography, the presence of two separate regurgitant jets should arouse suspicion of DOMV and should therefore prompt to the use of TEE (Fig. 1). In this case, TEE revealed a second orifice with an own subvalvular apparatus (Fig. 2). DOMV has to be differentiated from acquired disorders such as perforation due to endocarditis, partial fusion of valve leaflets by inflammatory lesions, perforated aneurysm of a leaflet, traumatic ruptures and interventional procedures[8]. None of these illnesses were likely to be present in our patient.

This case demonstrates that even in elderly patients with mitral insufficiency and a double regurgitant jet during transthoracic echocardiography, DOMV should be suspected and assessed by TEE.


   References
Top
 Abstract
 Introduction
 Case Report
 Discussion
 References
 

  1. Baño-Rodrigo A., Van Praagh S., Trowitzsch E., Van Praagh R. Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol (1988) 61:152–160.[CrossRef][Web of Science][Medline]
  2. Purnode P., Rombaut E., Gerard M., Alkhori M., Marchandise B. Double orifice mitral valve with flail leaflet: a transoesophageal echocardiographic examination. Eur J Echocardiogr (2000) 1:144–146.[Abstract/Free Full Text]
  3. Hoffman P., Strüper O., Groundstroem K., Sutherland G. The transesophageal echocardiographic features of double-orifice left atrioventricular valve. J Am Soc Echocardiogr (1993) 6:94–100.[Medline]
  4. Mao J.T., Tang J., Li Y., Sun P. Double-orifice mitral valve with multiple papillary muscles—a report of two patients. Angiology (1999) 50:771–775.[Web of Science][Medline]
  5. Matsuaka R., Sakakibara T., Mitsuno M., Yagura A., Yoshikawa M., Ishikura F. Valve repair for mitral regurgitation associated with isolated double-orifice mitral valve. J Thorac Cardiovasc Surg (1996) 112:1666–1667.[Free Full Text]
  6. Iglesias L.F., de Tomas Labat E., Valdesuso Aguilar R., et al. Double orifice mitral valve: severe regurgitation secondary to chord rupture. Rev Esp Cardiol (1999) 52:632–634.[Web of Science][Medline]
  7. Kron J., Standerfer R.J., Starr A. Severe mitral regurgitation in a woman with a double orifice mitral valve. Br Heart J (1986) 55:109–111.[Abstract/Free Full Text]
  8. Ciampani N., Vecchiola D., Silenzi C., et al. The tensor apparatus in double-orifice mitral valve: interpretation of echocardiographic findings. J Am Soc Echocardiogr (1997) 10:869–873.[CrossRef][Web of Science][Medline]
  9. Espinola-Zavaleta N., Vargas-Barron J., Keirns C., et al. Three-dimensional echocardiography in congenital malformations of the mitral valve. J Am Soc Echocardiogr (2002) 15:468–472.[CrossRef][Web of Science][Medline]

Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?



This Article
Right arrow Abstract Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Heyse, A.M
Right arrow Articles by Versée, L
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Heyse, A.M
Right arrow Articles by Versée, L
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?