European Journal of Echocardiography Advance Access published online on March 5, 2008
European Journal of Echocardiography, doi:10.1093/ejechocard/jen109
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org
Incremental utility of real-time three-dimensional echocardiography in the diagnosis and preoperative assessment of cleft mitral valve in adults
Ashok Kondur,
Sridevi Pitta and
Luis Afonso*
Wayne State University/Detroit Medical Center, Harper University Hospital, 8 Brush, Division of Cardiology, 3990 John R. Detroit, MI 48201, USA
Received 5 December 2007; .
* Corresponding author. Tel: +1 313 745 2620; fax: +1 313 993 8627. E-mail address: lafonso{at}dmc.org
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Abstract
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Cleft mitral valve is a rare congenital anomaly that may present
in isolation or accompany other cardiac malformations. Our illustrative
case series highlights the advantages of three-dimensional over
conventional two-dimensional echocardiography in the diagnosis
and morphologic assessment cleft mitral valve in adults.
Keywords: Cleft mitral valve; 3D echocardiography; Mitral regurgitation
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Introduction
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Cleft mitral valve (MV) is a rare congenital anomaly, usually
associated with other cardiac malformations such as endocardial
cushion defects, secundum-type atrial septal defect (ASD), ventricular
septal defect (VSD), or transposition of the great arteries.
The cleft usually involves the anterior leaflet, and clinical
manifestations include mitral insufficiency, subaortic outflow
obstruction, or both. Data on the use of real-time three-dimensional
transthoracic echocardiography (RT3DE) in the evaluation of
cleft MVs and preoperative surgical planning are limited. Our
illustrative case series highlights the potential advantages
of RT3DE over conventional two-dimensional echocardiography
in the assessment cleft MV morphology.
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Case 1
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A 40-year-old female presented with exertional dyspnea for 3
months. Past medical history was significant for ASD repair
and angioplasty of coarctation of aorta. Physical examination
was significant for holosystolic murmur best heard at the apex
and at the left sternal border. Two-dimensional transthoracic
echocardiography (2DE) showed mild mitral regurgitation and
a suspicion of a cleft in the anterior mitral leaflet.
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Case 2
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A 46-year-old male with a significant history of injection drug
use, presented with shortness of breath for 6 months and fever
for 1 month. Previous history was significant for ASD repair
at age nine. Physical examination was significant for a loud
pansystolic murmur at the apex. A 2DE showed severe mitral regurgitation
with multiple jets and thickened mitral leaflets with vegetations
noted on both leaflets. A cleft in the anterior mitral leaflet
was suspected.
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Case 3
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A 34-year-old asymptomatic female with no significant medical
history was referred for a holosystolic murmur evaluation. Two-dimensional
echocardiogram revealed a perimembranous VSD, mild mitral regurgitation
(MR) with anterior mitral leaflet prolapse.
RT3DE examination was performed in all three patients using the X4 matrix array transducer (Sonos 7500, Philips Medical Systems). Full-volume datasets were also acquired and analysed off-line, using dedicated software (Q-Lab, Phillips). A prominent cleft in the anterior MV leaflet (Figure 1, Supplementary material online, Video files 1 and 2) was clearly visualized by RT3DE in all three patients. In addition, three-dimensional multiplane reconstruction allowed visualization of cleft disposition, presence of accessory mitral tissue/papillary muscle, accessory chordae attached to the left ventricular outflow tract (LVOT) (Figure 2), and VSD (Figure 3). Patient's clinical profile and echocardiography findings were summarized in Table 1.
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Figure 2 Short-axis still frame (3DE) of the left ventricle at the level of the mitral valve illustrating the entire extent (width and depth) of the anterior mitral cleft with accessory chordae at approximately 1’o clock position (arrow).
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Discussion
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Cleft MV is a rare congenital cause of mitral insuffiency in
adults. It can present either as an isolated cleft or with accompanying
cardiac malformations such as secondum-type ASDs, VSDs, or transposition
of the great arteries.
1 A cleft may involve the anterior or
posterior leaflets of the MV; however, anterior MV leaflet clefts
are most common. Clinical manifestations of this anomaly include
mitral regurgitation and LVOT obstruction resulting from abnormal
chordal attachments.
2 Individuals with cleft MV may remain asymptomatic
and undiagnosed for years because of mild MR, and absence of
LVOT obstruction.
Conventional 2DE is a commonly employed imaging modality for the routine evaluation of mitral valve disease. However, detailed anatomic assessment by 2DE is limited because of non-planar relationship of the leaflets and annulus.3 There have been several published series in the medical literature indicating that clefts were missed using this technique and detected for the first time during surgery for severe MR.4,5 Two-dimensional transesophageal echocardiography allows for better visualization of mitral valve anatomy and is considered the reference modality, but it is a semi-invasive procedure, occasionally associated with complications. Three-dimensional echocardiography enables precise assessment of mitral valve pathology as it provides a structural display in three dimensions from any perspective.6–8 While its utility has been extensively documented in acquired mitral valvular disease, data on its incremental value in congenital mitral valve pathology are sparse.9,10
Real-time 3DE imaging is highly sensitive in the diagnosis of cleft valves, providing accurate pathoanatomic definition, including the width and depth of the cleft, degree of fibrosis and edge retraction, presence of accessory chordae, origin, and mechanism of the regurgitant jet11 in addition to characterizing associated congenital malformations. RT3DE imaging also allows visualization of the mitral valve en face either from the left atrium or left ventricle and provides a view of the valve similar to that seen intraoperatively by the cardiac surgeon.8 It has an additional advantage of displaying the dynamic motion of the valve within the beating heart.12 However, image quality has to be recognized as a limitation in a small percentage of patients.3
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Conclusion
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In comparison to 2DE, 3DE imaging has a number of advantages;
it provides topographic details such as the spatial location,
width and depth of the cleft, degree of fibrosis, disposition
of accessory chordae, or papillary muscles and a virtual multiplanar
perspective of associated congenital defects, data invaluable
for preoperative surgical planning.
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Supplementary material
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Supplementary material is available at
EJECHO online.
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References
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- Kohl T, Silverman NH. Comparison of cleft and papillary muscle position in cleft mitral valve and atrioventricular septal defect. Am J Cardiol (1996) 77:164–9.[CrossRef][Web of Science][Medline]
- Di Segni E, Edwards JE. Cleft anterior leaflet of the mitral valve with intact septa. A study of 20 cases. Am J Cardiol (1983) 51:919–26.[CrossRef][Web of Science][Medline]
- Sharma R, Mann J, Drummond L, Livesey SA, Simpson IA. The evaluation of real-time 3-dimensional transthoracic echocardiography for the preoperative functional assessment of patients with mitral valve prolapse: a comparison with 2-dimensional transesophageal echocardiography. J Am Soc Echocardiogr (2007) 20:934–40.[CrossRef][Web of Science][Medline]
- Mohanty SR, Choudhary SK, Ramamurthy S, Kumar AS. Isolated congenital anterior mitral leaflet cleft: a rare cause of mitral insufficiency. J Heart Valve Dis (1999) 8:67–70.[Web of Science][Medline]
- Fraisse A, Massih TA, Kreitmann B, Metras D, Vouhe P, Sidi D, et al. Characteristics and management of cleft mitral valve. J Am Coll Cardiol (2003) 42:1988–93.[Abstract/Free Full Text]
- Seliem MA, Fedec A, Szwast A, Farrell PE Jr., Ewing S, Gruber PJ, et al. Atrioventricular valve morphology and dynamics in congenital heart disease as imaged with real-time 3-dimensional matrix-array echocardiography: comparison with 2-dimensional imaging and surgical findings. J Am Soc Echocardiogr (2007) 20:869–76.[CrossRef][Web of Science][Medline]
- Fabricius AM, Walther T, Falk V, Mohr FW. Three-dimensional echocardiography for planning of mitral valve surgery: current applicability? Ann Thorac Surg (2004) 78:575–8.[Abstract/Free Full Text]
- Sutaria N, Northridge D, Masani N, Pandian N. Three dimensional echocardiography for the assessment of mitral valve disease. Heart (2000) 84(Suppl. 2):II7–II10.[Medline]
- Sinha A, Kasliwal RR, Nanda NC, Chauhan N, Agrawal G, Khanna D, et al. Live three-dimensional transthoracic echocardiographic assessment of isolated cleft mitral valve. Echocardiography (2004) 21:657–61.[CrossRef][Web of Science][Medline]
- Lu Q, Lu X, Xie M, Wang X, Wang J, Yang Y, et al. Real-time three-dimensional echocardiography in assessment of congenital double orifice mitral valve. J Huazhong Univ Sci Technolog Med Sci (2006) 26:625–8.[CrossRef][Medline]
- Kuperstein R, Feinberg MS, Carasso S, Gilman S, Dror Z, Di Segni E. The added value of real-time 3-dimensional echocardiography in the diagnosis of isolated cleft mitral valve in adults. J Am Soc Echocardiogr (2006) 19:811–4.[CrossRef][Web of Science][Medline]
- Schwartz SL, Cao QL, Azevedo J, Pandian NG. Simulation of intraoperative visualization of cardiac structures and study of dynamic surgical anatomy with real-time three-dimensional echocardiography. Am J Cardiol (1994) 73:501–7.[CrossRef][Web of Science][Medline]
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Abstract
Introduction
