European Journal of Echocardiography 2007 8(4):296-298; doi:10.1016/j.euje.2006.03.013
Copyright © 2006, The European Society of Cardiology
Mitral valve ring dehiscence with an aorta–left atrial fistula
Davinder S. Jassala,*,
Tomas G. Neilana,
Umaima Fatimab,
Godtfred Holmvangb,
Arvind Agnihotric,
Igor Palaciosd and
Danita M. Yoergera
aCardiac Ultrasound Laboratory, Cardiology Division, Massachusetts General Hospital, VBK-508, 55 Fruit Street, Boston, MA 02114, USA
bCardiac MRI, Department of Radiology, Massachusetts General Hospital, Boston, MA, USA
cCardiac Surgery Division, Massachusetts General Hospital, Boston, MA, USA
dCardiac Catheterization Laboratory, Cardiology Division, Massachusetts General Hospital, Boston, MA, USA
Received 27 October 2005; received in revised form 20 March 2006; accepted after revision 22 March 2006.
* Corresponding author. Tel.: +1 617 726 8871; fax: +1 617 726 8383. djassal{at}partners.org
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Abstract
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In an era with the increasing use of various imaging modalities
including echocardiography, ventriculography and cardiac magnetic
resonance (CMR) imaging, one must be aware of the limitations
of each discipline. We report a case of an individual who presented
with both a partial dehiscence of a mitral valve annuloplasty
ring and an aorta–left atrium fistula following surgical
management of infective endocarditis that was correctly identified
using transesophageal echocardiographic imaging.
Keywords: Mitral valve repair; Mitral regurgitation; Transesophageal echocardiography; Cardiac magnetic resonance imaging
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Case summary
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A 59-year-old male with mitral valve endocarditis secondary
to
Streptococcus viridans presented with worsening heart failure
symptoms four months after completing a full course of antibiotics.
He underwent a complex mitral valve reconstruction procedure
with quadrangular resection of the affected posterior cleft
leaflet and reduction annuloplasty with a 28mm partial Cosgrove
band. There was no evidence of recurrent infection at the time
of the surgery.
Two months later, the patient presented with dyspnea on minimal exertion and was discovered to have a hemolytic anemia. During the workup of his anemia, an aorta to left atrium fistula was detected on cardiac magnetic resonance imaging (Fig. 1). The patient was referred to our centre for an attempt at percutaneous closure of the fistula. A preprocedural transesophageal echocardiogram confirmed the communication (Fig. 2), but also discovered dehiscence of the mitral annuloplasty ring with severe mitral regurgitation around both sides of the free portion of the ring (Fig. 3A and B, Movie 1). Aortography localized the fistulous channel between the noncoronary cusp of the aorta and the left atrium.

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Figure 1 Steady state free precession (SSFP) image in straight transverse view demonstrating the fistula between the noncoronary cusp of the aorta and the posteriorly located left atrium (arrow). LA, left atrium.
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Figure 2 A transesophageal short axis view of the aorta with color Doppler. A fistulous communication between the noncoronary cusp of the aorta and the left atrium is noted (large arrow). The origin of the fistula was located 1.0cm (green line) from the ostium of the left main coronary artery.
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Figure 3 A transesophageal long axis view of the left ventricle with and without color (A and B). A linear echodensity arises from the posterior portion of the ring consistent with a torn suture (Fig. 3A). The posterior portion of the mitral valve ring is dehisced with severe mitral regurgitation (large arrow) (Fig. 3B). A fistulous channel is identified between the aorta and the left atrium (small arrow) with a moderate amount of shunt by color Doppler. LV, left ventricle.
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The development of the dehiscence was attributed to the presence
of the fistula with resultant increase in LA pressure and dilation
of the LA and mitral annulus. The most likely source of the
hemolysis was the mitral inflow and regurgitation around the
dehisced portion of the mitral ring. The patient was referred
for surgical correction. During cardiopulmonary bypass, the
dehisced mitral valve ring was replaced with a 29mm St. Jude's
valve with successful closure of the aorta–left atrial
fistula. There was no evidence of active infection in the blood
stream or the excised tissue.
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Discussion
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Two surgical procedures confined to the treatment of mitral
regurgitation are valve repair and valve replacement. Mitral
valve repair, when feasible, has the advantages of preserving
left ventricular geometry by maintaining subvalvular apparatus,
eliminating the use of a prosthetic valve with its attendant
complications, and avoidance of chronic anticoagulation.
1
Dehiscence of an annular ring is a rare occurrence. A MEDLINE review of the literature published between 1966 and 2005 revealed only two previously described cases of mitral annular ring dehiscence2–3; our case is notable for the coexisting aorta to left atrial fistula as the likely mechanism of mitral incompetence.
In an era with the increasing use of various imaging modalities including echocardiography, ventriculography and cardiac magnetic resonance (CMR) imaging, one must be aware of the limitations of each discipline. Although the left atrial to aorta fistula was well delineated by CMR and confirmed by ventriculography, the advantage of transesophageal echocardiography using two-dimensional, color, and spectral Doppler imaging allowed for the correct identification of both pathophysiological processes. With the increasing number of mitral valve repairs thus, the occurrence of a mitral ring dehiscence and its inherent diagnostic findings must be understood.
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Supplementary data
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Supplementary information for this manuscript can be downloaded
at doi:
10.1016/j.euje.2006.03.013.
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References
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- Schwartz C.F., Gulkarov I., Bohmann K., Colvin S.B., Galloway A.C. The role of annuloplasty in mitral valve repair. J Cardiovasc Surg (2004 Oct) 45(5):419–425.[Medline]
- Gindea A.J., Schwinger M., Freedberg R.S., Colvin S.B., Kronzon I. Dehiscence of a carpentier mitral ring: diagnosis by transesophageal echocardiography. Am Heart J (1989) 118:841–843.[CrossRef][Web of Science][Medline]
- Masiello P., Itri I., Mastrogiovanni G., Di Benedetto G. Transesophageal echocardiographic diagnosis of a dehisced carpentier mitral ring. Echocardiography (2000) 17:731–732.[CrossRef][Web of Science][Medline]

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