Ann Thorac Surg 2006;82:2287-2289
© 2006 The Society of Thoracic Surgeons
Case Reports
Mitral Valve Cleft in Both Anterior and Posterior Leaflet: An Extremely Rare Anomaly
Siamak Mohammadi, MD,
Sébastien Bergeron, MD,
Pierre Voisine, MD,
Denis Desaulniers, MD*
Cardiac Surgery Department, Laval Hospital, Québec, Canada
Accepted for publication May 10, 2006.
* Address correspondence to Dr Desaulniers, Department of Cardiac Surgery, Laval Hospital, 2725 Chemin Ste-Foy, Québec, G1V 4G5 Canada. (Email: denis.desaulniers{at}chg.ulaval.ca).
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Abstract
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We herein report the case of a woman with clefts in the anterior and posterior mitral leaflets causing mitral insufficiency. The patient developed heart failure symptoms in the eighth decade of life and underwent mitral valve repair, with suture closure of both clefts. The reasons of its late presentation are discussed.
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Introduction
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Isolated cleft mitral valve (MV) is a relatively uncommon lesion predominantly involving the anterior MV leaflet. A cleft in both the anterior and posterior MV leaflets is extremely rare and has been reported only once [1]. Isolated cleft MV is morphologically different from a cleft seen in atrioventricular septal defects and the spectrum of its associated anatomic features, such as papillary muscles location, chordal attachment, and cleft direction have been described [2]. The otherwise normally structured MV in this entity makes its surgical correction possible with potentially good results. We hereby describe a unique case with mitral regurgitation caused by a cleft in both the anterior and posterior MV leaflets presenting in the eighth decade of life.
A 73-year-old woman was referred to our hospital for exertional dyspnea (New York Heart Association functional class II to III) and atypical chest pain. Medical history was notable for diabetes, breast cancer, and sinus node disease with pacemaker implantation. Auscultation revealed a grade 2/6 holosystolic murmur at the left sternal border. Cardiac catheterization revealed nonobstructive coronary artery disease and grade I–II mitral regurgitation (MR) by left ventriculography. Transthoracic echocardiography showed normal left ventricle dimensions and function. The left atrial and mitral valve (MV) annulus diameters were normal. There was evidence for minimal central MR without any prolapse or other MV abnormality. No associated left ventricular outflow tract obstruction or septal defect was seen. Exercise echocardiography revealed an isolated progressive increase in MR from trivial at rest to severe without any left ventricle wall motion abnormality. The jet of MR was central (Fig 1) again without any other MV abnormality. The estimated systolic pulmonary artery pressure increased from 35 mm Hg at rest to 77 mm Hg with exercise (Fig 2).
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Fig 2. Increasing systolic pulmonary pressure (A) from 35 mm Hg at rest (B) to 77 mm Hg with dobutamine infusion.
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After complete evaluation and because of heart failure symptoms, a MV surgery was proposed. An inverted "V"-shape cleft in the anterior leaflet without extension toward the annulus, and another cleft bisecting the posterior leaflet were identified (Fig 3). A small amount of calcium deposition was detected in the MV annulus adjacent to the posterior commissure. Both clefts had chordal attachments to the left ventricle wall. The clefts were closed by several direct separate sutures (Fig 4), and a 28 mm Carpentier-Edwards (Edwards Lifesciences, Irvine, CA) MV ring was deployed after repair. Intraoperative transesophageal echocardiography revealed trivial MR after repair at artificially created 200 mm Hg of systolic blood pressure, which completely disappeared in follow-up transthoracic and dobutamine echocardiography studies performed before hospital discharge. No MV clefts could be detected in a retrospective review of the preoperative echocardiograms.
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Comment
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Isolated cleft MV is a rare disorder in adults. Accordingly, detailed descriptions of this pathology, its clinical presentation and diagnostic modalities are limited. Functional consequences of this anomaly include MV regurgitation and left ventricular outflow tract obstruction in the presence of abnormal chordal attachments. The wide spectrum of age distribution in patients with this congenital anomaly, described in previously published series, is associated with varying anatomic features contributing to MR.
Owing to the normal atrioventricular junction, individuals with isolated cleft, mild MR, and no subaortic outflow obstruction may remain asymptomatic and undiagnosed for years. This was the case with the patient herein described, who presented in the eight decade of life with exertional dyspnea. Late presentation of cleft MV in our patient could be related to the presence of sufficient MV leaflet tissue, its relative functional integrity, and the absence of other abnormalities. The cause of MR in the majority of cases has been reported to originate through the cleft site; however annular dilatation or restricted motion of the anterior MV, due to accessory chordal attachment or retraction of cleft edges, or both, has also been implicated. Angiographic features alone were found to be insufficient to reliably diagnose cleft MV.
Echocardiography is a useful method for MV disease. However, detailed anatomic analysis is not always possible with this 2-dimensional method. Cleft MV in adult patients has been missed by this technique in several published series [3, 4] and clefts were detected for the first time during surgery for severe MR. Recently the usefulness of three-dimensional transthoracic echocardiographic in the assessment of the cleft MV in the adult was studied [5]. The degree of MR depends on interactions between the annulus, leaflets, chordae tendinea, papillary muscles, left atrium, and left ventricle free wall, as well as the degree of their abnormality. The importance of MR can thus differ at rest and in exertional conditions. The absence of severe MR at rest could probably be due to minimal functional abnormality related to the presence of cleft chordal attachments, and absence of leaflets prolapse. Chordal attachments of the cleft to the ventricular septum has been described as a constant finding by some authors [4]. Kohl and Silverman [6] stated that "without such attachment the divided anterior leaflet would be flail." The presence of these chordal attachments in both clefts and the absence of MV prolapse in echocardiographic studies may be other reasons explaining the relative MV competence and its late presentation. Some authors [7] suggested that the degree of MR can be underestimated, owing to pulmonary venous systolic blunting and severe eccentricity of the MR jet.
Treatment options for cleft MV with significant MR range from surgical repair (with simple suturing of the cleft edges or patch repair) to MV replacement. In the cleft with otherwise normal MV, surgical repair is the technique of choice. Surgical repair is accomplished by approximation of the two breach edges in both the anterior and posterior MV leaflets, as was the case in our patient. The good results that we obtained with this approach can be related to the normal mitral annular size and the absence of cleft edge thickness and retraction. This was probably unusual because the MV cleft edges become thicker and rolled as patients get older, making simple suture repair less feasible [4]. Because such findings are very commonly associated with cleft MV, Perier and Clausnizer [8] suggest autologous pericardial patch augmentation repair as a preferred surgical approach.
Although isolated cleft of the MV is a rare entity, a careful examination of both leaflets is warranted in an individual with a history of MR.
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References
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- McDonald RW, Ott GY, Pantely GA. Cleft in the anterior and posterior leaflet of the mitral valve: a rare anomaly J Am Soc Echocardiogr 1994;7:422-424.[Medline]
- Sigfusson G, Ettedgui JA, Silverman NH, Anderson RH. Is a cleft in the anterior leaflet of an otherwise normal mitral valve an atrioventricular canal malformation? J Am Coll Cardiol 1995;26:508-515.[Abstract]
- 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.[Medline]
- Fraisse A, Massih TA, Kreitmann B, et al. Characteristics and management of cleft mitral valve J Am Coll Cardiol 2003;42:1988-1993.[Abstract/Free Full Text]
- Sinha A, Kasliwal RR, Nanda NC, et al. Live three-dimensional transthoracic echocardiographic assessment of isolated cleft mitral valve Echocardiography 2004;21:657-661.[Medline]
- Kohl T, Silverman NH. Comparison of cleft and papillary muscle position in cleft mitral valve and atrioventricular septal defect Am J Cardiol 1996;77:164-169.[Medline]
- Schwalm SA, Spencer KT, Neithardt G, Lang RM. Two cases of isolated cleft mitral valve with presentation in adulthood J Am Soc Echocardiogr 2004;17:783-785.[Medline]
- Perier P, Clausnizer B. Isolated cleft mitral valve: valve reconstruction techniques Ann Thorac Surg 1995;59:56-59.[Abstract/Free Full Text]
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Abstract
Introduction
