Isolated Cleft Mitral Valve

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J Am Soc Echocardiogr. 2006 Jun;19(6):811-4.
The added value of real-time 3-dimensional echocardiography in the diagnosis of isolated cleft mitral valve in adults.
Kuperstein R, Feinberg MS, Carasso S, Gilman S, Dror Z, Di Segni E.
Heart Institute, Sheba Medical Center, Tel Hashomer, Israel.

We evaluated the potential advantage of real-time 3-dimensional echocardiography on the assessment of mitral valve morphology and function in patients with isolated cleft mitral valve. Five patients, aged 33 +/- 9 years, with isolated cleft mitral valve and no associated cardiac anomalies, were studied. Real-time 3-dimensional echocardiography demonstrated the cleft in each case and allowed the measurement of its width and depth, the extent of cleft edge fibrosis and retraction, and the presence of accessory chordae and their attachment to the septum. Four patients had moderate or severe mitral regurgitation and in one patient the regurgitation was mild. In all patients the mitral regurgitant jet originating from the cleft was detected, and in one patient, a second jet was detected and not seen in the 2-dimensional study. Real-time 3-dimensional echocardiography is a reliable and reproducible technique that provides accurate and detailed echocardiographic characterization of the isolated cleft mitral valve in adults.

Eur J Echocardiogr. 2006 Jan 20;
Isolated cleft of the anterior mitral valve leaflet.
Timoteo A, Galrinho A, Fiarresga A, Branco L, Banazol N, Leal A, Fragata J, Quininha J.
Echocardiography Laboratory, Cardiology Department, Santa Marta Hospital, R. Santa Marta, 1169-024 Lisbon, Portugal.

Isolated anterior mitral leaflet cleft (not associated with atrio-ventricular septal defect) is a rare cause of congenital mitral regurgitation, and the treatment consists of direct suturing of the cleft. We present a clinical case with this entity.

Circ J. 2005 Jan;69(1):114-5.
Mitral regurgitation with an isolated anterior mitral leaflet cleft: a case report.
Oshima K, Takahashi T, Sato Y, Mohara J, Ishikawa S, Morishita Y.
Second Department of Surgery, Gunma University Faculty of Medicine, Maebashi, Japan.

A 20-year-old man was admitted to hospital because of general fatigue during exercise. He had had a heart murmur since the age of 6 years. Echocardiography showed severe mitral regurgitation (MR, IV), probably caused by an anterior leaflet cleft or tendon rupture. During surgery, a cleft measuring 9 mm in length was found in the center of the anterior leaflet of the mitral valve. The cleft was closed directly, together with annuloplasty using the bilateral Kay's method. A Cosgrove ring (32 mm) was added because the mitral valve annulus was dilated. The patient's postoperative course was uneventful and echocardiography after surgery demonstrated no MR. An isolated cleft of the anterior mitral leaflet is a rare cause of MR and in this case, direct closure of the cleft with additional annuloplasty gave a good functional result.

Echocardiography. 2004 Oct;21(7):657-61.
Live three-dimensional transthoracic echocardiographic assessment of isolated cleft mitral valve.
Sinha A, Kasliwal RR, Nanda NC, Chauhan N, Agrawal G, Khanna D, Dod HS.
Division of Cardiovascular Disease, University of Alabama at Birmingham, Birmingham, Alabama, USA.

We report the usefulness of live three-dimensional transthoracic echocardiography in the comprehensive assessment of isolated cleft mitral valve in two adult patients studied by us.

J Am Soc Echocardiogr. 2004 Jul;17(7):783-5.
Two cases of isolated cleft mitral valve with presentation in adulthood.
Schwalm SA, Spencer KT, Neithardt G, Lang RM.
Department of Medicine, University of Chicago Medical Center, Chicago, IL 60637, USA.

J Am Coll Cardiol. 2000 Mar 1;35(3):764-70.
Clinical features and management of isolated cleft mitral valve in childhood.
Tamura M, Menahem S, Brizard C.
Department of Cardiology, Royal Children's Hospital, Melbourne, Australia.

OBJECTIVES: We reviewed an institutional experience of isolated cleft mitral valve (ICMV), its clinical features, and management in a pediatric population. BACKGROUND: As ICMV is relatively uncommon, earlier reports highlighted its anatomical and echocardiographic features. Few studies have collated their clinical features with their outcome. METHODS: All patients with ICMV were retrospectively reviewed. Patients who were considered to have an atrioventricular septal defect or variant were excluded. RESULTS: Twenty patients (9 male, 11 female) were diagnosed with ICMV. Seven patients had associated cardiac lesions. The median age of diagnosis was 5.2 years (range 0.4 to 13.6 years). Echocardiography aided by color Doppler demonstrated the ICMV in all patients. However, an incomplete diagnosis was made in 4 of 20 patients before surgery. The severity of the mitral regurgitation (MR) at presentation was mild in 11, moderate in 8, and severe in 1 patient. In the 13 patients without associated cardiac lesions, 5 underwent mitral valve (MV) repair at median age of 5.2 years (range 1.2 to 7.7 years) for moderate to severe MR, 4 being symptomatic. The severity of the MR in seven of the eight unoperated patients has remained unchanged over the follow-up period (median 8.3 years, range 0.7 to 14.4 years). In total, 10 patients underwent MV repair (median 6.4, range 0.4 to 13.8 years). No patient required MV replacement. None of the 10 patients had more than mild MR over the follow-up period (median 0.6, range 0.2 to 11.0 years). CONCLUSIONS: Now readily diagnosable by echocardiography, ICMV is a correctable cause of MR with a good outcome. Surgery is indicated in those patients with moderate to severe MR and probably should be done early following diagnosis.

J Heart Valve Dis. 1999 Jan;8(1):67-70.
Isolated congenital anterior mitral leaflet cleft: a rare cause of mitral insufficiency.
Mohanty SR, Choudhary SK, Ramamurthy S, Kumar AS.
Cardiothoracic Sciences Center, All India Institute of Medical Sciences, New Delhi.

BACKGROUND AND AIM OF THE STUDY: Isolated cleft of the anterior mitral leaflet is a rare cause of mitral insufficiency. Although an established entity, due to its rarity the exact anatomic diagnosis is difficult to establish unless sought specifically. METHODS: Four patients (age range: 16 to 26 years) with isolated cleft of the anterior mitral leaflet were treated at the authors' institute. Clinical symptoms were typical of mitral insufficiency; the exact anatomic diagnosis was not established preoperatively in any patient. The cleft was directly sutured in all four patients and additional annuloplasty was performed in three. RESULTS: Postoperative echocardiography confirmed satisfactory results. After a mean follow up of 46.7 months (range: 3 to 84 months), one patient had mild mitral insufficiency and the remaining patients had no mitral regurgitation. CONCLUSION: In severe mitral insufficiency with no obvious mitral valve pathology and an intact atrial septum, a cleft of the anterior mitral leaflet should be sought. Repair of the cleft can restore normal mitral valve function.

Am J Cardiol. 1995 Dec 15;76(17):1284-91.
Echocardiographic evaluation of congenital mitral valve anomalies in children.
Banerjee A, Kohl T, Silverman NH.
Cardiovascular Research Institute, University of California, San Francisco 94143-0214, USA.

Congenital mitral valve anomalies were diagnosed in 65 children, whose ages ranged from newborn to 18 years, using 2-dimensional, color, pulsed-, and continuous-wave Doppler ultrasound. Data were collected over 7.5 years from 13,400 new studies. Data in these patients were compared with those obtained by cardiac catheterization, cardiac surgery, and autopsy. We detected 4 different lesions: (1) congenital mitral stenosis with 2 papillary muscles (n = 24); (2) parachute mitral valve, with a single papillary muscle (n = 24); (3) isolated cleft in the mitral valve (n = 10); and (4) double-orifice mitral valve (n = 7). A supravalvar mitral ring was detected in 21 patients with mitral stenosis; however, it never occurred as an isolated lesion and was invariably associated with some other left ventricular inflow or outflow obstruction. The supravalvar ring was associated with a parachute deformity of the mitral valve in 17 patients; in only 4 was this abnormality associated with mitral stenosis with 2 papillary muscles. In patients with congenital mitral stenosis, the peak and mean transmitral Doppler velocities were increased significantly compared with those in controls (peak velocity 1.53 +/- 0.74 vs 0.86 +/- 0.25 m/s, respectively, p < 0.01; mean velocity 1.13 +/- 0.61 vs 0.58 +/- 0.11 m/s, respectively, p < 0.01). The correlation between mean transmitral pressure gradient obtained by Doppler and cardiac catheterization was fair (r = 0.75). However, the correlation between the mitral valve areas calculated by the Doppler pressure half-time method and by the Gorlin formula was poor (r = 0.57).(ABSTRACT TRUNCATED AT 250 WORDS)

J Am Coll Cardiol. 1995 Aug;26(2):508-15.
Is a cleft in the anterior leaflet of an otherwise normal mitral valve an atrioventricular canal malformation?
Sigfusson G, Ettedgui JA, Silverman NH, Anderson RH.
Division of Cardiology, Children's Hospital of Pittsburgh, Pennsylvania, USA.

OBJECTIVES. This study sought to ascertain the surgical anatomy of a cleft in the left atrioventricular (AV) valve. BACKGROUND. Important morphologic differences exist between hearts with a cleft in the anterior leaflet of an otherwise normal mitral valve and those with a so-called cleft in the left AV valve when there is an AV septal defect, but it has been customary to link the lesions together on developmental grounds. METHODS. Eight autopsied specimens with a cleft in the aortic (or anterior) leaflet of the mitral valve were studied in detail, and echocardiograms from 21 patients with such a cleft were compared with the specimens and with findings typical of the so-called partial AV canal and other forms of AV septal defect. RESULTS. The structure and direction of the cleft, location of the papillary muscles within the left ventricle and AV junctional morphology of hearts with an otherwise normally structured mitral valve were significantly different from typical findings in hearts with AV septal defects. CONCLUSIONS. It is necessary to distinguish morphologically a cleft in an otherwise normally structured mitral valve in hearts with separate right and left AV junctions from the trifoliate left component of a common AV valve in hearts with an AV septal defect and a common AV junction because the disposition of the AV conduction tissues varies markedly between the lesions.

Ann Thorac Surg. 1995 Jan;59(1):56-9.
Isolated cleft mitral valve: valve reconstruction techniques.
Perier P, Clausnizer B.
Herz und Gefass Klinik, Bad Neustadt/Saale, Germany.

Reports concerning an isolated cleft of the anterior mitral valve are rare. This congenital anomaly of the mitral valve is usually repaired by suturing the edges of the cleft. We report 4 cases of isolated anterior mitral cleft. The patients ranged in age from 13 to 41 years. The clinical symptoms were those typical of mitral insufficiency. In all 4 patients, preoperative echocardiography was able to establish the exact anatomic diagnosis. In 1 patient, the cleft was directly sutured, whereas, in the other 3 patients, a fibrous reaction of the edges of the cleft with a subsequent lack of valvular tissue made direct suture technically impossible. Instead, the fibrous edges of the cleft were resected and the anterior leaflet of the mitral valve was reconstructed using an autologous pericardial patch pretreated with buffered glutaraldehyde. All 4 patients underwent annuloplasty together with placement of a Carpentier mitral ring. Postoperative echocardiograms have confirmed good results of the repair; 1 patient has a trivial insufficiency and 3 have a completely competent mitral valve.

J Am Soc Echocardiogr. 1994 Jul-Aug;7(4):422-4.
Cleft in the anterior and posterior leaflet of the mitral valve: a rare anomaly.
McDonald RW, Ott GY, Pantely GA.
Division of Adult Cardiology, School of Medicine, Oregon Health Sciences University, Portland 97201-3098.

A rare entity that causes congenital mitral regurgitation is an isolated cleft mitral valve. The cleft in the mitral valve can be seen in either the anterior or posterior leaflet of the valve. We present a unique case of an individual with a history of congenital mitral regurgitation caused by a cleft in both the anterior and posterior leaflets of the mitral valve.

Chest. 1992 Jan;101(1):12-5.
Color Doppler echocardiography of isolated cleft mitral valve. Roles of the cleft and the accessory chordae.
Di Segni E, Kaplinsky E, Klein HO.
Heart Institute, Sheba Medical Center, Tel-Hashomer, Israel.

To study the mechanism of altered mitral function in the presence of an isolated cleft mitral valve (ICMV) with regard to the relative roles of the cleft and of the accessory chordae, seven patients with ICMV were studied with color Doppler echocardiography. Mitral insufficiency ranging from mild to severe was demonstrated in six cases. The regurgitant jet originated in each case from the site of the cleft: in five patients the regurgitant jet had a narrow base originating exactly from the cleft; in the sixth patient, the regurgitant flow presented as a broad-based jet suggesting that accessory chordae restricted the motion of the anterior mitral leaflet. Turbulent flow in the left ventricular outflow tract, starting at the level of the accessory chordae, was found in one patient in whom a pressure drop of 44 mm Hg was detected with continuous-wave Doppler imaging. The altered function of the mitral valve cleft stems from two elements, the cleft itself and the accessory chordae. Color Doppler flow imaging showed that the cleft was the main factor causing mitral insufficiency. The accessory chordae played an additional pathogenetic role in two patients by causing restricted mitral motion or left ventricular outflow tract obstruction.

Ann Cardiol Angeiol (Paris). 1987 Jul-Sep;36(7):347-50.
Latent congenital mitral insufficiency caused by an isolated mitral cleft. Diagnosis by two-dimensional echocardiography and pulsed Doppler
Petitalot JP, Chaix AF, Bonneau G, Rousseau G, Barraine R.
Service de Cardiologie A, CHU La Miletrie, Poitiers.

In this paper, the authors report the case of an 8-year-old boy with an isolated slit in his mitral valve, and who did not have a heart murmur detectable either by auscultation or by phonocardiography and who had no symptoms of heart failure. Two-dimensional echocardiography, with a left parasternal direction, in a transversal view revealed the presence of this abnormality of the endocardial leaflets accompanied by an accessory chorda tendina, and this procedure confirmed the absence of hemodynamic percussion. Doppler ultrasonography detected an abnormal turbulent systolic blood flow immediately behind the mitral valve, suggesting a minimal to moderate degree latent regurgitation. Two-dimensional echocardiography together with the Doppler ultrasound makes possible the diagnosis of an isolated slit of the mitral valve by non-invasive technique and the detection of concomitant latent mitral valve regurgitation, especially when the left atrium is not dilated and if the patient does not have low cardiac output.

Am Heart J. 1984 Dec;108(6):1554-6.
Two-dimensional echocardiographic documentation of accessory chordae tendineae accompanying isolated anterior mitral cleft.
Yamamoto Y, Shimada R, Kaseda S, Tomoike H, Takeshita A, Nakamura M.

Am J Cardiol. 1983 Mar 15;51(6):919-26.
Cleft anterior leaflet of the mitral valve with intact septa. A study of 20 cases.
Di Segni E, Edwards JE.

Twenty pathologic specimens of heart, each with a cleft in the anterior leaflet of the mitral valve not associated with septal defects of persistent common atrioventricular canal (isolated cleft of the mitral valve), were studied. In 9 cases, there were either no associated anomalies or ones not of functional significance. In each of the other 11 cases there were other significant associated cardiac anomalies, including ventricular septal defect and d-transposition of the great arteries. Functional consequences of the mitral anomaly were mitral insufficiency, subaortic stenosis, or both. Mitral insufficiency was related principally to the width of the cleft. Subaortic stenosis, when present, was due to the position of insertion of accessory chordae in the ventricular septum in a position under the commissure, between the left and right aortic cusps or under the right aortic cusp.

Am J Cardiol. 1983 Mar 15;51(6):927-31.
Isolated cleft mitral valve: a variety of congenital mitral regurgitation identified by 2-dimensional echocardiography.
Di Segni E, Bass JL, Lucas RV Jr, Einzig S.

Nine children with isolated cleft mitral valve, aged 1 day to 12 years, were studied. The electrocardiogram showed a normal QRS axis in 5 subjects. Cardiac catheterization was performed in 4 patients and demonstrated severe mitral insufficiency in 3 but failed to clearly demonstrate a cleft mitral valve or gooseneck deformity. The mitral cleft was confirmed at operation in 2 patients. Two-dimensional echocardiography demonstrated a cleft dividing the anterior mitral leaflet into 2 portions in each patient. The mitral anulus was normally positioned and the atrioventricular septum present. Atrial and ventricular septa were intact. Features similar to anatomic studies such as accessory chordae and thickening of the edges of the cleft with increasing age were also seen. Two-dimensional echocardiography is the only method available to reliably diagnose isolated cleft of the mitral valve.

Br Heart J. 1982 Aug;48(2):109-16.
Isolated anterior mitral cleft. Two dimensional echocardiographic assessment and differentiation from "clefts" associated with atrioventricular septal defect.
Smallhorn JF, de Leval M, Stark J, Somerville J, Taylor JF, Anderson RH, Macartney FJ.

Five patients with isolated clefts in the anterior leaflet of the mitral valve, unassociated with atrioventricular septal defects, are described. All had significant mitral regurgitation, with the cleft being the only abnormality in three. Two patients had an associated ventricular septal defect, one with a straddling right atrioventricular valve. Angiocardiography in four showed moderate regurgitation, but was not able to delineate the aetiology. Two dimensional echocardiography showed a constant defect in the anterior leaflet, pointing towards the left ventricular outflow tract. This differed from 30 cases with atrioventricular septal defects where the "cleft" pointed towards the interventricular septum and was situated between the anterior and posterior bridging leaflets. All cases with isolated clefts had surgical correction, with minimal residual regurgitation on follow-up examination in two cases. Our current policy in patients with uncomplicated isolated cleft involves non-invasive assessment of these children and surgical correction if the regurgitation is significant.

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