A new anatomical approach to congenital valve diseases by three-dimensional echocardiography]

Congenital valvular heart disease in childhood is often complex. Conventional echocardiography provides two-dimensional views which require mental reconstruction for three-dimensional assessment. This problem may be solved by the use of three-dimensional (3D) echocardiography which obtains images of valves comparable to those seen at surgery. This was confirmed by 4 cases of congenital valvular heart disease studied by 3D echocardiography: stenotic bicuspid aortic valve disease, parachute mitral valve and two cases of mitral regurgitation in patients with atrioventricular canal. The 3D views of the aortic valve showed the commissural opening after percutaneous balloon valvuloplasty of the bicuspid valve. The surface of the aortic orifice and the surface of the two mitral leaflets were measured from 3D reconstructions. The longitudinal 3D view analysed the extension of the single obstructive mitral papillary muscle of the parachute malformation. The 3D ventricular views allowed assessment of the extension of the cleft and the surface of the three mitral leaflets of the 2 cases of atrioventricular canal. In one of these cases, the results of surgical valvuloplasty were evaluated after 3D reconstruction of the valve. 3D echocardiography is not only a diagnostic tool for congenital heart disease but also a very useful complementary investigation for accurate evaluation of congenital valvular lesions to optimise possible valve repair.     

Isolated Cleft Mitral Valve with Posterior and Anterior Clefts: a Rare Cause of Congenital Valve Regurgitation

We report a case of isolated cleft mitral valve with two clefts in the posterior and one in the anterior leaflet. Our case adds to the few reports of posterior and multiple mitral valve clefts and to our knowledge is the first using real‐time transoesophageal three‐dimensional echocardiography (3DE) for assessment of isolated cleft mitral valve. (Echocardiography 2010;27:E50‐E52)     

Isolated cleft of both the anterior and posterior mitral valve leaflets

A 37 year‐old female patient in whom the transthoracic echocardiography examination revealed dilatation of left heart chambers with left ventricular ejection fraction of 30% and moderate‐to‐severe mitral valve regurgitation was admitted to our hospital. On 2DTEE examination, mitral valve was normal; however, on 3D images, clefts of both anterior and posterior leaflets were revealed. Isolated cleft mitral valve without any other feature of atrioventricular septal defect is uncommon. 2D echocardiography has limited capability in defining the complex 3D anatomic characteristics of the cleft. 3DTEE allows to visualize the cleft position, morphology, and size, and it is important for surgical planning.     

An interatrial tunnel: a rare form of atrial septal defects

Atrial septal defects (ASDs) account for approximately 6%–10% of congenital heart defects. The well‐known types of atrial septal communications are the ostium secundum, ostium primum, sinus venosus types, and coronary sinus defects. A 50‐year‐old female was referred for TEE for better assessment of MR severity and mechanism. 2D/3D‐TEE showed a rare combination of different abnormalities; bi‐leaflet mitral valve prolapse, cleft P2, cor triatriatum sinister, and a tunnel‐shaped IAS communication. To the best of our knowledge, this is a very rare case with a rare form of atrial septal defect that was not described before. We named this defect an interatrial tunnel.     

Isolated congenital anterior mitral leaflet cleft: a rare cause of mitral insufficiency

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.     

Mitral insufficiency due to an isolated anterior-leaflet cleft: correction with an autologous pericardial patch supported by artificial chordae

Congenital clefts of the mitral valve without an associated atrioventricular canal defect are rare, and they may cause mitral insufficiency that requires surgical correction. Repair is typically by direct suture; however, if the cleft is especially wide, the use of this technique may distort the valve leaflet and cause poor coaptation with valvular insufficiency.Herein, we present the case of a 39-year-old woman who had severe mitral valve insufficiency secondary to a wide isolated cleft of the anterior mitral leaflet. The valve was reconstructed with an autologous pericardial patch supported by polytetrafluoroethylene neochordae and an implanted annuloplasty ring. Echocardiographic examination 1 year postoperatively showed excellent competence of the mitral valve and good coaptation of the leaflets. To our knowledge, this is the 1st report that describes the use of artificial neochordae to support an autologous pericardial patch in the repair of a cleft in the anterior mitral valve leaflet.     

Isolated cleft posterior mitral valve leaflet: an uncommon cause of mitral regurgitation

A 53-year-old woman with a history of hypertension was referredfor an echocardiogram by her primary care physician after anunspecified abnormal ECG. The echocardiogram showed normal leftventricular size and function; however, an isolated cleft posteriormitral valve leaflet was identified with concomitant bileafletprolapse and mild mitral regurgitation. She was subsequentlyreferred to a cardiologist for clinical evaluation. Cleft mitralvalve leaflet (CMVL) is an uncommon congenital cause of mitralregurgitation. Clefts, defined as slit-like holes or defects,are hypothesized to be a result of incomplete expression ofan endocardial cushion defect which most commonly involves theanterior mitral valve leaflet with a paediatric incidence of1:1340. Clefts affecting only the posterior mitral valve leafletare extremely rare with only four cases being reported in themedical literature. Important co-existing anomalies with eitherposterior and/or anterior CMVL include counterclockwise rotationof the papillary muscles, the presence of an accessory papillarymuscle or mitral valve leaflet, atrial septal defects, and mitralvalve prolapse. Regurgitation from CMVL can lead to importantphysiological and anatomical changes within the cardiac system.Regurgitation results from blood flow directly through the cleftitself or from malcoaptation from accessory chordae with orwithout papillary muscle distortion. Significant chronic mitralregurgitation elevates left atrial filling pressures and leadsto chamber enlargement and eccentric left ventricular hypertrophy.Early detection through two-dimensional echocardiography canprovide accurate anatomical images of the various mitral valvestructures and identify associated congenital anomalies. Earlysurgical correction is preferred before mitral regurgitationcauses unfavourable remodelling. Most mitral valve cleft defectscan easily be repaired by suturing the edges of the cleft. Ifa cleft resection leads to limited residual valve tissue, theleaflet of the mitral valve can be reconstructed using an autologouspericardial patch pre-treated with buffered glutaraldehyde.Posterior CMVL is an uncommon but clinically important causeof mitral insufficiency. Early recognition of this rare clinicalentity and possible co-existent anomalies can identify the patientswho would benefit from surgical intervention before compensatoryleft ventricular remodelling and contractile dysfunction develop.     

Isolated cleft mitral valve: a variety of congenital mitral regurgitation identified by 2-dimensional echocardiography

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.     

Double orifice mitral valve associated with endocardial cushion defect

Duplication of the mitral valve is a rare congenital cardiac anomaly. We encountered a case of duplication of the mitral valve associated with a partial form of endocardial cushion defect in a 6-year-old girl. The mitral orifice was separated by a fibrous tissue and each orifice provided papillary muscle and complete subvalvular mechanisms except for the cleft region. The fibrous tissue also provided a subvalvular apparatus. The cleft was repaired without complication. The short axis view of the two-dimensional echocardiogram demonstrated two separate holes in the mitral valve, which constituted the most obvious diagnostic sign prior to surgery.     

Usefulness of transthoracic freehand three-dimensional echocardiography for the evaluation of mitral valve prolapse

BACKGROUND: Two-dimensional (2D) echocardiography is routinely used in evaluating patients with mitral valve prolapse but requires a systematic examination for accurate assessment of the involved lesion of mitral valve prolapse, because the sonographer is required to mentally reconstruct two-dimensional images into three dimensions. Recently, freehand three-dimensional (3D) echocardiography has been introduced in the clinical setting for three-dimensional visualization of the mitral valve apparatus. OBJECTIVES: To evaluate the accuracy of the freehand 3D echocardiography system in assessing the involved lesion in patients with mitral valve prolapse. METHODS: This study consisted of 25 consecutive patients (15 men, 10 women, mean age 55 +/- 17 years) with mitral valve prolapse who were scheduled for 3D echocardiography. Mitral valve was reconstructed in the view from the left atrium (surgeon's view) by 3D echocardiography. The location of the involved lesion in mitral valve was classified as the medial, middle and lateral portions of the anterior leaflet, and the medial, middle and lateral scallops of the posterior leaflet, respectively. The results by 3D echocardiography were compared with those of 2D echocardiography as the clinical standard. RESULTS: An adequate three-dimensional display of the entire mitral valve for analysis of the involved lesion could be reconstructed in all 25 patients (feasibility 100%). The sensitivity of 3D echocardiography for detecting the lesions at the medial, middle and lateral portions of the anterior leaflet was 80%, 100% and 75%, and the medial, middle and lateral scallops of the posterior leaflet was 100%, 100% and 0%, respectively. The specificities were 100% at all locations in the mitral valve. CONCLUSIONS: These results indicate that freehand 3D echocardiography is useful for assessment of the involved lesion of the mitral valve in patients with mitral valve prolapse.     

Congenital mitral valve anomalies in transposition of the great arteries

In 165 hearts with transposition of the great arteries, including 16 with a ventricular septal defect and overriding pulmonary trunk and 5 with a so-called posterior transposition, the left ventricle was studied with emphasis on the morphology of the mitral valve. Distinct mitral valve anomalies were found in 36 cases (22 percent), and four categories of anomalies could be identified. Group A Included 16 specimens with a cleft anterior mitral valve leaflet. The cleft was complete or partial. Partial clefts continued as a fibrous cord within the leaflet. In eight cases the cleft was situated posterior or lateral to the pulmonary ostlum; in these cases the left ventricular outflow tract was not narrowed. Severe outflow tract stenosis was present in another eight cases in which the cleft was located anterior to the pulmonary ostlum, usually in combination with a ventricular septal defect, and in four of these specimens there was straddling of the mitral valve.

Group B included eight hearts with an abnormal size or position of the mitral valve, or both, the valve being hypoplastic or rotated clockwise, or both. Group C comprised seven hearts showing redundant left ventricular structures involving the mitral valve, among which were anomalous tissue strands, subpulmonary rings and redundant valve tissue. Group D included five specimens with deficient papillary muscles.

It is concluded that an abnormal mitral valve is not unusual in hearts with transposition of the great arteries. The findings are notably Important for those patients for whom anatomic surgical correction of the transposition is considered. In comparison with the venous baffle procedure, this operation makes greater demands on the structure of the mitral valve because the pressure in the left ventricle remains at systemic level. Thorough Investigation of mitral valve anatomy and function is necessary before anatomic correction is considered.     

Mitral regurgitation with an isolated anterior mitral leaflet cleft: a case report.

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.     

A case of accessory mitral valve leaflet associated with solitary mitral cleft.

Accessory mitral valve leaflet is a rare congenital anomaly. More than half of the cases show other congenital cardiac defects and almost all of the cases show subaortic obstruction. We report a case of an accessory mitral valve tissue without outflow obstruction associated with mitral cleft of the posterior mitral leaflet. To our knowledge, this is the first reported case of the combination of these two congenital anomalies.     

Multiplane two-dimensional versus real time three-dimensional transesophageal echocardiography in ischemic mitral regurgitation

Objectives: Intraoperative three‐dimensional (3D) transesophageal echocardiography (TEE) has been suggested to be a valuable technique for the evaluation of the mechanisms of ischemic mitral regurgitation (IMR). Studies comparing multiplane two‐dimensional (2D) with 3D TEE reconstruction of the mitral valve using the new mitral valve quantification (MVQ) software are lacking. We undertook a prospective comparison between multiplane 2D and 3D TEE for the assessment of IMR. Methods: We evaluated echocardiographically 45 patients with IMR who underwent mitral valve surgery in our institution. 2D and 3D TEE examinations followed by a 3D offline assessment of the mitral valve apparatus were performed in all patients. Offline analysis of mitral valve apparatus was conducted with QLAB–MVQ. Results: 3D TEE image acquisitions were performed in a short period of time and were feasible in all patients. Real time 3D TEE imaging was superior to 2D in identifying specific mitral scallops (A1, A3, P1, P3) and commissures. When compared with 2D TEE, 3D offline reconstruction of the mitral valve allows an accurate quantification of the shape and diameters of the mitral annulus. Both approaches provide almost similar values for the tenting area and the coaptation depth. The 3D approach gave the advantage of direct calculation of the leaflets angles, tenting volume, and surface of the leaflets. The interpapillary muscles distance at the level of the papillary muscle head was greater in 2D than in 3D. Conclusions: 3D TEE imaging provides valuable and complementary information to multiplane 2D TEE for the assessment of patients with IMR. (Echocardiography 2011;28:1125‐1132)     

Color Doppler echocardiography of isolated cleft mitral valve. Roles of the cleft and the accessory chordae.

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.     

Isolated Cleft of the Mitral Valve: Clinical Spectrum and Course

We have reviewed the clinical presentations, courses, and outcomes of 90 patients seen at Texas Children''s Hospital from 1983 through 2008 who had an isolated cleft of the mitral valve without some form of endocardial cushion defect. Additional congenital cardiac defects were present in 61 of the 90 patients, 35 of whom had a congenital syndrome. Seven patients had isolated cleft of the mitral valve without other intracardiac defects, and in these 7 there was a progressive increase in the degree of mitral regurgitation during a median time of 26.5 months from diagnosis to surgery. The patients ranged from a gestational age of approximately 32 weeks to 21.9 years of age. No death was observed among the 39 surgical patients, including 32 who had additional cardiac defects. There was a significant reduction in the degree of mitral regurgitation in all patients who underwent surgery. Among the 51 patients who did not have surgery, the degree of regurgitation did not change significantly over the course of 1 to 27 years'' observation.Isolated cleft of the mitral valve is an uncommon (but not rare) congenital malformation of the mitral valve that can cause all degrees of mitral regurgitation but can be managed medically or surgically.Key words: Adolescent, child, echocardiography, Doppler, heart defects, congenital/surgery/ultrasonography, infant, mitral valve/abnormalities/ultrasonography, mitral valve insufficiency, retrospective studiesIsolated cleft of the mitral valve, not associated with endocardial cushion defects, is an uncommon congenital anomaly of the mitral valve. Most reports^1–4 describe moderate-to-severe regurgitation of the mitral valve, which often requires surgical intervention. We reviewed our experience with this condition at Texas Children''s Hospital and found that many patients had only minor clefting of the septal leaflet of the mitral valve, which did not require surgery. With use of high-resolution echocardiography, skilled technicians are able to identify even minor clefts in the mitral valve. This condition is probably more common than previously realized.     

Real-time three-dimensional transesophageal echocardiography assessment of the mitral valve: perioperative advantages and game-changing findings

Real-time three-dimensional transesophageal echocardiography (RT-3D-TEE) represents a unique perioperative cardiovascular imaging tool which, without any need for off-line reconstruction, has been shown to be highly valuable for evaluating mitral valve and other intracardiac structures. It is highly probable that, in the near future, RT-3D-TEE will have a positive effect on the perioperative assessment of complex 3D mitral valve structures, as it provides important approaches to the pathophysiology of various mitral valve diseases, including prosthetic valves, and will become incorporated into everyday perioperative practice. The clinical applications and therapeutic implications of perioperative RT-3D-TEE in the thorough assessment of the mitral valve are briefly summarized in this review.     

Congenital submitral aneurysm with rupture into the left atrium: assessment by 2D and 3D transesophageal echocardiography

We describe two cases of congenital submitral aneurysms (SMAs) in which three-dimensional transesophageal echocardiography (3D TEE) proved useful to define the spatial extent of these aneurysms. In both cases, rupture into the left atrium was accurately delineated. 3D TEE was useful in case 1 as it depicted the precise site of rupture into the left atrium as well as pseudoprolapse of the P2 segment of the mitral valve. In case 2 it also localized the rupture into the left atrium in relation to the annulus to be adjacent to the anterolateral commissure. In addition, a cleft between the A1 and A2 scallops were identified and together with failure of the leaflets to coapt enabled the mechanisms contributing to the mitral regurgitation to be elucidated. Thus, imaging from the left atrial perspective using 3D TEE provided superior spatial anatomical delineation of the rupture and its relationship to the mitral valve, as well as accurate anatomical definition of the mitral leaflets. This information provides added benefit to the surgeon in planning a transatrial surgical repair of the SMAs. 3D TEE is superior to conventional 2D TEE in defining the spatial anatomy of SMAs as well as the mechanisms contributing to mitral regurgitation.     

Echocardiographic 3D‐guided 2D planimetry in quantifying left‐sided valvular heart disease

Echocardiographic 3D‐guided 2D planimetry can improve the accuracy of valvular disease assessment. Acquisition of 3D pyramidal dataset allows subsequent multiplanar reconstruction with accurate orthogonal plane alignment to obtain the correct borders of an anatomic orifice or flow area. Studies examining the 3D‐guided 2D planimetry approach in left‐sided valvular heart disease were identified and reviewed. The strongest evidence exists for estimating mitral valve area in patients with rheumatic mitral valve stenosis and vena contracta area in patients with mitral regurgitation (both primary and secondary). 3D‐guided approach showed excellent feasibility and reproducibility in most studies, as well as time efficiency and good correlation with reference and comparator methods. Therefore, 3D‐guided 2D planimetry can be used as an important clinical tool in quantifying left‐sided valvular heart disease, especially mitral valve disorders.     

Cleft of the mitral valve in patients with Down's syndrome

Differentiation between a cleft of the mitral valve and the cleft of the left side of an atrioventricular septal defect--a lesion commonly found in patients with Down's syndrome--is surgically important since the distribution of the conduction tissue varies between the 2 lesions. We sought to determine if cleft of the mitral valve occurs also in patients with Down's syndrome. We studied 5 patients with Down's syndrome and cleft of the mitral valve followed in our institution. Echocardiography showed in all 5 patients a cleft dividing the anterior (aortic) leaflet of mitral valve with normal papillary muscle position, mural leaflet size, and ratio of the inlet/outlet dimension of the left ventricle. Associated cardiac lesions were present in all 5 patients: perimembranous ventricular septal defect in 3, ostium secundum atrial septal defect in 2 and patent ductus arteriosus in 2 patients. During the 5.6 years (0.2-11) of the follow-up period, surgical repair of the cleft was never indicated since the mitral regurgitation through the cleft remained mild or absent in all the patients. Two patients underwent closure of a ventricular septal defect, with atrial septal defect closure in one and ductal ligation in 2. One patient died suddenly at home, without evidence of a cardiac cause. In conclusion, a cleft of the mitral valve has important developmental and morphologic differences with atrioventricular septal defect and may occur in patients with Down's syndrome. If surgical repair of the cleft or of associated cardiac lesion is indicated, it is necessary to distinguish it from atrioventricular septal defect where the conduction axis is displaced posteriorly and may be exposed during surgery.