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a Service of Pediatric Cardiology, Centre Hospitalo-Universitaire La Timone, Marseille, France
b Service of Thoracic and Cardiovascular Surgery, Centre Hospitalo-Universitaire La Timone, Marseille, France
Accepted for publication June 1, 2009.
* Address correspondence to Dr Fraisse, Cardiologie pédiatrique, Hôpital d'enfants de la Timone, 264 rue Saint Pierre, Marseille Cedex 5, 13385, France (Email: alain.fraisse{at}ap-hm.fr).
| Abstract |
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Methods: Patients with surgical repair of isolated cleft of the mitral valve were included.
Results: Ten patients (9 female) underwent repair at a mean age of 12.1 ± 10.5 years and mean weight of 32.1 ± 17.8 kg. Preoperative echocardiography showed mild or less than mild mitral regurgitation in 6 cases and moderate to severe regurgitation in 4. Intraoperative examination confirmed in all cases a cleft dividing the anterior leaflet of an otherwise normal mitral valve. Attachment of the cleft to the ventricular septum by accessory chordae was found in 3 cases whereas preoperative echocardiography found such attachments in 5. Direct suture of the cleft was performed in 9 cases, associated with repair of tricuspid valve straddling (n = 1), subaortic stenosis (n = 1), and ventricular septal defect (n = 1). One patient with thickened cleft's edges required an Alfieri-type repair. After a mean follow-up of 4.9 years (range, 1.3 to 11.9), all patients are asymptomatic without significant mitral regurgitation.
Conclusions: Echocardiographic description of isolated cleft of the mitral valve is not always as accurate as intraoperative analysis. This is a distinct morphologic entity from the cleft of the left-sided valve of atrioventricular septal defect, and seems associated with a strong female predominance, with various cardiac and extracardiac features. Surgical repair is successful with excellent midterm results.
| Introduction |
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Also, previous studies failed to provide precise information about the indication and timing for surgery as well as its long-term results. In the largest published series of 22 ICMV patients with echocardiographic or intraoperative analysis, we confirmed the important morphologic differences between ICMV and AVC type cleft [3]. Furthermore, surgical repair seemed to be always possible with a good functional result. However, the morphologic description of the ICMV relied mainly on echocardiographic data, and fewer than half of the patients had surgical repair, with only short-term follow-up.
To further clarify the morphologic features of ICMV, its surgical management, and its midterm results, we retrospectively studied all the patients with ICMV undergoing surgical repair in our center.
| Material and Methods |
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We recorded clinical data (age, weight, and symptoms), as well as chest roentgenogram and electrocardiogram (ECG) interpretations. Preoperative two-dimensional transthoracic echocardiography (TTE) tape or compact disk were reviewed with the specific goal to describe the mitral clefts, chordal attachments, and papillary muscles, measuring mitral and tricuspid annuli, and evaluating left and right ventricular sizes and functions. Preoperative mitral regurgitation (MR) was evaluated semiquantitatively and graded as absent, trivial, mild, moderate, or severe according to the jet width at its origin and area as seen by color Doppler flow mapping. All echocardiographic descriptions were compared with surgical findings. Other congenital heart malformations were collected as well as extracardiac anomalies. Five patients included in the present study have previously been described: 4 in a previous large study that was based partly or totally on echocardiographic diagnosis only [3], and 1 as a case report [11].
Intraoperative Analysis
Surgery was performed at our institution through sternotomy or right posterolateral thoracotomy with standard procedures of extracorporeal circulation and aortic cross-clamping in all cases. After opening the left atrium, the mitral valve was exposed and carefully inspected by the surgeon to precisely describe the ICMV. Intraoperative pictures or videos were taken in the majority (n = 9) of the cases. Anatomic findings and procedural timings were recorded.
Follow-Up
Clinical and echocardiographic evaluations were performed postoperatively and every 6 to 12 months during follow-up. Transthoracic echocardiography looked for mitral stenosis or residual MR, which was graded as previously described.
Statistical Analysis
Results are expressed as mean value ± SD, or median value (range) when appropriate.
| Results |
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200 ms). Median QRS axis was 45 degrees (range, –35 to 90 degrees). The oldest patient of our series, with severe MR, experienced atrial fibrillation before surgical repair. Sinus rhythm was successfully restored with amiodarone.
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Follow-Up
Postoperative course was uneventful in all cases. All patients were extubated within 24 hours, and there were no postoperative complications. Postoperative TTE showed no MR in 6 patients and trivial regurgitation in 4 patients. No patient had mitral stenosis. After a mean follow up of 4.9 years (range, 1.25 to 11.9), all patients are asymptomatic with a normal physical examination. Echocardiography demonstrates an excellent midterm surgical result, with no residual MR in 6 children and trivial MR in 4. No late complications occurred.
| Comment |
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Morphologic Considerations
From a morphologic standpoint, ICMV is a different entity from AVC. Whereas previous postmortem or echocardiographic analysis assimilated ICMV to partial AVC [10], no features of AVC were found in our series. Our combined echocardiographic and intraoperative analysis showed that the size of the left mural leaflet, the position of the posteromedial papillary muscle, and the left ventricular inlet/outlet ratio were normal, as previously stated by others [4, 6]. Our description of ICMV also agrees with Anderson's writing more than 2 decades ago: the cleft appears very much like an "artificial cleft produced in a normal valve with a scalpel" [12]. To further enhance this morphologic distinction between AVC and ICMV, the median QRS axis on the ECG of our patients clearly differed from the classic left QRS axis deviation in AVC defects.
A recent postmortem study analyzing cases with cleft of the MV and normally related great arteries reported AVC features in all cases [10]. Clearly, AVC without ostium primum exists independently of ICMV and is even possibly more frequently encountered. However, natural history and postoperative outcome of ICMV and AVC are different as well. Isolated cleft of the mitral valve is a simple cardiac congenital defect: apart from the cleft, cardiac anatomy is either normal or associated with simple malformation. The likelihood to survive even without surgery is very high. Similarly, surgical repair of ICMV carry a very low risk. Conversely, AVC comprises a wide spectrum of defects, some of which are potentially lethal or with a greater operative risk (eg, umbalanced ventricles, multiple outflow tract obstructions). We suggest that postmortem specimen of ICMV is not frequently encountered simply because patients with ICMV are seldom at risk for death. The exception is when complex congenital heart diseases are associated, especially in the case of ventriculoarterial discordance [10, 13].
Diagnosis of ICMV and Pathogenic Considerations
The female predominance (90%) is a surprising finding in the present study that was not noted in previous reports, including those from our institution [3, 4, 9, 10, 13]. This bias may represent a male lethal effect during pregnancy, in which only females can compensate for the deleterious effects of a missing or mutated copy of a causative gene. We speculate that this effect is likely to be gene-dosage dependant, with affected males exhibiting defects so severe as to cause early fetal demise. Similar effects have been well described in other studies [14]. Yet no syndromes with exclusive heart disease are know to exhibit male embryonic lethality. Moreover, this female predominance was not shown in most of the previous studies on ICMV that were based partly or totally on echocardiographic diagnosis only [3, 4, 9, 10, 13], and that raises the concern of the diagnosis accuracy and a possible mismatch between patients with ICMV and "forme fruste" of AVC septal defect when the diagnosis is only based on echocardiographic features. In agreement with the potential inaccuracy of echocardiography for ICMV diagnosis and description is the postmortem study by Van Praagh and colleagues [10]. In that report, the cleft was only diagnosed by echocardiography in 62% of cases (8 of 13) [10].
Our study is the first to really confront echocardiographic and intraoperative findings in all patients. We suggest that the sensitivity of echocardiography is high, at least when it is associated with mild or more than mild MR. During the entire inclusion period, ICMV was never diagnosed intraoperatively without being diagnosed preoperatively by echocardiography. However, ultrasound screening is not always accurate for the precise description of ICMV. We experienced numerous errors with the echocardiographic description of chordal attachments (2 of 5) and in the morphology of the MV. Interestingly, we found, as did other authors [9], that transesophageal echocardiography did not provide more precise information about ICMV morphologic assessment and chordal attachments. Conversely, other authors claimed that echocardiography can accurately describe ICMV. In a study only based on echocardiographic findings, Khol and associates [4] reported that "Accessory chordae arising from the cleft leaflet commissure and attaching to the ventricular septum were a constant finding in our patients. Without such attachment, the divided anterior leaflet would be flail" [4]. Based on our findings that the majority of the ICMV do not have septal attachments, such a statement seems incorrect. Consequently, the spectrum of extracardiac features that was reported in previous studies that only rely on echocardiography may not be accurate.
Of interest, 1 patient in the present study had Down syndrome, as this is frequently encountered in AVC patients [15]. However, Down syndrome has already been reported in other series of ICMV [3, 9], as well as in postmortem series of cleft mitral valve without ostium primum defect [6, 10]. That raises the hypothesis of a common genetic link between ICMV and AVC. Although the two entities represent different phenotypes, they are likely to have common genetic underpinnings, as was suggested in our previous study [3]. If further advances in genetic analysis confirm this hypothesis in the future, it may have important implications for prenatal diagnosis and counseling.
Surgical Considerations
Our series confirmed that surgical treatment of ICMV associated with MR can be carried out without mitral valve replacement [9, 16], even in the presence of moderate to severe MR, as was the case in 4 of our patients. As a matter of fact, repair with a direct suture of the cleft seems to be feasible in the majority of the cases. Of concern is the lack of valvular tissue due to retraction of the cleft's edges that potentially increases with time. For patients with severe MR due to wideness of the cleft, Perier and coworkers [16] reported the need of an additional pericardial patch to accomplish a successful cleft suture. In our series, only 1 patient with severe MR and important retraction of the cleft's edges could not be repaired though direct suture and needed Alfieri-type repair. Interestingly, patients were operated on at a younger age in our study than in the Perier study, with possibly less retraction of the cleft edges by a long-standing MR. Finally, ICMV repair is a straightforward operation in the majority of the cases and can be done with short cardiopulmonary bypass and aortic cross-clamp times. The approach can be very esthetic and minimally invasive with a short posterior right thoracotomy before breast development, or alternatively with an anterolateral submammary incision. The risk for reoperation or other morbidity seems low, as opposed to AV septal defect repair [17]. Although we cannot comment on the long-term fate of repaired ICMV, our study provides midterm follow-up showing that initial good results endure, with no increase of postoperative MR at midterm TTE evaluations. Patients operated on later in life may have more retracted cleft edges and may consequently experience a more complicated surgical repair with interposition of patches on the mitral valve [16], which can presumably impair the long-term results. All this suggests that surgical repair should be indicated for cases of mild or more than mild MR, even for asymptomatic patients. For patients with trivial or no significant MR, elective MV repair cannot be recommended, but careful follow-up is warranted.
Limitations of the Study
This study has the inherent limitations of its retrospective nature. Our series of 10 patients is a homogeneous but small one. We cannot fully assess the accuracy of echocardiography since only the patients in whom ICMV was diagnosed by surgery were included. Hence, although none of the surgeons of the team remembers such a case, we cannot comment on potential false positive diagnosis of ICMV by echocardiography for which intraoperative analysis demonstrated AVC variant.
In conclusion, through echocardiographic and intraoperative description, we show that the echocardiographic description of ICMV is not always as accurate as intraoperative analysis. We also confirm that ICMV is a distinct morphologic entity from the cleft of the left-sided valve of AV septal defect. It seems characterized by a strong female predominance, and various cardiac and extracardiac features may be associated. Surgical repair of ICMV is safe and effective, especially when this is accomplished before valve deterioration, with excellent midterm results.
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R. T. Collins II, S. Peyvandi, and M. S. Cohen Atrioventricular Valve Dyssynchrony Resulting From Severe Mitral Regurgitation Circulation, February 15, 2011; 123(6): 686 - 690. [Full Text] [PDF] |
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