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a AP-HP, Assistance Publique - Hôpitaux de Paris, Service de Chirurgie Cardiovasculaire, Hôpital Européen Georges Pompidou, Paris, France
b Université René Descartes, Paris V, France
Received 5 November 2007; received in revised form 5 June 2008; accepted 9 June 2008.
* Corresponding author. Address: Hôpital Européen Georges Pompidou, Service de Chirurgie Cardiovasculaire, 20, rue Leblanc 75908, Paris, France. Tel.: +33 1 56 09 37 48; fax: +33 1 56 09 22 19. (Email: rzegdi{at}hotmail.com).
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Abstract |
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 Top Abstract 1. Introduction2. Material and methods3. Results4. DiscussionReferences |
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Key Words: Valve • Repair • Congenital • Regurgitation
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1. Introduction |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionReferences |
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Congenital MVR is primarily a disease seen in infants or children. Many case reports of MVR due to congenital mitral valve lesions (such as mitral cleft or mitral hammock valve) have been reported in adult patients [5–11]. However, to the best of our knowledge, there are no data available regarding the incidence, the pathology, the feasibility and the results of MVRep in congenital MVR in this setting.
In this paper, we describe our surgical experience of congenital MVR in adults during a 6-year period.
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2. Material and methods |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionReferences |
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All but one patient were in functional class NYHA I–II. Thirteen patients were in sinus rhythm whereas a history of atrial fibrillation was present in six (40%) patients. Preoperative echocardiography revealed severe MVR in all instances. Left ventricular ejection fraction was 
60% in 13 (87%) patients. Pulmonary hypertension (>50 mmHg) was detected in two (13%) patients.
All the operations were performed through a median sternotomy on full cardiopulmonary bypass between the two vena cava and the ascending aorta. Mild systemic hypothermia (28–30 °C) was used in all cases. Myocardial protection was identical for all patients and consisted in an anterograde cold blood crystalloid cardioplegia. Mitral valve exposure was achieved through a standard left atriotomy along the interatrial groove.
Based on surgical analysis, the functional classification was type I (one patient), type II (five patients), type IIIb (one patient), type I + II (five patients), type II + IIIa (two patients) and type II + IIIb (one patient). Operative findings of the mitral valve are shown in Table 1 . Coexistence of several lesions in a same patient was observed in all but one patient. There were no other cardiac lesions associated to the mitral valve disease.
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Long-term follow-up data were obtained through questionnaires and telephone contacts with patients or relatives, physicians and cardiologists. Long-term results were assessed on the basis of NYHA functional class, electrocardiogram and echocardiography. Cardiac rhythm, thromboembolic or bleeding events, endocarditis and reoperations were systematically recorded.
Data were expressed as median (range) for continuous variables and as percentage for categorical variables.
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3. Results |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionReferences |
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On echocardiography, at discharge, 14 (93%) patients had no or trivial regurgitation. MVR was considered mild in one (7%) patient. There was no residual mitral valve stenosis. Mean transmitral valve diastolic gradient was 3 mmHg (range, 2.5–5 mmHg). Left ventricular ejection fraction was less than 50% in four (27%) patients.
Follow-up was complete in all patients. Median follow-up was 60 months (range, 6–83 months). There was no late mortality. All patients were in NYHA functional class I–II and in sinus rhythm. Last Doppler echocardiography study as part of routine follow-up revealed no or trivial mitral regurgitation in 11 (73%) patients and mild regurgitation in 4 (27%) patients. Mean transmitral diastolic gradient was 3.1 mmHg (range, 2–4.5 mmHg). Left ventricular ejection fraction was less than 50% in three (20%) patients.
There was no thromboembolic or hemorrhagic episode during the follow-up period. Endocarditis did not occur and all patients remained free from reoperation.
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4. Discussion |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionReferences |
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Congenital MVR is a well-known but infrequent disease in a pediatric population. When severe enough, mitral valve surgery is mandatory owing to the poor prognosis of the disease, responsible for left ventricular failure or pulmonary hypertension [2,3,13]. Several large surgical experiences have been reported [1–3,13]. MVRep is feasible in most cases and desirable owing to its satisfying long-term results contrasting with the poor results of mitral valve replacement [2,4].
Congenital MVR is a rare disease in adults, and to the best of our knowledge, no series have been reported to date. In our experience, congenital MVR represented 2.1% of all adult patients undergoing mitral valve surgery. The rarity of the disease in adults and its ignorance might explain why the diagnosis was not made preoperatively in 60% of cases. Even when a mitral cleft was surgically identified, this lesion was often misdiagnosed (three cases out of five).
A classification of the lesions encountered in pediatric congenital MVR has been proposed by Carpentier et al. [14]. These lesions may affect any level of the mitral valve subvalvular apparatus ‘complex’. Except for the parachute valve and the commissural fusion syndrome, all the lesions previously described in infancy or childhood were also found in our adult population.
Valvular prolapse was the leading cause of MVR in the present series (13 patients, 87%). In other pediatric series, the incidence of valvular prolapse ranged from 44% to 55% [1,2]. Valvular prolapse is also the main mechanism of regurgitation in various conditions like degenerative mitral valve disease and endocarditis. Therefore, identifying the congenital origin of a valvular prolapse in adults may be difficult in some instances. The decision is relatively easy when the valvular prolapse coexists with a specific condition (Williams–Beuren syndrome, for example) or a specific lesion (valvular cleft, for example). In the other cases, a careful analysis of the subvalvular apparatus may reveal uncommon pathologic findings that can only be of congenital origin. In our experience, these findings included aberrant chordae (spanning from a valvular segment to an adjacent one) and malpositioned or malformed papillary muscles. Malformation in our series (four cases) always consisted of a severely hypotrophic posterior papillary muscle.
Isolated cleft mitral valve is a well-known congenital abnormality usually diagnosed during infancy or childhood. Late diagnosis (at the time of surgery) has already been reported in adults [10]. The cleft almost always involves the anterior leaflet but rare cases of posterior or bileaflet cleft have also been published [10]. The present series included five patients with a mitral valvular cleft. In three cases, the cleft was typically anterior. In the two other cases, the cleft involved the mid part of P2 (Carpentier's classification), giving to P2 a ‘bifid’ aspect. These lesions were totally different from common marked indentations between P1 and P2 or P2 and P3.
There are several important differences between adults and children regarding congenital MVR. In pediatric series, many patients were in NYHA functional class III or IV (68% for Chauvaud et al.) [2]. This was the case in only one (7%) patient in our series. This may be explained by the fact that the natural history of severe mitral regurgitation is better known in adults and that early intervention in experienced centers has been recommended [15]. Conversely, in children, and more specifically in infants, surgery may be delayed especially if MVRep is deemed to be difficult.
In pediatric series, preoperative left ventricular failure or pulmonary hypertension is more frequent. This is partly due to the frequent coexistence of other cardiac lesions (such as septal defect) up to 83.7% in the series of Ohno et al. [3]. In our practice, for many years, MVRep has been performed as early as possible provided MVR was considered to be severe. Thus, all but one patient in our series were pauci- or asymptomatic, with usually preserved left ventricular ejection fraction and no pulmonary hypertension. Consequently, 5-year survival was 100% contrasting with a 5-year survival in pediatric series of 87.2% for Yoshimura et al. [1] and 93% for Chauvaud et al. [2], despite the older age of our patients.
MVRep for congenital MVR in adults has already been reported in many case reports [5–11]. However, the feasibility of MVRep in a non-selected cohort of adult patients with congenital MVR has not been fully determined so far. In the present series, MVRep was performed in all patients with excellent results in the postoperative period and at mid-term follow-up (no MR grade 2+ at a median follow-up of 60 months). The feasibility of MVRep depends on two factors (‘the golden rules’): that is, the availability of a sufficient amount of leaflet tissue, and for the tissue to be pliable (non-fibrotic or non-calcified). These two prerequisites are present in congenital MVR explaining the potentially high rate of MVRep in this condition.
The prosthetic annuloplasty is perceived as an important factor for the reproducibility of the repair as well as its stability on the long run. Proper sizing depends on the height of the anterior leaflet [12]. In our experience, all patients have had a prosthetic annuloplasty. In pediatric series, the rate of prosthetic annuloplasty varied from 0% to 48% [1,2]. This lower rate may be explained by the fear of early reoperation due to mitral valve stenosis (secondary to somatic growth) when the small height of the anterior leaflet would have imposed the choice of a small prosthetic ring.
In conclusion, congenital MVR is a rare disease in adult patients. Lesions are usually the same as those described in pediatric series and are often misdiagnosed preoperatively. MVRep is feasible in a high percentage of cases (100% in the present series) with excellent immediate and mid-term results (100% freedom from grade 2+ mitral regurgitation at 5 years).
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References |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionReferences |
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