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J Thorac Cardiovasc Surg 2007;133:1226-1233
© 2007 The American Association for Thoracic Surgery
Surgery for Acquired Cardiovascular Disease |
Klinik für Herzchirurgie, Universitaetsklinikum Schleswig-Holstein, Campus Luebeck, Luebeck, Germany.
Received for publication August 10, 2006; revisions received January 10, 2007; accepted for publication January 23, 2007. * Address for reprints: Professor Dr med Hans-H. Sievers, Klinik für Herzchirurgie UKSH, Campus Luebeck, Ratzeburger Allee 160, 23538 Luebeck, Germany. (Email: h.sievers{at}herzchirurgie-luebeck.de).
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Abstract |
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 Top Abstract IntroductionPatients and MethodsResultsDiscussionReferences |
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Methods: Between 1999 and 2003 a precise description of valve pathology was obtained from operative reports of 304 patients with a diseased bicuspid aortic valve. Several different characteristics of bicuspid aortic valves were tested to generate a pithy and easily applicable classification system.
Results: Three characteristics for a systematic classification were found appropriate: (1) number of raphes, (2) spatial position of cusps or raphes, and (3) functional status of the valve. The first characteristic was found to be the most significant and therefore termed "type." Three major types were identified: type 0 (no raphe), type 1 (one raphe), and type 2 (two raphes), followed by two supplementary characteristics, spatial position and function. These characteristics served to classify and codify the bicuspid aortic valves into three categories. Most frequently, a bicuspid aortic valve with one raphe was identified (type 1, n = 269). This raphe was positioned between the left (L) and right (R) coronary sinuses in 216 (type 1, L/R) with a hemodynamic predominant stenosis (S) in 119 (type 1, L/R, S). Only 21 patients had a "purely" bicuspid aortic valve with no raphe (type 0).
Conclusions: A classification system for the bicuspid aortic valve with one major category ("type") and two supplementary categories is presented. This classification, even if used in the major category (type) alone, might be advantageous to better define bicuspid aortic valve disease, facilitate scientific communication, and improve treatment.
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Introduction |
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TopAbstractIntroductionPatients and MethodsResultsDiscussionReferences |
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Bicuspid aortic valve (BAV) is the most common congenital cardiac anomaly, with an estimated incidence of 0.9% to 2% in the general population.1,2 The term "BAV" includes different morphologic phenotypes3-6 presenting with different hemodynamic conditions. The "purely" BAV is composed of two cusps, morphologically and functionally. However, the most frequent form of a BAV consists of three developmental anlagen of cusps and commissures instead of two. Associated with a certain proportion of BAVs is a dilatation of the ascending aorta, especially in young patients,7 exposing these patients to an increased risk of comorbidity owing to aneurysm formation and dissection. In recent years, BAV has gained increasing interest in research and treatment. Owing to improved knowledge of aortic valve anatomy and function, more refined reconstructive techniques are applied to the different phenotypes of BAV. A systematic classification of BAV phenotypes seems advantageous to more precisely describe BAV, to better relate valve morphologic characteristics to surgical intervention, to gain more insight into the association of BAV with other disease of the ascending aorta,8 and to allow more appropriate comparison of the different reports on the BAV. The presented classification system is based on our surgical experience with BAVs over a 5-year period. |
Patients and Methods |
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TopAbstractIntroductionPatients and MethodsResultsDiscussionReferences |
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Patient age at operation, gender, functional state of the patient (according to New York Heart Association classification), concomitant vascular diseases, and cardiac procedures, as well as the functional state of the aortic valve, were collected from the medical records.
Functional State of the BAV and Indications for Operation
The functional state of the BAV was determined before the operation by echocardiography and/or angiocardiography. The severity of aortic stenosis was assessed echocardiographically by the maximal pressure gradient (= 4 x v
2 ([m/s], where v is the peak systolic transvalvular velocity) and by the aortic valve area (= [CSA
LVOT x VTILVOT]/VTIAS, where CSA is cross-sectional area, LVOT is left ventricular outflow tract, VTI is velocity time integral, and AS is aortic stenosis). Aortic stenosis was also assessed angiographically by measuring the transvalvular pressure gradient directly and by the orifice area according to Gorlin and Gorlin.
An aortic stenosis was defined as severe if presenting with an orifice area of 1 cm2 or less or a mean pressure gradient of 50 mm Hg or more. Aortic insufficiency was assessed by color flow Doppler techniques and graded by the ratio of jet height to left ventricular outflow tract height. Grade III aortic insufficiency (47% to 64% of the aforementioned ratio) was considered an indication for operation in conjunction with the presence of symptoms and left ventricular size and function. Patients with severe aortic stenosis and symptoms or patients with an orifice area less than 0.7 cm2 were considered candidates for operative intervention. If aortic stenosis was combined with aortic insufficiency and both of moderate degree, indicating an operation, this valve condition was called a balanced lesion.
Definition of BAVs
In this study the term "BAV" stands for congenital bicuspid aortic valve disease comprising a spectrum of deformed aortic valves presenting on gross examination with two functional cusps forming a valve mechanism with less than three zones of parallel apposition between cusps. Thus, abnormal aortic valves with two raphes resulting in a restricted orifice area that extends from the periphery to the center were included in the BAV group (type 2, valve with two raphes) and not considered to be unicuspid, unicommissural, or monocuspid as described earlier for similar valve diseases.9-11 This is supported by the similarities in anatomy of BAV type 2 (valve with two raphes) to BAV type 1 (valve with one raphe).
Although the term "cusp" is a description more of a tip than an area and the term "leaflet" may be more appropriate, the term "cusp" is commonly used for the aortic valve and furthermore defines pathologic entities like the bicuspid and quadricuspid aortic valve. Therefore, we retain the term "cusp." The term "raphe" defines the conjoint or sometimes called "fused" area of the two underdeveloped cusps turning into a malformed commissure between both cusps (Figures 1 and 2).
These raphes can be developed partially or totally. The characteristic appearance of a raphe is a ridge with many elastic fibers.12 The commissure of the aortic valve is the space between the two coronet-shaped, lateral, and parallel attachments of two adjacent cusps to the aortic wall normally not adhering to each other and is different from the zone of apposition between the free edges of adjacent cusps. In BAV, however, an obliteration of the commissural area is present (Figure 2).
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to a more or less underdevelopment of one or two commissures and the adjacent cusps, which occurs in the majority of cases presenting with one or two raphes (Figures 1 and 5). In these cases the developmental anlagen of three cusps, commissures, and sinuses can be identified. Taken together, there are certain different morphologic characteristics of a BAV that allow, on gross examination, separation of the BAV from other aortic valve phenotypes (Figure 3), as already addressed in part by Osler13 in 1886.
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The second subcategory is determined by the functional status of the valve: predominant insufficiency (I), predominant stenosis (S), balanced insufficiency and stenosis (B), or no insufficiency and stenosis (No).
A missing or nonclassifiable subcategory is presented by an "X."
Thus, the classification system of a BAV may be presented in three blocks—type, spatial position, and valvular function—using the aforementioned codifications.
Statistical Analysis
Categorical data are given as total numbers and relative frequencies. Continuous data are given as mean ± standard deviation. Characteristics for patients of different groups were compared by the Fisher exact test for categorical variables and the U test and Kruskal–Wallis test for continuous variables. Statistical analyses were performed with statistical software SAS (SAS 8.2; SAS Inc, Cary, NC).
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Results |
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TopAbstractIntroductionPatients and MethodsResultsDiscussionReferences |
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The underlying aortic valve disease was an insufficiency in 117 (38.5%), stenosis in 156 (51.3%), balanced combined valve disease in 27 (8.9%), and active endocarditis in 15 (4.9%) patients; 4 of these were without valve dysfunction but had large vegetations.
Aortic aneurysms (diameter > 5 cm) were present in 90 (29.6%) patients, with involvement of the aortic root in 18 (5.9%), ascending aorta in 88 (28.9%), aortic arch in 2 (0.7%), and descending aorta in 1 (0.3%). A significantly higher proportion of aneurysms of the ascending aorta was present in BAV type 2 (valve with two raphes; P = .022; Figure 7).
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in 5 (1.6%), and remodeling technique according to Yacoub and associates15 in 3 (1.0%). There was no difference in coronary artery disease between types of BAVs (33% for type 0 [valve with no raphe], 29% for type 1 [valve with one raphe], and 14% for type 2 [valve with two raphes]).
Concomitant procedures were performed as follows: coronary artery bypass grafting in 43 (14.1%), maze procedure in 4 (1.3%), left ventricular myectomy in 7 (2.3%), mitral valve reconstruction in 16 (5.3%), closure of a patent foramen ovale in 3 (1.0%), and closure of a ventricular septum defect in 1 (0.3%).
Classification of Surgical Specimens
The results are summarized in detail in Table 1. There were 21 (7%) patients with BAV Type 0 (valve with no raphe). One of these specimens with predominant valvular stenosis could not be categorized into the first subcategory owing to lack of specification of the orientation of cusps in the operative report, resulting in the codification type 0, X, S. Most patients (n = 269, 88%) had BAV type 1 (valve with one raphe). Within this group the predominant finding was type 1, L/R, S (n = 119, 39%), which means a BAV with one raphe between the left and right coronary sinuses and with valvular stenosis. There were 14 patients with BAV type 2 (valve with two raphes).
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Discussion |
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TopAbstractIntroductionPatients and MethodsResultsDiscussionReferences |
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and has gained increasing interest in the medical community from a pathogenetic and especially surgical point of view.19,20 The BAV represents a certain segment of the developmental spectrum of aortic valve phenotypic continuity. Developmentally, the BAV is not simply the fusion or nonseparation of two cusps but a more complex process involving the cusps, sinuses, commissures, position of the coronary orifices, and possibly the texture of the wall of the ascending aorta. BAVs are not equal among each other in morphologic, positional, and functional aspects, which is of significance for treatment. We found the number of raphes to be the most practical and readily recognizable distinctive characteristic of BAVs, representing the main category of this classification, called "type." Typically, no raphe is present in the so-called "purely" BAV with two completely developed cusps and commissures (type 0), which is, however, the case in the minority of patients (7% in this series); this is comparable with the results of Angelini and colleagues.21 In this configuration, valve implantation in general, and especially the Ross procedure, is more challenging, and a circular, not scalloped, proximal suture line seems preferable. In addition, the vis-à-vis orientation of the coronary ostia requires special consideration when arranging the three new commissures of the autograft. In roots with this kind of unfavorable anatomy, it is sometimes advisable not to perform a Ross procedure.
In BAV type 1 (valve with one raphe), there are two smaller malformed cusps bearing the conjoint part or raphe and one larger cusp, most frequently the noncoronary. The commissure between both underdeveloped cusps is also malformed. This is, however, of no major surgical concern because the surgical annulus for implantation of prostheses and also for the Ross procedure is largely maintained (Figure 2) or even flattened to a more ringlike configuration in relation to the extent of malformation of this commissural area. The surgical annulus is composed of the proximal part of the semilunar attachments of the cusps to the wall, extending from the nadir of the sinuses to the point where this line approaches that of the adjacent cusp.22 The larger noncoronary annulus and sinus can easily be matched by a plication suture at the nadir and/or the sinus during closure of the aortotomy. Even in BAV type 2 with two raphes, three sinuses are identifiable, facilitating the pulmonary autograft procedure or conventional valve replacement.
In patients with an insufficient BAV type 1 (valve with one raphe), usually the two conjoint leaflets with the raphe are prolapsing, giving the appearance that there is some cusp tissue missing at the area of the raphe. This can be replaced by prosthetic material or pericardial tissue, but preferably the raphe is plicated or resected and thus the cusps are raised up to the coaptation area of the nonprolapsing cusp. This procedure, however, produces inevitably some degree of stenosis, which seems not to be of clinical significance even at exercise.23 Nevertheless, the deficiency of cusp tissue may require other surgical techniques, such as annuloplasty or cusp extension, to increase coaptation area or elevation of commissures.
In BAVs type 0 (valve with no raphe), plication of the prolapsing cusp also restores coaptation area. Long-term results of reconstruction of insufficient BAV type 1 (valve with one raphe) and type 0 (valve with no raphe) are likely different partly as a result of different morphologic characteristics.
The first subcategory is related to the position of the raphes. This is of minor direct surgical impact but may have some pathophysiologic relevance. In this respect, it is of interest that BAVs type 2 (valve with two raphes), L/R-R/N were associated with the highest incidence of aneurysms of the ascending aorta (Figure 7). The flow through this reduced opening area is directed to the convexity of the ascending aorta, where typically the aneurysms are located. Whether this increased local wall stress acts as a trigger for aneurysm formation is questionable. Probably these typical dilatations of the ascending aorta are related more to BAV intrinsic aortic disease than to hemodynamics.
The second subcategory provides information on the functional status of the valve, which is not only of diagnostic interest but also may have some prognostic information on ventricular performance, timing of operation, and operative technique.
With this classification system, it was possible to group all BAV phenotypes at least by the main category. For other studies on BAV morphology it was also possible to apply this classification system to the main category.3,4,6,21 Other authors4,5,9,21 principally found similar results concerning the distribution of purely BAVs and those with one raphe, as well as the position of the raphes. They also used the position of both the cusps and the raphes, the number of sinuses, the interleaflet triangles from below, and the cusp sizes for BAV description (Figure 8). These classifications, however, are not uniform, not widely used, and are not condensed in a concise, easily applicable, graduated classification and codification including morphology (number of raphes), spatial position of cusps or raphes, and valvular function, nor were abnormal aortic valves with two raphes included in the BAV group. From a surgical point of view especially, the number and position of raphes is easily assessable and applicable for classifying and codifying BAVs in the vast majority of patients.
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In addition, the coronary artery anatomy with a higher incidence of left main dominance7 might help the identification of a BAV in these cases. On the other hand, if there is some kind of a raphe in a "purely" BAV with two cusps, which is the proposed type 0 (valve with no raphe), there is no category in the described system for classifying this valve. However, these cases are extremely uncommon and may be described separately.
It is imaginable that additional subcategories could even more precisely specify the valve, for example, size of cusps,4 the concomitant existence of an ascending aortic aneurysm, extension and size of raphes, microscopic details, genetic markers, heritability signs,24 and annulus dilatation, just to mention some. However, these additional items would make the classification system more complex and probably less practical.
We believe that the proposed classification, with the main category termed "type" alone or preferably together with the two subcategories, would yield advantages to more precisely and comparably describing the BAV as a further step for improved management of this entity.
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Acknowledgments |
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References |
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TopAbstractIntroductionPatients and MethodsResultsDiscussionReferences |
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