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Evaluation of surgically excised mitral valves: Revised recommendations based on changing operative procedures in the 1990s
Perspectives in Pathology Evaluation of Surgically Excised Mitral Valves: Revised Recommendations Based on Changing Operative Procedures in the 1990s ANTHONY J. DARE, MD, PHILLIP J. HARRITY, MD, HENRY D. TAZELAAR, MD, WILLIAM D. EDWARDS, AND CHARLES J. MULLANY, MD In 1990, 95 mitral
valves from 54 women and 41 men (mean age,
61 years; age range, 8 to 85 years) were replaced (76%) or repaired (24%) at the Mayo Clinic. Functionally, 58% of the valves were purely regurgitant (MR), 25% were stenotic and regurgitant 17% were
purely
stenotic
(MS).
rheumatic) disease accounted
(MS-MR),
Postinflammatory
and
(presumably
for 100% of MS cases, 92% of MS-
MR cases, and 16% of MR cases. Other causes of pure MR included floppy valves (49%), ischemic heart disease (130/o),infective endocarditis (9%), miscellaneous
(9%), and indeterminate
(4%). Thus,
postinflammatory disease represented the major cause of both mitral stenosis (MS and MS-MR) surgical population.
and overall mitral valve disease in our
In contrast, floppy valves were the most com-
monly observed cause of pure MR. Among postinflammatory valves, 55% were completely excised and 45% had only the anterior leaflet removed; all were replaced. In contrast, floppy valves were incompletely excised in 96%; 67% were repaired
phologic
an accurate etiologic diagnosis
excised,
requires not only a mor-
assessment of resected tissues but also a knowledge
the clinical history, operative valve. Hm
PATHOL
details, and functional
24:1286-1293.
underlying etiology by gross inspection alone may now be more difficult than in the past. Moreover, in a previous study from our institution appreciable temporal changes in the various causes of mitral valve dysfunction were observed between 1965 and 1985.” Thus, the current investigation was undertaken to determine whether an accurate etiologic diagnosis could still be attained with evaluation of only portions of the mitral valve and to further define any temporal changes in the causes of mitral valve disease in patients undergoing operative replacement or repair. This represents a continuation of our ongoing study of the surgical pathology of valvular heart disease.4,6-i”
and only 33% were re-
placed. Because mitral valves frequently are incompletely rendering
MD,
Copyright
0
METHODS
of
state of the
1993 by W.B.
Saunders Company During the past 11 years several investigators have evaluated the athologic features of surgically excised mitral valves.‘--$ In general, conclusions regarding the underlying etiology have been based on gross inspection of the entire valve, removed during valve replacement procedures. In recent years, however, there has been a tendency for surgeons to remove only the anterior mitral leaflet during valve replacement and to remove only portions of the valve (usually from the posterior leaflet) during reparative procedures. As a result, determination of the
From the Department of Pathology, Prince Charles Hospital, Brisbane, Australia; and the Divisions of Anatomic Pathology and Cardiothoracic Surgery, Mayo Clinic and Foundation, Rochester, MN. Accepted for publication July 26, 1993. Presented in part at the 17th World Congress on Diseases of the Chest, Amsterdam, The Netherlands, June 1993. Key wor&: floppy mitral valve, mitral valve disease, rheumatic heart disease, surgical pathology. Address correspondence and reprint requests to William D. Edwards, MD, Hilton (1 1) Pathology, Mayo Clinic. Rochester, MN 55905. Copyright 0 1993 by W.B. Saunders Company 0046-S177/93/2412-0003$5.00/O
1286
All mitral valves that were either wholly or partially excised at the Mayo Clinic during 1990 were obtained from our tissue registry for review. All 95 specimens were evaluated grossly, as recommended by Roberts and MorrowI and Davies.15 Histologic examination was performed only when there was evidence to suggest endocarditis or another specific disease process requiring histologic confirmation (storage disease OI papillary muscle infarction, for example). For each valve the number and origin of excised fragments were recorded. Further pathologic examination, clinical data retrieval. and categorization of valve function (pure stenosis, pure regurgitation, or combined stenosis and regurgitation) were performed as described in previous studies.4.6-‘3 Based on the pathologic and clinical findings, each valve was classified as postinflammatory, floppy, or associated with ischemic heart disease, infective endocarditis, cardiomyopathy, congenital malformation, or another disorder. Valves that could not be classified confidently were considered to be of indeterminate etiology. The term “postinflammatory disease” was used to refer to a chronic noninfectious fibrosing process resulting in valvular distortion indistinguishable from confirmed cases of chronic rheumatic valvular disease. In accordance with the recommendations of Pomerance,16 such valves were not designated as “rheumatic” because a clinical history of acute rheumatic fever was not obtained in all cases and because other conditions (such as systemic lupus erythematosus, ankylosing spondylitis, rheumatoid arthritis, and radiation-induced valve disease) may produce identical morphologic changes.
SURGICAL
Etiolow Postinflam Postinflam Postinflam Floppy Ischemia Endocarditis Other Other Indeterminate
MS MS-MR MR MR MR MR MR MS-MR MR
NO. (%) 16 22 9 27 7 5 5 2 2
Male
Abbreviations:
Age of Patients
Female
M:F Ratio
Mean
Range
O-9
lo-19
F’~PPY lschemia Endocarditis Other Other Indeterminate Total
MS
MS-MR MR MR MR MR MR MS-MR MR
80-89
0 0 1 0 0 0 0 0 0
0 0 1 0 1 0 1 0
1 1 0 1 0 0 2 0 0
4 3 0 2 0 1 0 0 0
0 7 1 1 0 0 1 1 1
5 5 4 12 3 3 1 0 1
6 6 3 9 4 0 0 0 0
0 0 0 1 0 0 0 0 0
41
54
1:1.3
61
8-85
1
1
3
5
10
12
34
28
1
MS, mitral stenosis;
Postinflam,
postinflammatory
(presumably
rheumatic).
between the three functional groups (MS, 62 years; MS-MR, 59 years; and MR, 62 years). The oldest patients included those with regurgitation due to ischemic heart disease (mean age, 70 years). In contrast, the youngest patients were those with endocarditis (mean age, 52 years) and two “other diseases” (mean ages, 39 and 44 years). For all other etiologic categories the range of mean ages was 61 to 65 years (Table 1). The study included valves from 54 female and 41 male patients. Women predominated in the stenosis (MS and MSMR) groups (3: 1 ratio) and in the postinflammatory MR group (2:l ratio). In contrast, men had regurgitation more commonly due to floppy valves (1.3: 1 ratio) and to ischemic heart disease (2.5:1 ratio). All five patients with mitral incompetence due to endocarditis were males.
Relationship
of Procedure to Etiology
Seventy-two valves (76%) were replaced and 23 (24%) were repaired (Table 2). When valves were replaced the anterior leaflet was excised either alone or with part or all of the posterior leaflet (Fig 1). In contrast, when valves were repaired only a portion of the posterior leaflet or its chordae tendineae was excised (Fig 2). Among the 72 patients with valve replacement the entire valve was excised in 30 cases (42%), the anterior leaflet alone was excised in 34 cases (47%), and the anterior leaflet plus portions of the posterior leaflet were excised in the remaining
The overall mean age was 61 years (age range, 8 to 85 years) and there was no significant difference in mean age
Postinflam Postinflam Postinflam
70-79
0 0 0 0 0 0 1 0 0
Age and Gender Distribution
Functional Status
60-69
38-77 37-78 13-79 28-85 60-78 28-65 8-68 34-53 -
State of Valve
Surgical Procedures, Composition of Specimens, and Associated in 95 Surgically Excised Mitral Valves Valve Replacr“lent
50-59
62 61 63 65 70 52 39 44 61
Of the 95 valves, 16 (17%) had mitral stenosis (MS), 24 (25%) had combined mitral stenosis and regurgitation (MSMR), and 55 (58%) had pure mitral regurgitation (MR). All 16 cases in the MS group and 22 of the 24 cases (92%) in the MS-MR group resulted from postinflammatory disease (Table 1). One case each of type VI mucopolysaccharidosis and ergotamine-induced valvular disease accounted for the remaining two cases with MS-MR. The 55 cases of MR resulted from several disease processes, of which a floppy mitral valve was the most common (49%). Other significant causes were postinflammatory disease (16%), ischemic heart disease (13%), and endocarditis (9%). Two valves showed acute endocarditis (Staphylococcus aureus and Streptococcusfuecalis) and three were affected by healed endocarditis. Other conditions causing MR involved two cases of carcinoid heart disease and one case each of radiation-induced valve disease, valve disease associated with hypertrophic cardiomyopathy, and a congenitally dysplastic valve. Two cases (4%) were of indeterminate etiology.
Etiology
0
40-49
1:3 1:2.7 1:2 1.3:1 2.5:1 5:o 1:1.5 0:2 1:l
MR, mitral regurgitation;
TABLE 2.
30-39
12 16 6 12 2 0 3 2 1
RESULTS Functional
20-29
(yr)
4 6 3 15 5 5 2 0 1
(17) (23) (9) (28) (7) (5) (5) (2) (2)
95
Total
(Dare et al)
Gender
of Valves
Functional Status
OF THE MITRAL VALVE
Age and Gender of Patients, and Etiology and Functional Classification of 95 Surgically Excised Mitral Valves
TABLE 1.
Classification
PATHOLOGY
Valve Repair
Old MV Surgery
Associated AV Replace“lent
Complete
AL Only
AL and Pal-t of PL
Portion of PL
16 22 9 9 6 2 5 2 1
0 0 0 18 1 3 0 0 1
3 8 2 1 0 0 0 0 0
3 5 1 1 0 0 1 1 0
8 14 4 1 0 1 1 1 0
5 7 4 5 6 1 4 1 1
3 1 1 3 0 0 0 0 0
0 0 0 17 0 3 0 0 1
72
23
14
12
30
34
8
21
Abbreviations: AL. anteriorleaflet; AV. aortic valve; MR, mitral regurgitation; flammatory (presumably rheumatic).
1287
MS, mitral stenosis;
Chordae
Findings
Mitral Prolapse
MV Annular Calcification
Chordal Rupture
0
0
0
0
0
0
0
0
MV, mitral valve; PL. posterior
0
0
22 1 4 0 0 0
6 2 0 1 0 0
0 0 19 0 1 0 0 0
27
9
20
lea&t;
posti”&m,
postin-
HUMAN PATHOLOGY
Volume 24, No. 12 (December
1993)
FIGURE 1. Mitral valve specimens removed during valve replacement. (Top left) The entire valve, removed for postinflammatory MS. (Top right) The anterior leaflet and a portion of the posterior leaflet, excised for postinflammatory MS and regurgitation. (Bottom left) The anterior leaflet alone, removed for postischemic mitral insufficiency. (Bottom right) The anterior leaflet, with large perforation (arrow), excised for postendocarditis MR.
For the 23 casts in which valves were repaired the specimen c.onsisled of either a portion of the posterior leaflet (2 1 cases) or chordal fragments (two cases). A floppy valve was by far the most common etiology (78%) and the next most frequent cause was infective endocarditis (13%). The two specimens comprising only chordal fragments represented floppy valve disease and ischemic heart disease.
eight cases (1 1%). When the entire valve was removed, postinflammatory disease accounted for 26 of the 30 cases (87%). The remaining four cases included one case each of congenital valvular dysplasia, floppy mitral valve, infective endocarditis, and type VI mucopolysaccharidosis. In contrast, the 42 cases with removal of the anterior leaflet, with or without a portion of the posterior leaflet, showed a more diverse range of underlying etiologies (Table 2). Postinflammatory disease still accounted for the majority of cases (50%), with floppy valve disease being the next most common cause (19%).
Relationship
of Etiology to Procedure
All 47 postinflarlllrrator valves required replacement, and in 26 (55%) the entire valve was excised. For the remaining
1288
SURGICAL
PATHOLOGY
OF THE MITRAL VALVE
(Dare et al)
FIGURE 2. Mitral valve specimens removed during valve repair. (Top) A wedge resection from a posterior leaflet, showing hooding deformity (atrial and ventricular views), due to regurgitant floppy mitral valve. (Bottom) Resected chordae tendineae from a posterior leaflet of a patient with a history of mitral valve prolapse, chordal rupture, flail leaflet, and mitral insufficiency.
21 (45%) the anterior leaflet was excised with or without a portion of the posterior leaflet. For valvular dysfunction due to ischemic heart disease six of seven (86%) valves were replaced. Specimens consisted of the anterior leaflet for all valves that were replaced and of chordae only in the one case in which repair was performed. In contrast, 18 of the 27 (67%) floppy valves were repaired. Resected specimens included a portion of the posterior leaflet alone (63%), the anterior leaflet (30%), the complete valve (4%). or chordae only (4%). Among the five valves with infective endocarditis, three were repaired and two were replaced. Specimens from repaired valves consisted of posterior leaflet tissue, whereas those from replaced valves included either the anterior leaflet or the entire valve. For the nine cases with other etiologies resected specimens included the anterior leaflet (six cases) and the entire valve
1289
(Two cases) from the eight patients with valve replacement and the posterior leaflet from the one patient with a repaired valve.
Relationship
of Valve Function to Procedure
All 40 stenotic valves (pure MS and MS-MR groups combined) required replacement; 95% of these were involved by postinflammatory disease. In contrast, only 32 of the 55 (58%) purely regurgitant valves required replacement; the remaining 23 were repaired.
Simultaneous
Replacement
of Other Valves
In 13 cases valves other than the mitral also were replaced. Nine of the 13 cases (69%) represented combined aortic and mitral valve replacement for postinflammatory disease. Three (23%) included combined aortic and mitral replacement for
HUMANPATHOLOGY
Volume 24, No. 12 (December
one case each of radiation-induced valve disease, floppy aortic valve disease, and ergotamine-induced valve disease. In the remaining case combined pulmonary, tricuspid, and mitral valve replacement was performed for carcinoid heart disease.
1993)
DISCUSSION General Trends At the Mayo Clinic in 1990 postinflammatory disease was the most common disorder responsible for excision of mitral valve tissue (49%), followed by floppy valve disease (29%). In a recent review of 1,288 surgically excised mitral valves removed between 198 1 and 1989, Agozzino et al5 also reported postinflammatory and floppy valve disease as the two most common etiologies. Our 1990 data, however, showed a continuing decline in the relative frequency of postinflammatory disease, apparent at our institution since 1970 (Fig 3).” In contrast, the frequency of floppy mitral valves has continued to increase over the same time period. The declining incidence of postinflammatory mitral valve disease has paralleled the trend observed with aortic valve diseases-” and presumably corresponds to the declining incidence of acute rheumatic fever in the United States since the 1950s. The lower occurrence of postinflammatory disease, as well as changes in patient age and referral practices, may have contributed to the relative increase in the incidence of floppy mitral valve disease in our series. The frequency of MS with or without regurgitation, which was generally rheumatic, decreased from 79% in 1965 to 42% in 1990. It is unclear, however, why the decline primarily affected only the group with combined stenosis and insufficiency, while the frequency of pure stenosis remained relatively constant (Fig 3). During the
Previous Valve Surgery Fourteen patients previously had undergone surgical mitral commissurotomy or valvuloplasty, 13 of whom had postinflammatory disease. In the remaining case a floppy valve had been repaired. The aortic valve had been replaced previously in two patients, one for postinflammatory disease and the other for healed endocarditis.
Annular Calcification Mitral annular calcification was observed in nine specimens and occurred most frequently in the setting of a floppy valve (six cases). Of the remaining three cases, two were associated with ischemic heart disease and one had hypertrophic cardiomyopathy. When annular calcification is extensive it usually is not resected surgically.
Mitral Valve Prolapse and Chordal Rupture Mitral valve prolapse was diagnosed either preoperatively (by echocardiography) or intraoperatively (by surgical inspection) in 27 cases; in 22 (81%) of these cases the valve was floppy. Four cases (15%) had endocarditis and one (4%) represented ischemic heart disease. Twenty examples of chordal rupture were recorded, 19 of which were associated with a floppy valve; the remaining case was due to infective endocarditis.
Postinflammatory W8
I -w-
51
49
%IaO30Xl-
17
1965
1970
1975
1980
1985
1930
I ‘“1, 0'
1965
Year 90
,
1
1970
I
1
1975
1980
1
1995
17
I
1990
Year I
FIGURE 3. Temporal changes in mitral valve disease from 1965 to 1990. (Top left) Etiology for all valves (807 cases). (Top right) Functional state of all valves (807 cases). (Bottom) Etiology of pure mitral insufficiency (355 cases).
Year
1290
SURGICAL
TABLE 3.
PATHOLOGY
Comparison
OF THE MITRAL VALVE
of Studies of Surgically
(Dare et al)
Excised Mitral Valves Gender
Source Hanson et al, 1985’ Olson et al, 19874 Agozzino et al, 19925 Dare et al (current study)
Years of Study
Temporal Changes Reported
No. of Valves
Completely Excised (%)
Ratios
Postinflammatory MS
MS-MR
MR
Floppy
Other
Postinflammatory (F:M)
Floppy (M:F)
1980-1983
No
100
NS
35
19
0
32
14
5.0
1.7
1965-1985*
Yes
712
NS
173
277
80
100
82
1.9
3.2
1981-1989
No
1.288
NS
360
747
72
84
25
1.8
1.5
95
32
16
22
9
27
21
2.6
1.3
1990
Yes
Abbreviations: MS, mitral stenosis; MR. mitral regurgitation; NS, not stated * Includes only the years 1965, 1970, 1975, 1980, and 1985.
same time period the frequency of pure MR increased from 21% in 1965 to 58% in 1990, primarily as a result of increasing numbers of floppy valves. Of particular interest were the gender ratios for various disease etiologies. Women were much more likely than men to have stenosis or combined stenosis and regurgitation, primarily due to a higher incidence of postinflammatory disease in women. Even with pure MR female patients more often had postinflammatory disease than males. Men, in contrast, were more likely than women to have regurgitation rather than stenosis, and the regurgitation was more likely to be on the basis of floppy valve disease or ischemic heart disease rather than postinflammatory disease. Other investigators have reported similar findings (Table 3).2.4,.5 Trends in Stenotic
Mitral Valves
In 1990, as in former years, there continued to be a striking preponderance of postinflammatory disease as a cause of MS. Among 40 cases, all but two were postinflammatory. These two included one case each of type VI mucopolysaccharidosis and ergotamine-induced valvular disease, both of which have been reported.“-” In our previous study 450 of 452 stenotic mitral valves had postinflammatory disease and two were congenitally stenotic.” All 54 stenotic valves reported by Hanson et al* and all 1 ,107 valves described by Agozzino et al5 were considered to be rheumatic (postinflammatory) in etiology. Thus, nonrheumatic causes of MS are rare. Trends in Purely Regurgitant
Mitral Valves
Since 1975 floppy mitral valve disease has been the most common cause of MR among surgical patients at the Mayo Clinic; in 1990 it represented 49% of the cases (Fig 3). These findings are similar to those reported by Agozzino et a1,5 in which 84 of their 181 (46%) purely regurgitant mitral valves were floppy. Even higher percentages have been reported by other investigators, ranging from the 62% (60 of 97 cases) of Waller et al’ to the 70% (32 of 46 cases) of Hanson et al.’ The preponderance of floppy disease among regurgitant valves may be related to the increasing relative percentage of 1291
elderly citizens in the US population and the increasing willingness of surgeons to operate on such patients, particularly when valve repair, rather than replacement, is feasible. In 1990 postinflammatory disease accounted for only 16% of our cases, and other causes were even less commonly observed. Other studies from the United States also have shown low percentages of postinflammatory mitral incompetence, with only 3% identified by Waller et al’ and no cases reported by Hanson et al.” This is in contrast to the Italian study of Agozzino et al,’ in which postinflammatory disease accounted for 72 of their 18 1 cases (40%) with MR. Trends in Surgical Procedures In the past mitral valve replacement entailed excision of the entire valve, often as a single intact specimen. Currently, however, only the anterior mitral leaflet usually is removed, and the posterior leaflet and its chordal attachments to the papillary muscles generally are left in place. This change in surgical technique may better preserve left ventricular function and appears to be one factor leading to the virtual elimination of fatal left ventricular rupture following mitral valve replacement z”.z’ During mitral valve reparative procedures (rather than replacement) only portions of the valve are excised, usually a wedge from the posterior leaflet. As a result, the nature of mitral valve tissue received by surgical pathologists at our institution has changed dramatically since our previous study of the years between 1965 and 1985.4 During that time 87% of the valves were removed entirely, in one or two pieces. In contrast, during 1990 only 30 of 95 valves (32%) were excised in their entirety. It is therefore apparent that morphologic assessment of mitral valve specimens has become more difficult today than in previous decades, because valves are now frequently excised incompletely. To facilitate interpretation of these valves information other than the patient’s age and gender and the morphologic appearance of the resected tissues is needed. It also is important to know the preoperative functional state of the valve; the presence of annular dilatation, leaflet prolapse, or
HUMAN PATHOLOGY
CPW!
Volume 24, No. 12 (December
Date
U(CISED
case
Age, Gender -
NallK Patient IM
1993)
Date
U(CISED
Name
hm
Att%G~
Patient ID#
Yes_
NO-
unknown
Hx. of Endocarditir:
Yes_
No-
Unknown _
HI. of Rheumatic Fever
Yes_
No-
Unluwmn-
tlx. of Mitral
Yes_
No-
Unknown _
Hx. of Endocrrditir:
Yes_
No-
Unknmn-
Yes -
No-
Unknown _
Aortic Rod
Yes -
No-
Awtftis
-
Trtmcal
Prolapsez
lschemic Heart Dim
_
-
Hx. of Rheumatic Fever
Other Pertinent Info: Valve:
Miiral
ha+on
Dilatatfon:
Other Pertinent
_
Tricuqii_
L AV Valve
Common AV Valve
_
_
R. AV Valve -
Valve:
Description:
Ruptured Chordae:
No.of
Aortic Cusps:
_
Pldmowy
_
None-
Mild _
Moderate _
severe-
Stenosir:
None-
Mild -Moderate-
Inwfficiency:
None _
Mild _
Moderate_
Severe _
Inefficiency:
None _
Mild _
Case
SPECIMEN
U(CISED
_
Descrfptioa
Stenosis:
PLEASE SEND CARD WITH
_
Info:
Sewre-
MO&rate
PLEASE SEND CARD WITH
-
Severe-
SPECIMEN
Date
Name
Age, Gender -
Patient KM
bacon Size
Type of Pmsthesfr Position fe.~., MiiaD:
FIGURE 4. Forms to be completed by the surgeon at the time of valve surgery. (Top left) Atrioventricular valve. (Top right) Semilunar valve. (Bottom) Prosthetic valve.
Reason for Excision: Other Pertinent
Info:
Stenosis:
Non-
Mild -Moderate-
Severe-
Inwfficiency:
None _
Mild _
Severe _
Moderate_
PLEASE SEND CARD WITH
SPECIMEN
chordal rupture; the co-existence of other valvular dysfunction or cardiac disease; and a clinical history of endocarditis or past rheumatic fever. To obtain this information quickly and accurately, we provide printed forms to be completed by our surgical colleagues in the operating room. These forms are printed on index cards, with different colored cards representing semilunar, atrioventricular, and prosthetic valves (Fig 4). Cooperation is facilitated by the collegial working relationship that exists between pathologists and surgeons at our institution. Nonetheless, the composition of a specimen can provide valuable clues concerning the procedure and the underlying valvular disease. For example, if the anterior leaflet alone is received, then valve replacement has been performed. If the entire valve has been excised, then postinflammatory disease is by far the most likely etiology (87%). If the aortic valve also is replaced at the time of mitral valve surgery or there is a history of previous mitral commissurotomy, then postinflammatory disease is again the most likely cause (69% and 93%, respectively). In contrast, if the specimen consists of only a portion of the posterior leaflet, then a reparative procedure has been performed and floppy valve disease is the most probable etiology (78%). The presence of ruptured 1292
chordae tendineae also makes floppy valve disease highly likely (95%) as does a history of mitral valve prolapse (81%). However, it should be noted that valves affected by infective endocarditis may produce chordal rupture and flail leaflets and also are amenable in some instances to posterior leaflet repair. In conclusion, when the entire mitral valve is resected an accurate determination of the underlying etiology usually can be made based on the gross morphologic features alone. However, diagnostic difficulties arise when only a portion of the valve is removed, and microscopic evaluation is not necessarily helpful. In such cases rendering a clinicopathologic diagnosis is recommended. REFERENCES 1. Wailer BF, Morrow AG, Maron BJ, et al: Etiology of clinically isolated, severe, chronic, pure mitral regurgitation: Analysis of 97 patients over 30 years of age having mitral valve replacement. Am Heart J 104:276-288, 1982 2. Hanson TP, Edwards B.S. Edwards JE: Pathology of surgically excised mitral valves: One hundred consecutive cases. Arch Pathol Lab Med 109:823-828, 1985 3. Van der Bel-Kahn J, Becker AE: The surgical pathology of rheumatic and floppy mitral valves: Distinctive morphologic features upon gross examination. Am J Surg Pathol 10:282-292, 1986
SURGICAL
PATHOLOGY
OF THE MITRAL VALVE
4. Olson LT, Subramanian R, Ackermann DM, et al: Surgical pathology of the mitral valve: A study of 7 12 cases spanning 2 1 years. Mayo Clin Proc 62:22-34, 1987 5. Agozzino L, Falco A, de Vivo F, et al: Surgical pathology of the mitral valve: Gross and histological study of 1288 surgically excised valves. Int J Cardiol 17:79-89, 1992 6. Subramanian R, Olson LJ, Edwards WD: Surgical pathology of pure aortic stenosis: A study of 374 cases. Mayo Clin Proc 59:683690, 1984 7. Olson LJ, Subramanian R, Edwards WD: Surgical pathology of pure aortic insufficiency: A study of 225 cases. Mayo Clin Proc 59: 835-841, 1984 8. Suhramanian R, Olson LJ, Edwards WD: Surgical pathology of combined aortic stenosis and insufficiency: A study of 213 cases. Mayo Clin Proc 60:247-254, 1985 9. Pdssick CS, Ackermann DM, Pluth JR, et al: Temporal changes in the causes of aortic stenosis: A surgical pathologic study of 646 cases. Mayo Clin Proc 62: 119-l 23, 1987 10. Hauck AJ, Freeman DP. Ackermann DM, et al: Surgical pathology of the tricuspid valve: A study of 363 cases spanning 25 years. Mayo Clin Proc 63:85 l-863, 1988 11. Fuglestad SJ. Puga FJ, Danielson GK, et al: Surgical pathology of the truncal valve: A study of 12 cases. Am J Cardiovasc Path01 2: 39-47, 1988 12. Fuglestad SJ, Danielson CK, Puga FJ, et al: Surgical pathology of the common atrioventricular valve: A study of 11 cases. Am J Cardiovasc Pathol 2:49-55, 1988
(Dare et al)
13. Altrichter PM, Olson LJ, Edwards WD, et al: Surgical pathology of the pulmonary valve: A study of 116 cases spanning 15 years. Mayo Clin Proc 64:1352-1360, 1989 14. Roberts WC, Morrow AG: Cardiac valves and the surgical pathologist. Arch Pathol 82:309-3 13, 1966 15. Davies MJ: Pathology of Cardiac Valves. Boston, MA, Butterworth, 1980, pp 62-l 23 16. Pomerance A: Chronic rheumatic and other inflammatory valve disease, in Pomerance A, Davies MJ (eds): Pathology of the Heart. London, UK, Blackwell Scientific, 1975, pp 307-326 17. Tan CTT, Schall’ HV, Miller FA, Jr, et al: Valvular heart disease in four patients with Maroteaux-Lamy syndrome. Circulation 85:188-195, 1992 18. Hauck AJ, Edwards WD, Danielson GK, et al: Mitral and aortic valve disease associated with ergotamine therapy for migraine. Arch Pathol Lab Med 114:62-64, 1990 19. Redfield MM, Nicholson WJ, Edwards WD, et al: Valve disease associated with ergot alkaloid use: Echocardiographic and pathologic correlations. Ann Intern Med 117:50-52, 1992 20. Azariades M, Lennox SC: Rupture of the posterior wall of the left ventricle after mitral valve replacement: Etiological and technical considerations. Ann Thorac Surg 46:491-494, 1988 2 1. Karlson KJ, Ashraf MM, Berger RL: Rupture of left ventricle following mitral valve replacement. Ann Thorac Surg 46:590-597, 1988
1293
valves from 54 women and 41 men (mean age,
61 years; age range, 8 to 85 years) were replaced (76%) or repaired (24%) at the Mayo Clinic. Functionally, 58% of the valves were purely regurgitant (MR), 25% were stenotic and regurgitant 17% were
purely
stenotic
(MS).
rheumatic) disease accounted
(MS-MR),
Postinflammatory
and
(presumably
for 100% of MS cases, 92% of MS-
MR cases, and 16% of MR cases. Other causes of pure MR included floppy valves (49%), ischemic heart disease (130/o),infective endocarditis (9%), miscellaneous
(9%), and indeterminate
(4%). Thus,
postinflammatory disease represented the major cause of both mitral stenosis (MS and MS-MR) surgical population.
and overall mitral valve disease in our
In contrast, floppy valves were the most com-
monly observed cause of pure MR. Among postinflammatory valves, 55% were completely excised and 45% had only the anterior leaflet removed; all were replaced. In contrast, floppy valves were incompletely excised in 96%; 67% were repaired
phologic
an accurate etiologic diagnosis
excised,
requires not only a mor-
assessment of resected tissues but also a knowledge
the clinical history, operative valve. Hm
PATHOL
details, and functional
24:1286-1293.
underlying etiology by gross inspection alone may now be more difficult than in the past. Moreover, in a previous study from our institution appreciable temporal changes in the various causes of mitral valve dysfunction were observed between 1965 and 1985.” Thus, the current investigation was undertaken to determine whether an accurate etiologic diagnosis could still be attained with evaluation of only portions of the mitral valve and to further define any temporal changes in the causes of mitral valve disease in patients undergoing operative replacement or repair. This represents a continuation of our ongoing study of the surgical pathology of valvular heart disease.4,6-i”
and only 33% were re-
placed. Because mitral valves frequently are incompletely rendering
MD,
Copyright
0
METHODS
of
state of the
1993 by W.B.
Saunders Company During the past 11 years several investigators have evaluated the athologic features of surgically excised mitral valves.‘--$ In general, conclusions regarding the underlying etiology have been based on gross inspection of the entire valve, removed during valve replacement procedures. In recent years, however, there has been a tendency for surgeons to remove only the anterior mitral leaflet during valve replacement and to remove only portions of the valve (usually from the posterior leaflet) during reparative procedures. As a result, determination of the
From the Department of Pathology, Prince Charles Hospital, Brisbane, Australia; and the Divisions of Anatomic Pathology and Cardiothoracic Surgery, Mayo Clinic and Foundation, Rochester, MN. Accepted for publication July 26, 1993. Presented in part at the 17th World Congress on Diseases of the Chest, Amsterdam, The Netherlands, June 1993. Key wor&: floppy mitral valve, mitral valve disease, rheumatic heart disease, surgical pathology. Address correspondence and reprint requests to William D. Edwards, MD, Hilton (1 1) Pathology, Mayo Clinic. Rochester, MN 55905. Copyright 0 1993 by W.B. Saunders Company 0046-S177/93/2412-0003$5.00/O
1286
All mitral valves that were either wholly or partially excised at the Mayo Clinic during 1990 were obtained from our tissue registry for review. All 95 specimens were evaluated grossly, as recommended by Roberts and MorrowI and Davies.15 Histologic examination was performed only when there was evidence to suggest endocarditis or another specific disease process requiring histologic confirmation (storage disease OI papillary muscle infarction, for example). For each valve the number and origin of excised fragments were recorded. Further pathologic examination, clinical data retrieval. and categorization of valve function (pure stenosis, pure regurgitation, or combined stenosis and regurgitation) were performed as described in previous studies.4.6-‘3 Based on the pathologic and clinical findings, each valve was classified as postinflammatory, floppy, or associated with ischemic heart disease, infective endocarditis, cardiomyopathy, congenital malformation, or another disorder. Valves that could not be classified confidently were considered to be of indeterminate etiology. The term “postinflammatory disease” was used to refer to a chronic noninfectious fibrosing process resulting in valvular distortion indistinguishable from confirmed cases of chronic rheumatic valvular disease. In accordance with the recommendations of Pomerance,16 such valves were not designated as “rheumatic” because a clinical history of acute rheumatic fever was not obtained in all cases and because other conditions (such as systemic lupus erythematosus, ankylosing spondylitis, rheumatoid arthritis, and radiation-induced valve disease) may produce identical morphologic changes.
SURGICAL
Etiolow Postinflam Postinflam Postinflam Floppy Ischemia Endocarditis Other Other Indeterminate
MS MS-MR MR MR MR MR MR MS-MR MR
NO. (%) 16 22 9 27 7 5 5 2 2
Male
Abbreviations:
Age of Patients
Female
M:F Ratio
Mean
Range
O-9
lo-19
F’~PPY lschemia Endocarditis Other Other Indeterminate Total
MS
MS-MR MR MR MR MR MR MS-MR MR
80-89
0 0 1 0 0 0 0 0 0
0 0 1 0 1 0 1 0
1 1 0 1 0 0 2 0 0
4 3 0 2 0 1 0 0 0
0 7 1 1 0 0 1 1 1
5 5 4 12 3 3 1 0 1
6 6 3 9 4 0 0 0 0
0 0 0 1 0 0 0 0 0
41
54
1:1.3
61
8-85
1
1
3
5
10
12
34
28
1
MS, mitral stenosis;
Postinflam,
postinflammatory
(presumably
rheumatic).
between the three functional groups (MS, 62 years; MS-MR, 59 years; and MR, 62 years). The oldest patients included those with regurgitation due to ischemic heart disease (mean age, 70 years). In contrast, the youngest patients were those with endocarditis (mean age, 52 years) and two “other diseases” (mean ages, 39 and 44 years). For all other etiologic categories the range of mean ages was 61 to 65 years (Table 1). The study included valves from 54 female and 41 male patients. Women predominated in the stenosis (MS and MSMR) groups (3: 1 ratio) and in the postinflammatory MR group (2:l ratio). In contrast, men had regurgitation more commonly due to floppy valves (1.3: 1 ratio) and to ischemic heart disease (2.5:1 ratio). All five patients with mitral incompetence due to endocarditis were males.
Relationship
of Procedure to Etiology
Seventy-two valves (76%) were replaced and 23 (24%) were repaired (Table 2). When valves were replaced the anterior leaflet was excised either alone or with part or all of the posterior leaflet (Fig 1). In contrast, when valves were repaired only a portion of the posterior leaflet or its chordae tendineae was excised (Fig 2). Among the 72 patients with valve replacement the entire valve was excised in 30 cases (42%), the anterior leaflet alone was excised in 34 cases (47%), and the anterior leaflet plus portions of the posterior leaflet were excised in the remaining
The overall mean age was 61 years (age range, 8 to 85 years) and there was no significant difference in mean age
Postinflam Postinflam Postinflam
70-79
0 0 0 0 0 0 1 0 0
Age and Gender Distribution
Functional Status
60-69
38-77 37-78 13-79 28-85 60-78 28-65 8-68 34-53 -
State of Valve
Surgical Procedures, Composition of Specimens, and Associated in 95 Surgically Excised Mitral Valves Valve Replacr“lent
50-59
62 61 63 65 70 52 39 44 61
Of the 95 valves, 16 (17%) had mitral stenosis (MS), 24 (25%) had combined mitral stenosis and regurgitation (MSMR), and 55 (58%) had pure mitral regurgitation (MR). All 16 cases in the MS group and 22 of the 24 cases (92%) in the MS-MR group resulted from postinflammatory disease (Table 1). One case each of type VI mucopolysaccharidosis and ergotamine-induced valvular disease accounted for the remaining two cases with MS-MR. The 55 cases of MR resulted from several disease processes, of which a floppy mitral valve was the most common (49%). Other significant causes were postinflammatory disease (16%), ischemic heart disease (13%), and endocarditis (9%). Two valves showed acute endocarditis (Staphylococcus aureus and Streptococcusfuecalis) and three were affected by healed endocarditis. Other conditions causing MR involved two cases of carcinoid heart disease and one case each of radiation-induced valve disease, valve disease associated with hypertrophic cardiomyopathy, and a congenitally dysplastic valve. Two cases (4%) were of indeterminate etiology.
Etiology
0
40-49
1:3 1:2.7 1:2 1.3:1 2.5:1 5:o 1:1.5 0:2 1:l
MR, mitral regurgitation;
TABLE 2.
30-39
12 16 6 12 2 0 3 2 1
RESULTS Functional
20-29
(yr)
4 6 3 15 5 5 2 0 1
(17) (23) (9) (28) (7) (5) (5) (2) (2)
95
Total
(Dare et al)
Gender
of Valves
Functional Status
OF THE MITRAL VALVE
Age and Gender of Patients, and Etiology and Functional Classification of 95 Surgically Excised Mitral Valves
TABLE 1.
Classification
PATHOLOGY
Valve Repair
Old MV Surgery
Associated AV Replace“lent
Complete
AL Only
AL and Pal-t of PL
Portion of PL
16 22 9 9 6 2 5 2 1
0 0 0 18 1 3 0 0 1
3 8 2 1 0 0 0 0 0
3 5 1 1 0 0 1 1 0
8 14 4 1 0 1 1 1 0
5 7 4 5 6 1 4 1 1
3 1 1 3 0 0 0 0 0
0 0 0 17 0 3 0 0 1
72
23
14
12
30
34
8
21
Abbreviations: AL. anteriorleaflet; AV. aortic valve; MR, mitral regurgitation; flammatory (presumably rheumatic).
1287
MS, mitral stenosis;
Chordae
Findings
Mitral Prolapse
MV Annular Calcification
Chordal Rupture
0
0
0
0
0
0
0
0
MV, mitral valve; PL. posterior
0
0
22 1 4 0 0 0
6 2 0 1 0 0
0 0 19 0 1 0 0 0
27
9
20
lea&t;
posti”&m,
postin-
HUMAN PATHOLOGY
Volume 24, No. 12 (December
1993)
FIGURE 1. Mitral valve specimens removed during valve replacement. (Top left) The entire valve, removed for postinflammatory MS. (Top right) The anterior leaflet and a portion of the posterior leaflet, excised for postinflammatory MS and regurgitation. (Bottom left) The anterior leaflet alone, removed for postischemic mitral insufficiency. (Bottom right) The anterior leaflet, with large perforation (arrow), excised for postendocarditis MR.
For the 23 casts in which valves were repaired the specimen c.onsisled of either a portion of the posterior leaflet (2 1 cases) or chordal fragments (two cases). A floppy valve was by far the most common etiology (78%) and the next most frequent cause was infective endocarditis (13%). The two specimens comprising only chordal fragments represented floppy valve disease and ischemic heart disease.
eight cases (1 1%). When the entire valve was removed, postinflammatory disease accounted for 26 of the 30 cases (87%). The remaining four cases included one case each of congenital valvular dysplasia, floppy mitral valve, infective endocarditis, and type VI mucopolysaccharidosis. In contrast, the 42 cases with removal of the anterior leaflet, with or without a portion of the posterior leaflet, showed a more diverse range of underlying etiologies (Table 2). Postinflammatory disease still accounted for the majority of cases (50%), with floppy valve disease being the next most common cause (19%).
Relationship
of Etiology to Procedure
All 47 postinflarlllrrator valves required replacement, and in 26 (55%) the entire valve was excised. For the remaining
1288
SURGICAL
PATHOLOGY
OF THE MITRAL VALVE
(Dare et al)
FIGURE 2. Mitral valve specimens removed during valve repair. (Top) A wedge resection from a posterior leaflet, showing hooding deformity (atrial and ventricular views), due to regurgitant floppy mitral valve. (Bottom) Resected chordae tendineae from a posterior leaflet of a patient with a history of mitral valve prolapse, chordal rupture, flail leaflet, and mitral insufficiency.
21 (45%) the anterior leaflet was excised with or without a portion of the posterior leaflet. For valvular dysfunction due to ischemic heart disease six of seven (86%) valves were replaced. Specimens consisted of the anterior leaflet for all valves that were replaced and of chordae only in the one case in which repair was performed. In contrast, 18 of the 27 (67%) floppy valves were repaired. Resected specimens included a portion of the posterior leaflet alone (63%), the anterior leaflet (30%), the complete valve (4%). or chordae only (4%). Among the five valves with infective endocarditis, three were repaired and two were replaced. Specimens from repaired valves consisted of posterior leaflet tissue, whereas those from replaced valves included either the anterior leaflet or the entire valve. For the nine cases with other etiologies resected specimens included the anterior leaflet (six cases) and the entire valve
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(Two cases) from the eight patients with valve replacement and the posterior leaflet from the one patient with a repaired valve.
Relationship
of Valve Function to Procedure
All 40 stenotic valves (pure MS and MS-MR groups combined) required replacement; 95% of these were involved by postinflammatory disease. In contrast, only 32 of the 55 (58%) purely regurgitant valves required replacement; the remaining 23 were repaired.
Simultaneous
Replacement
of Other Valves
In 13 cases valves other than the mitral also were replaced. Nine of the 13 cases (69%) represented combined aortic and mitral valve replacement for postinflammatory disease. Three (23%) included combined aortic and mitral replacement for
HUMANPATHOLOGY
Volume 24, No. 12 (December
one case each of radiation-induced valve disease, floppy aortic valve disease, and ergotamine-induced valve disease. In the remaining case combined pulmonary, tricuspid, and mitral valve replacement was performed for carcinoid heart disease.
1993)
DISCUSSION General Trends At the Mayo Clinic in 1990 postinflammatory disease was the most common disorder responsible for excision of mitral valve tissue (49%), followed by floppy valve disease (29%). In a recent review of 1,288 surgically excised mitral valves removed between 198 1 and 1989, Agozzino et al5 also reported postinflammatory and floppy valve disease as the two most common etiologies. Our 1990 data, however, showed a continuing decline in the relative frequency of postinflammatory disease, apparent at our institution since 1970 (Fig 3).” In contrast, the frequency of floppy mitral valves has continued to increase over the same time period. The declining incidence of postinflammatory mitral valve disease has paralleled the trend observed with aortic valve diseases-” and presumably corresponds to the declining incidence of acute rheumatic fever in the United States since the 1950s. The lower occurrence of postinflammatory disease, as well as changes in patient age and referral practices, may have contributed to the relative increase in the incidence of floppy mitral valve disease in our series. The frequency of MS with or without regurgitation, which was generally rheumatic, decreased from 79% in 1965 to 42% in 1990. It is unclear, however, why the decline primarily affected only the group with combined stenosis and insufficiency, while the frequency of pure stenosis remained relatively constant (Fig 3). During the
Previous Valve Surgery Fourteen patients previously had undergone surgical mitral commissurotomy or valvuloplasty, 13 of whom had postinflammatory disease. In the remaining case a floppy valve had been repaired. The aortic valve had been replaced previously in two patients, one for postinflammatory disease and the other for healed endocarditis.
Annular Calcification Mitral annular calcification was observed in nine specimens and occurred most frequently in the setting of a floppy valve (six cases). Of the remaining three cases, two were associated with ischemic heart disease and one had hypertrophic cardiomyopathy. When annular calcification is extensive it usually is not resected surgically.
Mitral Valve Prolapse and Chordal Rupture Mitral valve prolapse was diagnosed either preoperatively (by echocardiography) or intraoperatively (by surgical inspection) in 27 cases; in 22 (81%) of these cases the valve was floppy. Four cases (15%) had endocarditis and one (4%) represented ischemic heart disease. Twenty examples of chordal rupture were recorded, 19 of which were associated with a floppy valve; the remaining case was due to infective endocarditis.
Postinflammatory W8
I -w-
51
49
%IaO30Xl-
17
1965
1970
1975
1980
1985
1930
I ‘“1, 0'
1965
Year 90
,
1
1970
I
1
1975
1980
1
1995
17
I
1990
Year I
FIGURE 3. Temporal changes in mitral valve disease from 1965 to 1990. (Top left) Etiology for all valves (807 cases). (Top right) Functional state of all valves (807 cases). (Bottom) Etiology of pure mitral insufficiency (355 cases).
Year
1290
SURGICAL
TABLE 3.
PATHOLOGY
Comparison
OF THE MITRAL VALVE
of Studies of Surgically
(Dare et al)
Excised Mitral Valves Gender
Source Hanson et al, 1985’ Olson et al, 19874 Agozzino et al, 19925 Dare et al (current study)
Years of Study
Temporal Changes Reported
No. of Valves
Completely Excised (%)
Ratios
Postinflammatory MS
MS-MR
MR
Floppy
Other
Postinflammatory (F:M)
Floppy (M:F)
1980-1983
No
100
NS
35
19
0
32
14
5.0
1.7
1965-1985*
Yes
712
NS
173
277
80
100
82
1.9
3.2
1981-1989
No
1.288
NS
360
747
72
84
25
1.8
1.5
95
32
16
22
9
27
21
2.6
1.3
1990
Yes
Abbreviations: MS, mitral stenosis; MR. mitral regurgitation; NS, not stated * Includes only the years 1965, 1970, 1975, 1980, and 1985.
same time period the frequency of pure MR increased from 21% in 1965 to 58% in 1990, primarily as a result of increasing numbers of floppy valves. Of particular interest were the gender ratios for various disease etiologies. Women were much more likely than men to have stenosis or combined stenosis and regurgitation, primarily due to a higher incidence of postinflammatory disease in women. Even with pure MR female patients more often had postinflammatory disease than males. Men, in contrast, were more likely than women to have regurgitation rather than stenosis, and the regurgitation was more likely to be on the basis of floppy valve disease or ischemic heart disease rather than postinflammatory disease. Other investigators have reported similar findings (Table 3).2.4,.5 Trends in Stenotic
Mitral Valves
In 1990, as in former years, there continued to be a striking preponderance of postinflammatory disease as a cause of MS. Among 40 cases, all but two were postinflammatory. These two included one case each of type VI mucopolysaccharidosis and ergotamine-induced valvular disease, both of which have been reported.“-” In our previous study 450 of 452 stenotic mitral valves had postinflammatory disease and two were congenitally stenotic.” All 54 stenotic valves reported by Hanson et al* and all 1 ,107 valves described by Agozzino et al5 were considered to be rheumatic (postinflammatory) in etiology. Thus, nonrheumatic causes of MS are rare. Trends in Purely Regurgitant
Mitral Valves
Since 1975 floppy mitral valve disease has been the most common cause of MR among surgical patients at the Mayo Clinic; in 1990 it represented 49% of the cases (Fig 3). These findings are similar to those reported by Agozzino et a1,5 in which 84 of their 181 (46%) purely regurgitant mitral valves were floppy. Even higher percentages have been reported by other investigators, ranging from the 62% (60 of 97 cases) of Waller et al’ to the 70% (32 of 46 cases) of Hanson et al.’ The preponderance of floppy disease among regurgitant valves may be related to the increasing relative percentage of 1291
elderly citizens in the US population and the increasing willingness of surgeons to operate on such patients, particularly when valve repair, rather than replacement, is feasible. In 1990 postinflammatory disease accounted for only 16% of our cases, and other causes were even less commonly observed. Other studies from the United States also have shown low percentages of postinflammatory mitral incompetence, with only 3% identified by Waller et al’ and no cases reported by Hanson et al.” This is in contrast to the Italian study of Agozzino et al,’ in which postinflammatory disease accounted for 72 of their 18 1 cases (40%) with MR. Trends in Surgical Procedures In the past mitral valve replacement entailed excision of the entire valve, often as a single intact specimen. Currently, however, only the anterior mitral leaflet usually is removed, and the posterior leaflet and its chordal attachments to the papillary muscles generally are left in place. This change in surgical technique may better preserve left ventricular function and appears to be one factor leading to the virtual elimination of fatal left ventricular rupture following mitral valve replacement z”.z’ During mitral valve reparative procedures (rather than replacement) only portions of the valve are excised, usually a wedge from the posterior leaflet. As a result, the nature of mitral valve tissue received by surgical pathologists at our institution has changed dramatically since our previous study of the years between 1965 and 1985.4 During that time 87% of the valves were removed entirely, in one or two pieces. In contrast, during 1990 only 30 of 95 valves (32%) were excised in their entirety. It is therefore apparent that morphologic assessment of mitral valve specimens has become more difficult today than in previous decades, because valves are now frequently excised incompletely. To facilitate interpretation of these valves information other than the patient’s age and gender and the morphologic appearance of the resected tissues is needed. It also is important to know the preoperative functional state of the valve; the presence of annular dilatation, leaflet prolapse, or
HUMAN PATHOLOGY
CPW!
Volume 24, No. 12 (December
Date
U(CISED
case
Age, Gender -
NallK Patient IM
1993)
Date
U(CISED
Name
hm
Att%G~
Patient ID#
Yes_
NO-
unknown
Hx. of Endocarditir:
Yes_
No-
Unknown _
HI. of Rheumatic Fever
Yes_
No-
Unluwmn-
tlx. of Mitral
Yes_
No-
Unknown _
Hx. of Endocrrditir:
Yes_
No-
Unknmn-
Yes -
No-
Unknown _
Aortic Rod
Yes -
No-
Awtftis
-
Trtmcal
Prolapsez
lschemic Heart Dim
_
-
Hx. of Rheumatic Fever
Other Pertinent Info: Valve:
Miiral
ha+on
Dilatatfon:
Other Pertinent
_
Tricuqii_
L AV Valve
Common AV Valve
_
_
R. AV Valve -
Valve:
Description:
Ruptured Chordae:
No.of
Aortic Cusps:
_
Pldmowy
_
None-
Mild _
Moderate _
severe-
Stenosir:
None-
Mild -Moderate-
Inwfficiency:
None _
Mild _
Moderate_
Severe _
Inefficiency:
None _
Mild _
Case
SPECIMEN
U(CISED
_
Descrfptioa
Stenosis:
PLEASE SEND CARD WITH
_
Info:
Sewre-
MO&rate
PLEASE SEND CARD WITH
-
Severe-
SPECIMEN
Date
Name
Age, Gender -
Patient KM
bacon Size
Type of Pmsthesfr Position fe.~., MiiaD:
FIGURE 4. Forms to be completed by the surgeon at the time of valve surgery. (Top left) Atrioventricular valve. (Top right) Semilunar valve. (Bottom) Prosthetic valve.
Reason for Excision: Other Pertinent
Info:
Stenosis:
Non-
Mild -Moderate-
Severe-
Inwfficiency:
None _
Mild _
Severe _
Moderate_
PLEASE SEND CARD WITH
SPECIMEN
chordal rupture; the co-existence of other valvular dysfunction or cardiac disease; and a clinical history of endocarditis or past rheumatic fever. To obtain this information quickly and accurately, we provide printed forms to be completed by our surgical colleagues in the operating room. These forms are printed on index cards, with different colored cards representing semilunar, atrioventricular, and prosthetic valves (Fig 4). Cooperation is facilitated by the collegial working relationship that exists between pathologists and surgeons at our institution. Nonetheless, the composition of a specimen can provide valuable clues concerning the procedure and the underlying valvular disease. For example, if the anterior leaflet alone is received, then valve replacement has been performed. If the entire valve has been excised, then postinflammatory disease is by far the most likely etiology (87%). If the aortic valve also is replaced at the time of mitral valve surgery or there is a history of previous mitral commissurotomy, then postinflammatory disease is again the most likely cause (69% and 93%, respectively). In contrast, if the specimen consists of only a portion of the posterior leaflet, then a reparative procedure has been performed and floppy valve disease is the most probable etiology (78%). The presence of ruptured 1292
chordae tendineae also makes floppy valve disease highly likely (95%) as does a history of mitral valve prolapse (81%). However, it should be noted that valves affected by infective endocarditis may produce chordal rupture and flail leaflets and also are amenable in some instances to posterior leaflet repair. In conclusion, when the entire mitral valve is resected an accurate determination of the underlying etiology usually can be made based on the gross morphologic features alone. However, diagnostic difficulties arise when only a portion of the valve is removed, and microscopic evaluation is not necessarily helpful. In such cases rendering a clinicopathologic diagnosis is recommended. REFERENCES 1. Wailer BF, Morrow AG, Maron BJ, et al: Etiology of clinically isolated, severe, chronic, pure mitral regurgitation: Analysis of 97 patients over 30 years of age having mitral valve replacement. Am Heart J 104:276-288, 1982 2. Hanson TP, Edwards B.S. Edwards JE: Pathology of surgically excised mitral valves: One hundred consecutive cases. Arch Pathol Lab Med 109:823-828, 1985 3. Van der Bel-Kahn J, Becker AE: The surgical pathology of rheumatic and floppy mitral valves: Distinctive morphologic features upon gross examination. Am J Surg Pathol 10:282-292, 1986
SURGICAL
PATHOLOGY
OF THE MITRAL VALVE
4. Olson LT, Subramanian R, Ackermann DM, et al: Surgical pathology of the mitral valve: A study of 7 12 cases spanning 2 1 years. Mayo Clin Proc 62:22-34, 1987 5. Agozzino L, Falco A, de Vivo F, et al: Surgical pathology of the mitral valve: Gross and histological study of 1288 surgically excised valves. Int J Cardiol 17:79-89, 1992 6. Subramanian R, Olson LJ, Edwards WD: Surgical pathology of pure aortic stenosis: A study of 374 cases. Mayo Clin Proc 59:683690, 1984 7. Olson LJ, Subramanian R, Edwards WD: Surgical pathology of pure aortic insufficiency: A study of 225 cases. Mayo Clin Proc 59: 835-841, 1984 8. Suhramanian R, Olson LJ, Edwards WD: Surgical pathology of combined aortic stenosis and insufficiency: A study of 213 cases. Mayo Clin Proc 60:247-254, 1985 9. Pdssick CS, Ackermann DM, Pluth JR, et al: Temporal changes in the causes of aortic stenosis: A surgical pathologic study of 646 cases. Mayo Clin Proc 62: 119-l 23, 1987 10. Hauck AJ, Freeman DP. Ackermann DM, et al: Surgical pathology of the tricuspid valve: A study of 363 cases spanning 25 years. Mayo Clin Proc 63:85 l-863, 1988 11. Fuglestad SJ. Puga FJ, Danielson GK, et al: Surgical pathology of the truncal valve: A study of 12 cases. Am J Cardiovasc Path01 2: 39-47, 1988 12. Fuglestad SJ, Danielson CK, Puga FJ, et al: Surgical pathology of the common atrioventricular valve: A study of 11 cases. Am J Cardiovasc Pathol 2:49-55, 1988
(Dare et al)
13. Altrichter PM, Olson LJ, Edwards WD, et al: Surgical pathology of the pulmonary valve: A study of 116 cases spanning 15 years. Mayo Clin Proc 64:1352-1360, 1989 14. Roberts WC, Morrow AG: Cardiac valves and the surgical pathologist. Arch Pathol 82:309-3 13, 1966 15. Davies MJ: Pathology of Cardiac Valves. Boston, MA, Butterworth, 1980, pp 62-l 23 16. Pomerance A: Chronic rheumatic and other inflammatory valve disease, in Pomerance A, Davies MJ (eds): Pathology of the Heart. London, UK, Blackwell Scientific, 1975, pp 307-326 17. Tan CTT, Schall’ HV, Miller FA, Jr, et al: Valvular heart disease in four patients with Maroteaux-Lamy syndrome. Circulation 85:188-195, 1992 18. Hauck AJ, Edwards WD, Danielson GK, et al: Mitral and aortic valve disease associated with ergotamine therapy for migraine. Arch Pathol Lab Med 114:62-64, 1990 19. Redfield MM, Nicholson WJ, Edwards WD, et al: Valve disease associated with ergot alkaloid use: Echocardiographic and pathologic correlations. Ann Intern Med 117:50-52, 1992 20. Azariades M, Lennox SC: Rupture of the posterior wall of the left ventricle after mitral valve replacement: Etiological and technical considerations. Ann Thorac Surg 46:491-494, 1988 2 1. Karlson KJ, Ashraf MM, Berger RL: Rupture of left ventricle following mitral valve replacement. Ann Thorac Surg 46:590-597, 1988
1293