- Email: [email protected]
Surgical Pathology of the Tricuspid Valve: A Study of 363 Cases Spanning 25 Years
^ ? Proceedings ROCHESTER, MINNESOTA
Vol. 63
SEPTEMBER 1988
Surgical Pathology of the Tricuspid Valve: A Study of 363 Cases Spanning 25 Years
ARTHUR J. HAUCK, M.D., Division of Pathology; DANIEL P. FREEMAN, M.D., Department of Neurology; DOUGLAS M. ACKERMANN, M.D.,* Division of Pathology; GORDON K. DANIELSON, M.D., Division of Thoracic and Cardiovascular Surgery; WILLIAM D. EDWARDS, M.D., Division of Pathology
Surgical pathologic features of the tricuspid valve were reviewed in 3 6 3 patients w h o had undergone tricuspid valve replacement at our institution during the period 1963 through 1987. Valves were purely regurgitant in 74%, stenotic and regurgitant in 23%, and purely stenotic in 2%; t w o valves were neither stenotic nor regurgitant. Among 2 6 9 purely insufficient tricuspid valves, the four most common causes were postinflammatory disease (41%), congenital disorder (32%), pulmonary venous hypertension (21%), and infective endocarditis (4%). Of 92 cases of tricuspid stenosis, with or without regurgitation, postinflammatory disease w a s observed in 92%. Female patients accounted for 66% of the 3 6 3 cases, including 84% of those with postinflammatory disease and 64% of those with pulmonary venous hypertension. In contrast, male patients accounted for 73% of cases with endocarditis and 61% w i t h congenital heart disease. Although postinflammatory disease accounted for 53% of the 3 6 3 cases, its relative frequency diminished from 79% during 1963 through 1967 to only 24% during 1983 through 1987. This trend may reflect the decreasing incidence of acute rheumatic fever reported in Western countries. During the same time interval, the relative frequency of congenital heart disease as a cause of tricuspid dysfunction increased from 7% to 53%, and it is currently the most common cause in our surgical population. This finding apparently reflects changes in patient referral practices and the development of n e w operative procedures.
A l t h o u g h t h e characteristic pathologic features of various types of tricuspid valve disease have been described in specimens obtained from au topsy, 1 - 7 little information h a s been published *Current address: University of Louisville, Louisville, Kentucky. Address reprint requests to Dr. W. D. Edwards, Section of Medical Pathology, Mayo Clinic, Rochester, MN 55905. Mayo Clin Proc 63:851-863,1988
about t h e d i s t i n g u i s h i n g features of tricuspid valves removed a t operation. 8 " 1 0 Moreover, to our knowledge, c h a n g e s i n t h e relative frequencies of t h e various causes of tricuspid valve disease have n o t been investigated. With these considerations in mind, t h e c u r r e n t study, which is a continua tion of t h a t b e g u n with t h e aortic a n d m i t r a l valves, 1 1 " 1 5 w a s u n d e r t a k e n to determine t h e fre quency of e a c h etiologic type of tricuspid valve
851
852
SURGICAL PATHOLOGY OF TRICUSPID VALVE
disease in a surgical population and to document temporal changes in these frequencies that have occurred during the past 25 years. METHODS Study Group.—From the tissue registry at our institution, 363 tricuspid valves that had been excised surgically during the 25 years between January 1963 and December 1987 were examined. For gross examination of the valves, we used the methods described by Davies 16 and by Roberts. 17 Patients in this study may also have had replace ment or surgical repair of additional cardiac valves. Excluded from this study, however, were patients who underwent repair, rather than ex cision and replacement, of tricuspid valves. For each valve, the number of pieces excised was recorded. The leaflets were examined for fibrosis, perforation, vegetations, calcification, congenital malformations, and hooding defor mity. The commissures were examined for fusion, and the chordae tendineae were evaluated for fusion, fibrosis, stretching, rupture, and congen ital defects. When present, the papillary muscles were examined for fibrosis and rupture. The age and sex of each patient, date of op eration, clinical cardiac diagnosis, and func tional status of the tricuspid and other cardiac valves (as determined by cardiac catheterization, by echocardiography, and at operation) were ob tained from review of the patient's clinical records. Functional Groups.—Tricuspid valves desig nated as purely stenotic had, at most, a mild component of insufficiency. Similarly, insufficient valves that also had only mild stenosis were classi fied as purely regurgitant. Only valves with at least moderate stenosis and insufficiency were classified in the combined functional group.
Mayo Clin Proc, September 1988, Vol 63
Etiologic Groups.—On the basis of patho logic findings and clinical information, tricuspid valves were assigned to one of eight etiologic categories (Table 1). Valves that could not be classified on the basis of gross inspection or clinical history were considered indeterminate. As in our previous studies, 1 1 1 5 the term "postinflammatory disease," as suggested by Pomerance, 18 was favored over "rheumatic disease" be cause a history of rheumatic fever could not be verified in every case. This designation applied to valves characterized by a chronic, apparently noninfectious, fibrosing process that involved leaflets, commissures, and chordae tendineae and that produced structural distortions indistinguish able from confirmed cases of chronic rheumatic valvular disease. In the setting of congenital heart disease, func tional tricuspid disease was also categorized as con genital unless it was due to infective endocarditis. In the setting of left-sided rheumatic valve disease with chronic pulmonary venous hyper tension, the tricuspid valve was categorized as postinflammatory if it was involved by diffuse leaflet fibrosis and by commissural or chordal fusion, and dysfunction was considered solely due to pulmonary hypertension if the valve was involved only by annular dilatation or focal leaflet fibrosis, without commissural or chordal fusion. It is understood that pulmonary venous hypertension provided the basis for pulmonary arterial and right ventricular hypertension in these cases. RESULTS Functional Classification.—Of the 363 tricus pid valves reviewed (Table 1), 269 (74%) were purely insufficient, 84 (23%) were both insufficient and
Table 1.—Etiologic Distribution of 363 Surgically Excised Tricuspid Valves Year of study Classification 1963-1967 1968-1972 1973-1977 1978-1982 1983-1987 Postinflammatory disease 65 11 16 72 30 Congenital disorder 6 15 17 20 35 Pulmonary venous hypertension 9 28 8 3 8 Infective endocarditis 2 2 1 3 3 Carcinoid syndrome 0 0 0 1 4 Trauma 0 1 0 0 0 Iatrogenic 0 0 0 1 0 Indeterminate 0 1 1 0 0 Total 82 119 57 39 66
Total No. % 194 53 93 26 56 15 11 3 5 1 1 <1 1 <1 2 <1 363 100
Mayo Clin Proc, September 1988, Vol 63
stenotic, 8 (2%) were purely stenotic, and 2 (0.6%) were neither insufficient nor stenotic (Table 2). Two functionally normal valves were removed, one during repair of a double-outlet left ventricle because of straddling chordae tendineae and the other to allow septation during correction of a double-inlet left ventricle. Morphologic Classification.—Postinflammatory changes were the most common cause of all types of tricuspid valve dysfunction in this series, but the relative frequency of such changes de creased from 79% during the period from 1963 through 1967 to 24% during 1983 through 1987 (Fig. 1 and Table 1). In contrast, the frequency of congenital heart disease increased from 7% to 53% during the same time intervals. Accordingly, congenital heart disease is currently the most commonly observed cause for tricuspid valve re placement at our institution. mean age Age and Sex Distribution.—The of patients at the time of a tricuspid valve sur gical procedure (Fig. 2 and Table 3) was higher in the postinflammatory (51 years) and pulmonary venous hypertension (55 years) groups than in the group with congenital disorders (21 years). Female patients accounted for 241 cases (66%) of tricuspid valve removal and for 84% of the postinflammatory group and 64% of the pulmonary venous hypertension group. In contrast, male patients accounted for 73% of the infective endo carditis cases and 61% of the congenital disorders. Postinflammatory Disease.—The most com mon underlying cause for tricuspid valve excision was postinflammatory disease, which accounted for 194 of the 363 cases (53%) (Table 1). Postinflammatory disease was particularly prevalent among tricuspid valves with a functional element
Table 2.—Functional Distribution of 363 Surgically Excised Tricuspid Valves* Classification TS TS + TI TI Total 6 109 194 Postinflammatory disease 79 93t 1 87 Congenital disorder 3 56 0 56 Pulmonary venous hypertension 0 11 0 11 Infective endocarditis 0 5 1 2 Carcinoid syndrome 2 1 0 1 Trauma 0 1 0 1 Iatrogenic 0 2 0 2 Indeterminate 0 8 269 363 Total 84 *TI = tricuspid insufficiency; TS = tricuspid stenosis. tTwo valves were functionally normal (see text).
SURGICAL PATHOLOGY OF TRICUSPID VALVE
80
·χ79
70
Postinflammatory Congenital
60
'
S? 50 |
40
O
30 20 10
853
iL
^^30
1963-1967
28 24
H-"·"— 7
53
-5?
■
Pulm ven htn 8
I
I
— I
1966-1972
1973-1977
1978-1982
■
12
1983-1987
Years
Fig. 1. Temporal changes in cause of surgical excision of tricuspid valves during period from 1963 through 1987. Rela tive frequency of postinflammatory (rheumatic) disease de creased and that of congenital heart disease increased during study period. Pulm ven htn = pulmonary venous hypertension.
of stenosis (Table 2) and accounted for 6 of 8 cases (75%) of pure stenosis and for 79 of 84 examples (94%) of combined stenosis and incompetence. Furthermore, among 269 purely regurgitant tri cuspid valves, postinflammatory disease was the most common cause, accounting for 109 cases (41%). All 194 patients with postinflammatory tri cuspid valve disease also had mitral valve dis ease: 132 (68%) had combined mitral stenosis and insufficiency, 50 (26%) had pure mitral stenosis, and 12 (6%) had pure mitral insufficiency. The aortic valve was also diseased in 122 of the 194 patients (63%): 62 (32%) had combined aortic stenosis and insufficiency, 49 (25%) had pure aortic insufficiency, 11 (6%) had pure aortic stenosis, and 72 (37%) had normally functioning aortic valves. The manner in which the valvular components were distorted tended to determine the functional state (Fig. 3). Tricuspid stenosis generally re sulted from diffuse leaflet fibrosis and commissural fusion in a closed position. In contrast, postinflammatory tricuspid regurgitation was most commonly characterized by fibrotic retraction of leaflet elements, chordal fusion, and fusion of commissures in an opened position. Among 194 valves, commissural fusion was observed in each of 6 purely stenotic valves, 75 of 79 (95%) stenotic and regurgitant valves, and only 56 of 109 (51%o) purely incompetent valves. Chordal fusion was a feature of 40 of 109 (37%)
854
SURGICAL PATHOLOGY OF TRICUSPID VALVE
Postinflammatory
20-29
30-39
40-49
Mayo Clin Proc, September 1988, Vol 63
<40 yr old (n = 119)
>40 yr old (n = 244)
50-59
Age of patient, yr
Fig. 2. Relationship between age of patients and cause of tricuspid valve disease. A and B, Congenital heart disease is most common cause in younger patients, and postinflammatory disease is most common cause in older patients. Pulm ven htn - pulmonary venous hypertension.
purely incompetent valves, 2 of 6 (33%) purely stenotic valves, and 26 of 79 valves (33%) with stenosis and insufficiency. Calcification was ob served in only three cases (two purely regurgi tant, one both stenotic and regurgitant) and was considered mild in each specimen. Congenital Disorder.—Oi 97 patients in whom tricuspid valve disease was associated with under lying congenital heart disease, valvular dysfunc tion was due to the congenital malformation or secondary pulmonary hypertension in 93 and resulted from infective endocarditis in 4. Among the 93 specimens, the most common anomaly was Ebstein's malformation (Fig. 4 and Table 4); this defect accounted for 39 cases (42%), of which 38 were purely regurgitant and 1 was both stenotic and insufficient.
The anomalies associated with the other 54 cases were numerous (Table 4) and tended to be associated with hypoplasia, dysplasia (dysgenesis), or annular dilatation (Fig. 4); 51 of the 54 valves (94%) were purely regurgitant. All five patients with floppy tricuspid valves also under went operative replacement of regurgitant floppy mitral valves. Pulmonary Venous Hypertension.—In the group with pulmonary venous hypertension, tri cuspid regurgitation resulted primarily from an nular dilatation, and focal leaflet thickening was presumed to have occurred as a secondary hemodynamic phenomenon (Fig. 5). Each of the 56 patients with pulmonary venous hypertension (excluding cases of congenital heart disease) had underlying mitral valve disease: 26 (46%) had
Table 3.—Sex and Age of 363 Patients With Surgically Excised Tricuspid Valves Sex Age of patients (yr) M:F Mean Range Classification M 0-9 10-19 20-29 30-39 40-49 50-59 60-69 70-79 F ratio Postinflammatory disease 0 0.2 51 32 19-71 1 162 2 28 51 63 43 6 Congenital disorder 21 57 1-76 23 32 36 1.6 15 7 7 5 2 2 Pulmonary venous hypertension 55 20 35-78 0 0 36 0.6 0 3 11 20 19 3 Infective endocarditis 32 8 6-70 2 3 3 2.7 1 1 2 0 1 1 Carcinoid syndrome 56 3 46-69 0 0 2 0 0 3 1 1 0 1.5 Trauma 17 1 0 1 0 0 0 0 0 0 0 Iatrogenic 52 0 0 0 1 0 0 0 1 0 0 Indeterminate 61 1 55-66 0 0 1 0 0 0 1 1 0 1.0 0.5 Total 44 122 1-78 25 37 241 18 39 74 91 67 12
Mayo Clin Proc, September 1988, Vol 63
SURGICAL PATHOLOGY OF TRICUSPID VALVE
855
Fig. 3. Postinflammatory (rheumatic) tricuspid valve disease. A, Pure tricuspid stenosis (atrial aspect) in 41-year-old woman. Commissures are fused, leaflets are diffusely fibrotic, and functional orifice is small; no annular dilatation is present. B, Pure tricuspid insufficiency (atrial aspect) in 45-year-old man. Leaflets are diffusely thickened, but commissural fusion is mild; annulus is dilated. C and D, Combined tricuspid stenosis and insufficiency in 45-year-old woman. Leaflets are diffusely fibrotic and commissures are fused; the result is a fixed orifice that is both stenotic and regurgitant. Valve is viewed from atrial (C) and from ventricular (D) aspects, showing two tricuspid papillary muscles (PM).
combined mitral stenosis and insufficiency, 12 (21%) had mitral stenosis, and 16 (29%) had mitral insufficiency; in 2 (4%) who had undergone pre vious mitral valve replacement elsewhere, the functional status of the mitral valve was un known. Moreover, aortic valve disease was also present in 23 of the 56 patients (41%): 3 (5%) had aortic stenosis, 7 (12%) had aortic stenosis and insufficiency, 13 (23%) had aortic insufficiency,
and 33 (59%) had normal aortic valve function. The cause of left-sided valvular dysfunction was rheumatic in 46 (82%), ischemic in 3 (5%), floppy mitral valve in 1 (2%), left atrial myxoma in 1 (2%), cardiomyopathy in 1 (2%), and unknown in 4 (7%). Infective Endocarditis.—All 11 patients with infective endocarditis had purely regurgitant tri cuspid valves (Fig. 6). Vegetations were observed
856
SURGICAL PATHOLOGY OF TRICUSPID VALVE
Mayo Clin Proc, September 1988, Vol 63
Fig. 4. Congenitally abnormal tricuspid valves. A, Ebstein's malformation (atrial aspect) in 74-year-old man. Anterior leaflet (arrows) is large and irregularly formed. B, Ebstein's malformation (atrial aspect) in 14-year-old girl. Leaflets are sheetlike and have poor chordal development (arrows). C, Regurgitant double-orifice (*) tricuspid valve (atrial aspect) in 26-year-old woman with double-inlet left ventricle. Annulus is dilated, and leaflet thickening is result of regurgitant stream. D, Dysplastic tricuspid valve (atrial aspect) with stenosis and regurgitation in 2-year-old girl with pulmonary atresia and intact ventricular septum. Leaflets are thickened, commissures are fused, chordae tendineae are poorly formed, and annulus is hypoplastic.
in four cases, leaflet perforations in four, ruptured valves in three; and tricuspid and aortic valves chordae tendineae in three, leaflet destruction in in one. Associated conditions in these seven pa tients included congenital heart disease in two, two, and leaflet calcification in one. The infection was active in four patients, among presumptive rheumatic disease in two, and floppy whom the endocarditis was diagnosed preopera- mitral valve, prosthetic aortic valve, and chronic tively in two and at operation in two; cultures, alcoholism in one each. which were obtained in three patients, revealed Carcinoid Syndrome.—Carcinoid heart dis Candida albicans in two and viridans strepto ease caused pure tricuspid stenosis in one patient, cocci in one. Of the seven cases of healed tricuspid combined tricuspid stenosis and insufficiency in endocarditis, the causative organism was known two patients, and pure tricuspid insufficiency in in three (Streptococcus pneumoniae, cr-hemolytic two. The valve leaflets and chordae tendineae were thickened and distorted by dense fibrous streptococcus, and Staphylococcus). Endocarditis involved the tricuspid valve alone plaques typical of carcinoid heart disease (Fig. 7). in four patients, of whom two had congenital In this group of patients, the pulmonary valve heart disease, one had a history of intravenous was stenotic in three and both stenotic and in drug abuse, and one had a history of meningitis. sufficient in two. Trauma.—In one patient, chest trauma during Among the other seven cases of infective endo carditis, tricuspid and mitral valves were in a motor vehicle accident caused rupture of the volved in three; tricuspid, mitral, and aortic anterior tricuspid papillary muscle (Fig. 8) and
Mayo Clin Proc, September 1988, Vol 63
Table 4.—Functional Distribution of 93 Surgically Excised Tricuspid Valves Associated With Congenital Heart Disease* Total Functional category No. % TS TS + TI TI Malformation 39 42 1 38 0 Ebstein's anomaly Complete transposition 0 12 13 0 12 Double-inlet left ventricle 0 8 0 6 7t Atrial septal defect 0 6 6 0 6 Floppy tricuspid valve 0 5 5 0 5 Isolated tricuspid insufficiency 0 4 4 0 4 Tetralogy of Fallot 0 4 4 0 4 Double-outlet left ventricle 0 2 0 1 2t Double-outlet right 2 ventricle 0 2 0 2 Pulmonary atresia, intact 2 ventricular septum 0 2 2 0 2 Ventricular septal defect 0 2 0 2 1 Atrioventricular canal 0 1 0 1 1 Congenital mitral stenosis 0 1 0 1 Congenital tricuspid 1 stenosis 1 1 0 0 1 Mitral atresia 0 1 0 1 1 0 1 0 1 Patent ductus arteriosus Pulmonary atresia, 1 ventricular septal defect 0 1 0 1 Ventricular septal defect, straddling tricuspid 1 valve 0 1 0 1 1 Corrected transposition 0 1 0 1 93 Total 1 100 3 87 *TI = tricuspid insufficiency; TS = tricuspid stenosis. fOne valve was functionally normal (see text).
produced valvular prolapse and severe tricuspid insufficiency. Iatrogenic.—During repair of a ventricular septal defect, placement of a suture through the
SURGICAL PATHOLOGY OF TRICUSPID VALVE
857
anterior tricuspid leaflet resulted in tricuspid in sufficiency and necessitated valve replacement. Other Valve Procedures.—In addition to tri cuspid valve replacement, 293 of the 363 patients (81%) also had undergone replacement, repair, or excision of other cardiac valves (Table 5). Of the 272 patients who underwent tricuspid and mitral valve surgical procedures, with or without aortic or pulmonary valve procedures, 194 (71%) had postinflammatory disease and 56 (21%) had chronic pulmonary venous hypertension. Viewed from another perspective, all 250 patients with postinflammatory disease or pulmonary venous hypertension also had a mitral valve operation. Among 23 patients with tricuspid and pulmo nary valve surgical procedures, with or without operative procedures on left-sided valves, 15 (65%) had congenital heart disease, 5 had carcinoid heart disease, and 3 had congenital heart disease with infective endocarditis. Furthermore, of the 70 patients with tricuspid valve replacement alone, 64 (91%) had underlying congenital heart disease. DISCUSSION Postinflammatory Disease.—In patients who have undergone a tricuspid valve surgical proce dure, tricuspid valve dysfunction is most com monly caused by postinflammatory disease, which is presumably a manifestation of chronic rheu matic disease in most instances. It accounted for 39 of 41 cases (95%) of valve replacement or repair reported by Kratz and associates, 9 for 194 of 363 valve replacements (53%) in the current study,
Fig. 5. Pure tricuspid insufficiency associated with pulmonary venous hypertension in 57-year-old man. Leaflets are focally thickened, and annulus is greatly dilated (atrial aspect).
858
Mayo Clin Proc, September 1988, Vol 63
SURGICAL PATHOLOGY OF TRICUSPID VALVE
and for 9 of 21 valve replacements (43%) reported inflammatory disease (41%); it resulted from dif by Waller.19 A striking female preponderance has fuse leaflet fibrosis and retraction and from been noted in this group of patients, accounting commissural and chordal fusion that fixed the for 84% of the postinflammatory cases in the valve in a partially opened position. Tricuspid current study. This finding is similar to the re stenosis, with or without regurgitation, was over ported sex incidence for rheumatic mitral valve whelmingly caused by postinflammatory disease disease. 15,20 (92%) and resulted from diffuse leaflet fibrosis In the current investigation, pure tricuspid re- and from commissural fusion in a closed position. gurgitation was most commonly caused by post- Pure tricuspid stenosis was rare and accounted
/IETRIC 1
2
3
4
7
6
5
(
8
T ^_._
mSw
^LF·»
3EL*
.
,:
Fig. 6. Infective endocarditis. A through C, Pure tricuspid insufficiency due to Candida albicans infection in 46-year-old man with history of chronic alcoholism. Mitral and aortic valves were also infected. Extensive vegetations, shown along atrial aspect, involved all leaflets (A, arrows) and produced perforation (B, *). Candida organisms were plentiful microscopically (C). (Methenamine silver; x360.)
Mayo Clin Proc, September 1988, Vol 63
SURGICAL PATHOLOGY OF TRICUSPID VALVE
859
Fig. 7. Carcinoid heart disease. A, Tricuspid insufficiency (atrial aspect) in 47-year-old woman. Leaflets and chordae tendineae are thickened by carcinoid plaques. B, Tricuspid stenosis and insufficiency (atrial aspect) in 46-year-old woman. Carcinoid plaques appreciably thickened and distorted the leaflets and chordae tendineae. C, Severe tricuspid stenosis (atrial aspect) in 45-year-old man. D, Photomicrograph of tricuspid valve shown in C. Carcinoid plaque is adherent to valvular endocardium but does not produce destruction of overlying leaflet tissue (area between arrowheads). (Elastic van Gieson; x36.)
for only 3% of the postinflammatory valves; this finding is in contrast to pure mitral stenosis, which accounted for 33% of postinflammatory valves in a previous surgical pathology study. 15 Calcification was a feature of only three postinflammatory valves in the current series.
Fig. 8. Posttraumatic tricuspid insufficiency (atrial aspect) associated with rupture of necrotic anterior and medial tri cuspid papillary muscles (arrows) in 17-year-old boy who was involved in motor vehicle accident.
During the 25 years studied, the frequency of postinflammatory disease decreased appreciably over time; this trend may be a reflection of the decreasing incidence of acute rheumatic fever reported in Western countries during the past several decades. 21 This result corresponds to ob servations for the aortic and mitral valves. 14,15 Congenital Disorder.— Tricuspid valve dys function in the setting of congenital heart disease may be due to either intrinsic abnormalities of the valve apparatus or secondary annular dila tation. Ebstein's anomaly accounted for 61% of 129 cases in a recent review by Pasque and col leagues 10 of tricuspid valve replacement in chil dren and was observed in 40% of 97 patients with congenital heart disease in the current study. At our institution, the most common reason for tricuspid valve replacement is now congenital heart disease (53%) rather than postinflammatory dis ease (24%). This finding probably reflects changes in patient referral practices and advances in sur gical techniques. Pulmonary Venous Hypertension.— Tricus pid regurgitation was associated with chronic pulmonary hypertension in 15% of 363 cases in the current study and 38% of 21 operatively ex-
860
SURGICAL PATHOLOGY OF TRICUSPID VALVE
Mayo Clin Proc, September 1988, Vol 63
Table 5.—Operative Valve Procedures (Replacement, Repair, or Excision)* in 363 Patients With Tricuspid Valve Replacement TV + TV + TV + TV + MV MV + AV TV alone TV + PV PV + MV PV + AV Total Classification 194 101 93 0 0 0 0 Postinflammatory disease Congenital disorder 12 2 64 14 1 0 93 Pulmonary venous hypertension 38 18 0 0 0 0 56 Infective endocarditis 2 2 4 1 1 1 11 Carcinoid syndrome 0 0 0 5 0 0 5 Trauma 0 0 1 0 0 0 1 Iatrogenic 0 0 1 0 0 0 1 Indeterminate 2 0 0 0 0 0 2 Total 155 115 70 20 2 1 363 *When pulmonary valves were involved, only excision was performed. AV = aortic valve; MV : mitral valve; PV = pulmonary valve; TV = tricuspid valve.
cised valves reported by Waller.19 In autopsy series, tricuspid regurgitation was associated with conditions that produce pulmonary hyper tension in 47% of 45 patients described by Waller19 and in 67% of 24 patients with mitral stenosis described by Banerjee and co-workers.22 Among regurgitant tricuspid valves in patients with rheumatic heart disease, Waller19 noted a mean annular circumference of 13.6 cm in those with "functional" regurgitation (anatomically normal tricuspid valve) in comparison with 11.4 cm in those with "organic" regurgitation (ana tomically abnormal valves). In Waller's study, systolic pulmonary artery pressures were 40 mm Hg or less in 79% of patients with abnormal tricuspid valves and 55 mm Hg or more in 76% of patients with normal tricuspid valves. The remaining patients in each group had systolic pulmonary artery pressures between 40 and 55 mm Hg. In a combined echocardiographic and angiographic study, 23 however, no significant hemodynamic differences were noted between the organic and functional groups. Similarly, in 25 patients with tricuspid regurgitation associated with acquired pure mitral regurgitation in a re port by Cohen and associates, 24 a comparison of preoperative clinical and hemodynamic findings revealed no features that would distinguish those with anatomically normal valves from those with organic valve disease. Increased maximal diameter of the tricuspid valve has been demonstrated angiographically in patients with functional tricuspid regurgitation, and patients with moderate or severe regurgita tion have had reduced annular shortening. 25 By echocardiography, annular area (corrected for
body surface area) has been shown to increase with the grade of functional tricuspid regurgitation. 26 Infective Endocarditis.—In pediatric patients, infective endocarditis occurs most commonly in the setting of congenital heart disease. 27 " 29 In adults, however, rheumatic heart disease, mitral valve prolapse, degenerative valve disease, pros thetic valves, and congenital heart disease are all important underlying conditions, 30-32 as the cur rent study confirms. Among patients with rheu matic heart disease, infective endocarditis seems to involve native valves less often and prosthetic valves more frequently than 20 years ago. 31 More over, infections of the tricuspid valve, like the left-sided valves, involve men more often than 12,15,30-32
women. ' ■ With involvement of right-sided valves, intra venous drug abuse emerges as an important pre disposing factor for infective endocarditis, along with alcoholism, generalized infections in pa tients with immunologic disorders, congenital heart disease, and dermal infections. 33 When only heroin addicts are considered, the tricuspid valve is involved alone in most cases. 34 Excision 35 or replacement 36 of the tricuspid valve may be nec essary when sepsis persists despite adequate anti biotic treatment, when septic pulmonary emboli recur, or when acute right-sided heart failure develops.36 Carcinoid Syndrome.—Although tricuspid valve replacement may prolong life and alleviate symptoms in selected patients with metastatic tumor and carcinoid heart disease,37 only 18 cases of tricuspid valve operation for carcinoid heart disease have been reported previously (Table g^ 38-54 y a i v u i a r d i s e a s e m a y progress even after
Mayo Clin Proc, September 1988, Vol 63
SURGICAL PATHOLOGY OF TRICUSPID VALVE
861
successful extirpation of the tumor, and valve surgical population (94% of cases in the current replacement may be considered in such cases. 55 series), other causes not present in our 363 Carcinoid plaques observed at autopsy on a Han cases have been the subject of several reports cock porcine heterograft 8 months after implan (Table 7). 62-67 tation 49 raise questions about the suitability of tissue prostheses in patients with carcinoid heart REFERENCES disease. 1. Silver MD: Obstruction to blood flow related to tricuspid, pulmonary, and mitral valves. In Cardiovascular Pathol Trauma.—Ruptured chordae tendineae,56"58 rup ogy. Vol 1. Edited by MD Silver. New York, Churchill tured papillary muscles, 59,60 and dislocated leaf Livingstone, 1983, pp 551-574 61 lets, all of which caused severe tricuspid regur- 2. Edwards JE: The spectrum and clinical significance of tricuspid regurgitation. Pract Cardiol 6:86-95, 1980 gitation, have been described after chest trauma 3. Sugiura M, Matsushita S, Ueda K: A clinicopathological and may lead to tricuspid valve replacement. study on valvular diseases in 3,000 consecutive autopsyes Other Tricuspid Valve Disease.—Although [sic] of the aged. Jpn Circ J 46:337-345,1982 postinflammatory disease, pulmonary hyperten 4. Rose AG: Etiology of acquired valvular heart disease in adults: a survey of 18,132 autopsies and 100 consecutive sion, and congenital heart disease account for valve-replacement operations. Arch Pathol Lab Med most cases of tricuspid valve dysfunction in the 110:385-388,1986
Table 6.—Tricuspid Valve Operations in Patients With Carcinoid Heart Disease Age (yr) Procedure* Reference and sex Tricuspid valve 35 F Wright & Mulder,38 1963 annuloplasty, pulmonary valvotomy TVR, pulmonary 55 F Aroesty et al, 39 1966 valvotomy 40 TVR, pulmonary 26 F Carpena et al, 1973 valvotomy TVR 60 M Lund et al,41 197442 46 F TVR Honey & Paneth, 1975 26 F TVR, PVR McGuire et al, 43 1978 70 F TVR Herreman et al, 44 1978 TVR, PVR 64 F Nielsen & Manners, 45 1979 46 TVR, pulmonary 30 F Okada et al, 1979 valvectomy TVR 60 F Sworn et al, 47481980 TVR, pulmonary 34 M Hendel et al, 1980 valvotomy 49 53 M TVR Schoen et al, 50 1981 TVR, PVR 62 M Gutierrez et al, 1982 TVR 56 F Kessler,51 1983 TVR 56 F Miller et al, 52 1983 53 TVR, pulmonary 56 F DiSesa et al, 1985 valvectomy TVR, pulmonary 32 F valvectomy TVR, PVR 78 F Blick et al, 54 1988 TVR, pulmonary 69 M Current study, 1988 valvectomy TVR, pulmonary 55 M valvectomy 45 M TVR, pulmonary valvectomy TVR, pulmonary 47 F valvectomy TVR, pulmonary 46 F valvectomy
5. Waller BF, Moriarty AT, Eble JN, Davey DM, Hawley DA, Pless JE: Etiology of pure tricuspid regurgitation based on anular circumference and leaflet area: analysis of 45 necropsy patients with clinical and morphologic evidence of pure tricuspid regurgitation. J Am Coll Car diol 7:1063-1074,1986 6. Lev M, Liberthson RR, Joseph RH, Seten CE, Kunske RD, Eckner FAO, Miller RA: The pathologic anatomy of Ebstein's disease. Arch Pathol 90:334-343,1970 7. Zuberbuhler JR, Allwork SP, Anderson RH: The spec trum of Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 77:202-211, 1979 8. Waller BF, Bloch T, Barker BG, Roe SJ, Brown JW, Mahomed Y: Evaluation of operatively excised cardiac valves: etiologic determination of valvular heart disease. Cardiol Clin 2:687-716, November 1984 9. Kratz JM, Crawford FA Jr, Stroud MR, Appleby DC Jr, Hanger KH: Trends and results in tricuspid valve sur gery. Chest 88:837-840,1985 10. Pasque M, Williams WG, Coles JG, Trusler GA, Freedom RM: Tricuspid valve replacement in children. Ann Thorac Surg 44:164-168,1987 11. Subramanian R, Olson LJ, Edwards WD: Surgical pa thology of pure aortic stenosis: a study of 374 cases. Mayo Clin Proc 59:683-690,1984 12. Olson LJ, Subramanian R, Edwards WD: Surgical pa thology of pure aortic insufficiency: a study of 225 cases. Mayo Clin Proc 59:835-841,1984 13. Subramanian R, Olson LJ, Edwards WD: Surgical pa thology of combined aortic stenosis and insufficiency: a study of 213 cases. Mayo Clin Proc 60:247-254, 1985
Table 7.—Additional Reported Causes of Tricuspid Valve Dysfunction in Surgically Treated Patients Functional status* Cause Reference 62 Aneurysm of sinus of Valsalva TS + TI Right atrial and ventricular thrombosis TS 63 Right ventricular infarction TI 64 Libman-Sacks endocarditis TI 65 Giant blood cyst of tricuspid valve TS 66 Hypereosinophilic syndrome TI 67
*PVR = pulmonary valve replacement; TVR = tricuspid valve *TI = tricuspid insufficiency; TS = tricuspid stenosis. replacement.
862
SURGICAL PATHOLOGY OF TRICUSPID VALVE
14. Passik CS, Ackermann DM, Pluth JR, Edwards WD: Temporal changes in the causes of aortic stenosis: a surgical pathologic study of 646 cases. Mayo Clin Proc 62:119-123,1987 15. Olson LJ, Subramanian R, Ackermann DM, Orszulak TA, Edwards WD: Surgical pathology of the mitral valve: a study of 712 cases spanning 21 years. Mayo Clin Proc 62:22-34,1987 16. Davies MJ: Pathology of Cardiac Valves. Boston, Butterworth Publishers, 1980, pp 124-129 17. Roberts WC: Examining operatively excised cardiac valves (editorial). Am J Cardiol 59:1434-1436,1987 18. Pomerance A: Chronic rheumatic and other inflamma tory valve disease. In The Pathology of the Heart. Edited by A Pomerance, MJ Davies. Oxford, England, Blackwell Scientific Publications, 1975, pp 307-326 19. Waller BF: Etiology of pure tricuspid regurgitation. Cardiovasc Clin 17 (No. 2):53-95,1987 20. Hanson TP, Edwards BS, Edwards JE: Pathology of sur gically excised mitral valves: one hundred consecutive cases. Arch Pathol Lab Med 109:823-828,1985 21. Annegers J F , Pillman NL, Weidman WH, Kurland LT: Rheumatic fever in Rochester, Minnesota, 1935-1978. Mayo Clin Proc 57:753-757,1982 22. Banerjee A, Khanna SK, Beohar PC, Narayanan PS, Malhotra V, Mehta VK, Khalilullah M, Gupta MP: Tri cuspid regurgitation associated with mitral stenosis: a clinico-pathological study. Indian Heart J 34:8-12, 1982 23. Daniels SJ, Mintz GS, Kotler MN: Rheumatic tricuspid valve disease: two-dimensional echocardiographic, hemodynamic, and angiographic correlations. Am J Cardiol 51:492-496,1983 24. Cohen SR, Sell JE, Mclntosh CL, Clark RE: Tricuspid regurgitation in patients with acquired, chronic, pure mitral regurgitation. I. Prevalence, diagnosis, and com parison of preoperative clinical and hemodynamic fea tures in patients with and without tricuspid regurgita tion. J Thorac Cardiovasc Surg 94:481-487,1987 25. Ubago JL, Figueroa A, Ochoteco A, Colman T, Duran RM, Duran CG: Analysis of the amount of tricuspid valve anular dilatation required to produce functional tricuspid regurgitation. Am J Cardiol 52:155-158,1983 26. Mikami T, Kudo T, Sakurai N, Sakamoto S, Tanabe Y, Yasuda H: Mechanisms for development of functional tricuspid regurgitation determined by pulsed Doppler and two-dimensional echocardiography. Am J Cardiol 53:160-163,1984 27. Johnson CM, Rhodes KH: Pediatric endocarditis. Mayo Clin Proc 57:86-94,1982 28. Van Hare GF, Ben-Shachar G, Liebman J, Boxerbaum B, Riemenschneider TA: Infective endocarditis in infants and children during the past 10 years: a decade of change. Am Heart J 107:1235-1240,1984 29. Sholler GF, Hawker RE, Celermajer JM: Infective endo carditis in childhood. Pediatr Cardiol 6:183-186,1986 30. Kaye D: Changing pattern of infective endocarditis. Am J Med 78 (Suppl 6B):157-162,1985 31. Griffin MR, Wilson WR, Edwards WD, O'Fallon WM, Kurland LT: Infective endocarditis: Olmsted County, Minnesota, 1950 through 1981. JAMA 254:1199-1202, 1985 32. McKinsey DS, Ratts TE, Bisno AL: Underlying cardiac lesions in adults with infective endocarditis: the chang ing spectrum. Am J Med 82:681-688,1987 33. Roberts WC, Buchbinder NA: Right-sided valvular infec tive endocarditis: a clinicopathologic study of twelve necropsy patients. Am J Med 53:7-19,1972 34. Banks T, Fletcher R, Ali N: Infective endocarditis in heroin addicts. Am J Med 55:444-451, 1973
Mayo Clin Proc, September 1988, Vol 63
35. Arbulu A, Thorns NW, Chiscano A, Wilson RF: Total tricuspid valvulectomy without replacement in the treat ment of Pseudomonas endocarditis. Surg Forum 22:162164,1971 36. Stern HJ, Sisto DA, Strom JA, Soeiro R, Jones SR, Frater RWM: Immediate tricuspid valve replacement for endo carditis: indications and results. J Thorac Cardiovasc Surg 91:163-167,1986 37. Strickman NE, Rossi PA, Massumkhani GA, Hall RJ: Carcinoid heart disease: a clinical, pathologic, and thera peutic update. Curr Probl Cardiol 6:1-42, February 1982 38. Wright PW, Mulder DG: Carcinoid heart disease: report of a case treated by open heart surgery. Am J Cardiol 12:864-868,1963 39. Aroesty JM, DeWeese JA, Hoffman MJ, Yu PN: Car cinoid heart disease: successful repair of the valvular lesions under cardiopulmonary bypass. Circulation 34:105110,1966 40. Carpena C, Kay JH, Mendez AM, Redington JV, Zubiate P, Zucker R: Carcinoid heart disease: surgery for tricus pid and pulmonary valve lesions. Am J Cardiol 32:229233,1973 41. Lund HG, Cleveland RJ, Greenberg LH, Lippmann M, Dollinger MR: Tricuspid valve replacement in carcinoid heart disease. West J Med 120:412-415,1974 42. Honey M, Paneth M: Carcinoid heart disease: successful tricuspid valve replacement. Thorax 30:464-469,1975 43. McGuire MR, Pugh DM, Dunn MI: Carcinoid heart dis ease: restrictive cardiomyopathy as a late complication. J Kansas Med Soc 79:661-662; 665,1978 44. Herreman F, Vernant P, Cachera JP, Monier P: Insuffisance tricuspidienne sovere et tumeur carcinoide primi tive de l'ovaire: succes du remplacement valvulaire ä long terme; a propos d'une observation. Arch Mal Coeur 71:7280,1978 45. Nielsen MS, Manners JM: Valve replacement in carcinoid syndrome: anaesthetic management for tricuspid and pulmonary valve surgery. Anaesthesia 34:474-499, 1979 46. Okada RD, Ewy GA, Copeland JG: Echocardiography and surgery in tricuspid and pulmonary valve stenosis due to carcinoid syndrome. Cardiovasc Med 4:871-874; 879; 881, 1979 47. Sworn MJ, Edlin GP, McGill DAF, Mousley JS, Monro JL: Tricuspid valve replacement in carcinoid syndrome due to ovarian primary. Br Med J [Clin Res] 280:85-86, 1980 48. Hendel N, Leckie B, Richards J: Carcinoid heart disease: eight-year survival following tricuspid valve replacement and pulmonary valvotomy. Ann Thorac Surg 30:391-395, 1980 49. Schoen FJ, Hausner RJ, Howell J F , Beazley HL, Titus JL: Porcine heterograft valve replacement in carcinoid heart disease. J Thorac Cardiovasc Surg 81:100-105,1981 50. Gutierrez FR, McKnight RC, Jaffe AS, Ludbrook PA, Biello D, Weldon CS: Double porcine valve replacement in carcinoid heart disease. Chest 81:101-103, 1982 51. Kessler MR: Carcinoid heart disease: a unique case of postvalvotomy diagnosis. Arch Intern Med 143:1615-1616, 1983 52. Miller BR, Vohr FH, Christian FV, Singh AK: Cardiac valvular replacement in carcinoid heart disease. Am J Med 75:896-898, 1983 53. DiSesa VJ, Mills RM Jr, Collins J J Jr: Surgical man agement of carcinoid heart disease. Chest 88:789-791, 1985 54. Blick DR, Zoghbi WA, Lawrie GM, Verani MS: Carcinoid heart disease presenting as right-to-left shunt and con gestive heart failure: successful surgical treatment. Am Heart J 115:201-203,1988
Mayo Clin Proc, September 1988, Vol 63
55. Trell E, Rausing A, Ripa J, Torp A, Waldenström J: Carcinoid heart disease: clinicopathologic findings and follow-up in 11 cases. Am J Med 54:433-444,1973 56. Katz NM, Pallas RS: Traumatic rupture of the tricuspid valve: repair by chordal replacements and annuloplasty. J Thorac Cardiovasc Surg 91:310-314,1986 57. Watanabe T, Katsume H, Matsukubo H, Furukawa K, Ijichi H: Ruptured chordae tendineae of the tricuspid valve due to nonpenetrating trauma: echocardiographic findings. Chest 80:751-753,1981 58. Stephenson LW, Mac Vaugh H III, Kastor JA: Tricuspid valvular incompetence and rupture of the ventricular septum caused by nonpenetrating trauma. J Thorac Cardiovasc Surg 77:768-772,1979 59. Berkery W, Hare C, Warner RA, Battaglia J, Potts JL: Nonpenetrating traumatic rupture of the tricuspid valve: formation of ventricular septal aneurysm and subsequent septal necrosis; recognition by two-dimensional Doppler echocardiography. Chest 91:778-780,1987 60. Fracasso A, Pothen P, Gallucci V: Tricuspid regurgitation caused by blunt chest trauma in association with pericardial agenesis: surgical correction after eight years. Thorax 37:75-76,1982 61. Gayet C, Pierre B, Delahaye J-P, Champsaur G, AndreFouet X, Rueff P: Traumatic tricuspid insufficiency: an underdiagnosed disease. Chest 92:429-432,1987
SURGICAL PATHOLOGY OF TRICUSPID VALVE
863
62. Gibbs KL, Reardon MJ, Strickman NE, de Castro CM, Gerard JA, Rycyna JL, Hall RJ, Cooley DA: Hemodynamic compromise (tricuspid stenosis and insufficiency) caused by an unruptured aneurysm of the sinus of Valsalva. J Am Coll Cardiol 7:1177-1181,1986 63. Mestres CA, de Lacy AM, Pomar JL: Massive right atrial and ventricular thrombosis after peritoneovenous shunt ing treated by thrombectomy and tricuspid valvectomy. Ann Thorac Surg 44:205-206,1987 64. Korr KS, Levinson H, Bough EW, Gheorghiade M, Stone J, McEnany MT, Shulman RS: Tricuspid valve replace ment for cardiogenic shock after acute right ventricular infarction. JAMA 244:1958-1960,1980 65. Laufer J, Frand M, Milo S: Valve replacement for severe tricuspid regurgitation caused by Libman-Sacks endo carditis. Br Heart J 48:294-297,1982 66. Gallucci V, Stritoni P, Fasoli G, Thiene G: Giant blood cyst of tricuspid valve: successful excision in an infant. Br Heart J 38:990-992,1976 67. Harley JB, Mclntosh CL, Kirklin JJW, Maron BJ, Gottdiener J, Roberts WC, Fauci AS: Atrioventricular valve replacement in the idiopathic hypereosinophilic syn drome. Am J Med 73:77-81,1982
TRANSESOPHAGEAL ECHOCARDIOGRAPHY Bound reprints of the article from July 1988 issue are available at a cost of $6.50. Please send check with order, made payable to MAYO CLINIC PRO CEEDINGS, Room 1035 Plummer Building, Mayo Clinic, Rochester, MN 55905.
Vol. 63
SEPTEMBER 1988
Surgical Pathology of the Tricuspid Valve: A Study of 363 Cases Spanning 25 Years
ARTHUR J. HAUCK, M.D., Division of Pathology; DANIEL P. FREEMAN, M.D., Department of Neurology; DOUGLAS M. ACKERMANN, M.D.,* Division of Pathology; GORDON K. DANIELSON, M.D., Division of Thoracic and Cardiovascular Surgery; WILLIAM D. EDWARDS, M.D., Division of Pathology
Surgical pathologic features of the tricuspid valve were reviewed in 3 6 3 patients w h o had undergone tricuspid valve replacement at our institution during the period 1963 through 1987. Valves were purely regurgitant in 74%, stenotic and regurgitant in 23%, and purely stenotic in 2%; t w o valves were neither stenotic nor regurgitant. Among 2 6 9 purely insufficient tricuspid valves, the four most common causes were postinflammatory disease (41%), congenital disorder (32%), pulmonary venous hypertension (21%), and infective endocarditis (4%). Of 92 cases of tricuspid stenosis, with or without regurgitation, postinflammatory disease w a s observed in 92%. Female patients accounted for 66% of the 3 6 3 cases, including 84% of those with postinflammatory disease and 64% of those with pulmonary venous hypertension. In contrast, male patients accounted for 73% of cases with endocarditis and 61% w i t h congenital heart disease. Although postinflammatory disease accounted for 53% of the 3 6 3 cases, its relative frequency diminished from 79% during 1963 through 1967 to only 24% during 1983 through 1987. This trend may reflect the decreasing incidence of acute rheumatic fever reported in Western countries. During the same time interval, the relative frequency of congenital heart disease as a cause of tricuspid dysfunction increased from 7% to 53%, and it is currently the most common cause in our surgical population. This finding apparently reflects changes in patient referral practices and the development of n e w operative procedures.
A l t h o u g h t h e characteristic pathologic features of various types of tricuspid valve disease have been described in specimens obtained from au topsy, 1 - 7 little information h a s been published *Current address: University of Louisville, Louisville, Kentucky. Address reprint requests to Dr. W. D. Edwards, Section of Medical Pathology, Mayo Clinic, Rochester, MN 55905. Mayo Clin Proc 63:851-863,1988
about t h e d i s t i n g u i s h i n g features of tricuspid valves removed a t operation. 8 " 1 0 Moreover, to our knowledge, c h a n g e s i n t h e relative frequencies of t h e various causes of tricuspid valve disease have n o t been investigated. With these considerations in mind, t h e c u r r e n t study, which is a continua tion of t h a t b e g u n with t h e aortic a n d m i t r a l valves, 1 1 " 1 5 w a s u n d e r t a k e n to determine t h e fre quency of e a c h etiologic type of tricuspid valve
851
852
SURGICAL PATHOLOGY OF TRICUSPID VALVE
disease in a surgical population and to document temporal changes in these frequencies that have occurred during the past 25 years. METHODS Study Group.—From the tissue registry at our institution, 363 tricuspid valves that had been excised surgically during the 25 years between January 1963 and December 1987 were examined. For gross examination of the valves, we used the methods described by Davies 16 and by Roberts. 17 Patients in this study may also have had replace ment or surgical repair of additional cardiac valves. Excluded from this study, however, were patients who underwent repair, rather than ex cision and replacement, of tricuspid valves. For each valve, the number of pieces excised was recorded. The leaflets were examined for fibrosis, perforation, vegetations, calcification, congenital malformations, and hooding defor mity. The commissures were examined for fusion, and the chordae tendineae were evaluated for fusion, fibrosis, stretching, rupture, and congen ital defects. When present, the papillary muscles were examined for fibrosis and rupture. The age and sex of each patient, date of op eration, clinical cardiac diagnosis, and func tional status of the tricuspid and other cardiac valves (as determined by cardiac catheterization, by echocardiography, and at operation) were ob tained from review of the patient's clinical records. Functional Groups.—Tricuspid valves desig nated as purely stenotic had, at most, a mild component of insufficiency. Similarly, insufficient valves that also had only mild stenosis were classi fied as purely regurgitant. Only valves with at least moderate stenosis and insufficiency were classified in the combined functional group.
Mayo Clin Proc, September 1988, Vol 63
Etiologic Groups.—On the basis of patho logic findings and clinical information, tricuspid valves were assigned to one of eight etiologic categories (Table 1). Valves that could not be classified on the basis of gross inspection or clinical history were considered indeterminate. As in our previous studies, 1 1 1 5 the term "postinflammatory disease," as suggested by Pomerance, 18 was favored over "rheumatic disease" be cause a history of rheumatic fever could not be verified in every case. This designation applied to valves characterized by a chronic, apparently noninfectious, fibrosing process that involved leaflets, commissures, and chordae tendineae and that produced structural distortions indistinguish able from confirmed cases of chronic rheumatic valvular disease. In the setting of congenital heart disease, func tional tricuspid disease was also categorized as con genital unless it was due to infective endocarditis. In the setting of left-sided rheumatic valve disease with chronic pulmonary venous hyper tension, the tricuspid valve was categorized as postinflammatory if it was involved by diffuse leaflet fibrosis and by commissural or chordal fusion, and dysfunction was considered solely due to pulmonary hypertension if the valve was involved only by annular dilatation or focal leaflet fibrosis, without commissural or chordal fusion. It is understood that pulmonary venous hypertension provided the basis for pulmonary arterial and right ventricular hypertension in these cases. RESULTS Functional Classification.—Of the 363 tricus pid valves reviewed (Table 1), 269 (74%) were purely insufficient, 84 (23%) were both insufficient and
Table 1.—Etiologic Distribution of 363 Surgically Excised Tricuspid Valves Year of study Classification 1963-1967 1968-1972 1973-1977 1978-1982 1983-1987 Postinflammatory disease 65 11 16 72 30 Congenital disorder 6 15 17 20 35 Pulmonary venous hypertension 9 28 8 3 8 Infective endocarditis 2 2 1 3 3 Carcinoid syndrome 0 0 0 1 4 Trauma 0 1 0 0 0 Iatrogenic 0 0 0 1 0 Indeterminate 0 1 1 0 0 Total 82 119 57 39 66
Total No. % 194 53 93 26 56 15 11 3 5 1 1 <1 1 <1 2 <1 363 100
Mayo Clin Proc, September 1988, Vol 63
stenotic, 8 (2%) were purely stenotic, and 2 (0.6%) were neither insufficient nor stenotic (Table 2). Two functionally normal valves were removed, one during repair of a double-outlet left ventricle because of straddling chordae tendineae and the other to allow septation during correction of a double-inlet left ventricle. Morphologic Classification.—Postinflammatory changes were the most common cause of all types of tricuspid valve dysfunction in this series, but the relative frequency of such changes de creased from 79% during the period from 1963 through 1967 to 24% during 1983 through 1987 (Fig. 1 and Table 1). In contrast, the frequency of congenital heart disease increased from 7% to 53% during the same time intervals. Accordingly, congenital heart disease is currently the most commonly observed cause for tricuspid valve re placement at our institution. mean age Age and Sex Distribution.—The of patients at the time of a tricuspid valve sur gical procedure (Fig. 2 and Table 3) was higher in the postinflammatory (51 years) and pulmonary venous hypertension (55 years) groups than in the group with congenital disorders (21 years). Female patients accounted for 241 cases (66%) of tricuspid valve removal and for 84% of the postinflammatory group and 64% of the pulmonary venous hypertension group. In contrast, male patients accounted for 73% of the infective endo carditis cases and 61% of the congenital disorders. Postinflammatory Disease.—The most com mon underlying cause for tricuspid valve excision was postinflammatory disease, which accounted for 194 of the 363 cases (53%) (Table 1). Postinflammatory disease was particularly prevalent among tricuspid valves with a functional element
Table 2.—Functional Distribution of 363 Surgically Excised Tricuspid Valves* Classification TS TS + TI TI Total 6 109 194 Postinflammatory disease 79 93t 1 87 Congenital disorder 3 56 0 56 Pulmonary venous hypertension 0 11 0 11 Infective endocarditis 0 5 1 2 Carcinoid syndrome 2 1 0 1 Trauma 0 1 0 1 Iatrogenic 0 2 0 2 Indeterminate 0 8 269 363 Total 84 *TI = tricuspid insufficiency; TS = tricuspid stenosis. tTwo valves were functionally normal (see text).
SURGICAL PATHOLOGY OF TRICUSPID VALVE
80
·χ79
70
Postinflammatory Congenital
60
'
S? 50 |
40
O
30 20 10
853
iL
^^30
1963-1967
28 24
H-"·"— 7
53
-5?
■
Pulm ven htn 8
I
I
— I
1966-1972
1973-1977
1978-1982
■
12
1983-1987
Years
Fig. 1. Temporal changes in cause of surgical excision of tricuspid valves during period from 1963 through 1987. Rela tive frequency of postinflammatory (rheumatic) disease de creased and that of congenital heart disease increased during study period. Pulm ven htn = pulmonary venous hypertension.
of stenosis (Table 2) and accounted for 6 of 8 cases (75%) of pure stenosis and for 79 of 84 examples (94%) of combined stenosis and incompetence. Furthermore, among 269 purely regurgitant tri cuspid valves, postinflammatory disease was the most common cause, accounting for 109 cases (41%). All 194 patients with postinflammatory tri cuspid valve disease also had mitral valve dis ease: 132 (68%) had combined mitral stenosis and insufficiency, 50 (26%) had pure mitral stenosis, and 12 (6%) had pure mitral insufficiency. The aortic valve was also diseased in 122 of the 194 patients (63%): 62 (32%) had combined aortic stenosis and insufficiency, 49 (25%) had pure aortic insufficiency, 11 (6%) had pure aortic stenosis, and 72 (37%) had normally functioning aortic valves. The manner in which the valvular components were distorted tended to determine the functional state (Fig. 3). Tricuspid stenosis generally re sulted from diffuse leaflet fibrosis and commissural fusion in a closed position. In contrast, postinflammatory tricuspid regurgitation was most commonly characterized by fibrotic retraction of leaflet elements, chordal fusion, and fusion of commissures in an opened position. Among 194 valves, commissural fusion was observed in each of 6 purely stenotic valves, 75 of 79 (95%) stenotic and regurgitant valves, and only 56 of 109 (51%o) purely incompetent valves. Chordal fusion was a feature of 40 of 109 (37%)
854
SURGICAL PATHOLOGY OF TRICUSPID VALVE
Postinflammatory
20-29
30-39
40-49
Mayo Clin Proc, September 1988, Vol 63
<40 yr old (n = 119)
>40 yr old (n = 244)
50-59
Age of patient, yr
Fig. 2. Relationship between age of patients and cause of tricuspid valve disease. A and B, Congenital heart disease is most common cause in younger patients, and postinflammatory disease is most common cause in older patients. Pulm ven htn - pulmonary venous hypertension.
purely incompetent valves, 2 of 6 (33%) purely stenotic valves, and 26 of 79 valves (33%) with stenosis and insufficiency. Calcification was ob served in only three cases (two purely regurgi tant, one both stenotic and regurgitant) and was considered mild in each specimen. Congenital Disorder.—Oi 97 patients in whom tricuspid valve disease was associated with under lying congenital heart disease, valvular dysfunc tion was due to the congenital malformation or secondary pulmonary hypertension in 93 and resulted from infective endocarditis in 4. Among the 93 specimens, the most common anomaly was Ebstein's malformation (Fig. 4 and Table 4); this defect accounted for 39 cases (42%), of which 38 were purely regurgitant and 1 was both stenotic and insufficient.
The anomalies associated with the other 54 cases were numerous (Table 4) and tended to be associated with hypoplasia, dysplasia (dysgenesis), or annular dilatation (Fig. 4); 51 of the 54 valves (94%) were purely regurgitant. All five patients with floppy tricuspid valves also under went operative replacement of regurgitant floppy mitral valves. Pulmonary Venous Hypertension.—In the group with pulmonary venous hypertension, tri cuspid regurgitation resulted primarily from an nular dilatation, and focal leaflet thickening was presumed to have occurred as a secondary hemodynamic phenomenon (Fig. 5). Each of the 56 patients with pulmonary venous hypertension (excluding cases of congenital heart disease) had underlying mitral valve disease: 26 (46%) had
Table 3.—Sex and Age of 363 Patients With Surgically Excised Tricuspid Valves Sex Age of patients (yr) M:F Mean Range Classification M 0-9 10-19 20-29 30-39 40-49 50-59 60-69 70-79 F ratio Postinflammatory disease 0 0.2 51 32 19-71 1 162 2 28 51 63 43 6 Congenital disorder 21 57 1-76 23 32 36 1.6 15 7 7 5 2 2 Pulmonary venous hypertension 55 20 35-78 0 0 36 0.6 0 3 11 20 19 3 Infective endocarditis 32 8 6-70 2 3 3 2.7 1 1 2 0 1 1 Carcinoid syndrome 56 3 46-69 0 0 2 0 0 3 1 1 0 1.5 Trauma 17 1 0 1 0 0 0 0 0 0 0 Iatrogenic 52 0 0 0 1 0 0 0 1 0 0 Indeterminate 61 1 55-66 0 0 1 0 0 0 1 1 0 1.0 0.5 Total 44 122 1-78 25 37 241 18 39 74 91 67 12
Mayo Clin Proc, September 1988, Vol 63
SURGICAL PATHOLOGY OF TRICUSPID VALVE
855
Fig. 3. Postinflammatory (rheumatic) tricuspid valve disease. A, Pure tricuspid stenosis (atrial aspect) in 41-year-old woman. Commissures are fused, leaflets are diffusely fibrotic, and functional orifice is small; no annular dilatation is present. B, Pure tricuspid insufficiency (atrial aspect) in 45-year-old man. Leaflets are diffusely thickened, but commissural fusion is mild; annulus is dilated. C and D, Combined tricuspid stenosis and insufficiency in 45-year-old woman. Leaflets are diffusely fibrotic and commissures are fused; the result is a fixed orifice that is both stenotic and regurgitant. Valve is viewed from atrial (C) and from ventricular (D) aspects, showing two tricuspid papillary muscles (PM).
combined mitral stenosis and insufficiency, 12 (21%) had mitral stenosis, and 16 (29%) had mitral insufficiency; in 2 (4%) who had undergone pre vious mitral valve replacement elsewhere, the functional status of the mitral valve was un known. Moreover, aortic valve disease was also present in 23 of the 56 patients (41%): 3 (5%) had aortic stenosis, 7 (12%) had aortic stenosis and insufficiency, 13 (23%) had aortic insufficiency,
and 33 (59%) had normal aortic valve function. The cause of left-sided valvular dysfunction was rheumatic in 46 (82%), ischemic in 3 (5%), floppy mitral valve in 1 (2%), left atrial myxoma in 1 (2%), cardiomyopathy in 1 (2%), and unknown in 4 (7%). Infective Endocarditis.—All 11 patients with infective endocarditis had purely regurgitant tri cuspid valves (Fig. 6). Vegetations were observed
856
SURGICAL PATHOLOGY OF TRICUSPID VALVE
Mayo Clin Proc, September 1988, Vol 63
Fig. 4. Congenitally abnormal tricuspid valves. A, Ebstein's malformation (atrial aspect) in 74-year-old man. Anterior leaflet (arrows) is large and irregularly formed. B, Ebstein's malformation (atrial aspect) in 14-year-old girl. Leaflets are sheetlike and have poor chordal development (arrows). C, Regurgitant double-orifice (*) tricuspid valve (atrial aspect) in 26-year-old woman with double-inlet left ventricle. Annulus is dilated, and leaflet thickening is result of regurgitant stream. D, Dysplastic tricuspid valve (atrial aspect) with stenosis and regurgitation in 2-year-old girl with pulmonary atresia and intact ventricular septum. Leaflets are thickened, commissures are fused, chordae tendineae are poorly formed, and annulus is hypoplastic.
in four cases, leaflet perforations in four, ruptured valves in three; and tricuspid and aortic valves chordae tendineae in three, leaflet destruction in in one. Associated conditions in these seven pa tients included congenital heart disease in two, two, and leaflet calcification in one. The infection was active in four patients, among presumptive rheumatic disease in two, and floppy whom the endocarditis was diagnosed preopera- mitral valve, prosthetic aortic valve, and chronic tively in two and at operation in two; cultures, alcoholism in one each. which were obtained in three patients, revealed Carcinoid Syndrome.—Carcinoid heart dis Candida albicans in two and viridans strepto ease caused pure tricuspid stenosis in one patient, cocci in one. Of the seven cases of healed tricuspid combined tricuspid stenosis and insufficiency in endocarditis, the causative organism was known two patients, and pure tricuspid insufficiency in in three (Streptococcus pneumoniae, cr-hemolytic two. The valve leaflets and chordae tendineae were thickened and distorted by dense fibrous streptococcus, and Staphylococcus). Endocarditis involved the tricuspid valve alone plaques typical of carcinoid heart disease (Fig. 7). in four patients, of whom two had congenital In this group of patients, the pulmonary valve heart disease, one had a history of intravenous was stenotic in three and both stenotic and in drug abuse, and one had a history of meningitis. sufficient in two. Trauma.—In one patient, chest trauma during Among the other seven cases of infective endo carditis, tricuspid and mitral valves were in a motor vehicle accident caused rupture of the volved in three; tricuspid, mitral, and aortic anterior tricuspid papillary muscle (Fig. 8) and
Mayo Clin Proc, September 1988, Vol 63
Table 4.—Functional Distribution of 93 Surgically Excised Tricuspid Valves Associated With Congenital Heart Disease* Total Functional category No. % TS TS + TI TI Malformation 39 42 1 38 0 Ebstein's anomaly Complete transposition 0 12 13 0 12 Double-inlet left ventricle 0 8 0 6 7t Atrial septal defect 0 6 6 0 6 Floppy tricuspid valve 0 5 5 0 5 Isolated tricuspid insufficiency 0 4 4 0 4 Tetralogy of Fallot 0 4 4 0 4 Double-outlet left ventricle 0 2 0 1 2t Double-outlet right 2 ventricle 0 2 0 2 Pulmonary atresia, intact 2 ventricular septum 0 2 2 0 2 Ventricular septal defect 0 2 0 2 1 Atrioventricular canal 0 1 0 1 1 Congenital mitral stenosis 0 1 0 1 Congenital tricuspid 1 stenosis 1 1 0 0 1 Mitral atresia 0 1 0 1 1 0 1 0 1 Patent ductus arteriosus Pulmonary atresia, 1 ventricular septal defect 0 1 0 1 Ventricular septal defect, straddling tricuspid 1 valve 0 1 0 1 1 Corrected transposition 0 1 0 1 93 Total 1 100 3 87 *TI = tricuspid insufficiency; TS = tricuspid stenosis. fOne valve was functionally normal (see text).
produced valvular prolapse and severe tricuspid insufficiency. Iatrogenic.—During repair of a ventricular septal defect, placement of a suture through the
SURGICAL PATHOLOGY OF TRICUSPID VALVE
857
anterior tricuspid leaflet resulted in tricuspid in sufficiency and necessitated valve replacement. Other Valve Procedures.—In addition to tri cuspid valve replacement, 293 of the 363 patients (81%) also had undergone replacement, repair, or excision of other cardiac valves (Table 5). Of the 272 patients who underwent tricuspid and mitral valve surgical procedures, with or without aortic or pulmonary valve procedures, 194 (71%) had postinflammatory disease and 56 (21%) had chronic pulmonary venous hypertension. Viewed from another perspective, all 250 patients with postinflammatory disease or pulmonary venous hypertension also had a mitral valve operation. Among 23 patients with tricuspid and pulmo nary valve surgical procedures, with or without operative procedures on left-sided valves, 15 (65%) had congenital heart disease, 5 had carcinoid heart disease, and 3 had congenital heart disease with infective endocarditis. Furthermore, of the 70 patients with tricuspid valve replacement alone, 64 (91%) had underlying congenital heart disease. DISCUSSION Postinflammatory Disease.—In patients who have undergone a tricuspid valve surgical proce dure, tricuspid valve dysfunction is most com monly caused by postinflammatory disease, which is presumably a manifestation of chronic rheu matic disease in most instances. It accounted for 39 of 41 cases (95%) of valve replacement or repair reported by Kratz and associates, 9 for 194 of 363 valve replacements (53%) in the current study,
Fig. 5. Pure tricuspid insufficiency associated with pulmonary venous hypertension in 57-year-old man. Leaflets are focally thickened, and annulus is greatly dilated (atrial aspect).
858
Mayo Clin Proc, September 1988, Vol 63
SURGICAL PATHOLOGY OF TRICUSPID VALVE
and for 9 of 21 valve replacements (43%) reported inflammatory disease (41%); it resulted from dif by Waller.19 A striking female preponderance has fuse leaflet fibrosis and retraction and from been noted in this group of patients, accounting commissural and chordal fusion that fixed the for 84% of the postinflammatory cases in the valve in a partially opened position. Tricuspid current study. This finding is similar to the re stenosis, with or without regurgitation, was over ported sex incidence for rheumatic mitral valve whelmingly caused by postinflammatory disease disease. 15,20 (92%) and resulted from diffuse leaflet fibrosis In the current investigation, pure tricuspid re- and from commissural fusion in a closed position. gurgitation was most commonly caused by post- Pure tricuspid stenosis was rare and accounted
/IETRIC 1
2
3
4
7
6
5
(
8
T ^_._
mSw
^LF·»
3EL*
.
,:
Fig. 6. Infective endocarditis. A through C, Pure tricuspid insufficiency due to Candida albicans infection in 46-year-old man with history of chronic alcoholism. Mitral and aortic valves were also infected. Extensive vegetations, shown along atrial aspect, involved all leaflets (A, arrows) and produced perforation (B, *). Candida organisms were plentiful microscopically (C). (Methenamine silver; x360.)
Mayo Clin Proc, September 1988, Vol 63
SURGICAL PATHOLOGY OF TRICUSPID VALVE
859
Fig. 7. Carcinoid heart disease. A, Tricuspid insufficiency (atrial aspect) in 47-year-old woman. Leaflets and chordae tendineae are thickened by carcinoid plaques. B, Tricuspid stenosis and insufficiency (atrial aspect) in 46-year-old woman. Carcinoid plaques appreciably thickened and distorted the leaflets and chordae tendineae. C, Severe tricuspid stenosis (atrial aspect) in 45-year-old man. D, Photomicrograph of tricuspid valve shown in C. Carcinoid plaque is adherent to valvular endocardium but does not produce destruction of overlying leaflet tissue (area between arrowheads). (Elastic van Gieson; x36.)
for only 3% of the postinflammatory valves; this finding is in contrast to pure mitral stenosis, which accounted for 33% of postinflammatory valves in a previous surgical pathology study. 15 Calcification was a feature of only three postinflammatory valves in the current series.
Fig. 8. Posttraumatic tricuspid insufficiency (atrial aspect) associated with rupture of necrotic anterior and medial tri cuspid papillary muscles (arrows) in 17-year-old boy who was involved in motor vehicle accident.
During the 25 years studied, the frequency of postinflammatory disease decreased appreciably over time; this trend may be a reflection of the decreasing incidence of acute rheumatic fever reported in Western countries during the past several decades. 21 This result corresponds to ob servations for the aortic and mitral valves. 14,15 Congenital Disorder.— Tricuspid valve dys function in the setting of congenital heart disease may be due to either intrinsic abnormalities of the valve apparatus or secondary annular dila tation. Ebstein's anomaly accounted for 61% of 129 cases in a recent review by Pasque and col leagues 10 of tricuspid valve replacement in chil dren and was observed in 40% of 97 patients with congenital heart disease in the current study. At our institution, the most common reason for tricuspid valve replacement is now congenital heart disease (53%) rather than postinflammatory dis ease (24%). This finding probably reflects changes in patient referral practices and advances in sur gical techniques. Pulmonary Venous Hypertension.— Tricus pid regurgitation was associated with chronic pulmonary hypertension in 15% of 363 cases in the current study and 38% of 21 operatively ex-
860
SURGICAL PATHOLOGY OF TRICUSPID VALVE
Mayo Clin Proc, September 1988, Vol 63
Table 5.—Operative Valve Procedures (Replacement, Repair, or Excision)* in 363 Patients With Tricuspid Valve Replacement TV + TV + TV + TV + MV MV + AV TV alone TV + PV PV + MV PV + AV Total Classification 194 101 93 0 0 0 0 Postinflammatory disease Congenital disorder 12 2 64 14 1 0 93 Pulmonary venous hypertension 38 18 0 0 0 0 56 Infective endocarditis 2 2 4 1 1 1 11 Carcinoid syndrome 0 0 0 5 0 0 5 Trauma 0 0 1 0 0 0 1 Iatrogenic 0 0 1 0 0 0 1 Indeterminate 2 0 0 0 0 0 2 Total 155 115 70 20 2 1 363 *When pulmonary valves were involved, only excision was performed. AV = aortic valve; MV : mitral valve; PV = pulmonary valve; TV = tricuspid valve.
cised valves reported by Waller.19 In autopsy series, tricuspid regurgitation was associated with conditions that produce pulmonary hyper tension in 47% of 45 patients described by Waller19 and in 67% of 24 patients with mitral stenosis described by Banerjee and co-workers.22 Among regurgitant tricuspid valves in patients with rheumatic heart disease, Waller19 noted a mean annular circumference of 13.6 cm in those with "functional" regurgitation (anatomically normal tricuspid valve) in comparison with 11.4 cm in those with "organic" regurgitation (ana tomically abnormal valves). In Waller's study, systolic pulmonary artery pressures were 40 mm Hg or less in 79% of patients with abnormal tricuspid valves and 55 mm Hg or more in 76% of patients with normal tricuspid valves. The remaining patients in each group had systolic pulmonary artery pressures between 40 and 55 mm Hg. In a combined echocardiographic and angiographic study, 23 however, no significant hemodynamic differences were noted between the organic and functional groups. Similarly, in 25 patients with tricuspid regurgitation associated with acquired pure mitral regurgitation in a re port by Cohen and associates, 24 a comparison of preoperative clinical and hemodynamic findings revealed no features that would distinguish those with anatomically normal valves from those with organic valve disease. Increased maximal diameter of the tricuspid valve has been demonstrated angiographically in patients with functional tricuspid regurgitation, and patients with moderate or severe regurgita tion have had reduced annular shortening. 25 By echocardiography, annular area (corrected for
body surface area) has been shown to increase with the grade of functional tricuspid regurgitation. 26 Infective Endocarditis.—In pediatric patients, infective endocarditis occurs most commonly in the setting of congenital heart disease. 27 " 29 In adults, however, rheumatic heart disease, mitral valve prolapse, degenerative valve disease, pros thetic valves, and congenital heart disease are all important underlying conditions, 30-32 as the cur rent study confirms. Among patients with rheu matic heart disease, infective endocarditis seems to involve native valves less often and prosthetic valves more frequently than 20 years ago. 31 More over, infections of the tricuspid valve, like the left-sided valves, involve men more often than 12,15,30-32
women. ' ■ With involvement of right-sided valves, intra venous drug abuse emerges as an important pre disposing factor for infective endocarditis, along with alcoholism, generalized infections in pa tients with immunologic disorders, congenital heart disease, and dermal infections. 33 When only heroin addicts are considered, the tricuspid valve is involved alone in most cases. 34 Excision 35 or replacement 36 of the tricuspid valve may be nec essary when sepsis persists despite adequate anti biotic treatment, when septic pulmonary emboli recur, or when acute right-sided heart failure develops.36 Carcinoid Syndrome.—Although tricuspid valve replacement may prolong life and alleviate symptoms in selected patients with metastatic tumor and carcinoid heart disease,37 only 18 cases of tricuspid valve operation for carcinoid heart disease have been reported previously (Table g^ 38-54 y a i v u i a r d i s e a s e m a y progress even after
Mayo Clin Proc, September 1988, Vol 63
SURGICAL PATHOLOGY OF TRICUSPID VALVE
861
successful extirpation of the tumor, and valve surgical population (94% of cases in the current replacement may be considered in such cases. 55 series), other causes not present in our 363 Carcinoid plaques observed at autopsy on a Han cases have been the subject of several reports cock porcine heterograft 8 months after implan (Table 7). 62-67 tation 49 raise questions about the suitability of tissue prostheses in patients with carcinoid heart REFERENCES disease. 1. Silver MD: Obstruction to blood flow related to tricuspid, pulmonary, and mitral valves. In Cardiovascular Pathol Trauma.—Ruptured chordae tendineae,56"58 rup ogy. Vol 1. Edited by MD Silver. New York, Churchill tured papillary muscles, 59,60 and dislocated leaf Livingstone, 1983, pp 551-574 61 lets, all of which caused severe tricuspid regur- 2. Edwards JE: The spectrum and clinical significance of tricuspid regurgitation. Pract Cardiol 6:86-95, 1980 gitation, have been described after chest trauma 3. Sugiura M, Matsushita S, Ueda K: A clinicopathological and may lead to tricuspid valve replacement. study on valvular diseases in 3,000 consecutive autopsyes Other Tricuspid Valve Disease.—Although [sic] of the aged. Jpn Circ J 46:337-345,1982 postinflammatory disease, pulmonary hyperten 4. Rose AG: Etiology of acquired valvular heart disease in adults: a survey of 18,132 autopsies and 100 consecutive sion, and congenital heart disease account for valve-replacement operations. Arch Pathol Lab Med most cases of tricuspid valve dysfunction in the 110:385-388,1986
Table 6.—Tricuspid Valve Operations in Patients With Carcinoid Heart Disease Age (yr) Procedure* Reference and sex Tricuspid valve 35 F Wright & Mulder,38 1963 annuloplasty, pulmonary valvotomy TVR, pulmonary 55 F Aroesty et al, 39 1966 valvotomy 40 TVR, pulmonary 26 F Carpena et al, 1973 valvotomy TVR 60 M Lund et al,41 197442 46 F TVR Honey & Paneth, 1975 26 F TVR, PVR McGuire et al, 43 1978 70 F TVR Herreman et al, 44 1978 TVR, PVR 64 F Nielsen & Manners, 45 1979 46 TVR, pulmonary 30 F Okada et al, 1979 valvectomy TVR 60 F Sworn et al, 47481980 TVR, pulmonary 34 M Hendel et al, 1980 valvotomy 49 53 M TVR Schoen et al, 50 1981 TVR, PVR 62 M Gutierrez et al, 1982 TVR 56 F Kessler,51 1983 TVR 56 F Miller et al, 52 1983 53 TVR, pulmonary 56 F DiSesa et al, 1985 valvectomy TVR, pulmonary 32 F valvectomy TVR, PVR 78 F Blick et al, 54 1988 TVR, pulmonary 69 M Current study, 1988 valvectomy TVR, pulmonary 55 M valvectomy 45 M TVR, pulmonary valvectomy TVR, pulmonary 47 F valvectomy TVR, pulmonary 46 F valvectomy
5. Waller BF, Moriarty AT, Eble JN, Davey DM, Hawley DA, Pless JE: Etiology of pure tricuspid regurgitation based on anular circumference and leaflet area: analysis of 45 necropsy patients with clinical and morphologic evidence of pure tricuspid regurgitation. J Am Coll Car diol 7:1063-1074,1986 6. Lev M, Liberthson RR, Joseph RH, Seten CE, Kunske RD, Eckner FAO, Miller RA: The pathologic anatomy of Ebstein's disease. Arch Pathol 90:334-343,1970 7. Zuberbuhler JR, Allwork SP, Anderson RH: The spec trum of Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 77:202-211, 1979 8. Waller BF, Bloch T, Barker BG, Roe SJ, Brown JW, Mahomed Y: Evaluation of operatively excised cardiac valves: etiologic determination of valvular heart disease. Cardiol Clin 2:687-716, November 1984 9. Kratz JM, Crawford FA Jr, Stroud MR, Appleby DC Jr, Hanger KH: Trends and results in tricuspid valve sur gery. Chest 88:837-840,1985 10. Pasque M, Williams WG, Coles JG, Trusler GA, Freedom RM: Tricuspid valve replacement in children. Ann Thorac Surg 44:164-168,1987 11. Subramanian R, Olson LJ, Edwards WD: Surgical pa thology of pure aortic stenosis: a study of 374 cases. Mayo Clin Proc 59:683-690,1984 12. Olson LJ, Subramanian R, Edwards WD: Surgical pa thology of pure aortic insufficiency: a study of 225 cases. Mayo Clin Proc 59:835-841,1984 13. Subramanian R, Olson LJ, Edwards WD: Surgical pa thology of combined aortic stenosis and insufficiency: a study of 213 cases. Mayo Clin Proc 60:247-254, 1985
Table 7.—Additional Reported Causes of Tricuspid Valve Dysfunction in Surgically Treated Patients Functional status* Cause Reference 62 Aneurysm of sinus of Valsalva TS + TI Right atrial and ventricular thrombosis TS 63 Right ventricular infarction TI 64 Libman-Sacks endocarditis TI 65 Giant blood cyst of tricuspid valve TS 66 Hypereosinophilic syndrome TI 67
*PVR = pulmonary valve replacement; TVR = tricuspid valve *TI = tricuspid insufficiency; TS = tricuspid stenosis. replacement.
862
SURGICAL PATHOLOGY OF TRICUSPID VALVE
14. Passik CS, Ackermann DM, Pluth JR, Edwards WD: Temporal changes in the causes of aortic stenosis: a surgical pathologic study of 646 cases. Mayo Clin Proc 62:119-123,1987 15. Olson LJ, Subramanian R, Ackermann DM, Orszulak TA, Edwards WD: Surgical pathology of the mitral valve: a study of 712 cases spanning 21 years. Mayo Clin Proc 62:22-34,1987 16. Davies MJ: Pathology of Cardiac Valves. Boston, Butterworth Publishers, 1980, pp 124-129 17. Roberts WC: Examining operatively excised cardiac valves (editorial). Am J Cardiol 59:1434-1436,1987 18. Pomerance A: Chronic rheumatic and other inflamma tory valve disease. In The Pathology of the Heart. Edited by A Pomerance, MJ Davies. Oxford, England, Blackwell Scientific Publications, 1975, pp 307-326 19. Waller BF: Etiology of pure tricuspid regurgitation. Cardiovasc Clin 17 (No. 2):53-95,1987 20. Hanson TP, Edwards BS, Edwards JE: Pathology of sur gically excised mitral valves: one hundred consecutive cases. Arch Pathol Lab Med 109:823-828,1985 21. Annegers J F , Pillman NL, Weidman WH, Kurland LT: Rheumatic fever in Rochester, Minnesota, 1935-1978. Mayo Clin Proc 57:753-757,1982 22. Banerjee A, Khanna SK, Beohar PC, Narayanan PS, Malhotra V, Mehta VK, Khalilullah M, Gupta MP: Tri cuspid regurgitation associated with mitral stenosis: a clinico-pathological study. Indian Heart J 34:8-12, 1982 23. Daniels SJ, Mintz GS, Kotler MN: Rheumatic tricuspid valve disease: two-dimensional echocardiographic, hemodynamic, and angiographic correlations. Am J Cardiol 51:492-496,1983 24. Cohen SR, Sell JE, Mclntosh CL, Clark RE: Tricuspid regurgitation in patients with acquired, chronic, pure mitral regurgitation. I. Prevalence, diagnosis, and com parison of preoperative clinical and hemodynamic fea tures in patients with and without tricuspid regurgita tion. J Thorac Cardiovasc Surg 94:481-487,1987 25. Ubago JL, Figueroa A, Ochoteco A, Colman T, Duran RM, Duran CG: Analysis of the amount of tricuspid valve anular dilatation required to produce functional tricuspid regurgitation. Am J Cardiol 52:155-158,1983 26. Mikami T, Kudo T, Sakurai N, Sakamoto S, Tanabe Y, Yasuda H: Mechanisms for development of functional tricuspid regurgitation determined by pulsed Doppler and two-dimensional echocardiography. Am J Cardiol 53:160-163,1984 27. Johnson CM, Rhodes KH: Pediatric endocarditis. Mayo Clin Proc 57:86-94,1982 28. Van Hare GF, Ben-Shachar G, Liebman J, Boxerbaum B, Riemenschneider TA: Infective endocarditis in infants and children during the past 10 years: a decade of change. Am Heart J 107:1235-1240,1984 29. Sholler GF, Hawker RE, Celermajer JM: Infective endo carditis in childhood. Pediatr Cardiol 6:183-186,1986 30. Kaye D: Changing pattern of infective endocarditis. Am J Med 78 (Suppl 6B):157-162,1985 31. Griffin MR, Wilson WR, Edwards WD, O'Fallon WM, Kurland LT: Infective endocarditis: Olmsted County, Minnesota, 1950 through 1981. JAMA 254:1199-1202, 1985 32. McKinsey DS, Ratts TE, Bisno AL: Underlying cardiac lesions in adults with infective endocarditis: the chang ing spectrum. Am J Med 82:681-688,1987 33. Roberts WC, Buchbinder NA: Right-sided valvular infec tive endocarditis: a clinicopathologic study of twelve necropsy patients. Am J Med 53:7-19,1972 34. Banks T, Fletcher R, Ali N: Infective endocarditis in heroin addicts. Am J Med 55:444-451, 1973
Mayo Clin Proc, September 1988, Vol 63
35. Arbulu A, Thorns NW, Chiscano A, Wilson RF: Total tricuspid valvulectomy without replacement in the treat ment of Pseudomonas endocarditis. Surg Forum 22:162164,1971 36. Stern HJ, Sisto DA, Strom JA, Soeiro R, Jones SR, Frater RWM: Immediate tricuspid valve replacement for endo carditis: indications and results. J Thorac Cardiovasc Surg 91:163-167,1986 37. Strickman NE, Rossi PA, Massumkhani GA, Hall RJ: Carcinoid heart disease: a clinical, pathologic, and thera peutic update. Curr Probl Cardiol 6:1-42, February 1982 38. Wright PW, Mulder DG: Carcinoid heart disease: report of a case treated by open heart surgery. Am J Cardiol 12:864-868,1963 39. Aroesty JM, DeWeese JA, Hoffman MJ, Yu PN: Car cinoid heart disease: successful repair of the valvular lesions under cardiopulmonary bypass. Circulation 34:105110,1966 40. Carpena C, Kay JH, Mendez AM, Redington JV, Zubiate P, Zucker R: Carcinoid heart disease: surgery for tricus pid and pulmonary valve lesions. Am J Cardiol 32:229233,1973 41. Lund HG, Cleveland RJ, Greenberg LH, Lippmann M, Dollinger MR: Tricuspid valve replacement in carcinoid heart disease. West J Med 120:412-415,1974 42. Honey M, Paneth M: Carcinoid heart disease: successful tricuspid valve replacement. Thorax 30:464-469,1975 43. McGuire MR, Pugh DM, Dunn MI: Carcinoid heart dis ease: restrictive cardiomyopathy as a late complication. J Kansas Med Soc 79:661-662; 665,1978 44. Herreman F, Vernant P, Cachera JP, Monier P: Insuffisance tricuspidienne sovere et tumeur carcinoide primi tive de l'ovaire: succes du remplacement valvulaire ä long terme; a propos d'une observation. Arch Mal Coeur 71:7280,1978 45. Nielsen MS, Manners JM: Valve replacement in carcinoid syndrome: anaesthetic management for tricuspid and pulmonary valve surgery. Anaesthesia 34:474-499, 1979 46. Okada RD, Ewy GA, Copeland JG: Echocardiography and surgery in tricuspid and pulmonary valve stenosis due to carcinoid syndrome. Cardiovasc Med 4:871-874; 879; 881, 1979 47. Sworn MJ, Edlin GP, McGill DAF, Mousley JS, Monro JL: Tricuspid valve replacement in carcinoid syndrome due to ovarian primary. Br Med J [Clin Res] 280:85-86, 1980 48. Hendel N, Leckie B, Richards J: Carcinoid heart disease: eight-year survival following tricuspid valve replacement and pulmonary valvotomy. Ann Thorac Surg 30:391-395, 1980 49. Schoen FJ, Hausner RJ, Howell J F , Beazley HL, Titus JL: Porcine heterograft valve replacement in carcinoid heart disease. J Thorac Cardiovasc Surg 81:100-105,1981 50. Gutierrez FR, McKnight RC, Jaffe AS, Ludbrook PA, Biello D, Weldon CS: Double porcine valve replacement in carcinoid heart disease. Chest 81:101-103, 1982 51. Kessler MR: Carcinoid heart disease: a unique case of postvalvotomy diagnosis. Arch Intern Med 143:1615-1616, 1983 52. Miller BR, Vohr FH, Christian FV, Singh AK: Cardiac valvular replacement in carcinoid heart disease. Am J Med 75:896-898, 1983 53. DiSesa VJ, Mills RM Jr, Collins J J Jr: Surgical man agement of carcinoid heart disease. Chest 88:789-791, 1985 54. Blick DR, Zoghbi WA, Lawrie GM, Verani MS: Carcinoid heart disease presenting as right-to-left shunt and con gestive heart failure: successful surgical treatment. Am Heart J 115:201-203,1988
Mayo Clin Proc, September 1988, Vol 63
55. Trell E, Rausing A, Ripa J, Torp A, Waldenström J: Carcinoid heart disease: clinicopathologic findings and follow-up in 11 cases. Am J Med 54:433-444,1973 56. Katz NM, Pallas RS: Traumatic rupture of the tricuspid valve: repair by chordal replacements and annuloplasty. J Thorac Cardiovasc Surg 91:310-314,1986 57. Watanabe T, Katsume H, Matsukubo H, Furukawa K, Ijichi H: Ruptured chordae tendineae of the tricuspid valve due to nonpenetrating trauma: echocardiographic findings. Chest 80:751-753,1981 58. Stephenson LW, Mac Vaugh H III, Kastor JA: Tricuspid valvular incompetence and rupture of the ventricular septum caused by nonpenetrating trauma. J Thorac Cardiovasc Surg 77:768-772,1979 59. Berkery W, Hare C, Warner RA, Battaglia J, Potts JL: Nonpenetrating traumatic rupture of the tricuspid valve: formation of ventricular septal aneurysm and subsequent septal necrosis; recognition by two-dimensional Doppler echocardiography. Chest 91:778-780,1987 60. Fracasso A, Pothen P, Gallucci V: Tricuspid regurgitation caused by blunt chest trauma in association with pericardial agenesis: surgical correction after eight years. Thorax 37:75-76,1982 61. Gayet C, Pierre B, Delahaye J-P, Champsaur G, AndreFouet X, Rueff P: Traumatic tricuspid insufficiency: an underdiagnosed disease. Chest 92:429-432,1987
SURGICAL PATHOLOGY OF TRICUSPID VALVE
863
62. Gibbs KL, Reardon MJ, Strickman NE, de Castro CM, Gerard JA, Rycyna JL, Hall RJ, Cooley DA: Hemodynamic compromise (tricuspid stenosis and insufficiency) caused by an unruptured aneurysm of the sinus of Valsalva. J Am Coll Cardiol 7:1177-1181,1986 63. Mestres CA, de Lacy AM, Pomar JL: Massive right atrial and ventricular thrombosis after peritoneovenous shunt ing treated by thrombectomy and tricuspid valvectomy. Ann Thorac Surg 44:205-206,1987 64. Korr KS, Levinson H, Bough EW, Gheorghiade M, Stone J, McEnany MT, Shulman RS: Tricuspid valve replace ment for cardiogenic shock after acute right ventricular infarction. JAMA 244:1958-1960,1980 65. Laufer J, Frand M, Milo S: Valve replacement for severe tricuspid regurgitation caused by Libman-Sacks endo carditis. Br Heart J 48:294-297,1982 66. Gallucci V, Stritoni P, Fasoli G, Thiene G: Giant blood cyst of tricuspid valve: successful excision in an infant. Br Heart J 38:990-992,1976 67. Harley JB, Mclntosh CL, Kirklin JJW, Maron BJ, Gottdiener J, Roberts WC, Fauci AS: Atrioventricular valve replacement in the idiopathic hypereosinophilic syn drome. Am J Med 73:77-81,1982
TRANSESOPHAGEAL ECHOCARDIOGRAPHY Bound reprints of the article from July 1988 issue are available at a cost of $6.50. Please send check with order, made payable to MAYO CLINIC PRO CEEDINGS, Room 1035 Plummer Building, Mayo Clinic, Rochester, MN 55905.