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Surgical treatment of floppy mitral valve syndrome in children
Surgical treatment of floppy mitral valve syndrome in children Five children whose ages ranged from 9 to 14 years underwent operation for floppy mitral valve syndrome. The clinical course of this syndrome in young patients was characterized by exceptionally rapid deterioration in congestive heart failure after the onset of symptoms. The children responded poorly to medical treatment. Early surgical treatment should be recommended soon after the onset of congestive heart failure. Three out of 5 patients required an associated tricuspid valve replacement for a floppy tricuspid valve, whose structure showed marked myxomatous transformation. Because of the high incidence of myxomatous transformation of the tricuspid valve in children, significant tricuspid insufficiency in this syndrome should be managed by valve replacement. Shoji Eguchi, M.D., Chiharu Nakamura, M.D., and Ken-ichi Asano, M.D., Niigata, Japan, and Makoto Tanaka, M.D., Nagaoka, Japan
An recent years, attention has been drawn to mitral insufficiency caused by myxomatous transformation. However, reports on this condition in children are scarce. We have operated upon five patients whose ages ranged from nine to fourteen years. The purpose of this paper is to report the special problems encountered in the treatment of younger age groups. Patients and methods In this institution, 11 patients ranging in age from 9 to 55 years of age underwent valve replacement for mitral insufficiency caused by myxomatous transformation between September, 1965, and October, 1975. This represents 5.4 per cent of the total number of 205 patients undergoing mitral valve replacement during this period and 11.5 per cent of 96 patients with pure mitral insufficiency treated by valve replacement. Among the 11 patients, 5 were children, and their ages at the time of operation were 9, 10, 13, 13, and 14 years. The criteria for including patients in this series were characteristic gross appearance of the valve and confirmation by histologic examination with hematoxylin and eosin, Weigert, elastic-van Gieson, alcian blue, and toluidine blue stains.1 None of the patients From the Department of Thoracic and Cardiovascular Surgery, University of Niigata Hospital, Niigata, Japan, and the Cardiovascular Center, Tachikawa Hospital, Nagaoka, Japan. Received for publication June 30, 1975. Accepted for publication Jan. 14, 1976.
exhibited features usually associated with Marfan's syndrome. No patient had a family history of valvular disease or a past history of rheumatic fever. In one patient (Case 3), the onset of congestive heart failure apparently coincided with the development of a heart murmur (see Table II). In 2 patients (Cases 2 and 4), a heart murmur had been noted 8 and 11 years earlier, respectively. One child (Case 4) presented a heart murmur at the age of 3 years. All but one patient (Case 5) manifested refractory congestive failure. Four patients were in Class IV and one (Case 5) in Class III of the New York Heart Association classification. Two patients complained of marked dyspnea with orthopnea. The duration of symptoms of congestive heart failure before the operation was 2 to 9 months, with rapid deterioration despite medical treatment. A Grade 2 to 3/6 holosystolic apical murmur was present in 4 patients. One child (Case 5) had a late systolic murmur following a midsystolic click and was unique among the patients in this respect. A third heart sound was present in all cases, and a fourth heart sound was described in Case 4. The pulmonic second sound was accentuated. The liver was enlarged to two to three fingerbreadths below the costal margin except in Case 5. In Cases 2 and 3, accumulation of ascites and pitting edema of the lower limbs were noted. Chest roentgenograms demonstrated cardiomegaly (cardiothoracic ratio 58 to 78 per cent, average 65 per cent) and pulmonary congestion. In all cases, except in the late stage, the posteroanterior radiogram revealed a 899
9 00
The Journal of Thoracic and Cardiovascular Surgery
Eguchi et al.
Fig. 1. A, Chest roentgenogram from Case 4 at the time of onset of symptoms (June 30, 1973) demonstrates a relatively small heart with protrusion of the left atrial appendage in addition to a disproportionate amount of pulmonary congestion. B, Chest roentgenogram 11 days before the operation (March 1, 1974) shows progressive cardiac enlargement. distinctive cardiac contour, that is, a relatively small heart with protrusion of the left atrial appendage in addition to a disproportionate amount of pulmonary vascular congestion (Fig. 1). These findings were confirmed by angiocardiography. The electrocardiogram showed regular sinus rhythm, left atrial enlargement, and left ventricular hypertrophy with nonspecific ST-T wave changes in all cases. Cardiac catheterization studies indicated pulmonary hypertension with increased left atrial pressure and prominent v waves, increased left ventricular enddiastolic pressure, and reduction in cardiac index (Table I). Left ventriculograms showed severe mitral regurgitation in all patients. Operative procedures and results are summarized in Table II. The valve cusps were soft, thin, and stretched, with irregular thickening of cusp edges. The mitral annulus was noted to be large and dilated. The chordae were elongated and thin in some areas. There was no evidence of rupture of chordae tendineae. The papillary muscles were normal. The mitral valve was replaced with a Starr-Edwards ball valve in 4 patients and with a Bjork-Shiley valve in one patient. Buttressed mattress sutures were applied to fix the prosthesis in 4 patients because the mitral annulus was fragile. Tricuspid insufficiency was present in 3 cases. In Case 1, tricuspid annuloplasty afforded complete correction. However, at the time of reoperation 4
months after the initial operation, the tricuspid annulus was again widely dilated and the tricuspid leaflets thin and friable. The valve was replaced with a Kay-Shiley disc valve. At the same time, a small thrombus which had caused mitral regurgitation was removed from the mitral prosthetic ring. Two patients underwent concomitant tricuspid valve replacement. Resected tricuspid valves also showed diffuse myxomatous degeneration (Fig. 2). Two patients died postoperatively, one (Case 1) because of cerebral embolism 42 days after the second operation and the other (Case 2) because of a low cardiac output syndrome on the seventh postoperative day. In the latter, heart surgery was undertaken despite intractable heart failure, because the congestive heart failure responded poorly to intensive medical therapy. To date, 3 patients are alive and doing well. Comment The floppy mitral valve syndrome has been reported to be more common in older people and rare in children, although not a few patients with this syndrome had heart murmurs during childhood.3' 4 In McKay's3 series of 38 mitral valve replacements for this condition, all patients were older than 30 years of age except for 3 with Marfan's syndrome. However, murmurs were known to be present for as long as 65 years preceding the onset of symptoms. On the other hand, Read5 reported 5 cases of mitral valve replace-
Volume 71 Number 6 June, 1976
Floppy mitral valve syndrome
9 01
Table I. Hemodynamic data Pressures (mm. Hg) Case No.
RA
RV
PA
PCW
LA
LV
16/10(13)
74/14-18
74/40
16/ 4(12)
50/ 0-14
58/26 (38)
a 24 v 28 (22)
100/10-30
14/ 6 ( 9)
78/-4-14
80/39 (52)
a 28 v41 (33)
120/ 5-32
( 7)
55/-1-10
59/33 (44)
a 33 v39 (30)
8/-2 ( 4)
60/ 2- 7
60/30 (45)
AO
Cl (L.lmin.lsq.
108/90
M.)
1.6 2.1
a 24 v40 (21)
77/ 6-13
2.1
a 25 v40
108/15-30
2.5
Legend: Figures in parentheses indicate mean pressures. CI, Cardiac index. RA, Right atrial. RV, Right ventricular. PA, Pulmonary arterial. PCW, Pulmonary capillary wedge. LA, Left atrial. LV, Left ventricular. AO, Aortic.
Table II Case Age (yr.)
Age when murmur first noted (yr.)
Period between onset of CHF and surgery (mo.)
No.
Sex
1
M
9
7
6
2
F
13
5
3
3 4
M F
13 14
13 3
6 9
5
M
10
10
2
Surgical treatment
Result
MVR (No. 2 S-E ball valve); TAP; removal of thrombus at valve seat; TVR (K-S valve) MVR (No. 3 S-E ball valve) TVR (No. 3 S-E disc valve) MVR (No. 3 S-E ball valve) MVR (No. 3 S-E ball valve) TVR (No. 4 S-E ball valve) MVR (No. 25 B-S valve)
Death (CVA) Death (LOS) Good Good Good
Legend: CHF, Congestive heart failure. MVR, Mitral valve replacement. TAP, Tricuspid annuloplasty. TVR, Tricuspid valve replacement. K-S, Kay-Shiley. S-E, Starr-Edwards. B-S, Bjork-Shiley. CVA, Cerebrovascular accident. LOS, Low output syndrome.
merit for the floppy valve syndrome, and 2 of the patients were 15 and 17 years old. These 2 Negro patients had no typical stigmata of Marfan's syndrome and presented with heart murmurs at the ages of 8 and 3 years. Shahin6 reported the case of a 3-year-old boy who required mitral valve replacement for myxomatous degeneration of the mitral valve. Sherman7 reported on an 8-year-old Negro girl who underwent mitral valve replacement for this condition. In this institution, 5 among 11 patients with this syndrome who underwent valve replacement were younger than 15 years of age. There may be a racial difference in the progress of this syndrome in addition to the high incidence of associated tricuspid valve disease seen in our series. Rapid deterioration after the onset of symptoms in this syndrome has been described by McKay3 and Hill.4
According to the report by Davis,8 3 out of 5 patients underwent valve replacement within 3 to 10 months after the initial onset of congestive heart failure. In our series, the clinical course of this syndrome in the younger age group was characterized by exceptionally rapid deterioration in congestive heart failure after the onset of symptoms as comparing with the older age group. The interval between the initial onset of congestive heart failure and surgery was 2 to 9 months in the younger age group (Table II). They responded poorly to treatment with digitalis and diuretics. In young patients with this syndrome, surgery seems to be indicated soon after the onset of congestive heart failure. Weakening of the valve annulus because of myxomatous changes, in addition to changes in valve leaflets and chordae, seems to contribute to the rapid
9 0 2 Eguchi et al.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 2. Mitral (A) and tricuspid fSj leaflets from Case 4. Myxomatous tissue with degeneration of connective tissue structure is evident. (Original magnification x 100.) deterioration in this syndrome. Disproportionate mitral annular dilatation seen at surgery suggests this speculation. Similarly, the mitral insufficiency in Marfan's syndrome tends to progress rapidly, especially in children. 9-13 The rapid progression of this syndrome is also manifested by the following findings: protrusion of the left atrial appendage and only moderate enlargement of the left atrium on chest roentgenography and angiocardiography; a tall and sharp v wave and ventricularization of the left atrial pressure; and the preservation of sinus rhythm despite severe and obviously catastrophic mitral regurgitation. Mitral valvuloplasty has been virtually useless in this syndrome.4, 15, 16 Diffuse involvement of valve apparatus can be managed only by valve replacement. Early reports of valve replacement in this syndrome indicated a high incidence of prosthesis dehiscence. 1,5
The tissue at the valve ring is fragile and holds sutures poorly. A continuous suture should not be used for fixing a prosthesis.1 Many interrupted sutures may provide firm fixation without impairment of blood supply in the annulus. We now use felt pledgets on the host annulus to minimize tension on the sutures. Recent reports indicate no incidence of prosthesis dehiscence owing to recognition of this serious complication.2, 4 Tricuspid insufficiency in this syndrome has rarely been reported. 12,17,18 Kern2 reported 2 cases of isolated tricuspid replacement for myxomatous degeneration of the tricuspid valve. However, to our knowledge, no case of combined mitral and tricuspid replacement for this syndrome has been reported in the English literature. Gooch and his associates,19 reporting prolapse of both mitral and tricuspid leaflets in the systolic murmur-click syndrome, suggested that both atrioventricular valves may be involved. Moreover, Shankar
Volume 71 Number 6 June, 1976
Floppy mitral valve syndrome
and his associates 12 presented the case of a very young infant with Marfan's syndrome who had fibromyxomatous involvement of both mitral and tricuspid valves and their associated annuli. Associated tricuspid insufficiency in this syndrome was rather common in our series, and it seemed to be irreversible and progressive in the younger age group. Resected tricuspid valves demonstrated marked myxomatous degeneration. Accordingly, significant tricuspid insufficiency in this syndrome should be managed by valve replacement because of the probability of myxomatous degeneration of the valve as well as secondary tricuspid insufficiency owing to mitral valve disease. REFERENCES 1 Cooley, D. A., Gerami, S., Hallman, G. L., Wukash, D. C , and Hall, R. J.: Mitral Insufficiency Due to Myxomatous Transformation "Floppy Valve Syndrome," J. Cardiovasc. Surg. 13: 346, 1972. 2 Kern, W. H., and Tucker, B. L.: Myxoid Changes in Cardiac Valves: Pathologic, Clinical, and Ultrastructural Studies, Am. Heart J. 84: 294, 1972. 3 McKay, R., and Yacoub, M. H.: Clinical and Pathological Findings in Patients With "Floppy" Valves Treated Surgically, Circulation 47, 48: 63, 1973 (Suppl. III). 4 Hill, D. G., Davies, M. J., and Braimbridge, M. V.: The Natural History and Surgical Management of the Redundant Cusp Syndrome (Floppy Mitral Valve), J. THORAC. CARDIOVASC. SURG. 67: 519,
1974.
5 Read, R. C , Thai, A. P., and Wendt, V. P.: Symptomatic Valvular Myxomatous Transformation (The Floppy Valve Syndrome): A Possible Forme Fruste of the Marfan Syndrome, Circulation 32: 897, 1965. 6 Shahin, W., Eshkol, D., and Levy, M. J.: Valve Replacement for Mitral Insufficiency in an Infant With Marfan's Syndrome, J. Pediatr. Surg. 4: 350, 1969. 7 Sherman, E. B., Char, F., Dungan, W. T., and Campbell, G. S.: Myxomatous Transformation of the
8 9 10 11
12 13
903
Mitral Valve Producing Mitral Insufficiency: Floppy Valve Syndrome, Am. J. Dis. Child. 119: 171, 1970. Davis, R. H., Schuster, B., Knoebel, S. B., and Fish, C : Myxomatous Degeneration of the Mitral Valve, Am. J. Cardiol. 28: 449, 1971. Simpson, J. W., Nora, J. J., and McNamara, D. G.: Marfan's Syndrome and Mitral Valve Disease, Am. Heart J. 77: 96, 1969. Raghib, G., Jue, K. L., Anderson, R. C , and Edwards, J. E.: Marfan's Syndrome With Mitral Insufficiency, Am. J. Cardiol. 16: 127, 1965. Bowden, D. H., Favaro, B., and Donahoe, J.: Marfan's Syndrome Accelerated Course in Childhood Associated With Lesions of Mitral Valve and Pulmonary Artery, Am. Heart J. 69: 26, 1965. Shankar, K. R., Hultgren, M. K., Lauer, R. M., and Diehl, A.M.: Lethal Tricuspid and Mitral Regurgitation in Marfan's Syndrome, Am. J. Cardiol. 20: 122, 1967. Pifarre, R., Caralps, J. M., and Sinha, S. N.: Mitral Valve Replacement for Regurgitation in an Infant With Marfan's Syndrome, J. THORAC. CARDIOVASC. SURG. 67:
233, 1974. 14 Nadas, A. S., and Fyler, D. C : Pediatric Cardiology, Philadelphia, 1972, W. B. Saunders Company, p. 287. 15 Sirak, H. D., and Ressalat, M. M.: Surgical Correction of Mitral Insufficiency in Marfan's Syndrome: Late Follow-up Results in 2 Cases, J. THORAC. CARDIOVASC. SURG. 55: 493,
1968.
16 Gerbode, F., Kerth, W. J., Hill, J. D., Sanchez, P. A., and Puryear, G. H.: Surgical Treatment of Non-rheumatic Mitral Insufficiency, J. Cardiovasc. Surg. 10: 103, 1969. 17 Castleman, B., and McNeely, B. U.: Case Records of the Massachusetts General Hospital, N. Engl. J. Med. 277: 92, 1967. 18 Pomerance,. A.: Ballooning Deformity (Mucoid Degenerations) of Atrioventricular Valves, Br. Heart J. 31: 343, 1969. 19 Gooch, A. S., Maranhao V., Scampardonis, G., Cha, S. D., and Yang, S. S.: Prolapse of Both Mitral and Tricuspid Leaflets in Systolic Murmur-Click Syndrome, N. Engl. J. Med. 287: 1218, 1972.
An recent years, attention has been drawn to mitral insufficiency caused by myxomatous transformation. However, reports on this condition in children are scarce. We have operated upon five patients whose ages ranged from nine to fourteen years. The purpose of this paper is to report the special problems encountered in the treatment of younger age groups. Patients and methods In this institution, 11 patients ranging in age from 9 to 55 years of age underwent valve replacement for mitral insufficiency caused by myxomatous transformation between September, 1965, and October, 1975. This represents 5.4 per cent of the total number of 205 patients undergoing mitral valve replacement during this period and 11.5 per cent of 96 patients with pure mitral insufficiency treated by valve replacement. Among the 11 patients, 5 were children, and their ages at the time of operation were 9, 10, 13, 13, and 14 years. The criteria for including patients in this series were characteristic gross appearance of the valve and confirmation by histologic examination with hematoxylin and eosin, Weigert, elastic-van Gieson, alcian blue, and toluidine blue stains.1 None of the patients From the Department of Thoracic and Cardiovascular Surgery, University of Niigata Hospital, Niigata, Japan, and the Cardiovascular Center, Tachikawa Hospital, Nagaoka, Japan. Received for publication June 30, 1975. Accepted for publication Jan. 14, 1976.
exhibited features usually associated with Marfan's syndrome. No patient had a family history of valvular disease or a past history of rheumatic fever. In one patient (Case 3), the onset of congestive heart failure apparently coincided with the development of a heart murmur (see Table II). In 2 patients (Cases 2 and 4), a heart murmur had been noted 8 and 11 years earlier, respectively. One child (Case 4) presented a heart murmur at the age of 3 years. All but one patient (Case 5) manifested refractory congestive failure. Four patients were in Class IV and one (Case 5) in Class III of the New York Heart Association classification. Two patients complained of marked dyspnea with orthopnea. The duration of symptoms of congestive heart failure before the operation was 2 to 9 months, with rapid deterioration despite medical treatment. A Grade 2 to 3/6 holosystolic apical murmur was present in 4 patients. One child (Case 5) had a late systolic murmur following a midsystolic click and was unique among the patients in this respect. A third heart sound was present in all cases, and a fourth heart sound was described in Case 4. The pulmonic second sound was accentuated. The liver was enlarged to two to three fingerbreadths below the costal margin except in Case 5. In Cases 2 and 3, accumulation of ascites and pitting edema of the lower limbs were noted. Chest roentgenograms demonstrated cardiomegaly (cardiothoracic ratio 58 to 78 per cent, average 65 per cent) and pulmonary congestion. In all cases, except in the late stage, the posteroanterior radiogram revealed a 899
9 00
The Journal of Thoracic and Cardiovascular Surgery
Eguchi et al.
Fig. 1. A, Chest roentgenogram from Case 4 at the time of onset of symptoms (June 30, 1973) demonstrates a relatively small heart with protrusion of the left atrial appendage in addition to a disproportionate amount of pulmonary congestion. B, Chest roentgenogram 11 days before the operation (March 1, 1974) shows progressive cardiac enlargement. distinctive cardiac contour, that is, a relatively small heart with protrusion of the left atrial appendage in addition to a disproportionate amount of pulmonary vascular congestion (Fig. 1). These findings were confirmed by angiocardiography. The electrocardiogram showed regular sinus rhythm, left atrial enlargement, and left ventricular hypertrophy with nonspecific ST-T wave changes in all cases. Cardiac catheterization studies indicated pulmonary hypertension with increased left atrial pressure and prominent v waves, increased left ventricular enddiastolic pressure, and reduction in cardiac index (Table I). Left ventriculograms showed severe mitral regurgitation in all patients. Operative procedures and results are summarized in Table II. The valve cusps were soft, thin, and stretched, with irregular thickening of cusp edges. The mitral annulus was noted to be large and dilated. The chordae were elongated and thin in some areas. There was no evidence of rupture of chordae tendineae. The papillary muscles were normal. The mitral valve was replaced with a Starr-Edwards ball valve in 4 patients and with a Bjork-Shiley valve in one patient. Buttressed mattress sutures were applied to fix the prosthesis in 4 patients because the mitral annulus was fragile. Tricuspid insufficiency was present in 3 cases. In Case 1, tricuspid annuloplasty afforded complete correction. However, at the time of reoperation 4
months after the initial operation, the tricuspid annulus was again widely dilated and the tricuspid leaflets thin and friable. The valve was replaced with a Kay-Shiley disc valve. At the same time, a small thrombus which had caused mitral regurgitation was removed from the mitral prosthetic ring. Two patients underwent concomitant tricuspid valve replacement. Resected tricuspid valves also showed diffuse myxomatous degeneration (Fig. 2). Two patients died postoperatively, one (Case 1) because of cerebral embolism 42 days after the second operation and the other (Case 2) because of a low cardiac output syndrome on the seventh postoperative day. In the latter, heart surgery was undertaken despite intractable heart failure, because the congestive heart failure responded poorly to intensive medical therapy. To date, 3 patients are alive and doing well. Comment The floppy mitral valve syndrome has been reported to be more common in older people and rare in children, although not a few patients with this syndrome had heart murmurs during childhood.3' 4 In McKay's3 series of 38 mitral valve replacements for this condition, all patients were older than 30 years of age except for 3 with Marfan's syndrome. However, murmurs were known to be present for as long as 65 years preceding the onset of symptoms. On the other hand, Read5 reported 5 cases of mitral valve replace-
Volume 71 Number 6 June, 1976
Floppy mitral valve syndrome
9 01
Table I. Hemodynamic data Pressures (mm. Hg) Case No.
RA
RV
PA
PCW
LA
LV
16/10(13)
74/14-18
74/40
16/ 4(12)
50/ 0-14
58/26 (38)
a 24 v 28 (22)
100/10-30
14/ 6 ( 9)
78/-4-14
80/39 (52)
a 28 v41 (33)
120/ 5-32
( 7)
55/-1-10
59/33 (44)
a 33 v39 (30)
8/-2 ( 4)
60/ 2- 7
60/30 (45)
AO
Cl (L.lmin.lsq.
108/90
M.)
1.6 2.1
a 24 v40 (21)
77/ 6-13
2.1
a 25 v40
108/15-30
2.5
Legend: Figures in parentheses indicate mean pressures. CI, Cardiac index. RA, Right atrial. RV, Right ventricular. PA, Pulmonary arterial. PCW, Pulmonary capillary wedge. LA, Left atrial. LV, Left ventricular. AO, Aortic.
Table II Case Age (yr.)
Age when murmur first noted (yr.)
Period between onset of CHF and surgery (mo.)
No.
Sex
1
M
9
7
6
2
F
13
5
3
3 4
M F
13 14
13 3
6 9
5
M
10
10
2
Surgical treatment
Result
MVR (No. 2 S-E ball valve); TAP; removal of thrombus at valve seat; TVR (K-S valve) MVR (No. 3 S-E ball valve) TVR (No. 3 S-E disc valve) MVR (No. 3 S-E ball valve) MVR (No. 3 S-E ball valve) TVR (No. 4 S-E ball valve) MVR (No. 25 B-S valve)
Death (CVA) Death (LOS) Good Good Good
Legend: CHF, Congestive heart failure. MVR, Mitral valve replacement. TAP, Tricuspid annuloplasty. TVR, Tricuspid valve replacement. K-S, Kay-Shiley. S-E, Starr-Edwards. B-S, Bjork-Shiley. CVA, Cerebrovascular accident. LOS, Low output syndrome.
merit for the floppy valve syndrome, and 2 of the patients were 15 and 17 years old. These 2 Negro patients had no typical stigmata of Marfan's syndrome and presented with heart murmurs at the ages of 8 and 3 years. Shahin6 reported the case of a 3-year-old boy who required mitral valve replacement for myxomatous degeneration of the mitral valve. Sherman7 reported on an 8-year-old Negro girl who underwent mitral valve replacement for this condition. In this institution, 5 among 11 patients with this syndrome who underwent valve replacement were younger than 15 years of age. There may be a racial difference in the progress of this syndrome in addition to the high incidence of associated tricuspid valve disease seen in our series. Rapid deterioration after the onset of symptoms in this syndrome has been described by McKay3 and Hill.4
According to the report by Davis,8 3 out of 5 patients underwent valve replacement within 3 to 10 months after the initial onset of congestive heart failure. In our series, the clinical course of this syndrome in the younger age group was characterized by exceptionally rapid deterioration in congestive heart failure after the onset of symptoms as comparing with the older age group. The interval between the initial onset of congestive heart failure and surgery was 2 to 9 months in the younger age group (Table II). They responded poorly to treatment with digitalis and diuretics. In young patients with this syndrome, surgery seems to be indicated soon after the onset of congestive heart failure. Weakening of the valve annulus because of myxomatous changes, in addition to changes in valve leaflets and chordae, seems to contribute to the rapid
9 0 2 Eguchi et al.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 2. Mitral (A) and tricuspid fSj leaflets from Case 4. Myxomatous tissue with degeneration of connective tissue structure is evident. (Original magnification x 100.) deterioration in this syndrome. Disproportionate mitral annular dilatation seen at surgery suggests this speculation. Similarly, the mitral insufficiency in Marfan's syndrome tends to progress rapidly, especially in children. 9-13 The rapid progression of this syndrome is also manifested by the following findings: protrusion of the left atrial appendage and only moderate enlargement of the left atrium on chest roentgenography and angiocardiography; a tall and sharp v wave and ventricularization of the left atrial pressure; and the preservation of sinus rhythm despite severe and obviously catastrophic mitral regurgitation. Mitral valvuloplasty has been virtually useless in this syndrome.4, 15, 16 Diffuse involvement of valve apparatus can be managed only by valve replacement. Early reports of valve replacement in this syndrome indicated a high incidence of prosthesis dehiscence. 1,5
The tissue at the valve ring is fragile and holds sutures poorly. A continuous suture should not be used for fixing a prosthesis.1 Many interrupted sutures may provide firm fixation without impairment of blood supply in the annulus. We now use felt pledgets on the host annulus to minimize tension on the sutures. Recent reports indicate no incidence of prosthesis dehiscence owing to recognition of this serious complication.2, 4 Tricuspid insufficiency in this syndrome has rarely been reported. 12,17,18 Kern2 reported 2 cases of isolated tricuspid replacement for myxomatous degeneration of the tricuspid valve. However, to our knowledge, no case of combined mitral and tricuspid replacement for this syndrome has been reported in the English literature. Gooch and his associates,19 reporting prolapse of both mitral and tricuspid leaflets in the systolic murmur-click syndrome, suggested that both atrioventricular valves may be involved. Moreover, Shankar
Volume 71 Number 6 June, 1976
Floppy mitral valve syndrome
and his associates 12 presented the case of a very young infant with Marfan's syndrome who had fibromyxomatous involvement of both mitral and tricuspid valves and their associated annuli. Associated tricuspid insufficiency in this syndrome was rather common in our series, and it seemed to be irreversible and progressive in the younger age group. Resected tricuspid valves demonstrated marked myxomatous degeneration. Accordingly, significant tricuspid insufficiency in this syndrome should be managed by valve replacement because of the probability of myxomatous degeneration of the valve as well as secondary tricuspid insufficiency owing to mitral valve disease. REFERENCES 1 Cooley, D. A., Gerami, S., Hallman, G. L., Wukash, D. C , and Hall, R. J.: Mitral Insufficiency Due to Myxomatous Transformation "Floppy Valve Syndrome," J. Cardiovasc. Surg. 13: 346, 1972. 2 Kern, W. H., and Tucker, B. L.: Myxoid Changes in Cardiac Valves: Pathologic, Clinical, and Ultrastructural Studies, Am. Heart J. 84: 294, 1972. 3 McKay, R., and Yacoub, M. H.: Clinical and Pathological Findings in Patients With "Floppy" Valves Treated Surgically, Circulation 47, 48: 63, 1973 (Suppl. III). 4 Hill, D. G., Davies, M. J., and Braimbridge, M. V.: The Natural History and Surgical Management of the Redundant Cusp Syndrome (Floppy Mitral Valve), J. THORAC. CARDIOVASC. SURG. 67: 519,
1974.
5 Read, R. C , Thai, A. P., and Wendt, V. P.: Symptomatic Valvular Myxomatous Transformation (The Floppy Valve Syndrome): A Possible Forme Fruste of the Marfan Syndrome, Circulation 32: 897, 1965. 6 Shahin, W., Eshkol, D., and Levy, M. J.: Valve Replacement for Mitral Insufficiency in an Infant With Marfan's Syndrome, J. Pediatr. Surg. 4: 350, 1969. 7 Sherman, E. B., Char, F., Dungan, W. T., and Campbell, G. S.: Myxomatous Transformation of the
8 9 10 11
12 13
903
Mitral Valve Producing Mitral Insufficiency: Floppy Valve Syndrome, Am. J. Dis. Child. 119: 171, 1970. Davis, R. H., Schuster, B., Knoebel, S. B., and Fish, C : Myxomatous Degeneration of the Mitral Valve, Am. J. Cardiol. 28: 449, 1971. Simpson, J. W., Nora, J. J., and McNamara, D. G.: Marfan's Syndrome and Mitral Valve Disease, Am. Heart J. 77: 96, 1969. Raghib, G., Jue, K. L., Anderson, R. C , and Edwards, J. E.: Marfan's Syndrome With Mitral Insufficiency, Am. J. Cardiol. 16: 127, 1965. Bowden, D. H., Favaro, B., and Donahoe, J.: Marfan's Syndrome Accelerated Course in Childhood Associated With Lesions of Mitral Valve and Pulmonary Artery, Am. Heart J. 69: 26, 1965. Shankar, K. R., Hultgren, M. K., Lauer, R. M., and Diehl, A.M.: Lethal Tricuspid and Mitral Regurgitation in Marfan's Syndrome, Am. J. Cardiol. 20: 122, 1967. Pifarre, R., Caralps, J. M., and Sinha, S. N.: Mitral Valve Replacement for Regurgitation in an Infant With Marfan's Syndrome, J. THORAC. CARDIOVASC. SURG. 67:
233, 1974. 14 Nadas, A. S., and Fyler, D. C : Pediatric Cardiology, Philadelphia, 1972, W. B. Saunders Company, p. 287. 15 Sirak, H. D., and Ressalat, M. M.: Surgical Correction of Mitral Insufficiency in Marfan's Syndrome: Late Follow-up Results in 2 Cases, J. THORAC. CARDIOVASC. SURG. 55: 493,
1968.
16 Gerbode, F., Kerth, W. J., Hill, J. D., Sanchez, P. A., and Puryear, G. H.: Surgical Treatment of Non-rheumatic Mitral Insufficiency, J. Cardiovasc. Surg. 10: 103, 1969. 17 Castleman, B., and McNeely, B. U.: Case Records of the Massachusetts General Hospital, N. Engl. J. Med. 277: 92, 1967. 18 Pomerance,. A.: Ballooning Deformity (Mucoid Degenerations) of Atrioventricular Valves, Br. Heart J. 31: 343, 1969. 19 Gooch, A. S., Maranhao V., Scampardonis, G., Cha, S. D., and Yang, S. S.: Prolapse of Both Mitral and Tricuspid Leaflets in Systolic Murmur-Click Syndrome, N. Engl. J. Med. 287: 1218, 1972.