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Surgery for mitral valvular disease during childhood and adolescence
Surgery for mitral valvular disease during childhood and adolescence Harold A. Collins, M.D., Rollin A. Daniel, Jr., M.D., H. William Scott, Jr., M.D., and Francis A. Puyau, M.D., Nashville, Tenn.
kJurgery for mitral valvular disease is rarely proposed for patients in the first two decades of life. This is particularly true of those patients with rheumatic valvular disease. The cogent reasons for this attitude are: (1) the difficulty in distinguishing between symptoms due to chronic rheumatic activity and those due to valvular deformity; (2) fear of producing an exacerbation of acute rheumatic fever by surgical trauma; and (3) the widespread belief that virtually all the cardiac manifestations during childhood and adolescence are due to active myocarditis. Despite these facts, the advisability of an operation remains an important practical problem. It arises when symptoms of increasing magnitude and significance occur in a young patient with recognized valvular disease and the benefit of a definitive surgical procedure must be assessed against the implied or real threat of activation of the rheumatic process. Consideration of surgery is even more cogent in those patients with congenital mitral valvular disease. The present report attempts to clarify certain of the issues regarding the indications for and results of surgery in mitral valvular disease during the first two decades From the Departments of Surgery and Pediatrics, Vanderbilt University School of Medicine, Nashville, Tenn. Supported in part by United States Public Health Service Grants HE-08195 and FR-95. Received for publication Nov. 10, 1965.
of life. During the past 8 years, 25 patients with rheumatic or congenital mitral valvular disease underwent operation at the Vanderbilt University Hospital. The experience with this group of patients provides the basis for this report. Mitral stenosis Sixteen patients had rheumatic mitral stenosis and 1 patient had congenital mitral stenosis. The patients' ages at the time of operation in the group with rheumatic heart disease varied from 13 to 20 years. There were 6 males and 10 females. Operation was performed at the age of 8 years in a girl with congenital mitral stenosis. The symptoms in this group of patients were similar to those of adults with mitral stenosis. Occasional hemoptysis and exertional and nocturnal dyspnea were the chief symptoms of patients with lesser degrees of stenosis. Weakness, easy fatigability, and tiredness were frequent complaints in individuals with more advanced grades of mitral stenosis. In those patients with the most severe stenosis, dyspnea at rest, gross cardiomegaly, and right ventricular failure were common. Two patients, 1 with and 1 without atrial fibrillation, had experienced cerebral embolization. It was noted frequently that these adolescent patients tended to minimize their symptoms whereas the parents sometimes tended to over-emphasize them. 639
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In the majority of patients it was impossible to ascertain the interval between the onset of acute rheumatic fever and the development of significant mitral stenosis. In several instances the parents were completely unaware of any illness preceding the onset of symptoms due to valvular disease. In no instance was operation advised when clinical or laboratory evidence suggested the presence of acute rheumatic activity. The physical findings were characteristic for patients with mitral stenosis. In all patients, a moderately loud diastolic murmur was present. The second sound at the pulmonary area was loud and, frequently, an opening snap was audible at the lower left sternal border. Three patients had systolic murmurs of varying intensity compatible with mitral insufficiency. One patient had systolic and diastolic murmurs over the aortic valvular area, and 1 patient had auscultatory findings compatible with tricuspid insufficiency. The electrocardiograms in those patients with pure mitral stenosis usually showed right axis deviation and right ventricular hypertrophy and, occasionally, abnormalities of the p waves. Left ventricular hypertrophy was present in the electrocardiograms of 1 patient with coexistent mitral insufficiency and 1 with aortic insufficiency. Two patients had atrial fibrillation. The roentgenograms characteristically demonstrated left atrial enlargement, prominence of the main pulmonary arterial segment, and right ventricular hypertrophy. In no patient was valvular calcification identified. Cardiac catheterization was accomplished in 11 of the 17 patients with mitral stenosis in an attempt to assess the severity of the stenosis or evaluate the presence of multivalvular disease. Eight patients had catheterization of both sides of the heart, and catheterization of the right side only was performed in 3 patients. The resting pulmonary artery systolic pressure exceeded 40 mm. Hg in every patient except 1 (range— 30 to 102 mm. Hg, with an average of 62 mm. Hg). The average mean diastolic gradient between the left atrium and left ven-
Thoracic and Cardiovascular Surgery
tricle in 8 patients was 16 mm. Hg, with a range of 9 to 27 mm. Hg. In 1 patient each, aortic insufficiency and tricuspid insufficiency were identified and quantitated at the time of cardiac catheterization. The indication for operation in this group of children and adolescents was the presence of symptoms attributable to mechanical obstruction, when clinical and laboratory evidence indicated that the rheumatic process was not active. Prior to 1960 the operative procedure usually consisted of finger fracture of the posteromedial and anterolateral zones of commissural fusion by the "closed" technique. Since 1960, virtually every patient in whom the closed technique was utilized had the valve opened by transventricular passage of the Gerbode dilator and splitting of the commissures to a minimum distance of 3.5 cm. The valvular fusion tended to be fibrotic in each patient. In 3 patients, correction of mitral stenosis was accomplished by the open technique with the aid of temporary cardiopulmonary bypass. This method was utilized in 1 child with congenital mitral stenosis. In another patient, the occurrence of previous systemic embolization, as well as the presence of tricuspid insufficiency, were the indications for use of this technique. In a third patient, the simultaneous correction of aortic insufficiency necessitated the use of cardiopulmonary bypass during mitral commissurotomy. No patient died as a result of operation for mitral stenosis. When operative pressures were obtained before and after opening of the mitral valve in 9 patients, the mean mitral diastolic gradient was reduced to an acceptable or negligible level in all. In 6 of 16 patients with rheumatic mitral stenosis, it was thought that slight mitral regurgitation had been produced or preexistent mitral insufficiency had been increased slightly. The surgically produced or enhanced mitral insufficiency did not affect the ultimate clinical result except in 1 patient. A portion of the left atrial appendage was submitted for microscopic study
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in each patient. In 10 of the 16 patients with rheumatic mitral stenosis, the pathologic specimen demonstrated either Aschoff bodies or other microscopic changes compatible with rheumatic fever. There were few major postoperative complications. One patient developed atrial fibrillation and spontaneous return of a normal sinus rhythm in the first postoperative week. The postpericardiotomy syndrome was observed in 2 patients. In 1 patient, the clinical course was suggestive of reactivated rheumatic fever in the postoperative period, and mitral insufficiency produced by commissurotomy seemed to increase in severity. The group has been observed postoperatively from 9 months to 8 years. Fourteen of the 16 patients with acquired stenosis are considered to be improved and their symptoms are nonexistent or minimal. The improvement in symptoms has correlated relatively well with changes in the auscultatory findings, as well as reversion of roentgenographic and electrocardiographic changes toward normal (Fig. 1). One child developed evidence of acute rheumatic fever
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after operation and her clinical status is considered worse than it was preoperatively. Another patient who was not catheterized was found at operation to have atrial thrombosis with predominant mitral insufficiency and minimal stenosis, and commissurotomy was not attempted. This patient was considered to be unimproved. The 8-year-old child with congenital mitral stenosis was subjected to operation in 1958 with the aid of temporary cardiopulmonary bypass. The mitral valve appeared to be the site of a membranous obstruction. Despite the valve being opened to the annulus anteriorly and posteriorly, the clinical course and auscultatory findings were unmodified. She was last examined Wi years postoperatively at which time she was unimproved. Mitral insufficiency Eight patients, ranging in age from 6 to 18 years, with an average of 13, underwent operation for severely incapacitating mitral insufficiency. In 6 of the patients, the mitral regurgitation resulted from rheumatic fever. Five of the 6 patients with acquired disease
Fig. 1. Chest roentgenograms obtained preoperatively (left) and 1 year after (right) mitral commissurotomy in a 15-year-old girl.
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were female. The interval between the initial attack of acute rheumatic fever and the occurrence of mitral insufficiency, severe enough to warrant operation, ranged from 3 to 12 years, with an average of 8 years. Two girls, 6 and 10 years of age, had congenital mitral insufficiency. In 1, the mitral valvular lesion was associated with corrected transposition of the great vessels* and in the other a ventricular septal defect was a coexistent lesion. The symptoms of the patients with mitral regurgitation were much more striking than those of the patients with mitral stenosis. All patients had been or were in congestive cardiac failure and were receiving digitalis. In many instances, repeated hospitalization had been necessary for control of cardiac failure. Most patients manifested extreme dyspnea with any type of activity. The functional cardiac capacity prior to surgery was was Class III in 3 patients and Class IV in 5 patients. Physical examination showed the heart to be enlarged and a diffuse ventricular heave was present. In 3 patients the anterior chest was deformed by cardiac enlargement. An apical systolic thrill and a loud apical pansystolic murmur were present in each. A diastolic murmur of variable intensity was audible at the apex in 5 of the 6 patients with rheumatic mitral incompetence. Conventional preoperative electrocardiograms were obtained in each patient. Atrial fibrillation was present in 3 of the 6 patients with rheumatic background. A pattern of left ventricular hypertrophy was noted in 4 patients and biventricular hypertrophy was noted in 4. Roentgenograms revealed striking cardiomegaly in all of the patients. Left ventricular enlargement, left atrial enlargement, prominence of the pulmonary arterial segment, and pulmonary parenchymal congestion were present in varying degrees in all patients. Six patients had catheterization of both •This patient has been previously reported. Puyau, F. A., Little, J. A., and Collins, H. A.: Mitral Valve Prosthesis in Childhood, Am. J. Dis. Child. 108:651, 1964.
Journal of Thoracic and Cardiovascular Surgery
sides of the heart and 1 patient had only catheterization of the right side. In each instance an attempt was made to quantitate the mitral insufficiency by injection of indocyanine green in the left ventricle and simultaneous withdrawal from the left atrium and a peripheral artery or by cineangiography. The resting pulmonary artery systolic pressure ranged from 60 to 100 mm. Hg (average 72 mm. Hg) in each of the patients studied. The average mitral regurgitant flow was three times forward flow with a range of one and two-tenths to five times forward flow. In 3 out of 4 patients with rheumatic mitral incompetence in whom left heart catheterization was accomplished, a significant mitral diastolic gradient was also observed. Coexistent lesions were identified in 4 patients at the time of cardiac catheterization. A 15-year-old girl with rheumatic mitral incompetence was also found to have mild aortic insufficiency. Massive tricuspid insufficiency was found in an 8-year-old girl with acquired mitral insufficiency. A 9-yearold girl had a large ventricular septal defect in association with congenital mitral insufficiency. Corrected transposition of the great vessels in association with congenital "mitral" insufficiency was encountered in a 6year-old girl. The indication for operation in this group of patients was congestive cardiac failure which was refractory to or poorly controlled by medical therapy. No patient had clinical or laboratory evidence of acute rheumatic activity at the time of operation. The mitral valve was repaired in 2 patients and replaced in 6 patients under direct vision with the aid of extracorporeal circulation. Plication of the mitral annulus was the main procedure in 2 patients with rheumatic mitral incompetence whose operations were in 1957 and 1958. The remaining 6 patients have had surgery since 1961 and the mitral valve was resected and replaced with a 3M Starr-Edwards mitral prosthesis in each. The tricuspid valve was replaced and a ventricular septal defect was closed simultaneously in each of 2 patients with mitral valve replacements. Anticoagulation was
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started 48 hours postoperatively and has been continued to date in the survivors. The mitral valvular abnormality in patients with rheumatic insufficiency was characterized by fibrosis and scarring of cusps and shortening of the chordae tendineae. Four of the 6 patients with rheumatic involvement also had commissural fusion with significant stenosis. In the patient with congenital mitral insufficiency and a ventricular septal defect, both the anterior and posterior mitral leaflets were short and bound down by very short chordae. The patient with corrected transposition had a tricuspid "mitral" valve with gross deficiency of all leaflets. Microscopic changes in the left atrial appendage were compatible with rheumatic fever in 5 of the 6 rheumatic patients. Two patients in whom mitral annuloplasty was utilized died soon after operation. One died almost immediately after operation with inadequately controlled mitral regurgitation. The other patient died 2 days postoperatively with pulmonary insufficiency. Four of the 6 patients with mitral valvular re-
Mitral valvular disease 6 4 3
placements survived operation. Death in an 8-year-old girl with simultaneous mitral and tricuspid replacement was attributed to low cardiac output postoperatively. The other death occurred as a result of air embolism in a child with simultaneous mitral valve replacement and closure of a ventricular septal defect. Major postoperative complications consisted of jaundice of 3 weeks' duration in 1 patient and a loculated extrapleural hematoma which persisted for 6 weeks in another patient. The patients surviving mitral valve replacement have been followed for periods varying from IVi to 3 years. Anticoagulant therapy has been controlled with relative ease and no secondary complications experienced. No patient has had recurrent rheumatic fever. Each survivor has noted striking, and sometimes dramatic, improvement which has been sustained. The reversion of roentgenographic and electrocardiographic changes toward normalcy has been noteworthy (Fig. 2). One of the survivors was killed in a motorcycle accident approximately \Vi years postoperatively. The re-
Fig. 2. Chest roentgenograms obtained preoperatively (left) and 6 months after (right) mitral valvular replacement in a 16-year-old girl.
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maining patients are leading relatively normal and active lives. Comment The first successful mitral valvulotomy was accomplished in a 12-year-old girl on May 20, 1923, and reported by Cutler and Levine.8 On May 16, 1925, Souttar17 performed the second successful mitral valvulotomy in a 19-year-old girl. These were the only 2 cases of survival after mitral valvular surgery reported until the past two decades.7 With the re-introduction and popularization of methods for mitral valvular surgery, the major emphasis has been on surgical treatment of adults with rheumatic disease. Wood23 has postulated that mitral valvulotomy in patients under the age of 20 years should be deferred as long as possible in view of the likelihood of activity and the presumed great risk of re-stenosis. Such diffidence has not been characteristic of the therapeutic approach to patients with congenital mitral valvular lesions. The early appearance and rapid progression of symptoms in patients with congenital mitral valvular disease has usually dictated surgical therapy within the first few years of life. The apparent reticence regarding surgical treatment for rheumatic mitral valvular disease has been predicated on several premises. It has been postulated that the symptoms of heart failure in patients under 20 years of age arise from active carditis rather than from a surgically correctable valvular defect. The possible negation of the benefits of operation by reactivation of rheumatic fever induced by surgery has also been considered. In addition, divergent views have arisen concerning prognosis in patients with rheumatic heart disease. Bland and Jones2 reported the prognosis in a series of 1,000 consecutive cases of patients with acute rheumatic fever followed for 20 years. The average age at onset was 8 years. Almost a third died within two decades of onset, primarily with complications of rheumatic fever. Wilson and Lubshez22 noted 226 deaths among 1,042 children with acute rheumatic fever. Two thirds
Thoracic and Cardiovascular Surgery
of the deaths occurred in the 1 to 15 year age group and most were ascribed to rheumatic carditis. Wilson and Lim21 described the occurrence of mitral stenosis in 269 children out of 757 with acute rheumatic fever. The diagnosis was established in 79 per cent within 1 to 2 years of an observed attack of active carditis, and, in the majority of cases, when the patient was under 15 years of age. It is apparent from these observations that a considerable mortality occurs from rheumatic heart disease within the first two decades of life and that mitral valvular disease can ensue relatively soon after the initial episode of rheumatic fever. The comparative importance of the mechanical valvular abnormality and the myocardial damage in producing mortality remains problematic. It is our impression that the gravity of the valvular defect has been significantly underestimated. The fear of reactivation of acute rheumatic fever by mitral valvular surgery in children and adolescents is exaggerated. In only 1 of the 22 patients with rheumatic heart disease in this study did reactivation of the rheumatic process appear to be related to surgery. Cherian and associates6 reported a series of 126 patients less than 20 years of age who underwent mitral valvotomy. Fifteen per cent of these patients had rheumatic reactivation in the follow-up period which was about the same incidence they noted in their older patients. Reale and coworkers10 reported the cases of 48 patients, ranging in age from 8 to 15 years, in whom mitral commissurotomy was performed. Only one instance of postoperative clinical activation of rheumatic fever was observed. Borman and his associates3 performed mitral commissurotomy in 2 children during an acute phase of rheumatic activity without significant postoperative difficulty. In the current study, microscopic examination of the excised atrial appendage revealed changes compatible with rheumatic activity in 15 of the 22 patients with acquired disease. This observation had no apparent correlation with clinical assessment or laboratory criteria of activity.
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The hemodynamic data in this group of patients reveal the rather profound changes which can occur relatively quickly in children and adolescents following acute rheumatic fever. Every patient in whom catheterization was performed had pulmonary hypertension. The degree of mitral stenosis and insufficiency determined from the catheterization data in these patients was comparable to that seen in adults with advanced disease. There was no evident relationship in this group between the degree of hemodynamic disturbance and the age of the patient. Admittedly the children and adolescents having cardiac catheterization in this series are a selected group and are not representative of all such individuals following acute rheumatic fever. Wider utilization of cardiac catheterization appears prudent, however, in the younger age group with residual cardiac symptoms after subsidence of acute rheumatic fever. This appears to be the only method of delineating with any accuracy the relative importance of the valvular defect in continuing disability. The indications for mitral commissurotomy in this age group have been delineated by various authors. We are in essential agreement with Lurie and Shumacker15 that the criteria for operation are the same as those applicable to adults. Basically it is the disease and its manifestations, rather than age, which dictate operation. The timing of mitral commissurotomy in adolescents and children with acquired mitral stenosis assumes some importance. Age, per se, is not a factor, since mitral commissurotomy can be accomplished with technical ease in even the very young. The most important factors appear to be that the rheumatic process be quiescent and that cardiac decompensation be under optimal medical control. Although Borman and associates3 believe that the presence of rheumatic activity does not contraindicate mitral valvotomy in selected cases, we believe that this viewpoint is justifiable only in extreme circumstances. The only patient in this group who was worse following surgery was 1 in whom the rheumatic process was clini-
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cally activated coincident with or shortly after operation. For the majority of children and adolescents, mitral commissurotomy can be accomplished satisfactorily by use of the closed technique of transventricular dilatation. The use of extracorporeal circulation is indicated only in those patients with coexistent lesions or with various complications secondary to mitral stenosis. Under the age of 20 years, advanced valvular pathological changes are unusual with acquired mitral stenosis and the need for valvular replacement should be minimal. Congenital mitral stenosis is a difficult lesion to treat surgically because of its various anatomic forms and frequent associated conditions. Operation under direct vision appears mandatory. Simple valvotomy has sufficed for some of these children18 but it appears likely that insertion of a prosthetic valve will be the only hope for insuring adequate valvular function for many of these patients. The operative morbidity should be low and operative mortality virtually nonexistent in this age group of patients following mitral commissurotomy for acquired stenosis. The greatest operative hazard in patients with mitral stenosis is embolization and this is a relatively infrequent occurrence under the age of 20, although 2 patients in this series had sustained systemic embolization preoperatively. Various authors have reported no operative mortality for mitral commissurotomy in patients under the age of 20 years.1* 3>i'5' 15'16 Cherian and associates6 reported an operative mortality following mitral commissurotomy of 13 per cent in 126 patients under the age of 20. The reason for this mortality rate is not clear. Based upon our own observations, as well as those of others, we are of the opinion that the value of mitral commissurotomy for acquired stenosis in children and adolescents has been underemphasized. Although the myocardial factor is of obvious importance in many individuals, a certain proportion have symptoms related to severe valvular stenosis. The indications for operation in this group are indisputable. Operation
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should not be deterred because of the implied effects of subclinical rheumatic activity. Rheumatic mitral insufficiency in the pediatric age group is common but may be compatible with survival for many years.12 Certain patients with mitral regurgitation, however, may progress rapidly to marked cardiomegaly, pulmonary hypertension, congestive heart failure, arrhythmias, and death. The patients in the current series exemplify the latter category. The large regurgitant flow with mitral insufficiency results in elevation of the mean left atrial pressure, the development of increasing pulmonary vascular resistance, and progressive anatomical changes in the pulmonary vascular bed. Such changes were apparent in our group of patients with both acquired and congenital mitral regurgitation. Congenital mitral insufficiency occurs either as an isolated anomaly or more commonly in association with other defects. Edwards and BurchelP have described and classified the pathologic anatomy of congenital mitral insufficiency. The association of ventricular septal defects with congenital mitral insufficiency has also been reported.10- " The indications for operation in children and adolescents with mitral insufficiency are the same as those for adults. Despite some natural reluctance to use foreign materials within the heart, especially in children, it is judicious to do so before the appearance of severe irreversible pulmonary, cardiac, and hepatic damage. Because of some of the known disadvantages and undefined long-term results of prosthetic valvular replacement, we have limited this procedure to patients with progressively severe symptoms despite intensive medical management. The choice of the most satisfactory operation for restoring competence to the mitral valve is problematic. In our experience, annuloplastic and valvuloplastic procedures have been of limited applicability and benefit, despite the obvious appeal of such techniques. Others, however, have achieved sat-
Journal of Thoracic and Cardiovascular Surgery
isfactory results with these methods.13'20' -* It is our impression that complete correction of the hemodynamic valvular defect transcends the importance of possible future complications arising from a foreign body in the heart. Clearly if the valvular deformity is conducive to complete direct repair, this is the preferred method. Such situations have been rare in our experience and that of others.14 We were initially concerned about size of prosthesis which could be utilized in children and adults. The massive cardiomegaly and mitral annular dilatation have permitted the use of a size 3M Starr-Edwards valve10 for each patient. Until more information is available concerning the ultimate fate of prosthetic valves, we have maintained patients with valve replacements on anticoagulants permanently. This has been accomplished with relative ease and no complications in this group of children and adolescents. The mortality and morbidity rates for operations to alleviate mitral insufficiency will probably always be higher than those associated with mitral commissurotomy. If patients have valve replacement before the terminal phases of mitral insufficiency, however, the mortality rate should continue to decrease. Two deaths in this series were related to inadequate relief of mitral insufficiency. With the availability of suitable valvular prostheses, such deaths should be avoidable at the present time. One death occurred as a result of inadequate cardiac output in a preterminal patient with simultaneous mitral and tricuspid replacements. It would be desirable to correct the lesions in such a patient before the clinical situation became so precarious. The other death which occurred following air embolization was preventable. The striking improvement in 4 long-term survivors following mitral valve replacement encourages us to recommend a more aggressive approach in children and adolescents with mitral insufficiency. In selected individuals under the age of 20 with progressive symptoms and an ineffective response to
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medical therapy, mitral replacement appears to be justified. Summary Congenital and acquired lesions of the mitral valve can produce symptoms of sufficient severity during the first two decades of life to warrant operation. During the past 8 years, 25 patients under the age of 21 years have required surgery for mitral valvular disease at the Vanderbilt University Medical Center. Sixteen patients had rheumatic mitral stenosis, and 1 patient had congenital mitral stenosis. The age range was from 8 to 20 years. Pulmonary hypertension was a constant finding in those patients who had cardiac catheterization. Transatrial or transventricular fracture of the mitral valve was possible for 14 patients, but open cardiotomy was necessary for 3 patients. Microscopic examination of the atrial appendage revealed an unexpectedly high incidence of active myocarditis. No patient died as a result of operation. All but 3 patients had sustained improvement postoperatively. Eight patients, ranging in age from 6 to 18 years, had surgical treatment for severely incapacitating mitral insufficiency. In 2 of the patients the lesion was congenital. Mitral annuloplasty was utilized in 2 early cases; both of these patients succumbed. Replacement of the mitral valve with a Starr prosthesis of adult size was accomplished in the other 6 patients. Four of these patients survived and are greatly improved. The two deaths occurred in patients with coexistent lesions which were simultaneously corrected. This study indicates the feasibility and generally good results following surgery for severe mitral valvular disease during childhood and adolescence. REFERENCES 1 Angelino, P. F., Levi, V., Brusca, A., and Actis-Dato, A.: Mitral Commissurotomy in the Younger Age Group, Am. Heart J. 51: 916, 1956. 2 Bland, E. F., and Jones, T. D.: Rheumatic Fever and Rheumatic Heart Disease. A Twenty Year Report on 1,000 Patients Followed Since
Childhood, Circulation 4: 836, 1951. 3 Borman, J. B., Stern, S., Shapira, T., Milwidsky, H., and Braun, K.: Mitral Valvotomy in Children, Am. Heart J. 61: 763, 1961. 4 Brest, A. N., Uricchio, J., and Likoff, W.: Valvular Surgery in the Young Patient With Rheumatic Heart Disease, J.A.M.A. 171: 249, 1959. 5 Castle, R. F., and Baylin, G. J.: Severe Acquired Mitral Stenosis in Childhood and Adolescence, J. Pediat. 58: 404, 1961. 6 Cherian, G., Vytilingam, K. I., Sukumar, I. P., and Gopinath, N.: Mitral Valvotomy in Young Patients, Brit. Heart J. 26: 157, 1964. 7 Cutler, E. C , and Beck, C. S.: The Present Status of the Surgical Procedures in Chronic Valvular Disease of the Heart. Final Report of All Surgical Cases, Arch. Surg. 18: 403, 1929. 8 Cutler, E. C , and Levine, S. A.: Cardiotomy and Valvulotomy for Mitral Stenosis. Experimental Observations and Clinical Notes Concerning an Operated Case With Recovery, Boston M. & S. J. 188: 1023, 1923. 9 Edwards, J. E., and Burchell, H. B.: Pathologic Anatomy of Mitral Insufficiency, Proc. Staff. Meet. Mayo Clin. 33: 497, 1958. 10 Gilbert, G., Aerichide, N., Lapointe, L., and David, P.: Congenital Mitral Insufficiency Associated With a Ventricular Septal Defect, Am. Heart J. 68: 107, 1964. 11 Hollman, A., and Hamed, M.: Mitral Valve Disease With Ventricular Septal Defect, Brit. Heart J. 27: 274, 1965. 12 Jhaveri, S., Czoniczer, G., Reider, R. B., and Massell, B. F.: Relatively Benign "Pure" Mitral Regurgitation of Rheumatic Origin, Circulation 22: 39, 1960. 13 Levy, M. J., Varco, R. L., Lillehei, C. W., and Edwards, J. E.: Mitral Insufficiency in Infants, Children, and Adolescents, J. THORACIC & CARDIOVAS. SURG. 45: 434,
1963.
14 Linde, L. M., Harper, J. A., Chuang, K. A., and Mulder, D. G.: Mitral Valve Replacement
in Children, J. THORACIC & CARDIOVAS. SURG.
15 16 17 18
49: 475, 1965. Lurie, P. R., and Shumacker, H. B., Jr.: Mitral Commissurotomy in Childhood, Peditrics 13: 454, 1954. Real, A., Colella, C , and Bruno, A. M.: Mitral Stenosis in Childhood: Clinical and Therapeutic Aspects, Am. Heart J. 66: 15, 1963. Souttar, H. S.: The Surgical Treatment of Mitral Stenosis, Brit. M. J. 2: 603, 1925. Starkey, G. W. B.: Surgical Experience in the Treatment of Congenital Mitral Stenosis and Mitral Insufficiency, J. THORACIC & CARDIOVAS.
SURG. 38: 336,
1959.
19 Starr, A., and Edwards, M. L.: Mitral Replacement: Clinical Experience With a Ball Valve
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Prosthesis, Ann. Surg. 154: 726, 1961. 20 Talner, N. S., Stern, A. M., and Sloan, H. E., Jr.: Congenital Mitral Insufficiency, Circulation 23: 339, 1961. 21 Wilson, M. G., and Lim, W. N.: The Natural History of Rheumatic Heart Disease in the Third, Fourth and Fifth Decades of Life. I. Prognosis With Special Reference to Survivorship, Circulation 16: 700, 1957.
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22 Wilson, M. G., and Lubshez, R.: Longevity in Rheumatic Fever, J.A.M.A. 138: 794, 1948. 23 Wood, P.: Diseases of the Heart and Circulation, ed. 2, London, 1956, Eyre & Spottiswoode, Ltd. 24 Yuan, S. H., Doyle, E. F., Pisacano, J. C , and Reed, G. E.: Severe Rheumatic Mitral Insufficiency in Childhood Amenable to Surgery, Pediatrics 33: 571, 1964.
kJurgery for mitral valvular disease is rarely proposed for patients in the first two decades of life. This is particularly true of those patients with rheumatic valvular disease. The cogent reasons for this attitude are: (1) the difficulty in distinguishing between symptoms due to chronic rheumatic activity and those due to valvular deformity; (2) fear of producing an exacerbation of acute rheumatic fever by surgical trauma; and (3) the widespread belief that virtually all the cardiac manifestations during childhood and adolescence are due to active myocarditis. Despite these facts, the advisability of an operation remains an important practical problem. It arises when symptoms of increasing magnitude and significance occur in a young patient with recognized valvular disease and the benefit of a definitive surgical procedure must be assessed against the implied or real threat of activation of the rheumatic process. Consideration of surgery is even more cogent in those patients with congenital mitral valvular disease. The present report attempts to clarify certain of the issues regarding the indications for and results of surgery in mitral valvular disease during the first two decades From the Departments of Surgery and Pediatrics, Vanderbilt University School of Medicine, Nashville, Tenn. Supported in part by United States Public Health Service Grants HE-08195 and FR-95. Received for publication Nov. 10, 1965.
of life. During the past 8 years, 25 patients with rheumatic or congenital mitral valvular disease underwent operation at the Vanderbilt University Hospital. The experience with this group of patients provides the basis for this report. Mitral stenosis Sixteen patients had rheumatic mitral stenosis and 1 patient had congenital mitral stenosis. The patients' ages at the time of operation in the group with rheumatic heart disease varied from 13 to 20 years. There were 6 males and 10 females. Operation was performed at the age of 8 years in a girl with congenital mitral stenosis. The symptoms in this group of patients were similar to those of adults with mitral stenosis. Occasional hemoptysis and exertional and nocturnal dyspnea were the chief symptoms of patients with lesser degrees of stenosis. Weakness, easy fatigability, and tiredness were frequent complaints in individuals with more advanced grades of mitral stenosis. In those patients with the most severe stenosis, dyspnea at rest, gross cardiomegaly, and right ventricular failure were common. Two patients, 1 with and 1 without atrial fibrillation, had experienced cerebral embolization. It was noted frequently that these adolescent patients tended to minimize their symptoms whereas the parents sometimes tended to over-emphasize them. 639
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In the majority of patients it was impossible to ascertain the interval between the onset of acute rheumatic fever and the development of significant mitral stenosis. In several instances the parents were completely unaware of any illness preceding the onset of symptoms due to valvular disease. In no instance was operation advised when clinical or laboratory evidence suggested the presence of acute rheumatic activity. The physical findings were characteristic for patients with mitral stenosis. In all patients, a moderately loud diastolic murmur was present. The second sound at the pulmonary area was loud and, frequently, an opening snap was audible at the lower left sternal border. Three patients had systolic murmurs of varying intensity compatible with mitral insufficiency. One patient had systolic and diastolic murmurs over the aortic valvular area, and 1 patient had auscultatory findings compatible with tricuspid insufficiency. The electrocardiograms in those patients with pure mitral stenosis usually showed right axis deviation and right ventricular hypertrophy and, occasionally, abnormalities of the p waves. Left ventricular hypertrophy was present in the electrocardiograms of 1 patient with coexistent mitral insufficiency and 1 with aortic insufficiency. Two patients had atrial fibrillation. The roentgenograms characteristically demonstrated left atrial enlargement, prominence of the main pulmonary arterial segment, and right ventricular hypertrophy. In no patient was valvular calcification identified. Cardiac catheterization was accomplished in 11 of the 17 patients with mitral stenosis in an attempt to assess the severity of the stenosis or evaluate the presence of multivalvular disease. Eight patients had catheterization of both sides of the heart, and catheterization of the right side only was performed in 3 patients. The resting pulmonary artery systolic pressure exceeded 40 mm. Hg in every patient except 1 (range— 30 to 102 mm. Hg, with an average of 62 mm. Hg). The average mean diastolic gradient between the left atrium and left ven-
Thoracic and Cardiovascular Surgery
tricle in 8 patients was 16 mm. Hg, with a range of 9 to 27 mm. Hg. In 1 patient each, aortic insufficiency and tricuspid insufficiency were identified and quantitated at the time of cardiac catheterization. The indication for operation in this group of children and adolescents was the presence of symptoms attributable to mechanical obstruction, when clinical and laboratory evidence indicated that the rheumatic process was not active. Prior to 1960 the operative procedure usually consisted of finger fracture of the posteromedial and anterolateral zones of commissural fusion by the "closed" technique. Since 1960, virtually every patient in whom the closed technique was utilized had the valve opened by transventricular passage of the Gerbode dilator and splitting of the commissures to a minimum distance of 3.5 cm. The valvular fusion tended to be fibrotic in each patient. In 3 patients, correction of mitral stenosis was accomplished by the open technique with the aid of temporary cardiopulmonary bypass. This method was utilized in 1 child with congenital mitral stenosis. In another patient, the occurrence of previous systemic embolization, as well as the presence of tricuspid insufficiency, were the indications for use of this technique. In a third patient, the simultaneous correction of aortic insufficiency necessitated the use of cardiopulmonary bypass during mitral commissurotomy. No patient died as a result of operation for mitral stenosis. When operative pressures were obtained before and after opening of the mitral valve in 9 patients, the mean mitral diastolic gradient was reduced to an acceptable or negligible level in all. In 6 of 16 patients with rheumatic mitral stenosis, it was thought that slight mitral regurgitation had been produced or preexistent mitral insufficiency had been increased slightly. The surgically produced or enhanced mitral insufficiency did not affect the ultimate clinical result except in 1 patient. A portion of the left atrial appendage was submitted for microscopic study
Volume 51 Number 5 May, 1966
in each patient. In 10 of the 16 patients with rheumatic mitral stenosis, the pathologic specimen demonstrated either Aschoff bodies or other microscopic changes compatible with rheumatic fever. There were few major postoperative complications. One patient developed atrial fibrillation and spontaneous return of a normal sinus rhythm in the first postoperative week. The postpericardiotomy syndrome was observed in 2 patients. In 1 patient, the clinical course was suggestive of reactivated rheumatic fever in the postoperative period, and mitral insufficiency produced by commissurotomy seemed to increase in severity. The group has been observed postoperatively from 9 months to 8 years. Fourteen of the 16 patients with acquired stenosis are considered to be improved and their symptoms are nonexistent or minimal. The improvement in symptoms has correlated relatively well with changes in the auscultatory findings, as well as reversion of roentgenographic and electrocardiographic changes toward normal (Fig. 1). One child developed evidence of acute rheumatic fever
Mitral valvular disease 6 4 1
after operation and her clinical status is considered worse than it was preoperatively. Another patient who was not catheterized was found at operation to have atrial thrombosis with predominant mitral insufficiency and minimal stenosis, and commissurotomy was not attempted. This patient was considered to be unimproved. The 8-year-old child with congenital mitral stenosis was subjected to operation in 1958 with the aid of temporary cardiopulmonary bypass. The mitral valve appeared to be the site of a membranous obstruction. Despite the valve being opened to the annulus anteriorly and posteriorly, the clinical course and auscultatory findings were unmodified. She was last examined Wi years postoperatively at which time she was unimproved. Mitral insufficiency Eight patients, ranging in age from 6 to 18 years, with an average of 13, underwent operation for severely incapacitating mitral insufficiency. In 6 of the patients, the mitral regurgitation resulted from rheumatic fever. Five of the 6 patients with acquired disease
Fig. 1. Chest roentgenograms obtained preoperatively (left) and 1 year after (right) mitral commissurotomy in a 15-year-old girl.
6 4 2 Collins et al.
were female. The interval between the initial attack of acute rheumatic fever and the occurrence of mitral insufficiency, severe enough to warrant operation, ranged from 3 to 12 years, with an average of 8 years. Two girls, 6 and 10 years of age, had congenital mitral insufficiency. In 1, the mitral valvular lesion was associated with corrected transposition of the great vessels* and in the other a ventricular septal defect was a coexistent lesion. The symptoms of the patients with mitral regurgitation were much more striking than those of the patients with mitral stenosis. All patients had been or were in congestive cardiac failure and were receiving digitalis. In many instances, repeated hospitalization had been necessary for control of cardiac failure. Most patients manifested extreme dyspnea with any type of activity. The functional cardiac capacity prior to surgery was was Class III in 3 patients and Class IV in 5 patients. Physical examination showed the heart to be enlarged and a diffuse ventricular heave was present. In 3 patients the anterior chest was deformed by cardiac enlargement. An apical systolic thrill and a loud apical pansystolic murmur were present in each. A diastolic murmur of variable intensity was audible at the apex in 5 of the 6 patients with rheumatic mitral incompetence. Conventional preoperative electrocardiograms were obtained in each patient. Atrial fibrillation was present in 3 of the 6 patients with rheumatic background. A pattern of left ventricular hypertrophy was noted in 4 patients and biventricular hypertrophy was noted in 4. Roentgenograms revealed striking cardiomegaly in all of the patients. Left ventricular enlargement, left atrial enlargement, prominence of the pulmonary arterial segment, and pulmonary parenchymal congestion were present in varying degrees in all patients. Six patients had catheterization of both •This patient has been previously reported. Puyau, F. A., Little, J. A., and Collins, H. A.: Mitral Valve Prosthesis in Childhood, Am. J. Dis. Child. 108:651, 1964.
Journal of Thoracic and Cardiovascular Surgery
sides of the heart and 1 patient had only catheterization of the right side. In each instance an attempt was made to quantitate the mitral insufficiency by injection of indocyanine green in the left ventricle and simultaneous withdrawal from the left atrium and a peripheral artery or by cineangiography. The resting pulmonary artery systolic pressure ranged from 60 to 100 mm. Hg (average 72 mm. Hg) in each of the patients studied. The average mitral regurgitant flow was three times forward flow with a range of one and two-tenths to five times forward flow. In 3 out of 4 patients with rheumatic mitral incompetence in whom left heart catheterization was accomplished, a significant mitral diastolic gradient was also observed. Coexistent lesions were identified in 4 patients at the time of cardiac catheterization. A 15-year-old girl with rheumatic mitral incompetence was also found to have mild aortic insufficiency. Massive tricuspid insufficiency was found in an 8-year-old girl with acquired mitral insufficiency. A 9-yearold girl had a large ventricular septal defect in association with congenital mitral insufficiency. Corrected transposition of the great vessels in association with congenital "mitral" insufficiency was encountered in a 6year-old girl. The indication for operation in this group of patients was congestive cardiac failure which was refractory to or poorly controlled by medical therapy. No patient had clinical or laboratory evidence of acute rheumatic activity at the time of operation. The mitral valve was repaired in 2 patients and replaced in 6 patients under direct vision with the aid of extracorporeal circulation. Plication of the mitral annulus was the main procedure in 2 patients with rheumatic mitral incompetence whose operations were in 1957 and 1958. The remaining 6 patients have had surgery since 1961 and the mitral valve was resected and replaced with a 3M Starr-Edwards mitral prosthesis in each. The tricuspid valve was replaced and a ventricular septal defect was closed simultaneously in each of 2 patients with mitral valve replacements. Anticoagulation was
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started 48 hours postoperatively and has been continued to date in the survivors. The mitral valvular abnormality in patients with rheumatic insufficiency was characterized by fibrosis and scarring of cusps and shortening of the chordae tendineae. Four of the 6 patients with rheumatic involvement also had commissural fusion with significant stenosis. In the patient with congenital mitral insufficiency and a ventricular septal defect, both the anterior and posterior mitral leaflets were short and bound down by very short chordae. The patient with corrected transposition had a tricuspid "mitral" valve with gross deficiency of all leaflets. Microscopic changes in the left atrial appendage were compatible with rheumatic fever in 5 of the 6 rheumatic patients. Two patients in whom mitral annuloplasty was utilized died soon after operation. One died almost immediately after operation with inadequately controlled mitral regurgitation. The other patient died 2 days postoperatively with pulmonary insufficiency. Four of the 6 patients with mitral valvular re-
Mitral valvular disease 6 4 3
placements survived operation. Death in an 8-year-old girl with simultaneous mitral and tricuspid replacement was attributed to low cardiac output postoperatively. The other death occurred as a result of air embolism in a child with simultaneous mitral valve replacement and closure of a ventricular septal defect. Major postoperative complications consisted of jaundice of 3 weeks' duration in 1 patient and a loculated extrapleural hematoma which persisted for 6 weeks in another patient. The patients surviving mitral valve replacement have been followed for periods varying from IVi to 3 years. Anticoagulant therapy has been controlled with relative ease and no secondary complications experienced. No patient has had recurrent rheumatic fever. Each survivor has noted striking, and sometimes dramatic, improvement which has been sustained. The reversion of roentgenographic and electrocardiographic changes toward normalcy has been noteworthy (Fig. 2). One of the survivors was killed in a motorcycle accident approximately \Vi years postoperatively. The re-
Fig. 2. Chest roentgenograms obtained preoperatively (left) and 6 months after (right) mitral valvular replacement in a 16-year-old girl.
644
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Collins et al.
maining patients are leading relatively normal and active lives. Comment The first successful mitral valvulotomy was accomplished in a 12-year-old girl on May 20, 1923, and reported by Cutler and Levine.8 On May 16, 1925, Souttar17 performed the second successful mitral valvulotomy in a 19-year-old girl. These were the only 2 cases of survival after mitral valvular surgery reported until the past two decades.7 With the re-introduction and popularization of methods for mitral valvular surgery, the major emphasis has been on surgical treatment of adults with rheumatic disease. Wood23 has postulated that mitral valvulotomy in patients under the age of 20 years should be deferred as long as possible in view of the likelihood of activity and the presumed great risk of re-stenosis. Such diffidence has not been characteristic of the therapeutic approach to patients with congenital mitral valvular lesions. The early appearance and rapid progression of symptoms in patients with congenital mitral valvular disease has usually dictated surgical therapy within the first few years of life. The apparent reticence regarding surgical treatment for rheumatic mitral valvular disease has been predicated on several premises. It has been postulated that the symptoms of heart failure in patients under 20 years of age arise from active carditis rather than from a surgically correctable valvular defect. The possible negation of the benefits of operation by reactivation of rheumatic fever induced by surgery has also been considered. In addition, divergent views have arisen concerning prognosis in patients with rheumatic heart disease. Bland and Jones2 reported the prognosis in a series of 1,000 consecutive cases of patients with acute rheumatic fever followed for 20 years. The average age at onset was 8 years. Almost a third died within two decades of onset, primarily with complications of rheumatic fever. Wilson and Lubshez22 noted 226 deaths among 1,042 children with acute rheumatic fever. Two thirds
Thoracic and Cardiovascular Surgery
of the deaths occurred in the 1 to 15 year age group and most were ascribed to rheumatic carditis. Wilson and Lim21 described the occurrence of mitral stenosis in 269 children out of 757 with acute rheumatic fever. The diagnosis was established in 79 per cent within 1 to 2 years of an observed attack of active carditis, and, in the majority of cases, when the patient was under 15 years of age. It is apparent from these observations that a considerable mortality occurs from rheumatic heart disease within the first two decades of life and that mitral valvular disease can ensue relatively soon after the initial episode of rheumatic fever. The comparative importance of the mechanical valvular abnormality and the myocardial damage in producing mortality remains problematic. It is our impression that the gravity of the valvular defect has been significantly underestimated. The fear of reactivation of acute rheumatic fever by mitral valvular surgery in children and adolescents is exaggerated. In only 1 of the 22 patients with rheumatic heart disease in this study did reactivation of the rheumatic process appear to be related to surgery. Cherian and associates6 reported a series of 126 patients less than 20 years of age who underwent mitral valvotomy. Fifteen per cent of these patients had rheumatic reactivation in the follow-up period which was about the same incidence they noted in their older patients. Reale and coworkers10 reported the cases of 48 patients, ranging in age from 8 to 15 years, in whom mitral commissurotomy was performed. Only one instance of postoperative clinical activation of rheumatic fever was observed. Borman and his associates3 performed mitral commissurotomy in 2 children during an acute phase of rheumatic activity without significant postoperative difficulty. In the current study, microscopic examination of the excised atrial appendage revealed changes compatible with rheumatic activity in 15 of the 22 patients with acquired disease. This observation had no apparent correlation with clinical assessment or laboratory criteria of activity.
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The hemodynamic data in this group of patients reveal the rather profound changes which can occur relatively quickly in children and adolescents following acute rheumatic fever. Every patient in whom catheterization was performed had pulmonary hypertension. The degree of mitral stenosis and insufficiency determined from the catheterization data in these patients was comparable to that seen in adults with advanced disease. There was no evident relationship in this group between the degree of hemodynamic disturbance and the age of the patient. Admittedly the children and adolescents having cardiac catheterization in this series are a selected group and are not representative of all such individuals following acute rheumatic fever. Wider utilization of cardiac catheterization appears prudent, however, in the younger age group with residual cardiac symptoms after subsidence of acute rheumatic fever. This appears to be the only method of delineating with any accuracy the relative importance of the valvular defect in continuing disability. The indications for mitral commissurotomy in this age group have been delineated by various authors. We are in essential agreement with Lurie and Shumacker15 that the criteria for operation are the same as those applicable to adults. Basically it is the disease and its manifestations, rather than age, which dictate operation. The timing of mitral commissurotomy in adolescents and children with acquired mitral stenosis assumes some importance. Age, per se, is not a factor, since mitral commissurotomy can be accomplished with technical ease in even the very young. The most important factors appear to be that the rheumatic process be quiescent and that cardiac decompensation be under optimal medical control. Although Borman and associates3 believe that the presence of rheumatic activity does not contraindicate mitral valvotomy in selected cases, we believe that this viewpoint is justifiable only in extreme circumstances. The only patient in this group who was worse following surgery was 1 in whom the rheumatic process was clini-
Mitral valvular disease 6 4 5
cally activated coincident with or shortly after operation. For the majority of children and adolescents, mitral commissurotomy can be accomplished satisfactorily by use of the closed technique of transventricular dilatation. The use of extracorporeal circulation is indicated only in those patients with coexistent lesions or with various complications secondary to mitral stenosis. Under the age of 20 years, advanced valvular pathological changes are unusual with acquired mitral stenosis and the need for valvular replacement should be minimal. Congenital mitral stenosis is a difficult lesion to treat surgically because of its various anatomic forms and frequent associated conditions. Operation under direct vision appears mandatory. Simple valvotomy has sufficed for some of these children18 but it appears likely that insertion of a prosthetic valve will be the only hope for insuring adequate valvular function for many of these patients. The operative morbidity should be low and operative mortality virtually nonexistent in this age group of patients following mitral commissurotomy for acquired stenosis. The greatest operative hazard in patients with mitral stenosis is embolization and this is a relatively infrequent occurrence under the age of 20, although 2 patients in this series had sustained systemic embolization preoperatively. Various authors have reported no operative mortality for mitral commissurotomy in patients under the age of 20 years.1* 3>i'5' 15'16 Cherian and associates6 reported an operative mortality following mitral commissurotomy of 13 per cent in 126 patients under the age of 20. The reason for this mortality rate is not clear. Based upon our own observations, as well as those of others, we are of the opinion that the value of mitral commissurotomy for acquired stenosis in children and adolescents has been underemphasized. Although the myocardial factor is of obvious importance in many individuals, a certain proportion have symptoms related to severe valvular stenosis. The indications for operation in this group are indisputable. Operation
6 4 6 Collins et al.
should not be deterred because of the implied effects of subclinical rheumatic activity. Rheumatic mitral insufficiency in the pediatric age group is common but may be compatible with survival for many years.12 Certain patients with mitral regurgitation, however, may progress rapidly to marked cardiomegaly, pulmonary hypertension, congestive heart failure, arrhythmias, and death. The patients in the current series exemplify the latter category. The large regurgitant flow with mitral insufficiency results in elevation of the mean left atrial pressure, the development of increasing pulmonary vascular resistance, and progressive anatomical changes in the pulmonary vascular bed. Such changes were apparent in our group of patients with both acquired and congenital mitral regurgitation. Congenital mitral insufficiency occurs either as an isolated anomaly or more commonly in association with other defects. Edwards and BurchelP have described and classified the pathologic anatomy of congenital mitral insufficiency. The association of ventricular septal defects with congenital mitral insufficiency has also been reported.10- " The indications for operation in children and adolescents with mitral insufficiency are the same as those for adults. Despite some natural reluctance to use foreign materials within the heart, especially in children, it is judicious to do so before the appearance of severe irreversible pulmonary, cardiac, and hepatic damage. Because of some of the known disadvantages and undefined long-term results of prosthetic valvular replacement, we have limited this procedure to patients with progressively severe symptoms despite intensive medical management. The choice of the most satisfactory operation for restoring competence to the mitral valve is problematic. In our experience, annuloplastic and valvuloplastic procedures have been of limited applicability and benefit, despite the obvious appeal of such techniques. Others, however, have achieved sat-
Journal of Thoracic and Cardiovascular Surgery
isfactory results with these methods.13'20' -* It is our impression that complete correction of the hemodynamic valvular defect transcends the importance of possible future complications arising from a foreign body in the heart. Clearly if the valvular deformity is conducive to complete direct repair, this is the preferred method. Such situations have been rare in our experience and that of others.14 We were initially concerned about size of prosthesis which could be utilized in children and adults. The massive cardiomegaly and mitral annular dilatation have permitted the use of a size 3M Starr-Edwards valve10 for each patient. Until more information is available concerning the ultimate fate of prosthetic valves, we have maintained patients with valve replacements on anticoagulants permanently. This has been accomplished with relative ease and no complications in this group of children and adolescents. The mortality and morbidity rates for operations to alleviate mitral insufficiency will probably always be higher than those associated with mitral commissurotomy. If patients have valve replacement before the terminal phases of mitral insufficiency, however, the mortality rate should continue to decrease. Two deaths in this series were related to inadequate relief of mitral insufficiency. With the availability of suitable valvular prostheses, such deaths should be avoidable at the present time. One death occurred as a result of inadequate cardiac output in a preterminal patient with simultaneous mitral and tricuspid replacements. It would be desirable to correct the lesions in such a patient before the clinical situation became so precarious. The other death which occurred following air embolization was preventable. The striking improvement in 4 long-term survivors following mitral valve replacement encourages us to recommend a more aggressive approach in children and adolescents with mitral insufficiency. In selected individuals under the age of 20 with progressive symptoms and an ineffective response to
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Mitral valvular disease 6 4 7
Number 5 May, 1964
medical therapy, mitral replacement appears to be justified. Summary Congenital and acquired lesions of the mitral valve can produce symptoms of sufficient severity during the first two decades of life to warrant operation. During the past 8 years, 25 patients under the age of 21 years have required surgery for mitral valvular disease at the Vanderbilt University Medical Center. Sixteen patients had rheumatic mitral stenosis, and 1 patient had congenital mitral stenosis. The age range was from 8 to 20 years. Pulmonary hypertension was a constant finding in those patients who had cardiac catheterization. Transatrial or transventricular fracture of the mitral valve was possible for 14 patients, but open cardiotomy was necessary for 3 patients. Microscopic examination of the atrial appendage revealed an unexpectedly high incidence of active myocarditis. No patient died as a result of operation. All but 3 patients had sustained improvement postoperatively. Eight patients, ranging in age from 6 to 18 years, had surgical treatment for severely incapacitating mitral insufficiency. In 2 of the patients the lesion was congenital. Mitral annuloplasty was utilized in 2 early cases; both of these patients succumbed. Replacement of the mitral valve with a Starr prosthesis of adult size was accomplished in the other 6 patients. Four of these patients survived and are greatly improved. The two deaths occurred in patients with coexistent lesions which were simultaneously corrected. This study indicates the feasibility and generally good results following surgery for severe mitral valvular disease during childhood and adolescence. REFERENCES 1 Angelino, P. F., Levi, V., Brusca, A., and Actis-Dato, A.: Mitral Commissurotomy in the Younger Age Group, Am. Heart J. 51: 916, 1956. 2 Bland, E. F., and Jones, T. D.: Rheumatic Fever and Rheumatic Heart Disease. A Twenty Year Report on 1,000 Patients Followed Since
Childhood, Circulation 4: 836, 1951. 3 Borman, J. B., Stern, S., Shapira, T., Milwidsky, H., and Braun, K.: Mitral Valvotomy in Children, Am. Heart J. 61: 763, 1961. 4 Brest, A. N., Uricchio, J., and Likoff, W.: Valvular Surgery in the Young Patient With Rheumatic Heart Disease, J.A.M.A. 171: 249, 1959. 5 Castle, R. F., and Baylin, G. J.: Severe Acquired Mitral Stenosis in Childhood and Adolescence, J. Pediat. 58: 404, 1961. 6 Cherian, G., Vytilingam, K. I., Sukumar, I. P., and Gopinath, N.: Mitral Valvotomy in Young Patients, Brit. Heart J. 26: 157, 1964. 7 Cutler, E. C , and Beck, C. S.: The Present Status of the Surgical Procedures in Chronic Valvular Disease of the Heart. Final Report of All Surgical Cases, Arch. Surg. 18: 403, 1929. 8 Cutler, E. C , and Levine, S. A.: Cardiotomy and Valvulotomy for Mitral Stenosis. Experimental Observations and Clinical Notes Concerning an Operated Case With Recovery, Boston M. & S. J. 188: 1023, 1923. 9 Edwards, J. E., and Burchell, H. B.: Pathologic Anatomy of Mitral Insufficiency, Proc. Staff. Meet. Mayo Clin. 33: 497, 1958. 10 Gilbert, G., Aerichide, N., Lapointe, L., and David, P.: Congenital Mitral Insufficiency Associated With a Ventricular Septal Defect, Am. Heart J. 68: 107, 1964. 11 Hollman, A., and Hamed, M.: Mitral Valve Disease With Ventricular Septal Defect, Brit. Heart J. 27: 274, 1965. 12 Jhaveri, S., Czoniczer, G., Reider, R. B., and Massell, B. F.: Relatively Benign "Pure" Mitral Regurgitation of Rheumatic Origin, Circulation 22: 39, 1960. 13 Levy, M. J., Varco, R. L., Lillehei, C. W., and Edwards, J. E.: Mitral Insufficiency in Infants, Children, and Adolescents, J. THORACIC & CARDIOVAS. SURG. 45: 434,
1963.
14 Linde, L. M., Harper, J. A., Chuang, K. A., and Mulder, D. G.: Mitral Valve Replacement
in Children, J. THORACIC & CARDIOVAS. SURG.
15 16 17 18
49: 475, 1965. Lurie, P. R., and Shumacker, H. B., Jr.: Mitral Commissurotomy in Childhood, Peditrics 13: 454, 1954. Real, A., Colella, C , and Bruno, A. M.: Mitral Stenosis in Childhood: Clinical and Therapeutic Aspects, Am. Heart J. 66: 15, 1963. Souttar, H. S.: The Surgical Treatment of Mitral Stenosis, Brit. M. J. 2: 603, 1925. Starkey, G. W. B.: Surgical Experience in the Treatment of Congenital Mitral Stenosis and Mitral Insufficiency, J. THORACIC & CARDIOVAS.
SURG. 38: 336,
1959.
19 Starr, A., and Edwards, M. L.: Mitral Replacement: Clinical Experience With a Ball Valve
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Prosthesis, Ann. Surg. 154: 726, 1961. 20 Talner, N. S., Stern, A. M., and Sloan, H. E., Jr.: Congenital Mitral Insufficiency, Circulation 23: 339, 1961. 21 Wilson, M. G., and Lim, W. N.: The Natural History of Rheumatic Heart Disease in the Third, Fourth and Fifth Decades of Life. I. Prognosis With Special Reference to Survivorship, Circulation 16: 700, 1957.
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22 Wilson, M. G., and Lubshez, R.: Longevity in Rheumatic Fever, J.A.M.A. 138: 794, 1948. 23 Wood, P.: Diseases of the Heart and Circulation, ed. 2, London, 1956, Eyre & Spottiswoode, Ltd. 24 Yuan, S. H., Doyle, E. F., Pisacano, J. C , and Reed, G. E.: Severe Rheumatic Mitral Insufficiency in Childhood Amenable to Surgery, Pediatrics 33: 571, 1964.