(Circulation. 1997;96:4280-4285.)
© 1997 American Heart Association, Inc.
Articles |
From the Columbia University College of Physicians and Surgeons and the Department of Pediatrics, Division of Pediatric Cardiology, Babies and Children's Hospital, Presbyterian Hospital, New York, NY.
Correspondence to Howard D. Apfel, MD, Division of Pediatric Cardiology, Babies and Children's Hospital, Columbia University, 3959 Broadway, New York, NY 10032. E-mail hda3{at}columbia.edu
| Abstract |
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Methods and Results Thirty-three patients (0.5 to 19 years old) operated on for mitral regurgitation as a single hemodynamically significant lesion were studied. All but 3 had medically refractory symptoms. One patient died during surgery, and 32 were followed for 0.3 to 17.1 years (mean, 4.5 years). The mean preoperative left ventricular shortening fraction was 0.38±0.09. Successful mitral valvuloplasty or replacement was documented by long-term normalization of end-diastolic dimensions. Early postoperative shortening fraction was significantly reduced (0.28±0.1, P<.01), but it improved to 0.40±0.07 (P<.01) on late follow-up, at which time only 1 patient had ventricular dysfunction. Preoperative shortening fractions did not correlate well with early or late postoperative values (r=.18 and r=.31, respectively). Seven of 32 surviving patients had preoperative shortening fractions <0.33 (mean, 0.26±0.05) and 25 >0.33 (mean, 0.39±0.08). Analysis of these subgroups showed no significant differences between the groups in early or late postoperative function. Duration of mitral insufficiency appeared to be associated with the development of atrial arrhythmias.
Conclusions Late left ventricular function normalizes in children after surgical correction of mitral insufficiency. In contrast to adults, delay of surgery in children with significant mitral regurgitation until the onset of severe symptoms does not increase the risk for long-term ventricular dysfunction, although late atrial arrhythmias are more likely to be encountered.
Key Words: mitral valve regurgitation surgery pediatrics
| Introduction |
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In contrast, guidelines for treatment of children with chronic mitral regurgitation are less well defined.10 Medical and surgical management related to body size problems have influenced decisions to delay mitral valve surgery in infants and children as long as possible.1113 Severe symptoms are often the indication for operation,10 and less emphasis is placed on echocardiographic criteria for intervention. However, late ventricular function has not been reported, nor have potential risk factors for poor outcome been analyzed in children after mitral valve repair or replacement.
We reviewed preoperative and early and late postoperative clinical, echocardiographic, and hemodynamic data in children undergoing mitral valve repair or replacement for chronic symptomatic mitral regurgitation to assess outcome.
| Methods |
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Nineteen patients had mitral valve repair and 14 mitral valve
replacement. One patient died during surgery, and 32 were followed for
4 months to 17 years (mean, 4.5±4.8 years). The clinical,
hemodynamic, and echocardiographic data
from all patients are shown in Table 1
.
Data Analysis
Patient records were reviewed for preoperative clinical and
hemodynamic status. Thirty patients were severely
symptomatic. Four patients had refractory congestive heart
failure requiring inotropic support and/or intubation and were in an
intensive care unit before surgery, and 26 were ambulatory and were
referred for surgical repair because of refractory symptoms despite
maximal medical therapy. Three patients with severe mitral
regurgitation and left atrial and/or
ventricular enlargement by
echocardiography had mild symptoms controlled by
digoxin and/or diuretics.
Duration of mitral regurgitation was calculated in the atrioventricular canal patients, assuming the day of initial atrioventricular canal surgery as the onset of isolated mitral regurgitation. The presence of at least mild to moderate mitral regurgitation in the immediate postoperative period was confirmed by color or pulse Doppler echocardiography in 21 of 24 patients. For the remaining patients in the series, audible mitral regurgitation, usually with echocardiographic confirmation, was considered to be the onset of regurgitation for purposes of analysis.
Preoperative cardiac catheterization had been performed in 23 patients. Parameters analyzed included cardiac index, pulmonary artery pressures, and pulmonary capillary wedge pressures.
On the basis of the change in cardioplegia solution from crystalloid to blood prime, the surgical era was divided into two periods (1976 to 1989 and 1990 to 1996). Nine patients had surgery in the earlier era and 24 in the later. Total cardiopulmonary bypass time and cross-clamp times were obtained for each operation.
Follow-up data were obtained by contacting the family and the physician caring for the patient, as well as from hospital charts. End points for follow-up were cardiac-related death, diagnosis of prosthetic valve stenosis, or the most recent follow-up date.
Echocardiographic Function Analysis
Three echocardiographic studies were
analyzed for each patient. These included the latest
preoperative study, a median of 8.5 days before surgery (range, 1 day
to 5 months); the prehospital discharge study after surgery (median, 7
days before surgery; range, 5 to 21 days); and the most recent
available echocardiogram in the survivors, used as the late follow-up
study. Echocardiograms were performed on an ATL Mark 500 A, GE Pass II,
HP Sonos 500, 1000, or 1500, Accuson 128, or SP 10
echocardiography machine. Two-dimensional and color
Doppler images in the subxyphoid, apical, and parasternal views
were used to assess the degree of regurgitation and to
rule out other significant lesions. Left ventricular
function was studied by use of M-mode measurements of the internal
dimensions in diastole and systole, measured at the level
of the papillary muscles in the parasternal short-axis
view.14 Fractional shortening was computed from
the left ventricular dimensions. Z scores were
used to standardize dimension measurements to patient size by the
following formula: Z score=observed-expected mean
dimension/SD of mean. Expected mean values for body surface area were
obtained from previously published values.14 The
degree of mitral regurgitation was qualitatively
assessed as mild, moderate, or severe according to the width and area
of the regurgitant jet on color Doppler
interrogation.15
Risk Analysis
Patients were considered to have a good outcome if they were
asymptomatic, were taking no medication, and had good left
ventricular function on follow-up (group 1). Suboptimal
outcome was defined as death, persistent left ventricular
dysfunction, or presence of atrial arrhythmias (group 2). The
potential risk factors analyzed are listed in Table 2
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Statistical Analysis
Group statistics were expressed as mean±SD. Comparisons of
indices of systolic function between preoperative and early and
late postoperative time periods for all patients were done by ANOVA.
Post hoc comparisons were made by Tukey's procedure. Comparisons
between individual subgroups were performed with unpaired t
tests. Correlation coefficients were calculated for specific
variables by standard methods.
Univariate and multivariate logistic regression analyses were performed on clinical, surgical, and echocardiographic data to determine the potential risk factors for suboptimal outcome.
| Results |
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Late Left Ventricular Function
Shortening Fraction
The mean preoperative SF for the group of 32 surviving patients
was 0.38±0.09 (original group, n=33). The early postoperative SF was
significantly lower (0.28±0.1, P<.01), and the mean SF at
late follow-up was 0.40±0.07, which was significantly higher than the
early postoperative study (P<.01) (Fig 2
).
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Preoperative SF for the group of 32 surviving patients did not predict
early or late postoperative values (r=.18 and
r=.31). Seven of the 32 patients had preoperative SF <0.33
(mean, 0.26±0.05) versus 25 patients who were >0.33 (mean,
0.37±0.07; P<.001). Follow-up analysis of these
subgroups showed no significant differences on early (0.20±0.07 versus
0.27±0.10, P=.07) or late postoperative quantitative
assessment (0.36±0.1 versus 0.39±0.08, P=.17) (Fig 3
). Regardless of preoperative or early
postoperative ventricular performance, there was
complete recovery of function on late follow-up in all but 1 of the 32
patients.
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End-Systolic Dimensions
Preoperative end-systolic dimension Z scores
did not correlate strongly with early or late postoperative left
ventricular function (r=.31 and
r=.40). Sixteen of 32 survivors had preoperative
end-systolic dimension Z scores >2 (mean,
3.3±1.5), and 16 were <2 (mean, 0.2±1.0). Analysis of the
subgroups with preoperative Z scores >2 and <2 showed a
significant difference in early postoperative SF (0.24±0.09 versus
0.31±0.09, P=.03). Nevertheless, all but 1 patient showed
normalization of function on late follow-up, albeit with a statistical
difference in final values between the two groups (0.37±0.1 versus
0.43±0.06, P=.03, Fig 4
).
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Risk Analysis
The patients were separated into two groups for risk
analysis. Twenty-seven patients (82%) were
asymptomatic with good left ventricular
function and were taking no medication (group 1). Six patients had
suboptimal outcomes (group 2). One patient died during surgery, and
another had persistent left ventricular dysfunction and
atrial fibrillation. Four children had preserved
ventricular function but developed atrial
arrhythmias requiring long-term antiarrhythmic therapy. The two
groups were compared for possible risk factors predicting poor outcome.
On multivariate analysis, only duration of
mitral regurgitation (P=.01) was found to be
a risk factor for poor outcome (Table 2
).
| Discussion |
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The differences between children and adults in late left ventricular performance after mitral surgery may be partially explained by the relatively shorter overall duration of mitral insufficiency, limiting the time that ventricular volume overload must be endured. Recent onset of regurgitation as an independent favorable predictor of recovery of ejection fraction before surgical correction has been well documented in adults.5 Interestingly, pediatric patients with mitral regurgitation appear to become symptomatic earlier than adults; this shortens the time from diagnosis to surgery and results in the likelihood of normalization of ventricular function. Almost all of the patients in this study were severely symptomatic at the time of surgery, in contrast to adult series, in which detection of ventricular dysfunction by echocardiography in minimally symptomatic or asymptomatic patients is more often the indication for surgery.7,8 In a large natural history study of adult patients with chronic mitral regurgitation, 77% of the patients were noted to be in NYHA class III or IV by 4 to 5 years after the onset of regurgitation.16 Of the patients in this study, 31 of 32 (94%) developed severe symptoms even at rest within 3 years after the initial diagnosis of mitral regurgitation. It would seem that mitral regurgitation that develops more insidiously, as seen in older patients, allows for greater accommodation by a compliant left atrium, and symptoms of pulmonary congestion are delayed.17
The greater resilience in left ventricular function in children than in older patients has been noted previously in other contexts and may reflect a greater potential for recovery in younger myocardium.1822 This is perhaps best illustrated in infancy by the well-documented recovery of left ventricular function from marked dysfunction after successful repair of anomalous left coronary artery from the pulmonary artery.18,19 In addition, animal studies have demonstrated that when myocardial damage is assessed by mechanical parameters such as ejection fraction, younger myocardium shows significantly less vulnerability to ischemia-reperfusion injury, as may be seen during and before cardiac bypass procedures.2022
Preoperative Risk Analysis
In adults, long-standing mitral regurgitation with
progressive left atrial enlargement has been shown to increase the risk
for the development of atrial
arrhythmias.8,23,24 Persistent or
recurrent atrial fibrillation before surgical correction necessitates
chronic anticoagulation before valve repair, even without mechanical
valve replacement. The association between prolonged duration of mitral
regurgitation resulting in significant atrial
enlargement and the ultimate development or persistence of atrial
arrhythmia before surgery is documented by several
studies.23,24 These reports led Gaasch and
Aurigemma to conclude in a recent review8 that
early surgical correction may be appropriate before development of
symptoms, left ventricular functional changes, or
substantial left atrial enlargement so that atrial fibrillation and its
complications might be avoided. Late atrial arrhythmias were
the primary reason for suboptimal outcome classification in 4 of 6
children in the present study. On the basis of the emergence of the
duration of regurgitation as the single predictive
variable for suboptimal outcome, it is reasonable also to attribute
atrial arrhythmias in children to increasing atrial enlargement
over time before mitral valve surgery. This creates a potential
clinical paradox in that prolonged duration of mitral
regurgitation before surgery does not affect late
postoperative left ventricular function, but it may place
the patient at a higher risk for developing rhythm
disturbances. In view of the well-known risks in operating on
the mitral valve in children,1113 the
risk-benefit decisions for early versus delayed surgery in pediatric
patients may be difficult.
Study Limitations
Mitral regurgitation as an isolated
hemodynamic lesion is uncommon in children. This most
likely accounts for the scarcity of pediatric studies in this area, and
although this study provides the only data specifically addressing this
issue in children, the sample is relatively small.
Furthermore, to maximize patient numbers, the definition of hemodynamically "isolated" mitral regurgitation was broadened to include many patients with previously repaired atrioventricular canal defects; cases with nonmitral residual hemodynamic abnormalities were excluded. The importance of this limitation is diminished by the fact that ventricular function was preserved during the time when isolated mitral insufficiency was present, before mitral valvuloplasty or replacement. This indicates that no other residual hemodynamic abnormality was present. Also, the late recovery after mitral valve surgery negates the potential importance of residual myocardial dysfunction secondary to the original atrioventricular defect hemodynamics.
Complex load-independent measures of left ventricular function were not included in this retrospective format. The goal of this study was to analyze preoperative risk factors regularly assessed at routine patient evaluations that could be analyzed to predict late ventricular function and outcome after surgical intervention. It is possible that more sophisticated techniques for quantification of ventricular function, such as load-independent methods, might unmask subclinical degrees of myocardial dysfunction beyond what can be recognized by routine qualitative and quantitative echocardiographic methods.
Conclusions
Long-term postoperative left ventricular function
normalized after successful surgery for mitral insufficiency in
symptomatic children. These findings are in contrast to
adult data and suggest that delay of surgery for mitral
regurgitation until the onset of severe symptoms does
not significantly increase the risk for late postoperative
ventricular dysfunction in pediatric patients. There were
suboptimal outcomes in a small number of patients, manifested mainly by
the development of atrial arrhythmia after prolonged duration
of mitral regurgitation before surgery, and this
finding potentially could speak for earlier intervention.
Decision making regarding the timing of mitral valve surgery in children continues to be difficult. Delaying intervention, even in symptomatic patients, has the advantage of being able to achieve a successful valvuloplasty or use a larger prosthetic valve at a later time at lower risk, with the expectation of good recovery of ventricular function. However, the relationship of prolonged duration of mitral regurgitation to the risk of atrial arrhythmias must also be a consideration.
Received May 30, 1997; revision received August 12, 1997; accepted September 7, 1997.
| References |
|---|
|
|
|---|
2.
Hochreiter C, Niles N, Devereux RB, Kligfield P, Borer
JS. Mitral regurgitation: relationship of noninvasive
descriptors of right and left ventricular
performance to clinical and hemodynamic
findings and to prognosis in medically and surgically treated patients.
Circulation. 1986;73:900912.
3. Ross J Jr. Assessment of cardiac function and myocardial contractility. In: Schlant RC, Alexander RW, eds. The Heart: Arteries and Veins. 8th ed. New York, NY: McGraw-Hill; 1994;1:487502.
4. Zile MR, Gaasch WH, Carroll JD, Levine HJ. Chronic mitral regurgitation: predictive value of preoperative echocardiographic indexes of left ventricular function and wall stress. J Am Coll Cardiol. 1984;3:235242.[Abstract]
5. Enriquez-Sarano M, Tajik J, Schaff HV, Orszulak TA, McGoon MD, Bailey KR, Frye RL. Echocardiographic prediction of left ventricular function after correction of mitral regurgitation: results and clinical implications. J Am Coll Cardiol. 1994;24:15361543.[Abstract]
6.
Starling MR. Effects of valve surgery on left
ventricular contractile function in patients with long-term
mitral regurgitation. Circulation. 1995;92:811818.
7.
Ross J Jr. The timing of surgery for severe mitral
regurgitation. N Engl J Med. 1996;335:14561458.
8. Gaasch WH, Aurigemma GP. Is corrective surgery ever indicated in the symptomatic patient with mitral regurgitation? Cardiol Rev. 1994;2:138144.
9. Ross J Jr. Afterload mismatch in aortic and mitral valve disease: implications for surgical therapy. J Am Coll Cardiol. 1985;5:811826.[Abstract]
10. Sanyal SK. Long term sequelae of the first attack of acute rheumatic fever in childhood. In: Emmanouilides GC, Riemenschneider TA, Allen HD, Gutgesell HP, eds. Heart Disease in Infants, Children and Adolescents. Baltimore, Md: Williams & Wilkins; 1995:14161440.
11. Kadoba K, Jonas RA, Mayer JE, Castaneda AR. Mitral valve replacement in the first year of life. J Thorac Cardiovasc Surg. 1990;100:762768.[Abstract]
12. Borkon AM, Soule L, Reitz BA, Gott VL, Gardner TJ. Five-year follow-up after valve replacement with the St. Jude medical valve in infants and children. Circulation. 1986;74(suppl I):I-110I-115.
13. Schaffer MS, Clarke DR, Campbell DN, Madigan CK, Wiggins JW, Wolfe RR. The St. Jude Medical cardiac valve in infants and children: role of anticoagulant therapy. J Am Coll Cardiol. 1987;9:235239.[Abstract]
14.
Henry WL, Ware J, Gardin JM, Hepner SI, McKay J, Weiner
M. Echocardiographic measurements in normal subjects.
Circulation. 1978;57:278285.
15.
Helmcke F, Nanda NC, Hsiung MC, Soto B, Adey CK, Goyal
RG, Gatewood RP Jr. Color Doppler assessment of mitral
regurgitation with orthogonal planes.
Circulation. 1987;75:175183.
16.
Rein AJJT, Colan SD, Parness IA, Sanders SP. Regional
and global left ventricular function in infants with
anomalous origin of the left coronary artery from the
pulmonary trunk: preoperative and postoperative assessment.
Circulation. 1987;75:115123.
17. Munoz S, Gallardo J, Diaz-Gorrin J, Medina O. Influence of surgery on the natural history of rheumatic mitral and aortic valve disease. Am J Cardiol. 1975;35:234242.[Medline] [Order article via Infotrieve]
18. Rappaport E. Natural history of aortic and mitral valve disease. Am J Cardiol. 1975;35:221228.[Medline] [Order article via Infotrieve]
19. Levitsky S, Van der Horst RL, Hastreiter AR, Fisher EA. Anomalous left coronary artery in the infant: recovery of ventricular function following early direct aortic implantation. J Thorac Cardiovasc Surg. 1980;79:598602.[Abstract]
20. Quantz M, Tchervenkov C, Chiu RC-J. Unique responses of immature hearts to ischemia. J Thorac Cardiovasc Surg. 1992;103:927935.[Abstract]
21.
Baker EJ, Boerboom LE, Olinger GN, Baker JE. Tolerance
of the developing heart to ischemia: impact of hypoxemia from
birth. Am J Physiol. 1995;268:H1165H1173.
22. Murashita T, Borgers M, Hearse DJ. Developmental changes in tolerance to ischemia in the rabbit heart: disparity between interpretations of structural, enzymatic and functional indices of injury. J Mol Cell Cardiol. 1987;19:12371246.[Medline] [Order article via Infotrieve]
23. Flugelman MY, Hasin Y, Katznelson N, Kriwisky M, Shefer A, Gotsman MS. Restoration and maintenance of sinus rhythm after mitral valve surgery for mitral stenosis. Am J Cardiol. 1984;54:617619.[Medline] [Order article via Infotrieve]
24.
Reed D, Abbott RD, Smucker ML, Kaul S. Prediction of
outcome after mitral valve replacement in patients with
symptomatic chronic mitral regurgitation:
the importance of left atrial size. Circulation. 1991;84:2334.
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