Fate of Mitral Regurgitation Following Repair of Atrioventricular Septal Defect Jonathan Rhodes, MD, Kenneth G. Warner, Barbara A. Romero, BS, Christopher H. Schmid,
MD, PhD,
David R. Fulton, MD, and Gerald R. Marx,
MD
The purpose of this study was to evaluate the fate of mitral regurgitation (MR) following repair of atrioventricular septal defects (AVSDs). Echocardiograms of all survivors of isolated AVSD surgery between 1986 and 1996, who had had >2 postoperative color Doppler studies (39 patients), were reviewed. On each study, MR severity was graded on a 11 to 41 scale, based upon the size of the MR jet. Median age at surgery was 9 months (range 3 to 169); median age at postoperative follow-up was 45 months (range 3 to 107). Mild deterioration of mitral valve function was fairly common. MR severity increased by >1 grade in 16 patients (41%) during the course of the study. However, the deterioration in mitral valve function oc-
curred primarily during the early postoperative time intervals. After the initial 32 postoperative months, MR worsened on only 4 occasions and in each instance worsened by only 1 grade. Deterioration to 41 MR occurred in only 3 patients, and was not observed after the initial 30 postoperative months. Survival curve analysis predicted a 90% probability of not having severe (41) MR after 30 months (lower 95% confidence bound: 80%). Postoperative MR remains fairly stable following AVSD repair. Serious deterioration is rare, especially after the initial 30 postoperative months. Q1997 by Excerpta Medica, Inc. (Am J Cardiol 1997;80:1194 –1197)
itral regurgitation (MR) is common among paM tients who have undergone repair of atriovenAlthough this tricular septal defects (AVSDs).
All postoperative color Doppler echocardiographic studies from each patient were retrospectively reviewed. Precordial long and apical 4-chamber views were used to assess MR severity. MR was graded on a 11 to 41 scale, based upon the appearance of the regurgitant jet in the 2 echocardiographic views. (11: none, or a thin jet visible a short distance above the leaflets; 21: a thin jet extending to the wall of the atrium; 31: a broad jet extending to the wall of the atrium; and 41: a broad jet occupying more than half of the left atrium.) An average of 4.3 6 1.9 studies (range 2 to 8) were analyzed for each patient. The data from these analyses were used to generate survival curves for freedom from 41 MR. In addition, the distribution of patients with various grades of MR was assessed at intervals of 0 to 3, 3 to 12, 12 to 24, 24 to 36, 36 to 48, 48 to 60, and 601 months after the operation. Patients who underwent reoperation because of residual MR were dropped from the analysis at the time of their reoperation. Statistical analysis: The paired sign test and Wilcoxon signed rank test were used to compare the distribution of MR grades in the various postoperative time intervals. Kaplan-Meier statistical methods were used to generate the survival curves. A p value of ,0.05 was considered significant. Twenty-five randomly selected studies were independently reviewed by 2 echocardiographers. MR was graded identically in 23 cases and differed by 1 grade in 2 cases. Twenty-five studies were also read twice on different occasions by the same reviewer. MR was graded identically on 24 occasions and differed by 1 grade on 1 occasion. The lower grade was chosen in cases where the grading differed.
1– 4
problem often appears well tolerated, MR may, in some cases, be progressive. Past studies have indicated that up to 18% of patients ultimately require reoperation for MR.1–10 Since its introduction in the 1980s, color Doppler echocardiography has become an important component of the noninvasive assessment of MR.11–15 We therefore reviewed our institution’s experience with the color Doppler echocardiographic assessment of MR following AVSD repair in order to obtain a better understanding of the fate of MR following AVSD repair.
METHODS Between 1986 and 1996, 47 patients were discharged from our institution following primary repair of an isolated AVSD. Of these, 39 had $2 postoperative color Doppler echocardiographic studies from which the function of the mitral valve could be assessed; these patients were the focus of this study (Table I). Median age at surgery was 9 months (range 3 to 169) and median age at follow-up was 45 months (range 3 to 107); 21 patients had complete AVSD’s, 8 had transitional AVSD’s, (i.e., restrictive ventricular communications), and 10 had partial AVSDs (isolated primum atrial septal defects with cleft anterior mitral valve leaflets). Trisomy 21 was present in 26 patients (67%). The mitral valve cleft was closed in 34 cases (87%). From the Division of Pediatric Cardiology, New England Medical Center, Boston, Massachusetts. Manuscript received February 5, 1997; revised manuscript received and accepted July 2, 1997. Address for reprints: Jonathan Rhodes, MD, Division of Pediatric Cardiology, 750 Washington St, #313, Boston, Massachusetts 02111.
1194
©1997 by Excerpta Medica, Inc. All rights reserved.
RESULTS
MR increased by $1 grade in 16 patients (41%) during the course of the study (Table I). However, the 0002-9149/97/$17.00 PII S002-9149(97)00655-3
TABLE I Clinical Features of Study Group Patient
DX
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 Mean
C C C C C C C C C C C C C C C C C C C C C T T T T T T T T P P P P P P P P P P
Age at Surgery
Initial MR
Final MR
Change
F/U
3.5 3.5 4.0 5.5 5.5 5.5 5.5 6.0 6.0 6.0 6.0 6.5 6.5 6.5 7.0 7.5 8.0 8.0 8.0 8.5 9.0 10.0 10.0 11.0 12.0 14.0 14.0 39.0 45.0 14.0 25.0 33.0 36.0 37.0 39.0 53.0 73.0 73.0 169.0 21.5 6 30.5
3 2 1 1 3 1 1 1 2 1 2 1 2 2 2 1 1 1 2 1 1 1 2 1 1 3 1 1 2 1 1 2 1 2 1 1 1 1 2 1.5 6 0.8
3 3 2 2 4 3 1 3 3 3 2 1 2 1 1 1 1 3 4 2 1 2 2 1 4 3 1 2 2 1 2 2 1 1 1 1 2 1 2 2.0 6 1.0
0 11 11 11 11 12 0 12 11 12 0 0 0 21 21 0 0 12 12 11 0 11 0 0 13 0 0 11 0 0 11 0 0 21 0 0 11 0 0 0.5 6 0.9
12 19 21 26 27* 28 77 63 88 94 83 12 51 57 45 73 60 61 2* 19 107 71 19 37 31* 17 28 45 84 21 28 24 61 20 3 7 46 55 90 44 6 29
patients with ,41 MR were doing well, without cardiopulmonary symptoms, at the time of last follow-up. All but 4 were receiving no cardiovascular medication.
DISCUSSION
Although MR is quite common following AVSD repair, criteria for surgical intervention have not been established. Rational decisions regarding the need for surgery in this setting require an understanding of the natural history of this condition. In this study, we characterized the fate of MR after AVSD repair by analyzing multiple, temporally separate color Doppler echocardiographic studies from all survivors of AVSD surgery. This analysis revealed that severe MR was rare following an initially successful AVSD repair. Furthermore, no patient developed severe MR after the initial 30 postoperative months; all patients with milder degrees of MR are currently doing well. These findings are generally supported by the reoperation statistics from previous surgical series.1–10 However, the criteria employed to determine the need for reoperation have not always been provided in past surgical series and, indeed, these criteria may be neither uniform nor consistent. Hence. reoperation statistics alone may convey an inaccurate picture of the natural history of MR *Underwent reoperation for residual MR. following AVSD repair. Age at surgery 5 age (in months) at time of AVSD; C 5 complete AVSD; Change 5 change in MR grade between initial and last follow-up; DX 5 diagnosis; Final MR 5 MR grade at time of last follow-up; Several previous investigators F/U 5 time interval (in months) between surgery and last follow-up; Initial MR 5 initial MR grade; P 5 have also stated that moderate departial AVSD; T 5 transitional AVSD. grees of MR are well tolerated and do not progress over time.2,3,5,9 However, these opinions were based upon deterioration was usually mild and occurred primarily general clinical impressions and/or studies that did not during the early postoperative time intervals (Figure employ consistent, serial analyses of mitral valve 1). After the initial 32 postoperative months, MR function. Although this study supports the opinons of worsened on only 4 occasions, and in each instance it previous investigators, it differs from past studies in worsened by only 1 grade. When compared with the that a consistent analytic technique (i.e., color Doppler distribution of patients in the 0- to 3-month time echocardiography) was used to serially assess MR interval, a statistically significant deterioration in mi- severity. Our data and conclusions regarding the fate tral valve function was detectable by the 12- to 24- of MR following AVSD repair may therefore be more month time interval. Thereafter, additional deteriora- reliable than those available from previous studies. tion did not achieve statistical significance. Clinical implications: Reoperation for MR following During the period of follow-up, 3 patients devel- AVSD repair often requires mitral valve replacement. oped severe (41) MR at 1, 1.5, and 30 months post- In recent series, 28 of 67 patients (42%) who underoperatively. All 3 ultimately underwent successful went surgery for residual MR received a prosthetic mitral valvuloplasty. Survival curve analysis predicted valve.1–9 Mitral valve replacement is a particularly a 90% probability of not having severe (41) MR after unattractive option in pediatric patients. Our observa30 months (lower 95% confidence bound 80%; Figure tion that MR following AVSD repair is generally 2). The progression of MR was not influenced by the stable and well tolerated for years after the initial type of AVSD or the presence of trisomy 21. All surgery is therefore an important consideration to facCONGENITAL HEART DISEASE/MITRAL REGURGITATION FOLLOWING AVSD REPAIR
1195
FIGURE 1. Progression of mitral regurgitation following AVSD repair. Roman numerals on the left side, the grade of MR. Each speckled column, divided into 4 parts (corresponding to the 4 grades of MR), represents the distribution of patients with the various grades of MR at the time interval (in months) indicated at the bottom of the column. White numbers within the columns, the number of patients, in that time interval, who had the indicated grade of MR. Black numbers within the small boxes, the number of patients from a particular category and time interval who moved to the indicated category in the next time interval. Small boxes that do not have a line exiting from the right side, the number of patients in the indicated category who underwent reoperation for residual mitral insufficiency during the subsequent time interval, or had no additional echocardiographic studies after the indicated time interval. The total number of patients within each time interval is also indicated, in parentheses, at the bottom of each column.
ings, etc., are important factors for the assessment of the patient with MR. However, although color Doppler echocardiography, in isolation, has limitations visa-vis the quantification MR, we believe it is one of the best modalities by which changes in MR (in an individual patient) may be assessed.14 Furthermore, we believe that the collection of these data from a group of patients is one of the best methods by which the natural history of MR may be ascertained. It must also be noted that this was a retrospective study in which the median follow-up period was only 45 months, and only 13 patients were followed for .60 months. Consequently, conclusions regarding the long-term function of the mitral valve following AVSD surgery must remain somewhat speculative.
FIGURE 2. Survival curve, with 95% confidence intervals, for freedom from 41 MR. Solid line, the survival curve. Dotted lines, the 95% confidence intervals. Tick marks on the survival curve, points where patients had their last echocardiographic studies. The numbers in parentheses indicate the number of patients in the analysis at the indicated time.
tor into decisions regarding the timing of surgical intervention for residual MR. Study limitations: The correspondence between the color Doppler echocardiographic appearance of a MR jet and the clinical significance of the MR is only approximate.16,17 Certainly other clinical and hemodynamic considerations, such as the degree of chamber enlargement, the level of symptoms, the presence of significant physical or cardiac catheterization find1196
THE AMERICAN JOURNAL OF CARDIOLOGYT
VOL. 80
1. Bonnetts PL, Goldberg SJ, Copeland JG. Frequency of left atrioventricular regurgitation postoperatively after repair of complete atrioventricular defect. Am J Cardiol 1994;74:1157–1160. 2. Han L, Kang SU, Park SC, Ettedgui JA, Neches WH. Long-term left atrioventricular valve function following surgical repair of atrioventricular septal defect. Cardiol Young 1995;5:230 –237. 3. Weintraub RG, Brawn WJ, Venables AW, Mee RBB. Two patch repair of complete atrioventricular septal defect in the first year of life. J Thorac Cardiovasc Surg 1990;99:320 –326. 4. Capouya ER, Laks H, Drinkwater DC, Pearl JM, Milgalter E. Management of the left atrioventricular valve in the repair of complete atrioventricular septal defects. J Thorac Cardiovasc Surg 1992;104:196 –203. 5. Ross DA, Nanton M, Gillis A, Murphy DA. Atrioventricular canal defects: results of repair in the current era. J Card Surg 1991;6:367–372. 6. Michielon G, Stellon G, Rizzoli G, Milanesi O, Rubino M, Moreolo GS, Casarotto D. Left atrioventricular valve incompetence after repair of common atrioventricular canal defects. Ann Thorac Surg 1995;60:S604 –S609. 7. McGrath LB, Gonzalez-Lavin L. Actuarial survival, freedom from reoperation, and other events after repair of atrioventricular septal defects. J Thorac Cardiovasc Surg 1987;94:582–590. 8. Pozzi M, Remig J, Fimmers R, Urban AE. Atrioventricular septal defects: analysis of short- and medium-term results. J Thorac Cardiovasc Surg 1991;101: 138 –142.
NOVEMBER 1, 1997
9. Lacour-Gayet F, Comas J, Bruniaux J, Serraf A, Losay J, Petit J, Dervanian P, Planche C. Management of the left atrioventricular valve in 95 patients with atrioventricular septal defects and a common atrioventricular orifice—a ten year review. Cardiol Young 1991;1:367–373. 10. Hanley FL, Fenton KN, Jonas RA, Mayer JE, Cook NR, Wernovsky G, Castenada AR. Surgical repair of complete atrioventricular canal defects in infancy. J Thorac Cardiovasc Surg 1993;106:387–397. 11. Miyatake K, Izumi S, Okamoto M, Kinoshita N, Asonuma H, Nakagawa H, Yamamoto K, Takamiya M, Sakakibara H, Nimura Y. Semiquantitative grading of severity of mitral regurgitation by real-time two-dimensional Doppler flow imaging technique. J Am Coll Cardiol 1986;7:82– 88. 12. Wu Y, Chang AC, Chin AJ. Semiquantitative assessment of mitral regurgitation by Doppler color flow imaging in patients aged ,20 years. Am J Cardiol 1993;71:727–732. 13. Spain MG, Smith MD, Grayburn PA, Harlamert EA, DeMaria AN.
Quantitative ssessment of mitral regurgitation by color Doppler flow imaging: angiographic and hemodynamic correlations. J Am Coll Cardiol 1989;13: 585–590. 14. Otsuji Y, Tei C, Kisanuki A, Natsugoe K, Kawazoe Y. Color Doppler echocardiographic assessment of the change in mitral regurgitant volume. Am Heart J 1987;114:349 –354. 15. Helmcke F, Nanda NC, Hsiung MC, Soto B, Adey CK, Goyal RG, Gatewood RP. Color Doppler assessment of mitral regurgitation with orthogonal planes. Circulation 1987;75:175–183. 16. Vandervoort PM, Homa DA, Thomas JD. Color flow Doppler assessment of valvular regurgitation: qualitative limitations and quantitative promise. Am J Card Imag 1995;9:195–198. 17. Shah PM. Quantitative assessment of mitral regurgitation. J Am Coll Cardiol 1989;13:591–593.
CONGENITAL HEART DISEASE/MITRAL REGURGITATION FOLLOWING AVSD REPAIR
1197