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Ann Thorac Surg 1996;62:419-424
© 1996 The Society of Thoracic Surgeons
Department of Cardiothoracic Surgery, Division of Surgery, and Department of Cardiology, Division of Pediatrics, aChildren's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin
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Abstract |
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 TopFootnotes Abstract IntroductionMaterial and MethodsResultsCommentAcknowledgmentsReferences |
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Methods. For the entire experience the operative mortality was 13.9% (16 patients). During the most recent era, January 1988 to December 1993, operative mortality was 3.6% (2 of 55 patients). This was significantly improved from the two previous eras, January 1974 to December 1980, 28% (7 of 25) and January 1981 to December 1987, 20% (7 of 35 patients) (p = 0.02). There were seven late deaths; 10-year actuarial survival, including operative mortality was 81%. Age at complete repair decreased; before 1982 all patients were more than 12 months of age, whereas after 1982 64% (56 of 88 patients) were 12 months of age or less.
Results. Moderate or severe preoperative left atrioventricular valve regurgitation was not a risk factor for operative mortality. For operative survivors with moderate to severe preoperative left atrioventricular valve regurgitation (n = 17), late postoperative left atrioventricular valve regurgitation (follow-up data available on 15 patients) was significantly reduced (severe = 1, moderate = 5, mild = 9; p = 0.007).
Conclusions. Early mortality was predicted by the era of surgical repair. Conversion to routine repair during infancy was achieved with a simultaneous decrease in operative mortality. For patients with moderate to severe preoperative left atrioventricular valve regurgitation, significant improvement in the degree of left atrioventricular valve regurgitation can be expected without an increase in operative or late mortality or morbidity.
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Introduction |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentAcknowledgmentsReferences |
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Material and Methods |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentAcknowledgmentsReferences |
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We analyzed the impact of preoperative, operative, and postrepair characteristics on operative and long-term mortality and morbidity. Morbid outcomes evaluated are: operative morbidity (duration of mechanical ventilation [more than 72 hours], magnitude of inotropic support [none, low, moderate, or high], duration of inotropic support [more than 72 hours]); and long-term morbidity (degree of LAVR [moderate or severe], LAV operation [either repair or replacement], placement of a permanent pacemaker, poor ventricular function [ejection fraction 55% or less]).
Inotropes used for postoperative support included isoproterenol, dopamine, dobutamine, epinephrine, and amrinone. The predominant level of inotropic support used during the first 48 hours after operation was used as the magnitude of inotropic support that we evaluated for subsequent analysis. This was graded on the following scale:
Complete patient selection was assured by reviewing operative lists and computerized patient data bases. Hospital records, cardiac catheterization, and echocardiographic reports were reviewed.
Operative Method
All procedures were performed using multidose cold crystalloid cardioplegia. A left ventricular vent either apical (early in the series) or through the right superior pulmonary vein (later) was used in all patients. One- and two-patch repairs were performed as previously described [5]. Injury to the bundle of His was prevented using the following technique. After the ventricular septal portion of the defect was closed and the common atrioventricular valve separated into right and left sides, closure of the atrial component of the defect was begun. Interrupted sutures were placed beneath the coronary sinus in the region of the bundle of His and passed through the atrial segment of the patch but not tied. The cross-clamp was released and when sinus rhythm resumed, the interrupted sutures were tied and the rhythm carefully observed. If heart block developed the sutures were removed and replaced. The routine use of a left ventricular vent and moderate hypothermia prevented ejection of air during this part of the procedure. The coronary sinus drainage remained to the right atrium.
Statistical Analysis
Contingency tables were used for single analysis of all variables (Sigmastat Software, Jandel Scientific Software, San Rafael, CA) to determine predictors of early and late morbidity and mortality. Factors deemed significant by initial univariate analysis were entered into multiple logistic regression analysis to identify the independent contribution of potential risk factors (SAS Institute, Inc, Cary, NC). The impact of age (as a continuous variable), operative technique (one- versus two-patch) and era of operation on operative mortality was assessed using multiple logistic regression analysis. Actuarial survival curves were constructed using the Kaplan-Meier method. The change in operative mortality between each of the three periods was made by 
2 for linear trends.
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Results |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentAcknowledgmentsReferences |
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). Down's syndrome was present in 82% (94 patients). Associated congenital heart abnormalities (other than secundum atrioventricular septal defect, patent ductus arteriosus, right-sided aortic arch) were present in 17% (19), and included 16 patients with CAVSD combined with tetralogy of Fallot (infundibular or valvar pulmonic stenosis, n = 15; pulmonary atresia, n = 1), left-sided obstruction in 2 patients (outflow tract narrowing, n = 1; coarctation of the aorta, n = 1), and double orifice mitral valve (n = 1). Palliative pulmonary artery banding was used as an initial step in 16% (18 patients). There was no patient who underwent pulmonary artery banding who did not survive to undergo complete repair. Data on preoperative LAVR were available for 111 patients. The LAVR was graded as moderate or severe in 19% (21 patients). The impact of PVR and Qp/Qs on outcome was not evaluated in patients with structural limitation of pulmonary blood flow, (patients with tetralogy of Fallot or patients who had undergone pulmonary artery banding). Among patients without structural limitation of pulmonary flow, PVR was 5.4 ± 0.4 Wood units (range, 1.0 to 14 Wood units); the PVR was considered to be elevated (6 or more Wood units) in 33% (23 of 68 patients). As an indicator of poorly compensated congestive heart failure we looked at patients with elevated Qp/Qs (4 or more). The average Qp/Qs was 3.5 ± 0.3 (range, 0.9 to 16.2) and the Qp/Qs was elevated (4 or more) in 27% (20 of 75 patients).
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Outcome
OPERATIVE MORTALITY.
There were 16 operative deaths. Poor cardiac function with or without evidence of acute elevation of PVR was the cause of death in 69% (11 of 16 patients). Other causes included progressive renal failure (n = 1), cardiac tamponade after removal of intracardiac lines (n = 1), cerebral edema (n = 1), and sepsis (n = 2). Table 1 summarizes the operative mortality and era of operation.
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). Average follow-up was 5.2 ± 0.5 years. Actuarial survival curves were also calculated separately for patients with mild or less preoperative LAVR and compared with patients with moderate or severe preoperative LAVR. For patients with mild or less preoperative LAVR, 10-year actuarial survival was 82% compared with 76% for patients with moderate to severe preoperative LAVR (p = NS) (Fig 4)fig 4.
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LATE MORBIDITY.
The degree of late LAVR was evaluated as an indicator of late morbidity. Echocardiographic or cardiac catheterization data was used to evaluate late LAVR (more than 30 days postoperatively) and data were available on 76% (75 of 99 patients) of the operative survivors. Late LAVR was severe in 3% (2 of 75 patients), moderate in 17% (13), mild in 32% (24), trace in 29% (22), and absent in 19% (14 patients). Therefore, late LAVR was moderate to severe in 20% (15 patients) and less than or equal to mild in 80% (60 patients). Data (either echocardiogram or ventriculogram) on late ventricular function were available for 74% (73 of 99 patients). Poor late ventricular function was identified in 3% (2 of 73 patients) and was used as an indicator of late morbidity. Six patients (6%) underwent a total of seven late LAV operations. One patient underwent mitral valvuloplasty (1 year postoperatively) and subsequently underwent a mitral valve replacement (6 years postoperatively). This patient had the only mitral valve replacement in the series. Five additional patients underwent mitral valve repair. The need for additional LAV operation as well as moderate or severe LAVR were both considered to be late morbid outcomes for the purposes of analysis. Permanent pacemakers were implanted in 2% (2 of 99) of the patients in this series. One patient had a pacemaker placed in the early postoperative period and 1 patient underwent pacemaker placement 8 years after complete repair.
Predictors of Operative and Late Mortality and Morbidity
OPERATIVE MORTALITY AND LONG-TERM SURVIVAL.
Only the era of operation had an impact on operative survival with mortality decreasing significantly in the most recent era (Table 1
). To determine the possible independent contributions of age, operative technique, and era of operation on operative mortality, these variables were entered into multiple logistic regression analysis. Only era of operation was found to be significant (p = 0.03). Ten-year actuarial survival was 81% for all 115 patients in the series (Fig 3) and the degree of preoperative LAVR did not have an impact on long-term survival (Fig 4).
OPERATIVE MORBIDITY.
Univariate analysis indicated that repair during early infancy, the presence of Down's syndrome, associated congenital heart disease, elevated PVR, and two-patch repair technique predicted a need for increased levels of intensive care unit support. Entering these factors into a multiple logistic regression analysis model, only the use of a two-patch repair technique was associated with an increased level of intensive care unit support (Table 2).
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IMPACT OF PREOPERATIVE LAVR ON LATE POSTOPERATIVE LAVR.
The degree of preoperative LAVR did not predict late postoperative LAVR. Among the 21 patients with moderate to severe preoperative LAVR, there were 17 operative survivors with late follow-up available on 15. Among these 15 patients, preoperative LAVR was severe in 1 and moderate in 14. Late LAVR was severe in 1, moderate in 4, mild in 6, trace in 2, and absent in 2 patients. This improvement was significant, p = 0.007, by Wilcoxon signed rank test (Fig 5). Among patients with mild or less preoperative LAVR, late LAVR was moderate or severe in 17% (10 of 60 patients) compared with a 33% (5 of 15 patients) incidence of moderate or severe late LAVR among patients with moderate or severe preoperative LAVR; this difference was not significant.
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Comment |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentAcknowledgmentsReferences |
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Coincident with a decrease in mortality over time is an increase in the rate of repair of CAVSD during infancy. The transition to routine repair during early infancy was accomplished without an increased risk of mortality, and repair in early infancy avoids the use of palliative procedures [6]. In addition, progressive ventricular dilatation from volume overload with progressive atrioventricular valve annular dilatation may be avoided by correction during early infancy, thus decreasing the complexity of the repair and the potential for residual LAVR [3]. Because results of repair during early infancy are excellent and because of the potential for decreased late morbid outcomes we, as well as many other investigators, advocate complete repair during early infancy.
In this series, the technique of repair shifted from a one-patch repair to a two-patch repair. Potential advantages of the one-patch repair is that it provides excellent exposure of the crest of the interventricular septum; however, in the presence of significant bridging leaflets, the leaflet must be incised to place the patch [7–9]. The structural integrity of the valve is violated, and perhaps competence of the reconstructed LAV sacrificed [10]. Because most patients come to CAVSD operation with mild or less LAVR, techniques that preserve LAV competence should be superior. The aim of the two-patch repair is to close the interventricular communication with minimal alteration of valve architecture, therefore preserving LAV competence [10, 11]. Although at present the two-patch repair is our technique of choice, our findings do not clearly define statistically one technique as superior. The apparent association between the two-patch repair and increased need for postoperative support coincides with a decrease in the age at the time of correction and may be difficult to separate from a general increase in the intensive care unit support directed at infants undergoing cardiac repair. Univariate analysis identified the one-patch repair as a risk factor for late LAVR. However, because the two-patch repair has been the dominate technique used only since 1989 (Fig 2), the follow-up for the two-patch repair technique is shorter and by multivariate analysis, one-patch repair was not identified as a risk factor for late LAVR. Other factors such as a critical volume of surgical cases, as well as cumulative experience with one or the other repair technique are also likely to be important factors.
Multivariate analysis identified only ischemic time of 105 minutes or more as a marginal risk factor (p = 0.05) for unsatisfactory LAV function. A longer ischemic time, however, was not associated with an increase in early morbidity or mortality indicating that within the range of ischemic times observed in this study myocardial preservation techniques were adequate. Longer ischemic time probably indicates only a more difficult initial repair and one that is less likely to yield satisfactory long-term results.
To avoid injury to the conduction system, sutures placed in the vicinity of the bundle of His were not tied until the cross-clamp was released and the heart began to beat in sinus rhythm. If heart block developed, sutures were removed and replaced. Routine use of left ventricular venting as well as moderate hypothermia prevented ejection of air during this phase of the procedure. The incidence of injury to the conduction system was minimal. Two patients required a permanent pacemaker, one in the early postoperative period and one 8 years after complete repair.
Is preoperative moderate to severe LAVR a risk factor for mortality and morbidity after repair of CAVSD? Others have suggested that patients with moderate to severe preoperative LAVR are at increased risk for mortality and morbidity after repair of CAVSD [1, 2]. In this series we found no increase in early or late mortality among patients with moderate to severe preoperative LAVR compared with patients who came to operation with mild or less LAVR. Patients with moderate to severe preoperative LAVR had significantly improved postoperative LAVR. Most patients undergoing operation with moderate to severe LAVR had mild or less LAVR in the late period. Satisfactory and durable valve reconstruction can be achieved in patients with moderate to severe preoperative LAVR with survival similar to patients who undergo operation with mild or less LAVR. Our findings are in agreement with McGrath and Gonzalez-Lavin [12] and Weintraub and colleagues [13] who found that satisfactory LAV reconstruction neutralized preoperative LAVR as a risk factor for mortality and morbidity.
The accuracy of a retrospective study might be questioned. In particular, the degree of LAVR was assessed using different techniques, multiple observers, and spanning a period of 20 years. However, we believe the studies were carefully evaluated and the reports are reasonably accurate. A prospective echocardiographic study could be undertaken to confirm our results.
In summary, in the current era repair of CAVSD can be carried out successfully in infants with low mortality and minimal long-term morbidity. The two-patch repair technique may allow for superior LAV reconstruction with a lower incidence of late moderate to severe LAVR. Patients with moderate to severe preoperative LAVR can be expected to have a significant reduction in postrepair LAVR with long-term survival similar to patients who undergo operation with mild or less LAVR.
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Acknowledgments |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentAcknowledgmentsReferences |
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Footnotes |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentAcknowledgmentsReferences |
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Address reprint requests to Dr Tweddell, Department of Cardiovascular Surgery, MS 715, Children's Hospital of Wisconsin, 9000 W Wisconsin Ave, PO Box 1997, Milwaukee, WI 53201.
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentAcknowledgmentsReferences |
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