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Ann Thorac Surg 2002;73:622-627
© 2002 The Society of Thoracic Surgeons
a Division of Paediatric Cardiology, Grantham Hospital, The University of Hong Kong, Hong Kong, People's Republic of China
b Division of Cardiothoracic Surgery, Grantham Hospital, The University of Hong Kong, Hong Kong, People's Republic of China
Accepted for publication October 9, 2001.
* Address reprint requests to Dr Cheung, Division of Paediatric Cardiology, Department of Paediatrics, Grantham Hospital, The University of Hong Kong, 125 Wong Chuk Hang Rd, Aberdeen, Hong Kong, People's Republic of China
e-mail: xfcheung{at}hkucc.hku.hk
| Abstract |
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Methods. We reviewed the long-term outcome of 135 patients who underwent closure of their defect and, in light of the findings, assessed the impact of preoperative aortic cusp prolapse and surgical interventions on occurrence of aortic regurgitation (AR) in the long-term. The patients were categorized into three groups for comparison: group I consisted of 79 patients with no aortic cusp prolapse and underwent simple closure of ventricular septal defect, group II comprised 39 patients with mild to moderate cusp prolapse who similarly had only closure of the defect performed, whereas group III comprised 17 patients who had additional aortic valvoplasty for greater than moderate to severe cusp prolapse.
Results. Group I patients had significantly higher pulmonary arterial pressure (p < 0.001) and ratio of pulmonary blood flow to systemic blood flow (p < 0.001). None of these patients had AR before their operation, and none experienced AR afterward at a median follow-up of 6.1 years. Of the 39 group II patients, 30 (77%) had trivial or mild AR preoperatively. The AR improved in 15 patients, remained trivial or mild in 14 and absent in 7, but progressed to trivial or mild in 3 at a median follow-up of 3.1 years. None required further interventions. In contrast, 14 (82%) of the 17 group III patients had moderate to severe AR before operation. The regurgitation improved in 10, but remained moderate or severe in 4 and worsened further in 3 at a median follow-up of 4.6 years. The freedom from failure of aortic valvoplasty was (mean ± standard error of the mean) 71% ± 11%, 64% ± 12%, and 43% ± 19% at 1, 5, and 10 years, respectively. An older age at latest follow-up was the only identifiable significant risk factor (p = 0.03).
Conclusions. Our data do not support the need of aortic valvoplasty for mild to moderate aortic cusp prolapse. Close follow-up is warranted in those with greater than moderate to severe cusp prolapse despite valvoplasty as there is continued failure on follow-up. Nothing, however, is better than early closure of defects before development of aortic valve complications.
| Introduction |
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We reviewed the long-term outcome of 135 patients who underwent surgical closure of subarterial VSDs, 17 of whom had additional aortic valvoplasty and, in light of our findings, assessed the impact of preoperative aortic cusp prolapse and surgical interventions on occurrence of AR in the long-term.
| Patients and methods |
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On the basis of the severity of aortic cusp prolapse as elaborated below and the surgical procedures performed, the patients were categorized into three groups for comparison. Group I consisted of 79 patients without aortic cusp prolapse who underwent simple patch repair of the defect. Group II comprised 39 patients who, despite the presence of mild to moderate cusp prolapse, only had patch repair of the defect. Group III comprised 17 patients who had greater than moderate degree of cusp prolapse and underwent aortic valvoplasty in addition to closure of the defect.
Aortic cusp prolapse
Cusp prolapse was graded on a three-point scale [18, 19] by echocardiographic imaging: mild (buckling of aortic cusp with minimal herniation, aortic cusp protrudes slightly into defect only during early systole), moderate (prolapse of cusp with obvious herniation into the septal defect), and severe (prolapse of cusp and its sinus through the defect into the right ventricular outflow tract during both systole and diastole). The severity of prolapse was further confirmed during operation.
Aortic regurgitation
Using color Doppler echocardiography, the degree was graded [5] as trivial (slight AR under the aortic valve), mild (AR not reaching the tip of mitral valve leaflet), moderate (AR reaching the tip of mitral valve leaflet), and severe (AR beyond the tip of mitral valve leaflet). The severity of AR was assessed within 1 week of operation and serially during the postoperative period. Immediate postbypass assessment in the operating theater was performed since 1992 when transesophageal echocardiography became available to us. Transthoracic echocardiography was performed at the time of discharge (5 to 7 days after operation), on early follow-up at 2 and 4 weeks after operation, and thereafter every 6 months to 1 year during late follow-up. The severity of AR at latest follow-up was used in the outcome analysis. Failure of aortic valve repair was defined by persistence of moderate degree of AR despite aortoplasty, progression to moderate or severe AR on follow-up, or the need to reoperate for worsening regurgitation.
Surgical procedure
The transpulmonary approach was used. Aortic cusp prolapse was identified and graded by visualizing through the defect during infusion of cardioplegic solution into the coronary sinus. A transverse aortotomy was made when aortic valve exploration was required. Aortic valvoplasty was performed if cusp prolapse was of greater than moderate to severe degree. A stay suture was then placed in the corpus arantii of the normal leaflets and the center of the prolapsed leaflet, and excessive valve tissue was plicated and sutured with pledgets to the commissural margins close to the aortic wall [20]. The septal defect was closed with a prosthetic patch.
Associated structural cardiac lesions that were surgically corrected in the same setting or in stage included patent ductus arteriosus (n = 26), atrial septal defect or foramen ovale (n = 21), coarctation of aorta (n = 19), valvar pulmonary stenosis (n = 6), and interrupted aortic arch (n = 4).
Statistical analysis
Data are presented as mean ± standard deviation or median (range) as appropriate. Differences in parametric variables among the three patient groups were compared by one-way analysis of variance with post hoc comparison by Bonferroni test, whereas nonparametric analysis was performed using Kruskal-Wallis test with post hoc comparison by Dunns multiple comparison test. Freedom from failure of aortic valvoplasty was analyzed by Kaplan-Meier actuarial survival analysis. Univariate analysis was used to determine risk factors for failure of aortic valvoplasty, using unpaired Students t test for continuous variables and Fishers exact test for nominal variables. A p value of less than 0.05 was considered significant. All analyses were performed using SPSS version 8.0 (SPSS, Inc, Chicago, IL).
| Results |
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The differences among the three groups are summarized in Table 1. Group I had congestive heart failure with significantly higher pulmonary arterial pressure (p < 0.001) and ratio of pulmonary blood flow to systemic blood flow (p < 0.001), hence requiring operation at an earlier age (p < 0.001). Despite having VSDs of size similar to those of group I, group II had lower pulmonary arterial pressure (p < 0.001) and a smaller ratio of pulmonary blood flow to systemic blood flow (p < 0.001), related probably to reduction of shunt by the prolapsing aortic cusp. Among all groups, group III had the largest VSDs (p < 0.001). Nonetheless, their pulmonary arterial pressure and ratio of pulmonary blood flow to systemic blood flow, although similar to those of group II (p = 1.00 for both), were significantly lower than those of group I (p = 0.001 for both).
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Surgical outcome
Group I
The indication for surgical closure of the defect was a large left-to-right shunt that caused pulmonary hypertension and congestive heart failure. None of the 79 patients had aortic cusp prolapse or AR at the time of operation. Aortic regurgitation was not detected in any of these patients after operation on a median follow-up of 6.1 years (range, 0.5 months to 18.4 years).
Group II
The impact of surgical closure of the defect on AR in this group of 39 patients with mild to moderate aortic cusp prolapse is shown in Figure 1A.
Nine (23%) patients had no AR before operation, whereas 30 (77%) had trivial or mild AR. Of the latter 30 patients, reduction of AR after operation was shown in 15, whereas AR remained either trivial or mild in 14 and absent in 7. Worsening of AR was noted in 3 (7.7%) patients, with progression from none to trivial or mild in 2, and trivial to mild in 1. Nevertheless, none of the patients exhibited moderate or severe AR that required further surgical interventions on a median follow-up of 3.1 years (range, 0.7 months to 16.0 years).
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Failure of aortic valve repair
Seven of the 17 group III (41%) patients had failure of aortic valvoplasty. Two patients required prosthetic valve replacement as described earlier, and another 3 are awaiting valve replacement. The freedom from failure (mean ± standard error of mean) of valve repair was 71% ± 11%, 64% ± 12%, and 43% ± 19% at 1, 5, and 10 years, respectively (Fig 2).
Univariate analysis revealed that age at latest follow-up was the only significant factor associated with failure (p = 0.03; Table 2).
The number of aortic cusps involved, degree of AR before operation, size of VSD, and hemodynamic factors were not found to be significant.
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| Comment |
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It is obvious, therefore, that preoperative aortic cusp prolapse has important bearings on the severity of AR after operation. Furthermore, we have shown that severity of cusp prolapse correlates positively with defect size, in contrast to previous reports of an association between large defects and absence of cusp prolapse [23, 24]. The use of Doppler color flow [23] or angiography coupled with hemodynamic studies [24] to assess VSD size previously, rather than surgical measurements, might have led to underestimation of the actual defect size once prolapse occurs. Not surprisingly, our results concur with those of the surgical series reported by Ishikawa and colleagues [25].
For subarterial VSDs without concomitant aortic valve complications, early patch closure prevents the occurrence of the latter. This agrees with the findings of Sim and associates [14], who reported on the outcome of 45 surgical patients. As surgical risk is extremely low, universal surgical closure has been advocated [9, 10]. Nonetheless, we have recently shown that defects less than 5 mm in size are unlikely to be associated with aortic valve deformities or AR [26]. Given this finding, it would perhaps be more rational to suggest closure of subarterial VSDs of at least 5 mm as early as possible to prevent development of AR in the long-term.
Whether aortic valvoplasty should be routinely performed in patients with both aortic cusp prolapse and AR has been controversial [16, 2731]. This controversy might in part be related to failure of risk stratification based on severity of cusp prolapse. The severity of AR, which occurs secondarily to aortic cusp deformity [32], is commonly used instead for such purpose in previous studies [1114]. It is important to note, however, that, severity of AR may vary from none to mild in patients with mild to moderate cusp prolapse (Fig 1A) and trivial to severe in those with moderate to severe cusp prolapse (Fig 1B). Discordance between the two phenomena might have confounded the interpretation. We have therefore categorized our patients on the basis of severity of cusp prolapse. We have shown that in patients with mild to moderate degree of aortic cusp prolapse before operation, simple closure of the defect without aortic valvoplasty effectively reduces or halts the progression of AR in 92% of our patients. Closure of the defect alone is believed to prevent progression of AR by reducing the Venturi effect [7, 14]. However, in contrast to the outcome of group I patients, progression of AR, albeit uncommon, may occur. This finding is similar to those previously reported [14, 17]. Nonetheless, the degree of residual AR is at most trivial to mild in our patients who required no further interventions. Hence, it appears unjustified to recommend aortic valvoplasty as a routine in this patient group.
On the other hand, aortic valvoplasty was performed in all of our patients with greater than moderate to severe aortic cusp prolapse, the majority of whom had moderate to severe AR preoperatively. The long-term outcome, however, is discouraging, with a freedom from failure of the procedure at 10 years of only 43%. We identified an older age at latest follow-up as the only significant risk factor for failure, relating probably to an older age at operation and a longer duration of follow-up (Table 2). The latter two variables were not statistically significant, however, possibly as a result of the relatively small number of patients in this group. Secondary aortic annular dilation and cuspal retraction may account for the poorer outcome in those operated on late. Almost all of the previous reports on long-term outcome after aortic valvoplasty have included patients with both subarterial and perimembranous VSDs [1113, 15, 16]. Okita and coworkers [13] reported a 74.2% and 55.3% freedom of valvoplasty failure at 10 and 15 years, respectively. Risk factors that were identified in their study included older age at operation, a greater cardiothoracic ratio, perimembranous VSD, and multiple previous valvoplasties. Whereas Trusler and colleagues [11] reported a similar freedom of valvoplasty failure at 10 years of 76%, they found that neither age at repair nor VSD position are risk factors. Rather, associated structural defect of the aortic valve was found to be significant. The only patient in our series with a bicuspid aortic valve had severe AR before operation, and valve repair resulted only in transient improvement with failure noted by 1 year of follow-up. Using solely the need for reoperation as the definition of failure of valve repair, Elgamal and associates [12] reported a 10-year to 15-year actuarial freedom from reoperation of 81% and identified the degree of AR at the time of hospital discharge (the only factor with p < 0.05) as a risk factor. In none of these previous reports, however, is the severity of cusp prolapse mentioned. The degree of preoperative AR in their patients varies from mild to severe. It might be possible, therefore, that the better outcome of their patients is related in part to inclusion of those with a milder degree of cusp prolapse.
Although aortic valvoplasty was performed in our group III patients using the technique as described by Trusler and colleagues [20], alternative approaches have been described [3335]. Yacoub and colleagues [33] proposed reconstruction of the dilated sinus of Valsalva with elevation of aortic annulus and cusp, thus increasing cusp coaptation and reducing or abolishing AR. However, whether this is superior to the Trusler technique is uncertain. Another approach that might result in a lower early and late failure of valve repair, as compared with the Trusler technique, has been described by Carpentier [34] and Chauvaud and associates [35]. This approach involves triangular resection of the prolapsed cusp, reinforcement of the aortic wall, and aortic annuloplasty. We, however, do not have experience with either of these techniques.
Subarterial VSD with greater than moderate to severe aortic cusp prolapse is hence a progressive cardiac lesion despite closure of the defect and repair of the valve. Close monitoring is warranted especially in patients who are operated on late and those with structurally abnormal aortic valve. Our data do not support aortic valvoplasty in patients with mild to moderate degree of cusp prolapse. Nothing, however, is better than early closure of defects before development of aortic valve complications.
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