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J Thorac Cardiovasc Surg 2003;126:521-528
© 2003 The American Association for Thoracic Surgery
Surgery for congenital heart disease |
a Childrens Heart Center, Wilhelmina Childrens Hospital UMC, Utrecht, The Netherlands
Received for publication June 18, 2002; revisions received August 20, 2002; revisions received September 16, 2002; accepted for publication October 8, 2002.
* Address for reprints: Ger B. W. E. Bennink, MD, PhD, Wilhelmina Childrens Hospital, Department of Cardiology/Cardiac Surgery, University of Utrecht, PO Box 18009, 3501 CA Utrecht, The Netherlands
g.bennink{at}azu.nl
| Abstract |
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METHODS: Two hundred sixty-two children had surgical repair of coarctation of the aorta by means of either resection and end-to-end anastomosis (n = 137; mean age, 1.85 ± 3.1 years) or polytetrafluoroethylene patch aortoplasty (n = 118; mean age, 1.09 ± 1.9 years) during a 28-year period. Coarctation was isolated in 109 (41.6%), associated with ventricular septal defect in 77 (29.4%), and associated with complex intracardiac anomalies in 76 (29.0%) patients. Follow-up ranged from 2 days to 29.3 years (median, 11.9 years). Seven patients were lost to follow-up. Kaplan-Meier survival curves were estimated, and multivariable Cox regression analysis was performed for several outcome variables.
RESULTS: Mortality was 8.2% and was associated with intracardiac pathology in all cases. Recoarctation occurred in 53 patients, 23 after resection and anastomosis and 30 after patch repair, not differing statistically (P = .4, log-rank test). Aneurysm formation occurred in 8 patients after patch repair that included ridge resection in 7 of the 8 patients. Late hypertension occurred in less patients (n = 3) after resection and anastomosis than after patch repair (n = 8) (P < .03). Arch hypoplasia (P < .01) and age less than 1 month (P < .001) were found to be independent risk factors for recoarctation.
CONCLUSIONS: Polytetrafluoroethylene patch repair including coarctation ridge resection was found to be a risk factor for aneurysm formation and late hypertension. Arch hypoplasia and young age must be considered to predispose to recoarctation.
Aneurysm formation after Dacron patch aortoplasty for coarctation of the aorta has been reported on extensively, in as much as 51% of patients.1 However, aneurysm incidence after repair with polytetrafluoroethylene (PTFE) appears to be comparable with that after other techniques.2 Among a variety of surgical techniques, we have continued to perform patch aortoplasty with PTFE as patch material in the recent past. The objectives of this retrospective review were 3-fold. Our first intent was to evaluate the immediate results and long-term follow-up regarding mortality, recoarctation, aneurysm formation, and late hypertension after coarctation repair in all age groups with resection and end-to-end anastomosis (RETE) and patch angioplasty. The second goal was to compare those results for both surgical techniques to identify the ideal form of surgical management or to specify indications for these techniques. The third and final objective was to identify risk factors for various outcomes on which our surgical management can be based.
| Patients and methods |
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The immediate results of surgical intervention were considered satisfactory if a pulsatile flow in the descending aorta was registered by means of palpation after the operation and the resultant pressure gradient between the upper and lower limbs was less than 20 mm Hg.
Follow-up
The length of follow-up ranged from 2 days to 29.3 years (median, 11.9 years). Seven patients were lost to follow-up: 4 patients after RETE and 3 after patch angioplasty. Follow-up was limited by death in 26 patients. Follow-up ranged from 1 to 10 years in 115 (44%) of 262 patients and from 10 to 20 years in 102 (39% of 262) patients and was greater than 20 years in 45 (17%) of 262 patients. Follow-up included clinical evaluation every 3 months in the first year after intervention and yearly thereafter. Arm and leg cuff pressures were registered, and chest radiography and 2-dimensional continuous-wave echocardiographic Doppler ultrasound studies performed. Follow-up catheterization and angiography were performed in patients in whom aneurysm formation or recoarctation was suspected. An aneurysm was defined as an aortic ratio of greater than 1.5, measuring aortic diameters at the coarctation repair site and thoracic aorta at the level of the diaphragm. A gradient of 20 mm Hg and a registered continuation of flow in diastole in echocardiographic Doppler ultrasound studies or blood pressure gradients between the upper and lower extremities exceeding 20 mm Hg were considered to correspond with recoarctation, requiring reintervention. Magnetic resonance imaging was performed in patients suspected of having aneurysm formation. Recoarctation was treated with repeat surgery or BA after additional angiography.
Statistical methods
Kaplan-Meier survival curves were estimated to determine intervention-free probabilities for various groups. Cumulative probabilities in these groups were compared against guidelines by using the log-rank test. Multivariable Cox regression analyses were performed for the various outcomes for which sufficient events were encountered. Potential risk factors in these analyses included era (1973-1987 vs 1988-2000), age (<1 month, 1 month to 1 year, or >1 year), type of repair (PTFE patch aortoplasty vs RETE), and aortic arch hypoplasia, isthmus hypoplasia, or both.
| Results |
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Mortality
Mortality is specified in Table 2.
Overall mortality was 21 (8.2%) of 255 patients. Seven patients died of postoperative complications after primary repair of coarctation. The procedure used was RETE in 3 of 7 patients and patch aortoplasty in 4 of 7 patients. Kaplan-Meier analysis for survival was performed, differentiating groups 1, 2, and 3. The cumulative survival probability at 10 years follow-up was 96.8% ± 1.8% for patients in group 1, 87.0% ± 4.4% in group 2, and 80.6% ± 4.9% in group 3. Log-rank testing revealed a significant difference between these groups regarding mortality (P = .001, Figure 2).
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Aneurysm formation
Aneurysm formation was encountered in 8 (3.1%) of 255 patients, all after patch aortoplasty (Figure 6).
The interval to aneurysm formation varied from 4 days to 18.6 years (median, 12.3 years). The actuarial 15-year probability of freedom from aneurysm formation after patch aortoplasty was found to be 93% ± 3.1%. The aneurysm that developed within 4 days after surgical intervention was identified to be a false aneurysm. All other aneurysms developed opposite the patch along the medial aspect of the aorta. All these aneurysms developed after procedures involving coarctation ridge resection performed before 1991. Transverse arch hypoplasia was present in 1 of these patients, and both arch and isthmus hypoplasia were present in 2 of these patients. Patients in whom aneurysm formation was encountered underwent successful aneurysm resection and patch graft interpositioning. Neither age nor transverse arch-isthmus hypoplasia were predictors of aneurysm formation.
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| Discussion |
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Recoarctation and late hypertension
Echocardiography played a central role in the screening for recoarctation of our patients. When optimal imaging is feasible, being the case in infants and children, the literature supports combined 2-dimensional and Doppler color flow echocardiography to image the aortic arch, isthmus, and coarctation site. A sensitivity of 100% for recoarctation can thus be achieved by means of echocardiography and clinical visit, as was pointed out by Therrien and colleagues.8 The primary focus in this and most other studies investigating risk factors for recurrent arch obstruction after early coarctation repair is the relationship between the technique of repair and recoarctation. The type of surgical repair might influence the prevalence of residual or recurrent coarctation by incomplete resection of ductal tissue, suture material, and the width of the anastomosis. The possible mechanisms in the process of recoarctation related to these surgical factors consist of inadequate growth of the anastomosis, active fibrosis and narrowing at the anastomotic site, thrombosis at the suture line, and retention of abnormal, possibly ductal, tissue.9 In the absence of a large randomized study, superiority of one technique over another is less likely to be demonstrated. On the whole, these studies demonstrate no difference in recoarctation incidence between RETE and subclavian flap,5,6 although the probability of recoarctation appears to be higher after simple patch aortoplasty, when patch material used was Dacron or unspecified.7,10 Reported recoarctation rates after PTFE patch aortoplasty, although sparse, are comparable with those after other techniques.2,11-13 Although more recoarctation after patch aortoplasty has been found in this series (30 [25%] of 118 patients vs 19 [14%] of 133 patients for RETE), type of repair was no independent predictor for recoarctation. Furthermore, significant differences could not be established in stratifying patients younger and older than 1 month of age, although a younger age has been suggested to predispose to recoarctation after PTFE patch repair.2 The age at operation has been found to be related to the development of recoarctation and, in an inverse manner, to the development of late hypertension. Brouwer and associates14 determined the optimal age for elective aortic coarctation repair for the infant with isolated aortic coarctation who is symptom free to be 1.5 years. The risk for recoarctation decreases rapidly and levels off at 1.5 years of age, and the risk for late hypertension and premature death increases progressively after that age range.14,15 It has been pointed out that the age of 1.5 years might be rather conservative because recoarctation rates decrease to less than 5% in current surgical experience with repair performed at age less than 1 year16, and chronic hypertension develops in as much as 60% of patients thereafter in other studies.17 We therefore stratified patients younger and older than 1 year of age but could not find any significant difference in those age groups regarding late hypertension. Nevertheless, this difference was found for type of repair because patch aortoplasty was associated with a higher late hypertension rate. We hypothesize that in patch aortoplasty the combination of residual ductal tissue and diminished aortic wall compliance is responsible for the occurrence of late hypertension in this group. These phenomena might play a role in recoarctation in long-term follow-up also. This might explain the disproportional hazards for recoarctation that were found after 5 years in patients undergoing after different types of repair (Figure 2).
Arch hypoplasia
Several reports demonstrated that the anatomy of the aortic arch was a predictor of reintervention for recurrent obstruction after repair.9,14 Transverse arch hypoplasia was present in 32 (13%) of 255 patients in this study and was demonstrated to be an independent risk factor for recoarctation. The presence of a hypoplastic arch usually increases the left ventricular obstruction created by the coarctation. Nevertheless, a conservative approach toward the hypoplastic arch seems to be justified in most cases because Siewers and associates18 have suggested that when the ratio of transverse aortic arch to ascending aorta exceeds 0.25, the arch can be expected to grow normally without obstruction and that extended resections should be reserved for those with ratios of less than 0.25.
Aneurysm formation
The interval to aneurysm formation varied from 2 to almost 19 years, thereby warranting a follow-up for a long enough period. The follow-up protocol for patients in this series, including clinical visits, echocardiography and chest radiography as a screening test and performing magnetic resonance imaging on patients with positive results, was identified as an appropriate strategy in adult patients for both aneurysm formation and recoarctation by Therrien and colleagues.8 Because we consider echocardiography more sensitive and specific at lower patient ages, we believe that this policy is appropriate for children as well.
RETE proved to be superior to PTFE patch aortoplasty most apparently in respect to aneurysm formation. All aneurysm formation in this series, in 8 (6.4%) of 125 patients, was encountered after patch aortoplasty. We suggest that indication for PTFE patch aortoplasty, despite its relative easy performance and favorable surgical record, must be narrowed in favor of RETE. Aneurysm formation after patch aortoplasty has been reported on extensively, with the reported incidence varying between 2% and 51%.1,19,20 The cause of aneurysm formation after patch aortoplasty has been attributed to several different factors that are discussed subsequently.
Material
There appears to be a striking difference between patch aortoplasty using Dacron grafts compared with using PTFE. Considerable foreign body giant-cell infiltration is demonstrated in Dacron grafts,21 which is not the case with PTFE.19 Probably this inflammatory response to a Dacron patch makes it more susceptible to aneurysm formation than a PTFE patch, despite Dacron being 20% more compliant, a condition that was considered to be favorable.19,22,23 The stretching and degeneration of fibers of Dacron, with subsequent dilatation of the material,24 has not been found in PTFE, which does not appear to show any signs of breakdown through chemical or biologic processes over time.25 Indeed, a higher incidence of aneurysm formation has been reported after Dacron patch aortoplasty than after repair with PTFE in studies in which both materials were applied.13,26 Additionally, no aneurysm formation has been encountered in several reports on use of PTFE2,12,13 compared with aneurysm rates of between 3% to 22% after patch aortoplasty with Dacron patch.20,22,23 As regards the use of nonprosthetic material, observations are sparse. Autogenous internal thoracic artery was applied as patch material in several studies, with good results in limited follow-up periods.27
Resection of coarctation ridge
The coarctation ridge was excised in the performance of patch aortoplasty in all our patients until 1991. At that time, we changed our policy with regard to resecting the coarctation ridge because violating the intimal aortic layer by resection of the fibrous coarctation membrane has been found to be highly related to aneurysm formation in patients in several studies.22,23 DeSanto and associates28 performed patch aortoplasty with and without concomitant intimal excision in dogs experimentally. Aneurysms formed in 8 of 12 animals who had intimal excisions, and no aneurysms formed in the control animals. All true aneurysm formation we observed might be caused by ridge resection in patients before the recent era. With a modified patch technique, leaving the coarctation ridge intact, we only encountered one false aneurysm. These observations correspond with those of Backer and coworkers.2
Strain and stress
The experimental in vitro findings of Smaill and associates29 suggest that synthetic patch aortoplasty might produce increases in wall strain and disproportionate increases in stress adjacent to the patch associated with changes in local aortic wall geometry that are a direct result of patch implantation. The altered hemodynamics that result form the different tensile strengths of a Dacron patch and the posterior aortic wall, with the pulsatile waveform being completely directed to the posterior aortic wall by the inflexible anterior patch, might cause dilatation of the pliable aortic wall.20,22,23 We do not know of any report on PTFE patch material or modification of the technique of patch aortoplasty in which these effects were found to be successfully prevented.
Age
None of the age categories were found to be risk factors for aneurysm formation in our series. On the contrary, previous reports on Dacron patch aortoplasty supported early surgical repair.1,15 The risk of aneurysm formation was reduced substantially by operating earlier.1 Although the aortic wall might be structurally different in infants, thus enabling it to adapt more rapidly to a patch aortoplasty than the aortic wall of patients who undergo surgical repair at an older age, this might depend on Dacron. According to several authors, prosthetic patch aortoplasty should be abandoned in adolescents and adults, in whom a high incidence of aneurysm formation was found with advanced age. Tubular interpositioning grafting and patch aortoplasty with the autogenous arterial wall are proposed as viable alternatives.13,20,27 However, there appears to be a minimum age because Backer and associates2 found a high recurrence rate in infants younger than 1 month of age, whereas the recoarctation rate was 4 of 97 in patients older than 1 month of age after patch aortoplasty with PTFE.
Aortic arch hypoplasia
Different from recoarctation, arch hypoplasia did not reach statistical significance as a risk factor for aneurysm formation. In contrast to our findings, Bogaert and associates30 have identified transverse arch hypoplasia as a condition highly related to aneurysm formation after patch aortoplasty. They suggest that narrowing of an aortic segment leads to blood flow acceleration and poststenotic turbulence, which might induce aneurysm development in the distal aortic segments. Because aneurysm formation has been encountered after RETE,11 in which the coarctated segment is fully resected, and no abnormal patch material is present, wall abnormalities by themselves cannot be (solely) responsible for aneurysm formation, despite statements in several reports.11,20,28 Abnormal flow patterns originating in a concomitant hypoplastic arch might have a similar influence on RETE and patch aortoplasty.30
Limitations
The design of this study is limited by the fact that it is retrospective and spans 3 decades. During this period, including patients for RETE and patch aortoplasty has been rather constant. However, both surgical techniques have been improved gradually in the course of time, although era (1971-1987 vs 1988-2000) was not related to any outcome. Furthermore, patients were not randomized for both techniques. The choice of either RETE or patch aortoplasty was based on the surgeons preference in general, but selection bias could not be ruled out in this study design.
Conclusions
The present study findings do not allow extensive conclusions to be drawn in the treatment of coarctation associated with complex intracardiac malformations, for which this association was the most important risk factor. Comparing both surgical techniques, the resection of the coarctation ridge seems to be an important factor with regard to aneurysm formation. Considerable aneurysm formation was found after patch aortoplasty combined with coarctation ridge resection. Our findings demonstrate no differences between recoarctation and aneurysm formation with a modified patch aortoplasty. Arch hypoplasia and age younger than 1 month must be considered to predispose to recoarctation in patients with coarctation managed surgically. Although a slightly increased risk of late hypertension was identified after patch repair, we consider both surgical techniques to be comparable.
| Acknowledgments |
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