Author + information
- Received February 11, 2015
- Revision received April 13, 2015
- Accepted May 7, 2015
- Published online August 24, 2015.
- José Suárez de Lezo, MD, PhD∗∗ (, )
- Miguel Romero, MD, PhD∗,
- Manuel Pan, MD, PhD∗,
- Javier Suárez de Lezo, MD, PhD∗,
- José Segura, MD, PhD∗,
- Soledad Ojeda, MD, PhD∗,
- Djordje Pavlovic, MD, PhD∗,
- Francisco Mazuelos, MD, PhD∗,
- José López Aguilera, MD∗ and
- Simona Espejo Perez, MD†
- ∗Department of Cardiology, Reina Sofia University Hospital, University of Córdoba and Instituto Maimónides para la Investigación Biomédica en Córdoba, Córdoba, Spain
- †Department of Radiology, Reina Sofia University Hospital, University of Córdoba and IMIBIC, Córdoba, Spain
- ↵∗Reprint requests and correspondence:
Dr. José Suárez de Lezo, Department of Cardiology, Reina Sofía University Hospital, Avenida Menéndez Pidal s/n, 14004 Córdoba, Spain.
Objectives This study sought to determine whether several anatomic or evolving characteristics of the coarctation may create challenging conditions for treatment.
Background Stent repair of coarctation of aorta is an alternative to surgical correction.
Methods We analyzed our 21-year experience in the percutaneous treatment of complex coarctation of aorta. Adverse conditions for treatment were as follow: 1) complete interruption of the aortic arch (n = 11); 2) associated aneurysm (n = 18); 3) complex stenosis (n = 30); and 4) the need for re-expansion and/or restenting (n = 21). Twenty patients (33%) belonged to more than 1 group. Ten interruptions were type A and 1 was type B. The mean length of the interrupted aorta was 9 ± 11 mm. The associated aneurysms were native in 8 patients and after previous intervention in 10 patients. Aneurysm shapes were fusiform in 8 patients and saccular in 10. The following characteristics defined complex stenosis as long diffuse stenosis, very tortuous coarctation, or stenosis involving a main branch or an unusual location. Patients previously stented at an early age, required re-expansion and/or restenting after reaching 16 ± 5 years of age.
Results Two patients had died by 1-month follow-up. The remaining 58 patients did well and were followed-up for a mean period of 10 ± 6 years. Late adverse events occurred in 3 patients (5%). All remaining patients are symptom-free, with normal baseline blood pressure. Imaging techniques revealed good patency at follow-up without associated aneurysm or restenosis. The actuarial survival free probability of all complex patients at 15 years was 92%.
Conclusions Stent repair of complex coarctation of aorta is feasible and safe. Initial results are maintained at later follow-up.
Stent repair of coarctation of aorta is an alternative to surgical correction (1,2). However, several anatomic or evolving characteristics of the coarctation throughout life may create challenging conditions for surgical and percutaneous treatment. The coarctation segment in previously treated patients may evolve over time and many of these patients require several interventions throughout their lifetime. The growth of the aorta in children with previously stented coarctation leads to slowly progressive recoarctation at the fixed stented segment. This may require re-expansion of the stent to accommodate this segment to aortic growth (3,4). However, scarce information is available on stent re-expansion. In other adult patients, not previously treated, the aortic segments may evolve over time suffering adverse hemodynamic conditions. Evolution of the entire aortic arch and collateral network may create very adverse conditions for treatment. However, the anatomy, length, and location of the coarctation segment are widely diverse and may involve associated aneurysms or main aortic arch trunks. Therefore, the complexity of certain coarctations may make therapeutic decisions difficult. This paper retrospectively analyzes our 21-year experience in the percutaneous treatment of complex coarctation of aorta.
We selected for study 60 patients considered to have a complex coarctation to treat. They all gave written informed consent; 46 were male and 14 female. Fourteen of them (23%) had associated malformations. Twenty-three (38%) had 1 or more previous interventions on the coarctation, surgical, percutaneous, or both. Twelve patients underwent 2 or more previous interventions on the coarctation. Thirty-seven patients (62%), mean age 26 ± 17 years, presented with a native coarctation of complex treatment. The adverse conditions for treatment were diverse and patients were divided into 4 groups: 1) complete interruption of the aortic arch (n = 11); 2) associated aneurysm (n = 18); 3) complex stenosis (n = 30); and 4) the need for re-expansion and/or restenting (n = 21). Twenty patients (33%) belonged to more than 1 group. All therapeutic procedures were performed under anesthesia and endotracheal intubation. The access site was percutaneous in 57 patients and subclavian in 3 patients. Two arterial and 1 venous pressure lines were obtained. Proximal and distal aortic pressures and pulmonary arterial pressure were monitored during the entire procedure. A final angiography and simultaneous pressure gradient determination were performed. Closure of the femoral puncture site was performed using a Prostar system in 39 patients. The patients were monitored after treatment for at least 24 h. A close follow-up schedule was established after discharge. Five patients had ulterior surgical procedures to treat associated aortic valve disease.
Complete interruption of the aortic arch
Figure 1 shows angiographic examples of this entity. The age ranged from 5 to 66 years; 3 were children and 8 were adults. All patients had severe hypertension and 7 presented with heart failure; 1 had atrial fibrillation. Three of them had associated aortic valve disease and 2 associated coronary artery disease. Following the classification of Celoria and Patton, 10 were type A and 1 was type B. The mean length of the interrupted aorta was 9 ± 11 mm. Distortion between proximal and distal aortas was present in 6 patients, whereas they were well aligned in 5. An extended network of collaterals was always present. Two patients had an associated native saccular aneurysm.
Recanalization of the occluded aorta was performed using a rigid coronary wire in 6 patients and a radiofrequency guidewire in 5 instances. Recanalization was reached antegradely in 4 patients and retrogradely in 7. Once into the opposed lumen, the guidewire was captured with a snare and kept fixed. This allowed crossing with a 3-mm diameter balloon catheter. After dilation, an angiographic catheter was interchanged and angiography was performed in the proximal aorta. Measurements were confirmed and a rigid guidewire was used to allow interchanging the angiographic catheter to a 12- to 14-F Cook cannula (Cook Medical, Bloomington, Indiana), by using a Dotter effect. Once the cannula was in the upper segment, a covered Cheatham (NuMED Canada Inc., Cornwall, Ontario, Canada) (n = 6) or nude stent (n = 5) was implanted.
Thirteen patients were treated 1 or more times at the coarctation surgically, percutaneously, or both. Computed tomography (CT) or angiographic studies revealed the anatomy of the related aneurysm. The aneurysms were native in 7 patients, post-surgery in 3 patients, and post–percutaneous intervention in 7 patients. The aneurysm was located at the coarctation level in 10 instances, at proximal aorta in 2 patients, at distal aorta in 3 patients, and at collaterals in 3 patients. The aneurysm shape was fusiform in 8 patients and saccular in 10. The mean maximal diameter of the aneurysm was 18 ± 8 mm. The clinical condition was stable with hypertension, and 11 patients had effort dyspnea.
The coarctation segments were analyzed and measured angiographically and the aneurysm was delineated in different projections. In 3 patients, the aneurysm was first covered with a stent and then the aneurysm was partially or totally occluded by filling the sac with coils through the struts (2); in 2 of them, a second stent was implanted to increase the mesh and fix the occluded sac. After the introduction of covered stents, all remaining patients were treated with a Cheatham covered stent. A custom-made expandable covered stent was designed according to the anatomy of the aorta in 3 patients.
The following characteristics defined complex stenosis: 1) a long diffuse stenosis (>45 mm) (n = 12); 2) a very tortuous coarctation that required an extremely acute remodeling for repair (n = 6); 3) a stenosis involving a main branch (n = 14); or 4) a coarctation of an unusual location (n = 8). Nine of these patients had stenosis with more than 1 complex characteristics. Eleven patients received previous interventions on the coarctation and presented for recoarctation. The clinical presentation was effort dyspnea in 20 patients and hypertension in all.
CT or angiographic measurements allowed for individual design of the stent covering. In patients with long diffuse stenosis, the mean stented length was 66 ± 16 mm requiring 1 to 3 stents to cover the long stenotic segment. One patient with a native long abdominal coarctation had an associated severe ostial stenosis of the right renal artery; a stent in the renal artery was implanted before stenting the coarctation. Patients with a tortuous coarctation always exhibited a post-stenotic dilation. The stent was implanted in increasing diameters to gradually perform the extremely acute remodeling of the aorta. The distal stent was always overexpanded to adapt the aortic wall. In 5 patients with an associated ductus arteriosus as a main branch, a covered stent treated both malformations by stenting the coarctation and closing the ductus. The branches to cover in the remaining 9 patients were the right renal artery in 1 patient, the left subclavian artery in 4 patients, the left carotid artery in 1 patients, and both arteries in 3 patients. The branches were protected with wires before conventional stent implantation in these patients. The branches were angiographically evaluated after implantation. Balloon post-dilation of the branch origin was required in 6 instances, and kissing balloon was performed in 2 patients. Eight patients had severe coarctation in an unusual location: at the transverse arch in 5 patients and at the abdominal aorta in 3 patients.
The need for re-expansion and/or restenting
Patients had been previously stented at an early age (mean 3 ± 3 years) as a palliative treatment to initially resolve a severe clinical condition and allow them to grow up without heart failure. The Palmaz stent diameter that was implanted at that time was 9 ± 1 mm. They all had a significant clinical improvement after the first stenting, which permitted normal growth in these children. Continuous follow-up of these patients monitored their aortic growth in relation to the fixed stented segment. Re-expansion was performed at a mean of 13 ± 4 years after first treatment. All these patients were asymptomatic and 10 exhibited hypertension. Three patients had an associated aneurysm.
Angiography at different projections always showed the anatomy of the aorta and the stented segment, which provided measurements and evaluation of fractures or recoils of the first stent before and after re-expansion. A new in-stent stent was implanted after re-expansion in 9 patients; 4 of these in-stent stents were covered. Re-expansion was performed in the remaining 12 patients using a balloon catheter of 18 ± 5 mm inflation diameter.
All patients were monitored and 58 were discharged 2 to 4 days after treatment. Follow-up studies included telephone calls, clinical evaluations on demand, and scheduled clinical and echocardiographic evaluations at 6 months, 1 year, and every subsequent year. A CT-scan follow-up was performed in all patients since 2008. Five patients required rehospitalization for the surgical treatment of aortic valve disease 5 ± 5 years after stent treatment. Acute and late major events were recorded.
Quantitative data are expressed as the mean ± SD. The paired t-test was used to compare 2 mean values. Survival curves were constructed using the Kaplan-Meier method.
Stent implantation and successful revascularization was always achieved. The peak gradient across coarctation decreased and the minimal lumen diameter increased significantly. Table 1 shows the baseline and immediate findings. One 52-year-old patient had a sudden cardiac death 3 h after the procedure. The patient underwent surgery during his youth, and he exhibited a long diffuse recoarctation. He received 3 overlapped Palmaz stents at treatment. One 53-year-old patient had a thalamic ictus following the procedure that had mostly recovered. No other major complications occurred. Four patients had an absent or decreased femoral pulse at the puncture site, without limb ischemia; 2 patients had hemorrhagic complications needing blood supply. Repair of the access site was uneventful in 3 patients where surgical access was performed through the right subclavian artery. Clinical outcome was favorable in 58 patients. Basal blood pressure became normal in all of them and those having symptoms of heart failure recovered after treatment. During follow-up, 43 patients did not need medical treatment to maintain baseline blood pressure under normal limits; 12 other patients were on beta-blockers and/or angiotensin-converting enzyme inhibitors.
Interruption of the aortic arch
Two patients with associated coronary disease were revascularized using stents in the same procedure. The interruption was always successfully recanalized and stented. The gradient between ascending and descending aortas after stenting decreased significantly. The covered stent completely excluded the aneurysm in 2 patients with a related aneurysm. The significant hemodynamic relief correlated with a marked clinical improvement in patients with previous heart failure except 1 patient who needed early aortic valve replacement. Figure 2 shows the recanalization process of a complete interruption of the aorta and the angiographic result after a covered stent implantation. Figure 3 shows the baseline angiographies and CT images before and after treatment. The patient with associated aortic valve disease and refractory heart failure died after aortic valve replacement 3 weeks after coarctation repair. After a mean follow-up of 3 ± 3 years, 2 patients had successful valve replacement 1 and 6 years after stent treatment, respectively. One 66-year-old patient died of a noncardiac cause 2 years after stent repair and another 63-year-old patient with surgical aortic valve replacement and coronary artery disease died suddenly 5 years after stent repair. The remaining 8 patients are alive and symptom-free without baseline hypertension.
The aneurysm was covered by a nude stent in 3 patients, and the aneurysmal sac was filled with coils through the struts to obliterate the aneurysm. Figure 4 shows an angiographic example of this type of obliteration. A second in-stent stent was implanted in 2 patients to increase the mesh and avoid coil embolization. The aneurysm was excluded with a covered stent in the remaining 15 patients. Figures 5 and 6 show the angiographies and CT images before and after treatment in 2 patients with associated native and post-surgical aneurysms, respectively. Covered custom designed stents were implanted in both patients. The gradient across the coarctation segment decreased significantly after treatment (Table 1). At follow-up, all 18 patients are alive without significant Doppler gradient across coarctation. CT imaging studies revealed that all aneurysms remained occluded at follow-up.
Figure 7 shows an angiographic example of a long-diffuse coarctation before and after treatment. A covered stent was implanted at the coarctation level in 5 patients with associated ductus arteriosus to completely occlude the ductus. Nine other patients had coarctation that was near a major branch partially covered by a provisional nude stent. Ulterior treatment of the branch was required in 7 patients. A stent at the renal artery was implanted before coarctation treatment. No angiographic flow limitations of the covered branch were observed following these procedures. The final stent diameter at the coarctation level was 17 ± 8 mm. All 30 patients are alive and symptom-free without baseline hypertension after a mean follow-up of 10 ± 8 years. Imaging techniques revealed adequate persistent scaffolding of the treated segment with patency of partially covered main branches (Figure 8) and persistent occlusion of associated ductus arteriosus.
The need for re-expansion and/or restenting
Three patients underwent coil obliteration of an associated aneurysm. The following changes in transcoarctation peak gradients were observed: at an early age: 53 ± 17 mm Hg; post-stent: 5 ± 4 mm Hg; at late follow-up: 46 ± 14 mm Hg; and post–re-expansion: 5 ± 5 mm Hg. Figure 4 shows an example of re-expansion and partial restenting. New angiography after expansion showed minor stent fractures in 7 patients and mild recoil in 2 patients. These anomalies were treated with a new in-stent stent implantation in 9 patients; 4 of them were covered. There were no complications associated with the balloon dilation of the stent and/or new stent implantation. No damage of the aorta was observed after treatment. At follow-up, adequate scaffolding persisted.
Patients were followed-up closely for a mean period of 10 ± 6 years. Associated late surgery of the aortic valve was needed in 5 patients, at a mean follow-up time of 5 ± 5 years. Evaluations continue in 4 other patients with mild aortic valve disease. Late mortality occurred in 3 patients (5%). The remaining 55 patients were symptom-free with normal baseline blood pressure. The last limb Doppler gradient was 4 ± 5 mm Hg. Imaging techniques revealed a good patency of the aorta without associated aneurysms or restenosis at late follow-up. The latest CT study was performed at a mean of 6 ± 8 years after intervention. Figure 9 shows the actuarial survival probability that was observed in this series of patients together with that from noncomplex coarctation repaired with stents at our institution in the same study period.
Repair of coarctation of aorta has evolved significantly in the past 70 years and the outcome and late results have improved. Stent repair has been an alternative to surgical correction since 1995 (1) but both treatments may be combined throughout life. Many factors influence conditions for treatment. The age at presentation, the wide diversity in coarctation anatomy and location, and evolution of the aortic segments and collaterals over time may be important determinants for the selection of the best treatment for each patient at a given moment of their life. Indeed, many patients require several interventions at the coarctation throughout their lifetime. Therefore, the repair may be a simple, single stage procedure or may present very complex conditions for management depending on numerous factors. The complexity for treatment may be present at birth and continues throughout life or it may appear as an evolving anatomical and/or pathophysiological feature that leads to a difficult management. This retrospective 21-year study analyzed the immediate and long-term results of stent repair in patients with complex coarctation of aorta.
Interruption of the aortic arch
This uncommon congenital anomaly, with frequent associated cardiac anomalies, is well known, and it usually presents as a complicated neonatal surgical emergency, because the median age at death in untreated neonates is 10 days. Surgery demonstrated excellent results using a variety of individualized techniques for repair (5). However, aortic interruption may also be present in young children, adolescents, adults, and elderly patients. Survival in these patients depends on a dense collateral network. Several references in the published data are case reports of a diagnostic curiosity in adulthood (6,7). Whether the interruption is congenital or acquired during life from a critical coarctation is not well known and both pathogeneses are possible. The treatment of this entity is extremely difficult, and it has not been completely established because there is lack of data in treated patients. Interruption length may vary from millimeters to >1 cm. Both interrupted aortic segments may be well aligned or completely distorted, which adds additional complexity for recanalization and stent treatment. Collateral flow may provide complex networks around the coarctation segment, with associated saccular aneurysms in some instances. Surgical case reports prefer the performance of an extra-anatomical bypass between ascending and descending aortas (8) to avoid approaching the site of interruption. Case reports of percutaneous recanalization and stent repair were also published (9,10), but there is little information on the long-term results of either surgical or percutaneous treatments.
This study presents a series of patients with interruption of the aortic arch who were treated with stent repair and followed-up for a mean period of 3 years. Delineation of the anatomy using imaging techniques is mandatory before treatment decisions and these techniques provide an adequate planning approach. Antegrade or retrograde guidewire recanalization allows for guidewire capture in the opposed lumen segment, which becomes fixed for balloon crossing and cannulation of the interrupted segment. The stent repair is an easy and safe procedure that reestablished the flow with no significant gradient across. We implanted nude metallic stents in our initial experience, but we strongly recommend the implantation of covered stents to prevent possible wall damage and exclude aneurysms. The initial good results are maintained at follow-up, without restenosis or evidence of aortic wall damage.
Aortic aneurysm formation complicates aortic coarctation, and it carries a risk of rupture with a high mortality rate. Coarctation of the aorta continues to have a high risk for aneurysmal formation late after treatment, despite advanced techniques for surgical or percutaneous treatment. Native aneurysms may also be associated with severe coarctations. The frequency, anatomical types, location, risk factors, and mechanisms of aortic aneurysms late after repair were reviewed (11). The presence of cystic necrosis of the media in resected coarctation segments (12) may explain this association in native or treated coarctations. A bicuspid aortic valve and the inherent weakness of the aortic wall are risk factors for aneurysm formation. Therefore, continuous monitoring of patients with treated or untreated coarctations appears mandatory despite the absence of symptoms because aneurysms may grow over time. The size and location of the aneurysm are important issues for treatment. Open, hybrid, and endovascular techniques may be used for repair (13). Percutaneous management has evolved over time and this treatment appears to be an effective and safe procedure to combine coarctation repair and exclusion of the aneurysm. Coil and microcoil embolization and an Amplatzer vascular plug were successfully used (2,14). However, different studies demonstrated that the deployment of covered stents may be a safe and definitive therapeutic option, without endoleaks and other complications (15,16).
We started to obliterate small aneurysms using coils (2) (Figure 4). However, this treatment is limited to small aneurysms, and it requires stent jailing of the deployed coils. Since the introduction of covered stents, we only used this device to combine coarctation treatment and aneurysm obliteration. We may use commercially available covered stents or demand a custom-tailored covered stent for certain patients depending on the anatomy (Figures 4 and 5). All patients had successful immediate exclusion of the aneurysm, which remain obliterated at long-term follow-up. Therefore, our results confirm the findings of other studies and suggest that e-poly-tetra-fluoro-ethylene-covered Cheatham-platinum stents provide a safe and promising option for the treatment of coarctation of aorta with associated aneurysm.
Long-segment coarctation of aorta is infrequent, but it may complicate treatment, primarily when it affects the main branches (17,18). Stent implantation was attempted in long-segment thoracic coarctation (18) and the use of more than 1 stent should always be considered. A hypoplastic aortic arch and coarctation involving the trunks also represent challenging conditions for treatment. Surgical (17) or percutaneous (19) treatments have been described. Open-cell bare-metal stents are chosen when there is a likelihood of jailing a neck trunk, which normally preserves the flow through the branches. However, provisional stent techniques with or without a final kissing balloon should be used to guarantee open access to the covered branches (Figure 8). In contrast, implantation of a covered stent is a good option in patients with associated ductus arteriosus. An adequate repair was always obtained. Therefore, complex stenosis at the coarctation level can be repaired using stents to provide good initial and late results.
The need for re-expansion and/or restenting
Stents implantation in severe coarctations at an early age is controversial, but it seems effective and safe in the short term (1,20). The aortic segments grow with age despite a fixed stented segment. This growth may require re-expansion of the stent to accommodate the segment to normal aortic growth. However, scarce information on stent re-expansion of coarcted segments because of growth is available. Morrow et al. (3) demonstrated the feasibility of re-expanding implanted stents in normal juvenile swine after growth in a short-term follow-up, without causing significant injury to the arterial layers. More recently, Zanjani et al. (4) reported the feasibility and efficacy of stent re-expansion in 22 patients with aortic coarctation. The intervals between stent implantation and redilation ranged between 2.5 months to 11 years in this study. Pressure gradients always decreased and stent diameters always increased, without reports of detectable damage to the aortic wall. No other information on stent re-expansion or restenting after growth is available in the published data. There is also no information on the late follow-up of patients with re-expanded stents. The mean time from initial stent implantation to re-expansion in our study, was 13 years. No luminal damage or stent deformation was observed after re-expansion or restenting, and the repair was adequate (Figure 4). This experience is the longest follow-up showing good evolution after stent re-expansion or restenting.
Stent repair of several types of complex coarctation of aorta is a safe and feasible management strategy. This 21-year percutaneous experience demonstrates that good initial results are maintained at late follow-up.
WHAT IS KNOWN? Stent repair of coarctation of aorta is an alternative to surgical correction. However, several anatomic or evolving characteristics of the coarctation throughout life may create challenging conditions for both surgical and percutaneous treatment.
WHAT IS NEW? This retrospective 21-year study analyzed the immediate and long-term results of stent repair in patients with different types of complex coarctation of aorta (interruption of the aortic arch, associated aneurysm, complex stenosis, and stent re-expansion). Stent repair in these complex patients was a safe and feasible management strategy.
WHAT IS NEXT? This percutaneous experience demonstrates that good initial results are maintained at late follow-up.
Dr. Pan has received lecture fees from Abbott. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Abbreviations and Acronyms
- computed tomography
- Received February 11, 2015.
- Revision received April 13, 2015.
- Accepted May 7, 2015.
- 2015 American College of Cardiology Foundation
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