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Original Article
Late complication after repair of aortic coarctation
Asian Cardiovascular & Thoracic Annals 0(0) 1–7 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314557872 aan.sagepub.com
Anaı¨s Lemaire1, Fabio Cuttone1, Julien Desgue´1, Calin Ivascau1, Sabino Caprio1, Vladimir Saplacan1, Annette Belin2 and Ge´rard Babatasi1
Abstract Background: Coarctation of the aorta is a congenital malformation that has long been considered completely correctable with appropriate surgery in childhood. However, with the aging of these patients, many late complications have been reported, and this notion must be reevaluated. Methods: We retrospectively reviewed all patients who underwent reoperation between 1992 and 2012 in our adult cardiac surgery department following surgical correction of coarctation in childhood; 18 patients over 15-years old were included in the study. Results: The median time from coarctation repair to reoperation was 25 years. Patients were reoperated on for several late complications: aortic valve disease secondary to bicuspid aortic valve, ascending aortic aneurysm, recoarctation, aortic arch hypoplasia, pseudoaneurysm, associated recoarctation and pseudoaneurysm, subvalvular aortic obstruction, and descending thoracic aortic aneurysm. One patient died due to an intraoperative complication. In the other cases, the surgical results were satisfactory at the 6-month follow-up. According to literature data, age at coarctation repair and surgical technique appear to be essential factors in late complications: older age and surgical repair with prosthesis interposition are associated with a higher rate of reintervention. Conclusion: Patients who have undergone repair of aortic coarctation frequently remain asymptomatic for a long time. Late complications can be appropriately treated when diagnosed early. Consequently, all coarctation patients need careful lifelong follow-up, especially those with congenital aortic valve disease or surgery in childhood with interposition of prosthetic material.
Keywords Aortic aneurysm, Aortic coarctation, Aortic valve, Blood vessel prosthesis implantation, Vascular surgical procedures
Introduction Coarctation of the aorta accounts for approximately 5% of all congenital heart malformations,1 with an incidence of 0.2 to 0.6 per 1000 live births.2 This anomaly was first described by Johann Freidrich Meckel in 1750. The first successful surgical correction was made by Clarence Crafoord in 1944 by resection and endto-end anastomosis.3 The surgical repair technique quickly spread worldwide and was advanced by the introduction of diverse techniques such as subclavian flap aortoplasty.4 Hence aortic coarctation was considered completely correctable after appropriate surgery in childhood. With the aging of patients operated on in childhood, this notion has now been
questioned following the emergence of many late complications, and it must be reevaluated. To address these issues, we retrospectively studied patients referred to our adult cardiac surgery department with late surgical complications after aortic coarctation repair. 1 Department of Cardiac Surgery, University of Caen Basse-Normandie and University Hospital of Caen, France 2 Department of Cardiology, University of Caen Basse-Normandie and University Hospital of Caen, France
Corresponding author: Anaı¨s Lemaire, MD, Cardiac Surgery Department, University Hospital of Caen, Avenue de la Coˆte de Nacre, 14033 Caen Cedex 9, France. Email: [email protected]
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Patients and methods We performed a retrospective review of all patients referred to the University Hospital of Caen, France, with adult congenital heart disease requiring surgical intervention following aortic coarctation repair. Patients’ data were taken from our department’s database and medical records from January 1, 1992 to December 31, 2012. Inclusion criteria were patients over 15-years old and previously operated on for aortic coarctation (in our center or another) who underwent reoperation in our department. Because the association of bicuspid aortic valve with aortic coarctation is well described (with a prevalence of 25%–85%),5 we included patients referred to our center for complications either directly related to the coarctation or secondary to associated bicuspid aortic valve. Eighteen patients met these criteria and were included in our study (Table 1). The median age at the first operation for coarctation repair was 8 years (range 22 days to 55 years). Several surgical techniques were used in the first operation; in 4 cases, the technique was not described in detail. Thirteen (72.2%) patients had associated cardiac anomalies (Table 1). These 18 patients were referred for reoperation for
various reasons (Table 1). The median time between aortic coarctation repair and reintervention was 25 years (range 5–43 years; Table 2).
Results Among the 12 patients with bicuspid aortic valve, 6 developed significant aortic valve disease requiring surgery: 3 had isolated aortic valve disease, 2 had associated significant coronary lesions, and 1 had an ascending aortic aneurysm. In 4 of these patients, the aortic valvulopathy consisted of bicuspid valve stenosis only, and the other 2 had a combination of stenosis and regurgitation. Surgery on the aortic valve was performed at a median age of 47.5 years after follow-up of 31.5 years after coarctation repair. The echocardiographic mean pressure gradient across the aortic valve was 49 mm Hg (range 30–60 mm Hg) and the maximal pressure gradient was 68 mm Hg (range 50–80 mm Hg). In the 2 cases of regurgitation, aortic valve insufficiency was grade 2/4. All 6 patients were operated on via a sternotomy with standard cardiopulmonary bypass (CPB). Aortic valve replacement was performed in all cases; 5 had a mechanical prosthesis and one had a
Table 1. Data of 12 patients who required reintervention after repair of aortic coarctation. Technique of Patient no. Age at repair coarctation repair
Associated cardiac anomaly
Late complications requiring reoperation
1 2
15 years 1 year
IVC Bicuspid aortic valve
Recoarctation Recoarctation
3 4 5 6 7
6 years 3.5 months 10 years 14 years 2.5 years
Prosthesis interposition Crafoord þ prosthesis interposition at 13 years Crafoord Waldhausen Prosthesis interposition Prosthesis interposition Waldhausen
8 9 10 11 12 13 14 15 16
23 years 3 months 5 years 12 years 24 years 20 years 4 years 16 years 1 month
Isthmus patch plasty Crafoord Crafoord ND Crafoord ND ND ND Waldhausen
17 18
22 days 55 years
Crafoord Ascending aorta-to-coeliac aorta prosthesis interposition
Bicuspid aortic valve
Bicuspid aortic valve, subvalvular aortic stenosis, arteria lusoria Left inferior vena cava Bicuspid aortic valve Bicuspid aortic valve, arteria lusoria Bicuspid aortic valve Bicuspid aortic valve Subvalvular aortic valve stenosis Bicuspid aortic valve Bicuspid aortic valve, subvalvular aortic valve stenosis Bicuspid aortic valve Bicuspid aortic valve
Hypoplastic aortic arch Hypoplastic aortic arch Recoarctation Recoarctation þ pseudoaneurysm Ascending aortic aneurysm Isthmic pseudoaneurysm Ascending aortic aneurysm Ascending aortic aneurysm Ascending aortic aneurysm þ AVS AVS þ coronaropathy AVS þ coronaropathy Subvalvular aortic obstruction AVS þ AVI AVS þ AVI AVS Descending thoracic aortic aneurysm
AVI: aortic valve insufficiency; AVS: aortic valve stenosis; IVC: interventricular communication; ND: not documented.
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Table 2. Time between aortic coarctation repair and reoperation, according to late surgical complication.
Late complication Isolated bicuspid aortic valve disease Ascending aortic aneurysm Recoarctation Aortic arch hypoplasia Pseudoaneurysm Recoarctation þ pseudoaneurysm Subvalvular aortic obstruction Descending aortic aneurysm
Median coarctation repair-to-reoperation Incidence time (years) [range] 5
35 [18–43]
4 3 2 1 1
24 [22–27] 27 [24–38] 13 [9–17] 29 27
1 1
20 5
bioprosthesis (at 56 years of age) due to severe uncontrolled hypertension involving a significant risk of hypertensive intracranial hemorrhage with anticoagulation therapy. At the 6-month follow-up, 5 patients were doing well but one developed prosthetic valve endocarditis 6 months after implantation, necessitating a third intervention. Pathology of the ascending or horizontal aorta was found in 8 (44.4%) patients: 4 had ascending aortic aneurysms, 2 had pseudoaneurysms, and 2 had a hypoplastic aortic arch. The median age at reintervention was 27 years (range 15–52 years): 27 years for ascending aortic aneurysm, 46 years for pseudoaneurysm, and 1 6 years for aortic arch hypoplasia. The 4 patients who underwent surgery for ascending aortic aneurysm had associated bicuspid aortic valves. Their clinical presentation was mainly the onset or worsening of dyspnea (New York Heart Association class II/III) and asthenia; 2 presented with chest pain (one acute and miming symptoms of aortic dissection, the other chronic and associated with abdominal pain). All 4 patients had transthoracic echocardiography, 3 had computed tomography, and one had aortic angiography. The maximal diameter of the aneurysm was 59 mm (range 40–85 mm). Aortic dilatation included the coronary sinus in all cases and extended to the aortic arch in one. Surgery was performed in all 4 cases by median sternotomy, under standard central CPB in 3, and femorofemoral CPB in one. Two patients underwent a Bentall procedure and the other 2 had ectasia resection and direct end-to-end aortic anastomosis after mobilization of both the aortic arch and the heart (one had additional mechanical aortic valve replacement). The median CPB time was 207 min (range 127–322 min); 280 min for the Bentall technique and 133 min for resection-anastomosis. The median aortic crossclamp time was 118 min (range 85–
156 min); 150 min for the Bentall operation and 86 min for resection-anastomosis. The patient operated on with femorofemoral CPB suffered a right ventricle wound during the sternotomy, which resulted air emboli and diffuse brain damage in the early postoperative period. The patient remained in a chronic neurovegetative state and died on the 43rd postoperative day. In the other 3 cases, the postoperative course was uneventful with a median hospital stay of 11 days (range 6–17 days). Two patients were treated for pseudoaneurysm development after aortic isthmus plasty using a Dacron interposition graft for coarctation repair at the ages of 14 and 23 years. Reintervention occurred at 27 and 28 years after the first operation. Both patients presented with chest pain and class II dyspnea. Evaluation included transthoracic echocardiography and magnetic resonance imaging, with aortography in one case and transesophageal echocardiography and computed tomography in the other. Both aortic pseudoaneurysms (diameters 32 and 60 mm) developed at the distal site of Dacron patch implantation. Intraoperatively, ectasia associated with avulsion of the isthmus prosthesis was found. Resection of the aneurysm and prosthesis was followed by isthmic reconstruction by direct endto-end anastomosis in one case and by interposition of a 20-mm tubular Hemashield prosthesis in the other. The postoperative courses were marked only by persistent well-controlled hypertension. In our 2 cases of aortic arch hypoplasia, coarctation repair had been performed before the age of 6 months by resection and end-to-end anastomosis in one and Waldhausen aortoplasty in the other. Reintervention occurred after 9 and 17 years. Clinical presentations were typical with blood pressure asymmetry, hypertension in the right upper limb, loss of femoral pulses, and anisosphygmia. Transthoracic echocardiography and aortic angiography revealed excellent development of the isthmic area but major hypoplasia of the horizontal aorta (diameters between the 2 carotids were 7 and 10 mm) with systolic flow acceleration (3.5 m s1). Central aortic arch plasty with a left subclavian flap was performed in one case via an iterative posterolateral left anterior thoracotomy without CPB; resection of the atretic area and extended end-to-end anastomosis was carried out under CPB and circulatory arrest via a median sternotomy in the other. The measured diameters of the aortic arch after repair were 25 and 17 mm. The postoperative periods were event-free, and hospital stay was 10 and 11 days. Clinical and echographic examinations after 2 months were satisfactory except for residual hypertension, well-controlled on drug-therapy. Four patients were reoperated for recoarctation, including one with an associated pseudoaneurysm. The initial coarctations had been repaired using Dacron prostheses (diameters 10, 11, 12 and 16 mm).
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The median age at first intervention was 12 years (range 1–15 years) and the median age at reoperation was 40 years (range 34–41 years). Diagnosis was based on typical symptoms such as labile hypertension resistant to medical treatment, blood pressure discrepancy between the two upper limbs, asthenia, dyspnea, effort paresthesia, and loss of strength in the left upper and lower limbs. Evaluation included Doppler and aortic angiography, with computed tomography in 2 cases and magnetic resonance-angiography in one. The average pressure gradient across the stenosis was 46.3 mm Hg (range 25–64 mm Hg). In 2 cases, resection of the prosthetic tube and terminoterminal implantation of a larger isthmic prosthesis (18 and 20 mm) was performed under CPB with aortic crossclamping (22 and 54 min). One case was treated by extraanatomic bypass with a 16-mm prosthetic tube between the left subclavian artery and the descending thoracic aorta. The 4th patient underwent a bypass between the ascending and coeliac aorta (Figure 1) by interposing a 16-mm vascular prosthesis via a median sternotomy; the other 3 has an iterative left posterolateral thoracotomy. Postoperative evolutions were satisfying with complete regression of symptoms and good echographic results at the 6-month follow-up. Subvalvular aortic obstruction was found in one patient who was operated on at the age of 4 years, but the surgical technique and associated anatomy were not recorded. Twenty years later, he had no clinical symptoms and was participating in competitive
sport. A routine cardiology assessment revealed a subvalvular fibrous diaphragm with left ventricular hypertrophy and moderate aortic valve insufficiency. Surgery via a ministernotomy revealed left ventricular outflow tract obstruction due to a fibrous membrane, and a normal tricuspid aortic valve. The membrane was resected via a transaortic approach and the postoperative course was uneventful. One patient presented with a complex history: late diagnosis of aortic coarctation, aortic arch hypoplasia, and bicuspid aortic valve at the age of 23 years (incidentally discovered during a medical examination for military repatriation). At the time, he refused surgery but then developed progressively increasing poststenotic aortic ectasia. At the age of 66 years, a large thoracic descending aortic aneurysm was discovered due to dysphonia related to compressive left recurrent paralysis. The aneurysm and the coarctation area were excluded by prosthesis interposition between the ascending aorta and the coeliac aorta, with the expectation of gradual aneurysm thrombosis. However, the postoperative course was marked by continuing expansion of the aneurysm, causing tracheoesophageal compression with respiratory failure 4 years later (Figure 2). He underwent aneurysm ligation and exclusion, and was discharged from the hospital without any complication. Another specific problem of note in our series was premature coronary artery disease; patients with coarctation history often develop important arterial collaterals from the internal mammary arteries (IMA). Two of the patients with bicuspid aortic valve stenosis also presented with coronary lesions; both had undergone surgery for well-tolerated coarctation at the age of 20 and 24 years and were reoperated on 36 and 43 years later. One had a single
Figure 1. Postoperative computed tomography with 3-dimensional reconstruction in the patient operated on by extraanatomic bypass between the ascending aorta and coeliac aorta for isthmic recoarctation (ventral aorta). The coarctation area is indicated by the white arrow.
Figure 2. Chest radiograph 4 years after the first intervention (ventral aorta) following the appearance of respiratory insufficiency. The aortic aneurysm had greatly increased size (white arrow) and caused significant compression of the upper airways, with major tracheal deviation (dotted arrow).
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lesion in the proximal left anterior descending artery (LAD), and the other presented with 2 lesions and significant stenosis LAD. Because of the late coarctation repair, both patients had developed an important arterial collateral network, particularly at the expense of the IMA, which were very dilated and unusable (Figure 3). In the first case, the LAD stenosis was moderate and the patient was asymptomatic, so we decided not carry out coronary artery bypass and consider angiography later, if necessary. The second patient presented with clinical symptoms and severe stenosis of the LAD; the left IMA was extremely dilated (mean diameter 8 mm) and therefore unusable. A saphenous graft was used for coronary revascularization. Pathologic examination of the IMA revealed concentric partially calcified atheroma.
Discussion Adults operated on in childhood for congenital heart diseases now confront cardiac surgeons with a new category of patient with specific conditions. Due to the excellent results of surgical treatment for aortic coarctation, it was believed that these patients were completely cured after a successful intervention, but we now see the onset of late complications in adulthood. A significant percentage of these patients present with various complications either directly related to the
Figure 3. Computed tomography-angiography with 3-dimensional reconstruction in an adult operated on in late childhood for aortic coarctation. Note the very dilated internal mammary artery, consequent to the development of an important collateral arterial network.
coarctation and previous surgery (recoarctation, aneurysm or pseudoaneurysm, bacterial infection),6 or to associated pathologies (particularly bicuspid aortic valve disease). Approximately 11% of patients operated on for aortic coarctation require reoperation during follow-up.7 According to the literature, the surgical technique in the first intervention is an important factor in the subsequent evolution. Interposition of prosthetic material is associated with a substantial risk of recoarctation because the prosthetic tube gradually becomes stenosed with growth of the child, giving rise to typical symptoms of coarctation with a significant transprosthetic gradient. In our series, the mean transprosthetic gradient was 46.3 mm Hg; the diagnosis of aortic recoarctation is based on a mean gradient > 20 mm Hg. Prosthesis interposition is also associated with an increased rate of aortic aneurysms and pseudoaneurysms (5%–10% of all patients undergoing aortic coarctation repair, and 38% of patients with prosthesis interposition)8–10 which arise opposite the patch suture line or sometimes due to degeneration of the Dacron patch fibers.11 These aneurysms may remain asymptomatic for a long time and can be brutally revealed by aortic rupture or dissection, especially in patients with persistent hypertension.12 Our findings are in agreement with the literature data because all of our patients with aortic recoarctation or pseudoaneurysm had undergone prosthesis interposition during coarctation repair (Figure 4). These results confirm the current strategy of avoiding the use of prosthetic material, if possible. Ascending or horizontal aortic pathology was the reason for reoperation in 44.4% of cases in our series, and in agreement with the literature, a strong association between ascending aortic dilatation and bicuspid aortic valve was seen: all patients who were operated on for ascending aorta aneurysm had a bicuspid aortic valve. Once diagnosed, post-coarctectomy aortic aneurysms should be handled surgically. There is only one report of conservative treatment of these lesions; it recorded a 100% failure rate at 15 years.13 Management of these redo patients can be a challenge for cardiac surgeons because of the complexity of the anatomical lesions and postoperative adhesions due to the primary procedure. Given these complex conditions, we must use numerous preventive techniques to secure the intervention, such as femorofemoral cannulation prior to sternotomy and hypothermic circulatory arrest if necessary. Occasionally, the implementation of these preventive strategies may be insufficient and the consequences can be dramatic, as illustrated by one of our patients who suffered a cerebral embolism during the intervention, responsible for irreversible neurological damage and death. An attractive and promising alternative to major surgery is endovascular stent
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Figure 4. Relationship between the first surgical intervention and the late surgical complication. All cases in our series referred for aortic recoarctation or pseudoaneurysm underwent prosthesis interposition (Dacron patch or tube interposition).
implantation, but the indications are limited depending on the anatomical features.14 Surgery remains the treatment of choice in many cases. Bicuspid aortic valve was found in 66.7% of our patients, which is consistent with the literature data.15,16 The reason for this association is not clearly understood,17 but these aortic valve anomalies are responsible for significant morbidity and mortality,12 with the appearance of ‘‘bicuspid aortic valve syndrome’’ (both aortic valve stenosis and insufficiency). The majority of patients with bicuspid aortic valve develop abnormal valve calcifications before the age of 20 years, and almost all before 30-years old. The resulting aortic stenosis progresses rapidly (average gradient increase of 27 mm Hg per decade).12 Comparative studies showed a higher incidence of significant aortic valve disease in cases of both aortic coarctation and bicuspid aortic valve compared to isolated bicuspid valve. Thus coarctation seems to have an additional role in the genesis of symptomatic bicuspid aortic valve pathology.18 Besides the early aortic valve disease in these patients (in our series, mean age at surgery for aortic valve disease was 40 years), we are confronted with strategic issues during surgery, such as the choice of prosthetic valve when replacement is necessary. Indeed, we need to balance the risks and benefits of each prosthesis type. Another specific problem that appeared in our series was surgical treatment of coronary artery disease, sparsely described in the literature. It is widely known that patients with aortic coarctation tend to develop early coronary artery disease, although the mechanism is still unknown.14,16,18 Coronary disease appears to be the leading cause of late death after coarctation repair,19 responsible for 37% of deaths in these patients.7 Lawrie and colleagues20 first drew attention to premature coronary artery disease in patients with aortic coarctation,
noting that two-thirds of late deaths were attributed to acute myocardial ischemic events. Subsequent studies have confirmed this association and highlighted the development of severe atherosclerotic lesions in young patients with aortic coarctation.7 However, few studies have focused on the management of this pathology, which cannot be based on the general population, especially in cases of late coarctation repair. Coarctation repaired in adulthood induces the development of an extensive network of collaterals between the proximal and distal aorta, particularly via the IMA. The IMA are often extremely dilated and therefore unusable for coronary artery bypass grafting. In our series, we were faced to this problem twice. The treatment strategy must be discussed case by case to choose the best option for each patient. This particular factor during follow-up and monitoring of patients operated on for aortic coarctation deserves further studies in larger series with long-term results. Long-term results after aortic coarctation repair are overall satisfying, with 82% of patients asymptomatic after 20 years of follow-up.14 However, some patients present with long-term cardiovascular complications, possibly causing early death. In addition to the medical problem of late uncontrolled hypertension, these patients may develop specific complications requiring technically challenging surgical treatment. Late complications frequently remain asymptomatic for a long time, but can be treated appropriately when diagnosed early. Because of the various complications that can occur late or very late during follow-up, coarctation of the aorta, even appropriately repaired during childhood, is a condition that requires careful lifelong attention and follow-up, especially in patients with congenital aortic valve disease and those who have undergone coarctation repair with prosthetic material interposition.
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Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Jenkins NP and Ward C. Coarctation of the aorta: natural history and outcome after surgical treatment. QJM 1999; 92: 365–371. 2. Sudhayakumar N, Nair B and Satpathy M. Coarctation of the aorta. In: Satpathy M (ed.) Clinical Diagnosis of Congenital Heart Disease. New Delhi: Jaypee Brothers, 2008, pp. 160–169. 3. Crafoord C and Nylin F. Congenital coarctation of aorta and its surgical treatment. J Thorac Surg 1945; 14: 347–361. 4. Waldhausen JA and Nahrwold DL. Repair of coarctation of the aorta with a subclavian flap. J Thorac Cardiovasc Surg 1966; 51: 512–513. 5. Tawes RL Jr, Berry CL and Aberdeen E. Congenital bicuspid aortic valves associated with coarctation of the aorta in children. Br Heart J 1969; 31: 127–128. 6. Svensson LG. Congenital anomalies in adult. In: Coselli JS, LeMaire SA (eds) Aortic Arch Surgery: Principles, Strategies and Outcomes. Chichester, UK: WileyBlackwell, 2008, pp. 249–257. 7. Cohen M, Fuster V, Steele PM, Driscoll D and McGoon DC. Coarctation of the aorta. Long-term follow-up and prediction of outcome after surgical correction. Circulation 1989; 80: 840–845. 8. von Kodolitsch Y, Aydin MA, Koschyk DH, et al. Predictors of aneurysmal formation after surgical correction of aortic coarctation. J Am Coll Cardiol 2002; 39: 617–624. 9. Cramer JW, Ginde S, Bartz PJ, Tweddell JS, Litwin SB and Earing MG. Aortic aneurysms remain a significant source of morbidity and mortality after use of Dacron(Õ ) patch aortoplasty to repair coarctation of the aorta: results from a single center. Pediatr Cardiol 2013; 34: 296–301.
10. Walhout RJ, Braam RL, Schepens MA, Mulder BJ and Plokker HW. Aortic aneurysm formation following coarctation repair by Dacron patch aortoplasty. Neth Heart J 2010; 18: 376–377. 11. Chiesa R, Melissano G, Civilini E, Bertoglio L, Setacci F and Baccellieri D. Giant aneurysm 25 years after patch aortoplasty for aortic coarctation. Tex Heart Inst J 2008; 35: 220–221. 12. Roos-Hesselink JW, Scho¨lzel BE, Heijdra RJ, et al. Aortic valve and aortic arch pathology after coarctation repair. Heart 2003; 89: 1074–1077. 13. Knyshov GV, Sitar LL, Glagola MD and Atamanyuk MY. Aortic aneurysms at the site of the repair of coarctation of the aorta: a review of 48 patients. Ann Thorac Surg 1996; 61: 935–939. 14. Ho¨rmann M, Pavlidis D, Brunkwall J and Gawenda M. Long-term results of endovascular aortic repair for thoracic pseudoaneurysms after previous surgical coarctation repair. Interact Cardiovasc Thorac Surg 2011; 13: 401–404. 15. Tawes RL, Berry CL and Aberdeen E. Congenital bicuspid aortic valves associated with coarctation of the aorta in children. Br Heart J 1969; 31: 127–128. 16. Becker AE, Becker MJ and Edwards JE. Anomalies associated with coarctation of the aorta: particular reference to infancy. Circulation 1970; 41: 1067–1075. 17. Netter FH. The Ciba collection of medical illustrations. Heart 1969; 5: 163. 18. Ward C. Clinical significance of the bicuspid aortic valve. Heart 2000; 83: 81–85. 19. Toro-Salazar OH, Steinberger J, Thomas W, Rocchini AP, Carpenter B and Moller JH. Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol 2002; 89: 541–547. 20. Lawrie GM, DeBakey ME, Morris GC Jr, Crawford ES, Wagner WF and Glaeser DH. Late repair of coarctation of the descending thoracic aorta in 190 patients. Results up to 30 years after operation. Arch Surg 1981; 116: 1557–15560.
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Original Article
Late complication after repair of aortic coarctation
Asian Cardiovascular & Thoracic Annals 0(0) 1–7 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314557872 aan.sagepub.com
Anaı¨s Lemaire1, Fabio Cuttone1, Julien Desgue´1, Calin Ivascau1, Sabino Caprio1, Vladimir Saplacan1, Annette Belin2 and Ge´rard Babatasi1
Abstract Background: Coarctation of the aorta is a congenital malformation that has long been considered completely correctable with appropriate surgery in childhood. However, with the aging of these patients, many late complications have been reported, and this notion must be reevaluated. Methods: We retrospectively reviewed all patients who underwent reoperation between 1992 and 2012 in our adult cardiac surgery department following surgical correction of coarctation in childhood; 18 patients over 15-years old were included in the study. Results: The median time from coarctation repair to reoperation was 25 years. Patients were reoperated on for several late complications: aortic valve disease secondary to bicuspid aortic valve, ascending aortic aneurysm, recoarctation, aortic arch hypoplasia, pseudoaneurysm, associated recoarctation and pseudoaneurysm, subvalvular aortic obstruction, and descending thoracic aortic aneurysm. One patient died due to an intraoperative complication. In the other cases, the surgical results were satisfactory at the 6-month follow-up. According to literature data, age at coarctation repair and surgical technique appear to be essential factors in late complications: older age and surgical repair with prosthesis interposition are associated with a higher rate of reintervention. Conclusion: Patients who have undergone repair of aortic coarctation frequently remain asymptomatic for a long time. Late complications can be appropriately treated when diagnosed early. Consequently, all coarctation patients need careful lifelong follow-up, especially those with congenital aortic valve disease or surgery in childhood with interposition of prosthetic material.
Keywords Aortic aneurysm, Aortic coarctation, Aortic valve, Blood vessel prosthesis implantation, Vascular surgical procedures
Introduction Coarctation of the aorta accounts for approximately 5% of all congenital heart malformations,1 with an incidence of 0.2 to 0.6 per 1000 live births.2 This anomaly was first described by Johann Freidrich Meckel in 1750. The first successful surgical correction was made by Clarence Crafoord in 1944 by resection and endto-end anastomosis.3 The surgical repair technique quickly spread worldwide and was advanced by the introduction of diverse techniques such as subclavian flap aortoplasty.4 Hence aortic coarctation was considered completely correctable after appropriate surgery in childhood. With the aging of patients operated on in childhood, this notion has now been
questioned following the emergence of many late complications, and it must be reevaluated. To address these issues, we retrospectively studied patients referred to our adult cardiac surgery department with late surgical complications after aortic coarctation repair. 1 Department of Cardiac Surgery, University of Caen Basse-Normandie and University Hospital of Caen, France 2 Department of Cardiology, University of Caen Basse-Normandie and University Hospital of Caen, France
Corresponding author: Anaı¨s Lemaire, MD, Cardiac Surgery Department, University Hospital of Caen, Avenue de la Coˆte de Nacre, 14033 Caen Cedex 9, France. Email: [email protected]
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Patients and methods We performed a retrospective review of all patients referred to the University Hospital of Caen, France, with adult congenital heart disease requiring surgical intervention following aortic coarctation repair. Patients’ data were taken from our department’s database and medical records from January 1, 1992 to December 31, 2012. Inclusion criteria were patients over 15-years old and previously operated on for aortic coarctation (in our center or another) who underwent reoperation in our department. Because the association of bicuspid aortic valve with aortic coarctation is well described (with a prevalence of 25%–85%),5 we included patients referred to our center for complications either directly related to the coarctation or secondary to associated bicuspid aortic valve. Eighteen patients met these criteria and were included in our study (Table 1). The median age at the first operation for coarctation repair was 8 years (range 22 days to 55 years). Several surgical techniques were used in the first operation; in 4 cases, the technique was not described in detail. Thirteen (72.2%) patients had associated cardiac anomalies (Table 1). These 18 patients were referred for reoperation for
various reasons (Table 1). The median time between aortic coarctation repair and reintervention was 25 years (range 5–43 years; Table 2).
Results Among the 12 patients with bicuspid aortic valve, 6 developed significant aortic valve disease requiring surgery: 3 had isolated aortic valve disease, 2 had associated significant coronary lesions, and 1 had an ascending aortic aneurysm. In 4 of these patients, the aortic valvulopathy consisted of bicuspid valve stenosis only, and the other 2 had a combination of stenosis and regurgitation. Surgery on the aortic valve was performed at a median age of 47.5 years after follow-up of 31.5 years after coarctation repair. The echocardiographic mean pressure gradient across the aortic valve was 49 mm Hg (range 30–60 mm Hg) and the maximal pressure gradient was 68 mm Hg (range 50–80 mm Hg). In the 2 cases of regurgitation, aortic valve insufficiency was grade 2/4. All 6 patients were operated on via a sternotomy with standard cardiopulmonary bypass (CPB). Aortic valve replacement was performed in all cases; 5 had a mechanical prosthesis and one had a
Table 1. Data of 12 patients who required reintervention after repair of aortic coarctation. Technique of Patient no. Age at repair coarctation repair
Associated cardiac anomaly
Late complications requiring reoperation
1 2
15 years 1 year
IVC Bicuspid aortic valve
Recoarctation Recoarctation
3 4 5 6 7
6 years 3.5 months 10 years 14 years 2.5 years
Prosthesis interposition Crafoord þ prosthesis interposition at 13 years Crafoord Waldhausen Prosthesis interposition Prosthesis interposition Waldhausen
8 9 10 11 12 13 14 15 16
23 years 3 months 5 years 12 years 24 years 20 years 4 years 16 years 1 month
Isthmus patch plasty Crafoord Crafoord ND Crafoord ND ND ND Waldhausen
17 18
22 days 55 years
Crafoord Ascending aorta-to-coeliac aorta prosthesis interposition
Bicuspid aortic valve
Bicuspid aortic valve, subvalvular aortic stenosis, arteria lusoria Left inferior vena cava Bicuspid aortic valve Bicuspid aortic valve, arteria lusoria Bicuspid aortic valve Bicuspid aortic valve Subvalvular aortic valve stenosis Bicuspid aortic valve Bicuspid aortic valve, subvalvular aortic valve stenosis Bicuspid aortic valve Bicuspid aortic valve
Hypoplastic aortic arch Hypoplastic aortic arch Recoarctation Recoarctation þ pseudoaneurysm Ascending aortic aneurysm Isthmic pseudoaneurysm Ascending aortic aneurysm Ascending aortic aneurysm Ascending aortic aneurysm þ AVS AVS þ coronaropathy AVS þ coronaropathy Subvalvular aortic obstruction AVS þ AVI AVS þ AVI AVS Descending thoracic aortic aneurysm
AVI: aortic valve insufficiency; AVS: aortic valve stenosis; IVC: interventricular communication; ND: not documented.
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Table 2. Time between aortic coarctation repair and reoperation, according to late surgical complication.
Late complication Isolated bicuspid aortic valve disease Ascending aortic aneurysm Recoarctation Aortic arch hypoplasia Pseudoaneurysm Recoarctation þ pseudoaneurysm Subvalvular aortic obstruction Descending aortic aneurysm
Median coarctation repair-to-reoperation Incidence time (years) [range] 5
35 [18–43]
4 3 2 1 1
24 [22–27] 27 [24–38] 13 [9–17] 29 27
1 1
20 5
bioprosthesis (at 56 years of age) due to severe uncontrolled hypertension involving a significant risk of hypertensive intracranial hemorrhage with anticoagulation therapy. At the 6-month follow-up, 5 patients were doing well but one developed prosthetic valve endocarditis 6 months after implantation, necessitating a third intervention. Pathology of the ascending or horizontal aorta was found in 8 (44.4%) patients: 4 had ascending aortic aneurysms, 2 had pseudoaneurysms, and 2 had a hypoplastic aortic arch. The median age at reintervention was 27 years (range 15–52 years): 27 years for ascending aortic aneurysm, 46 years for pseudoaneurysm, and 1 6 years for aortic arch hypoplasia. The 4 patients who underwent surgery for ascending aortic aneurysm had associated bicuspid aortic valves. Their clinical presentation was mainly the onset or worsening of dyspnea (New York Heart Association class II/III) and asthenia; 2 presented with chest pain (one acute and miming symptoms of aortic dissection, the other chronic and associated with abdominal pain). All 4 patients had transthoracic echocardiography, 3 had computed tomography, and one had aortic angiography. The maximal diameter of the aneurysm was 59 mm (range 40–85 mm). Aortic dilatation included the coronary sinus in all cases and extended to the aortic arch in one. Surgery was performed in all 4 cases by median sternotomy, under standard central CPB in 3, and femorofemoral CPB in one. Two patients underwent a Bentall procedure and the other 2 had ectasia resection and direct end-to-end aortic anastomosis after mobilization of both the aortic arch and the heart (one had additional mechanical aortic valve replacement). The median CPB time was 207 min (range 127–322 min); 280 min for the Bentall technique and 133 min for resection-anastomosis. The median aortic crossclamp time was 118 min (range 85–
156 min); 150 min for the Bentall operation and 86 min for resection-anastomosis. The patient operated on with femorofemoral CPB suffered a right ventricle wound during the sternotomy, which resulted air emboli and diffuse brain damage in the early postoperative period. The patient remained in a chronic neurovegetative state and died on the 43rd postoperative day. In the other 3 cases, the postoperative course was uneventful with a median hospital stay of 11 days (range 6–17 days). Two patients were treated for pseudoaneurysm development after aortic isthmus plasty using a Dacron interposition graft for coarctation repair at the ages of 14 and 23 years. Reintervention occurred at 27 and 28 years after the first operation. Both patients presented with chest pain and class II dyspnea. Evaluation included transthoracic echocardiography and magnetic resonance imaging, with aortography in one case and transesophageal echocardiography and computed tomography in the other. Both aortic pseudoaneurysms (diameters 32 and 60 mm) developed at the distal site of Dacron patch implantation. Intraoperatively, ectasia associated with avulsion of the isthmus prosthesis was found. Resection of the aneurysm and prosthesis was followed by isthmic reconstruction by direct endto-end anastomosis in one case and by interposition of a 20-mm tubular Hemashield prosthesis in the other. The postoperative courses were marked only by persistent well-controlled hypertension. In our 2 cases of aortic arch hypoplasia, coarctation repair had been performed before the age of 6 months by resection and end-to-end anastomosis in one and Waldhausen aortoplasty in the other. Reintervention occurred after 9 and 17 years. Clinical presentations were typical with blood pressure asymmetry, hypertension in the right upper limb, loss of femoral pulses, and anisosphygmia. Transthoracic echocardiography and aortic angiography revealed excellent development of the isthmic area but major hypoplasia of the horizontal aorta (diameters between the 2 carotids were 7 and 10 mm) with systolic flow acceleration (3.5 m s1). Central aortic arch plasty with a left subclavian flap was performed in one case via an iterative posterolateral left anterior thoracotomy without CPB; resection of the atretic area and extended end-to-end anastomosis was carried out under CPB and circulatory arrest via a median sternotomy in the other. The measured diameters of the aortic arch after repair were 25 and 17 mm. The postoperative periods were event-free, and hospital stay was 10 and 11 days. Clinical and echographic examinations after 2 months were satisfactory except for residual hypertension, well-controlled on drug-therapy. Four patients were reoperated for recoarctation, including one with an associated pseudoaneurysm. The initial coarctations had been repaired using Dacron prostheses (diameters 10, 11, 12 and 16 mm).
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The median age at first intervention was 12 years (range 1–15 years) and the median age at reoperation was 40 years (range 34–41 years). Diagnosis was based on typical symptoms such as labile hypertension resistant to medical treatment, blood pressure discrepancy between the two upper limbs, asthenia, dyspnea, effort paresthesia, and loss of strength in the left upper and lower limbs. Evaluation included Doppler and aortic angiography, with computed tomography in 2 cases and magnetic resonance-angiography in one. The average pressure gradient across the stenosis was 46.3 mm Hg (range 25–64 mm Hg). In 2 cases, resection of the prosthetic tube and terminoterminal implantation of a larger isthmic prosthesis (18 and 20 mm) was performed under CPB with aortic crossclamping (22 and 54 min). One case was treated by extraanatomic bypass with a 16-mm prosthetic tube between the left subclavian artery and the descending thoracic aorta. The 4th patient underwent a bypass between the ascending and coeliac aorta (Figure 1) by interposing a 16-mm vascular prosthesis via a median sternotomy; the other 3 has an iterative left posterolateral thoracotomy. Postoperative evolutions were satisfying with complete regression of symptoms and good echographic results at the 6-month follow-up. Subvalvular aortic obstruction was found in one patient who was operated on at the age of 4 years, but the surgical technique and associated anatomy were not recorded. Twenty years later, he had no clinical symptoms and was participating in competitive
sport. A routine cardiology assessment revealed a subvalvular fibrous diaphragm with left ventricular hypertrophy and moderate aortic valve insufficiency. Surgery via a ministernotomy revealed left ventricular outflow tract obstruction due to a fibrous membrane, and a normal tricuspid aortic valve. The membrane was resected via a transaortic approach and the postoperative course was uneventful. One patient presented with a complex history: late diagnosis of aortic coarctation, aortic arch hypoplasia, and bicuspid aortic valve at the age of 23 years (incidentally discovered during a medical examination for military repatriation). At the time, he refused surgery but then developed progressively increasing poststenotic aortic ectasia. At the age of 66 years, a large thoracic descending aortic aneurysm was discovered due to dysphonia related to compressive left recurrent paralysis. The aneurysm and the coarctation area were excluded by prosthesis interposition between the ascending aorta and the coeliac aorta, with the expectation of gradual aneurysm thrombosis. However, the postoperative course was marked by continuing expansion of the aneurysm, causing tracheoesophageal compression with respiratory failure 4 years later (Figure 2). He underwent aneurysm ligation and exclusion, and was discharged from the hospital without any complication. Another specific problem of note in our series was premature coronary artery disease; patients with coarctation history often develop important arterial collaterals from the internal mammary arteries (IMA). Two of the patients with bicuspid aortic valve stenosis also presented with coronary lesions; both had undergone surgery for well-tolerated coarctation at the age of 20 and 24 years and were reoperated on 36 and 43 years later. One had a single
Figure 1. Postoperative computed tomography with 3-dimensional reconstruction in the patient operated on by extraanatomic bypass between the ascending aorta and coeliac aorta for isthmic recoarctation (ventral aorta). The coarctation area is indicated by the white arrow.
Figure 2. Chest radiograph 4 years after the first intervention (ventral aorta) following the appearance of respiratory insufficiency. The aortic aneurysm had greatly increased size (white arrow) and caused significant compression of the upper airways, with major tracheal deviation (dotted arrow).
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lesion in the proximal left anterior descending artery (LAD), and the other presented with 2 lesions and significant stenosis LAD. Because of the late coarctation repair, both patients had developed an important arterial collateral network, particularly at the expense of the IMA, which were very dilated and unusable (Figure 3). In the first case, the LAD stenosis was moderate and the patient was asymptomatic, so we decided not carry out coronary artery bypass and consider angiography later, if necessary. The second patient presented with clinical symptoms and severe stenosis of the LAD; the left IMA was extremely dilated (mean diameter 8 mm) and therefore unusable. A saphenous graft was used for coronary revascularization. Pathologic examination of the IMA revealed concentric partially calcified atheroma.
Discussion Adults operated on in childhood for congenital heart diseases now confront cardiac surgeons with a new category of patient with specific conditions. Due to the excellent results of surgical treatment for aortic coarctation, it was believed that these patients were completely cured after a successful intervention, but we now see the onset of late complications in adulthood. A significant percentage of these patients present with various complications either directly related to the
Figure 3. Computed tomography-angiography with 3-dimensional reconstruction in an adult operated on in late childhood for aortic coarctation. Note the very dilated internal mammary artery, consequent to the development of an important collateral arterial network.
coarctation and previous surgery (recoarctation, aneurysm or pseudoaneurysm, bacterial infection),6 or to associated pathologies (particularly bicuspid aortic valve disease). Approximately 11% of patients operated on for aortic coarctation require reoperation during follow-up.7 According to the literature, the surgical technique in the first intervention is an important factor in the subsequent evolution. Interposition of prosthetic material is associated with a substantial risk of recoarctation because the prosthetic tube gradually becomes stenosed with growth of the child, giving rise to typical symptoms of coarctation with a significant transprosthetic gradient. In our series, the mean transprosthetic gradient was 46.3 mm Hg; the diagnosis of aortic recoarctation is based on a mean gradient > 20 mm Hg. Prosthesis interposition is also associated with an increased rate of aortic aneurysms and pseudoaneurysms (5%–10% of all patients undergoing aortic coarctation repair, and 38% of patients with prosthesis interposition)8–10 which arise opposite the patch suture line or sometimes due to degeneration of the Dacron patch fibers.11 These aneurysms may remain asymptomatic for a long time and can be brutally revealed by aortic rupture or dissection, especially in patients with persistent hypertension.12 Our findings are in agreement with the literature data because all of our patients with aortic recoarctation or pseudoaneurysm had undergone prosthesis interposition during coarctation repair (Figure 4). These results confirm the current strategy of avoiding the use of prosthetic material, if possible. Ascending or horizontal aortic pathology was the reason for reoperation in 44.4% of cases in our series, and in agreement with the literature, a strong association between ascending aortic dilatation and bicuspid aortic valve was seen: all patients who were operated on for ascending aorta aneurysm had a bicuspid aortic valve. Once diagnosed, post-coarctectomy aortic aneurysms should be handled surgically. There is only one report of conservative treatment of these lesions; it recorded a 100% failure rate at 15 years.13 Management of these redo patients can be a challenge for cardiac surgeons because of the complexity of the anatomical lesions and postoperative adhesions due to the primary procedure. Given these complex conditions, we must use numerous preventive techniques to secure the intervention, such as femorofemoral cannulation prior to sternotomy and hypothermic circulatory arrest if necessary. Occasionally, the implementation of these preventive strategies may be insufficient and the consequences can be dramatic, as illustrated by one of our patients who suffered a cerebral embolism during the intervention, responsible for irreversible neurological damage and death. An attractive and promising alternative to major surgery is endovascular stent
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Figure 4. Relationship between the first surgical intervention and the late surgical complication. All cases in our series referred for aortic recoarctation or pseudoaneurysm underwent prosthesis interposition (Dacron patch or tube interposition).
implantation, but the indications are limited depending on the anatomical features.14 Surgery remains the treatment of choice in many cases. Bicuspid aortic valve was found in 66.7% of our patients, which is consistent with the literature data.15,16 The reason for this association is not clearly understood,17 but these aortic valve anomalies are responsible for significant morbidity and mortality,12 with the appearance of ‘‘bicuspid aortic valve syndrome’’ (both aortic valve stenosis and insufficiency). The majority of patients with bicuspid aortic valve develop abnormal valve calcifications before the age of 20 years, and almost all before 30-years old. The resulting aortic stenosis progresses rapidly (average gradient increase of 27 mm Hg per decade).12 Comparative studies showed a higher incidence of significant aortic valve disease in cases of both aortic coarctation and bicuspid aortic valve compared to isolated bicuspid valve. Thus coarctation seems to have an additional role in the genesis of symptomatic bicuspid aortic valve pathology.18 Besides the early aortic valve disease in these patients (in our series, mean age at surgery for aortic valve disease was 40 years), we are confronted with strategic issues during surgery, such as the choice of prosthetic valve when replacement is necessary. Indeed, we need to balance the risks and benefits of each prosthesis type. Another specific problem that appeared in our series was surgical treatment of coronary artery disease, sparsely described in the literature. It is widely known that patients with aortic coarctation tend to develop early coronary artery disease, although the mechanism is still unknown.14,16,18 Coronary disease appears to be the leading cause of late death after coarctation repair,19 responsible for 37% of deaths in these patients.7 Lawrie and colleagues20 first drew attention to premature coronary artery disease in patients with aortic coarctation,
noting that two-thirds of late deaths were attributed to acute myocardial ischemic events. Subsequent studies have confirmed this association and highlighted the development of severe atherosclerotic lesions in young patients with aortic coarctation.7 However, few studies have focused on the management of this pathology, which cannot be based on the general population, especially in cases of late coarctation repair. Coarctation repaired in adulthood induces the development of an extensive network of collaterals between the proximal and distal aorta, particularly via the IMA. The IMA are often extremely dilated and therefore unusable for coronary artery bypass grafting. In our series, we were faced to this problem twice. The treatment strategy must be discussed case by case to choose the best option for each patient. This particular factor during follow-up and monitoring of patients operated on for aortic coarctation deserves further studies in larger series with long-term results. Long-term results after aortic coarctation repair are overall satisfying, with 82% of patients asymptomatic after 20 years of follow-up.14 However, some patients present with long-term cardiovascular complications, possibly causing early death. In addition to the medical problem of late uncontrolled hypertension, these patients may develop specific complications requiring technically challenging surgical treatment. Late complications frequently remain asymptomatic for a long time, but can be treated appropriately when diagnosed early. Because of the various complications that can occur late or very late during follow-up, coarctation of the aorta, even appropriately repaired during childhood, is a condition that requires careful lifelong attention and follow-up, especially in patients with congenital aortic valve disease and those who have undergone coarctation repair with prosthetic material interposition.
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Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Jenkins NP and Ward C. Coarctation of the aorta: natural history and outcome after surgical treatment. QJM 1999; 92: 365–371. 2. Sudhayakumar N, Nair B and Satpathy M. Coarctation of the aorta. In: Satpathy M (ed.) Clinical Diagnosis of Congenital Heart Disease. New Delhi: Jaypee Brothers, 2008, pp. 160–169. 3. Crafoord C and Nylin F. Congenital coarctation of aorta and its surgical treatment. J Thorac Surg 1945; 14: 347–361. 4. Waldhausen JA and Nahrwold DL. Repair of coarctation of the aorta with a subclavian flap. J Thorac Cardiovasc Surg 1966; 51: 512–513. 5. Tawes RL Jr, Berry CL and Aberdeen E. Congenital bicuspid aortic valves associated with coarctation of the aorta in children. Br Heart J 1969; 31: 127–128. 6. Svensson LG. Congenital anomalies in adult. In: Coselli JS, LeMaire SA (eds) Aortic Arch Surgery: Principles, Strategies and Outcomes. Chichester, UK: WileyBlackwell, 2008, pp. 249–257. 7. Cohen M, Fuster V, Steele PM, Driscoll D and McGoon DC. Coarctation of the aorta. Long-term follow-up and prediction of outcome after surgical correction. Circulation 1989; 80: 840–845. 8. von Kodolitsch Y, Aydin MA, Koschyk DH, et al. Predictors of aneurysmal formation after surgical correction of aortic coarctation. J Am Coll Cardiol 2002; 39: 617–624. 9. Cramer JW, Ginde S, Bartz PJ, Tweddell JS, Litwin SB and Earing MG. Aortic aneurysms remain a significant source of morbidity and mortality after use of Dacron(Õ ) patch aortoplasty to repair coarctation of the aorta: results from a single center. Pediatr Cardiol 2013; 34: 296–301.
10. Walhout RJ, Braam RL, Schepens MA, Mulder BJ and Plokker HW. Aortic aneurysm formation following coarctation repair by Dacron patch aortoplasty. Neth Heart J 2010; 18: 376–377. 11. Chiesa R, Melissano G, Civilini E, Bertoglio L, Setacci F and Baccellieri D. Giant aneurysm 25 years after patch aortoplasty for aortic coarctation. Tex Heart Inst J 2008; 35: 220–221. 12. Roos-Hesselink JW, Scho¨lzel BE, Heijdra RJ, et al. Aortic valve and aortic arch pathology after coarctation repair. Heart 2003; 89: 1074–1077. 13. Knyshov GV, Sitar LL, Glagola MD and Atamanyuk MY. Aortic aneurysms at the site of the repair of coarctation of the aorta: a review of 48 patients. Ann Thorac Surg 1996; 61: 935–939. 14. Ho¨rmann M, Pavlidis D, Brunkwall J and Gawenda M. Long-term results of endovascular aortic repair for thoracic pseudoaneurysms after previous surgical coarctation repair. Interact Cardiovasc Thorac Surg 2011; 13: 401–404. 15. Tawes RL, Berry CL and Aberdeen E. Congenital bicuspid aortic valves associated with coarctation of the aorta in children. Br Heart J 1969; 31: 127–128. 16. Becker AE, Becker MJ and Edwards JE. Anomalies associated with coarctation of the aorta: particular reference to infancy. Circulation 1970; 41: 1067–1075. 17. Netter FH. The Ciba collection of medical illustrations. Heart 1969; 5: 163. 18. Ward C. Clinical significance of the bicuspid aortic valve. Heart 2000; 83: 81–85. 19. Toro-Salazar OH, Steinberger J, Thomas W, Rocchini AP, Carpenter B and Moller JH. Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol 2002; 89: 541–547. 20. Lawrie GM, DeBakey ME, Morris GC Jr, Crawford ES, Wagner WF and Glaeser DH. Late repair of coarctation of the descending thoracic aorta in 190 patients. Results up to 30 years after operation. Arch Surg 1981; 116: 1557–15560.
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