Aortic aneurysms after correction of aortic coarctation - Hogrefe eContent

VASA 2010; 39: 3–16 © 2010 by Verlag Hans Huber, Hogrefe AG, Bern

Y. v. Kodolitsch et al., Volume 39, Issue 1, February 2010 DOI 10.1024/0301–1526/a000001

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Aortic aneurysms after correction of aortic coarctation: A systematic review Y. von Kodolitsch, A. M Aydin, A. M Bernhardt, C. Habermann, H. Treede, H. Reichenspurner, T. Meinertz and A. Dodge-Khatami University Heart Center, Hamburg, Germany Summary

Zusammenfassung

Despite advanced techniques for surgical or percutaneous therapy coarctation of the aorta continues to carry a high risk of aneurysmal formation. Mortality of these aneurysms ranges between 90%, reflecting remarkable differences in surgical strategies and the follow-up management of coarctation. We review the frequency, anatomical types, risk factors and mechanisms of aortic aneurysm forming late after surgical or percutaneous therapy of aortic coarctation. We emphasize that aneurysms do not form exclusively at the site of previous intervention, but also at remote locations such as the ascending aorta. Moreover, aneurysm formation may only in part be attributed to a specific technique of coarctation therapy, and we emphasize the role of a bicuspid aortic valve and inherent weakness of the aortic wall as significant risk factors for aneurysm after aortic coarctation. We report the presenting symptoms, follow-up protocols, and imaging criteria for local and proximal aneurysms. Finally, we discuss criteria for prophylactic intervention at the site of such aneurysms, and present therapeutic options for different types of aneurysms. With this systematic review, we wish to provide data for establishing more uniform strategies for preventing, diagnosing and treating aneurysms associated with aortic coarctation.

Aortenaneurysmen nach Korrektur einer Coarctatio aortae: Eine systematische Literaturanalyse Trotz des Einsatzes fortgeschrittener chirurgischer und interventioneller Techniken zur Therapie der Aortenisthmusstenose bleibt das Risiko der Entwicklung von Aneurysmen der Aorta erhöht. Die Mortalität dieser Aneurysmen wird in der Literatur mit zwischen < 1% und > 90% angegeben. Diese Unterschiede der Prognose sind durch verschiedene Techniken zur Korrektur der Koarktation und durch unterschiedliche Strategien der Nachsorge bedingt. Wir liefern einen Überblick über die Häufigkeit, die anatomischen Typen, die Risikofaktoren und Mechanismen der Aneurysmabildung, die nach chirurgischer wie nach interventioneller Therapie einer Koarktation der Aorta entstehen können. Wir betonen, dass diese Aneurysmen nicht ausschließlich im Bereich der ehemaligen Koarktation entstehen, sondern auch in entfernten Bereichen des Gefäßes, wie beispielsweise der Aorta aszendens beobachtet werden. Wir zeigen, dass die Aneurysmabildung nur zum Teil durch Anwendung spezifischer Techniken der Korrektur der Koarktation bedingt ist. In diesem Zusammenhang betonen wir die Rolle der bikuspidal angelegten Aortenklappe als signifikanten Risikofaktor der Aneurysmabildung ebenso wie eine oft multifaktoriell bedingte strukturelle Schwäche der Aortenwand bei Koarktation. Unsere Arbeit liefert zudem einen Überblick über typische Symptome, Strategien der Nachsorge, und diagnostische Kriterien für Aortenaneurysmen nach korrigierter Koarktation. Wir diskutieren publizierte Kriterien zur prophylaktischen Intervention bei verschiedenen Typen der Aortenaneurysmen. Mit unserer systematischen Analyse der Literatur beabsichtigen wir eine Übersicht zu schaffen, die die Entwicklung einheitlicherer Strategien zur Diagnose und Therapie von Aortenaneurysmen nach korrigierter Koarktation der Aorta erleichtert.

Key words: Coarctation, aorta, bicuspid aortic valve, aneurysm, endovascular stentgrafts

Introduction Without treatment, 50 % of patients suffering from aortic coarctation die before the age of 32 years. Rupture of aortic aneurysm is the second leading cause of death, occurring in 21 % of cases [16, 17, 29].

Life expectancy can be improved when aortic coarctation is corrected in early childhood. However, aortic aneurysms that develop despite successful repair of aortic coarctation emerge as a major risk for death despite successful repair [21].

Interestingly, the frequency of aneurysm formation varies between 1–51 % across different centers. This extensive variability may be explained to a large part by different diagnostic criteria for aneurysm and different follow-up strategies. For instance, the reported incidence has

Y. v. Kodolitsch et al., Volume 39, Issue 1, February 2010


aneurysms usually originate at the site of sutures for anastomosis of vascular grafts, and fistula formation may result in hemoptysis or gastrointestinal bleeding [78]. Conversely, true aneurysms form opposite to a patch graft, or above a tube or patch graft with involvement of the aortic arch [3, 20, 59, 111].

Dissections usually develop in true aneurysms and originate proximal to a tube graft, or proximal to an end-to-end anastomosis [3, 20, 117]. Local aneurysms ruptured in 50 % of 20 patients in the series of Parks and collagues, and in 12 % of 17 patients in our own series [81, 111] (Fig. 1).

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increased through the past two decades, most likely due to improved diagnosis by use of advanced imaging technology. In a follow-up study of 235 adults (mean age 27 ± 13 years) with coarctation, treated surgically (n = 181, group I), by balloon angioplasty or stenting (n = 28, group II), or who were left untreated (n=26, group III), Oliver and colleagues identified aneurysms in 37 individuals (16 %) requiring surgical or percutaneous intervention. Interestingly, aneurysm frequency (15 %, 18 %, and 15 % in groups I, II and III, respectively) and location of aneurysm in the ascending (9 %, 11 %, and 12 %), or descending aorta (4 % in all groups) were independent of the initial treatment type for aortic coarctation [78]. After coarctation repair, more than two thirds of aneurysms develop in males [17, 78, 111]. According to classical studies, the mean age at initial diagnosis of aneurysm formation is 33 ± 16 years [3, 11, 13, 20, 27, 43, 44, 59, 60, 62, 81–83, 86, 98] with a large range between six [69] and 63 years [109]. The mean time interval from initial intervention on coarctation to aneurysm formation ranges widely from 4–18 years. The risk of aneurysm formation increases with time, with exponential increase ten years after surgery for aortic coarctation [81].

Anatomical features Local aneurysms The majority of aneurysms are reported at the site of primary coarctation repair. At the time of initial diagnosis, these aneurysms exhibit maximum diameters of 38 ± 13 mm in the series of Bogaert and colleagues, and of 56 ± 18 mm in our series [11, 111]. Such aneurysms may be true, false, mycotic or dissecting [1, 3, 11, 13, 20, 27, 43, 44, 59, 60, 62, 78, 81–83, 86, 98]. False

Figure 1: Anatomical types of aneurysms, which may form after successful percutaneous or surgical repair of aortic coarctation. We distinguish local types of aneurysm, which form at the site of primary coarctation repair, and remote types, which form at sites of the thoracic aorta that are unrelated to the site of coarctation repair.



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Proximal aneurysms One third of post-surgical aneurysms are identified in the aortic root and ascending aorta [3, 5, 20, 36, 52, 67, 87, 89, 99, 110, 111, 117]. Interestingly, Oliver and colleagues observed that ascending aortic aneurysms outweigh descending aortic aneurysms by a ratio of 2 to 1 [78]. At the time of initial diagnosis, proximal aneurysms present with average diameters of 83 ± 21 mm. Such large aneurysms have probably formed, because follow-up imaging was not directed at this site of the aorta or was not performed at all. Most of these aneurysms are true, but dissection occurs in up to 25 percent [111]. Moreover, aortic valve regurgitation of at least moderate degree is present in the vast majority of patients [111]. Signs of degeneration of the aortic media are usually marked or moderate, and a congenitally bicuspid aortic valve is present in most cases (Fig. 2) [36, 52, 78, 110, 111].

tified only for complex types such as Turner syndrome caused by chromosomal abnormality [65], or the Alagille syndrome related to defects

of the Jagged1 gene (JAG1) [54, 85]; these syndromes are associated with generalized aortic disease. For noncomplex types of aortic coarctation,

Pathogenetic mechanisms We identify five major mechanisms leading to aneurysm formation after intervention for aortic coarctation: Generalized vascular abnormality Coarctation of the aorta may be (1) an isolated malformation, (2) occur with other non-complex cardiovascular abnormalities, or be part of (3) complex congenital cardiovascular malformations such as complete atrio-ventricular septal defect or transposition with subpulmonary ventricular septal defect (TaussigBing heart) and (4) syndromes with multiorgan involvement. A tendency to familial aggregation of coarctation is usually compatible with multi-factorial inheritance [12]. Isolated causes are currently iden-

Figure 2: Various techniques for surgical repair of aortic coarctation. Frequencies of post-surgical aneurysmal formation are reported as in our previous literature review [111].



arteries, the aortico-pulmonary septum, the coronary arteries and both semilunar heart valves [55, 57, 104]. A congenitally bicuspid aortic valve is identified as an independent predictor for post-surgical aneurysm in the ascending aorta [111] and may also be associated with local aneurysms [78]. Schaefer and colleagues identified three morphologies of congenitally bicuspid aortic valves. They defined type 1 by fusion of the right and left coronary cusp (80 % of their cases), type 2 by fusion of the right and non-coronary fusion

(20 %), and type 3, by left and noncoronary fusion, which they observed in only one of their patients (Fig. 3) [93]. According to this study, the aortic sinuses were larger in type 1, while type 2 exhibited larger arch dimensions. A normal shape of the ascending aorta was more common in type 1, and a dilated ascending aorta was more common in type 2. Mutations in the signalling and transcriptional regulator NOTCH1 can cause a bicuspid aortic valve and severe valve calcification in nonsyndromal autosomal dominant hu-

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there is evidence of family clustering with left ventricular outflow tract obstructions such as hypoplastic left heart syndrome, aortic valve stenosis, congenitally biscuspid aortic valves, interrupted aortic arch type A, Shone complex, and possibly also septal hypertrophy, mitral valve prolapse, and dilation of the ascending aorta. Defects of cardiac neural crest cells are also appealing to explain the coincidence of aortic abnormalities with cardiac outflow tract malformations, since these cells participate in the development of the aortic arch

Figure 3: Anatomical types of bicuspid aortic valve (BAV) according to a classiﬁcation system suggested by Schaefer and colleagues [93]. Type 1 exhibits congenital fusion of the right and left coronary cusp (also called anteriorposterior type [22]). Type 2 has a congenital fusion of the right and non-coronary cusp (also called right-left type [22]). Type 3 exhibits a congenital fusion of the non-coronary and left coronary cusp. The frequencies of all three types are given as reported from 191 individuals with BAV [93]. Echocardiographic features of the normal aortic valve and of all three types of BAV are given as seen on transthoracic (TTE) and transesophageal (TEE) echocardiography.



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man pedigrees [39]. Bicuspid aortic valves coincided with coarctation in 16–65 % of cases [50, 55, 83, 108], and diameters of the ascending aorta may still progress significantly after replacement of a bicuspid aortic valve [119]. Moreover, aortic tissue samples obtained during surgery exhibited reduced fibrillin-1 content and increased matrix metalloproteinase 2 activity in aortas with bicuspid aortic valves [92]. Interestingly, knock-out of a single gene, endothelium-derived nitric oxide synthase (eNOS) in 12 mature mice resulted in a high prevalence of a bicuspid aortic valve, however, aortic coarctation remains to be identified in larger samples [65]. A hypoplastic aortic arch is observed in 3–65 % of coarctations in infants of less than two years of age [7, 42, 61, 107] and in 22–40 % of coarctations diagnosed in adolescents or adults [8, 9, 15, 83]. Such malformation is indicative of a more widespread abnormality of the aorta, and was identified as an independent predictor of post-surgical aneurysm formation, particularly in the poststenotic region at the site of previous coarctation repair [11, 71]. Impaired vascular function in the pre-coarctational aortic segment The pre-coarctational aorta exhibits reduced contractility, less smooth muscle mass and increased collagen content [96]. Conduit artery function exhibits abnormal responses to flow and nitroglycerin, with increased vascular stiffness confined to the upper part of the body in patients after coarctation repair [24]. After coarctation repair, pathologic aortic arch geometry with acute angulation between the ascending and descending aorta with loss of a horizontal segment results in post-surgical arterial hypertension [79]. Interestingly, impaired vascular function

and abnormal arch geometry are less marked with early coarctation repair [24, 79]. Advanced age at coarctation repair is an independent predictive factor for postoperative mortality and aneurysm formation [21, 78, 111]. The risk for aneurysms increases when coarctation repair is performed ≥ 13.5–16 years [78]. Local tissue properties A so-called hemodynamic theory regards coarctation as a branch-point of the ductus arteriosus, that results from reduced aortic flow related to left ventricular outflow tract obstructions; this type of coarctation results in the formation of an intimal cushion that is histologically indistinguishable from normal blood vessels [48]. Conversely, in 1855 Skoda described aortic coarctation as a constriction caused by ectopic ductal tissue that invades the aorta as a sling that surrounds the entire aortic lumen (Skodaic theory) [45]. Elzenga and colleges surmised that migration of ductal tissue is flowdependent. According to their histologic findings, ductal tissue invades the aorta with increased right-toleft ductal flow caused by left-sided obstruction, but migrates into the pulmonary artery with increased left-to-right ductal flow caused by right-sided obstruction such as tetralogy [30, 31]. Numerous histologic studies confirm ectopic ductal tissue in aortic coarctation, and recent in vivo studies of intra-cardiac flow forces in zebrafish embryos confirm intra-cardiac hemodynamics as a key epigenetic factor in embryonic cardiogenesis [45, 47, 91] (Fig. 4). To date, there is no consensus for a uniform surgical strategy. Both persisting ductal tissue [1, 51] and extensive resection of the fibrous ridge have been identified as risk factors of late aneurysm development [44]. Cystic media degeneration and severe atherosclerosis are also associ-

ated with aneurysms at the site of previous repair [3, 59]. Surgical and interventional techniques Evidence is overwhelming that the patch graft technique carries a high risk [1, 3, 11, 13, 20, 27, 44, 59, 62, 81, 83, 86], and even is an independent predictor [5] for aneurysm formation. However, in children, localized dilatation of the aorta after patch aortoplasty is due mostly to a large patch and only to a lesser extent, due to aortic wall growth [2]. Moreover, Oliver and colleagues did not confirm preponderance of any surgical or interventional technique of coarctation repair in their unselected series of patients with local aneurysm formation [78]. A review of the classical surgical literature identifies 29 aneurysms after 165 subclavian-flap procedures (17 %), twelve aneurysms after 184 insertions of a tube graft (6 %), and 33 aneurysms after 1,089 end-to-end anastomosis (3 %) performed for coarctation repair [111]. Aneurysm formation has been documented after bypass grafting for long aortic coarctation [38, 41, 78], and after percutaneous interventions for aortic coarctation [46, 100]. Hornung and colleagues found an average of 5.5 % aneurysm formation after balloon angioplasty of coarctation in adults, and of 4.3 percent after endovascular stent implantation for aortic coarctation in adolescent and adult patients [46]. Flow disturbance and other factors Tubular hypoplasia of the aortic arch or recoarctation leads to flow disturbances. This may result in impaired axial flow and increased nonphysiologic shear stress of the distal aortic wall which causes aneurysm formation. Narrowing of an aortic segment leads to blood flow acceleration, and post-stenotic turbulence



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Figure 4: The two main theories for the pathogenesis of aortic coarctation. A) Skodaic hypothesis [45]: Postnatal contracture of ectopic ductal tissue in the aortic wall causing coarctation that results in circumferential obstruction. B) Hemodynamic hypothesis [48]: Coarctation is regarded as a branch-point of the ductus arteriosus opposite the aortic end of ductus arteriosus which leads to a localized shelf. The reason is increased ductal blood ﬂow during early development, particularly with presence of a bicuspid aortic valve.

may induce aneurysm development in the distal aortic segments. Although the cause for secondary aneurysm formation after repair of coarctation is most likely multifactorial, turbulent flow may play a mediating or enhancing role [11]. Non-physiologic stress at suture lines, suture dehiscence, patch laceration, ischemic aortic wall necrosis at intra-operative clamping sites, intra-operative intimal lesions and injury of the vasa vasorum are also discussed as potential mechanisms of local aneurysm formation [3, 28, 59]. Moreover, Dacron may be more prone to aneurysm formation than polytetrafluoroethylene (PTFE) [1, 6]. False aneurysms develop from aortic graft infection, but are usually encountered early some months

after coarctation repair [20, 58, 59, 68, 82, 110].

Prevention of aneurysm formation It is unknown whether aneurysm formation can be delayed or prevented by prophylactic measures [70, 115]. However, control of systemic hypertension is likely to reduce the risk of aneurysm formation [1, 21, 78, 81, 111]. Moreover, long-term beta-adrenergic blockade is effective for managing aortic dissection, in delaying progression of abdominal aneurysm, in preventing late progression of intramural hematoma, and in delaying aortic root dilatation in Marfan patients [96, 113, 114].

Thus, beta-adrenergic blockade is the standard for treating patients with systemic hypertension after coarctation repair, particularly when a bicuspid aortic valve is present [34, 35, 116]. Preliminary studies in Marfan patients provide experimental and clinical evidence, that angiotensin converting enzyme inhibitors and angiotensin II-receptor blockers prevent or retard aneurysmal formation [14, 77, 120]. These drugs may also protect the aorta in postoperative coarctation. Future experiments may elucidate the role of matrix metalloproteinases inhibitors and gene or protein therapy to strengthen the deficient extracellular matrix [35]. Most importantly, irrespective of the specific drug used to protect the aorta, patients require serial tomographic imaging to monitor the effect of drugs and to identify aortic aneurysmal formation [18, 105]. Moreover, similar to Marfan patients, future studies may help to establish functional measurements of aortic stiffness, such as applanation tonometry to a predict risk of aortic disease progression [75].

Presenting symptoms Presenting symptoms largely depend on the location of aneurysm formation. Proximal aneurysms usually present with symptoms resulting from aortic dissection, rupture, pericardial tamponade and severe regurgitation of the aortic valve [36, 37, 52, 67, 99, 110 113, 117]. Thus, chest pain, dyspnoea, hemoptysis, syncope, and cardiogenic shock are the most frequently observed symptoms in these patients. Conversely, local aneurysms usually present on serial follow-up investigations before they cause symptoms, with only one third presenting with signs of acute complications including pain, dyspnoea, septic fever, hemoptysis



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or syncope. Unfortunately, a substantial subset of the symptomatic patients is not diagnosed before autopsy [3, 11, 13, 20, 27, 44, 59, 62, 81, 83, 86]. Postoperative follow-up Screening for aneurysm can be performed with various modalities comprising transesophageal echocardiography (TEE), magnet resonance imaging (MRI), and contrast-enhanced computerized tomography (XCT) (95). However, there are few studies that employ TEE to screen for post-surgical aortic aneurysms [33, 112]. Single-slide or spiral computerized tomography is highly accurate for detecting aneurysm and dissection of the thoracic aorta, with the specific advantage of delineating thrombogenic plaque and calcification in the aortic wall. Many investigators employ computerized tomography to screen for post-surgical aneurysm, but none of their studies are designed to prospectively assess its diagnostic performance [13, 32, 43, 68, 111]. Using computerized tomography or TTE to measure the aortic diameter at the repair site and at the level of the diaphragm, Bromberg and colleagues determined an aortic ratio from these two measurements, and defined aneurysm as a ratio ≥ 1.5 [13]. Aebert and coworkers also use an aortic ratio ≥ 1.5 but, in addition consider aneurysm with a maximum aortic diameter exceeding 40 mm [1], whereas other surgeons prefer to take action, when they document progression of aneurysm diameters. Unfortunately there are no uniform criteria for elective intervention [90]. Currently, evidence-based protocols are not available to follow adult patients after coarctation repair. However, current guidelines consider MRI as the investigation of choice and do not recommend routine use of TEE (Fig. 5) [26].

Expert centers perform life-long clinical visits in yearly intervals including ECG, exercise testing, chest radiography and TTE; MRI is performed routinely every two to three or five years [5, 26, 105], or, in high risk patients such patients with a bicuspid aortic valve, or with a patch graft at the site of coarctation repair every 12 to 24 months (Table I) [103, 111].

Indications for reintervention Local aneurysm Controversy exists about optimal timing of elective surgery for local aneurysms. Some authors rec-

ommend to delay resection of local aneurysm, presuming that they regress with time [74]. However, Mendelsohn and colleagues do not observe spontaneous resolution of local aneurysm, but in contrast find that children with an aortic ratio ≥ 1.5 at the repair site develop significant progression of their ratios from 1.64 ± 0.06 at baseline to 2.04 ± 0.2 within three to five years of follow-up [72]. Parikh and colleagues find an aortic ratio ≥ 1.68 a better criterion for progression, since they have observed that in children aortic ratios < 1.68 usually decrease with growth [80]. Kron and colleagues recommend to resect aneurysms related to patch grafts with a diameter ≥ 60 mm [62]. Today,

Figure 5: Magnetic resonance angiogram in a patient with unrepaired aortic coarctation with detailed anatomical mapping of a marked aneurysm of the ascending aorta and dilated intercostal and retrosternal collateral arteries.



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Table I: Diagnostic criteria reported for aneurysm formation at the site of previous coarctation repair

Aortic contour increases in size compared to initial postoperative chest radiographs [84] Widening of the aortic contour or the mediastinal shadow on serial chest radiography [48, 108] Aortic ratio ≥ 1.5 (that is the ratio of the aortic diameter measured at the repair site and at the level of the diaphragm using tomographic imaging or angiography) [1, 11, 13, 71, 78, 79, 81] Maximum aortic diameter > 40 mm at the site of previous coarctation repair [1] New geometric changes at the site of coarctation repair [78]

however, surgeons would likely accept lower thresholds for intervention. Aebert and colleagues perform reoperation of the descending aorta when the diameter of a fusiform dilatation exceeds 45 mm, or when a saccular aneurysm is noted [1]. Some authors suggest yearly tomographic imaging in patients with local aneurysm and recommend surgical intervention only in cases with progressive aneurysm dilatation [13, 68, 86]. We feel that in case of a large diameter of aorta, 6 month followup period is advisable. Since a critical ratio for rupture is unknown and the mortality of ruptured aneurysms is high, many surgeons tend to operate soon after a local aneurysm is diagnosed [3, 20, 27, 59, 72]. The presence of a pseudo-aneurysm at a previous suture line is also considered an indication for reintervention [5] (Table II). Proximal aneurysm Most authors adopt guidelines for prophylactic aortic root replacement from Marfan patients, who are at a risk for aortic dissection and rupture that is considered similar to patients with concurrent bicuspid aortic valve and coarctation [35, 116]. Thus, prophylactic intervention is classically recommended with maximum aortic root diameters ≥ 55 mm [40]. Intervention may be carried out earlier in high risk patients, including patients with an annual increase of the aortic ratio

exceeding 5 percent, with dilatation of the aortic sinuses involving the ascending aorta, with severe aortic or mitral valve regurgitation, with a family history of aortic dissection, with other major surgery required in the near future, or, in women planning pregnancy [84, 88, 106].

Therapeutic options Local aneurysms Some surgeons repair small aortic aneurysms by aortorrhaphy [59], by external support with wrapping of the aneurysm [3], patch plasty [59] or end-to-end anastomosis [20]. In cases with concomitant cardiovascular malformations and technically demanding aneurysms, a palliative surgical approach may be used by implanting a prosthetic graft that bypasses the isthmic region [5, 19, 41]. However, these techniques are appropriate only in selected patients. The surgical gold standard is to insert a tube graft after the aneurym is resected [1, 3, 4, 20, 27, 59, 60, 62]. Usually, the aorta is cross-clamped and various types of artificial circulation are used to maintain adequate circulation [59]. Cardiopulmonary bypass with hypothermic circulatory arrest avoids aortic clamping and sacrifice of intercostal arteries, and provides adequate protection of the spinal cord and other vital organs. This technique is particularly useful in extended surgery for post-surgical

complications of complex coarctation with extensive aortic aneurysm, persistent hypoplastic aortic arch, or aneurysm of aberrant left subclavian arteries [63, 87]. When coarctation is associated with ascending aortic aneurysm, the risk of rupture during aortic clamping for coarctation repair may be high and thus some surgeons recommend repair of ascending aortic aneurysm before correction of coarctation. As anatomy, size and localization of the aneurysms varies greatly, surgical strategies may be individualized [5, 76]. Proximal aneurysms Severe regurgitation of a bicuspid aortic valve combined with aneurysm of the ascending aorta is usually treated with a composite root replacement. Alternatively, the aortic valve and the supracoronary ascending aorta may be replaced separately. In Marfan patients this procedure is not appropriate, since aneurysms develop in the diseased aortic tissue that is left between the valve graft and the aortic tube graft [64]. Unfortunately, there are no longterm studies on the postoperative course after surgery of bicuspid aortic valves comparing both surgical techniques. However, in a series of 27 procedures with bicuspid aortic valve replacement and separate tube graft implantation in the ascending aorta, no patient required re-intervention for recurrent aneurysm of the aortic root [102]. This strategy



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Table II: Reported indications for intervention for aneurysm formation at the site of previous coarctation repair

Aortic ratio at the repair site ≥ 1.5 [70], or ≥ 1.68 [77] Aneurysms related to patch grafts with a diameter ≥ 60 mm [60] Diameter of a fusiform dilatation of the descending aorta ≥ 45 mm [1]. Saccular aneurysm of the descending aorta [1] Pseudoaneurysm at a previous suture line [5] Surgical intervention only with progressive aneurysmal dilatation on annual tomographic images [13, 66, 83] Surgical intervention as soon after local aneurysm is diagnosed [3, 19, 26, 57, 70]

may especially be appropriate in older patients with valvular stenosis requiring extensive concomitant surgical procedures [102]. Surgeons also use the remodelling and reimplantation techniques for valvesparing operations in patients with aortic root aneurysms associated with a bicuspid aortic valve [25, 53, 94, 101, 118]. However, long-term results are not available, and at least from a theoretical point of view, this procedure does not eliminate the inherent susceptibility of a bicuspid aortic valve for endocarditis, or valve dysfunction [73]. Interventional therapy Endoluminal treatment of thoracic aortic aneurysms of atherosclerotic etiology is an established option, with the largest series published by the Stanford group [23]. In the setting of post-coarctation aneurysm, experience is more limited. Given the relative risks associated with surgical repair, not only with regards to procedural mortality, but also with morbidity related to recurrent laryngeal nerve paralysis (13–36 %) and phrenic nerve injury (5–6 %), more recent attempts have been made at interventional endoluminal stent covering of the aneurysms [10]. In small series of elective indications for aneurysm and pseudoaneurysm treatment using covered stents, both self-expanding and balloon-dilatable, successful deploy-

ment has been achieved percutaneously through the femoral artery, without mortality or major morbidity [10, 56, 66]. Although the safety and reproducibility of the technique is established, longer follow-up is required to assess the long-term stability and hence benefit to the patients of this less invasive therapeutic option, when compared to surgery. Prognosis Patients suffering acute symptoms from a previously unknown aortic aneurysm may die suddenly, or if referred to a hospital, have a poor prognosis because of severe complications. In hospitalised patients without immediate surgical intervention, reported lethality ranges between 26–100 %, with an average at 56 % [20, 44, 59, 78, 81, 82, 98]. Even when surgery is carried out immediately, the average lethality remains high with an average of 42 % [27, 44, 59, 60, 62, 78, 111]. Conversely, elective interventions are survived without complications in the majority of patients, with a reported surgical mortality as low as 2.2 % [3, 27, 44, 59, 60, 62, 78, 111]. Thus, survival of aneurysm formation depends on the quality of medical management, and close surveillance of the entire thoracic aorta is a cornerstone for preventing highly lethal complications of acute rupture or dissection of aneurysms, allowing for elective surgical therapy.

Prognosis of aneurysm after coarctation repair should probably change in future. In fact, the risk of aneurysm after coarctation repair demands elective strategies of aortic follow-up for these patients. Defining more uniform MRI protocols with aortic measurements, repeated examination according to baseline clinical and anatomical values and their progression are mandatory. The role of education also has to be considered essential in this population. Such approach should reduce the risk of death.

Conflicts of interest There are no conflicts of interest existing.

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Correspondence address PD Dr. Yskert von Kodolitsch MBA Department of Cardiology University Heart Center Martinistrasse 52 D-20246 Hamburg Germany E-mail: [email protected]

Submitted: 15.04.2009 Accepted after revsion: 28.06.2009