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Management of thoracic aortic aneurysm in adults

Management of thoracic aortic aneurysm in adults
Literature review current through: Jan 2024.
This topic last updated: Sep 18, 2023.

INTRODUCTION — Thoracic aortic aneurysm (TAA) can be due to one of several etiologies. The natural history of TAA is one of progressive expansion, the rate of which depends upon the location of the aneurysm and its underlying cause. Although most TAAs produce no symptoms, patients who become symptomatic or have complications related to the aneurysm (eg, acute aortic regurgitation, dissection, aortic rupture) should undergo repair [1-5]. Conservative management of asymptomatic TAA aims to lessen stress on the aorta and limit further aortic expansion. Asymptomatic patients who do not meet criteria for repair also require ongoing aneurysm surveillance. Any patient with additional clinical risk factors (eg, Marfanoid habitus, positive family history) should be evaluated for possible underlying genetic conditions known to be associated with thoracic aortic aneurysm and dissection (TAAD). Where expertise in the management of thoracic aortic disease is not available, the patient should be transferred to a high-volume cardiovascular center with a multidisciplinary aortic team to provide the best possible outcome [6].

The management of thoracic aortic aneurysm is reviewed here. The etiology, natural history, clinical features, and diagnosis of TAA, as well as specific techniques for repair of the thoracic aorta, are discussed separately. (See "Epidemiology, risk factors, pathogenesis, and natural history of thoracic aortic aneurysm and dissection" and "Clinical manifestations and diagnosis of thoracic aortic aneurysm" and "Endovascular repair of the thoracic aorta" and "Overview of open surgical repair of the thoracic aorta".)

INDICATIONS FOR REPAIR — We agree with major cardiovascular society guidelines from the American College of Cardiology, American Heart Association, and Society of Vascular Surgery that recommend repair for all symptomatic thoracic aortic aneurysm (TAA; ruptured, associated with dissection, causing pain) [1-5,7,8]. In general, repair of asymptomatic TAA is not recommended until the risk of rupture or other complications exceeds the risks associated with repair. Asymptomatic TAAs are selected for repair depending upon diameter, location, expansion rate, family history of rupture/dissection, and the presence of associated coronary heart disease or valve pathology requiring surgical intervention, with special considerations depending on the presence of underlying contributing etiologies (eg, connective tissue disorders, bicuspid aortic valve, familial thoracic aortic aneurysm/dissection). For patients who meet the criteria for repair, survival is improved for open surgery compared with medical therapy alone [4,5]. This should also be the case for endovascular repair given that endovascular repair compares favorably with open surgery. Asymptomatic patients with TAA who do not meet criteria for repair are managed medically with routine surveillance. (See 'Management of asymptomatic TAA' below.)

Summary of indications — Recommendations for repair for TAA in the ascending (table 1) and descending thoracic aorta are summarized and discussed in detail below. (See 'Symptomatic (nonruptured) and ruptured TAA' below and 'Asymptomatic TAA' below.)

Symptomatic (nonruptured) or ruptured TAA.

Asymptomatic ascending TAA [7]:

End-diastolic aortic diameter >5.5 cm [5,8], or aortic size index (aortic diameter [cm] divided by body surface area [m2]) ≥2.75 cm/m2 [9] or aortic area over height ratio (ie, maximal aortic cross-sectional area [cm2] divided by height [m]) >10 cm2/m [4,5]. A body surface area calculator can be found in the link (calculator 1). (See 'Degenerative TAA' below and 'Accounting for body size' below.)

-Surgery can be considered for patients with >5 cm aortic root/ascending aortic aneurysms when bicuspid aortic valve is present or for those with a trileaflet aortic valve, if surgery is performed at a comprehensive multidisciplinary aortic team.

-Surgery is indicated for patients with an aneurysm of the aortic root or ascending aorta of <5.5 cm, but an expansion rate of ≥0.3 cm per year in two consecutive years, or ≥0.5 cm in one year.

-For patients with a height >1 standard deviation above or below the mean who have an asymptomatic aneurysm of the aortic root or ascending aorta and a maximal cross-sectional aortic area/height ratio of >10 cm2/m, surgery is reasonable when performed by experienced surgeons in a multidisciplinary aortic team.

For patients with genetically mediated thoracic aortic aneurysm and dissection (TAAD), including syndromic conditions such as Marfan, Loeys-Dietz, vascular Ehlers-Danlos, and Turner syndromes, as well as nonsyndromic conditions like familial TAAD or bicuspid aortic valve, a lower diameter or aortic index is suggested as an indicator for repair (figure 1). The specific threshold diameter depends on the condition. (See 'Genetically mediated thoracic aortic aneurysm and dissection' below and 'Accounting for body size' below.)

For patients undergoing aortic valve surgery or coronary artery bypass grafting: End-diastolic aortic diameter >4.5 cm in diameter.

Asymptomatic descending TAA:

For most average-sized adults: Diameter of >5.5 cm [5,8].

Patients with high surgical risk for repair (eg, Society of Thoracic Surgeons Predicted Risk of Mortality [PROM] score >8 percent, frailty score index >2, two compromised organ systems [10]): Diameter ≥6 to 7 cm.

For patients with genetically mediated conditions (syndromic or nonsyndromic) or other factors that increase the risk of rupture (eg, aneurysm expansion >0.5 cm per year, saccular aneurysm, infectious aneurysm, female sex), a smaller diameter is suggested as an indicator for repair [7]. The specific threshold diameter depends on the condition.

For smaller patients, including many females, a diameter greater than twice the diameter of the nonaneurysmal aorta (normal segment) or the aortic size index can be used. (See 'Accounting for body size' below.)

Asymptomatic TAA with rapid expansion ≥5 mm per year for aneurysms <5 cm in diameter [5,11].

Symptomatic (nonruptured) and ruptured TAA — Although most TAAs are asymptomatic, TAA can produce a variety of symptoms and complications, which can be life-threatening. Chest pain associated with TAA could represent rapid aneurysm expansion, dissection, or rupture. Chest pain associated with evidence of acute dissection in a true thoracic or thoracoabdominal aortic aneurysm can be the first and only sign of a TAA [12]. Others may rarely present with dysphagia or dyspnea, usually related to compression of adjacent organs [4,5]. Patients whose symptoms can be attributed to the aneurysm should undergo repair [4]. (See "Clinical manifestations and diagnosis of thoracic aortic aneurysm", section on 'Symptomatic TAA' and 'Thoracic aneurysm repair' below.)

It has been suggested that patients with untreated large ascending or descending thoracic aneurysms are more likely to die of complications associated with their aneurysms than from any other cause [13]. In several series of patients, aneurysm rupture occurred in 32 to 68 percent of medically treated patients, and rupture accounted for 32 to 47 percent of deaths [14-18]. One-, three-, and five-year survival rates for unrepaired thoracic aneurysms are approximately 65, 35, and 20 percent, respectively. After rupture (or other complications), cardiovascular disease is the second most common cause of death among candidates who do not undergo repair. The overall mortality associated with ruptured TAA is high. Only approximately one half of patients with ruptured TAA live long enough to be transferred to the emergency department for treatment [19]. Without repair, ruptured TAA is nearly uniformly fatal. Unfortunately, regardless of the method of repair, a large portion of patients with ruptured TAA still do not survive [4,20].

Asymptomatic TAA — Elective repair of asymptomatic TAA may reduce the morbidity and mortality associated with TAA complications, but elective repair of asymptomatic TAA is not recommended until the risk of rupture or other complications exceeds the risks associated with repair. Favorable outcomes for open or endovascular repair reinforce the need to repair TAA before it reaches the thresholds described below (diameter, expansion rate), above which the risk of rupture increases sharply [21,22].

The most important determinant of TAA rupture is the diameter of the aneurysm [13,23-26]. Other factors, such as a rapidly expanding aortic diameter, concomitant bicuspid aortic valve, or connective tissue disease, also increase the risk for rupture [9]. (See "Epidemiology, risk factors, pathogenesis, and natural history of thoracic aortic aneurysm and dissection".)

Diameter criteria — The most important determinant of TAA rupture or other complications (eg, dissection) is the diameter of the aneurysm [17,23-25,27]. The diameter threshold to recommend elective repair varies by the location and etiology of the aneurysm, among other factors [17]. One group has shown that the clinical threshold for rupture mirrors the innate physical limits of the aortic wall. As the aorta approaches 6 cm, its distensibility rapidly falls [28]. At this diameter, the aorta loses its natural elasticity and effectively becomes a rigid tube. At a blood pressure of 200 mmHg, easily achieved through strenuous exercise or emotional distress, the stress generated in the wall of a 6 cm aorta can attain or exceed the maximum tensile strength of aortic tissue [28].

Degenerative TAA — For patients with degenerative TAA (ie, not associated with genetically mediated conditions), we suggest elective repair of ascending TAA for end-diastolic aortic diameter >5.5 cm, and elective repair of descending TAA with a diameter >5.5 cm for patients with a low risk for perioperative complications. For higher-risk patients, a descending aortic diameter >6 cm should be repaired [5,8,17,23-25,27]. What constitutes high risk is not well defined. The use of the Society of Thoracic Surgeons Predicted Risk of Mortality (PROM) score >8, frailty score >2, and more than one major organ system impairment has been used in an effort to stratify risk for aortic surgery in those with bicuspid aortic valves [10].

Based on observational studies, the risk of complications of TAA (rupture, dissection) increases with larger aortic diameter, in particular, once the ascending aorta expands beyond 6 cm, or the descending aorta expands beyond 7 cm [27].

An early study of 133 patients with ascending TAA, descending TAA, or both found that the overall five-year risk of rupture was 0 percent for those less than 4.0 cm compared with 16 and 31 percent for TAA 4.0 to 5.9 and ≥6.0 cm, respectively [23]. TAA diameter >6.0 cm was associated with a 15.6 percent per year combined endpoint of rupture, dissection, or death.

In another series of 370 patients, the median diameter at the time of rupture or dissection was 5.9 cm for ascending TAA and 7.2 cm for descending TAA; a diameter greater than 6 cm increased the risk of rupture or dissection by 25 percent for an ascending TAA, while a diameter greater than 7 cm increased the risk by 37 percent for descending TAA [25].

In a later prospective study that included 304 patients with mostly ascending TAA (72 percent), overall rates of dissection or rupture were 2, 3, and 7 percent per year for TAA less than 5.0 cm, 5.0 to 5.9 cm, and ≥6.0 cm, respectively [17]. The combined endpoint of rupture, dissection, or death overall occurred at a rate of 16 percent per year among patients with TAA ≥6.0 cm. The overall five-year survival rate in patients with TAA >6.0 cm was 56 percent. In patients who did not undergo surgery (diameter not specified), 54 percent were alive at five years. By comparison, elective surgery restored survival to a rate similar to a matched control population.

In another review of 216 patients assessed, 41 percent of patients were considered high risk for any intervention [18]. Median TAA diameter was 6 cm. Over a median follow-up of 12 months, 49 of 81 patients died, of which 23 deaths were due to rupture; the others were not aneurysm related.

Even though the risk of dissection or rupture increases with aortic diameter, most patients who present with dissection have smaller-diameter aneurysms. In a review of 591 patients with type A dissection enrolled in the International Registry of Acute Aortic Dissection (IRAD), the mean diameter was 5.3 cm [12]. Nearly 60 percent of patients had an ascending aortic diameter <5.5 cm, and 40 percent had an aortic diameter <5.0 cm.

Clinicians should understand the limitations of basing treatment decisions solely on diameter criteria. Data have called into question the traditional cutoff of 5.5 cm for degenerative TAA, and some have called for potentially lowering this threshold [29]. Reasons for this include prospective natural history studies showing a "hinge-point" for TAA complications at 5.25 cm (in addition to previously described 5.75 cm), recognition that the aorta "increases" in diameter by 7 mm at the moment of dissection, along with increased safety of contemporary elective TAA repair. Further work will be needed in this area to better predict those patients who would benefit from prophylactic TAA repair at smaller diameters.

Inflammatory TAA — Inflammatory diseases such as Takayasu arteritis, giant cell arteritis, Behçet disease, and ankylosing spondylitis are commonly associated with TAAD [4,5]. TAA formation may occur at multiple sites over a period of follow-up. Initial medical therapy for active Takayasu arteritis and active giant cell arteritis reduces the active inflammatory state. Elective surgical repair of TAA for patients with Takayasu arteritis and giant cell arteritis should be delayed until the acute inflammatory state is treated and quiescent. The diameter criteria for inflammatory TAA repair are the same as those for noninflammatory, degenerative TAA [4,5]. (See "Epidemiology, risk factors, pathogenesis, and natural history of thoracic aortic aneurysm and dissection", section on 'Inflammatory disorders' and "Overview of the management of vasculitis in adults".)

Genetically mediated thoracic aortic aneurysm and dissection — Genetically mediated TAAD can be part of a syndrome (ie, syndromic) such as Marfan syndrome (table 2), Loeys-Dietz syndrome, vascular Ehlers-Danlos syndrome, or Turner syndrome, or nonsyndromic, as with familial TAAD and bicuspid aortic valve [30,31]. Prophylactic surgery in the setting of many hereditary TAA disorders is uncertain and may depend upon the specific mutation, aneurysm location, family history, absolute aortic size, and growth rate. It is recognized that TAA due to mutations in SMAD3, ACTA2, and MYLK may lead to dissection at relatively small aortic diameters (figure 1). Decision making in patients with genetically mediated TAAD, particularly in younger patients (<60 years old), takes into account the diameter of the ascending aorta/proximal arch but also the diameter of the aortic root and aortic valve function (table 1) [32].

Marfan syndrome – Marfan patients should generally undergo elective operation at a smaller aortic diameter than patients with degenerative aortic aneurysms to reduce the risk of TAA complications [3,4,33,34]. Surgery is warranted to replace the aortic root and ascending aorta for aortic root diameter of ≥4.5 cm and features associated with an increased risk of aortic dissection, a family history of aortic dissection, rapid aortic expansion (> 0.3 mm per year), diffuse aortic root and ascending aortic dilation, and marked vertebral artery tortuosity. Elective repair at a smaller ascending aortic diameter in surgical centers with expertise in the repair of the aortic root and ascending aorta is reasonable [3,5,35,36]. (See "Management of Marfan syndrome and related disorders" and "Heritable thoracic aortic diseases: Pregnancy and postpartum care".)

Bicuspid aortic valve – A decision for elective repair in patients with bicuspid-aortic-valve-associated aortic dilation depends upon the diameter of the ascending aorta but also on the functional status of the valve [37-41]. Although the aortic valve eventually requires replacement in over 75 percent of patients due to progressive aortic stenosis and/or regurgitation, bicuspid aortic valve is often associated with ascending aortic aneurysm in the absence of significant valvular disease. For some patients, repair of TAA becomes necessary before aortic valve operation is indicated [42]. For asymptomatic patients with bicuspid aortic valve, an ascending aortic diameter >5.5 cm warrants repair (lower for smaller stature); however, for patients with additional risk factors for complications (eg, family history of aortic dissection, rapid expansion >0.5 cm/year), repair at an ascending aortic diameter 5.0 to 5.5 cm, or >4.5 cm in those with severe aortic stenosis or regurgitation, is reasonable to reduce the risk for complications [5]. For those at low risk for aneurysm resection with surgery performed in a center with expertise, surgery at >5.0 cm can be considered [10]. This recommendation is based on data from single-center observational studies of patients who underwent aortic valve replacement for valvular dysfunction. Patients with ascending aortic diameter of 4.0 to 4.5 cm at the time of isolated aortic valve replacement had an increased risk of ascending aortic complications [3,43]. Another retrospective study has also shown that the probability of aortic dissection increases dramatically at an ascending aortic diameter of 5.3 cm and increases gradually at a sinus diameter of 5.0 cm [44]. (See "Bicuspid aortic valve: General management in adults".)

Loeys Dietz syndrome – The Loeys-Dietz syndrome has the smallest recommended diameter threshold among the genetically mediated TAA syndromes owing to its aggressive natural history. Prophylactic aortic root and ascending aortic replacement is generally recommended for ascending aortic diameter between 4.0 to 4.5 cm [4,30,45]. However, the decision also depends on the specific genetic variant (eg, SMAD3, TGFB2, TGFB3, MYH11, MLCK and PRKG1), and other factors such as aortic expansion rate, family history, patient age, and sex [7]. (See 'Rapid expansion' below and "Management of Marfan syndrome and related disorders", section on 'Management of related conditions'.)

Accounting for body size — Absolute diameter criteria for intervention on the aorta do not take into account natural variation in aortic diameter for sex and body size (figure 2A-B). As an example, a small individual (body surface area [BSA] 1.5 m2) with an aortic measurement of 4.5 cm may be at a higher risk for aortic complications compared with a larger individual with a BSA of 2.1 and the same aortic measurement. As such, smaller patients may have a smaller diameter threshold for elective intervention due to the larger relative aneurysm diameter. The role of body size has also been evaluated in patients with Marfan syndrome, who tend to be taller [4].

One suggested criterion for smaller patients is to perform elective repair for aortic diameters greater than twice the diameter of a normal segment of aorta (nonaneurysmal segment). For smaller patients, including many females, we suggest elective repair using this criterion.

While not addressed in the later guidelines [46], some have suggested using an indexing method that helps correct for body size. Elective ascending aortic repair is recommended before the ascending aortic size (ASI) index (aortic diameter [cm] divided by body surface area [m2]) is 2.75 cm/m2 [9]. Additional investigation has explored the possible importance of using height alone, consisting of the aortic height index (AHI [cm/m]), which may be more predictive of aneurysm complications compared with using BSA [47]. However, this approach has not been widely adopted clinically.

Another method to account for body size suggests that for cases of Marfan syndrome, it may be reasonable to perform elective aortic root replacement for patients with an ascending aortic area (cm2) to height (m) ratio of 10 cm2/m [4,5,48]. This index is also being used for those with bicuspid aortic valve-associated aneurysm [49].

Rapid expansion — Once a TAA develops, the natural history is one of progressive enlargement with variable expansion rates [50-52]. Expansion rates range from 1 to 10 mm per year, depending upon TAA etiology, diameter, and location within the aorta [17,18,21,50,53-56]. Rapid expansion is defined as ≥0.5 cm in one year or ≥0.3 cm per year in two consecutive years for those with sporadic aneurysms, and ≥0.3 cm in one year for those with heritable thoracic aortic disease or bicuspid aortic valve. Although, rapid expansion is very rare in the absence of an aortic dissection.

Reports of rapid expansion can reflect measurement errors; however, when rapid expansion is seen, it should raise concern for aortic dissection or aortic infection [21]. Rapid expansion increases the risk of TAA complications; thus, we suggest repair of asymptomatic ascending TAA <5 cm that exhibits rapid expansion, defined as ≥5 mm per year [4,5,11,57]. It is important to note that follow-up should be at the same institution, on the same scanner, so that interobserver variability in assessing size or changes in size does not come into the decision-making process to justify a high-risk operation.

Similar to abdominal aortic aneurysm, larger-diameter TAAs expand more rapidly than smaller-diameter TAAs [58]. This was illustrated in a study of 67 patients with TAA who underwent serial computed tomography (CT) [54]. The rate of expansion for aneurysms >5.0 cm in diameter at diagnosis was 7.9 mm per year but was approximately 1.7 mm per year for aneurysms <5.0 cm. A history of hypertension did not affect the rate of aneurysm expansion. However, these studies are older, and the location and specific etiology of the aneurysm may not have been taken into account. Larger-diameter aortas (>5.0 cm at the time of diagnosis) can have expansion rates up to 15 mm/year (figure 3).

The anatomic location of the aneurysm is another factor associated with the rate of expansion. In a series of 87 patients who underwent vascular imaging, aneurysms located within the mid-portion of the descending aorta showed the most rapid expansion, while those in the ascending aorta had the slowest expansion rate, despite having a greater initial diameter (figure 4) [53]. In general, isolated degenerative ascending aortic aneurysms have an average expansion rate of 1 mm per year, whereas descending TAAs expand by approximately 3 mm per year [58,59].

Saccular aneurysm morphology is associated with aortic infection and increased rates of expansion and rupture compared with fusiform. In one study, the expansion rate of saccular aneurysms was 28 mm/year [60]. As such, saccular aneurysms are often treated more aggressively.

Patients with familial TAAs have faster average rates of expansion at 2 mm per year (combined ascending and descending TAA) [58,61-64]. Marfan syndrome is associated with expansion rates of up to 3 mm per year. An increased aortic expansion rate has also been reported during pregnancy for those with Marfan syndrome compared with those with Marfan syndrome who are not pregnant both during and following pregnancy, in some but not all studies [65-68]. TAAs associated with the particularly aggressive Loeys-Dietz syndrome can expand very rapidly up to 10 mm per year [45].

MANAGEMENT OF ASYMPTOMATIC TAA — Asymptomatic thoracic aortic aneurysms (TAAs) may be detected on routine chest radiograph or during surveillance in a patient with an underlying genetically mediated condition such as Marfan syndrome. (See "Clinical manifestations and diagnosis of thoracic aortic aneurysm", section on 'Incidental TAA'.)

Asymptomatic patients without indications for repair are managed conservatively, which includes the following (see 'Indications for repair' above):  

Measures to reduce cardiovascular risk. (See 'Cardiovascular risk reduction' below.)

Aggressive blood pressure control (≤130 mmHg), and other measures to limit aneurysm expansion. Beta-blockers are the first-line therapy. Angiotensin receptor blockers are a reasonable adjunct to beta blockers to achieve adequate blood pressure control. (See 'Antihypertensive therapies' below and 'Therapies to limit aortic expansion' below.)

Statin therapy is reasonable in those with thoracic aortic aneurysm. (See 'Statin therapy' below.)

Patient education regarding the symptoms and signs that may indicate the development of complications. (See 'Counseling and other evaluation' below.)

Counseling for those suspected of having genetically mediated disease. First-degree relatives of those with TAA disease should be screened, based on family studies demonstrating an approximately 20 percent chance of another first-degree relative having a TAA [58,61]. (See 'Identifying associated genetic conditions' below.)

Screening for associated aneurysmal disease. (See 'Identifying associated aneurysm' below.)

Serial imaging of the aneurysm to evaluate for expansion and changes in extent. (See 'Aneurysm surveillance' below.)

Counseling on exercise and activity limitations is individualized, but in general, contact sports are avoided and most patients are counseled against heavy lifting (defined as half of ideal body weight) and exercises involving sustained Valsalva maneuver.

Cardiovascular risk reduction — Managing cardiovascular risk is important for improving overall outcomes, including outcomes associated with aortic repair, when it is required. Lifestyle modification, including smoking cessation, is an important aspect to medical management in these patients [69-71]. Although it is unknown whether statins reduce the rate of thoracic aortic expansion, most patients with TAA have other indications for statin therapy. Statin therapy should be used to meet low-density lipoprotein (LDL) goals as outlined by multidisciplinary guidelines [4,5]. (See 'Statin therapy' below and "Overview of primary prevention of cardiovascular disease" and "Prevention of cardiovascular disease events in those with established disease (secondary prevention) or at very high risk".)

We agree with major society guidelines that advise patients with aneurysmal disease to stop smoking; these patients should be offered cessation interventions [4]. The management of smoking cessation is discussed in detail elsewhere. (See "Overview of vascular intervention and surgery for vascular anomalies" and "Overview of smoking cessation management in adults".)

Cigarette smoking is strongly associated with aneurysm formation, aneurysm expansion, and aneurysm rupture and is an important modifiable risk factor. Although the majority of studies that have defined these associations have been in patients with abdominal aortic aneurysm (AAA), smoking has also been linked to alterations in mechanical properties of the thoracic aorta in animal models [72]. (See "Management of asymptomatic abdominal aortic aneurysm", section on 'Smoking cessation'.)

Therapies to limit aortic expansion — Much of the data regarding pharmacologic therapy to limit expansion of TAA are derived from mouse model studies, such as fibrillin-1–deficient (Marfan) mice [69,71]. There are emerging studies on the treatment of TAA disease in humans. But, similarly, the majority of these studies have been performed on patients with Marfan syndrome. Whether these results can be generalized to the non-Marfan population is uncertain, but given some shared pathophysiologic pathways, findings of these investigations may indirectly support pharmacologic treatment (antihypertensive therapy, statin therapy) in non-Marfan subpopulations [71,73]. (See "Epidemiology, risk factors, pathogenesis, and natural history of thoracic aortic aneurysm and dissection", section on 'Pathogenesis'.)

Antihypertensive therapies — Based primarily on studies performed in Marfan patients, for patients with asymptomatic TAA who are being conservatively managed, we suggest blood pressure control primarily using beta blocker therapy with the aim of limiting further aortic expansion [4,74,75]. While the theoretical benefit of anti-impulse therapy is widely accepted, no controlled studies have demonstrated a benefit. The goal systolic pressure is 105 to 120 mmHg, if tolerated. The adequacy of beta blockade is usually judged by the heart rate response. An angiotensin-converting enzyme (ACE) inhibitor or angiotensin receptor blocker (ARB) is an acceptable alternative for blood pressure control among those who do not tolerate beta blocker therapy.

For patients with bicuspid aortic valves and a dilated ascending TAA without moderate or severe aortic regurgitation, guidelines on the treatment of valvular disease suggest any effective antihypertensive to control blood pressure. Beta blockers and ARBs have a conceptual advantage but have not been shown to be effective in clinical studies [46,76]. (See "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders".)

Beta blockers – Beta blockers reduce the inotropic state of the heart, decreasing left ventricular contractility (dP/dt) and shear stress, and the impact force of ejected blood on the aorta [77]. A clinical benefit has been demonstrated only in studies of Marfan patients [78-80]. It is not known if these results can be extrapolated to patients without Marfan syndrome, but it is biologically plausible to attempt such therapy. In a 10 year study in which 70 patients with Marfan syndrome were randomly assigned to propranolol or no beta blocker therapy, the treated group had a 73 percent slower rate of aortic expansion and lower mortality after the first four years of follow-up [78]. A retrospective nonrandomized study of beta blocker use in children with Marfan syndrome also demonstrated a slowing of aortic expansion in patients treated with beta blockers compared with untreated controls [80]. However, some studies suggest that beta blockers do not protect against TAA expansion [53,71,81].

ACE inhibitors and ARBs – Experimental research suggests a role for the renin-angiotensin pathways in the formation of aneurysms [80,82-84]. Studies on ACE inhibitors and ARBs have reported conflicting results [71,85-91]. ACE inhibition may have beneficial effects by modifying the inflammatory mediators and by decreasing vascular smooth muscle apoptosis [87]. ARBs may also be important in preventing aneurysm expansion in Marfan syndrome due to downregulation of transforming growth factor beta (TGF-beta) and its effects [88,89]. Losartan may have a protective effect by antagonizing the activity of transforming growth factor (TGF) [88]. In a nonrandomized study of young patients with Marfan syndrome and aortic root dilation, ARB therapy significantly reduced the rate of aortic expansion [88]. Another study reported no benefit of an angiotensin-receptor blocker (Losartan) over a beta blocker (atenolol) with respect to the rate of aortic-root dilation in patients with Marfan syndrome [90]. One interpretation of this study is that ARBs are as effective as beta blockers in the treatment of patients with Marfan syndrome; however, such an interpretation assumes that beta blockers are an effective treatment option. (See "Management of Marfan syndrome and related disorders", section on 'Drug therapy'.)

Statin therapy — Although mostly studied in the context of AAA [92-95], statin therapy may provide a protective effect by inhibiting matrix metalloproteinases (MMPs) and plasminogen activator [96]. In a Marfan mouse model, pravastatin was found to attenuate aortic root aneurysm formation [97]. In one retrospective review of TAA patients, mortality rates were significantly lower among those taking statins compared with those who were not (20 versus 33 percent); this effect was independent of angiotensin blockade [98]. Survival was attributed to a decreased need for surgical repair due to reduced aortic expansion. In another retrospective review, statin therapy was associated with a significant reduction in complications, including aortic dissection [96,99].

Other pharmacologic therapies — There are no data reporting a benefit for any other pharmacologic therapy for limiting aortic expansion for TAA in humans.

Doxycycline also reduces matrix metalloproteinase (MMP) activity, and thus elastin destruction, and has been shown to prevent aortic root aneurysm in fibrillin-1-deficient mice [100]. Small studies using doxycycline have demonstrated attenuation of AAA expansion in human subjects. (See "Management of asymptomatic abdominal aortic aneurysm", section on 'Doxycycline'.)

Avoidance of fluoroquinolones — Fluoroquinolones have been linked to an increased risk of aortic dissection and aneurysm rupture [101]. The US Food and Drug Administration (FDA) has issued a safety advisory that this class of drugs should be avoided in patients with aortic aneurysm or who are at risk for aortic aneurysm [102]. (See "Epidemiology, risk factors, pathogenesis, and natural history of thoracic aortic aneurysm and dissection", section on 'Rupture/dissection' and "Management of asymptomatic abdominal aortic aneurysm", section on 'Fluoroquinolone use'.)

Aneurysm surveillance — Asymptomatic patients who do not meet the diameter criteria for elective TAA repair (open or endovascular) require long-term aneurysm surveillance. Aneurysm surveillance includes ongoing clinical evaluation for the development of symptoms and signs of aneurysm complications, and serial imaging to evaluate the diameter and structure of the aneurysm. The preferred imaging technique depends on the location of the aortic aneurysm; options include echocardiography, CT angiography, or magnetic resonance (MR) angiography. Ideally, the serial studies should be performed using the same technique in the same center for consistency with future comparisons [103]. If a TAA is only moderate in size and remains relatively stable over time, MR rather than CT is reasonable to minimize the patient's radiation exposure [4,5].

In general, we suggest imaging at six months after the initial diagnosis to ensure the stability of the aneurysm diameter and extent [4,52,57]. Further imaging can be performed annually if there is no expansion or extension. However, the surveillance schedule may be modified based upon the etiology, site, and diameter of the aneurysm at presentation. If the aorta demonstrates rapid expansion or is approaching the threshold for surgery or endovascular repair, we suggest more frequent imaging at every three to six months.

A proposed approach to imaging these patients was adapted from guidelines on the management of thoracic aortic disease [7] .

Degenerative aortic root or ascending aortic aneurysm:

3.5 to 4.4 cm: Annual CT or MR angiography, echocardiogram to follow valvular disease (if needed)

4.5 to 5.4 cm: Biannual (every six months) CT or MR angiography, echocardiogram to follow valvular disease (if needed)

Genetically mediated aortic root or ascending aortic aneurysm:

3.5 to 4.4 cm: Annual echocardiogram, CT, or magnetic resonance imaging (MRI)

4.5 to 5.0 cm: Biannual (every six months) echocardiogram, CT, or MRI

Descending aortic aneurysm:

4.0 to 4.9 cm: Annual CT or MR angiography

5.0 to 6.0 cm: Biannual (every six months) CT or MR angiography

When an aortic root or ascending aortic aneurysm is present, transthoracic echocardiogram should be performed to evaluate for the presence of a bicuspid aortic valve (BAV), one of the most common causes of a dilated aorta [3,5]. It is also important to consider body size when evaluating the significance of aortic root dimensions [4]. (See 'Accounting for body size' above.)

COUNSELING AND OTHER EVALUATION

Identifying associated genetic conditions — Patients identified as having thoracic aortic aneurysm or dissection (TAAD) should be evaluated for possible underlying genetic or familial disorders known to be related to TAAD, which may increase their individual risk of aneurysm progression or complications. The evaluation should include a thorough family history to evaluate for sudden or unexplained death and history of aneurysm or dissection. (See "Epidemiology, risk factors, pathogenesis, and natural history of thoracic aortic aneurysm and dissection", section on 'Genetic predisposition'.)

TAA can occur as part of complex genetic syndromes, such as Marfan syndrome (most common), Ehlers-Danlos syndrome, Loeys-Dietz syndrome, and Turner syndrome. However, increasing numbers of genetic loci have been linked to nonsyndromic familial TAAD and suggest that the true proportion of familial TAADs is approximately 20 percent [104,105].

Young age at presentation is an important clue that a patient has genetically mediated TAAD. In a retrospective review at a single institution of 760 patients between 16 and 60 years of age diagnosed with aortopathy, differences in presentation and outcomes were studied [106]. The etiology of genetically mediated TAAD was nonsyndromic TAA in 311, Marfan syndrome in 221, and bicuspid aortic valve disease in 228. The average age was 37. Marfan patients were younger than nonsyndromic TAA and bicuspid aortic valve patients. However, a symptomatic presentation with aortic dissection was more common for nonsyndromic TAA compared with Marfan syndrome or bicuspid aortic valve. (See "Clinical manifestations and diagnosis of thoracic aortic aneurysm", section on 'Determining TAA etiology'.)

If one or more first-degree relatives of a patient with known TAAD are found to have thoracic aortic dilatation, aneurysm, dissection, an abdominal aortic aneurysm, or brain aneurysm, then referral to a geneticist is recommended. First-degree relatives of those with TAA disease should be screened, based on family studies demonstrating an approximately 20 percent chance of another first-degree relative having a TAA [58,61]. Family members (siblings, children) may also require screening. For those with a positive family history, genetic testing can be based on phenotypes most commonly associated with TAA, when they are present (algorithm 1) [107]. A panel of genes can be checked in those patients who do not have clinical characteristics that suggest a specific syndrome.

The 2022 American College of Cardiology (ACC)/American Heart Association (AHA) Aortic Disease Guidelines recommend genetic testing for aortopathy in the following scenarios [7]:

Obtain a complete aortic family history in patients with aortic root/ascending thoracic aortic disease (TAD) or thoracic aortic aneurysm or dissection (ie, TAAD)

Obtain testing for genetic aortopathy in patients with TAD or TAAD age <60 years, family history of TAD or intracranial aneurysm, and those with phenotypic features of genetic aortopathy.

For patients with positive genetic testing:

Obtain cascade testing of at-risk biologic relatives.

Obtain transthoracic echocardiogram or CT angiography to screen for aneurysm in family members with positive genetic testing.

Provide genetic counseling for positive aortopathy screening.

Inheritance patterns for familial bicuspid aortic valve associated with TAA are consistent with an autosomal dominant pattern with incomplete penetrance [108]. Recommendations for screening are discussed separately. (See "Clinical manifestations and diagnosis of bicuspid aortic valve in adults", section on 'Genetics'.)

Identifying associated aneurysm — Thoracic aortic aneurysm is associated with aneurysmal disease affecting other vascular beds. This association is likely related to a common pathophysiology, which is distinct from that of the ascending aorta [109]. As such, we have a low threshold to screen TAA patients for abdominal aortic aneurysm (AAA), intracranial aneurysm, and aneurysms affecting the lower extremities (iliac, femoral, popliteal). For patients with Loeys-Dietz syndrome, head-to-pelvis imaging is recommended upon diagnosis and periodically thereafter. (See "Epidemiology, risk factors, pathogenesis, and natural history of thoracic aortic aneurysm and dissection", section on 'Pathogenesis'.)

AAA is present in approximately 20 percent of patients diagnosed with TAA [110]. In an imaging review, AAA was more positively associated with arch and descending TAAs, particularly those located near the diaphragm [110]. The associations of lower extremity aneurysms with AAA and TAA are reviewed elsewhere. (See "Clinical features and diagnosis of abdominal aortic aneurysm", section on 'Imaging asymptomatic patients' and "Iliac artery aneurysm" and "Popliteal artery aneurysm".)

TAA is also associated with intracranial aneurysm (ICA). In a review of 212 patients with TAA, the prevalence of ICA was 9.0 percent, which was ninefold greater than that in the general population [111]. (See "Screening for intracranial aneurysm".)

Counseling the high-risk patient — Asymptomatic patients with TAA meeting criteria for repair who have severe medical comorbidities who are judged to be high risk for repair (open surgical or endovascular) may be best managed conservatively, reserving repair only if symptoms develop (including rupture).

Patient with Marfan syndrome who are pregnant, bicuspid aortic valve, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome have an increased risk for complications related to TAA [112]. Counseling females with TAA regarding the risks of pregnancy and determining the timing of repair relative to planned pregnancy is discussed separately. (See 'Symptomatic (nonruptured) and ruptured TAA' above.)

(See "Heritable thoracic aortic diseases: Pregnancy and postpartum care".)

(See "Bicuspid aortic valve: Management during pregnancy".)

(See "Overview of the management of Ehlers-Danlos syndromes", section on 'Reproductive options and pregnancy'.)

Recommendations for activity and sports participation among patients with connective tissue disorders, including Marfan syndrome, are discussed separately. (See "Management of Marfan syndrome and related disorders", section on 'Restriction of strenuous activity'.)

THORACIC ANEURYSM REPAIR — Prophylactic repair of thoracic aortic aneurysm (TAA) for appropriate diameter/expansion criteria is recommended to prevent the morbidity and mortality associated with aneurysm rupture/dissection. The five-year survival rate after elective open surgical repair of a TAA in contemporary series is approximately 85 percent. Emergency surgery for TAA complications has much worse outcomes, with a five-year survival rate of 37 percent [17]. There are no trials comparing endovascular repair versus medical management, but long-term survival is expected to be similar to open repair. (See 'Open versus endovascular repair of descending TAA' below.)

The timing of repair for a particular individual with TAA is individualized since the natural history is variable, particularly for aneurysms <5.0 cm in diameter [17], and the majority of patients have concomitant cardiovascular disease that increases the risks associated with surgery. Many patients die of other cardiovascular causes before the aneurysm ruptures. (See 'Indications for repair' above.)

Where expertise in the management of thoracic aortic disease is not available, the patient with TAA should be referred (or transferred for symptomatic patients) to a high-volume cardiovascular center to provide the best possible outcome [6].

Prior to TAA repair, the patient should undergo cardiovascular risk assessment. These issues are discussed in detail separately. (See "Evaluation of cardiac risk prior to noncardiac surgery".)

In addition:

Preoperative assessment should include evaluation of left ventricular function, valve disease, and potential concomitant coronary artery disease.

Patients with ascending aortic and particularly arch disease should undergo carotid artery duplex ultrasound examination. (See "Evaluation of carotid artery stenosis".)

Patients with descending thoracic aortic disease who require left thoracotomy should undergo pulmonary function testing if clinical symptoms of pulmonary disease are present. (See "Overview of pulmonary function testing in adults".)

Patients with TAA that extends distally should be assessed for symptoms and signs of peripheral artery disease. If present, noninvasive lower extremity pulse-volume recordings and pressures will determine the level and severity of disease to serve as a baseline for future comparison. (See "Noninvasive diagnosis of upper and lower extremity arterial disease".)

Approach to repair — Ascending TAA is managed with an open surgical approach using cardiopulmonary bypass and sometimes requires aortic root replacement with coronary artery reimplantation. Descending TAA can be repaired with an open or endovascular approach, or a combination of the two (hybrid repair).

Open surgery – The nature of open surgery varies depending upon the location and extent of the aneurysm. Open repair of ascending TAA is performed via a median sternotomy with cardiopulmonary bypass including cardioplegia and often requires aortic root replacement or coronary artery reimplantation. The diseased aortic segment is typically replaced with either a composite graft (ie, aortic valve replaced) or valve-sparing procedure [113]. For bicuspid aortic valve, the valve can be replaced or repaired at the time of aortic surgery [114]. When the aortic arch is involved, hypothermic circulatory arrest needs to be instituted typically with antegrade or retrograde cerebral perfusion. The aortic arch can be repaired via a hemi-arch technique or total arch replacement. Open repair of descending TAA is via a left thoracotomy and often does not require full cardiopulmonary bypass or cardioplegia; however, measures to protect the spinal cord are important. End-organ revascularization (visceral, renal) may require native arterial reimplantation with or without endarterectomy, or bypass grafting. (See "Overview of open surgical repair of the thoracic aorta".)

Endovascular repair – Thoracic endovascular aneurysm repair involves the placement of modular graft components delivered via the iliac or femoral arteries to line the descending thoracic aorta and exclude the aneurysm from the circulation. Endovascular repair requires fulfillment of specific anatomic criteria, and the greatest experience involves treatment of descending TAA that does not involve the abdominal visceral segment. The role of stent-grafting for extensive thoracoabdominal aortic disease, which requires debranching procedures or specialized grafts (fenestrated, branched endografts), is an area of active clinical research. Although endovascular repair is associated with lower perioperative mortality, late complications, including graft migration and aortic rupture, have been reported. Newer generation endografts specifically designed for use in the aortic arch, both single-branch and multi-branch, are in clinical trials for use in arch pathology. (See "Endovascular repair of the thoracic aorta".)

Hybrid repair – A hybrid approach involves typically an open approach to manage the ascending aorta or aortic arch, with an endovascular approach for the descending thoracic aorta (ie, frozen elephant trunk) [115-123]. (See "Overview of open surgical repair of the thoracic aorta".)

Open versus endovascular repair of descending TAA — The choice of approach to repair (open versus endovascular) for descending TAA should take into account the etiology (degenerative, genetically mediated, infectious), location in the descending aorta, and extent of the aneurysm, and the patient's expected survival (short-term and long-term), which depends upon the patient's age and medical comorbidities.

Degenerative versus genetically mediated aneurysm — Thoracic aortic aneurysm/dissection can be degenerative or related to genetically mediated disorders (nonsyndromic or syndromic). The etiology of descending aneurysmal disease determines the preferred approach to repair. (See "Clinical manifestations and diagnosis of thoracic aortic aneurysm", section on 'Determining TAA etiology'.)

For patients with degenerative descending TAA (asymptomatic, symptomatic) that does not involve the visceral segment, and anatomy that is otherwise suitable for endovascular repair, we use an endovascular rather than open surgical approach to repair. Endovascular repair is associated with reduced perioperative morbidity and mortality; however, the long-term durability of endovascular TAA repair compared with open surgical repair remains uncertain. Endovascular repair combined with a debranching procedure may still be an option for distal TAA but cannot generally be performed in an urgent setting given the need for customized endovascular devices. (See 'Thoracic aneurysm repair' above and "Endovascular repair of the thoracic aorta" and 'Elective repair' below and 'Emergency repair' below.)

For patients with syndromic descending TAA, we use an open surgical approach to replace the descending thoracic aorta, rather than endovascular stent-graft placement. Unfavorable late aortic remodeling at the site of stent-graft placement has been reported, likely related to the persistent radial force of these devices against the abnormal aortic tissue. However, some feel that stent-grafting may be justified in emergencies as a "bridging" method to later definitive surgical repair, though elective repair has also been reported [124]. The optimal approach for those with nonsyndromic TAA is still unknown as there are no large data sets evaluating stent grafting in nonsyndromic TAA. Therefore, depending on the extent of repair needed, patient age, and other comorbidities, either approach may be appropriate. (See "Endovascular repair of the thoracic aorta", section on 'Contraindications'.)

Elective repair — There are no randomized trials comparing endovascular with open surgery for the repair of descending TAA, but the bulk of the evidence from observational studies argues for an endovascular rather than open approach in those patients who are candidates for both, largely due to the 30 day morbidity and mortality benefit [125-127]. Long-term durability continues to be the main concern as the rate of secondary endovascular interventions following thoracic stent-grafting ranges from 10 to 32 percent, while the rate of secondary open operation ranges anywhere from 0.4 to 7.9 percent [128].

Systematic reviews of observational studies have found significantly reduced rates of perioperative morbidity and mortality in the short term (odds ratio [OR] 0.36; 95% CI 0.23-0.58 [125]), and reduced length of hospital stay for an endovascular compared with an open approach [125-127]. The risk of major neurologic injury was also lower for the endovascular approach (OR 0.39; 95% CI 0.25-0.62) [125].

In a prospective study comparing thoracic aortic aneurysm endovascular repair with open repair, perioperative events, including myocardial infarction, respiratory events such as pneumonia or ventilation for more than 24 hours, stroke, and paraplegia, were combined into a composite score [129]. The percentage of patients who experienced at least one event was significantly lower in the endovascular group compared with open surgery (9 versus 33 percent).

In two smaller studies, each of which included around 230 patients, perioperative mortality was significantly lower with endovascular repair (2.1 versus 11.7 percent early after surgery, and 1.9 versus 5.7 percent at 30 days) [129,130].

Patients in the study cited above were followed for five years [131]. Aneurysm-related mortality was lower for endovascular repair (2.8 versus 11.7 percent), most of which was attributable to fewer perioperative deaths. Major adverse events, defined as prolongation of treatment, new hospitalization, major disability, or death, were also lower (58 versus 79 percent). No significant differences were seen for survival (68 versus 67 percent) or the rate of aneurysm-related reintervention (3.6 versus 2.1 percent).

Nevertheless, there remain concerns over the validity of these comparisons due to the following concerns [132]:

Many studies compare endovascular repair with older surgical techniques or operations with greater extents of repair [133]. In a later single-center retrospective study of over 700 patients who received either endovascular repair or open surgery, mortality was not significantly different at 30 days (5.7 versus 8.3 percent, respectively) and 12 months (15.6 versus 15.9 percent, respectively) [134].

Many patients enrolled in endovascular repair studies were not eligible for open surgery due to comorbidities.

Many patients enrolled in open surgery studies include patients who would not be eligible for endovascular repair because of anatomic constraints [135].

Emergency repair — Patients treated for symptomatic TAA (eg, rupture, dissection) have increased perioperative mortality. Among this subpopulation, endovascular repair of symptomatic/ruptured TAA is also associated with improvements in perioperative morbidity and mortality [136-140]. The outcomes of endovascular versus open surgery in the emergency setting were specifically addressed in a nonrandomized study of 60 consecutive patients with acute rupture of the thoracic aorta; 28 patients were treated surgically and 32 were treated with an endovascular stent-graft [140]. The following findings were reported:

Perioperative mortality was significantly lower with the endovascular approach compared with open surgery (3.1 versus 17.8 percent).

At a mean follow-up of 36 months, four additional deaths occurred in patients who received stent-grafts; three were attributed to late procedural complications (one aneurysm, one dissection, and one traumatic transection). No additional procedure-related deaths occurred in the surgical patients.

Reintervention rates were similar between the groups. All of the surgical reinterventions were early in the postoperative course for bleeding. In the patients treated with stent-grafts, one required early drainage of an empyema, and two required late interventions for endovascular leaks (one repeat stent, complicated by paraplegia; one surgical repair with stent-graft removal).

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Aortic and other peripheral aneurysms".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

Basics topics (see "Patient education: Thoracic aortic aneurysm (The Basics)")

SUMMARY AND RECOMMENDATIONS

Patient evaluation Patients diagnosed with thoracic aortic aneurysm (TAA) should be evaluated for possible underlying genetic syndromes known to be related to thoracic aortic aneurysm and dissection (TAAD). The patient should also be evaluated for other associated aneurysms (eg, brain, abdominal aorta, mesenteric, iliac, femoral, or popliteal arteries) using CT angiography or ultrasound. Among symptomatic patients, this evaluation is obtained postoperatively. (See 'Management of asymptomatic TAA' above and 'Identifying associated genetic conditions' above and 'Identifying associated aneurysm' above.)

Repair of symptomatic TAA – Patients who develop symptoms attributable to TAA should undergo urgent repair (open surgical, endovascular), provided the risk for repair is not prohibitive. Symptoms in a patient with TAA (known or unknown) like chest pain can represent rapid aneurysm expansion or be due to a variety of life-threatening complications, including aortic dissection, acute aortic regurgitation, aortic leakage, or overt aortic rupture. (See 'Symptomatic (nonruptured) and ruptured TAA' above and "Clinical manifestations and diagnosis of thoracic aortic aneurysm", section on 'Symptomatic TAA'.)

Surveillance and medical management of asymptomatic TAA – Most TAAs produce no symptoms; however, the natural history of TAA is one of progressive expansion, which depends upon the location and diameter of the aneurysm and its underlying etiology. Aneurysm diameter is the most important factor determining the risk for TAA complications.

Patients with asymptomatic TAA should be followed for the development of signs and symptoms that may be associated with the TAA. The surveillance schedule is based upon the etiology, site, and diameter of the aneurysm at presentation, and expansion rates identified at follow-up. Ideally, serial CT or magnetic resonance (MR) angiography studies should be performed using the same imaging technique at the same center. (See 'Aneurysm surveillance' above.)

-Following the initial study that identified TAA, we suggest imaging six months after the initial study to ensure the stability of the aneurysm diameter and extent.

-If the TAA does not demonstrate expansion after six months, we suggest annual imaging.

-If the TAA demonstrates expansion or is approaching the diameter threshold for repair, we suggest imaging every three to six months.

For patients with asymptomatic TAA who are being conservatively managed, control of hypertension is recommended to limit further aortic expansion. We suggest beta blocker therapy rather than other agents (Grade 2C). The goal systolic pressure is 105 to 120 mmHg, if tolerated. An angiotensin-converting enzyme (ACE) inhibitor or angiotensin receptor blocker (ARB) for blood pressure control is an acceptable alternative for those who do not tolerate beta blocker therapy. Most patients have concomitant cardiovascular disease; thus, managing cardiovascular risk factors (eg, smoking cessation, antiplatelet therapy, statin therapy) is also important. Statin therapy may also reduce adverse outcomes related to TAA. (See 'Antihypertensive therapies' above and 'Management of asymptomatic TAA' above.)

Repair of asymptomatic TAA – Elective repair of asymptomatic TAA is not undertaken until the risk of rupture or other complications exceeds the risks associated with repair. For most patients with asymptomatic TAA, we use the defined threshold criteria defined above for elective repair (table 1). Candidates for elective repair are selected based upon diameter, location, expansion rate, and patient comorbidities, accounting the presence of underlying contributing etiologies. For patients who do not meet the defined criteria, we suggest not performing elective repair (Grade 2C). These patients are followed conservatively and are considered for repair if symptoms develop. (See 'Summary of indications' above.)

Approach to TAA repair – The approach to TAA repair (open, endovascular) takes into account the location and anatomic extent of the aneurysm, etiology, and the patient's expected survival (short-term and long-term), which depends upon the patient's age and medical comorbidities. (See 'Thoracic aneurysm repair' above and "Endovascular repair of the thoracic aorta" and "Overview of open surgical repair of the thoracic aorta".)

Ascending TAA is traditionally managed with an open surgical approach using cardiopulmonary bypass and often requires aortic root replacement and/or coronary artery reimplantation.

Descending TAA can be repaired with an open or endovascular approach, the choice of which depends upon whether the etiology is degenerative or genetically mediated. For patients with degenerative descending TAA, we suggest an endovascular approach for initial repair rather than an open approach, provided the thoracic aortic anatomy is suitable for endografting (Grade 2C). Endovascular repair is associated with reduced perioperative morbidity and mortality; however, the long-term durability of endovascular TAA repair compared with open surgical repair remains uncertain. An open approach should be used for those with syndromic TAAD. The approach for those with nonsyndromic TAAD is less well defined.

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Emile R Mohler, III, MD (deceased), who contributed to an earlier version of this topic review.

The UpToDate editorial staff acknowledges Y Joseph Woo, MD and Christina L Greene, MD, who contributed to an earlier version of this topic.

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Topic 8189 Version 34.0

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