Thoracic Aortic Aneurysmal Disease.pdf

CONSENSUS RECOMMENDATIONS Thoracic Aort...

CONSENSUS RECOMMENDATIONS Thoracic Aortic Aneurysmal Disease: Comprehensive Recommendations for the Primary Care Physician Prajwal Reddy, MD; Kaavya S. Nair, DO; Vinayak Kumar, MD, MBA; Juan M. Bowen, MD; David R. Deyle, MD; Alberto Pochettino, MD; Heidi M. Connolly, MD; and Nandan S. Anavekar, MB, BCh Abstract Thoracic aortic aneurysm (TAA) is a commonly encountered disease that is defined as aortic dilation with an increase in diameter of at least 50% greater than the expected age- and sex-adjusted size. Thoracic aortic aneurysms are described by their size, location, morphology, and cause. Primary care clinicians and other noncardiologists are often the first point of contact for patients with TAA. This review is intended to provide them with basic information on the differential diagnosis, diagnostic evaluation, and medical and surgical management of TAAs. Management decisions depend on having as precise a diagnosis as possible. Fortunately, this can often be achieved with a stepwise diagnostic approach that incorporates imaging and targeted genetic testing. Our review includes recommenda- tions. In this review, we discuss these issues at a basic level and include recommendations for patients considering pregnancy. ª 2023 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved. n Mayo Clin Proc. 2024;99(1):111-123 From the Department of A ortic aneurysmal disease is defined years, with overall stable incidence Cardiology (P.R., V.K., as aortic dilation with an increase based on the Rochester Epidemiology Proj- H.M.C., N.S.A.), Depart- in diameter of at least 50% greater ect database.3-5 ment of Internal Medicine (J.M.B.), Department of than expected for the same aortic segment We describe a methodical approach to Clinical Genomics in unaffected individuals of the same age the diagnosis and management of a dilated (D.R.D.), Department of and sex. Aortic aneurysms are described by ascending aorta. The steps include a thor- Cardiovascular Surgery (A.P.), Department of their size, location, morphology, and cause. ough family history, a directed physical ex- Radiology (N.A.S.), Mayo There are marked structural differences be- amination, multimodality imaging, and Clinic, Rochester, MN; and College of Osteopathic tween the thoracic and abdominal aorta. often genetic testing. Medicine, Kansas City Different embryologic origins of these seg- University, Kansas City, ments likely lead to these differences and MO (K.S.N.). CLASSIFICATION the resultant pathologic heterogeneity.1 Most individuals with aortic disease are diag- Genetic Diseases Affecting the Aorta nosed incidentally in the asymptomatic stage Marfan syndrome (MFS), Loeys-Dietz syn- or identified in the setting of syndromic dis- drome (LDS), vascular Ehlers-Danlos syn- ease patterns. Medial degeneration with frag- drome, Turner syndrome, bicuspid aortic mentation of elastic fibers is the underlying valve (BAV), and familial thoracic aortic mechanism for thoracic aortic aneurysm aneurysm disease are some of the common (TAA) formation.2 Microscopic changes in genetic conditions that are associated with genetic aortopathies resemble degenerative TAA. Some are single gene disorders, changes but occur at an earlier age.3 The whereas others are more complex/polygenic. incidence of TAA is estimated to be around The inheritance pattern in MFS and LDS is 10.4 to 13.8 cases per 100,000 person- autosomal dominant. It is possible for the Mayo Clin Proc. n January 2024;99(1):111-123 n https://doi.org/10.1016/j.mayocp.2023.07.004 111 www.mayoclinicproceedings.org n ª 2023 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved. Descargado para Anonymous User (n/a) en National Autonomous University of Mexico de ClinicalKey.es por Elsevier en febrero 21, 2024. Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2024. Elsevier Inc. Todos los derechos reservados.  MAYO CLINIC PROCEEDINGS family history to be negative when a new patients, those with likely syndromic aortop- genetic sequence variation has developed in athy, and those with a familial pattern of the index patient. In familial thoracic aortic aortic aneurysm. Disease-causing mutations aneurysm disease, genetic testing has a lower and the pattern of aortic involvement are yield than in syndromic aortopathies but still outlined in Table 2. identifies a disease-causing mutation in The use of genetic testing is clearly estab- approximately 20% of cases.6,7 The inheri- lished in syndromic conditions such as MFS tance pattern in BAV is best described as fa- and LDS. The 2022 American College of Car- milial clustering, and genetic testing results diology/American Heart Association (ACC/ are usually negative. AHA) guidelines for management of aortic A thorough family history may produce disease also recommend genetic testing in evidence of TAA, a syndromic condition, patients with a positive family history, espe- BAV, or premature death in family members. cially if they are younger than 60 years.9 A A directed physical examination looks for medical genetics consultation helps to deter- extracardiac and cardiac features of connec- mine which patients will benefit from aort- tive tissue dysplasia. Skin examination find- opathy panel testing and is also useful in ings include striae, atrophic scars, and the interpretation of results and in patient translucent skin. Musculoskeletal connective counseling. tissue findings include tall stature, long limbs, scoliosis, and pectus deformities. Car- Degenerative Aortic Disease diac findings are those that suggest BAV and Degenerative disease is the most common myxomatous mitral valve disease, including cause of TAA in older patients. Involvement aortic ejection clicks, aortic ejection and of the descending thoracic aorta is more regurgitant murmurs, and apical mid- common than in genetic aortopathies.10 It systolic clicks with late systolic or holosys- is important to consider age, sex, and body tolic apical murmurs. Table 1 lists the phys- size when interpreting aortic dimensions. ical examination findings that suggest The estimated normal rate of aortic expan- syndromic aortopathy. sion is about 0.9 mm in men and 0.7 mm Multimodality imaging is used to identify in women for each decade of life.11 aortic aneurysms, arterial aneurysms, and Unnecessary follow-up testing can be arterial tortuosity. In our clinic, physicians avoided when older patients with an appar- and advanced practice providers caring for pa- ently enlarged aorta are found to be within tients at risk for aortopathy obtain echocardi- normal limits when accounting for age, ography as well as computed tomography sex, and body size. angiography (CTA) or magnetic resonance angiography (MRA) of the entire aorta at the Inflammatory Aortic Disease initial evaluation. In patients with BAV, 4- Aortitis can be either noninfectious (rheuma- dimensional flow magnetic resonance imag- tologic) or infectious. Noninfectious ing (MRI) can be used to identify altered inflammatory aortopathies are more common flow patterns and aortic wall shear stress than infectious causes. In those older than 60 that can cause aortic dilation.8 Although this years, large vessel vasculitis caused by giant technology may provide additional insights cell arteritis (GCA) is the most common into aneurysm progression, it remains investi- form of aortitis.12 In those younger than 60 gational and is currently not used in the years, Takayasu arteritis is the most common decision-making algorithm to select patients form of aortitis. Other forms of aortitis for prophylactic surgery. include granulomatosis with polyangiitis, sys- Genetic testing is now widely available temic lupus erythematosus (SLE), rheuma- and lower in cost than in the past. Several toid arthritis, Behçet disease, and laboratories offer TAA genetic testing panels sarcoidosis. Infectious aortitis is less common that are often covered by medical insurance. but should be considered in the appropriate Genetic testing is most useful in young setting. Causative organisms include n n 112 Mayo Clin Proc. January 2024;99(1):111-123 https://doi.org/10.1016/j.mayocp.2023.07.004 www.mayoclinicproceedings.org Descargado para Anonymous User (n/a) en National Autonomous University of Mexico de ClinicalKey.es por Elsevier en febrero 21, 2024. Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2024. Elsevier Inc. Todos los derechos reservados. THORACIC AORTIC ANEURYSMAL DISEASE TABLE 1. Physical Examination Findings Coinciding With Genetic Syndromic Aortopathies Organ System Physical exam findings Ophthalmology Ectopia lentis, retinal detachment, myopia, glaucoma, cataract Skin Striae, atrophic scars, translucent skin, easy bruising Orthopedic Joint hypermobility, cervical vertebral abnormality, club feet or joint contractures, joint laxity, pectus deformity, scoliosis, dural ectasia, protrusio acetabuli Staphylococcus, Salmonella, Treponema pal- the aortic root and ascending aorta. Trans- lidum (syphilis), and Mycobacterium.12 thoracic echocardiography does not provide Giant cell arteritis is a disorder that gener- a comprehensive assessment of the entire ally affects persons of advanced age and is aorta but is useful in the initial diagnosis more common in White women.12,13 In the or for routine follow-up if the aneurysmal Olmsted County, Minnesota, population, the segment is reliably visualized. The aortic average age- and sex-adjusted incidence of root and proximal ascending aorta are gener- GCA in individuals 50 years or older during ally easily visualized, whereas the mid- a 50-year period was 18.8 per 100,000 per ascending aorta requires dedicated views. person-year.12 Patients may have typical tem- The distal ascending, arch, and remainder poral arteritis symptoms, but the clinical pre- of the descending thoracic aorta are gener- sentation is variable and aortitis may even be ally incompletely visualized with TTE in asymptomatic.13 A recent study in 1450 the adult patient. Echocardiography has the affected patients reported that GCA added benefit of assessment of valvular heart frequently affects the thoracic aorta and that disease, which is frequently seen with 45% of patients had aortic sequelae of dis- aortopathies. ease.13 The pattern of inflammation is often Transesophageal echocardiography (TEE) nonspecific with overlapping patterns.10 has a more limited role in follow-up of aortopa- Diagnosis is based on a combination of clin- thies given its invasive nature and difficulty ical, imaging, and pathologic findings. providing comparable views for comparing se- rial dimensions. However, the aorta including Chronic Dissection the aortic root, ascending aorta, arch, and Although mortality for acute dissection can be descending thoracic aorta are generally easily high, the mainstay for patients presenting with visualized. The role of TEE is primarily related type B dissection is medical therapy with to the diagnosis of aortopathies and intraoper- aggressive blood pressure and heart rate con- ative imaging. Assessment of the thoracic aorta trol.14 Despite initial stabilization with medical is part of routine TEE evaluation in most echo- treatment, many go on to have aneurysmal cardiography laboratories. dilation that warrants future intervention. In a systematic review, thoracic aortic growth Computed Tomography was seen between 7% and 25% across the Computed tomography angiography with selected studies in patients with chronic aortic intravenous iodinated contrast is the primary dissection.15,16 Studies are currently underway modality used in the comprehensive assess- to assess which patients presenting with type B ment of the thoracic aorta. The entire aorta dissection benefit from earlier interventional and the proximal branch vessels are easily therapy, with one of the outcomes being visualized with electrocardiogram-gated decreasing aneurysmal degeneration.14 acquisition. Computed tomography angiog- raphy can readily identify acute disease states such as aortic dissection, intramural IMAGING hematoma, or penetrating ulcer. Accurate Echocardiography reproducible measurement requires multi- Transthoracic echocardiography (TTE) is planar reconstructions, using the double frequently the initial test for screening of oblique method, which creates a reformatted Mayo Clin Proc. n January 2024;99(1):111-123 n https://doi.org/10.1016/j.mayocp.2023.07.004 113 www.mayoclinicproceedings.org Descargado para Anonymous User (n/a) en National Autonomous University of Mexico de ClinicalKey.es por Elsevier en febrero 21, 2024. Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2024. Elsevier Inc. Todos los derechos reservados.  MAYO CLINIC PROCEEDINGS TABLE 2. Genetic Causes of Aortopathies and the Corresponding Pattern of Aortic Involvementa Genetic Syndrome Geneb Usual pattern of vascular involvement Marfan syndrome Fibrillin-1 (FBN1) Aortic root Vascular Ehlers-Danlos syndrome COL3A1 Variable: aorta and branches of the aorta Loeys-Dietz syndrome TGF-b receptors (TGFBR1 or TGFRB2), TGFB2, Aortic root, ascending aorta, branches of the aorta TGFB3, SMAD3 including arteries of the head and neck Turner syndrome XO syndrome Aortic root, ascending aorta, arch Bicuspid aortic valve No single mutation Aortic root, ascending aorta Familial thoracic aortic aneurysm disease Multiple mutations: genetic testing results often Ascending aorta negative a TGF-b, transforming growth factor b. b For expansion of gene symbols, see www.genenames.org. anatomic view of the aorta in a plane perpen- MRA provides superior anatomic assessment dicular to blood flow. The double oblique of the entire aorta compared with TTE but method corrects the oblique course of the can also effectively characterize inflamma- ascending aorta along with accounting for tory medial changes to the aorta. Magnetic aortic tortuosity.17,18 This technique is the resonance imaging with gadolinium contrast standardized approach recommended in enhancement is the preferred noninvasive guideline statements, as nonstandard ap- modality for the diagnosis of aortitis, proaches to measurements may lead to unre- revealing late gadolinium enhancement, liable reporting with consequent conflict in vessel wall edema, and wall thickening using management approach.9 T2-weighted imaging.12 Despite specialized Computed tomography angiography has MRI imaging protocols, the use of this mo- several advantages over other imaging mo- dality for disease activity remains controver- dalities, including short scan time, the ability sial. One large cohort study in patients with to obtain a 3-dimensional data set of the Takayasu arteritis with assessment of vessel entire aorta, wide availability, and low oper- wall edema by MRA did not reveal a strong ator dependence.17 The disadvantages of correlation with clinical disease activity.12 CTA include poor characterization of active Overall limitations include general availabil- inflammation, along with the risk of iodin- ity, feasibility in patients with renal dysfunc- ated contrast nephropathy and ionizing radi- tion, and limitation in patients with ation. Many experts suggest performing nonconditional implants or devices. computed tomography (CT) or other cross- sectional imaging at the time of initial aneu- Positron Emission Tomography rysm diagnosis and before planned operative The above noninvasive modalities can find intervention because of the comprehensive aortic wall thickening and peri-aortic inflam- assessment of the entire aorta and branch mation. However, they can be unreliable in vessels. The ability to assess the coronary identifying early inflammatory changes and arterial system using CTA has a clear advan- structural changes after treatment, which tage in preoperative planning and may avoid typically lag in their resolution.19 18F-fluoro- the need for invasive coronary angiography. deoxyglucose positron emission tomography (FDG-PET) is increasingly used because of Magnetic Resonance Imaging its advantage of identifying and monitoring Magnetic resonance angiography is response to therapy with its qualitative commonly used in the surveillance of TAA, assessment of metabolically active cells. especially in young patients who require The normal vascular wall has no FDG up- routine follow-up, because of the benefit of take; however, areas of inflammatory change not requiring ionizing radiation. As in CT, reveal avid FDG uptake. Retrospective n n 114 Mayo Clin Proc. January 2024;99(1):111-123 https://doi.org/10.1016/j.mayocp.2023.07.004 www.mayoclinicproceedings.org Descargado para Anonymous User (n/a) en National Autonomous University of Mexico de ClinicalKey.es por Elsevier en febrero 21, 2024. Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2024. Elsevier Inc. Todos los derechos reservados. THORACIC AORTIC ANEURYSMAL DISEASE and varied measurement technique of the D C aortic root (Figure 1). B A The American Society of Echocardiogra- phy guidelines recommend a leading-to- leading edge measurement of the thoracic aorta for adults.22 The aortic root diameters are frequently underestimated by TTE because of the parasternal long axis cutting through the minor axis of the aortic root. The aortic root should be measured in long and short axis by TTE. Moreover, asymmetry of the aortic root is not uncommon, espe- FIGURE 1. A and B, Leading-to-leading edge cially in the context of BAV, in which the technique used with transthoracic echocardi- annulus commonly has a minor and a major ography. C and D, Variability in measurement axis in cross section.21 techniques for computed tomography and magnetic resonance imaging, with most using There is controversy over measurement the inner-to-inner edge method (panel C). techniques for CT and MRI, with most using the inner-to-inner edge method. In short, it is common to have a variation of 2 to 3 mm between modalities along with inter- studies using FDG uptake graded for reader variability.21 severity correlated well with traditionally Given the above discrepancies, a clini- used markers such as C-reactive protein cian should acknowledge these limitations level and erythrocyte sedimentation rate.19 in the clinical decision making for elective This suggests that FDG-PET/CT can reliably aneurysm repair or in serial monitoring stra- detect early improvement in disease post- tegies. The differences in anatomic land- therapy, and persistent activity is an marks used across the imaging modalities indicator of nonresponders to therapy.19 and the differences in imaging technique il- Semiquantitative methods of using standard- lustrates the etiology of discrepancies that ized uptake value can be a more objective are often observed in clinical practice, high- assessment of hypermetabolism, which is lighting the necessity of intra-institutional valuable for correlation with treatment standardization and integration of clinical response. One potential limitation of using and imaging practice. FDG-PET is differentiation of the uptake be- tween the atheromatous plaque and inflam- matory aortitis. Nuclear radiologists need Patients With Abnormal Renal Function to take great care in interpretation, as there Specialized scanning sequences with both are no clear quantitative criteria that can MRI and CT are available, including contrast differentiate these 2 entities, particularly in dose reduction strategies. Moreover, MRI elderly patients with substantial atheroscle- without contrast using bright blood or black rotic burden.20 blood sequences can be considered.23 Echo- cardiography remains a first-line assessment Measuring the Aorta in patients with abnormal renal function but It is common to see aortic measurement dis- is limited in its evaluation of the entire crepancies with both single and multimodal- thoracic aorta and ensuring measurement ity imaging; this is in large part due to of the largest diameter as outlined above. nonstandardized measuring techniques. In their review of discrepancies in aortic mea- Surveillance surement, Elefteriades et al21 highlighted The average rate of expansion of TAA is esti- the main issues including measuring in sys- mated to be up to 1 mm/y in degenerative tole vs diastole, including the aortic wall, aortic disease. Genetic aortopathies have Mayo Clin Proc. n January 2024;99(1):111-123 n https://doi.org/10.1016/j.mayocp.2023.07.004 115 www.mayoclinicproceedings.org Descargado para Anonymous User (n/a) en National Autonomous University of Mexico de ClinicalKey.es por Elsevier en febrero 21, 2024. Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2024. Elsevier Inc. Todos los derechos reservados.  MAYO CLINIC PROCEEDINGS the potential for greatest degree of expansion blood pressure control, and ultimately elec- (
2 mm/y) followed by inflammatory aorto- tive aorta repair. The use of pharmacologic pathies (up to 1.5 mm/y).13 Surveillance fre- agents to reduce hemodynamic stress has quency is individualized to the etiology of been studied using various agents, particu- TAA and the resulting risk of rapid larly in MFS. The central target for physi- expansion. cians caring for patients with TAA is Current recommendations suggest annual aggressive management of hypertension follow-up for degenerative aortopathies when regardless of the etiology.27 Most commonly, dimensions are between 35 and 45 mm and b-blockers are used as first-line therapy for biannual follow-up when between 45 and 55 TAA followed by losartan or other angio- mm.22 A baseline cross-sectional study with tensin II receptor blocker (ARB) therapy.28 either CT or MRI is helpful in identifying abnormal segments along with those that b-Blockers could be serially followed by TTE alone. The primary cited evidence relies on an This allows correlation between TTE and open-label randomized clinical trial of pro- cross-sectional imaging of the affected aortic pranolol in patients with MFS involving 70 dimension and allows TTE to be used as a sur- patients (mean age at enrollment, 14.5 years veillance technique when there is a close cor- for the untreated control group and 15.4 relation between modalities. years for the treatment group), which Unlike degenerative disease, genetic and revealed a significant reduction in aortic inflammatory aortopathies require an individ- root growth and clinical events in the 32 pa- ualized surveillance plan. A summary of the tients treated with propranolol who were fol- guidelines for genetically mediated TAAs us- lowed for a mean of 10.7 years.29 More ing conservative surveillance criteria and our recently, a meta-analysis of clinical trials in clinic’s practice patterns is illustrated in MFS found that both b-blockers and ARBs Figure 2.9,22,24,25 It is of particular importance have a similar efficacy, with an estimated to follow patients with certain genetic aorto- 50% reduction in the annual growth rate of pathies and those with GCA more closely the aorta.30 b-Blockers and more recently because of the high risk of aortic emergencies ARBs are the antihypertensives of choice in in these conditions. For example, a large recent guidelines.9,28,31 retrospective study in 1450 patients with GCA noted an aneurysmal growth rate of 1.5 Angiotensin II Receptor Blockers mm/y and approaching 4.5 mm/y in patients The development of pathologic changes in with a history of aortic dissection.13 Patients the aortic wall and the progressive dilation with aortic dissection had a 51 mm median of the aortic root were attenuated or pre- size of the aorta at the time of dissection. vented by systemic treatment with a trans- Bois et al26 suggested a surveillance schedule forming growth factor beneutralizing based on index imaging at the time of diag- antibody or losartan, an antihypertensive nosis: If index imaging is normal, reassess- medication known to inhibit transforming ment with repeat imaging in 3 to 5 years is growth factor b signaling.32,33 As noted recommended. If index imaging is abnormal above, a recent meta-analysis of MFS clinical with an aortic dimension of greater than 4.5 trials confirmed the efficacy of ARBs in cm, then reimage every 3 to 6 months to deter- MFS.30 Although the combination of b- mine the rate of change. For stable disease, blockers and ARBs has not rigorously been reimaging can be spaced out to annual studies, studied, it is likely that combination treat- and if the aortic dimension is less than 4.5 cm, ment is also effective, as this meta-analysis every 1.5 to 2 years. suggested a reduction in the rate of aortic root expansion using ARB therapy even in MEDICAL THERAPIES patients who were receiving b-blocker ther- Current management strategies incorporate apy. The use of ARBs in LDS has not been serial cardiac imaging, lifestyle modification, studied as extensively, but it is highly likely n n 116 Mayo Clin Proc. January 2024;99(1):111-123 https://doi.org/10.1016/j.mayocp.2023.07.004 www.mayoclinicproceedings.org Descargado para Anonymous User (n/a) en National Autonomous University of Mexico de ClinicalKey.es por Elsevier en febrero 21, 2024. Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2024. Elsevier Inc. Todos los derechos reservados. THORACIC AORTIC ANEURYSMAL DISEASE Genetically mediated aneurysm Syndromic Nonsyndromic Turner Loeys-Dietz Marfan Bicuspid aortic valve FTAAD Imaging TTE + (CTA or MRA)b TTE + (CTA or MRA) TTE + (CTA or MRA) TTE + (CTA or MRA) TTE + (CTA or MRA) modalitya Initialc 0 and 6 mo 0 and 6 mo 0 and 6 mo 0 and 6 mo 0 and 6 mo Surveillance No specific 5-10 yd 2-3 y 3-5 y 2-3 y (no TAA) recommendations ≤2.3 cm/m2 Yearly 45 mm or >40 mm in the patient with a family history of dissection or SCD or rapid expansion Vascular Ehlers-Danlos syndrome High (1%-10%) Avoid pregnancy Turner syndrome High (1%-10%) ASI >25 mm/m2 Familial thoracic aortic aneurysm disease Variable Ascending aorta >50 mm Acquired Low (55 mm ACOG, American College of Obstetricians and Gynecologists; ASI, aortic size index; ESC, European Society of Cardiology; SCD, sudden cardiac death. Data from 2018 ESC guidelines and 2019 ACOG bulletin. Smoking Cessation rupture.50 Stimulants used for attention Tobacco use is associated with the growth of deficit disorder have theoretical risk but TAA.39,40 Smoking cessation should be can be continued if their use is essential. strongly encouraged on the initial and follow-up visits given the clear link with Driving increased mortality. This is not specifically addressed in the Euro- pean Society of Cardiology (ESC) or AHA/ Exercise ACC guidelines for thoracic aortic disease. A For patients with TAA, it is important to position statement by the Canadian Cardiovas- maintain a healthy cardiovascular lifestyle. cular Society recommends preventing patients Physicians and nurse practitioners should from private driving if TAA is less than 6.0 cm discuss and encourage the benefits of regular or 5.5 cm for commercial drivers,51 which is exercise, which have been reported in animal also recommended by the US Federal Motor models.41,42 Yetman and McCrindle43 high- Carrier Safety Administration. lighted this in their study of obesity and its associated increased risk of aortic complica- tions in patients with MFS. However, recom- Screening mendations should be given on avoidance of Family screening is vital given the asymp- contact/competitive sports along with iso- tomatic nature of TAA. Many patients found metric exercise. Specific caution should be with TAA have a genetic contribution, and as exercised to avoid repetitive weightlifting a general rule, first-degree relatives should along with a general lifting restriction of have echocardiography as a screening test. approximately 50 lb.44 Pregnancy Sleep Disordered Breathing Clinicians should discuss birth control Identification and treatment of sleep disor- methods and the risk of pregnancy-related dered breathing is recommended. Risks asso- aortic complications including dissection.52 ciated with untreated sleep apnea and its The ESC guidelines outline the risk of dissec- relation with hypertension have been tion with genetic aortopathies with the highest described, which is also an indirect risk of risk in patients with MFS, LDS, and vascular aortic enlargement in those who have un- Ehlers-Danlos syndrome. The pregnancy rec- treated sleep disordered breathing.45-49 ommendations for this patient population vary given the heterogeneity of the disease pro- Medications to Avoid cess, which dictates specific counseling recom- Fluoroquinolones are generally avoided mendations (Table 3). Both the ESC and given the increased risk of aortic aneurysm American College of Obstetricians and n n 118 Mayo Clin Proc. January 2024;99(1):111-123 https://doi.org/10.1016/j.mayocp.2023.07.004 www.mayoclinicproceedings.org Descargado para Anonymous User (n/a) en National Autonomous University of Mexico de ClinicalKey.es por Elsevier en febrero 21, 2024. Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2024. Elsevier Inc. Todos los derechos reservados. THORACIC AORTIC ANEURYSMAL DISEASE Indications for prophylactic aortic surgery Degenerative aortic diseasea 5.5 cm Bicuspid aortic valve Marfan syndrome 5.0 cm Bicuspid + risk of dissection Bicuspid + low surgical risk at an established center 4.5 cm Bicuspid aortic valve undergoing valve surgery 4.0 cm Loeys-Dietz syndrome 2.5 cm/m2 Turner syndrome diameter Widest FIGURE 3. Diagram depicting values for thoracic aorta diameter for prophylactic aortic surgery. The light green circle is considered a normal adult thoracic aorta diameter at 3.5 cm, recognizing that the normal range is age, sex, and patient size dependent. a5.0 cm is reasonable in asymptomatic patients when performed by experienced surgeons in a multidisciplinary aortic team. Note: If there are high risk factors such as family history of aortic dissection, rapid growth of aneurysm, diffuse dilation, and marked vertebral arterial tortuosity, surgery may be indicated at a lower aneurysm size. Gynecologists guidelines53 for cardiovascular is recommended that the patient be con- disease in pregnant patients suggest compre- nected to a specialist if (1) the patient is hensive imaging of the aorta before pregnancy young or has a concerning family history/ge- in patients at risk of genetic aortic syndromes, netic syndrome, (2) there are high-risk fea- BAV, or family history. Repeated aortic imag- tures for dissection/rupture, (3) the patient ing during pregnancy is suggested every 4 to has multiple comorbidities, or (4) the size 12 weeks depending on the diagnosis and of the aorta warrants biannual follow-up or severity of aortic dilation.52,53 All patients meets criteria for intervention. In such cases, with aortic dilation or a history of dissection if possible, it would be ideal to refer patients should deliver in a center in which cardiotho- to a high-volume center for performing racic surgery is available. Decision on vaginal aortic procedures. vs cesarean delivery depends on the aorta dimension, cause of dilation, and aortic dimen- sion change during pregnancy.53 Similarly, When to Refer for Elective Surgery? depending on the size of the aorta and underly- The threshold for elective aortic repair de- ing cause of disease, b-blocker therapy is often pends on the etiology that has been estab- recommended, well tolerated, and continued lished after a systematic evaluation. The throughout pregnancy. Angiotensin II recep- current European society guidelines and tor blockers should not be used during ACC/AHA 2022 aortic disease guidelines pregnancy. provide recommendations based on the diag- nosis and modifying factors. Preventive aortic repair is recommended SURGICAL CONSIDERATIONS for patients with BAV-related TAA when When to Refer to a Specialist? the aorta dimension is greater than 55 mm Although routine surveillance can be fol- unless there are high-risk features.9 Howev- lowed as an outpatient by primary care, it er, the threshold for surgery is lowered to Mayo Clin Proc. n January 2024;99(1):111-123 n https://doi.org/10.1016/j.mayocp.2023.07.004 119 www.mayoclinicproceedings.org Descargado para Anonymous User (n/a) en National Autonomous University of Mexico de ClinicalKey.es por Elsevier en febrero 21, 2024. Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2024. Elsevier Inc. Todos los derechos reservados.  MAYO CLINIC PROCEEDINGS greater than 45 mm in patients with BAV thoracoabdominal aortic aneurysm repair with an independent indication for aortic carry the highest risk of mortality and post- valve surgery.3,11 In patients with MFS, the operative complications. Despite the higher threshold for operative intervention is low- risk, these cases are feasible, and outcomes ered to greater than 50 mm but may be lower are improved by using adjunctive cardiopul- if additional risk factors are present. These monary bypass techniques such as hypo- risk factors include a family history of thermic circulatory arrest, retrograde/ dissection, size increase of greater than 3 anterograde cerebral perfusion, and the mm/y, desire for pregnancy, or associated use of a cerebrospinal drain.57-59 Procedural valvular abnormalities such as severe aortic complexity may also differ on the basis of or mitral insufficiency that independently the shape of the aneurysm fusiform (sym- meet criteria for intervention. Acknowl- metric enlargement of all the walls) vs edging the increased risk of dissection in saccular (localized bulging at 1 side). familial TAA, BAV, and MFS, the 2022 The operative complexity depends on ACC/AHA guidelines suggest that it is which segment of the aorta is involved. A reasonable to refer for surgery at lower midline sternotomy is used to access the dimension thresholds when performed by ascending aorta and much of the aortic experienced surgeons in multidisciplinary arch. Thoracic endovascular aortic repair is aortic centers (class 2a).9 Meanwhile, greater now an established technique in degenera- than 42 mm has been the suggested tive aneurysms and dissection treatment, threshold for intervention in patients with but thoracic endovascular aortic repair is LDS,24 though other studies suggest greater generally avoided and contraindicated for than 40 mm for these patients.54,55 The rec- elective intervention in patients with geneti- ommendations for prophylactic surgery are cally mediated aortic disorders. illustrated in Figure 3. Some recommenda- tions use aortic diameter indexed to body Aortic Root surface area, particularly in genetic syn- Surgical repair of the aortic root is completed dromes in which patients have short statures via midline sternotomy with cardiopulmo- (eg, Turner syndrome) to ensure early surgi- nary bypass. The aortic root is replaced cal management.56 Given the complexity of with graft material with reimplantation of appropriate timing for intervention, the lat- the coronary arteries. Specific complications est guidelines emphasize referral to aortic to be aware of include coronary button aneu- centers with higher patient volumes with rysms or pseudoaneurysms at the site of multidisciplinary aortic teams to enhance reimplantation. The aortic valve is often outcomes.9 replaced as part of a valved conduit, but in cases with normal aortic valve, specialized Prophylactic Aortic Surgery surgeons may elect to resuspend the native The primary goal for surgical intervention aortic valve to avoid aortic valve prosthesis in patients with aortopathies is to prevent (valve-sparing root replacement). In Europe, aortic dissection or rupture. Surgery is there is a growing use of the personalized also often needed to correct associated external aortic root support system, in which valvular disease. Successful repair removes a sleeve is 3-dimensional printed and surgi- or excludes the aneurysmal segment with cally placed to wrap around the aorta to pro- normal artery proximal and distal to the vide additional compressive support and repair. In selected cases, repair can be reduce the risk of rupture and aortic valve completed using endovascular techniques, insufficiency.60 The personalized external depending on the location and aortic root support system is not currently etiology. Aortic arch replacement and approved for use in the United States. n n 120 Mayo Clin Proc. January 2024;99(1):111-123 https://doi.org/10.1016/j.mayocp.2023.07.004 www.mayoclinicproceedings.org Descargado para Anonymous User (n/a) en National Autonomous University of Mexico de ClinicalKey.es por Elsevier en febrero 21, 2024. Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2024. Elsevier Inc. Todos los derechos reservados. THORACIC AORTIC ANEURYSMAL DISEASE Ascending Aorta for patients with TAA that are found during The least complex repair can be completed routine clinical care. when TAA is limited to above the sinotubu- lar junction without root or aortic arch POTENTIAL COMPETING INTERESTS involvement. A supracoronary tubular graft The authors report no competing interests. repair is performed with a relatively short period of aortic clamping, and cardiopulmo- Abbreviations and Acronyms: ACC/AHA, American Col- lege of Cardiology/American Heart Association; ARB, nary bypass can be done without the need angiotensin II receptor blocker; BAV, bicuspid aortic valve; for circulatory arrest. Dissection and aneu- CT, computed tomography; CTA, computed tomography rysm can occur at the clamping site because angiography; ESC, European Society of Cardiology; FDG, 18 of fragile tissue; this risk is decreased with F-fluorodeoxyglucose; GCA, giant cell arteritis; LDS, Loeys-Dietz syndrome; MFS, Marfan syndrome; MRA, mag- circulatory arrest. Repair options include netic resonance angiography; MRI, magnetic resonance im- open midline sternotomy with an interposi- aging; PET, positron emission tomography; TAA, thoracic tion graft or an inclusion graft that is sur- aortic aneurysm; TEE, transesophageal echocardiography; rounded by the aneurysmal sac. TTE, transthoracic echocardiography Correspondence: Address to Prajwal Reddy, MD, Depart- ment of Cardiology, Mayo Clinic, 200 First St SW, Roches- Aortic Arch ter, MN 55905 ([email protected]). Complexity of TAA repair is the highest with aortic arch involvement. Historically, the ORCID Juan M. 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