View source for Thoracic aortic aneurysm ACC/ AHA guidelines for aortic imaging techniques

__NOTOC__
{{Thoracic aortic aneurysm}}
{{CMG}}; {{AE}} [[User:Mohammad Salih|Mohammad Salih, MD.]] {{AN}}; {{RT}}; {{HP}}

==Overview==
The diagnosis of a thoracic aortic aneurysm is often made as an incidental finding on either chest x-ray ([[widening of the mediastinum]]), on transthoracic echocardiography (dilated aortic root) or on trans esophageal echocardiography (enlarged ascending aorta or aortic arch).  The definitive diagnosis is made using [[CT angiography]] but [[MRA]] is also an excellent test. Once diagnosed, serial [[CTA]] (or [[MRA]]) are recommended every 6-12 months.  The frequency of surveillance imaging should be increased if the aneurysm is larger at baseline, if there is a recent history of accelerating expansion, if the patient is pregnant,  and if the patient has marfan syndrome, loeys-dietz syndrome or a confirmed genetic mutation known to predispose to aortic aneurysms and aortic dissections (TGFBR1, TGFBR2, FBN1, ACTA2, or MYH11).  Indeed in these high risk syndromes, the patient should undergo complete aortic imaging at the time of the initial diagnosis and then every 6 months to determine if enlargement is occurring.  [[Bicuspid aortic valve]], [[coarctation of the aorta]], and/or dilatation of the ascending thoracic aorta should be excluded in a patient with [[turner's syndrome]].  Follow-up imaging should be obtained every 5 to 10 years in the patient with turner's syndrome if the initial imaging is normal, and yearly if any abnormalities are present.  Patients with [[bicuspid aortic valve]] disease and first-degree relatives of patients with [[bicuspid aortic valve]] disease should undergo imaging to rule out the presence of the thoracic aortic aneurysm.

==2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients with Thoracic Aortic Disease (DO NOT EDIT)<ref name="pmid20233780">{{cite journal |author=Hiratzka LF, Bakris GL, Beckman JA, ''et al.'' |title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine |journal=[[Circulation]] |volume=121 |issue=13 |pages=e266–369 |year=2010 |month=April |pmid=20233780 |doi=10.1161/CIR.0b013e3181d4739e |url=}}</ref>==
===Aortic Imaging Techniques to Determine the Presence and Progression of Thoracic Aortic Disease (DO NOT EDIT)<ref name="pmid20233780">{{cite journal |author=Hiratzka LF, Bakris GL, Beckman JA, ''et al.'' |title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine |journal=[[Circulation]] |volume=121 |issue=13 |pages=e266–369 |year=2010 |month=April |pmid=20233780 |doi=10.1161/CIR.0b013e3181d4739e |url=}}</ref>===
{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
|bgcolor="LightGreen" |<nowiki>"</nowiki>'''1.''' Measurements of aortic diameter should be taken at reproducible anatomic landmarks, perpendicular to the axis of [[blood flow]], and reported in a clear and consistent format. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|bgcolor="LightGreen" |<nowiki>"</nowiki>'''2.''' For measurements taken by [[CT|computed tomographic imaging]] or [[magnetic resonance imaging]], the external diameter should be measured perpendicular to the axis of [[blood flow]]. For aortic root measurements, the widest diameter, typically at the mid-sinus level, should be used. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|bgcolor="LightGreen" |<nowiki>"</nowiki>'''3.''' For measurements taken by [[echocardiography]], the internal diameter should be measured perpendicular to the axis of blood flow. For aortic root measurements the widest diameter, typically at the mid-sinus level, should be used. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
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|bgcolor="LightGreen" |<nowiki>"</nowiki>'''4.''' Abnormalities of aortic morphology should be recognized and reported separately even when aortic diameters are within normal limits. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
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|bgcolor="LightGreen" |<nowiki>"</nowiki>'''5.''' The finding of [[aortic dissection]], aneurysm, traumatic injury and/or aortic rupture should be immediately communicated to the referring physician. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|bgcolor="LightGreen" |<nowiki>"</nowiki>'''6.''' Techniques to minimize episodic and cumulative radiation exposure should be utilized whenever possible.<ref name="pmid17467608">{{cite journal |author=Amis ES, Butler PF, Applegate KE, ''et al.'' |title=American College of Radiology white paper on radiation dose in medicine |journal=J Am Coll Radiol |volume=4 |issue=5 |pages=272–84 |year=2007 |month=May |pmid=17467608 |doi=10.1016/j.jacr.2007.03.002 |url=}}</ref><ref name="pmid18046031">{{cite journal |author=Brenner DJ, Hall EJ |title=Computed tomography--an increasing source of radiation exposure |journal=N. Engl. J. Med. |volume=357 |issue=22 |pages=2277–84 |year=2007 |month=November |pmid=18046031 |doi=10.1056/NEJMra072149 |url=}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
|}

{|class="wikitable"
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| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
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|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' If clinical information is available, it can be useful to relate aortic diameter to the patient's age and body size. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|}

===Genetic Syndromes (DO NOT EDIT)<ref name="pmid20233780">{{cite journal |author=Hiratzka LF, Bakris GL, Beckman JA, ''et al.'' |title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine |journal=[[Circulation]] |volume=121 |issue=13 |pages=e266–369 |year=2010 |month=April |pmid=20233780 |doi=10.1161/CIR.0b013e3181d4739e |url=}}</ref>===
{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
|bgcolor="LightGreen" |<nowiki>"</nowiki>'''1.''' An [[echocardiogram]] is recommended at the time of diagnosis of [[Marfan syndrome]] to determine the aortic root and ascending aortic diameters and 6 months thereafter to determine the rate of enlargement of the aorta. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>        
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|bgcolor="LightGreen" |<nowiki>"</nowiki>'''2.''' Annual imaging is recommended for patients with [[Marfan syndrome]] if stability of the aortic diameter is documented. If the maximal aortic diameter is 4.5 cm or greater, or if the aortic diameter shows significant growth from baseline, more frequent imaging should be considered. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
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|bgcolor="LightGreen" |<nowiki>"</nowiki>'''3.''' Patients with [[Loeys-Dietz syndrome]] or a confirmed genetic mutation known to predispose to aortic aneurysms and aortic dissections ([[TGFBR1]], [[TGFBR2]], [[FBN1]], [[ACTA2]], or [[MYH11]]) should undergo complete aortic imaging at initial diagnosis and 6 months thereafter to establish if enlargement is occurring.<ref name="pmid18695204">{{cite journal |author=Pearson GD, Devereux R, Loeys B, ''et al.'' |title=Report of the National Heart, Lung, and Blood Institute and National Marfan Foundation Working Group on research in Marfan syndrome and related disorders |journal=Circulation |volume=118 |issue=7 |pages=785–91 |year=2008 |month=August |pmid=18695204 |pmc=2909440 |doi=10.1161/CIRCULATIONAHA.108.783753 |url=}}</ref><ref name="pmid2766531">{{cite journal |author=Svensson LG, Crawford ES, Coselli JS, Safi HJ, Hess KR |title=Impact of cardiovascular operation on survival in the Marfan patient |journal=Circulation |volume=80 |issue=3 Pt 1 |pages=I233–42 |year=1989 |month=September |pmid=2766531 |doi= |url=}}</ref><ref name="pmid17307461">{{cite journal |author=Svensson LG, Blackstone EH, Feng J, ''et al.'' |title=Are Marfan syndrome and marfanoid patients distinguishable on long-term follow-up? |journal=Ann. Thorac. Surg. |volume=83 |issue=3 |pages=1067–74 |year=2007 |month=March |pmid=17307461 |doi=10.1016/j.athoracsur.2006.10.062 |url=}}</ref><ref name="pmid16444274">{{cite journal |author=Zhu L, Vranckx R, Khau Van Kien P, ''et al.'' |title=Mutations in myosin heavy chain 11 cause a syndrome associating thoracic aortic aneurysm/aortic dissection and patent ductus arteriosus |journal=Nat. Genet. |volume=38 |issue=3 |pages=343–9 |year=2006 |month=March |pmid=16444274 |doi=10.1038/ng1721 |url=}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>     
|-
|bgcolor="LightGreen" |<nowiki>"</nowiki>'''4.''' Patients with Loeys-Dietz syndrome should have yearly [[magnetic resonance imaging]] from the cerebrovascular circulation to the pelvis.<ref name="pmid17330129">{{cite journal |author=LeMaire SA, Pannu H, Tran-Fadulu V, Carter SA, Coselli JS, Milewicz DM |title=Severe aortic and arterial aneurysms associated with a TGFBR2 mutation |journal=Nat Clin Pract Cardiovasc Med |volume=4 |issue=3 |pages=167–71 |year=2007 |month=March |pmid=17330129 |pmc=2561071 |doi=10.1038/ncpcardio0797 |url=}}</ref><ref name="pmid16928994">{{cite journal |author=Loeys BL, Schwarze U, Holm T, ''et al.'' |title=Aneurysm syndromes caused by mutations in the TGF-beta receptor |journal=N. Engl. J. Med. |volume=355 |issue=8 |pages=788–98 |year=2006 |month=August |pmid=16928994 |doi=10.1056/NEJMoa055695 |url=}}</ref><ref name="pmid17257922">{{cite journal |author=Williams JA, Loeys BL, Nwakanma LU, ''et al.'' |title=Early surgical experience with Loeys-Dietz: a new syndrome of aggressive thoracic aortic aneurysm disease |journal=Ann. Thorac. Surg. |volume=83 |issue=2 |pages=S757–63; discussion S785–90 |year=2007 |month=February |pmid=17257922 |doi=10.1016/j.athoracsur.2006.10.091 |url=}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
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|bgcolor="LightGreen" |<nowiki>"</nowiki>'''5.''' Patients with [[Turner syndrome]] should undergo imaging of the [[heart]] and [[aorta]] for evidence of [[bicuspid aortic valve]], [[coarctation of the aorta]], or dilatation of the ascending thoracic aorta.<ref name="pmid17047017">{{cite journal| author=Bondy CA, Turner Syndrome Study Group| title=Care of girls and women with Turner syndrome: a guideline of the Turner Syndrome Study Group. | journal=J Clin Endocrinol Metab | year= 2007 | volume= 92 | issue= 1 | pages= 10-25 | pmid=17047017 | doi=10.1210/jc.2006-1374 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17047017  }} </ref> If initial imaging is normal and there are no risk factors for [[aortic dissection]], repeat imaging should be performed every 5 to 10 years or if otherwise clinically indicated. If abnormalities exist, annual imaging or follow-up imaging should be done. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|}

{|class="wikitable"
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| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
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|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' It is reasonable to consider surgical repair of the aorta in all adult patients with [[Loeys-Dietz syndrome]] or a confirmed [[TGFBR1]] or [[TGFBR2]] mutation and an aortic diameter of 4.2 cm or greater by [[transesophageal echocardiogram]] (internal diameter) or 4.4 to 4.6 cm or greater by [[CT|computed tomographic imaging]] and/or [[magnetic resonance imaging]] (external diameter).<ref name="pmid16928994">{{cite journal |author=Loeys BL, Schwarze U, Holm T, ''et al.'' |title=Aneurysm syndromes caused by mutations in the TGF-beta receptor |journal=N. Engl. J. Med. |volume=355 |issue=8 |pages=788–98 |year=2006 |month=August |pmid=16928994 |doi=10.1056/NEJMoa055695 |url=}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>    
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|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' For women with [[Marfan syndrome]] contemplating [[pregnancy]], it is reasonable to prophylactically replace the aortic root and [[ascending aorta]] if the diameter exceeds 4.0 cm.<ref name="pmid18695204">{{cite journal| author=Pearson GD, Devereux R, Loeys B, Maslen C, Milewicz D, Pyeritz R et al.| title=Report of the National Heart, Lung, and Blood Institute and National Marfan Foundation Working Group on research in Marfan syndrome and related disorders. | journal=Circulation | year= 2008 | volume= 118 | issue= 7 | pages= 785-91 | pmid=18695204 | doi=10.1161/CIRCULATIONAHA.108.783753 | pmc=PMC2909440 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18695204  }} </ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
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|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''3.''' If the maximal cross-sectional area in square centimeters of the [[ascending aorta]] or root divided by the patient's height in meters exceeds a ratio of 10, surgical repair is reasonable because shorter patients have dissection at a smaller size and 15% of patients with [[Marfan syndrome]] have dissection at a size smaller than 5.0 cm.<ref name="pmid17307461">{{cite journal |author=Svensson LG, Blackstone EH, Feng J, ''et al.'' |title=Are Marfan syndrome and marfanoid patients distinguishable on long-term follow-up? |journal=Ann. Thorac. Surg. |volume=83 |issue=3 |pages=1067–74 |year=2007 |month=March |pmid=17307461 |doi=10.1016/j.athoracsur.2006.10.062 |url=}}</ref><ref name="pmid10219065">{{cite journal |author=Gott VL, Greene PS, Alejo DE, ''et al.'' |title=Replacement of the aortic root in patients with Marfan's syndrome |journal=N. Engl. J. Med. |volume=340 |issue=17 |pages=1307–13 |year=1999 |month=April |pmid=10219065 |doi=10.1056/NEJM199904293401702 |url=}}</ref><ref name="pmid11828302">{{cite journal |author=Svensson LG, Khitin L |title=Aortic cross-sectional area/height ratio timing of aortic surgery in asymptomatic patients with Marfan syndrome |journal=J. Thorac. Cardiovasc. Surg. |volume=123 |issue=2 |pages=360–1 |year=2002 |month=February |pmid=11828302 |doi= |url=}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>          
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{|class="wikitable"
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| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIb]]
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|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' In patients with [[Turner syndrome]] with additional risk factors, including [[bicuspid aortic valve]], [[coarctation of the aorta]], and/or [[hypertension]], and in patients who attempt to become pregnant or who become pregnant, it may be reasonable to perform imaging of the [[heart]] and [[aorta]] to help determine the risk of [[aortic dissection]]. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>       
|}

===Familial Thoracic Aortic Aneurysms and Dissections (DO NOT EDIT)<ref name="pmid20233780">{{cite journal |author=Hiratzka LF, Bakris GL, Beckman JA, ''et al.'' |title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine |journal=[[Circulation]] |volume=121 |issue=13 |pages=e266–369 |year=2010 |month=April |pmid=20233780 |doi=10.1161/CIR.0b013e3181d4739e |url=}}</ref>===
{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
|bgcolor="LightGreen" |<nowiki>"</nowiki>'''1.''' Aortic imaging is recommended for first-degree relatives of patients with [[thoracic aortic aneurysm]] and/or [[aortic dissection|dissection]] to identify those with asymptomatic disease.<ref name="pmid16996941">{{cite journal |author=Albornoz G, Coady MA, Roberts M, ''et al.'' |title=Familial thoracic aortic aneurysms and dissections--incidence, modes of inheritance, and phenotypic patterns |journal=Ann. Thorac. Surg. |volume=82 |issue=4 |pages=1400–5 |year=2006 |month=October |pmid=16996941 |doi=10.1016/j.athoracsur.2006.04.098 |url=}}</ref><ref name="pmid10199307">{{cite journal |author=Coady MA, Davies RR, Roberts M, ''et al.'' |title=Familial patterns of thoracic aortic aneurysms |journal=Arch Surg |volume=134 |issue=4 |pages=361–7 |year=1999 |month=April |pmid=10199307 |doi= |url=}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki> 
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|bgcolor="LightGreen" |<nowiki>"</nowiki>'''2.''' If the mutant gene (FBN1, TGFBR1, TGFBR2, COL3A1, ACTA2, MYH11) associated with [[aortic aneurysm]] and/or dissection is identified in a patient, first-degree relatives should undergo counseling and testing. Then, only the relatives with the genetic mutation should undergo aortic imaging.  ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki> 
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{|class="wikitable"
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| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
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|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' If one or more first-degree relatives of a patient with known thoracic [[aortic aneurysm]] and/or [[aortic dissection|dissection]] are found to have thoracic aortic dilatation, aneurysm, or dissection, then imaging of second-degree relatives is reasonable.<ref name="pmid16996941">{{cite journal |author=Albornoz G, Coady MA, Roberts M, ''et al.'' |title=Familial thoracic aortic aneurysms and dissections--incidence, modes of inheritance, and phenotypic patterns |journal=Ann. Thorac. Surg. |volume=82 |issue=4 |pages=1400–5 |year=2006 |month=October |pmid=16996941 |doi=10.1016/j.athoracsur.2006.04.098 |url=}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
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|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' Sequencing of the [[ACTA2]] gene is reasonable in patients with a family history of thoracic aortic aneurysms and/or dissections to determine if ACTA2 mutations are responsible for the inherited predisposition.<ref name="pmid16027248">{{cite journal| author=Pannu H, Fadulu VT, Chang J, Lafont A, Hasham SN, Sparks E et al.| title=Mutations in transforming growth factor-beta receptor type II cause familial thoracic aortic aneurysms and dissections. | journal=Circulation | year= 2005 | volume= 112 | issue= 4 | pages= 513-20 | pmid=16027248 | doi=10.1161/CIRCULATIONAHA.105.537340 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16027248  }} </ref><ref name="pmid17994018">{{cite journal| author=Guo DC, Pannu H, Tran-Fadulu V, Papke CL, Yu RK, Avidan N et al.| title=Mutations in smooth muscle alpha-actin (ACTA2) lead to thoracic aortic aneurysms and dissections. | journal=Nat Genet | year= 2007 | volume= 39 | issue= 12 | pages= 1488-93 | pmid=17994018 | doi=10.1038/ng.2007.6 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17994018  }} </ref><ref name="pmid16444274">{{cite journal| author=Zhu L, Vranckx R, Khau Van Kien P, Lalande A, Boisset N, Mathieu F et al.| title=Mutations in myosin heavy chain 11 cause a syndrome associating thoracic aortic aneurysm/aortic dissection and patent ductus arteriosus. | journal=Nat Genet | year= 2006 | volume= 38 | issue= 3 | pages= 343-9 | pmid=16444274 | doi=10.1038/ng1721 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16444274  }} </ref><ref name="pmid16928994">{{cite journal| author=Loeys BL, Schwarze U, Holm T, Callewaert BL, Thomas GH, Pannu H et al.| title=Aneurysm syndromes caused by mutations in the TGF-beta receptor. | journal=N Engl J Med | year= 2006 | volume= 355 | issue= 8 | pages= 788-98 | pmid=16928994 | doi=10.1056/NEJMoa055695 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16928994  }} </ref><ref name="pmid18781618">{{cite journal| author=Stheneur C, Collod-Béroud G, Faivre L, Gouya L, Sultan G, Le Parc JM et al.| title=Identification of 23 TGFBR2 and 6 TGFBR1 gene mutations and genotype-phenotype investigations in 457 patients with Marfan syndrome type I and II, Loeys-Dietz syndrome and related disorders. | journal=Hum Mutat | year= 2008 | volume= 29 | issue= 11 | pages= E284-95 | pmid=18781618 | doi=10.1002/humu.20871 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18781618  }} </ref><ref name="pmid19409525">{{cite journal| author=Guo DC, Papke CL, Tran-Fadulu V, Regalado ES, Avidan N, Johnson RJ et al.| title=Mutations in smooth muscle alpha-actin (ACTA2) cause coronary artery disease, stroke, and Moyamoya disease, along with thoracic aortic disease. | journal=Am J Hum Genet | year= 2009 | volume= 84 | issue= 5 | pages= 617-27 | pmid=19409525 | doi=10.1016/j.ajhg.2009.04.007 | pmc=PMC2680995 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19409525  }} </ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
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{|class="wikitable"
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| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIb]]
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' Sequencing of other genes known to cause familial thoracic aortic aneurysms and/or dissection ([[TGFBR1]], [[TGFBR2]], [[MYH11]]) may be considered in patients with a family history and clinical features associated with mutations in these genes.<ref name="pmid16027248">{{cite journal| author=Pannu H, Fadulu VT, Chang J, Lafont A, Hasham SN, Sparks E et al.| title=Mutations in transforming growth factor-beta receptor type II cause familial thoracic aortic aneurysms and dissections. | journal=Circulation | year= 2005 | volume= 112 | issue= 4 | pages= 513-20 | pmid=16027248 | doi=10.1161/CIRCULATIONAHA.105.537340 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16027248  }} </ref><ref name="pmid17994018">{{cite journal| author=Guo DC, Pannu H, Tran-Fadulu V, Papke CL, Yu RK, Avidan N et al.| title=Mutations in smooth muscle alpha-actin (ACTA2) lead to thoracic aortic aneurysms and dissections. | journal=Nat Genet | year= 2007 | volume= 39 | issue= 12 | pages= 1488-93 | pmid=17994018 | doi=10.1038/ng.2007.6 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17994018  }} </ref><ref name="pmid16444274">{{cite journal| author=Zhu L, Vranckx R, Khau Van Kien P, Lalande A, Boisset N, Mathieu F et al.| title=Mutations in myosin heavy chain 11 cause a syndrome associating thoracic aortic aneurysm/aortic dissection and patent ductus arteriosus. | journal=Nat Genet | year= 2006 | volume= 38 | issue= 3 | pages= 343-9 | pmid=16444274 | doi=10.1038/ng1721 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16444274  }} </ref><ref name="pmid16928994">{{cite journal| author=Loeys BL, Schwarze U, Holm T, Callewaert BL, Thomas GH, Pannu H et al.| title=Aneurysm syndromes caused by mutations in the TGF-beta receptor. | journal=N Engl J Med | year= 2006 | volume= 355 | issue= 8 | pages= 788-98 | pmid=16928994 | doi=10.1056/NEJMoa055695 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16928994  }} </ref><ref name="pmid18781618">{{cite journal| author=Stheneur C, Collod-Béroud G, Faivre L, Gouya L, Sultan G, Le Parc JM et al.| title=Identification of 23 TGFBR2 and 6 TGFBR1 gene mutations and genotype-phenotype investigations in 457 patients with Marfan syndrome type I and II, Loeys-Dietz syndrome and related disorders. | journal=Hum Mutat | year= 2008 | volume= 29 | issue= 11 | pages= E284-95 | pmid=18781618 | doi=10.1002/humu.20871 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18781618  }} </ref><ref name="pmid19409525">{{cite journal| author=Guo DC, Papke CL, Tran-Fadulu V, Regalado ES, Avidan N, Johnson RJ et al.| title=Mutations in smooth muscle alpha-actin (ACTA2) cause coronary artery disease, stroke, and Moyamoya disease, along with thoracic aortic disease. | journal=Am J Hum Genet | year= 2009 | volume= 84 | issue= 5 | pages= 617-27 | pmid=19409525 | doi=10.1016/j.ajhg.2009.04.007 | pmc=PMC2680995 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19409525  }} </ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' If one or more first-degree relatives of a patient with known thoracic aortic aneurysm and/or dissection are found to have thoracic aortic dilatation, [[aortic aneurysm|aneurysm]], or [[aortic dissection|dissection]], then referral to a geneticist may be considered. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|}

===Bicuspid Aortic Valve and Associated Congenital Variants in Adults (DO NOT EDIT)<ref name="pmid20233780">{{cite journal |author=Hiratzka LF, Bakris GL, Beckman JA, ''et al.'' |title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine |journal=[[Circulation]] |volume=121 |issue=13 |pages=e266–369 |year=2010 |month=April |pmid=20233780 |doi=10.1161/CIR.0b013e3181d4739e |url=}}</ref>===
{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
|bgcolor="LightGreen" |<nowiki>"</nowiki>'''1.''' First-degree relatives of patients with a [[bicuspid aortic valve]], premature onset of thoracic aortic disease with minimal risk factors, and/or a familial form of [[thoracic aortic aneurysm]] and dissection should be evaluated for the presence of a bicuspid aortic valve and asymptomatic thoracic aortic disease. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|bgcolor="LightGreen" |<nowiki>"</nowiki>'''2.''' All patients with a [[bicuspid aortic valve]] should have both the aortic root and ascending thoracic [[aorta]] evaluated for evidence of aortic dilatation.<ref name="pmid16129122">{{cite journal |author=Braverman AC, Güven H, Beardslee MA, Makan M, Kates AM, Moon MR |title=The bicuspid aortic valve |journal=Curr Probl Cardiol |volume=30 |issue=9 |pages=470–522 |year=2005 |month=September |pmid=16129122 |doi=10.1016/j.cpcardiol.2005.06.002 |url=}}</ref><ref name="pmid16087084">{{cite journal |author=Borger MA, David TE |title=Management of the valve and ascending aorta in adults with bicuspid aortic valve disease |journal=Semin. Thorac. Cardiovasc. Surg. |volume=17 |issue=2 |pages=143–7 |year=2005 |pmid=16087084 |doi=10.1053/j.semtcvs.2005.02.005 |url=}}</ref><ref name="pmid14647130">{{cite journal |author=Svensson LG, Blackstone EH, Cosgrove DM |title=Surgical options in young adults with aortic valve disease |journal=Curr Probl Cardiol |volume=28 |issue=7 |pages=417–80 |year=2003 |month=July |pmid=14647130 |doi=10.1016/j.cpcardiol.2003.08.002 |url=}}</ref><ref name="pmid18431353">{{cite journal |author=Svensson LG |title=Aortic valve stenosis and regurgitation: an overview of management |journal=J Cardiovasc Surg (Torino) |volume=49 |issue=2 |pages=297–303 |year=2008 |month=April |pmid=18431353 |doi= |url=}}</ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
|}

===Takayasu Arteritis and Giant Cell Arteritis (DO NOT EDIT)<ref name="pmid20233780">{{cite journal |author=Hiratzka LF, Bakris GL, Beckman JA, ''et al.'' |title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine |journal=[[Circulation]] |volume=121 |issue=13 |pages=e266–369 |year=2010 |month=April |pmid=20233780 |doi=10.1161/CIR.0b013e3181d4739e |url=}}</ref>===
{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
|bgcolor="LightGreen" |<nowiki>"</nowiki>'''1.''' The initial evaluation of [[Takayasu arteritis]] or [[giant cell arteritis]] should include [[thoracic aorta]] and branch vessel [[CT|computed tomographic imaging]] or [[magnetic resonance imaging]] to investigate the possibility of aneurysm or occlusive disease in these vessels. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>      
|}

===Surveillance of Thoracic Aortic Disease or Previously Repaired Patients (DO NOT EDIT)<ref name="pmid20233780">{{cite journal |author=Hiratzka LF, Bakris GL, Beckman JA, ''et al.'' |title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine |journal=[[Circulation]] |volume=121 |issue=13 |pages=e266–369 |year=2010 |month=April |pmid=20233780 |doi=10.1161/CIR.0b013e3181d4739e |url=}}</ref>===
{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' [[Computed tomographic imaging]] or [[magnetic resonance imaging]] of the [[thoracic aorta]] is reasonable after a Type A or B aortic dissection or after prophylactic repair of the aortic root/ascending aorta.<ref name="pmid18695204">{{cite journal| author=Pearson GD, Devereux R, Loeys B, Maslen C, Milewicz D, Pyeritz R et al.| title=Report of the National Heart, Lung, and Blood Institute and National Marfan Foundation Working Group on research in Marfan syndrome and related disorders. | journal=Circulation | year= 2008 | volume= 118 | issue= 7 | pages= 785-91 | pmid=18695204 | doi=10.1161/CIRCULATIONAHA.108.783753 | pmc=PMC2909440 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18695204  }} </ref> ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' [[Computed tomographic imaging]] or [[magnetic resonance imaging]] of the [[aorta]] is reasonable at 1, 3, 6, and 12 months postdissection and, if stable, annually thereafter so that any threatening enlargement can be detected in a timely fashion.  ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''3.''' When following patients with imaging, utilization of the same modality at the same institution is reasonable, so that similar images of matching anatomic segments can be compared side by side.  ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''4.''' If a [[thoracic aortic aneurysm]] is only moderate in size and remains relatively stable over time, [[magnetic resonance imaging]] instead of [[computed tomographic imaging]] is reasonable to minimize the patient’s radiation exposure. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''5.''' Surveillance imaging similar to classic aortic dissection is reasonable in patients with intramural hematoma. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|}

==References==
{{reflist|2}}
[[Category:Disease]]
[[Category:Cardiology]]
[[Category:Vascular surgery]]
[[Category:Emergency medicine]]
{{WH}}
{{WS}}