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| name = {{PAGENAME}}
| synonyms = Cystic medial necrosis of aorta<ref>{{cite web|title=Orphanet: Familial aortic dissection |url=https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=229 |website=Orpha.net |accessdate=29 May 2019 |language=en}}</ref>
| synonyms = Cystic medial necrosis of aorta<ref>{{cite web|title=Orphanet: Familial aortic dissection |url=https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=229 |website=Orpha.net |accessdate=29 May 2019 |language=en}}</ref>
| image =
| image = Aorta segments.svg
| alt =
| alt =
| caption = Segments of the [[aorta]], including the [[thoracic aorta]], [[ascending aorta]], [[aortic arch]], [[descending thoracic aorta]], [[abdominal aorta]], suprarenal abdominal aorta, and infrarenal abdominal aorta.
| caption =
| pronounce =
| pronounce =
| field = cardiology, cardiothoracic surgery
| field = [[Cardiology]]
| symptoms =
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| complications =
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'''Annuloaortic ectasia''' is characterized by pure [[Aortic regurgitation|aortic valve regurgitation]] and aneurysmal dilatation of the [[ascending aorta]].<ref name="An Xu Poullis Tang 2004 pp. 61–66">{{cite journal | last=An | first=Zhao | last2=Xu | first2=Zhi-Yun | last3=Poullis | first3=Mike | last4=Tang | first4=Paul C. | last5=Oliveira | first5=Nilto De | last6=Rylski | first6=Bartosz | last7=Milewski | first7=Rita K. | last8=Urbanski | first8=Paul P. | last9=Diegeler | first9=Anno | last10=Kumar | first10=Neeraj | last11=Yang | first11=Laurence T. | title=Aortic valve sparing operations: basic concepts | journal=International Journal of Cardiology | publisher=Elsevier | volume=97 | date=December 1, 2004 | issn=0167-5273 | doi=10.1016/j.ijcard.2004.08.010 | pages=61–66 |url=https://www.sciencedirect.com/science/article/abs/pii/S0167527304006047 | access-date=December 29, 2023}}</ref> Men are more likely than women to develop idiopathic annuloaortic ectasia, which usually manifests in the fourth or sixth decades of life. Additional factors that contribute to this condition include [[osteogenesis imperfecta]], inflammatory aortic diseases, intrinsic valve disease, [[Loeys–Dietz syndrome|Loeys-Dietz syndrome]], [[Marfan syndrome]], and operated [[Congenital heart defect|congenital heart disease]].<ref name="Vascular Pathobiology"/>
'''Annuloaortic ectasia''' is a dilation of the proximal ascending aorta and [[aortic annulus]]. It may cause [[aortic regurgitation]], thoracic [[aortic dissection]], [[aneurysm]] and rupture. It is often associated with [[connective tissue diseases]] like [[Marfan syndrome]] and [[Ehlers Danlos Syndrome]].<ref>{{Cite web|url=https://radiopaedia.org/articles/annuloaortic-ectasia-2?lang=us|title=Annuloaortic ectasia &#124; Radiology Reference Article|first=Azza|last=Elgendy|website=Radiopaedia.org|access-date=25 April 2022}}</ref> It can also be a complication due to tertiary [[Syphilis#Tertiary|syphilis]]. In tertiary syphilis the aortic root becomes so dilated that the aortic valve becomes incompetent and [[cor bovinum]] results.


On a gross level, there is a pear-shaped, symmetric enlargement due to proximal aortic dilation. The aortic wall dilatation at the commissural level causes the [[Cusps of heart valves|cusps]] to effectively shorten and prevent them from converging during [[systole]], which results in [[aortic valve]] incompetence. The arch is typically spared from the aneurysmal process, though it may involve the entire ascending aorta. The ectatic aorta may experience dissections. Dissections of the ascending aorta are typically tiny, confined, and restricted. Aortic rupture can happen even if there is no dissection.<ref name="Vascular Pathobiology">{{cite web | last=Kinoshita | first=Ryoji | last2=Arai | first2=Hirokuni | last3=Barillà | first3=David | last4=Benedetto | first4=Filippo | last5=Ladich | first5=E. | last6=Virmani | first6=R. | last7=Seidman | first7=M.A. | last8=McManus | first8=B.M. | last9=Subbotin | first9=Vladimir M. | last10=Spanos | first10=K. | last11=Giannoukas | first11=A.D. | title=Vascular Pathobiology: Atherosclerosis and Large Vessel Disease | publisher=Academic Press | date=January 1, 2016 | doi=10.1016/B978-0-12-420219-1.00003-3 |url=https://www.sciencedirect.com/science/article/abs/pii/B9780124202191000033 | access-date=December 29, 2023 | page=85–124}}</ref>
The term was first coined by the American heart surgeon [[Denton Cooley]] in 1961.<ref>{{Cite book | url=https://books.google.com/books?id=yE8tBAAAQBAJ&q=annuloaortic+ectasia&pg=PA556 | title=Mastery of Cardiothoracic Surgery| isbn=9781451113150| last1=Kaiser| first1=Larry| last2=Kron| first2=Irving L.| last3=Spray| first3=Thomas L.| date=2013-12-24}}</ref>

The term was first coined by the American heart surgeon [[Denton Cooley]] in 1961.<ref>{{Cite book |url=https://books.google.com/books?id=yE8tBAAAQBAJ&q=annuloaortic+ectasia&pg=PA556 | title=Mastery of Cardiothoracic Surgery| isbn=9781451113150| last1=Kaiser| first1=Larry| last2=Kron| first2=Irving L.| last3=Spray| first3=Thomas L.| date=2013-12-24}}</ref>

==Signs and symptoms==
Symptoms are often neurological or circulatory.<ref name="giant cell arteritis">{{cite journal | last=S | first=Gelsomino | last2=S | first2=Romagnoli | last3=F | first3=Gori | last4=G | first4=Nesi | last5=C | first5=Anichini | last6=C | first6=Sorbara | last7=P | first7=Stefàno | last8=GF | first8=Gensini | title=Annuloaortic ectasia and giant cell arteritis | journal=The Annals of thoracic surgery | publisher=Ann Thorac Surg | volume=80 | issue=1 | issn=1552-6259 | pmid=15975349 | doi=10.1016/j.athoracsur.2005.01.063 |url=https://pubmed.ncbi.nlm.nih.gov/15975349/ | access-date=December 29, 2023 | page=}}</ref> Symptoms include [[dyspnea]], [[Acute respiratory distress syndrome|respiratory distress]], [[heart failure]], [[atrial tachycardia]],<ref name="G C E S p. ">{{cite journal | last=G | first=Nesi | last2=C | first2=Anichini | last3=E | first3=Pedemonte | last4=S | first4=Tozzini | last5=G | first5=Calamai | last6=GF | first6=Montesi | last7=F | first7=Gori | title=Giant cell arteritis presenting with annuloaortic ectasia | journal=Chest | publisher=Chest | volume=121 | issue=4 | issn=0012-3692 | pmid=11948080 | doi=10.1378/chest.121.4.1365 |url=https://pubmed.ncbi.nlm.nih.gov/11948080/ | access-date=December 29, 2023 | page=}}</ref> pain, [[cardiac tamponade]],<ref name="T S T M 2007 p. ">{{cite journal | last=T | first=Fujii | last2=S | first2=Sumiyoshi | last3=T | first3=Koga | last4=M | first4=Nishizaka | last5=R | first5=Matsukawa | last6=H | first6=Kuwano | last7=K | first7=Sueishi | title=An autopsy case report of annuloaortic ectasia with cardiac tamponade ruptured from an aneurysm of the right Valsalva sinus | journal=Pathology, research and practice | publisher=Pathol Res Pract | volume=203 | issue=9 | date=2007 | issn=0344-0338 | pmid=17646055 | doi=10.1016/j.prp.2007.05.007 |url=https://pubmed.ncbi.nlm.nih.gov/17646055/ | access-date=December 29, 2023 | page=}}</ref> [[palpitations]], and [[malaise]].<ref name="H Y Y T p. ">{{cite journal | last=H | first=Harada | last2=Y | first2=Honma | last3=Y | first3=Hachiro | last4=T | first4=Mawatari | last5=T | first5=Abe | title=Composite graft replacement after aortic valvuloplasty in Takayasu arteritis | journal=The Annals of thoracic surgery | publisher=Ann Thorac Surg | volume=73 | issue=2 | issn=0003-4975 | pmid=11845892 | doi=10.1016/s0003-4975(01)03363-x |url=https://pubmed.ncbi.nlm.nih.gov/11845892/ | access-date=December 29, 2023 | page=}}</ref> [[Aortic valve regurgitation]] is found in about 77% of all patients.<ref name="Yuan Lin p. ">{{cite journal | last=Yuan | first=Shi-Min | last2=Lin | first2=Hong | title=Annuloaortic Ectasia and Arteritis: Clinical Features, Treatments of Choice, and Causative Relations | journal=Brazilian Journal of Cardiovascular Surgery | publisher=Sociedade Brasileira de Cirurgia Cardiovascular | volume=34 | issue=4 | pmid=31454202 | doi=10.21470/1678-9741-2018-0252 |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6713369/ | access-date=December 29, 2023 | page=}}</ref>

==Treatment==
For many years, the gold standard treatment for patients with aortic valve disease and aortic root aneurysms was to replace both the [[aortic valve]] and the [[ascending aorta]] with a composite graft. This also applies to patients with different levels of AI and annuloaortic ectasia, where the aortic valve may be largely preserved without any structural abnormalities.<ref name="Martino Re Blasi Celiento p. ">{{cite journal | last=Martino | first=Andrea De | last2=Re | first2=Federico Del | last3=Blasi | first3=Stefania | last4=Celiento | first4=Michele | last5=Ravenni | first5=Giacomo | last6=Pratali | first6=Stefano | last7=Milano | first7=Aldo D. | last8=Bortolotti | first8=Uberto | title=Surgical Treatment of Annuloaortic Ectasia&nbsp;– Replace or Repair? | journal=AORTA Journal | publisher=Thieme Medical Publishers | volume=5 | issue=5 | pmid=29657952 | doi=10.12945/j.aorta.2017.17.044 |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5890764/ | access-date=December 29, 2023 | page=}}</ref>

==See also==
* [[Aortic regurgitation]]
* [[Aortic valve replacement]]


==References==
==References==
{{Reflist}}
{{Reflist}}

==Further reading==
* {{cite web | last=Mestres | first=Gaspar | last2=Riambau | first2=Vincent | last3=Seidman | first3=M.A. | last4=McManus | first4=B.M. | last5=Misra | first5=Durga Prasanna | last6=Negi | first6=Vir Singh | last7=Sato | first7=Hiroshi | last8=Tamiya | first8=Yukihiko | last9=Fishbein | first9=G.A. | last10=Buja | first10=L.M. | last11=Schoen | first11=F.J. | last12=Butany | first12=J. | title=Aneurysms of the Aorta: Ascending, Thoracic and Abdominal and Their Management | publisher=Academic Press | date=January 1, 2016 | doi=10.1016/B978-0-12-420219-1.00005-7 |url=https://www.sciencedirect.com/science/article/abs/pii/B9780124202191000057 | access-date=December 29, 2023 | ref=none}}
* {{cite book | last=Cooley | first=Denton A. | title=Cardiac Valve Replacement | chapter=Annuloaortic Ectasia: Surgical Repair Using a Composite ST. JUDE MEDICAL® Valve and DACRON® Tube Graft | publisher=Springer US | publication-place=Boston, MA | date=1985 | isbn=978-1-4612-9629-4 | doi=10.1007/978-1-4613-2601-4_10 | ref=none}}

==External links==
* [https://stanfordhealthcare.org/medical-conditions/blood-heart-circulation/annuloaortic-ectasia.html Stanford Health Care]
* [https://thoracickey.com/annuloaortic-ectasia/ Thoracic Key]


{{Medical resources
{{Medical resources
| ICD10 = I71.0
| ICD11 = {{ICD11|BD50.3Y}}
| ICD9 = <!--{{ICD9|xxx}}-->
| ICD10 = {{ICD10|I71.2}}
| ICDO =
| ICD10CM = <!-- {{ICD10CM|Xxx.xxxx}} -->
| OMIM = 607086
| ICD9 = {{ICD9|441.2}}
| DiseasesDB =
| ICDO =
| MedlinePlus =
| OMIM = 607086
| MeshID = C562834
| eMedicineSubj =
| DiseasesDB = 30073
| eMedicineTopic =
| MeSH =
| SNOMED CT = 253646008
| Curlie =
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| GARDName = Familial aortic dissection
| RP = 35726
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| OB =
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}}


[[Category:Vascular diseases]]
[[Category:Vascular diseases]]


{{circulatory-disease-stub}}

Revision as of 02:53, 29 December 2023

Annuloaortic ectasia
Other namesCystic medial necrosis of aorta[1]
Segments of the aorta, including the thoracic aorta, ascending aorta, aortic arch, descending thoracic aorta, abdominal aorta, suprarenal abdominal aorta, and infrarenal abdominal aorta.
SpecialtyCardiology

Annuloaortic ectasia is characterized by pure aortic valve regurgitation and aneurysmal dilatation of the ascending aorta.[2] Men are more likely than women to develop idiopathic annuloaortic ectasia, which usually manifests in the fourth or sixth decades of life. Additional factors that contribute to this condition include osteogenesis imperfecta, inflammatory aortic diseases, intrinsic valve disease, Loeys-Dietz syndrome, Marfan syndrome, and operated congenital heart disease.[3]

On a gross level, there is a pear-shaped, symmetric enlargement due to proximal aortic dilation. The aortic wall dilatation at the commissural level causes the cusps to effectively shorten and prevent them from converging during systole, which results in aortic valve incompetence. The arch is typically spared from the aneurysmal process, though it may involve the entire ascending aorta. The ectatic aorta may experience dissections. Dissections of the ascending aorta are typically tiny, confined, and restricted. Aortic rupture can happen even if there is no dissection.[3]

The term was first coined by the American heart surgeon Denton Cooley in 1961.[4]

Signs and symptoms

Symptoms are often neurological or circulatory.[5] Symptoms include dyspnea, respiratory distress, heart failure, atrial tachycardia,[6] pain, cardiac tamponade,[7] palpitations, and malaise.[8] Aortic valve regurgitation is found in about 77% of all patients.[9]

Treatment

For many years, the gold standard treatment for patients with aortic valve disease and aortic root aneurysms was to replace both the aortic valve and the ascending aorta with a composite graft. This also applies to patients with different levels of AI and annuloaortic ectasia, where the aortic valve may be largely preserved without any structural abnormalities.[10]

See also

References

  1. ^ "Orphanet: Familial aortic dissection". Orpha.net. Retrieved 29 May 2019.
  2. ^ An, Zhao; Xu, Zhi-Yun; Poullis, Mike; Tang, Paul C.; Oliveira, Nilto De; Rylski, Bartosz; Milewski, Rita K.; Urbanski, Paul P.; Diegeler, Anno; Kumar, Neeraj; Yang, Laurence T. (December 1, 2004). "Aortic valve sparing operations: basic concepts". International Journal of Cardiology. 97. Elsevier: 61–66. doi:10.1016/j.ijcard.2004.08.010. ISSN 0167-5273. Retrieved December 29, 2023.
  3. ^ a b Kinoshita, Ryoji; Arai, Hirokuni; Barillà, David; Benedetto, Filippo; Ladich, E.; Virmani, R.; Seidman, M.A.; McManus, B.M.; Subbotin, Vladimir M.; Spanos, K.; Giannoukas, A.D. (January 1, 2016). "Vascular Pathobiology: Atherosclerosis and Large Vessel Disease". Academic Press. p. 85–124. doi:10.1016/B978-0-12-420219-1.00003-3. Retrieved December 29, 2023.
  4. ^ Kaiser, Larry; Kron, Irving L.; Spray, Thomas L. (2013-12-24). Mastery of Cardiothoracic Surgery. ISBN 9781451113150.
  5. ^ S, Gelsomino; S, Romagnoli; F, Gori; G, Nesi; C, Anichini; C, Sorbara; P, Stefàno; GF, Gensini. "Annuloaortic ectasia and giant cell arteritis". The Annals of thoracic surgery. 80 (1). Ann Thorac Surg. doi:10.1016/j.athoracsur.2005.01.063. ISSN 1552-6259. PMID 15975349. Retrieved December 29, 2023.
  6. ^ G, Nesi; C, Anichini; E, Pedemonte; S, Tozzini; G, Calamai; GF, Montesi; F, Gori. "Giant cell arteritis presenting with annuloaortic ectasia". Chest. 121 (4). Chest. doi:10.1378/chest.121.4.1365. ISSN 0012-3692. PMID 11948080. Retrieved December 29, 2023.
  7. ^ T, Fujii; S, Sumiyoshi; T, Koga; M, Nishizaka; R, Matsukawa; H, Kuwano; K, Sueishi (2007). "An autopsy case report of annuloaortic ectasia with cardiac tamponade ruptured from an aneurysm of the right Valsalva sinus". Pathology, research and practice. 203 (9). Pathol Res Pract. doi:10.1016/j.prp.2007.05.007. ISSN 0344-0338. PMID 17646055. Retrieved December 29, 2023.
  8. ^ H, Harada; Y, Honma; Y, Hachiro; T, Mawatari; T, Abe. "Composite graft replacement after aortic valvuloplasty in Takayasu arteritis". The Annals of thoracic surgery. 73 (2). Ann Thorac Surg. doi:10.1016/s0003-4975(01)03363-x. ISSN 0003-4975. PMID 11845892. Retrieved December 29, 2023.
  9. ^ Yuan, Shi-Min; Lin, Hong. "Annuloaortic Ectasia and Arteritis: Clinical Features, Treatments of Choice, and Causative Relations". Brazilian Journal of Cardiovascular Surgery. 34 (4). Sociedade Brasileira de Cirurgia Cardiovascular. doi:10.21470/1678-9741-2018-0252. PMID 31454202. Retrieved December 29, 2023.
  10. ^ Martino, Andrea De; Re, Federico Del; Blasi, Stefania; Celiento, Michele; Ravenni, Giacomo; Pratali, Stefano; Milano, Aldo D.; Bortolotti, Uberto. "Surgical Treatment of Annuloaortic Ectasia – Replace or Repair?". AORTA Journal. 5 (5). Thieme Medical Publishers. doi:10.12945/j.aorta.2017.17.044. PMID 29657952. Retrieved December 29, 2023.

Further reading

External links