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. Elsevier. 97: 61–66. doi:10.1016/j.ijcard.2004.08.010. ISSN 0167-5273. PMID 15590080. Retrieved December 29, 2023.
  3. 1 2 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. pp. 85–124. doi:10.1016/B978-0-12-420219-1.00003-3. ISBN 9780124202191. Retrieved December 29, 2023.
  4. Kaiser, Larry; Kron, Irving L.; Spray, Thomas L. (2013-12-24). Mastery of Cardiothoracic Surgery. Lippincott Williams & Wilkins. ISBN 9781451113150.
  5. S, Gelsomino; S, Romagnoli; F, Gori; G, Nesi; C, Anichini; C, Sorbara; P, Stefàno; GF, Gensini (2005). "Annuloaortic ectasia and giant cell arteritis". The Annals of Thoracic Surgery. Ann Thorac Surg. 80 (1): 101–105. 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 (2002). "Giant cell arteritis presenting with annuloaortic ectasia". Chest. 121 (4): 1365–1367. 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. Pathol Res Pract. 203 (9): 671–675. 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 (2002). "Composite graft replacement after aortic valvuloplasty in Takayasu arteritis". The Annals of Thoracic Surgery. Ann Thorac Surg. 73 (2): 644–647. doi:10.1016/s0003-4975(01)03363-x. ISSN 0003-4975. PMID 11845892. Retrieved December 29, 2023.
  9. Yuan, Shi-Min; Lin, Hong (2019). "Annuloaortic Ectasia and Arteritis: Clinical Features, Treatments of Choice, and Causative Relations". Brazilian Journal of Cardiovascular Surgery. Sociedade Brasileira de Cirurgia Cardiovascular. 34 (4): 472–479. doi:10.21470/1678-9741-2018-0252. PMC 6713369. PMID 31454202.
  10. Martino, Andrea De; Re, Federico Del; Blasi, Stefania; Celiento, Michele; Ravenni, Giacomo; Pratali, Stefano; Milano, Aldo D.; Bortolotti, Uberto (2017). "Surgical Treatment of Annuloaortic Ectasia – Replace or Repair?". AORTA Journal. Thieme Medical Publishers. 5 (5): 139–147. doi:10.12945/j.aorta.2017.17.044. PMC 5890764. PMID 29657952.

Further reading

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