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Cystic medial necrosis

MedGen UID:
1638446
Concept ID:
C4551473
Disease or Syndrome
Synonyms: Arterial cystic medial necrosis; Cystic adventitial disease; Cystic medionecrosis
SNOMED CT: Cystic adventitial disease (234021009); Cystic medial necrosis (234021009); Cystic medial necrosis (42182000); Cystic medionecrosis (42182000)
 
HPO: HP:0012180

Definition

A disorder of large arteries, in particular the aorta, characterized by an accumulation of basophilic ground substance in the media with cyst-like lesions associated with degenerative changes of collagen, elastin and the vascular smooth muscle cells. [from HPO]

Conditions with this feature

Arterial dissection-lentiginosis syndrome
MedGen UID:
325345
Concept ID:
C1838122
Disease or Syndrome
A rare association syndrome, reported in several members of two families to date, characterized by arterial dissection, occurring at an early age and presenting with a range of manifestations depending on the vascular territory involved (ex. headache, dysphasia, hemiparesis), in association with cystic medial necrosis and multiple lentigines (brown and black in color and mainly affecting the skin of the trunk and extremities).
Aortic aneurysm, familial thoracic 4
MedGen UID:
338704
Concept ID:
C1851504
Disease or Syndrome
Any familial thoracic aortic aneurysm and aortic dissection in which the cause of the disease is a mutation in the MYH11 gene.
Idiopathic spontaneous coronary artery dissection
MedGen UID:
377701
Concept ID:
C1852540
Disease or Syndrome
A rare vascular disease characterized by idiopathic detachment of the layers of the walls of coronary arteries, creating a false lumen which limits the main coronary flow, leading to myocardial ischemia. Clinical manifestations include acute coronary syndromes, especially ST-segment elevation myocardial infarction (STEMI), syncope, cardiogenic shock, or sudden cardiac death. The condition typically affects young women.

Professional guidelines

PubMed

Halushka MK, Angelini A, Bartoloni G, Basso C, Batoroeva L, Bruneval P, Buja LM, Butany J, d'Amati G, Fallon JT, Gallagher PJ, Gittenberger-de Groot AC, Gouveia RH, Kholova I, Kelly KL, Leone O, Litovsky SH, Maleszewski JJ, Miller DV, Mitchell RN, Preston SD, Pucci A, Radio SJ, Rodriguez ER, Sheppard MN, Stone JR, Suvarna SK, Tan CD, Thiene G, Veinot JP, van der Wal AC
Cardiovasc Pathol 2016 May-Jun;25(3):247-257. Epub 2016 Mar 12 doi: 10.1016/j.carpath.2016.03.002. PMID: 27031798
Shumacker HB Jr
J Thorac Cardiovasc Surg 1972 Jan;63(1):1-13. PMID: 5008118

Recent clinical studies

Etiology

El-Hamamsy I, Yacoub MH
Nat Rev Cardiol 2009 Dec;6(12):771-86. Epub 2009 Nov 3 doi: 10.1038/nrcardio.2009.191. PMID: 19884902
Braverman AC, Güven H, Beardslee MA, Makan M, Kates AM, Moon MR
Curr Probl Cardiol 2005 Sep;30(9):470-522. doi: 10.1016/j.cpcardiol.2005.06.002. PMID: 16129122
Rich BS
Med Clin North Am 1994 Mar;78(2):267-88. doi: 10.1016/s0025-7125(16)30159-6. PMID: 8121211
Marsalese DL, Moodie DS, Lytle BW, Cosgrove DM, Ratliff NB, Goormastic M, Kovacs A
J Am Coll Cardiol 1990 Jul;16(1):68-73. doi: 10.1016/0735-1097(90)90458-2. PMID: 2358606
Epstein SE, Maron BJ
J Am Coll Cardiol 1986 Jan;7(1):220-30. doi: 10.1016/s0735-1097(86)80285-6. PMID: 3079780

Diagnosis

Halushka MK, Angelini A, Bartoloni G, Basso C, Batoroeva L, Bruneval P, Buja LM, Butany J, d'Amati G, Fallon JT, Gallagher PJ, Gittenberger-de Groot AC, Gouveia RH, Kholova I, Kelly KL, Leone O, Litovsky SH, Maleszewski JJ, Miller DV, Mitchell RN, Preston SD, Pucci A, Radio SJ, Rodriguez ER, Sheppard MN, Stone JR, Suvarna SK, Tan CD, Thiene G, Veinot JP, van der Wal AC
Cardiovasc Pathol 2016 May-Jun;25(3):247-257. Epub 2016 Mar 12 doi: 10.1016/j.carpath.2016.03.002. PMID: 27031798
El-Hamamsy I, Yacoub MH
Nat Rev Cardiol 2009 Dec;6(12):771-86. Epub 2009 Nov 3 doi: 10.1038/nrcardio.2009.191. PMID: 19884902
Braverman AC, Güven H, Beardslee MA, Makan M, Kates AM, Moon MR
Curr Probl Cardiol 2005 Sep;30(9):470-522. doi: 10.1016/j.cpcardiol.2005.06.002. PMID: 16129122
Prieto D, Antunes MJ
Rev Port Cardiol 2005 Apr;24(4):583-604. PMID: 15977781
Rich BS
Med Clin North Am 1994 Mar;78(2):267-88. doi: 10.1016/s0025-7125(16)30159-6. PMID: 8121211

Therapy

Seike Y, Matsuda H, Ishibashi-Ueda H, Morisaki H, Morisaki T, Minatoya K, Ogino H
Ann Thorac Cardiovasc Surg 2021 Feb 20;27(1):56-63. Epub 2021 Jan 6 doi: 10.5761/atcs.oa.20-00223. PMID: 33408307Free PMC Article
Rong LQ, Palumbo MC, Rahouma M, Meineri M, Arguelles GR, Kim J, Lau C, Devereux RB, Pryor KO, Girardi LN, Redaelli A, Gaudino MFL, Weinsaft JW
Eur J Vasc Endovasc Surg 2019 Oct;58(4):521-528. Epub 2019 Aug 21 doi: 10.1016/j.ejvs.2019.05.003. PMID: 31445862
El-Hamamsy I, Yacoub MH
Nat Rev Cardiol 2009 Dec;6(12):771-86. Epub 2009 Nov 3 doi: 10.1038/nrcardio.2009.191. PMID: 19884902
Isner JM, Donaldson RF, Fulton D, Bhan I, Payne DD, Cleveland RJ
Circulation 1987 Apr;75(4):689-95. doi: 10.1161/01.cir.75.4.689. PMID: 2951035
Nath PH, Zollikofer C, Castaneda-Zuniga WR, Velasquez G, Formanek A, Nicoloff D, Amplatz K
Radiology 1979 Apr;131(1):43-51. doi: 10.1148/131.1.43. PMID: 424605

Prognosis

Akutsu K, Kawamoto M, Sato N, Yamamoto T, Tamura K, Mizuno K, Tanaka K
J Nippon Med Sch 2012;79(2):159-62. doi: 10.1272/jnms.79.159. PMID: 22687361
Girdauskas E, Kuntze T, Borger MA, Doenst T, Mochalski M, Walther T, Falk V, Mohr FW
Ann Thorac Surg 2008 Mar;85(3):972-7. doi: 10.1016/j.athoracsur.2007.10.051. PMID: 18291180
Ueda T, Shimizu H, Aeba R, Shin H, Katogi T, Yozu R, Kawada S
Jpn J Thorac Cardiovasc Surg 1999 Feb;47(2):73-8. doi: 10.1007/BF03217945. PMID: 10097476
Marsalese DL, Moodie DS, Lytle BW, Cosgrove DM, Ratliff NB, Goormastic M, Kovacs A
J Am Coll Cardiol 1990 Jul;16(1):68-73. doi: 10.1016/0735-1097(90)90458-2. PMID: 2358606
Epstein SE, Maron BJ
J Am Coll Cardiol 1986 Jan;7(1):220-30. doi: 10.1016/s0735-1097(86)80285-6. PMID: 3079780

Clinical prediction guides

Schoenmakers E, Marelli F, Jørgensen HF, Visser WE, Moran C, Groeneweg S, Avalos C, Jurgens SJ, Figg N, Finigan A, Wali N, Agostini M, Wardle-Jones H, Lyons G, Rusk R, Gopalan D, Twiss P, Visser JJ, Goddard M, Nashef SAM, Heijmen R, Clift P, Sinha S, Pirruccello JP, Ellinor PT, Busch-Nentwich EM, Ramirez-Solis R, Murphy MP, Persani L, Bennett M, Chatterjee K
Nat Commun 2023 Dec 2;14(1):7994. doi: 10.1038/s41467-023-43851-6. PMID: 38042913Free PMC Article
Wada H, Sakata N, Tashiro T
Heart Vessels 2016 Nov;31(11):1855-1861. Epub 2016 Feb 18 doi: 10.1007/s00380-016-0813-2. PMID: 26892532
Prieto D, Antunes MJ
Rev Port Cardiol 2005 Apr;24(4):583-604. PMID: 15977781
Fukunaga S, Akashi H, Tayama K, Kawano H, Kosuga K, Aoyagi S
J Heart Valve Dis 1997 Mar;6(2):181-3. PMID: 9130129
Ondreyco SM, Lewis HD Jr, Hartman CR
Arch Intern Med 1980 Apr;140(4):547-9. PMID: 7362391

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