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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
Robicsek F, Cook JW, Reames MK Sr, Skipper ER
J Thorac Cardiovasc Surg 2004 Oct;128(4):562-70. doi: 10.1016/j.jtcvs.2004.04.032. PMID: 15457157
Millar AJ, Gilbert RD, Brown RA, Immelman EJ, Burkimsher DA, Cywes S
J Pediatr Surg 1996 Dec;31(12):1624-8. doi: 10.1016/s0022-3468(96)90034-2. PMID: 8986973
Rich BS
Med Clin North Am 1994 Mar;78(2):267-88. doi: 10.1016/s0025-7125(16)30159-6. PMID: 8121211

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
Tanaka A, Milner R, Ota T
Gen Thorac Cardiovasc Surg 2015 May;63(5):245-59. Epub 2015 Jan 31 doi: 10.1007/s11748-015-0521-3. PMID: 25636900
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

Therapy

Leontyev S, Borger MA, Legare JF, Merk D, Hahn J, Seeburger J, Lehmann S, Mohr FW
Eur J Cardiothorac Surg 2012 Mar;41(3):641-6. Epub 2011 Dec 1 doi: 10.1093/ejcts/ezr070. PMID: 22345184
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
Takayama T, Miyata T, Nagawa H
J Vasc Surg 2009 May;49(5):1162-5. doi: 10.1016/j.jvs.2008.12.007. PMID: 19307079
Sato J, Kawakami T, Nakabayashi K, Fukuoka K, Hirano K, Terado Y, Yokoyama K, Ohtsuka T, Ohkura Y, Fujioka Y, Kurata A
Pathol Res Pract 2008;204(11):845-50. Epub 2008 Jul 23 doi: 10.1016/j.prp.2008.05.009. PMID: 18653288
Soyer R, Bessou JP, Bouchart F, Redonnet M, Mouton-Schleifer D, Arrignon J
Ann Thorac Surg 1994 Aug;58(2):425-8. doi: 10.1016/0003-4975(94)92220-9. PMID: 8067843

Prognosis

Balistreri CR, Pisano C, Candore G, Maresi E, Codispoti M, Ruvolo G
Eur J Cardiothorac Surg 2013 Jun;43(6):e180-6. Epub 2012 Dec 17 doi: 10.1093/ejcts/ezs630. PMID: 23248206
Gravholt CH, Landin-Wilhelmsen K, Stochholm K, Hjerrild BE, Ledet T, Djurhuus CB, Sylvén L, Baandrup U, Kristensen BØ, Christiansen JS
Cardiol Young 2006 Oct;16(5):430-6. doi: 10.1017/S1047951106000928. PMID: 16984695
Wagner WH, Allins AD, Treiman RL, Cohen JL, Foran RF, Levin PM, Cossman DV
Ann Vasc Surg 1997 Jul;11(4):342-7. doi: 10.1007/s100169900058. PMID: 9236988
Millar AJ, Gilbert RD, Brown RA, Immelman EJ, Burkimsher DA, Cywes S
J Pediatr Surg 1996 Dec;31(12):1624-8. doi: 10.1016/s0022-3468(96)90034-2. PMID: 8986973
Messina LM, Reilly LM, Goldstone J, Ehrenfeld WK, Ferrell LD, Stoney RJ
Ann Surg 1986 Sep;204(3):331-9. doi: 10.1097/00000658-198609000-00012. PMID: 3753060Free PMC Article

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
Balistreri CR, Pisano C, Candore G, Maresi E, Codispoti M, Ruvolo G
Eur J Cardiothorac Surg 2013 Jun;43(6):e180-6. Epub 2012 Dec 17 doi: 10.1093/ejcts/ezs630. PMID: 23248206
Prieto D, Antunes MJ
Rev Port Cardiol 2005 Apr;24(4):583-604. PMID: 15977781
Nakashima Y, Shiokawa Y, Sueishi K
Lab Invest 1990 Jun;62(6):751-60. PMID: 2359259
Larson EW, Edwards WD
Am J Cardiol 1984 Mar 1;53(6):849-55. doi: 10.1016/0002-9149(84)90418-1. PMID: 6702637

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