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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).
See: Condition Record
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.
See: Condition Record
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.
See: Condition Record
Aortic aneurysm, familial thoracic 4
Arterial dissection-lentiginosis syndrome
Idiopathic spontaneous coronary artery dissection

Professional guidelines

PubMed

Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association For European Cardiovascular Pathology: II. Noninflammatory degenerative diseases - nomenclature and diagnostic criteria.
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
Operative treatment of aneurysms of the thoracic aorta due to cystic medial necrosis.
Shumacker HB Jr
J Thorac Cardiovasc Surg 1972 Jan;63(1):1-13. PMID: 5008118

Recent clinical studies

Etiology

Sudden death due to aortic rupture in acromegaly.
Maffei P, Menegazzo C, Michelotto M, Salmaso R, Martini C, De Carlo E, Carli M, Barban M, Fassina A, Vettor R, Sicolo N
Heart Vessels 2008 Jan;23(1):71-4. Epub 2008 Feb 14 doi: 10.1007/s00380-007-1012-y. PMID: 18273550
The bicuspid aortic valve.
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
Aortic dissection after ingestion of "ecstasy" (MDMA).
Duflou J, Mark A
Am J Forensic Med Pathol 2000 Sep;21(3):261-3. doi: 10.1097/00000433-200009000-00016. PMID: 10990289
Sudden death screening.
Rich BS
Med Clin North Am 1994 Mar;78(2):267-88. doi: 10.1016/s0025-7125(16)30159-6. PMID: 8121211
Sudden death and the competitive athlete: perspectives on preparticipation screening studies.
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

Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association For European Cardiovascular Pathology: II. Noninflammatory degenerative diseases - nomenclature and diagnostic criteria.
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
Acute aortic dissection.
Prieto D, Antunes MJ
Rev Port Cardiol 2005 Apr;24(4):583-604. PMID: 15977781
Turner's syndrome and cardiovascular anomalies: a case report and review of the literature.
Subramaniam PN
Am J Med Sci 1989 Apr;297(4):260-2. doi: 10.1097/00000441-198904000-00014. PMID: 2650544
Sudden death and the competitive athlete: perspectives on preparticipation screening studies.
Epstein SE, Maron BJ
J Am Coll Cardiol 1986 Jan;7(1):220-30. doi: 10.1016/s0735-1097(86)80285-6. PMID: 3079780
Cystic medial necrosis. A correctable cause of the superior vena cava syndrome.
Roberts AJ, Jaffe RB, Michaelis LL, Morrow AG
Arch Surg 1974 Jul;109(1):84-6. doi: 10.1001/archsurg.1974.01360010062015. PMID: 4834138

Therapy

Rapidly growing aortic arch aneurysm in Behcet's disease.
Kojima N, Sakano Y, Ohki S, Misawa Y
Interact Cardiovasc Thorac Surg 2011 Mar;12(3):502-4. Epub 2010 Dec 21 doi: 10.1510/icvts.2010.260976. PMID: 21177298
Cellular and molecular mechanisms of thoracic aortic aneurysms.
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
Aortic dissection after ingestion of "ecstasy" (MDMA).
Duflou J, Mark A
Am J Forensic Med Pathol 2000 Sep;21(3):261-3. doi: 10.1097/00000433-200009000-00016. PMID: 10990289
Aortic dissection complicating coronary angioplasty in cystic medial necrosis.
Pande AK, Gosselin G, Leclerc Y, Leung TK
Am Heart J 1996 Jun;131(6):1221-3. doi: 10.1016/s0002-8703(96)90101-5. PMID: 8644605
Operative treatment of aneurysms of the thoracic aorta due to cystic medial necrosis.
Shumacker HB Jr
J Thorac Cardiovasc Surg 1972 Jan;63(1):1-13. PMID: 5008118

Prognosis

Acute aortic dissection associated with cystic medial necrosis of unknown etiology.
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
Sudden death due to aortic rupture in acromegaly.
Maffei P, Menegazzo C, Michelotto M, Salmaso R, Martini C, De Carlo E, Carli M, Barban M, Fassina A, Vettor R, Sicolo N
Heart Vessels 2008 Jan;23(1):71-4. Epub 2008 Feb 14 doi: 10.1007/s00380-007-1012-y. PMID: 18273550
Aortic dissection after ingestion of "ecstasy" (MDMA).
Duflou J, Mark A
Am J Forensic Med Pathol 2000 Sep;21(3):261-3. doi: 10.1097/00000433-200009000-00016. PMID: 10990289
Spontaneous vertebral artery dissection as a complication of autosomal dominant polycystic kidney disease.
Larranaga J, Rutecki GW, Whittier FC
Am J Kidney Dis 1995 Jan;25(1):70-4. doi: 10.1016/0272-6386(95)90629-0. PMID: 7810537
Sudden death and the competitive athlete: perspectives on preparticipation screening studies.
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

Selenoprotein deficiency disorder predisposes to aortic aneurysm formation.
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
Sudden death of a young woman due to aortic dissection caused by Turner's syndrome.
Mimasaka S, Ohtsu Y, Tsunenari S, Matsukawa A, Hashiyada M, Takahashi S, Funayama M
Pathol Int 2007 Apr;57(4):219-23. doi: 10.1111/j.1440-1827.2007.02084.x. PMID: 17316418
Acute aortic dissection.
Prieto D, Antunes MJ
Rev Port Cardiol 2005 Apr;24(4):583-604. PMID: 15977781
Aortic root replacement for annuloaortic ectasia in Shprintzen-Goldberg syndrome: a case report.
Fukunaga S, Akashi H, Tayama K, Kawano H, Kosuga K, Aoyagi S
J Heart Valve Dis 1997 Mar;6(2):181-3. PMID: 9130129
Dissecting aortic aneurysm associated with congenital bicuspid aortic valve.
Edwards WD, Leaf DS, Edwards JE
Circulation 1978 May;57(5):1022-5. doi: 10.1161/01.cir.57.5.1022. PMID: 639201

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