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Idiopathic spontaneous coronary artery dissection

MedGen UID:
377701
Concept ID:
C1852540
Disease or Syndrome
Synonym: Coronary artery dissection, spontaneous
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0007385
OMIM®: 122455
Orphanet: ORPHA458718

Definition

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. [from ORDO]

Clinical features

From HPO
Coronary artery dissection
MedGen UID:
881843
Concept ID:
C0340648
Disease or Syndrome
Acute occurrence of a dissection (tear within the tunica intima and entry of blood into the tunica media) of a coronary artery.
Cystic medial necrosis
MedGen UID:
1638446
Concept ID:
C4551473
Disease or Syndrome
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.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIdiopathic spontaneous coronary artery dissection

Recent clinical studies

Etiology

Mkoko P, Pandie S, Ntsekhe M
S Afr Med J 2019 Jun 28;109(7):477-479. doi: 10.7196/SAMJ.2019.v109i7.13997. PMID: 31266572
Rohit MK, Garg PK, Hariram V, Gupta A, Grover A
Indian Heart J 2008 Jul-Aug;60(4):346-8. PMID: 19242014
Maeder M, Ammann P, Angehrn W, Rickli H
Int J Cardiol 2005 Jun 8;101(3):363-9. doi: 10.1016/j.ijcard.2004.03.045. PMID: 15907402
Mahenthiran J, Revankar R, Koka V, Hoo J, Shenoy M
J Natl Med Assoc 2000 Feb;92(2):87-90. PMID: 10800297Free PMC Article

Diagnosis

Mkoko P, Pandie S, Ntsekhe M
S Afr Med J 2019 Jun 28;109(7):477-479. doi: 10.7196/SAMJ.2019.v109i7.13997. PMID: 31266572
Datta G
J Invasive Cardiol 2012 Apr;24(4):E84-6. PMID: 22477765
Bicer M, Saba D, Ozdemir B, Ercan A
Thorac Cardiovasc Surg 2008 Dec;56(8):486-8. Epub 2008 Nov 14 doi: 10.1055/s-2007-989429. PMID: 19012216
Maeder M, Ammann P, Angehrn W, Rickli H
Int J Cardiol 2005 Jun 8;101(3):363-9. doi: 10.1016/j.ijcard.2004.03.045. PMID: 15907402
Mahenthiran J, Revankar R, Koka V, Hoo J, Shenoy M
J Natl Med Assoc 2000 Feb;92(2):87-90. PMID: 10800297Free PMC Article

Therapy

Maeder M, Ammann P, Angehrn W, Rickli H
Int J Cardiol 2005 Jun 8;101(3):363-9. doi: 10.1016/j.ijcard.2004.03.045. PMID: 15907402
Mahenthiran J, Revankar R, Koka V, Hoo J, Shenoy M
J Natl Med Assoc 2000 Feb;92(2):87-90. PMID: 10800297Free PMC Article

Prognosis

Bartolini D, Vischi M, Bellone P
J Cardiovasc Med (Hagerstown) 2008 Mar;9(3):308-10. doi: 10.2459/JCM.0b013e3280117079. PMID: 18301155
Maeder M, Ammann P, Angehrn W, Rickli H
Int J Cardiol 2005 Jun 8;101(3):363-9. doi: 10.1016/j.ijcard.2004.03.045. PMID: 15907402
Mahenthiran J, Revankar R, Koka V, Hoo J, Shenoy M
J Natl Med Assoc 2000 Feb;92(2):87-90. PMID: 10800297Free PMC Article

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