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Aortic elastic fiber thinning

MedGen UID:
1697352
•Concept ID:
C5139118
•
Pathologic Function
Synonym: Aortic elastic fibre thinning
 
HPO: HP:0032084

Definition

A thinning out of elastic fibers of the media of the aorta that creates widening of intralamellar spaces, as identified on a stain for elastic fibers. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAortic elastic fiber thinning

Professional guidelines

PubMed

Molecular genetic evaluation of pediatric renovascular hypertension due to renal artery stenosis and abdominal aortic coarctation in neurofibromatosis type 1.
Coleman DM, Wang Y, Yang ML, Hunker KL, Birt I, Bergin IL, Li JZ, Stanley JC, Ganesh SK
Hum Mol Genet 2022 Feb 3;31(3):334-346. doi: 10.1093/hmg/ddab241. PMID: 34476477Free PMC Article

Recent clinical studies

Etiology

The Etiological Heterogeneity of Bicuspid Aortopathy between Ascending and Root Morphotype.
Li F, Li X, Wang YT, Yu CT, Yin G, Chen XY, Zhao S, Wang W, Gao Q
Heart Surg Forum 2020 Dec 22;23(6):E913-E919. doi: 10.1532/hsf.3333. PMID: 33399529
Histopathological evidence of aortopathy in newborns and infants with Tetralogy of Fallot at the time of the surgical repair.
Fabian O, Gebauer R, Koblizek M, Hornofova L, Janousek J
Cardiovasc Pathol 2019 May-Jun;40:59-64. Epub 2019 Feb 28 doi: 10.1016/j.carpath.2019.02.004. PMID: 30904751
Aortic valve-mediated wall shear stress is heterogeneous and predicts regional aortic elastic fiber thinning in bicuspid aortic valve-associated aortopathy.
Bollache E, Guzzardi DG, Sattari S, Olsen KE, Di Martino ES, Malaisrie SC, van Ooij P, Collins J, Carr J, McCarthy PM, Markl M, Barker AJ, Fedak PWM
J Thorac Cardiovasc Surg 2018 Dec;156(6):2112-2120.e2. Epub 2018 Jun 12 doi: 10.1016/j.jtcvs.2018.05.095. PMID: 30060930Free PMC Article
Pathogenesis of aortic wall complications in Marfan syndrome.
Grewal N, Gittenberger-de Groot AC
Cardiovasc Pathol 2018 Mar-Apr;33:62-69. Epub 2018 Feb 2 doi: 10.1016/j.carpath.2018.01.005. PMID: 29433109
Aneurysmal and occlusive atherosclerosis of the human abdominal aorta.
Xu C, Zarins CK, Glagov S
J Vasc Surg 2001 Jan;33(1):91-6. doi: 10.1067/mva.2001.109744. PMID: 11137928

Diagnosis

Molecular genetic evaluation of pediatric renovascular hypertension due to renal artery stenosis and abdominal aortic coarctation in neurofibromatosis type 1.
Coleman DM, Wang Y, Yang ML, Hunker KL, Birt I, Bergin IL, Li JZ, Stanley JC, Ganesh SK
Hum Mol Genet 2022 Feb 3;31(3):334-346. doi: 10.1093/hmg/ddab241. PMID: 34476477Free PMC Article
The Etiological Heterogeneity of Bicuspid Aortopathy between Ascending and Root Morphotype.
Li F, Li X, Wang YT, Yu CT, Yin G, Chen XY, Zhao S, Wang W, Gao Q
Heart Surg Forum 2020 Dec 22;23(6):E913-E919. doi: 10.1532/hsf.3333. PMID: 33399529
Quantification of aortic and cutaneous elastin and collagen morphology in Marfan syndrome by multiphoton microscopy.
Cui JZ, Tehrani AY, Jett KA, Bernatchez P, van Breemen C, Esfandiarei M
J Struct Biol 2014 Sep;187(3):242-253. Epub 2014 Jul 30 doi: 10.1016/j.jsb.2014.07.003. PMID: 25086405
Noninvasive evaluation of arterial abnormalities in young patients with neurofibromatosis type 1.
Tedesco MA, Ratti G, Di Salvo G, Martiniello AR, Limongelli G, Grieco M, Calabrese E, Grassia C, Iacono A, Lama G
Angiology 2000 Sep;51(9):733-41. doi: 10.1177/000331970005100905. PMID: 10999614

Therapy

Pregnancy-associated aortopathy and sudden postpartum death.
Byard RW
Forensic Sci Med Pathol 2023 Jun;19(2):266-268. Epub 2023 Apr 5 doi: 10.1007/s12024-023-00606-5. PMID: 37020086Free PMC Article
Histopathological evidence of aortopathy in newborns and infants with Tetralogy of Fallot at the time of the surgical repair.
Fabian O, Gebauer R, Koblizek M, Hornofova L, Janousek J
Cardiovasc Pathol 2019 May-Jun;40:59-64. Epub 2019 Feb 28 doi: 10.1016/j.carpath.2019.02.004. PMID: 30904751
Histopathological abnormalities in the central arteries and veins of Fontan subjects.
Hays BS, Baker M, Laib A, Tan W, Udholm S, Goldstein BH, Sanders SP, Opotowsky AR, Veldtman GR
Heart 2018 Feb;104(4):324-331. Epub 2017 Sep 29 doi: 10.1136/heartjnl-2017-311838. PMID: 28970278
Systemic injection of planktonic forms of mammalian-derived nanoparticles alters arterial response to injury in rabbits.
Schwartz MK, Lieske JC, Hunter LW, Miller VM
Am J Physiol Heart Circ Physiol 2009 May;296(5):H1434-41. Epub 2009 Mar 13 doi: 10.1152/ajpheart.00993.2008. PMID: 19286948Free PMC Article

Prognosis

Histopathological evidence of aortopathy in newborns and infants with Tetralogy of Fallot at the time of the surgical repair.
Fabian O, Gebauer R, Koblizek M, Hornofova L, Janousek J
Cardiovasc Pathol 2019 May-Jun;40:59-64. Epub 2019 Feb 28 doi: 10.1016/j.carpath.2019.02.004. PMID: 30904751
Aortic valve-mediated wall shear stress is heterogeneous and predicts regional aortic elastic fiber thinning in bicuspid aortic valve-associated aortopathy.
Bollache E, Guzzardi DG, Sattari S, Olsen KE, Di Martino ES, Malaisrie SC, van Ooij P, Collins J, Carr J, McCarthy PM, Markl M, Barker AJ, Fedak PWM
J Thorac Cardiovasc Surg 2018 Dec;156(6):2112-2120.e2. Epub 2018 Jun 12 doi: 10.1016/j.jtcvs.2018.05.095. PMID: 30060930Free PMC Article
Floppy aortic valves without aortic root dilatation: clinical, histologic, and ultrastructural studies.
Tamura K, I-Ida T, Fujii T, Tanaka S, Asano G
J Nippon Med Sch 2002 Aug;69(4):355-64. doi: 10.1272/jnms.69.355. PMID: 12187368
Phenotypic alteration of vascular smooth muscle cells precedes elastolysis in a mouse model of Marfan syndrome.
Bunton TE, Biery NJ, Myers L, Gayraud B, Ramirez F, Dietz HC
Circ Res 2001 Jan 19;88(1):37-43. doi: 10.1161/01.res.88.1.37. PMID: 11139471
Coronary artery aneurysms develop in weanling rabbits with serum sickness but not in mature rabbits. An experimental model for Kawasaki disease in humans.
Onouchi Z, Ikuta K, Nagamatsu K, Tamiya H, Sakakibara Y, Ando M
Angiology 1995 Aug;46(8):679-87. doi: 10.1177/000331979504600806. PMID: 7639414

Clinical prediction guides

Molecular genetic evaluation of pediatric renovascular hypertension due to renal artery stenosis and abdominal aortic coarctation in neurofibromatosis type 1.
Coleman DM, Wang Y, Yang ML, Hunker KL, Birt I, Bergin IL, Li JZ, Stanley JC, Ganesh SK
Hum Mol Genet 2022 Feb 3;31(3):334-346. doi: 10.1093/hmg/ddab241. PMID: 34476477Free PMC Article
The Etiological Heterogeneity of Bicuspid Aortopathy between Ascending and Root Morphotype.
Li F, Li X, Wang YT, Yu CT, Yin G, Chen XY, Zhao S, Wang W, Gao Q
Heart Surg Forum 2020 Dec 22;23(6):E913-E919. doi: 10.1532/hsf.3333. PMID: 33399529
Aortic valve-mediated wall shear stress is heterogeneous and predicts regional aortic elastic fiber thinning in bicuspid aortic valve-associated aortopathy.
Bollache E, Guzzardi DG, Sattari S, Olsen KE, Di Martino ES, Malaisrie SC, van Ooij P, Collins J, Carr J, McCarthy PM, Markl M, Barker AJ, Fedak PWM
J Thorac Cardiovasc Surg 2018 Dec;156(6):2112-2120.e2. Epub 2018 Jun 12 doi: 10.1016/j.jtcvs.2018.05.095. PMID: 30060930Free PMC Article
Quantification of aortic and cutaneous elastin and collagen morphology in Marfan syndrome by multiphoton microscopy.
Cui JZ, Tehrani AY, Jett KA, Bernatchez P, van Breemen C, Esfandiarei M
J Struct Biol 2014 Sep;187(3):242-253. Epub 2014 Jul 30 doi: 10.1016/j.jsb.2014.07.003. PMID: 25086405
Floppy aortic valves without aortic root dilatation: clinical, histologic, and ultrastructural studies.
Tamura K, I-Ida T, Fujii T, Tanaka S, Asano G
J Nippon Med Sch 2002 Aug;69(4):355-64. doi: 10.1272/jnms.69.355. PMID: 12187368

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