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Aortic aneurysm, familial thoracic 9(AAT9)

MedGen UID:
863805
Concept ID:
C4015368
Disease or Syndrome
Synonym: AAT9
 
Gene (location): MFAP5 (12p13.31)
 
Monarch Initiative: MONDO:0014514
OMIM®: 616166

Definition

Any familial thoracic aortic aneurysm and aortic dissection in which the cause of the disease is a mutation in the MFAP5 gene. [from MONDO]

Clinical features

From HPO
Arachnodactyly
MedGen UID:
2047
Concept ID:
C0003706
Congenital Abnormality
Abnormally long and slender fingers (spider fingers).
Atrial fibrillation
MedGen UID:
445
Concept ID:
C0004238
Finding
An atrial arrhythmia characterized by disorganized atrial activity without discrete P waves on the surface EKG, but instead by an undulating baseline or more sharply circumscribed atrial deflections of varying amplitude an frequency ranging from 350 to 600 per minute.
Mitral valve prolapse
MedGen UID:
7671
Concept ID:
C0026267
Disease or Syndrome
One or both of the leaflets (cusps) of the mitral valve bulges back into the left atrium upon contraction of the left ventricle.
Thoracic aortic aneurysm
MedGen UID:
56525
Concept ID:
C0162872
Anatomical Abnormality
An abnormal localized widening (dilatation) of the thoracic aorta.
Ascending aortic dissection
MedGen UID:
322966
Concept ID:
C1836653
Disease or Syndrome
A separation of the layers within the wall of the ascending aorta. Tears in the intimal layer result in the propagation of dissection (proximally or distally) secondary to blood entering the intima-media space.
Aortic tortuosity
MedGen UID:
870555
Concept ID:
C4025003
Anatomical Abnormality
Abnormal tortuous (i.e., twisted) form of the aorta.
Disproportionate tall stature
MedGen UID:
323048
Concept ID:
C1836996
Finding
A tall and slim body build with increased arm span to height ratio (>1.05) and a reduced upper-to-lower segment ratio (<0.85), i.e., unusually long arms and legs. The extremities as well as the hands and feet are unusually slim.
Dural ectasia
MedGen UID:
377094
Concept ID:
C1851712
Finding
A widening or ballooning of the dural sac surrounding the spinal cord usually at the lumbosacral level.
Pectus carinatum
MedGen UID:
57643
Concept ID:
C0158731
Finding
A deformity of the chest caused by overgrowth of the ribs and characterized by protrusion of the sternum.
Pectus excavatum
MedGen UID:
781174
Concept ID:
C2051831
Finding
A defect of the chest wall characterized by a depression of the sternum, giving the chest ("pectus") a caved-in ("excavatum") appearance.
High palate
MedGen UID:
66814
Concept ID:
C0240635
Congenital Abnormality
Height of the palate more than 2 SD above the mean (objective) or palatal height at the level of the first permanent molar more than twice the height of the teeth (subjective).

Recent clinical studies

Etiology

Kimura N, Aizawa K, Kawahito K, Itagaki R, Yamaguchi A, Misawa Y, Siepe M, Czerny M, Beyersdorf F, Kari FA, Rylski B
Circ J 2019 Jan 25;83(2):285-294. Epub 2018 Dec 22 doi: 10.1253/circj.CJ-18-0969. PMID: 30584230
Tan L, Li Z, Zhou C, Cao Y, Zhang L, Li X, Cianflone K, Wang Y, Wang DW
Hum Mol Genet 2017 Dec 15;26(24):4814-4822. doi: 10.1093/hmg/ddx360. PMID: 28973303
Loeper F, Oosterhof J, van den Dorpel M, van der Linde D, Lu Y, Robertson E, Hambly B, Jeremy R
J Am Heart Assoc 2016 Nov 16;5(11) doi: 10.1161/JAHA.116.003705. PMID: 27852591Free PMC Article
Inoue Y, Minatoya K, Oda T, Itonaga T, Seike Y, Tanaka H, Sasaki H, Kobayashi J
Ann Thorac Surg 2016 Mar;101(3):1185-8. doi: 10.1016/j.athoracsur.2015.05.071. PMID: 26897206
Li Y, Hu J, Qian H, Gu J, Meng W, Zhang EY
J Renin Angiotensin Aldosterone Syst 2015 Dec;16(4):1130-4. Epub 2014 Sep 17 doi: 10.1177/1470320314549219. PMID: 25237166

Diagnosis

Arnaud P, Hanna N, Benarroch L, Aubart M, Bal L, Bouvagnet P, Busa T, Dulac Y, Dupuis-Girod S, Edouard T, Faivre L, Gouya L, Lacombe D, Langeois M, Leheup B, Milleron O, Naudion S, Odent S, Tchitchinadze M, Ropers J, Jondeau G, Boileau C
Genet Med 2019 Sep;21(9):2015-2024. Epub 2019 Feb 11 doi: 10.1038/s41436-019-0444-y. PMID: 30739908
Seike Y, Minatoya K, Sasaki H, Tanaka H, Itonaga T, Inoue Y, Morisaki H, Morisaki T, Ishibashi-Ueda H, Kobayashi J
Gen Thorac Cardiovasc Surg 2017 Dec;65(12):686-691. Epub 2017 Aug 14 doi: 10.1007/s11748-017-0810-0. PMID: 28808903
D'Souza RS, Slavov D, Graw S, Jirikowic J, Todd E, Rogers RK, Taylor MR
Int Angiol 2017 Oct;36(5):417-427. Epub 2017 Jan 31 doi: 10.23736/S0392-9590.17.03757-9. PMID: 28139901
Inoue Y, Minatoya K, Oda T, Itonaga T, Seike Y, Tanaka H, Sasaki H, Kobayashi J
Ann Thorac Surg 2016 Mar;101(3):1185-8. doi: 10.1016/j.athoracsur.2015.05.071. PMID: 26897206
Haruki T, Ito H, Sakata K, Kobayashi Y
Asian Cardiovasc Thorac Ann 2015 Nov;23(9):1072-4. Epub 2014 Apr 14 doi: 10.1177/0218492314532278. PMID: 24732088

Therapy

Yokawa K, Ikeno Y, Koda Y, Henmi S, Matsueda T, Takahashi H, Nakai H, Yamanaka K, Gotake Y, Tanaka H, Okita Y
Ann Thorac Surg 2019 May;107(5):1342-1347. Epub 2018 Dec 7 doi: 10.1016/j.athoracsur.2018.10.075. PMID: 30529676
Loeper F, Oosterhof J, van den Dorpel M, van der Linde D, Lu Y, Robertson E, Hambly B, Jeremy R
J Am Heart Assoc 2016 Nov 16;5(11) doi: 10.1161/JAHA.116.003705. PMID: 27852591Free PMC Article
Haruki T, Ito H, Sakata K, Kobayashi Y
Asian Cardiovasc Thorac Ann 2015 Nov;23(9):1072-4. Epub 2014 Apr 14 doi: 10.1177/0218492314532278. PMID: 24732088

Prognosis

Arnaud P, Hanna N, Benarroch L, Aubart M, Bal L, Bouvagnet P, Busa T, Dulac Y, Dupuis-Girod S, Edouard T, Faivre L, Gouya L, Lacombe D, Langeois M, Leheup B, Milleron O, Naudion S, Odent S, Tchitchinadze M, Ropers J, Jondeau G, Boileau C
Genet Med 2019 Sep;21(9):2015-2024. Epub 2019 Feb 11 doi: 10.1038/s41436-019-0444-y. PMID: 30739908
D'Souza RS, Slavov D, Graw S, Jirikowic J, Todd E, Rogers RK, Taylor MR
Int Angiol 2017 Oct;36(5):417-427. Epub 2017 Jan 31 doi: 10.23736/S0392-9590.17.03757-9. PMID: 28139901
Loeper F, Oosterhof J, van den Dorpel M, van der Linde D, Lu Y, Robertson E, Hambly B, Jeremy R
J Am Heart Assoc 2016 Nov 16;5(11) doi: 10.1161/JAHA.116.003705. PMID: 27852591Free PMC Article
Inoue Y, Minatoya K, Oda T, Itonaga T, Seike Y, Tanaka H, Sasaki H, Kobayashi J
Ann Thorac Surg 2016 Mar;101(3):1185-8. doi: 10.1016/j.athoracsur.2015.05.071. PMID: 26897206
Haruki T, Ito H, Sakata K, Kobayashi Y
Asian Cardiovasc Thorac Ann 2015 Nov;23(9):1072-4. Epub 2014 Apr 14 doi: 10.1177/0218492314532278. PMID: 24732088

Clinical prediction guides

Arnaud P, Hanna N, Benarroch L, Aubart M, Bal L, Bouvagnet P, Busa T, Dulac Y, Dupuis-Girod S, Edouard T, Faivre L, Gouya L, Lacombe D, Langeois M, Leheup B, Milleron O, Naudion S, Odent S, Tchitchinadze M, Ropers J, Jondeau G, Boileau C
Genet Med 2019 Sep;21(9):2015-2024. Epub 2019 Feb 11 doi: 10.1038/s41436-019-0444-y. PMID: 30739908
Benke K, Ágg B, Pólos M, Sayour AA, Radovits T, Bartha E, Nagy P, Rákóczi B, Koller Á, Szokolai V, Hedberg J, Merkely B, Nagy ZB, Szabolcs Z
BMC Psychiatry 2017 Jul 17;17(1):253. doi: 10.1186/s12888-017-1417-9. PMID: 28716062Free PMC Article
Vrsalovic M, Zeljkovic I, Presecki AV, Pintaric H, Kruslin B
Blood Press 2015;24(4):212-6. Epub 2015 May 2 doi: 10.3109/08037051.2015.1025607. PMID: 25936403
Li Y, Hu J, Qian H, Gu J, Meng W, Zhang EY
J Renin Angiotensin Aldosterone Syst 2015 Dec;16(4):1130-4. Epub 2014 Sep 17 doi: 10.1177/1470320314549219. PMID: 25237166
Balistreri CR, Maresi E, Pisano C, Di Maggio FM, Vaccarino L, Caruso C, Lio D, Ruvolo G, Candore G
Rejuvenation Res 2014 Apr;17(2):192-6. Epub 2014 Apr 8 doi: 10.1089/rej.2013.1505. PMID: 24066724

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