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Aortic dissection

MedGen UID:
83315
Concept ID:
C0340643
Disease or Syndrome
Synonyms: Aneurysm Aorta, Dissecting; Aneurysm, Aortic Dissecting; Aneurysm, Dissecting; Aorta, Dissecting Aneurysm; Aortic Dissecting Aneurysm; Aortic Dissecting Aneurysms; Aortic Dissection; Aortic Dissections; Dissecting Aneurysm; Dissecting Aneurysm Aorta; Dissecting Aneurysm Aortas; Dissecting Aneurysm, Aortic; Dissecting Aneurysms; Dissection, Aortic
SNOMED CT: Dissection of aorta (308546005)
 
HPO: HP:0002647

Definition

Aortic dissection refers to a tear in the intimal layer of the aorta causing a separation between the intima and the medial layers of the aorta. [from HPO]

Conditions with this feature

Fibromuscular dysplasia
MedGen UID:
4700
Concept ID:
C0016052
Disease or Syndrome
Fibromuscular dysplasia (FMDA) is a nonatherosclerotic, noninflammatory arterial disease that most commonly involves the renal and carotid arteries. The prevalence of symptomatic renal artery FMDA is about 4 in 1,000 and the prevalence of cervicocranial FMDA is about half of that. Histologic classification includes 3 main subtypes, intimal, medial, and perimedial, which may be associated in a single patient. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string of beads' appearance that is related to medial FMDA, and tubular and focal types, which are not clearly related to specific histologic lesions (summary by Plouin et al., 2007)
Marfan syndrome
MedGen UID:
44287
Concept ID:
C0024796
Disease or Syndrome
FBN1-related Marfan syndrome (Marfan syndrome), a systemic disorder of connective tissue with a high degree of clinical variability, comprises a broad phenotypic continuum ranging from mild (features of Marfan syndrome in one or a few systems) to severe and rapidly progressive neonatal multiorgan disease. Cardinal manifestations involve the ocular, skeletal, and cardiovascular systems. Ocular findings include myopia (>50% of affected individuals); ectopia lentis (seen in approximately 60% of affected individuals); and an increased risk for retinal detachment, glaucoma, and early cataracts. Skeletal system manifestations include bone overgrowth and joint laxity; disproportionately long extremities for the size of the trunk (dolichostenomelia); overgrowth of the ribs that can push the sternum in (pectus excavatum) or out (pectus carinatum); and scoliosis that ranges from mild to severe and progressive. The major morbidity and early mortality in Marfan syndrome relate to the cardiovascular system and include dilatation of the aorta at the level of the sinuses of Valsalva (predisposing to aortic tear and rupture), mitral valve prolapse with or without regurgitation, tricuspid valve prolapse, and enlargement of the proximal pulmonary artery. Severe and prolonged regurgitation of the mitral and/or aortic valve can predispose to left ventricular dysfunction and occasionally heart failure. With proper management, the life expectancy of someone with Marfan syndrome approximates that of the general population.
Juvenile polyposis/hereditary hemorrhagic telangiectasia syndrome
MedGen UID:
331400
Concept ID:
C1832942
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.
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.
Aneurysm-osteoarthritis syndrome
MedGen UID:
462437
Concept ID:
C3151087
Disease or Syndrome
Loeys-Dietz syndrome (LDS) is characterized by vascular findings (cerebral, thoracic, and abdominal arterial aneurysms and/or dissections), skeletal manifestations (pectus excavatum or pectus carinatum, scoliosis, joint laxity, arachnodactyly, talipes equinovarus, cervical spine malformation and/or instability), craniofacial features (widely spaced eyes, strabismus, bifid uvula / cleft palate, and craniosynostosis that can involve any sutures), and cutaneous findings (velvety and translucent skin, easy bruising, and dystrophic scars). Individuals with LDS are predisposed to widespread and aggressive arterial aneurysms and pregnancy-related complications including uterine rupture and death. Individuals with LDS can show a strong predisposition for allergic/inflammatory disease including asthma, eczema, and reactions to food or environmental allergens. There is also an increased incidence of gastrointestinal inflammation including eosinophilic esophagitis and gastritis or inflammatory bowel disease. Wide variation in the distribution and severity of clinical features can be seen in individuals with LDS, even among affected individuals within a family who have the same pathogenic variant.
Loeys-Dietz syndrome 4
MedGen UID:
766676
Concept ID:
C3553762
Disease or Syndrome
Loeys-Dietz syndrome (LDS) is characterized by vascular findings (cerebral, thoracic, and abdominal arterial aneurysms and/or dissections), skeletal manifestations (pectus excavatum or pectus carinatum, scoliosis, joint laxity, arachnodactyly, talipes equinovarus, cervical spine malformation and/or instability), craniofacial features (widely spaced eyes, strabismus, bifid uvula / cleft palate, and craniosynostosis that can involve any sutures), and cutaneous findings (velvety and translucent skin, easy bruising, and dystrophic scars). Individuals with LDS are predisposed to widespread and aggressive arterial aneurysms and pregnancy-related complications including uterine rupture and death. Individuals with LDS can show a strong predisposition for allergic/inflammatory disease including asthma, eczema, and reactions to food or environmental allergens. There is also an increased incidence of gastrointestinal inflammation including eosinophilic esophagitis and gastritis or inflammatory bowel disease. Wide variation in the distribution and severity of clinical features can be seen in individuals with LDS, even among affected individuals within a family who have the same pathogenic variant.
Meester-Loeys syndrome
MedGen UID:
934778
Concept ID:
C4310811
Disease or Syndrome
Meester-Loeys syndrome (MRLS) is an X-linked disorder characterized by early-onset aortic aneurysm and dissection. Other recurrent findings include hypertelorism, pectus deformity, joint hypermobility, contractures, and mild skeletal dysplasia (Meester et al., 2017).
Polymicrogyria with or without vascular-type Ehlers-Danlos syndrome
MedGen UID:
1675672
Concept ID:
C5193040
Disease or Syndrome
Polymicrogyria with or without vascular-type Ehlers-Danlos syndrome is an autosomal recessive disorder with a highly variable phenotype. Although all patients have polymicrogyria and other variable structural brain anomalies on imaging, only some show developmental delay and/or seizures. Similarly, only some patients have connective tissue defects that particularly affect the vascular system and can result in early death (summary by Vandervore et al., 2017).

Professional guidelines

PubMed

Writing Committee Members, Isselbacher EM, Preventza O, Hamilton Black Iii J, Augoustides JG, Beck AW, Bolen MA, Braverman AC, Bray BE, Brown-Zimmerman MM, Chen EP, Collins TJ, DeAnda A Jr, Fanola CL, Girardi LN, Hicks CW, Hui DS, Jones WS, Kalahasti V, Kim KM, Milewicz DM, Oderich GS, Ogbechie L, Promes SB, Ross EG, Schermerhorn ML, Times SS, Tseng EE, Wang GJ, Woo YJ
J Am Coll Cardiol 2022 Dec 13;80(24):e223-e393. Epub 2022 Nov 2 doi: 10.1016/j.jacc.2022.08.004. PMID: 36334952Free PMC Article
Isselbacher EM, Preventza O, Hamilton Black J 3rd, Augoustides JG, Beck AW, Bolen MA, Braverman AC, Bray BE, Brown-Zimmerman MM, Chen EP, Collins TJ, DeAnda A Jr, Fanola CL, Girardi LN, Hicks CW, Hui DS, Schuyler Jones W, Kalahasti V, Kim KM, Milewicz DM, Oderich GS, Ogbechie L, Promes SB, Gyang Ross E, Schermerhorn ML, Singleton Times S, Tseng EE, Wang GJ, Woo YJ; Peer Review Committee Members
Circulation 2022 Dec 13;146(24):e334-e482. Epub 2022 Nov 2 doi: 10.1161/CIR.0000000000001106. PMID: 36322642Free PMC Article
Erbel R, Aboyans V, Boileau C, Bossone E, Bartolomeo RD, Eggebrecht H, Evangelista A, Falk V, Frank H, Gaemperli O, Grabenwöger M, Haverich A, Iung B, Manolis AJ, Meijboom F, Nienaber CA, Roffi M, Rousseau H, Sechtem U, Sirnes PA, Allmen RS, Vrints CJ; ESC Committee for Practice Guidelines
Eur Heart J 2014 Nov 1;35(41):2873-926. Epub 2014 Aug 29 doi: 10.1093/eurheartj/ehu281. PMID: 25173340

Recent clinical studies

Etiology

Rylski B, Schilling O, Czerny M
Eur Heart J 2023 Mar 7;44(10):813-821. doi: 10.1093/eurheartj/ehac757. PMID: 36540036
Sen I, Erben YM, Franco-Mesa C, DeMartino RR
Semin Vasc Surg 2021 Mar;34(1):10-17. Epub 2021 Feb 5 doi: 10.1053/j.semvascsurg.2021.02.003. PMID: 33757630
Sayed A, Munir M, Bahbah EI
Curr Cardiol Rev 2021;17(4):e230421186875. doi: 10.2174/1573403X16666201014142930. PMID: 33059568Free PMC Article
Tadros RO, Tang GHL, Barnes HJ, Mousavi I, Kovacic JC, Faries P, Olin JW, Marin ML, Adams DH
J Am Coll Cardiol 2019 Sep 17;74(11):1494-1504. doi: 10.1016/j.jacc.2019.07.063. PMID: 31514953
Gawinecka J, Schönrath F, von Eckardstein A
Swiss Med Wkly 2017;147:w14489. Epub 2017 Aug 25 doi: 10.4414/smw.2017.14489. PMID: 28871571

Diagnosis

Meng X, Han J, Wang L, Wu Q
J Card Surg 2021 Jul;36(7):2510-2517. Epub 2021 Apr 29 doi: 10.1111/jocs.15575. PMID: 33928681
Zhu Y, Lingala B, Baiocchi M, Tao JJ, Toro Arana V, Khoo JW, Williams KM, Traboulsi AA, Hammond HC, Lee AM, Hiesinger W, Boyd J, Oyer PE, Stinson EB, Reitz BA, Mitchell RS, Miller DC, Fischbein MP, Woo YJ
J Am Coll Cardiol 2020 Oct 6;76(14):1703-1713. doi: 10.1016/j.jacc.2020.07.061. PMID: 33004136
Marroush TS, Boshara AR, Parvataneni KC, Takla R, Mesiha NA
Am J Med Sci 2017 Nov;354(5):513-520. Epub 2016 Nov 5 doi: 10.1016/j.amjms.2016.11.005. PMID: 29173364
Elsayed RS, Cohen RG, Fleischman F, Bowdish ME
Cardiol Clin 2017 Aug;35(3):331-345. Epub 2017 May 26 doi: 10.1016/j.ccl.2017.03.004. PMID: 28683905
Silaschi M, Byrne J, Wendler O
Heart 2017 Jan 1;103(1):78-87. Epub 2016 Oct 12 doi: 10.1136/heartjnl-2015-308284. PMID: 27733536

Therapy

Juraszek A, Czerny M, Rylski B
Trends Cardiovasc Med 2022 Oct;32(7):456-461. Epub 2021 Aug 16 doi: 10.1016/j.tcm.2021.08.008. PMID: 34411744
Rogers RK, Reece TB, Bonaca MP, Hess CN
Cardiol Clin 2021 Nov;39(4):495-503. doi: 10.1016/j.ccl.2021.06.002. PMID: 34686262
Nazerian P, Mueller C, Soeiro AM, Leidel BA, Salvadeo SAT, Giachino F, Vanni S, Grimm K, Oliveira MT Jr, Pivetta E, Lupia E, Grifoni S, Morello F; ADvISED Investigators
Circulation 2018 Jan 16;137(3):250-258. Epub 2017 Oct 13 doi: 10.1161/CIRCULATIONAHA.117.029457. PMID: 29030346
Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA
JAMA 2016 Aug 16;316(7):754-63. doi: 10.1001/jama.2016.10026. PMID: 27533160
Nienaber CA, Kische S, Rousseau H, Eggebrecht H, Rehders TC, Kundt G, Glass A, Scheinert D, Czerny M, Kleinfeldt T, Zipfel B, Labrousse L, Fattori R, Ince H; INSTEAD-XL trial
Circ Cardiovasc Interv 2013 Aug;6(4):407-16. Epub 2013 Aug 6 doi: 10.1161/CIRCINTERVENTIONS.113.000463. PMID: 23922146

Prognosis

Yang SS, Zhao ZL, Li T, Liang WT, Yuan D, Qian YJ
Clin Lab 2021 Nov 1;67(11) doi: 10.7754/Clin.Lab.2021.210220. PMID: 34758236
Grønberg BH, Killingberg KT, Fløtten Ø, Brustugun OT, Hornslien K, Madebo T, Langer SW, Schytte T, Nyman J, Risum S, Tsakonas G, Engleson J, Halvorsen TO
Lancet Oncol 2021 Mar;22(3):321-331. doi: 10.1016/S1470-2045(20)30742-7. PMID: 33662285
Sievers HH, Rylski B, Czerny M, Baier ALM, Kreibich M, Siepe M, Beyersdorf F
Interact Cardiovasc Thorac Surg 2020 Mar 1;30(3):451-457. doi: 10.1093/icvts/ivz281. PMID: 31755925
Takagi H, Ando T, Umemoto T; (ALICE [All-Literature Investigation of Cardiovascular Evidence] Group)
Am J Cardiol 2017 Aug 15;120(4):700-707. Epub 2017 May 30 doi: 10.1016/j.amjcard.2017.05.040. PMID: 28648392
Prisant LM, Nalamolu VR
J Clin Hypertens (Greenwich) 2005 Jun;7(6):367-71. doi: 10.1111/j.1524-6175.2005.04116.x. PMID: 16088302Free PMC Article

Clinical prediction guides

Otto CM
Heart 2020 Jun;106(12):867-869. doi: 10.1136/heartjnl-2020-317283. PMID: 32461257
Carroll BJ, Schermerhorn ML, Manning WJ
Heart 2020 Feb;106(3):182-189. Epub 2019 Dec 10 doi: 10.1136/heartjnl-2019-314897. PMID: 31822571
Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U, Ehrlich MP, Trimarchi S, Braverman AC, Myrmel T, Harris KM, Hutchinson S, O'Gara P, Suzuki T, Nienaber CA, Eagle KA; IRAD Investigators
Circulation 2018 Apr 24;137(17):1846-1860. doi: 10.1161/CIRCULATIONAHA.117.031264. PMID: 29685932
Nazerian P, Mueller C, Soeiro AM, Leidel BA, Salvadeo SAT, Giachino F, Vanni S, Grimm K, Oliveira MT Jr, Pivetta E, Lupia E, Grifoni S, Morello F; ADvISED Investigators
Circulation 2018 Jan 16;137(3):250-258. Epub 2017 Oct 13 doi: 10.1161/CIRCULATIONAHA.117.029457. PMID: 29030346
Cooper M, Hicks C, Ratchford EV, Salameh MJ, Malas M
Vasc Med 2016 Dec;21(6):547-552. Epub 2016 Apr 28 doi: 10.1177/1358863X16643601. PMID: 27126951

Recent systematic reviews

Siddiqi TJ, Usman MS, Rashid AM, Javaid SS, Ahmed A, Clark D 3rd, Flack JM, Shimbo D, Choi E, Jones DW, Hall ME
J Am Heart Assoc 2023 Jul 18;12(14):e029355. Epub 2023 Jul 8 doi: 10.1161/JAHA.122.029355. PMID: 37421281Free PMC Article
Gouda P, Kay R, Habib M, Aziz A, Aziza E, Welsh R
Int J Cardiol 2022 Sep 1;362:158-167. Epub 2022 Jun 1 doi: 10.1016/j.ijcard.2022.05.065. PMID: 35662564
D'Ascenzi F, Valentini F, Pistoresi S, Frascaro F, Piu P, Cavigli L, Valente S, Focardi M, Cameli M, Bonifazi M, Metra M, Mondillo S
Trends Cardiovasc Med 2022 Jul;32(5):299-308. Epub 2021 Jun 22 doi: 10.1016/j.tcm.2021.06.001. PMID: 34166791
Marroush TS, Boshara AR, Parvataneni KC, Takla R, Mesiha NA
Am J Med Sci 2017 Nov;354(5):513-520. Epub 2016 Nov 5 doi: 10.1016/j.amjms.2016.11.005. PMID: 29173364
Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA
JAMA 2016 Aug 16;316(7):754-63. doi: 10.1001/jama.2016.10026. PMID: 27533160

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