Skip to content

Master bibliography — thoracic aortic aneurysm

Last curated: 2026-08-27. One line per paper: full citation, PMID, tags, and the wiki pages that cite it. Every PMID was verified against a live PubMed query during the session that added it (see CONVENTIONS.md §1). 286 unique papers across six sections; a paper cited by two domains appears once per section — the recurrence is itself signal (they are the field's load-bearing papers, e.g. PMID 11834007 yearly rates, 17709637 size paradox, 36049495 ARB/BB meta-analysis, 16601194 losartan mouse).

Companion reference lists live beside this file: guidelines/REGISTRY.md (guideline documents), statistics/STATISTICS.md (statistical sources), patient-voice/sources.md (patient-experience sources), notes/ (landmark paper deep notes).

Foundations & natural history

Classification and definitions

  • Daily PO, et al. Management of acute aortic dissections. Ann Thorac Surg. 1970;10:237-47. PMID 5458238 — tags: [classification, stanford, dissection] — cited by: aortic-dissection.md
  • DeBakey ME, et al. Surgical management of dissecting aneurysms of the aorta. J Thorac Cardiovasc Surg. 1965;49:130-49. PMID 14261867 — tags: [classification, debakey, dissection] — cited by: aortic-dissection.md
  • Crawford ES, et al. Thoracoabdominal aortic aneurysms: preoperative and intraoperative factors determining immediate and long-term results of operations in 605 patients. J Vasc Surg. 1986;3:389-404. PMID 3951025 — tags: [classification, thoracoabdominal, crawford-extent, surgery] — cited by: overview.md, anatomy-and-classification.md, epidemiology-and-natural-history.md
  • Johnston KW, et al. Suggested standards for reporting on arterial aneurysms. J Vasc Surg. 1991;13:452-8. PMID 1999868 — tags: [definitions, aneurysm, reporting-standards] — cited by: overview.md, anatomy-and-classification.md

Yale natural-history program (hinge points, yearly rates, indexed size)

  • Coady MA, et al. What is the appropriate size criterion for resection of thoracic aortic aneurysms? J Thorac Cardiovasc Surg. 1997;113:476-91. PMID 9081092 — tags: [natural-history, hinge-points, size-thresholds, yale] — cited by: overview.md, epidemiology-and-natural-history.md, aortic-dissection.md
  • Coady MA, et al. Surgical intervention criteria for thoracic aortic aneurysms: a study of growth rates and complications. Ann Thorac Surg. 1999;67:1922-6. PMID 10391339 — tags: [natural-history, growth-rates, size-thresholds, yale] — cited by: epidemiology-and-natural-history.md
  • Davies RR, et al. Yearly rupture or dissection rates for thoracic aortic aneurysms: simple prediction based on size. Ann Thorac Surg. 2002;73:17-27. PMID 11834007 — tags: [natural-history, yearly-rates, rupture, dissection, yale] — cited by: overview.md, anatomy-and-classification.md, epidemiology-and-natural-history.md, aortic-dissection.md
  • Davies RR, et al. Novel measurement of relative aortic size predicts rupture of thoracic aortic aneurysms. Ann Thorac Surg. 2006;81:169-77. PMID 16368358 — tags: [aortic-size-index, indexed-size, risk-stratification, yale] — cited by: anatomy-and-classification.md, epidemiology-and-natural-history.md
  • Elefteriades JA. Natural history of thoracic aortic aneurysms: indications for surgery, and surgical versus nonsurgical risks. Ann Thorac Surg. 2002;74:S1877-80. PMID 12440685 — tags: [natural-history, hinge-points, growth-rates, surgical-risk, yale] — cited by: overview.md, epidemiology-and-natural-history.md, aortic-dissection.md
  • Elefteriades JA, et al. Indications and imaging for aortic surgery: size and other matters. J Thorac Cardiovasc Surg. 2015;149:S10-3. PMID 25218531 — tags: [natural-history, normal-size, size-paradox, review, yale] — cited by: anatomy-and-classification.md, epidemiology-and-natural-history.md
  • Kuzmik GA, et al. Natural history of thoracic aortic aneurysms. J Vasc Surg. 2012;56:565-71. PMID 22840907 — tags: [natural-history, review, silent-disease, yale] — cited by: overview.md, epidemiology-and-natural-history.md
  • Zafar MA, et al. Height alone, rather than body surface area, suffices for risk estimation in ascending aortic aneurysm. J Thorac Cardiovasc Surg. 2018;155:1938-1950. PMID 29395211 — tags: [aortic-height-index, indexed-size, risk-stratification, yale] — cited by: overview.md, anatomy-and-classification.md, epidemiology-and-natural-history.md

Population-based epidemiology

  • Bickerstaff LK, et al. Thoracic aortic aneurysms: a population-based study. Surgery. 1982;92:1103-8. PMID 7147188 — tags: [epidemiology, olmsted, natural-history, historical] — cited by: overview.md, anatomy-and-classification.md, epidemiology-and-natural-history.md
  • Clouse WD, et al. Improved prognosis of thoracic aortic aneurysms: a population-based study. JAMA. 1998;280:1926-9. PMID 9851478 — tags: [epidemiology, olmsted, rupture-risk, sex-differences] — cited by: overview.md, epidemiology-and-natural-history.md
  • Clouse WD, et al. Acute aortic dissection: population-based incidence compared with degenerative aortic aneurysm rupture. Mayo Clin Proc. 2004;79:176-80. PMID 14959911 — tags: [epidemiology, olmsted, dissection, incidence] — cited by: epidemiology-and-natural-history.md, aortic-dissection.md
  • Olsson C, et al. Thoracic aortic aneurysm and dissection: increasing prevalence and improved outcomes reported in a nationwide population-based study of more than 14,000 cases from 1987 to 2002. Circulation. 2006;114:2611-8. PMID 17145990 — tags: [epidemiology, nationwide, sweden, outcomes] — cited by: overview.md, epidemiology-and-natural-history.md
  • Howard DP, et al. Population-based study of incidence and outcome of acute aortic dissection and premorbid risk factor control: 10-year results from the Oxford Vascular Study. Circulation. 2013;127:2031-7. PMID 23599348 — tags: [epidemiology, dissection, prehospital-death, hypertension, oxvasc] — cited by: red-flags-and-safety-concerns.md, overview.md, epidemiology-and-natural-history.md, aortic-dissection.md
  • Landenhed M, et al. Risk profiles for aortic dissection and ruptured or surgically treated aneurysms: a prospective cohort study. J Am Heart Assoc. 2015;4:e001513. PMID 25609416 — tags: [epidemiology, risk-factors, hypertension, cohort, malmo] — cited by: red-flags-and-safety-concerns.md, overview.md, epidemiology-and-natural-history.md, aortic-dissection.md
  • Gouveia e Melo R, et al. Incidence and Prevalence of Thoracic Aortic Aneurysms: A Systematic Review and Meta-analysis of Population-Based Studies. Semin Thorac Cardiovasc Surg. 2022;34:1-16. PMID 33705940 — tags: [epidemiology, meta-analysis, prevalence, autopsy] — cited by: overview.md, epidemiology-and-natural-history.md
  • Gouveia e Melo R, et al. A systematic review and meta-analysis of the incidence of acute aortic dissections in population-based studies. J Vasc Surg. 2022;75:709-720. PMID 34560218 — tags: [epidemiology, meta-analysis, dissection, incidence] — cited by: overview.md, epidemiology-and-natural-history.md, aortic-dissection.md

Normal aortic dimensions and measurement

  • Devereux RB, et al. Normal limits in relation to age, body size and gender of two-dimensional echocardiographic aortic root dimensions in persons ≥15 years of age. Am J Cardiol. 2012;110:1189-94. PMID 22770936 — tags: [normal-values, aortic-root, echocardiography, nomograms] — cited by: anatomy-and-classification.md, epidemiology-and-natural-history.md
  • Rogers IS, et al. Distribution, determinants, and normal reference values of thoracic and abdominal aortic diameters by computed tomography (from the Framingham Heart Study). Am J Cardiol. 2013;111:1510-6. PMID 23497775 — tags: [normal-values, CT, framingham, determinants] — cited by: anatomy-and-classification.md, epidemiology-and-natural-history.md
  • Wolak A, et al. Aortic size assessment by noncontrast cardiac computed tomography: normal limits by age, gender, and body surface area. JACC Cardiovasc Imaging. 2008;1:200-9. PMID 19356429 — tags: [normal-values, CT, upper-normal-limits] — cited by: anatomy-and-classification.md
  • Goldstein SA, et al. Multimodality imaging of diseases of the thoracic aorta in adults: from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2015;28:119-82. PMID 25623219 — tags: [measurement-conventions, imaging, guideline, echocardiography] — cited by: anatomy-and-classification.md
  • Elefteriades JA, et al. Discrepancies in Measurement of the Thoracic Aorta: JACC Review Topic of the Week. J Am Coll Cardiol. 2020;76:201-217. PMID 32646571 — tags: [measurement-conventions, inter-modality-discrepancy, review] — cited by: anatomy-and-classification.md
  • Oladokun D, et al. Systematic Review of the Growth Rates and Influencing Factors in Thoracic Aortic Aneurysms. Eur J Vasc Endovasc Surg. 2016;51:674-81. PMID 26947541 — tags: [growth-rates, systematic-review, measurement-heterogeneity] — cited by: anatomy-and-classification.md, epidemiology-and-natural-history.md

IRAD and dissection outcomes

  • Hagan PG, et al. The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. JAMA. 2000;283:897-903. PMID 10685714 — tags: [IRAD, dissection, presentation, mortality] — cited by: red-flags-and-safety-concerns.md, overview.md, aortic-dissection.md
  • Mehta RH, et al. Predicting death in patients with acute type a aortic dissection. Circulation. 2002;105:200-6. PMID 11790701 — tags: [IRAD, type-A, mortality-prediction, malperfusion] — cited by: overview.md, aortic-dissection.md
  • Nienaber CA, et al. Gender-related differences in acute aortic dissection. Circulation. 2004;109:3014-21. PMID 15197151 — tags: [IRAD, sex-differences, dissection, outcomes] — cited by: epidemiology-and-natural-history.md, aortic-dissection.md
  • Pape LA, et al. Aortic diameter >or=5.5 cm is not a good predictor of type A aortic dissection: observations from the International Registry of Acute Aortic Dissection (IRAD). Circulation. 2007;116:1120-7. PMID 17709637 — tags: [IRAD, size-paradox, dissection, thresholds] — cited by: red-flags-and-safety-concerns.md, overview.md, epidemiology-and-natural-history.md, aortic-dissection.md
  • Pape LA, et al. Presentation, Diagnosis, and Outcomes of Acute Aortic Dissection: 17-Year Trends From the International Registry of Acute Aortic Dissection. J Am Coll Cardiol. 2015;66:350-8. PMID 26205591 — tags: [IRAD, trends, mortality, TEVAR] — cited by: red-flags-and-safety-concerns.md, overview.md, aortic-dissection.md
  • Evangelista A, et al. Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. Circulation. 2018;137:1846-1860. PMID 29685932 — tags: [IRAD, review, 20-year, trends] — cited by: red-flags-and-safety-concerns.md, aortic-dissection.md

Aortic size paradox

  • Paruchuri V, et al. Aortic Size Distribution in the General Population: Explaining the Size Paradox in Aortic Dissection. Cardiology. 2015;131:265-72. PMID 25997607 — tags: [size-paradox, population-denominator, relative-risk, yale] — cited by: overview.md, anatomy-and-classification.md, epidemiology-and-natural-history.md, aortic-dissection.md
  • Kim JB, et al. Risk of Aortic Dissection in the Moderately Dilated Ascending Aorta. J Am Coll Cardiol. 2016;68:1209-1219. PMID 27609684 — tags: [size-paradox, moderate-dilatation, absolute-risk] — cited by: overview.md, epidemiology-and-natural-history.md, aortic-dissection.md

Acute aortic syndrome variants (IMH, PAU)

  • Coady MA, et al. Penetrating ulcer of the thoracic aorta: what is it? How do we recognize it? How do we manage it? J Vasc Surg. 1998;27:1006-15. PMID 9652462 — tags: [penetrating-ulcer, acute-aortic-syndrome, rupture, yale] — cited by: aortic-dissection.md
  • Coady MA, et al. Pathologic variants of thoracic aortic dissections. Penetrating atherosclerotic ulcers and intramural hematomas. Cardiol Clin. 1999;17:637-57. PMID 10589337 — tags: [intramural-hematoma, penetrating-ulcer, acute-aortic-syndrome, yale] — cited by: epidemiology-and-natural-history.md, aortic-dissection.md
  • Tolenaar JL, et al. The differences and similarities between intramural hematoma of the descending aorta and acute type B dissection. J Vasc Surg. 2013;58:1498-504. PMID 24060392 — tags: [intramural-hematoma, type-B, IRAD, outcomes] — cited by: aortic-dissection.md

Malperfusion

  • Di Eusanio M, et al. Clinical presentation, management, and short-term outcome of patients with type A acute dissection complicated by mesenteric malperfusion: observations from the International Registry of Acute Aortic Dissection. J Thorac Cardiovasc Surg. 2013;145:385-390.e1. PMID 22341418 — tags: [malperfusion, mesenteric, type-A, IRAD, mortality] — cited by: aortic-dissection.md

Post-dissection degeneration and false-lumen biology

  • Tsai TT, et al. Partial thrombosis of the false lumen in patients with acute type B aortic dissection. N Engl J Med. 2007;357:349-59. PMID 17652650 — tags: [false-lumen, partial-thrombosis, type-B, prognosis, IRAD] — cited by: red-flags-and-safety-concerns.md, aortic-dissection.md
  • Fattori R, et al. Survival after endovascular therapy in patients with type B aortic dissection: a report from the International Registry of Acute Aortic Dissection (IRAD). JACC Cardiovasc Interv. 2013;6:876-82. PMID 23968705 — tags: [TEVAR, type-B, aneurysmal-degeneration, IRAD] — cited by: aortic-dissection.md
  • Kamman AV, et al. Predictors of Stable Aortic Dimensions in Medically Managed Acute Aortic Syndromes. Ann Vasc Surg. 2017;42:143-149. PMID 28390915 — tags: [type-B, growth-predictors, false-lumen-thrombosis, IRAD] — cited by: aortic-dissection.md
  • Nienaber CA, et al. Endovascular repair of type B aortic dissection: long-term results of the randomized investigation of stent grafts in aortic dissection trial. Circ Cardiovasc Interv. 2013;6:407-16. PMID 23922146 — tags: [INSTEAD-XL, TEVAR, RCT, type-B, remodeling] — cited by: aortic-dissection.md

Reviews, guidelines, burden

  • Isselbacher EM. Thoracic and abdominal aortic aneurysms. Circulation. 2005;111:816-28. PMID 15710776 — tags: [review, etiology, ascending-vs-descending] — cited by: overview.md
  • Svensson LG, et al. Expert consensus document on the treatment of descending thoracic aortic disease using endovascular stent-grafts. Ann Thorac Surg. 2008;85:S1-41. PMID 18083364 — tags: [consensus, burden, TEVAR, descending] — cited by: overview.md
  • Isselbacher EM, et al. 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease. J Am Coll Cardiol. 2022;80:e223-e393. PMID 36334952 — tags: [guideline, 2022-acc-aha, thresholds, measurement-conventions] — cited by: red-flags-and-safety-concerns.md, overview.md, anatomy-and-classification.md, aortic-dissection.md

Genetics

Familial clustering and heritability of non-syndromic TAAD

  • Biddinger A, Rocklin M, Coselli J, Milewicz DM. Familial thoracic aortic dilatations and dissections: a case control study. J Vasc Surg. 1997;25:506-11. PMID 9081132 — tags: [heritability, familial-clustering, case-control] — cited by: genetics-of-taa.md
  • Albornoz G, Coady MA, Roberts M, et al. Familial thoracic aortic aneurysms and dissections — incidence, modes of inheritance, and phenotypic patterns. Ann Thorac Surg. 2006;82:1400-5. PMID 16996941 — tags: [heritability, inheritance, growth-rate, pedigree] — cited by: genetics-of-taa.md, syndromic-aortopathies.md

Gene discovery — ECM and TGF-β pathway

  • Dietz HC, Cutting GR, Pyeritz RE, et al. Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature. 1991;352:337-9. PMID 1852208 — tags: [FBN1, marfan, gene-discovery, landmark] — cited by: genetics-of-taa.md, syndromic-aortopathies.md
  • Loeys BL, Chen J, Neptune ER, et al. A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2. Nat Genet. 2005;37:275-81. PMID 15731757 — tags: [TGFBR1, TGFBR2, loeys-dietz, gene-discovery, landmark] — cited by: genetics-of-taa.md, syndromic-aortopathies.md
  • Loeys BL, Schwarze U, Holm T, et al. Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med. 2006;355:788-98. PMID 16928994 — tags: [TGFBR1, TGFBR2, loeys-dietz, natural-history, vEDS-phenocopy, landmark] — cited by: genetics-of-taa.md, syndromic-aortopathies.md, bicuspid-aortopathy.md
  • van de Laar IMBH, van der Linde D, Oei EHG, et al. Phenotypic spectrum of the SMAD3-related aneurysms-osteoarthritis syndrome. J Med Genet. 2012;49:47-57. PMID 22167769 — tags: [SMAD3, aneurysms-osteoarthritis, LDS3, phenotype] — cited by: genetics-of-taa.md, syndromic-aortopathies.md
  • Lindsay ME, Schepers D, Bolar NA, et al. Loss-of-function mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm. Nat Genet. 2012;44:922-7. PMID 22772368 — tags: [TGFB2, gene-discovery, TGF-beta-paradox, mouse-model] — cited by: genetics-of-taa.md, syndromic-aortopathies.md
  • Leutermann R, Sheikhzadeh S, Brockstädt L, et al. A 1-bp duplication in TGFB2 in three family members with a syndromic form of thoracic aortic aneurysm. Eur J Hum Genet. 2013;22:944-8. PMID 24193348 — tags: [TGFB2, haploinsufficiency, case-series] — cited by: syndromic-aortopathies.md
  • Bertoli-Avella AM, Gillis E, Morisaki H, et al. Mutations in a TGF-β ligand, TGFB3, cause syndromic aortic aneurysms and dissections. J Am Coll Cardiol. 2015;65:1324-1336. PMID 25835445 — tags: [TGFB3, gene-discovery, LDS-spectrum] — cited by: genetics-of-taa.md, syndromic-aortopathies.md
  • Guo DC, Regalado ES, Gong L, et al. LOX mutations predispose to thoracic aortic aneurysms and dissections. Circ Res. 2016;118:928-34. PMID 26838787 — tags: [LOX, gene-discovery, cross-linking, unexplained-75-percent] — cited by: genetics-of-taa.md
  • Barbier M, Gross MS, Aubart M, et al. MFAP5 loss-of-function mutations underscore the involvement of matrix alteration in the pathogenesis of familial thoracic aortic aneurysms and dissections. Am J Hum Genet. 2014;95:736-43. PMID 25434006 — tags: [MFAP5, MAGP-2, gene-discovery, haploinsufficiency] — cited by: genetics-of-taa.md
  • Pepin M, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med. 2000;342:673-80. PMID 10706896 — tags: [COL3A1, vascular-EDS, natural-history, landmark] — cited by: genetics-of-taa.md, syndromic-aortopathies.md

Gene discovery — smooth muscle contractile unit

  • Zhu L, Vranckx R, Khau Van Kien P, et al. Mutations in myosin heavy chain 11 cause a syndrome associating thoracic aortic aneurysm/aortic dissection and patent ductus arteriosus. Nat Genet. 2006;38:343-9. PMID 16444274 — tags: [MYH11, gene-discovery, PDA, aortic-stiffness, landmark] — cited by: genetics-of-taa.md
  • Guo DC, Pannu H, Tran-Fadulu V, et al. Mutations in smooth muscle alpha-actin (ACTA2) lead to thoracic aortic aneurysms and dissections. Nat Genet. 2007;39:1488-93. PMID 17994018 — tags: [ACTA2, gene-discovery, SMC-contraction, landmark] — cited by: genetics-of-taa.md
  • Wang L, Guo DC, Cao J, et al. Mutations in myosin light chain kinase cause familial aortic dissections. Am J Hum Genet. 2010;87:701-7. PMID 21055718 — tags: [MYLK, gene-discovery, dissection-without-dilatation] — cited by: genetics-of-taa.md
  • Guo DC, Regalado E, Casteel DE, et al. Recurrent gain-of-function mutation in PRKG1 causes thoracic aortic aneurysms and acute aortic dissections. Am J Hum Genet. 2013;93:398-404. PMID 23910461 — tags: [PRKG1, gene-discovery, gain-of-function, cGMP] — cited by: genetics-of-taa.md
  • Kuang SQ, Medina-Martinez O, Guo DC, et al. FOXE3 mutations predispose to thoracic aortic aneurysms and dissections. J Clin Invest. 2016;126:948-61. PMID 26854927 — tags: [FOXE3, gene-discovery, SMC-apoptosis, p53] — cited by: genetics-of-taa.md
  • Guo DC, Gong L, Regalado ES, et al. MAT2A mutations predispose individuals to thoracic aortic aneurysms. Am J Hum Genet. 2015;96:170-7. PMID 25557781 — tags: [MAT2A, gene-discovery, bicuspid-aortic-valve, zebrafish] — cited by: genetics-of-taa.md, bicuspid-aortopathy.md
  • Lange M, Kasper B, Bohring A, et al. 47 patients with FLNA associated periventricular nodular heterotopia. Orphanet J Rare Dis. 2015;10:134. PMID 26471271 — tags: [FLNA, X-linked, PVNH, diagnostic-delay] — cited by: genetics-of-taa.md

Gene-validity curation, nosology and guidelines

  • Renard M, Francis C, Ghosh R, et al. Clinical validity of genes for heritable thoracic aortic aneurysm and dissection. J Am Coll Cardiol. 2018;72:605-615. PMID 30071989 — tags: [ClinGen, gene-curation, panel-design, landmark] — cited by: genetics-of-taa.md
  • Loeys BL, Dietz HC, Braverman AC, et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet. 2010;47:476-85. PMID 20591885 — tags: [marfan, ghent-criteria, diagnosis, nosology] — cited by: syndromic-aortopathies.md
  • Malfait F, Francomano C, Byers P, et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017;175:8-26. PMID 28306229 — tags: [EDS, nosology, classification, COL3A1] — cited by: syndromic-aortopathies.md
  • Michelena HI, Della Corte A, Evangelista A, et al. Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes. Ann Thorac Surg. 2021;112:1005-1022. PMID 34304861 — tags: [bicuspid-aortic-valve, nomenclature, consensus, fusion-phenotype] — cited by: bicuspid-aortopathy.md
  • Monda E, Lioncino M, Verrillo F, et al. The role of genetic testing in patients with heritable thoracic aortic diseases. Diagnostics (Basel). 2023;13:772. PMID 36832261 — tags: [genetic-testing, HTAD, review, risk-stratification] — cited by: genetics-of-taa.md
  • Milewicz DM, Braverman AC, De Backer J, et al. Marfan syndrome. Nat Rev Dis Primers. 2021;7:64. PMID 34475413 — tags: [marfan, FBN1, review, de-novo-variants] — cited by: genetics-of-taa.md, syndromic-aortopathies.md

Genotype-specific natural history and thresholds

  • Jondeau G, Ropers J, Regalado E, et al. International registry of patients carrying TGFBR1 or TGFBR2 mutations: results of the MAC (Montalcino Aortic Consortium). Circ Cardiovasc Genet. 2016;9:548-558. PMID 27879313 — tags: [TGFBR1, TGFBR2, registry, gene-specific-thresholds, sex-differences, landmark] — cited by: genetics-of-taa.md, syndromic-aortopathies.md, bicuspid-aortopathy.md
  • Regalado ES, Guo DC, Prakash SK, et al. Aortic disease presentation and outcome associated with ACTA2 mutations. Circ Cardiovasc Genet. 2015;8:457-64. PMID 25759435 — tags: [ACTA2, natural-history, codon-level-risk, penetrance] — cited by: genetics-of-taa.md
  • Silverman DI, Burton KJ, Gray J, et al. Life expectancy in the Marfan syndrome. Am J Cardiol. 1995;75:157-60. PMID 7810492 — tags: [marfan, survival, life-expectancy, era-effect] — cited by: syndromic-aortopathies.md, bicuspid-aortopathy.md
  • Poninska JK, Bilinska ZT, Franaszczyk M, et al. Next-generation sequencing for diagnosis of thoracic aortic aneurysms and dissections: diagnostic yield, novel mutations and genotype phenotype correlations. J Transl Med. 2016;14:115. PMID 27146836 — tags: [diagnostic-yield, NGS, panel-testing, event-free-survival] — cited by: genetics-of-taa.md

Turner syndrome and arterial tortuosity syndrome

  • Carlson M, Silberbach M. Dissection of the aorta in Turner syndrome: two cases and review of 85 cases in the literature. J Med Genet. 2007;44:745-9. PMID 17873120 — tags: [turner-syndrome, dissection, age-at-event, registry] — cited by: syndromic-aortopathies.md
  • Bolar K, Hoffman AR, Maneatis T, Lippe B. Long-term safety of recombinant human growth hormone in Turner syndrome. J Clin Endocrinol Metab. 2008;93:344-51. PMID 18000090 — tags: [turner-syndrome, growth-hormone, dissection-mortality, safety-cohort] — cited by: syndromic-aortopathies.md
  • Nijs J, Gelsomino S, Lucà F, et al. Unreliability of aortic size index to predict risk of aortic dissection in a patient with Turner syndrome. World J Cardiol. 2014;6:349-52. PMID 24944765 — tags: [turner-syndrome, aortic-size-index, case-report, threshold-failure] — cited by: syndromic-aortopathies.md
  • Coucke PJ, Willaert A, Wessels MW, et al. Mutations in the facilitative glucose transporter GLUT10 alter angiogenesis and cause arterial tortuosity syndrome. Nat Genet. 2006;38:452-7. PMID 16550171 — tags: [SLC2A10, GLUT10, arterial-tortuosity, autosomal-recessive, gene-discovery] — cited by: genetics-of-taa.md, syndromic-aortopathies.md

Bicuspid aortic valve — epidemiology, genetics and hemodynamics

  • Tutar E, Ekici F, Atalay S, Nacar N. The prevalence of bicuspid aortic valve in newborns by echocardiographic screening. Am Heart J. 2005;150:513-5. PMID 16169333 — tags: [bicuspid-aortic-valve, prevalence, newborn-screening, sex-ratio] — cited by: bicuspid-aortopathy.md
  • Ward C. Clinical significance of the bicuspid aortic valve. Heart. 2000;83:81-5. PMID 10618341 — tags: [bicuspid-aortic-valve, review] — cited by: bicuspid-aortopathy.md
  • Cripe L, Andelfinger G, Martin LJ, et al. Bicuspid aortic valve is heritable. J Am Coll Cardiol. 2004;44:138-43. PMID 15234422 — tags: [bicuspid-aortic-valve, heritability, family-study, landmark] — cited by: bicuspid-aortopathy.md
  • Garg V, Muth AN, Ransom JF, et al. Mutations in NOTCH1 cause aortic valve disease. Nature. 2005;437:270-4. PMID 16025100 — tags: [NOTCH1, gene-discovery, bicuspid-aortic-valve, calcification, RUNX2, landmark] — cited by: genetics-of-taa.md, bicuspid-aortopathy.md
  • McKellar SH, Tester DJ, Yagubyan M, et al. Novel NOTCH1 mutations in patients with bicuspid aortic valve disease and thoracic aortic aneurysms. J Thorac Cardiovasc Surg. 2007;134:290-6. PMID 17662764 — tags: [NOTCH1, bicuspid-aortic-valve, aneurysm, mutation-frequency] — cited by: bicuspid-aortopathy.md
  • Michelena HI, Khanna AD, Mahoney D, et al. Incidence of aortic complications in patients with bicuspid aortic valves. JAMA. 2011;306:1104-12. PMID 21917581 — tags: [bicuspid-aortic-valve, natural-history, dissection-incidence, community-cohort, landmark] — cited by: bicuspid-aortopathy.md
  • McKellar SH, MacDonald RJ, Michelena HI, et al. Frequency of cardiovascular events in women with a congenitally bicuspid aortic valve in a single community and effect of pregnancy on events. Am J Cardiol. 2011;107:96-9. PMID 21146694 — tags: [bicuspid-aortic-valve, pregnancy, women, progression] — cited by: bicuspid-aortopathy.md
  • Mahadevia R, Barker AJ, Schnell S, et al. Bicuspid aortic cusp fusion morphology alters aortic three-dimensional outflow patterns, wall shear stress, and expression of aortopathy. Circulation. 2014;129:673-82. PMID 24345403 — tags: [bicuspid-aortic-valve, 4D-flow, wall-shear-stress, fusion-phenotype] — cited by: bicuspid-aortopathy.md
  • Guzzardi DG, Barker AJ, van Ooij P, et al. Valve-related hemodynamics mediate human bicuspid aortopathy: insights from wall shear stress mapping. J Am Coll Cardiol. 2015;66:892-900. PMID 26293758 — tags: [bicuspid-aortic-valve, wall-shear-stress, elastin, MMP, paired-samples, landmark] — cited by: bicuspid-aortopathy.md

Mechanism

Histopathology and nomenclature of medial degeneration

  • Halushka MK, Angelini A, Bartoloni G, et al. 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. Cardiovasc Pathol. 2016;25:247-257. PMID 27031798 — tags: [histopathology, nomenclature, consensus, MEMA] — cited by: pathophysiology.md
  • Stone JR, Bruneval P, Angelini A, et al. Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases. Cardiovasc Pathol. 2015;24:267-78. PMID 26051917 — tags: [histopathology, aortitis, consensus, inflammation] — cited by: pathophysiology.md
  • Waters KM, Rooper LM, Guajardo A, Halushka MK. Histopathologic differences partially distinguish syndromic aortic diseases. Cardiovasc Pathol. 2017;30:6-11. PMID 28646716 — tags: [histopathology, Marfan, Loeys-Dietz, bicuspid, ageing] — cited by: pathophysiology.md, animal-models.md
  • Lesauskaite V, Tanganelli P, Sassi C, et al. Smooth muscle cells of the media in the dilatative pathology of ascending thoracic aorta: morphology, immunoreactivity for osteopontin, matrix metalloproteinases, and their inhibitors. Hum Pathol. 2001;32:1003-11. PMID 11567232 — tags: [MMP, TIMP, osteopontin, SMC-phenotype, apoptosis] — cited by: pathophysiology.md

Extracellular matrix, fibrillin-1 and the elastin-contractile unit

  • Karimi A, Milewicz DM. Structure of the Elastin-Contractile Units in the Thoracic Aorta and How Genes That Cause Thoracic Aortic Aneurysms and Dissections Disrupt This Structure. Can J Cardiol. 2015;32:26-34. PMID 26724508 — tags: [elastin-contractile-unit, ECM, genetics, review] — cited by: pathophysiology.md
  • Pereira L, Andrikopoulos K, Tian J, et al. Targetting of the gene encoding fibrillin-1 recapitulates the vascular aspect of Marfan syndrome. Nat Genet. 1997;17:218-22. PMID 9326947 — tags: [fibrillin-1, mouse-model, homeostasis, Marfan] — cited by: pathophysiology.md, animal-models.md
  • Pereira L, Lee SY, Gayraud B, et al. Pathogenetic sequence for aneurysm revealed in mice underexpressing fibrillin-1. Proc Natl Acad Sci U S A. 1999;96:3819-23. PMID 10097121 — tags: [fibrillin-1, mgR, mouse-model, elastolysis, threshold] — cited by: pathophysiology.md, animal-models.md
  • Ramirez F, Gayraud B, Pereira L. Marfan syndrome: new clues to genotype-phenotype correlations. Ann Med. 1999;31:202-7. PMID 10442675 — tags: [fibrillin-1, genotype-phenotype, review] — cited by: pathophysiology.md, animal-models.md
  • Judge DP, Biery NJ, Keene DR, et al. Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome. J Clin Invest. 2004;114:172-81. PMID 15254584 — tags: [fibrillin-1, haploinsufficiency, C1039G, mouse-model] — cited by: pathophysiology.md, animal-models.md
  • Chen M, Cavinato C, Hansen J, et al. FN (Fibronectin)-Integrin α5 Signaling Promotes Thoracic Aortic Aneurysm in a Mouse Model of Marfan Syndrome. Arterioscler Thromb Vasc Biol. 2023;43:e132-e150. PMID 36994727 — tags: [fibronectin, integrin, NF-kB, mgR, mechanosignalling] — cited by: pathophysiology.md, animal-models.md
  • Rodrigues Bento J, Meester J, Luyckx I, et al. The Genetics and Typical Traits of Thoracic Aortic Aneurysm and Dissection. Annu Rev Genomics Hum Genet. 2022;23:223-253. PMID 36044906 — tags: [genetics, review, elastin-contractile-unit, omics] — cited by: pathophysiology.md
  • Yamashiro Y, Yanagisawa H. Crossing Bridges between Extra- and Intra-Cellular Events in Thoracic Aortic Aneurysms. J Atheroscler Thromb. 2017;25:99-110. PMID 28943527 — tags: [review, mechanosensing, TGF-beta, ECM, convergence] — cited by: pathophysiology.md
  • Wang X, Tan Q, Xu J, et al. Genetic and molecular mechanisms of hereditary thoracic aortic aneurysm and dissection (Review). Mol Med Rep. 2026;34:232. PMID 42318957 — tags: [review, hereditary-TAAD, TGF-beta, SMC] — cited by: (background reading; not cited inline)

MMP / TIMP and matrix proteolysis

  • Ikonomidis JS, Jones JA, Barbour JR, et al. Expression of matrix metalloproteinases and endogenous inhibitors within ascending aortic aneurysms of patients with Marfan syndrome. Circulation. 2006;114(1 Suppl):I365-70. PMID 16820601 — tags: [MMP, TIMP, Marfan, TGFBR2, human-tissue] — cited by: pathophysiology.md, animal-models.md
  • Huusko T, Salonurmi T, Taskinen P, et al. Elevated messenger RNA expression and plasma protein levels of osteopontin and matrix metalloproteinase types 2 and 9 in patients with ascending aortic aneurysms. J Thorac Cardiovasc Surg. 2012;145:1117-1123. PMID 22571802 — tags: [MMP-2, MMP-9, osteopontin, plasma-biomarker] — cited by: pathophysiology.md
  • Mäki JM, Räsänen J, Tikkanen H, et al. Inactivation of the lysyl oxidase gene Lox leads to aortic aneurysms, cardiovascular dysfunction, and perinatal death in mice. Circulation. 2002;106:2503-9. PMID 12417550 — tags: [lysyl-oxidase, cross-linking, mouse-model, elastic-fibre] — cited by: pathophysiology.md, animal-models.md
  • LeMaire SA, Zhang L, Zhang NS, et al. Ciprofloxacin accelerates aortic enlargement and promotes dissection and rupture in Marfan mice. J Thorac Cardiovasc Surg. 2020;163:e215-e226. PMID 34586071 — tags: [fluoroquinolone, LOX, Marfan-mouse, drug-safety] — cited by: pathophysiology.md, animal-models.md

Smooth muscle cell phenotype, death and contractile function

  • Pedroza AJ, Tashima Y, Shad R, et al. Single-Cell Transcriptomic Profiling of Vascular Smooth Muscle Cell Phenotype Modulation in Marfan Syndrome Aortic Aneurysm. Arterioscler Thromb Vasc Biol. 2020;40:2195-2211. PMID 32698686 — tags: [scRNA-seq, SMC-modulation, KLF4, PAI-1, Marfan] — cited by: pathophysiology.md
  • Dalal AR, Pedroza AJ, Kim JL, et al. Chemokine (C-C Motif) Ligand 2 Expressing Adventitial Fibroblast Expansion During Loeys-Dietz Syndrome Aortic Aneurysm Formation. Arterioscler Thromb Vasc Biol. 2025;45:722-742. PMID 40109260 — tags: [scRNA-seq, Loeys-Dietz, adventitial-fibroblast, macrophage, CCL2] — cited by: pathophysiology.md, animal-models.md
  • Chakraborty A, Li Y, Zhang C, et al. Programmed cell death in aortic aneurysm and dissection: A potential therapeutic target. J Mol Cell Cardiol. 2021;163:67-80. PMID 34597613 — tags: [apoptosis, necroptosis, pyroptosis, ferroptosis, SMC-loss, review] — cited by: pathophysiology.md
  • Guo DC, Pannu H, Tran-Fadulu V, et al. Mutations in smooth muscle alpha-actin (ACTA2) lead to thoracic aortic aneurysms and dissections. Nat Genet. 2007;39:1488-93. PMID 17994018 — tags: [ACTA2, contractile-unit, human-genetics, vasa-vasorum] — cited by: pathophysiology.md, animal-models.md
  • Schildmeyer LA, Braun R, Taffet G, et al. Impaired vascular contractility and blood pressure homeostasis in the smooth muscle alpha-actin null mouse. FASEB J. 2000;14:2213-20. PMID 11053242 — tags: [Acta2-null, mouse-model, contractility, compensation] — cited by: animal-models.md
  • Bellini C, Wang S, Milewicz DM, Humphrey JD. Myh11(R247C/R247C) mutations increase thoracic aorta vulnerability to intramural damage despite a general biomechanical adaptivity. J Biomech. 2015;48:113-21. PMID 25433566 — tags: [MYH11, mouse-model, hypertension, delamination, mucoid-pooling] — cited by: animal-models.md, hemodynamics-and-biomechanics.md
  • Atash A, Mees BME, Cramer MJ, et al. MYH11 variants in thoracic aortic aneurysm pathophysiology: From bench to bedside. Eur J Clin Invest. 2026;56:e70196. PMID 41891259 — tags: [MYH11, review, penetrance, gene-therapy] — cited by: animal-models.md

TGF-β pathway and the TGF-β paradox

  • Neptune ER, Frischmeyer PA, Arking DE, et al. Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome. Nat Genet. 2003;33:407-11. PMID 12598898 — tags: [TGF-beta, fibrillin-1, landmark, mouse-model, lung] — cited by: pathophysiology.md, animal-models.md
  • Habashi JP, Judge DP, Holm TM, et al. Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome. Science. 2006;312:117-21. PMID 16601194 — tags: [losartan, TGF-beta, landmark, Marfan-mouse, ARB] — cited by: pathophysiology.md, animal-models.md
  • Loeys BL, Schwarze U, Holm T, et al. Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med. 2006;355:788-98. PMID 16928994 — tags: [Loeys-Dietz, TGFBR1, TGFBR2, natural-history] — cited by: pathophysiology.md, animal-models.md
  • Li W, Li Q, Jiao Y, et al. Tgfbr2 disruption in postnatal smooth muscle impairs aortic wall homeostasis. J Clin Invest. 2014;124:755-67. PMID 24401272 — tags: [TGF-beta-paradox, Tgfbr2, conditional-KO, MAPK, rapamycin] — cited by: pathophysiology.md, animal-models.md
  • Wei H, Hu JH, Angelov SN, et al. Aortopathy in a Mouse Model of Marfan Syndrome Is Not Mediated by Altered Transforming Growth Factor β Signaling. J Am Heart Assoc. 2017;6:e004968. PMID 28119285 — tags: [TGF-beta-paradox, Marfan-mouse, protective-TGF-beta] — cited by: pathophysiology.md, animal-models.md
  • Angelov SN, Hu JH, Wei H, et al. TGF-β (Transforming Growth Factor-β) Signaling Protects the Thoracic and Abdominal Aorta From Angiotensin II-Induced Pathology by Distinct Mechanisms. Arterioscler Thromb Vasc Biol. 2017;37:2102-2113. PMID 28729364 — tags: [TGF-beta-paradox, angiotensin-II, TAA-vs-AAA, macrophage] — cited by: pathophysiology.md, animal-models.md
  • Humphrey JD. Possible mechanical roles of glycosaminoglycans in thoracic aortic dissection and associations with dysregulated transforming growth factor-β. J Vasc Res. 2012;50:1-10. PMID 23018968 — tags: [glycosaminoglycan, MEMA, swelling-pressure, delamination, TGF-beta] — cited by: pathophysiology.md, hemodynamics-and-biomechanics.md
  • Humphrey JD, Milewicz DM. The aorta in Marfan syndrome: from molecular mechanisms to mechanobiological dysfunction. Cardiovasc Res. 2026;122:1427-1442. PMID 42140666 — tags: [review, Marfan, mechanobiology, computational-model, compensation] — cited by: pathophysiology.md, animal-models.md, hemodynamics-and-biomechanics.md
  • Asano K, Cantalupo A, Sedes L, Ramirez F. Pathophysiology and Therapeutics of Thoracic Aortic Aneurysm in Marfan Syndrome. Biomolecules. 2022;12:128. PMID 35053276 — tags: [review, Marfan, endothelial-dysfunction, angiotensin-II] — cited by: (background reading; not cited inline)

Inflammation and immune contribution

  • He R, Guo DC, Sun W, et al. Characterization of the inflammatory cells in ascending thoracic aortic aneurysms in patients with Marfan syndrome, familial thoracic aortic aneurysms, and sporadic aneurysms. J Thorac Cardiovasc Surg. 2008;136:922-9. PMID 18954631 — tags: [inflammation, T-cell, macrophage, human-tissue, TCR-clonality] — cited by: pathophysiology.md
  • Humphrey JD, Schwartz MA. Vascular Mechanobiology: Homeostasis, Adaptation, and Disease. Annu Rev Biomed Eng. 2021;23:1-27. PMID 34255994 — tags: [mechanobiology, feedback-loops, inflammation, review] — cited by: pathophysiology.md, hemodynamics-and-biomechanics.md
  • Suslov AV, Afanasyev MA, Degtyarev PA, et al. Molecular Pathogenesis and the Possible Role of Mitochondrial Heteroplasmy in Thoracic Aortic Aneurysm. Life (Basel). 2021;11:1395. PMID 34947926 — tags: [review, mitochondria, heteroplasmy, morphology] — cited by: pathophysiology.md

Embryology, lineage and regional heterogeneity

  • Sawada H, Rateri DL, Moorleghen JJ, Majesky MW, Daugherty A. Smooth Muscle Cells Derived From Second Heart Field and Cardiac Neural Crest Reside in Spatially Distinct Domains in the Media of the Ascending Aorta-Brief Report. Arterioscler Thromb Vasc Biol. 2017;37:1722-1726. PMID 28663257 — tags: [embryology, second-heart-field, neural-crest, lineage-tracing] — cited by: pathophysiology.md
  • Sawada H, Katsumata Y, Higashi H, et al. Second Heart Field-Derived Cells Contribute to Angiotensin II-Mediated Ascending Aortopathies. Circulation. 2022;145:987-1001. PMID 35143327 — tags: [second-heart-field, LRP1, PAI-1, angiotensin-II, transmural-gradient] — cited by: pathophysiology.md, animal-models.md
  • Ren P, Jiang B, Hassab AHM, et al. Heterogeneous Cardiac-Derived and Neural Crest-Derived Aortic Smooth Muscle Cells Exhibit Similar Transcriptional Changes After TGFβ Signaling Disruption. Arterioscler Thromb Vasc Biol. 2024;45:260-276. PMID 39697172 — tags: [scRNA-seq, lineage, neural-crest, TGF-beta, species-difference] — cited by: pathophysiology.md, animal-models.md

Mechanobiology and mechanosensing

  • Humphrey JD, Milewicz DM, Tellides G, Schwartz MA. Cell biology. Dysfunctional mechanosensing in aneurysms. Science. 2014;344:477-9. PMID 24786066 — tags: [mechanosensing, perspective, aneurysm] — cited by: pathophysiology.md
  • Humphrey JD, Schwartz MA, Tellides G, Milewicz DM. Role of mechanotransduction in vascular biology: focus on thoracic aortic aneurysms and dissections. Circ Res. 2015;116:1448-61. PMID 25858068 — tags: [mechanotransduction, review, focal-adhesion, actomyosin] — cited by: pathophysiology.md, hemodynamics-and-biomechanics.md

Ex vivo biomechanics of aneurysmal tissue

  • Vorp DA, Schiro BJ, Ehrlich MP, et al. Effect of aneurysm on the tensile strength and biomechanical behavior of the ascending thoracic aorta. Ann Thorac Surg. 2003;75:1210-4. PMID 12683565 — tags: [tensile-strength, stiffness, ex-vivo, ascending] — cited by: hemodynamics-and-biomechanics.md
  • Chung J, Lachapelle K, Wener E, et al. Energy loss, a novel biomechanical parameter, correlates with aortic aneurysm size and histopathologic findings. J Thorac Cardiovasc Surg. 2014;148:1082-8. PMID 25129601 — tags: [energy-loss, biaxial-testing, hinge-point, collagen-elastin] — cited by: hemodynamics-and-biomechanics.md
  • Chung J, Lachapelle K, Cartier R, Mongrain R, Leask RL. Loss of mechanical directional dependency of the ascending aorta with severe medial degeneration. Cardiovasc Pathol. 2016;26:45-50. PMID 27888778 — tags: [anisotropy, energy-loss, medial-degeneration, Movat] — cited by: hemodynamics-and-biomechanics.md
  • Salmasi MY, Sasidharan S, Frattolin J, et al. Regional variation in biomechanical properties of ascending thoracic aortic aneurysms. Eur J Cardiothorac Surg. 2022;62:ezac392. PMID 35894942 — tags: [delamination, peel-test, regional-heterogeneity, wall-thickness] — cited by: pathophysiology.md, hemodynamics-and-biomechanics.md
  • Pal S, Tsamis A, Pasta S, et al. A mechanistic model on the role of "radially-running" collagen fibers on dissection properties of human ascending thoracic aorta. J Biomech. 2014;47:981-8. PMID 24484644 — tags: [delamination, collagen-architecture, peel-force, modelling] — cited by: hemodynamics-and-biomechanics.md

4D-flow MRI and wall shear stress

  • Barker AJ, Markl M, Bürk J, et al. Bicuspid aortic valve is associated with altered wall shear stress in the ascending aorta. Circ Cardiovasc Imaging. 2012;5:457-66. PMID 22730420 — tags: [4D-flow, WSS, bicuspid, jet-eccentricity] — cited by: hemodynamics-and-biomechanics.md
  • Guzzardi DG, Barker AJ, van Ooij P, et al. Valve-Related Hemodynamics Mediate Human Bicuspid Aortopathy: Insights From Wall Shear Stress Mapping. J Am Coll Cardiol. 2015;66:892-900. PMID 26293758 — tags: [4D-flow, WSS, elastin, MMP, paired-samples, landmark] — cited by: pathophysiology.md, hemodynamics-and-biomechanics.md, animal-models.md
  • Bollache E, Guzzardi DG, Sattari S, et al. Aortic valve-mediated wall shear stress is heterogeneous and predicts regional aortic elastic fiber thinning in bicuspid aortic valve-associated aortopathy. J Thorac Cardiovasc Surg. 2018;156:2112-2120. PMID 30060930 — tags: [4D-flow, WSS, elastic-fibre-thickness, stiffness, bicuspid] — cited by: pathophysiology.md, hemodynamics-and-biomechanics.md
  • van Ooij P, Garcia J, Potters WV, et al. Age-related changes in aortic 3D blood flow velocities and wall shear stress: Implications for the identification of altered hemodynamics in patients with aortic valve disease. J Magn Reson Imaging. 2016;43:1239-49. PMID 26477691 — tags: [4D-flow, WSS, age-matching, methodology] — cited by: hemodynamics-and-biomechanics.md
  • Suwa K, Rahman OA, Bollache E, et al. Effect of Aortic Valve Disease on 3D Hemodynamics in Patients With Aortic Dilation and Trileaflet Aortic Valve Morphology. J Magn Reson Imaging. 2019;51:481-491. PMID 31169969 — tags: [4D-flow, WSS, tricuspid, stenosis, regurgitation] — cited by: hemodynamics-and-biomechanics.md
  • Bouaou K, Dietenbeck T, Soulat G, et al. Four-dimensional flow cardiovascular magnetic resonance aortic cross-sectional pressure changes and their associations with flow patterns in health and ascending thoracic aortic aneurysm. J Cardiovasc Magn Reson. 2024;26:101030. PMID 38403074 — tags: [4D-flow, pressure-gradient, vorticity, WSS, ATAA] — cited by: hemodynamics-and-biomechanics.md

Computational modelling: FEA, CFD, FSI

  • Xuan Y, Wang Z, Liu R, et al. Wall stress on ascending thoracic aortic aneurysms with bicuspid compared with tricuspid aortic valve. J Thorac Cardiovasc Surg. 2018;156:492-500. PMID 29656820 — tags: [FEA, wall-stress, bicuspid, diameter-uncoupling] — cited by: hemodynamics-and-biomechanics.md
  • Gomez A, Wang Z, Xuan Y, et al. Regional wall stress differences on tricuspid aortic valve-associated ascending aortic aneurysms. Interact Cardiovasc Thorac Surg. 2022;34:1115-1123. PMID 34718581 — tags: [FEA, wall-stress, regional, sinotubular-junction, n=204] — cited by: hemodynamics-and-biomechanics.md
  • Trabelsi O, Davis FM, Rodriguez-Matas JF, Duprey A, Avril S. Patient specific stress and rupture analysis of ascending thoracic aneurysms. J Biomech. 2015;48:1836-43. PMID 25979384 — tags: [FEA, bulge-inflation, rupture-risk-index, patient-specific] — cited by: hemodynamics-and-biomechanics.md
  • Condemi F, Campisi S, Viallon M, Croisille P, Avril S. Relationship Between Ascending Thoracic Aortic Aneurysms Hemodynamics and Biomechanical Properties. IEEE Trans Biomed Eng. 2019;67:949-956. PMID 31331875 — tags: [CFD, TAWSS, rupture-stretch, in-vivo-in-vitro-in-silico] — cited by: hemodynamics-and-biomechanics.md
  • Kaiser AD, Shad R, Schiavone N, Hiesinger W, Marsden AL. Controlled Comparison of Simulated Hemodynamics Across Tricuspid and Bicuspid Aortic Valves. Ann Biomed Eng. 2022;50:1053-1072. PMID 35748961 — tags: [FSI, simulation, bicuspid, cusp-fusion, jet] — cited by: hemodynamics-and-biomechanics.md

Animal models, comparative phenotyping and reporting standards

  • Bellini C, Bersi MR, Caulk AW, et al. Comparison of 10 murine models reveals a distinct biomechanical phenotype in thoracic aortic aneurysms. J R Soc Interface. 2017;14:20161036. PMID 28490606 — tags: [comparative-biomechanics, 10-models, circumferential-stiffness, landmark] — cited by: hemodynamics-and-biomechanics.md, animal-models.md, pathophysiology.md
  • Daugherty A, Milewicz DM, Dichek DA, et al. Recommendations for Design, Execution, and Reporting of Studies on Experimental Thoracic Aortopathy in Preclinical Models. Arterioscler Thromb Vasc Biol. 2025;45:609-631. PMID 40079138 — tags: [methodology, consensus, reporting-standards, mouse] — cited by: animal-models.md
  • Wang Y, Panicker IS, Anesi J, et al. Animal Models, Pathogenesis, and Potential Treatment of Thoracic Aortic Aneurysm. Int J Mol Sci. 2024;25:901. PMID 38255976 — tags: [review, animal-models, porcine, zebrafish, BAPN] — cited by: animal-models.md
  • Nistala H, Lee-Arteaga S, Carta L, et al. Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndrome. Hum Mol Genet. 2010;19:4790-8. PMID 20871099 — tags: [mgR, losartan, TGF-beta, osteopenia] — cited by: animal-models.md
  • He C, Jiang B, Wang M, et al. mTOR inhibition prevents angiotensin II-induced aortic rupture and pseudoaneurysm but promotes dissection in Apoe-deficient mice. JCI Insight. 2022;7:e155815. PMID 35132962 — tags: [angiotensin-II, pseudoaneurysm, mTOR, model-misclassification] — cited by: animal-models.md
  • Phillips EH, Yrineo AA, Schroeder HD, et al. Morphological and Biomechanical Differences in the Elastase and AngII apoE(-/-) Rodent Models of Abdominal Aortic Aneurysms. Biomed Res Int. 2015;2015:413189. PMID 26064906 — tags: [elastase, angiotensin-II, AAA, comparative] — cited by: pathophysiology.md, animal-models.md
  • Gao YX, Liu YT, Zhang YY, et al. [Establishment of β-aminopropionitrile-induced aortic dissection model in C57Bl/6J mice]. Zhonghua Xin Xue Guan Bing Za Zhi. 2018;46:137-142. PMID 29495238 — tags: [BAPN, lathyrism, dose-response, dissection, mouse] — cited by: animal-models.md
  • Lv X, Hu Y, Chen X, et al. Establishment and effect evaluation of an aortic dissection model induced by different doses of β-aminopropionitrile in rats. Vascular. 2020;29:832-840. PMID 33357159 — tags: [BAPN, angiotensin-II, rat, dissection, MRI] — cited by: animal-models.md
  • Bousbaa A, Fedoryshchenko I, Luyckx I, Loeys B, Verstraeten A. Preclinical modeling of Loeys-Dietz syndrome: insights into mechanisms and therapy. Orphanet J Rare Dis. 2026. PMID 42380922 — tags: [Loeys-Dietz, preclinical-models, TGF-beta-paradox, review] — cited by: animal-models.md

Translational gap: human trials of TGF-β/AT1 blockade

  • Lacro RV, Dietz HC, Sleeper LA, et al. Atenolol versus losartan in children and young adults with Marfan's syndrome. N Engl J Med. 2014;371:2061-71. PMID 25405392 — tags: [RCT, losartan, atenolol, Marfan, translational-gap] (NCT00429364) — cited by: pathophysiology.md, animal-models.md
  • Kang YN, Chi SC, Wu MH, Chiu HH. The effects of losartan versus beta-blockers on cardiovascular protection in marfan syndrome: A systematic review and meta-analysis. J Formos Med Assoc. 2019;119:182-190. PMID 31003918 — tags: [meta-analysis, losartan, beta-blocker, Marfan] — cited by: pathophysiology.md, animal-models.md

Clinical management

Guidelines and standards

  • Isselbacher EM, et al. 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease. Circulation. 2022;146:e334-e482. PMID 36322642. doi:10.1161/CIR.0000000000001106 — tags: [guideline, thresholds, surveillance, medical-therapy, surgery] — cited by: red-flags-and-safety-concerns.md, guidelines.md, imaging-and-surveillance.md, risk-stratification-and-size-thresholds.md, medical-therapy.md, surgical-and-endovascular-repair.md
  • Isselbacher EM, et al. 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease. J Am Coll Cardiol. 2022;80:e223-e393. PMID 36334952. doi:10.1016/j.jacc.2022.08.004 — tags: [guideline, co-publication] — cited by: guidelines.md
  • Hiratzka LF, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease. Circulation. 2010;121:e266-e369. PMID 20233780. doi:10.1161/CIR.0b013e3181d4739e — tags: [guideline, historical] — cited by: guidelines.md
  • Erbel R, et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases. Eur Heart J. 2014;35:2873-2926. PMID 25173340. doi:10.1093/eurheartj/ehu281 — tags: [guideline, ESC] — cited by: guidelines.md
  • Czerny M, et al. EACTS/STS Guidelines for diagnosing and treating acute and chronic syndromes of the aortic organ. Eur J Cardiothorac Surg. 2024;65(2):ezad426. PMID 38408364. doi:10.1093/ejcts/ezad426 — tags: [guideline, EACTS-STS, aortic-organ] — cited by: guidelines.md
  • Czerny M, et al. EACTS/STS Guidelines for diagnosing and treating acute and chronic syndromes of the aortic organ. Ann Thorac Surg. 2024;118:5-115. PMID 38416090. doi:10.1016/j.athoracsur.2024.01.021 — tags: [guideline, co-publication] — cited by: guidelines.md
  • Czerny M, et al. Clinical cases referring to the 2023 EACTS/STS Guidelines for diagnosing and treating acute and chronic syndromes of the aortic organ. Eur J Cardiothorac Surg. 2024;66(3):ezae294. PMID 39196761. doi:10.1093/ejcts/ezae294 — tags: [guideline, cases] — cited by: guidelines.md
  • Goldstein SA, et al. Multimodality imaging of diseases of the thoracic aorta in adults (ASE/EACVI). J Am Soc Echocardiogr. 2015;28:119-182. PMID 25623219. doi:10.1016/j.echo.2014.11.015 — tags: [imaging, standards, echo] — cited by: imaging-and-surveillance.md, guidelines.md

Natural history, hinge points, and size thresholds

  • Coady MA, et al. What is the appropriate size criterion for resection of thoracic aortic aneurysms? J Thorac Cardiovasc Surg. 1997;113:476-491. PMID 9081092. doi:10.1016/S0022-5223(97)70360-X — tags: [natural-history, hinge-point, thresholds, Yale] — cited by: risk-stratification-and-size-thresholds.md, imaging-and-surveillance.md, guidelines.md
  • Coady MA, et al. Surgical intervention criteria for thoracic aortic aneurysms: a study of growth rates and complications. Ann Thorac Surg. 1999;67:1922-1926. PMID 10391339. doi:10.1016/s0003-4975(99)00431-2 — tags: [natural-history, growth-rate, Yale] — cited by: risk-stratification-and-size-thresholds.md
  • Davies RR, et al. Yearly rupture or dissection rates for thoracic aortic aneurysms: simple prediction based on size. Ann Thorac Surg. 2002;73:17-27. PMID 11834007. doi:10.1016/s0003-4975(01)03236-2 — tags: [natural-history, yearly-risk, thresholds, Yale] — cited by: risk-stratification-and-size-thresholds.md, imaging-and-surveillance.md, medical-therapy.md, guidelines.md
  • Davies RR, et al. Novel measurement of relative aortic size predicts rupture of thoracic aortic aneurysms. Ann Thorac Surg. 2006;81:169-177. PMID 16368358. doi:10.1016/j.athoracsur.2005.06.026 — tags: [aortic-size-index, indexed-measures, Yale] — cited by: risk-stratification-and-size-thresholds.md, guidelines.md
  • Davies RR, et al. Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve. Ann Thorac Surg. 2007;83:1338-1344. PMID 17383337. doi:10.1016/j.athoracsur.2006.10.074 — tags: [BAV, growth-rate, natural-history] — cited by: imaging-and-surveillance.md
  • Pape LA, et al. Aortic diameter ≥5.5 cm is not a good predictor of type A aortic dissection (IRAD). Circulation. 2007;116:1120-1127. PMID 17709637. doi:10.1161/CIRCULATIONAHA.107.702720 — tags: [size-paradox, IRAD, dissection] — cited by: risk-stratification-and-size-thresholds.md, guidelines.md
  • Paruchuri V, et al. Aortic size distribution in the general population: explaining the size paradox in aortic dissection. Cardiology. 2015;131:265-272. PMID 25997607. doi:10.1159/000381281 — tags: [size-paradox, population, relative-risk] — cited by: risk-stratification-and-size-thresholds.md, imaging-and-surveillance.md, guidelines.md
  • Zafar MA, et al. Height alone, rather than body surface area, suffices for risk estimation in ascending aortic aneurysm. J Thorac Cardiovasc Surg. 2018;155:1938-1950. PMID 29395211. doi:10.1016/j.jtcvs.2017.10.140 — tags: [aortic-height-index, indexed-measures, Yale] — cited by: risk-stratification-and-size-thresholds.md, guidelines.md
  • Wu J, et al. Ascending aortic length and risk of aortic adverse events: the neglected dimension. J Am Coll Cardiol. 2019;74:1883-1894. PMID 31526537. doi:10.1016/j.jacc.2019.07.078 — tags: [aortic-length, indexed-measures, Yale] — cited by: risk-stratification-and-size-thresholds.md, imaging-and-surveillance.md
  • Svensson LG, Khitin L. Aortic cross-sectional area/height ratio timing of aortic surgery in asymptomatic patients with Marfan syndrome. J Thorac Cardiovasc Surg. 2002;123:360-361. PMID 11828302. doi:10.1067/mtc.2002.118497 — tags: [area-height-ratio, Marfan] — cited by: risk-stratification-and-size-thresholds.md, guidelines.md
  • Svensson LG, et al. Relationship of aortic cross-sectional area to height ratio and the risk of aortic dissection in patients with bicuspid aortic valves. J Thorac Cardiovasc Surg. 2003;126:892-893. PMID 14502185. doi:10.1016/s0022-5223(03)00608-1 — tags: [area-height-ratio, BAV] — cited by: risk-stratification-and-size-thresholds.md
  • Jondeau G, et al. International registry of patients carrying TGFBR1 or TGFBR2 mutations: results of the MAC (Montalcino Aortic Consortium). Circ Cardiovasc Genet. 2016;9:548-558. PMID 27879313. doi:10.1161/CIRCGENETICS.116.001485 — tags: [genotype-thresholds, Loeys-Dietz, sex-differences] — cited by: risk-stratification-and-size-thresholds.md
  • Nienaber CA, et al. Gender-related differences in acute aortic dissection. Circulation. 2004;109:3014-3021. PMID 15197151. doi:10.1161/01.CIR.0000130644.78677.2C — tags: [sex-differences, IRAD, dissection] — cited by: risk-stratification-and-size-thresholds.md, guidelines.md
  • Boczar KE, et al. Sex differences in thoracic aortic aneurysm growth. Hypertension. 2019;73:190-196. PMID 30571545. doi:10.1161/HYPERTENSIONAHA.118.11851 — tags: [sex-differences, growth-rate, stiffness] — cited by: risk-stratification-and-size-thresholds.md
  • Cote CL, et al. Sex differences in trends in incidence of thoracic aortic aneurysm repair and aortic dissection: 2005-2015. CJC Open. 2022;4:1081-1089. PMID 36562011. doi:10.1016/j.cjco.2022.08.012 — tags: [sex-differences, epidemiology] — cited by: risk-stratification-and-size-thresholds.md
  • Treatment in Thoracic Aortic Aneurysm: Surgery versus Surveillance (TITAN:SvS). ClinicalTrials.gov NCT03536312 (recruiting; n=610 target; verified 2026-08-27) — tags: [RCT, 5.0-5.4cm, thresholds] — cited by: risk-stratification-and-size-thresholds.md, guidelines.md

Imaging, measurement, and screening

  • Quint LE, et al. Proximal thoracic aortic diameter measurements at CT: repeatability and reproducibility according to measurement method. Int J Cardiovasc Imaging. 2013;29:479-488. PMID 22864960. doi:10.1007/s10554-012-0102-9 — tags: [measurement-error, CT, reproducibility] — cited by: imaging-and-surveillance.md, risk-stratification-and-size-thresholds.md
  • Mariscalco G, et al. Systematic review of studies that have evaluated screening tests in relatives of patients affected by nonsyndromic thoracic aortic disease. J Am Heart Assoc. 2018;7:e009302. PMID 30371227. doi:10.1161/JAHA.118.009302 — tags: [screening, family, nonsyndromic] — cited by: red-flags-and-safety-concerns.md, imaging-and-surveillance.md
  • Abbasciano RG, et al. Evaluating the feasibility of screening relatives of patients affected by nonsyndromic thoracic aortic diseases: the REST study. J Am Heart Assoc. 2022;11:e023741. PMID 35383466. doi:10.1161/JAHA.121.023741 — tags: [screening, family, feasibility, NCT03861741] — cited by: imaging-and-surveillance.md

Medical therapy — Marfan trials and mechanism

  • Shores J, et al. Progression of aortic dilatation and the benefit of long-term beta-adrenergic blockade in Marfan's syndrome. N Engl J Med. 1994;330:1335-1341. PMID 8152445. doi:10.1056/NEJM199405123301902 — tags: [beta-blocker, Marfan, RCT] — cited by: medical-therapy.md
  • Habashi JP, et al. Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome. Science. 2006;312:117-121. PMID 16601194. doi:10.1126/science.1124287 — tags: [losartan, TGF-beta, mouse] — cited by: medical-therapy.md
  • Brooke BS, et al. Angiotensin II blockade and aortic-root dilation in Marfan's syndrome. N Engl J Med. 2008;358:2787-2795. PMID 18579813. doi:10.1056/NEJMoa0706585 — tags: [ARB, pediatric, cohort] — cited by: medical-therapy.md
  • Groenink M, et al. Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial (COMPARE). Eur Heart J. 2013;34:3491-3500. PMID 23999449. doi:10.1093/eurheartj/eht334 — tags: [losartan, RCT, COMPARE] — cited by: medical-therapy.md
  • Milleron O, et al. Marfan Sartan: a randomized, double-blind, placebo-controlled trial. Eur Heart J. 2015;36:2160-2166. PMID 25935877. doi:10.1093/eurheartj/ehv151 — tags: [losartan, RCT, negative-trial] — cited by: medical-therapy.md
  • Lacro RV, et al. Atenolol versus losartan in children and young adults with Marfan's syndrome. N Engl J Med. 2014;371:2061-2071. PMID 25405392. doi:10.1056/NEJMoa1404731 — tags: [losartan, atenolol, RCT, pediatric, NCT00429364] — cited by: medical-therapy.md
  • Mullen M, et al. Irbesartan in Marfan syndrome (AIMS): a double-blind, placebo-controlled randomised trial. Lancet. 2019;394:2263-2270. PMID 31836196. doi:10.1016/S0140-6736(19)32518-8 — tags: [irbesartan, RCT, AIMS] — cited by: medical-therapy.md
  • Pitcher A, et al. Angiotensin receptor blockers and β blockers in Marfan syndrome: an individual patient data meta-analysis of randomised trials. Lancet. 2022;400:822-831. PMID 36049495. doi:10.1016/S0140-6736(22)01534-3 — tags: [IPD-meta-analysis, ARB, beta-blocker, Marfan-Treatment-Trialists] — cited by: medical-therapy.md, guidelines.md
  • van Andel MM, et al. Long-term clinical outcomes of losartan in patients with Marfan syndrome: follow-up of the COMPARE trial. Eur Heart J. 2020;41:4181-4187. PMID 32548624. doi:10.1093/eurheartj/ehaa377 — tags: [losartan, long-term, clinical-endpoints] — cited by: medical-therapy.md
  • Franken R, et al. Beneficial outcome of losartan therapy depends on type of FBN1 mutation in Marfan syndrome. Circ Cardiovasc Genet. 2015;8:383-388. PMID 25613431. doi:10.1161/CIRCGENETICS.114.000950 — tags: [FBN1, pharmacogenetics, losartan] — cited by: medical-therapy.md
  • Gersony DR, et al. The effect of beta-blocker therapy on clinical outcome in patients with Marfan's syndrome: a meta-analysis. Int J Cardiol. 2007;114:303-308. PMID 16831475. doi:10.1016/j.ijcard.2005.11.116 — tags: [beta-blocker, meta-analysis, skeptical] — cited by: medical-therapy.md
  • Sato T, et al. Statins reduce thoracic aortic aneurysm growth in Marfan syndrome mice via inhibition of the Ras-induced ERK signaling pathway. J Am Heart Assoc. 2018;7:e008543. PMID 30571378. doi:10.1161/JAHA.118.008543 — tags: [statins, mouse, ERK] — cited by: medical-therapy.md

Medical therapy — drug safety signals and lifestyle

  • Lee CC, et al. Risk of aortic dissection and aortic aneurysm in patients taking oral fluoroquinolone. JAMA Intern Med. 2015;175:1839-1847. PMID 26436523. doi:10.1001/jamainternmed.2015.5389 — tags: [fluoroquinolone, pharmacoepidemiology] — cited by: medical-therapy.md
  • Lee CC, et al. Oral fluoroquinolone and the risk of aortic dissection. J Am Coll Cardiol. 2018;72:1369-1378. PMID 30213330. doi:10.1016/j.jacc.2018.06.067 — tags: [fluoroquinolone, case-crossover] — cited by: medical-therapy.md
  • Pasternak B, et al. Fluoroquinolone use and risk of aortic aneurysm and dissection: nationwide cohort study. BMJ. 2018;360:k678. PMID 29519881. doi:10.1136/bmj.k678 — tags: [fluoroquinolone, cohort, Sweden] — cited by: red-flags-and-safety-concerns.md, medical-therapy.md
  • Dong YH, et al. Association of infections and use of fluoroquinolones with the risk of aortic aneurysm or aortic dissection. JAMA Intern Med. 2020;180:1587-1595. PMID 32897358. doi:10.1001/jamainternmed.2020.4192 — tags: [fluoroquinolone, confounding-by-indication, skeptical] — cited by: medical-therapy.md
  • Gopalakrishnan C, et al. Association of fluoroquinolones with the risk of aortic aneurysm or aortic dissection. JAMA Intern Med. 2020;180:1596-1605. PMID 32897307. doi:10.1001/jamainternmed.2020.4199 — tags: [fluoroquinolone, surveillance-bias, skeptical] — cited by: red-flags-and-safety-concerns.md, medical-therapy.md, guidelines.md
  • Doyle JJ, et al. A deleterious gene-by-environment interaction imposed by calcium channel blockers in Marfan syndrome. Elife. 2015;4:e08648. PMID 26506064. doi:10.7554/eLife.08648 — tags: [calcium-channel-blockers, harm-signal, mouse+human] — cited by: medical-therapy.md
  • Hatzaras I, et al. Weight lifting and aortic dissection: more evidence for a connection. Cardiology. 2007;107:103-106. PMID 16847387. doi:10.1159/000094530 — tags: [exercise, weightlifting, dissection-trigger] — cited by: red-flags-and-safety-concerns.md, medical-therapy.md

Surgery and endovascular repair

  • Bentall H, De Bono A. A technique for complete replacement of the ascending aorta. Thorax. 1968;23:338-339. PMID 5664694. doi:10.1136/thx.23.4.338 — tags: [Bentall, composite-graft, historical] — cited by: surgical-and-endovascular-repair.md
  • Yacoub MH, et al. Late results of a valve-preserving operation in patients with aneurysms of the ascending aorta and root. J Thorac Cardiovasc Surg. 1998;115:1080-1090. PMID 9605078. doi:10.1016/S0022-5223(98)70408-8 — tags: [remodeling, valve-sparing, Yacoub] — cited by: surgical-and-endovascular-repair.md
  • David TE. Current readings: aortic valve-sparing operations. Semin Thorac Cardiovasc Surg. 2014;26:231-238. PMID 25527017. doi:10.1053/j.semtcvs.2014.10.002 — tags: [valve-sparing, reimplantation-vs-remodeling, review] — cited by: surgical-and-endovascular-repair.md
  • David TE, et al. Outcomes of aortic valve-sparing operations in Marfan syndrome. J Am Coll Cardiol. 2015;66:1445-1453. PMID 26403341. doi:10.1016/j.jacc.2015.07.041 — tags: [valve-sparing, Marfan, long-term] — cited by: surgical-and-endovascular-repair.md
  • David TE, et al. A progress report on reimplantation of the aortic valve. J Thorac Cardiovasc Surg. 2021;161:890-899.e1. PMID 33008570. doi:10.1016/j.jtcvs.2020.07.121 — tags: [reimplantation, David-operation, 20-year] — cited by: surgical-and-endovascular-repair.md
  • Hughes GC, et al. Effects of institutional volumes on operative outcomes for aortic root replacement in North America. J Thorac Cardiovasc Surg. 2013;145:166-170. PMID 22306215. doi:10.1016/j.jtcvs.2011.10.094 — tags: [volume-outcome, root-replacement, STS] — cited by: surgical-and-endovascular-repair.md, guidelines.md
  • Chikwe J, et al. National outcomes in acute aortic dissection: influence of surgeon and institutional volume on operative mortality. Ann Thorac Surg. 2013;95:1563-1569. PMID 23562465. doi:10.1016/j.athoracsur.2013.02.039 — tags: [volume-outcome, type-A-dissection] — cited by: surgical-and-endovascular-repair.md
  • Pape LA, et al. Presentation, diagnosis, and outcomes of acute aortic dissection: 17-year trends from IRAD. J Am Coll Cardiol. 2015;66:350-358. PMID 26205591. doi:10.1016/j.jacc.2015.05.029 — tags: [IRAD, trends, mortality] — cited by: surgical-and-endovascular-repair.md
  • Roselli EE, et al. Evolution of simplified frozen elephant trunk repair for acute DeBakey type I dissection: midterm outcomes. Ann Thorac Surg. 2018;105:749-755. PMID 29217087. doi:10.1016/j.athoracsur.2017.08.037 — tags: [frozen-elephant-trunk, arch, dissection] — cited by: surgical-and-endovascular-repair.md
  • Nakhaei P, et al. Aortic remodeling, distal stent-graft induced new entry and endoleak following frozen elephant trunk: a systematic review and meta-analysis. J Card Surg. 2022;37:3848-3862. PMID 36069163. doi:10.1111/jocs.16918 — tags: [frozen-elephant-trunk, meta-analysis, dSINE] — cited by: surgical-and-endovascular-repair.md
  • Cheng D, et al. Endovascular aortic repair versus open surgical repair for descending thoracic aortic disease: a systematic review and meta-analysis of comparative studies. J Am Coll Cardiol. 2010;55:986-1001. PMID 20137879. doi:10.1016/j.jacc.2009.11.047 — tags: [TEVAR, open-repair, meta-analysis] — cited by: surgical-and-endovascular-repair.md
  • Coselli JS, et al. Outcomes of 3309 thoracoabdominal aortic aneurysm repairs. J Thorac Cardiovasc Surg. 2016;151:1323-1337. PMID 26898979. doi:10.1016/j.jtcvs.2015.12.050 — tags: [thoracoabdominal, open-repair, benchmark] — cited by: surgical-and-endovascular-repair.md
  • Coselli JS, et al. Cerebrospinal fluid drainage reduces paraplegia after thoracoabdominal aortic aneurysm repair: results of a randomized clinical trial. J Vasc Surg. 2002;35:631-639. PMID 11932655. doi:10.1067/mva.2002.122024 — tags: [spinal-cord-protection, CSF-drainage, RCT] — cited by: surgical-and-endovascular-repair.md, guidelines.md
  • Roselli EE, et al. ARISE: first-in-human evaluation of a novel stent graft to treat ascending aortic dissection. J Endovasc Ther. 2023;30:550-560. PMID 35587698. doi:10.1177/15266028221095018 — tags: [ascending-TEVAR, first-in-human, device] — cited by: surgical-and-endovascular-repair.md
  • Izgi C, et al. External aortic root support to prevent aortic dilatation in patients with Marfan syndrome. J Am Coll Cardiol. 2018;72:1095-1105. PMID 30165980. doi:10.1016/j.jacc.2018.06.053 — tags: [PEARS, Marfan, MRI-follow-up] — cited by: surgical-and-endovascular-repair.md
  • Treasure T, et al. Personalized external aortic root support in aneurysm disease. Curr Opin Cardiol. 2022;37:454-458. PMID 36094493. doi:10.1097/HCO.0000000000000990 — tags: [PEARS, review, registry] — cited by: surgical-and-endovascular-repair.md, guidelines.md

Research frontier

D-dimer and acute aortic syndrome diagnosis

  • Nazerian P, Mueller C, Soeiro AM, et al. Diagnostic Accuracy of the Aortic Dissection Detection Risk Score Plus D-Dimer for Acute Aortic Syndromes: The ADvISED Prospective Multicenter Study. Circulation. 2018;137:250-258. PMID 29030346 — tags: [d-dimer, diagnosis, acute-aortic-syndrome, prospective-cohort] — cited by: biomarkers.md
  • Nazerian P, Mueller C, Vanni S, et al. Integration of transthoracic focused cardiac ultrasound in the diagnostic algorithm for suspected acute aortic syndromes. Eur Heart J. 2019;40:1952-1960. PMID 31226214 — tags: [d-dimer, echocardiography, diagnosis, advised-substudy] — cited by: biomarkers.md
  • Morello F, Mueller C, Soeiro AM, et al. Response by Morello et al to Letters Regarding Article, "Diagnostic Accuracy of the Aortic Dissection Detection Risk Score Plus D-Dimer for Acute Aortic Syndromes". Circulation. 2018;138:448-449. PMID 30571366 — tags: [d-dimer, correspondence, methodology] — cited by: biomarkers.md
  • Watanabe H, Horita N, Shibata Y, et al. Diagnostic test accuracy of D-dimer for acute aortic syndrome: systematic review and meta-analysis of 22 studies with 5000 subjects. Sci Rep. 2016;6:26893. PMID 27230962 — tags: [d-dimer, meta-analysis, diagnostic-accuracy] — cited by: biomarkers.md
  • Yao J, Bai T, Yang B, Sun L. The diagnostic value of D-dimer in acute aortic dissection: a meta-analysis. J Cardiothorac Surg. 2021;16:343. PMID 34838062 — tags: [d-dimer, meta-analysis] — cited by: biomarkers.md
  • Cui JS, Jing ZP, Zhuang SJ, et al. D-dimer as a biomarker for acute aortic dissection: a systematic review and meta-analysis. Medicine (Baltimore). 2015;94:e471. PMID 25634194 — tags: [d-dimer, meta-analysis] — cited by: biomarkers.md
  • Suzuki T, Distante A, Eagle K. Biomarker-assisted diagnosis of acute aortic dissection: how far we have come and what to expect. Curr Opin Cardiol. 2010;25:541-545. PMID 20717014 — tags: [biomarkers, review, smooth-muscle-myosin, calponin] — cited by: biomarkers.md

Circulating candidate biomarkers of aneurysm presence and growth

  • Ikonomidis JS, Ivey CR, Wheeler JB, et al. Plasma biomarkers for distinguishing etiologic subtypes of thoracic aortic aneurysm disease. J Thorac Cardiovasc Surg. 2013;145:1326-1333. PMID 23312977 — tags: [mmp, timp, microrna, bav-vs-tav, plasma] — cited by: biomarkers.md
  • Li T, Jiang B, Li X, et al. Serum matrix metalloproteinase-9 is a valuable biomarker for identification of abdominal and thoracic aortic aneurysm: a case-control study. BMC Cardiovasc Disord. 2018;18:202. PMID 30373522 — tags: [mmp9, case-control, serum] — cited by: biomarkers.md
  • Matt P, Schoenhoff F, Habashi J, et al. Circulating transforming growth factor-beta in Marfan syndrome. Circulation. 2009;120:526-532. PMID 19635970 — tags: [tgf-beta, marfan, losartan, biomarker] — cited by: biomarkers.md
  • Suzuki T, Trimarchi S, Sawaki D, et al. Circulating transforming growth factor-beta levels in acute aortic dissection. J Am Coll Cardiol. 2011;58:775. PMID 21816317 — tags: [tgf-beta, dissection, letter] — cited by: biomarkers.md
  • Boileau A, Lino Cardenas CL, Courtois A, et al. MiR-574-5p: A Circulating Marker of Thoracic Aortic Aneurysm. Int J Mol Sci. 2019;20:3924. PMID 31409059 — tags: [microrna, biomarker, extracellular-vesicles] — cited by: biomarkers.md
  • Ekedi AVNB, Rozhkov AN, Shchekochikhin DY, et al. Evaluation of microRNA Expression Features in Patients with Various Types of Arterial Damage: Thoracic Aortic Aneurysm and Coronary Atherosclerosis. J Pers Med. 2023;13:1161. PMID 37511774 — tags: [microrna, biomarker, case-control] — cited by: biomarkers.md
  • Raffort J, Lareyre F, Clément M, et al. Diabetes and aortic aneurysm: current state of the art. Cardiovasc Res. 2018;114:1702-1713. PMID 30052821 — tags: [diabetes, metformin, review, aaa-vs-taa] — cited by: clinical-trials-landscape.md

Imaging biomarkers beyond diameter

  • Fletcher AJ, Nash J, Syed MBJ, et al. Microcalcification and Thoracic Aortopathy: A Window Into Disease Severity. Arterioscler Thromb Vasc Biol. 2022;42:1048-1059. PMID 35770666 — tags: [18f-naf, pet, microcalcification, histology, nanoindentation] — cited by: biomarkers.md
  • Debono S, Nash J, Fletcher AJ, et al. Aortic sodium [18F]fluoride uptake following endovascular aneurysm repair. Heart. 2023;109:1677-1682. PMID 37164479 — tags: [18f-naf, pet, disease-activity, evar] — cited by: biomarkers.md
  • Guala A, Evangelista A, Teixido-Tura G, et al. Leaflet fusion length is associated with aortic dilation and flow alterations in non-dysfunctional bicuspid aortic valve. Eur Radiol. 2021;31:9262-9272. PMID 33977309 — tags: [4d-flow, bicuspid, wall-shear-stress] — cited by: biomarkers.md
  • Zhu Y, Armour C, Li B, et al. A combined 4D flow MR imaging and fluid-structure interaction analysis of ascending thoracic aortic aneurysms. Biomech Model Mechanobiol. 2025;24:829-844. PMID 40067580 — tags: [4d-flow, fsi, wall-stress, biomechanics] — cited by: biomarkers.md
  • Stephens SB, Shalhub S, Dodd N, et al. Vertebral Tortuosity Is Associated With Increased Rate of Cardiovascular Events in Vascular Ehlers-Danlos Syndrome. J Am Heart Assoc. 2023;12:e029518. PMID 37776192 — tags: [tortuosity, veds, imaging-biomarker, gentac] — cited by: biomarkers.md, clinical-trials-landscape.md

GWAS, polygenic risk, and Mendelian randomization

  • Pirruccello JP, Chaffin MD, Chou EL, et al. Deep learning enables genetic analysis of the human thoracic aorta. Nat Genet. 2022;54:40-51. PMID 34837083 — tags: [gwas, deep-learning, uk-biobank, polygenic-score, svil] — cited by: omics-and-emerging-science.md, biomarkers.md
  • Nekoui M, Pirruccello JP, Di Achille P, et al. Spatially Distinct Genetic Determinants of Aortic Dimensions Influence Risks of Aneurysm and Stenosis. J Am Coll Cardiol. 2022;80:486-497. PMID 35902171 — tags: [gwas, deep-learning, segment-specific, polygenic-score] — cited by: omics-and-emerging-science.md
  • Klarin D, Devineni P, Sendamarai AK, et al. Genome-wide association study of thoracic aortic aneurysm and dissection in the Million Veteran Program. Nat Genet. 2023;55:1106-1115. PMID 37308786 — tags: [gwas, million-veteran-program, taad, non-atherosclerotic] — cited by: omics-and-emerging-science.md, clinical-trials-landscape.md
  • Malhotra R, Mauer AC, Lino Cardenas CL, et al. HDAC9 is implicated in atherosclerotic aortic calcification and affects vascular smooth muscle cell phenotype. Nat Genet. 2019;51:1580-1587. PMID 31659325 — tags: [gwas, hdac9, calcification, smc-phenotype] — cited by: omics-and-emerging-science.md
  • Pirruccello JP, Lin H, Khurshid S, et al. Development of a Prediction Model for Ascending Aortic Diameter Among Asymptomatic Individuals. JAMA. 2022;328:1935-1944. PMID 36378208 — tags: [prediction-model, screening, uk-biobank, framingham] — cited by: omics-and-emerging-science.md, biomarkers.md
  • Pirruccello JP, Khurshid S, Lin H, et al. AORTA Gene: Polygenic prediction improves detection of thoracic aortic aneurysm. medRxiv [preprint]. 2023. PMID 37662232 — tags: [polygenic-score, prediction, preprint, all-of-us] — cited by: omics-and-emerging-science.md, biomarkers.md
  • Kelemen M, Danesh J, Di Angelantonio E, et al. Evaluating the cost-effectiveness of polygenic risk score-stratified screening for abdominal aortic aneurysm. Nat Commun. 2024;15:8063. PMID 39277617 — tags: [polygenic-score, screening, cost-effectiveness, aaa] — cited by: omics-and-emerging-science.md
  • Georgakis MK, Malik R, Richardson TG, et al. Associations of genetically predicted IL-6 signaling with cardiovascular disease risk across population subgroups. BMC Med. 2022;20:245. PMID 35948913 — tags: [mendelian-randomization, il-6, inflammation, composite-endpoint] — cited by: omics-and-emerging-science.md
  • Zekavat SM, Viana-Huete V, Matesanz N, et al. TP53-mediated clonal hematopoiesis confers increased risk for incident atherosclerotic disease. Nat Cardiovasc Res. 2023;2:144-158. PMID 36949957 — tags: [clonal-hematopoiesis, chip, atherosclerosis] — cited by: omics-and-emerging-science.md

Single-cell and spatial transcriptomics of the aorta

  • Li Y, Ren P, Dawson A, et al. Single-Cell Transcriptome Analysis Reveals Dynamic Cell Populations and Differential Gene Expression Patterns in Control and Aneurysmal Human Aortic Tissue. Circulation. 2020;142:1374-1388. PMID 33017217 — tags: [scrna-seq, human-ataa, erg, mitochondrial-dysfunction] — cited by: omics-and-emerging-science.md
  • Pedroza AJ, Tashima Y, Shad R, et al. Single-Cell Transcriptomic Profiling of Vascular Smooth Muscle Cell Phenotype Modulation in Marfan Syndrome Aortic Aneurysm. Arterioscler Thromb Vasc Biol. 2020;40:2195-2211. PMID 32698686 — tags: [scrna-seq, marfan, modsmc, serpine1, klf4] — cited by: omics-and-emerging-science.md
  • Dawson A, Li Y, Li Y, et al. Single-Cell Analysis of Aneurysmal Aortic Tissue in Patients with Marfan Syndrome Reveals Dysfunctional TGF-β Signaling. Genes (Basel). 2022;13:95. PMID 35052435 — tags: [scrna-seq, marfan, tgf-beta-paradox] — cited by: omics-and-emerging-science.md, biomarkers.md
  • Chou EL, Chaffin M, Simonson B, et al. Aortic Cellular Diversity and Quantitative Genome-Wide Association Study Trait Prioritization Through Single-Nuclear RNA Sequencing of the Aneurysmal Human Aorta. Arterioscler Thromb Vasc Biol. 2022;42:1355-1374. PMID 36172868 — tags: [snrna-seq, gwas-prioritization, ltbp1, il34, pdlim5, actn4] — cited by: omics-and-emerging-science.md
  • Zhen K, Du Z, Wang P, et al. Spatial and single-cell RNA sequencing reveals the immune microenvironment of human ascending thoracic aortic aneurysms. Clin Transl Med. 2026;16:e70650. PMID 42007493 — tags: [spatial-transcriptomics, visium-hd, immune-atlas, nf-kb] — cited by: omics-and-emerging-science.md
  • Bramel EE, Espinoza Camejo WA, Creamer TJ, et al. Intrinsic expression sensitizes the aortic root to dilation in a Loeys-Dietz syndrome mouse model. Res Sq [preprint]. 2024. PMID 38883722 — tags: [spatial-transcriptomics, loeys-dietz, aortic-root, preprint] — cited by: omics-and-emerging-science.md

Proteomics and mechanism

  • Sawada H, Katsumata Y, Higashi H, et al. Second Heart Field-Derived Cells Contribute to Angiotensin II-Mediated Ascending Aortopathies. Circulation. 2022;145:987-1001. PMID 35143327 — tags: [proteomics, second-heart-field, lrp1, pai-1, angiotensin-ii] — cited by: omics-and-emerging-science.md
  • Lino Cardenas CL, Kessinger CW, Cheng Y, et al. An HDAC9-MALAT1-BRG1 complex mediates smooth muscle dysfunction in thoracic aortic aneurysm. Nat Commun. 2018;9:1009. PMID 29520069 — tags: [epigenetics, hdac9, malat1, brg1, drug-target] — cited by: omics-and-emerging-science.md
  • Lino Cardenas CL, Kessinger CW, Chou E, et al. HDAC9 complex inhibition improves smooth muscle-dependent stenotic vascular disease. JCI Insight. 2019;4:e124706. PMID 30674723 — tags: [antisense-oligonucleotide, malat1, ezh2, therapeutic-target] — cited by: omics-and-emerging-science.md
  • Huang K, Wang Y, Siu KL, Zhang Y, Cai H. Targeting feed-forward signaling of TGFβ/NOX4/DHFR/eNOS uncoupling/TGFβ axis with anti-TGFβ and folic acid attenuates formation of aortic aneurysms. Redox Biol. 2020;38:101757. PMID 33126053 — tags: [redox, enos-uncoupling, tetrahydrobiopterin, fbn1-mouse] — cited by: omics-and-emerging-science.md

Machine learning and automated imaging

  • Lo Piccolo F, Hinck D, Segeroth M, et al. Impact of retraining a deep learning algorithm for improving guideline-compliant aortic diameter measurements on non-gated chest CT. Eur J Radiol. 2023;168:111093. PMID 37716024 — tags: [deep-learning, automated-measurement, ct, aortic-root] — cited by: omics-and-emerging-science.md
  • Krebs JR, Imran M, Fazzone B, et al. Volumetric analysis of acute uncomplicated type B aortic dissection using an automated deep learning aortic zone segmentation model. J Vasc Surg. 2024;80:1025-1034.e4. PMID 38851467 — tags: [deep-learning, segmentation, growth-prediction, type-b-dissection] — cited by: omics-and-emerging-science.md
  • Kany S, Rämö JT, Hou C, et al. Assessment of valvular function in over 47,000 people using deep learning-based flow measurements. medRxiv [preprint]. 2023. PMID 37205587 — tags: [deep-learning, uk-biobank, flow-phenotyping, preprint] — cited by: omics-and-emerging-science.md
  • Ayhan C, Mekhaeil M, Channawi R, et al. Applications of Artificial Intelligence as a Prognostic Tool in the Management of Acute Aortic Syndrome and Aneurysm: A Comprehensive Review. J Clin Med. 2025;14:8420. PMID 41375721 — tags: [machine-learning, prognosis, review, tripod-ai] — cited by: omics-and-emerging-science.md

Trial methodology, registries, and devices

  • Pitcher A, Spata E, Emberson J, et al. Angiotensin receptor blockers and β blockers in Marfan syndrome: an individual patient data meta-analysis of randomised trials. Lancet. 2022;400:822-831. PMID 36049495 — tags: [ipd-meta-analysis, marfan, surrogate-endpoint, arb, beta-blocker] — cited by: clinical-trials-landscape.md, biomarkers.md
  • Roselli EE, Atkins MD, Brinkman W, et al. ARISE: First-In-Human Evaluation of a Novel Stent Graft to Treat Ascending Aortic Dissection. J Endovasc Ther. 2023;30:550-560. PMID 35587698 — tags: [ascending-tevar, arise, early-feasibility, device] — cited by: clinical-trials-landscape.md
  • Suh GK, Bondesson J, Zhu YD, et al. Ascending Aortic Endograft and Thoracic Aortic Deformation After Ascending Thoracic Endovascular Aortic Repair. J Endovasc Ther. 2023;32:7-17. PMID 37144300 — tags: [ascending-tevar, biomechanics, device-durability, arise-substudy] — cited by: clinical-trials-landscape.md
  • Kroner BL, Tolunay HE, Basson CT, et al. The National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC): results from phase I and scientific opportunities in phase II. Am Heart J. 2011;162:627-632.e1. PMID 21982653 — tags: [registry, gentac, biorepository] — cited by: clinical-trials-landscape.md
  • Asokan KL, Landes JR, Renders W, et al. Mitral Annular Disjunction in Heritable Thoracic Aortic Disease: Insights From the Montalcino Aortic Consortium. J Am Heart Assoc. 2024;13:e036274. PMID 39424426 — tags: [registry, montalcino-aortic-consortium, htad, smad3] — cited by: clinical-trials-landscape.md

Genotype-specific natural history (cited as genetic biomarkers)

  • Regalado ES, Mellor-Crummey L, De Backer J, et al. Clinical history and management recommendations of the smooth muscle dysfunction syndrome due to ACTA2 arginine 179 alterations. Genet Med. 2018;20:1206-1215. PMID 29300374 — tags: [acta2, smds, natural-history, genotype-phenotype] — cited by: biomarkers.md
  • Shalhub S, Regalado ES, Guo DC, Milewicz DM. The natural history of type B aortic dissection in patients with PRKG1 mutation c.530G>A (p.Arg177Gln). J Vasc Surg. 2019;70:718-723. PMID 30871887 — tags: [prkg1, natural-history, type-b-dissection, small-diameter-dissection] — cited by: biomarkers.md

Trial registrations (ClinicalTrials.gov, verified 2026-08-27)

Strategy and pharmacotherapy

  • NCT03536312 — TITAN:SvS, early surgery vs surveillance at 5.0–5.4 cm, RCT, n=610, Recruiting, primary completion 2035 — cited by: clinical-trials-landscape.md, biomarkers.md
  • NCT07483177 — HEART pilot, spironolactone vs placebo in degenerative TAA, phase 1/2, n=50, Recruiting — cited by: clinical-trials-landscape.md
  • NCT06280482 — Nicotinamide riboside in ACTA2 R179 smooth muscle dysfunction syndrome, phase 1, n=15, Recruiting — cited by: clinical-trials-landscape.md, omics-and-emerging-science.md
  • NCT04197648 — Exercise and blood pressure control in TAA, n=30, Recruiting — cited by: clinical-trials-landscape.md
  • NCT07495267 — Nutritional ketosis in Marfan chronic dissection, n=15, Not yet recruiting — cited by: clinical-trials-landscape.md
  • NCT07205250 — Aspirin in type B / non-A-non-B dissection and TAA, n=224, Not yet recruiting — cited by: clinical-trials-landscape.md
  • NCT05215587 — Scandinavian Trial of Uncomplicated Aortic Dissection Therapy (TEVAR), n=554, Recruiting — cited by: clinical-trials-landscape.md

Marfan / heritable aortopathy (status only; results on medical-therapy.md)

  • NCT00429364 — Atenolol vs losartan, Pediatric Heart Network, n=608, Completed — cited by: clinical-trials-landscape.md
  • NCT00763893 — Marfan Sartan, losartan vs placebo, n=303, Terminated — cited by: clinical-trials-landscape.md
  • NCT01949233 — Irbesartan ± doxycycline, Oxford, n=56, Unknown — cited by: clinical-trials-landscape.md
  • NCT00782327 — Losartan vs placebo on beta-blocker, Ghent, n=22, Completed — cited by: clinical-trials-landscape.md
  • NCT00723801 — Losartan vs atenolol, Brigham, n=40, Completed — cited by: clinical-trials-landscape.md
  • NCT01145612 — Losartan vs atenolol, Spain, n=140, Unknown — cited by: clinical-trials-landscape.md
  • NCT00683124 — Losartan vs nebivolol vs both, Pavia, n=291, Unknown — cited by: clinical-trials-landscape.md
  • NCT01715207 — Aliskiren vs control on atenolol, n=30, Completed — cited by: clinical-trials-landscape.md
  • NCT01295047 — Atenolol vs perindopril vs verapamil crossover, Cardiff, n=18, Completed — cited by: clinical-trials-landscape.md
  • NCT00485368 — Perindopril and aortic wall properties, n=17, Completed — cited by: clinical-trials-landscape.md
  • NCT00593710 — Losartan vs atenolol, PWV, UBC, n=17, Completed — cited by: clinical-trials-landscape.md
  • NCT01361087 — Circulating TGF-β in Marfan, n=0, Withdrawn — cited by: clinical-trials-landscape.md, biomarkers.md

Abdominal aortic aneurysm pharmacotherapy (TAA/AAA distinction)

  • NCT04500756 — LIMIT, metformin vs placebo in AAA, phase 2, n=314, Recruiting — cited by: clinical-trials-landscape.md
  • NCT04224051 — MAAAGI, metformin in AAA, phase 2, n=500, Unknown — cited by: clinical-trials-landscape.md
  • NCT03507413 — MetAAA, metformin in non-diabetic AAA, phase 2/3, n=170, Unknown — cited by: clinical-trials-landscape.md
  • NCT05361772 — Low-dose colchicine in AAA, n=230, Unknown — cited by: clinical-trials-landscape.md
  • NCT01756833 — N-TA³CT, doxycycline vs placebo in AAA, phase 2, n=261, Completed — cited by: clinical-trials-landscape.md
  • NCT00538967 — Doxycycline and MMP activity in AAA, phase 2, n=60, Completed — cited by: clinical-trials-landscape.md
  • NCT00126204 — Adjunctive doxycycline with EVAR, n=75, Completed — cited by: clinical-trials-landscape.md

Ascending and arch devices

  • NCT05800743 — ARISE II, GORE Ascending Stent Graft ± TBE, n=370, Enrolling by invitation, completion 2034 — cited by: clinical-trials-landscape.md
  • NCT06827990 — GORE ASG in de novo type A dissection, n=112, Recruiting — cited by: clinical-trials-landscape.md
  • NCT02201589 — Valiant PS-IDE ascending feasibility, n=20, Recruiting — cited by: clinical-trials-landscape.md
  • NCT03322033 — Valiant PS-IDE ascending feasibility (Baylor), n=1, Terminated — cited by: clinical-trials-landscape.md
  • NCT05174767 — PERSEVERE, AMDS in acute DeBakey I, n=115, Active not recruiting — cited by: clinical-trials-landscape.md
  • NCT06044259 — RADAR, hemiarch ± AMDS, RCT, n=72, Recruiting — cited by: clinical-trials-landscape.md
  • NCT02724072 — Thoraflex Hybrid IDE, n=84, Completed — cited by: clinical-trials-landscape.md
  • NCT03214601 — Relay Branch early feasibility (zone 0), n=30, Active not recruiting — cited by: clinical-trials-landscape.md
  • NCT06550986 / NCT06740721 — IMPEDE-FX RapidFill false-lumen embolisation, n=30 / 15, Recruiting / Not yet recruiting — cited by: clinical-trials-landscape.md
  • NCT04545502 — Terumo Aortic graft post-market registry, n=2,000, Recruiting — cited by: clinical-trials-landscape.md
  • NCT04381507 — CONFORM-TAA post-market study, n=1, Terminated — cited by: clinical-trials-landscape.md

Registries, biorepositories, mechanism-facing observational

  • NCT03440697 — Pathogenetic Basis of Aortopathy and Aortic Valve Disease (Yale / NHLBI), n=3,000, Active not recruiting, completion 2030 — cited by: clinical-trials-landscape.md
  • NCT02256163 — Identification of genes and pathogenesis in familial TAA (AP-HP), n=258, Completed — cited by: clinical-trials-landscape.md
  • NCT06783803 — Linkage analysis in familial aneurysms, n=20, Active not recruiting — cited by: clinical-trials-landscape.md
  • NCT03142074 — Biomechanical and microstructural properties of the aortic wall (KU Leuven), n=180, Unknown — cited by: clinical-trials-landscape.md
  • NCT03410420 — Pimonidazole hypoxia detection in human thoracic aorta, phase 1, n=9, Completed — cited by: clinical-trials-landscape.md
  • NCT03090763 — Micro AAA, circulating microRNA in abdominal and thoracic aneurysm, n=200, Unknown — cited by: clinical-trials-landscape.md
  • NCT05715203 — Aortic stiffness in genetic aortopathies, n=250, Recruiting — cited by: clinical-trials-landscape.md
  • NCT07034430 — Genetic risk stratification in ascending dilatation below surgical threshold, n=102, Active not recruiting — cited by: clinical-trials-landscape.md
  • NCT06377033 — Behavioural nudges for genomic medicine uptake including TAA (UPenn), n=1,000, Recruiting — cited by: clinical-trials-landscape.md
  • NCT02538822 — Rupture-risk assessment from dynamic imaging (Saint-Étienne), n=31, Terminated — cited by: clinical-trials-landscape.md
  • NCT02299947 — FIBTEG, fibrinogen after ascending aortic surgery, phase 4, n=27, Terminated — cited by: clinical-trials-landscape.md

Human dimension (patient experience & advocacy)

Sourced by the patient-voice layer; non-journal sources (organizations, campaigns, videos) live in patient-voice/sources.md.

  • Lovatt S, et al. Misdiagnosis of aortic dissection: A systematic review of the literature. Am J Emerg Med. 2022;53:16-22. PMID 34968970 — tags: [patient-experience] — cited by: red-flags-and-safety-concerns.md, patient-experience-and-advocacy.md
  • Harris KM, et al. Correlates of delayed recognition and treatment of acute type A aortic dissection: the International Registry of Acute Aortic Dissection (IRAD). Circulation. 2011;124:1911-8. PMID 21969019 — tags: [patient-experience] — cited by: red-flags-and-safety-concerns.md, patient-experience-and-advocacy.md
  • Holmes HR, et al. Misdiagnosis of Thoracic Aortic Disease Occurs Commonly in Emergency Transfers. Ann Thorac Surg. 2022;114:2202-2208. PMID 34838743 — tags: [patient-experience] — cited by: red-flags-and-safety-concerns.md, patient-experience-and-advocacy.md
  • Reed MJ. Diagnosis and management of acute aortic dissection in the emergency department. Br J Hosp Med (Lond). 2024;85:1-9. PMID 38708978 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Lee JR, et al. Lived experiences of people with or at risk for aortic dissection: A qualitative assessment. Semin Vasc Surg. 2022;35:78-87. PMID 35501045 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Pasadyn SR, et al. From Tear to Fear: Posttraumatic Stress Disorder in Patients With Acute Type A Aortic Dissection. J Am Heart Assoc. 2020;9:e015060. PMID 32340520 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Chaddha A, et al. Survivors of Aortic Dissection: Activity, Mental Health, and Sexual Function. Clin Cardiol. 2015;38:652-9. PMID 26769699 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Chaddha A, et al. Exercise and Physical Activity for the Post-Aortic Dissection Patient: The Clinician's Conundrum. Clin Cardiol. 2015;38:647-51. PMID 26769698 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Chaddha A, et al. Cardiology patient page: Activity recommendations for postaortic dissection patients. Circulation. 2014;130:e140-2. PMID 25311622 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Chaddha A, et al. Medication adherence patterns in aortic dissection survivors. Indian J Med Res. 2018;147:183-188. PMID 29806607 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Chaddha A, et al. The Clinical Impact of Imaging Surveillance and Clinic Visit Frequency after Acute Aortic Dissection. Aorta (Stamford). 2019;7:75-83. PMID 31614376 — tags: [patient-experience] — cited by: red-flags-and-safety-concerns.md, patient-experience-and-advocacy.md
  • McEntire A, et al. Psychological distress in response to physical activity restrictions in patients with non-syndromic thoracic aortic aneurysm/dissection. J Community Genet. 2021;12:631-641. PMID 34386933 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Millette TJ, et al. Exercise, Sports Participation, and Quality of Life in Young Patients with Heritable Thoracic Aortic Disease. Med Sci Sports Exerc. 2024 (print 2025);57:260-266. PMID 39352231 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Dreher H, et al. Symptoms of post-traumatic distress and quality of life in adults with aortopathy and congenital heart defects or hereditary connective tissue diseases. Cardiovasc Diagn Ther. 2025;15:781-791. PMID 40948724 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Liu C, et al. Psychological experiences of patients with Stanford type B aortic dissection during the preoperative waiting period: a qualitative study. BMC Cardiovasc Disord. 2026;26. PMID 42277648 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Bacour Y, et al. From Survival to Recovery: Understanding the Life Impact of an Acute Aortic Dissection Through Activity, Sleep, and Quality of Life. J Clin Med. 2025;14:859. PMID 39941528 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Luo B, et al. Comparative analysis of postoperative sexual dysfunction and quality of life in type A aortic dissection patients of different ages. J Cardiothorac Surg. 2021;16:117. PMID 33933114 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Feng J, et al. Attitudes and behavioral intentions of aortic dissection survivors towards exercise: an application of the health action process approach framework. Rev Cardiovasc Med. 2022;23:64. PMID 35229555 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Gao J, et al. Influencing factors of health promotion behaviour in patients with aortic dissection: a qualitative study using the COM-B model. BMJ Open. 2025;15:e076181. PMID 40044200 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Velvin G, et al. Quality of life in people with syndromic heritable thoracic aortic disease and their relatives: a qualitative interview based study. Orphanet J Rare Dis. 2025;20:12. PMID 39789589 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Velvin G, et al. Systematic review of quality of life in persons with hereditary thoracic aortic aneurysm and dissection diagnoses. Clin Genet. 2019;95:661-676. PMID 30788842 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Velvin G, et al. Satisfaction with life in adults with Marfan syndrome (MFS): associations with health-related consequences of MFS, pain, fatigue, and demographic factors. Qual Life Res. 2016;25:1779-90. PMID 26727916 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Velvin G, et al. Work participation in adults with Marfan syndrome: Demographic characteristics, MFS related health symptoms, chronic pain, and fatigue. Am J Med Genet A. 2015;167A:3082-90. PMID 26420568 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Bathen T, et al. Fatigue in adults with Marfan syndrome, occurrence and associations to pain and other factors. Am J Med Genet A. 2014;164A:1931-9. PMID 24719044 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Lidal IB, et al. A scoping review presenting a wide variety of research on paediatric and adolescent patients with Marfan syndrome. Acta Paediatr. 2020;109:1758-1771. PMID 31977115 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Abbasciano R, et al. Patient and family perspectives on cascade screening for thoracic aortic disease: a mixed-methods evaluation. Eur J Hum Genet. 2026;34:947-955. PMID 41772284 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Russo M, et al. Aortic dissection in pregnancy and the postpartum period. Semin Vasc Surg. 2022;35:60-68. PMID 35501042 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Somalo-Barranco G, et al. Management of pregnancy in women with rare multisystemic vascular diseases: a qualitative survey analysis. Orphanet J Rare Dis. 2026. PMID 42365315 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Lee JR, et al. The Aortic Dissection Collaborative: Methods for building capacity for patient-centered outcomes research in the aortic dissection community. Semin Vasc Surg. 2022;35:9-15. PMID 35501047 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Ilonzo N, et al. The mental health impact of aortic dissection. Semin Vasc Surg. 2022;35:88-99. PMID 35501046 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Talutis SD, et al. Stakeholder perspectives on education in aortic dissection. Semin Vasc Surg. 2022;35:69-77. PMID 35501043 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Pena RCF, et al. An assessment of the current medical management of thoracic aortic disease: A patient-centered scoping literature review. Semin Vasc Surg. 2022;35:16-34. PMID 35501038 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Nishath T, et al. Implementation of telemedicine in the care of patients with aortic dissection. Semin Vasc Surg. 2022;35:43-50. PMID 35501040 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Lee JR, et al. A mixed method approach to understanding the impact of COVID-19 on patients with or at risk for aortic dissection. Semin Vasc Surg. 2022;35:100-109. PMID 35501037 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Harky A, et al. COVID-19 and cardiac surgery: A perspective from United Kingdom. J Card Surg. 2020 (print 2021);36:1649-1658. PMID 32981073 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Lee MY, et al. Lost to follow-up: A narrative review of socioeconomic, psychosocial, and systemic barriers to aortic dissection surveillance. Semin Vasc Surg. 2026;39:166-172. PMID 42285644 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Grewal S, et al. From survival to lifelong care: The role of patient organisations in aortic dissection. Am Heart J Plus. 2026;68:100834. PMID 42491301 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Shan R, et al. Dissecting Aortas and Patient Reported Outcome Measures. Eur J Vasc Endovasc Surg. 2023;66:351. PMID 37451607 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Huang W, et al. Preliminary evaluation of the Chinese version of the patient-reported outcomes measurement information system 29-item profile in patients with aortic dissection. Health Qual Life Outcomes. 2022;20:94. PMID 35701761 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Smolderen KG, et al. Evolving dynamic needs for patient-reported outcomes assessment in individuals with an abdominal aortic aneurysm (AAA): A systematic review. Vasc Med. 2026;31:232-245. PMID 41854119 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md
  • Knops AM, et al. A decision aid regarding treatment options for patients with an asymptomatic abdominal aortic aneurysm: a randomised clinical trial. Eur J Vasc Endovasc Surg. 2014;48:276-83. PMID 24913683 — tags: [patient-experience] — cited by: patient-experience-and-advocacy.md

Audit additions — 2026-08-28

Papers added during the citation-audit pass; every PMID verified live this session. Grouped by the section they extend.

Foundations — dissection classification standards

  • Lombardi JV, et al. Society for Vascular Surgery (SVS) and Society of Thoracic Surgeons (STS) reporting standards for type B aortic dissections. J Vasc Surg. 2020;71:723-747. PMID 32001058 — tags: [classification, dissection, reporting-standards] — cited by: aortic-dissection.md

Genetics — gene discovery

  • van de Laar IM, et al. Mutations in SMAD3 cause a syndromic form of aortic aneurysms and dissections with early-onset osteoarthritis. Nat Genet. 2011;43:121-6. PMID 21217753 — tags: [SMAD3, gene-discovery, aneurysms-osteoarthritis] — cited by: genetics-of-taa.md

Mechanism — large-animal models

  • Eckhouse SR, et al. Reproducible porcine model of thoracic aortic aneurysm. Circulation. 2013;128(11 Suppl 1):S186-93. PMID 24030405 — tags: [animal-models, large-animal, porcine] — cited by: animal-models.md
  • Tian Y, et al. A reproducible swine model of proximal descending thoracic aortic aneurysm created with intra-adventitial application of elastase. J Vasc Surg. 2018;67:300-308.e2. PMID 28479097 — tags: [animal-models, large-animal, porcine, elastase] — cited by: animal-models.md
  • Kenawy DM, et al. A porcine model of thoracic aortic aneurysms created with a retrievable drug infusion stent graft mirrors human aneurysm pathophysiology. JVS Vasc Sci. 2024;5:100212. PMID 39188992 — tags: [animal-models, large-animal, porcine, endovascular] — cited by: animal-models.md
  • El Batti S, et al. Experimental Evaluation of Endovascular Fenestration Scissors in an Ovine Model of Aortic Dissection. Eur J Vasc Endovasc Surg. 2018;56:373-380. PMID 30005965 — tags: [animal-models, large-animal, ovine, dissection] — cited by: animal-models.md

Frontier — biomarkers (sST2, desmosine, lumican, FDG)

  • Wang Y, et al. Magnitude of Soluble ST2 as a Novel Biomarker for Acute Aortic Dissection. Circulation. 2018;137:259-269. PMID 29146682 — tags: [sST2, dissection, diagnosis] — cited by: biomarkers.md
  • Morello F, et al. Prospective diagnostic accuracy study of plasma soluble ST2 for diagnosis of acute aortic syndromes. Sci Rep. 2020;10:3103. PMID 32080259 — tags: [sST2, replication-failure, diagnosis] — cited by: biomarkers.md
  • Wren J, et al. Diagnostic accuracy of alternative biomarkers for acute aortic syndrome: a systematic review. Emerg Med J. 2024;41:678-685. PMID 39107052 — tags: [biomarkers, systematic-review, acute-aortic-syndrome] — cited by: biomarkers.md
  • Doukas P, et al. Plasma Desmosine Is Elevated in Thoracoabdominal Aortic Aneurysms and Is Associated with Intramural Proteolytic Activity. Int J Mol Sci. 2026;27. PMID 41683665 — tags: [desmosine, elastin-turnover, thoracoabdominal] — cited by: biomarkers.md
  • Mordi IR, et al. Plasma Desmosine and Abdominal Aortic Aneurysm Disease. J Am Heart Assoc. 2019;8:e013743. PMID 31595818 — tags: [desmosine, AAA, prognosis] — cited by: biomarkers.md
  • Kuzmanova E, et al. Accelerating isotope dilution LC-MS-based desmosine quantification for estimating elastin turnover. Bioanalysis. 2025;17:99-104. PMID 39891463 — tags: [desmosine, assay-methods] — cited by: biomarkers.md
  • Gombert A, et al. Desmosine in Aortic Disease: Biology, Measurement, and Clinical Applications in Aortic Pathologies. J Clin Med. 2026;15. PMID 41976840 — tags: [desmosine, review] — cited by: biomarkers.md
  • Gu G, et al. Lumican as a novel potential clinical indicator for acute aortic dissection: A comparative study, based on multi-slice computed tomography angiography. Exp Ther Med. 2016;11:923-928. PMID 26998013 — tags: [lumican, dissection, diagnosis] — cited by: biomarkers.md
  • Chen SW, et al. Expression and role of lumican in acute aortic dissection: A human and mouse study. PLoS One. 2021;16:e0255238. PMID 34310653 — tags: [lumican, mechanism] — cited by: biomarkers.md
  • Hsu ME, et al. Level of serum soluble lumican and risks of perioperative complications in patients receiving aortic surgery. PLoS One. 2021;16:e0247340. PMID 33661915 — tags: [lumican, perioperative] — cited by: biomarkers.md
  • Harrison OJ, et al. Candidate plasma biomarkers for predicting ascending aortic aneurysm in bicuspid aortic valve disease. J Cardiothorac Surg. 2018;13:76. PMID 29929532 — tags: [lumican, BAV, pilot] — cited by: biomarkers.md
  • Espitia O, et al. [Aortitis]. Rev Med Interne. 2024;45:767-775. PMID 39034261 — tags: [fdg-pet, aortitis] — cited by: biomarkers.md
  • Hasse B, et al. 18F-fluorodeoxyglucose uptake patterns in positron emission tomography/computed tomography caused by inflammation and/or infection after graft surgery for thoracic aortic dissection. J Nucl Cardiol. 2024;36:101865. PMID 38679286 — tags: [fdg-pet, postoperative] — cited by: biomarkers.md

Frontier — Mendelian randomization, proteomics, gene-targeting horizon

  • DePaolo J, et al. Relationship Between Ascending Thoracic Aortic Diameter and Blood Pressure: A Mendelian Randomization Study. Arterioscler Thromb Vasc Biol. 2023;43:359-366. PMID 36601961 — tags: [mendelian-randomization, blood-pressure] — cited by: omics-and-emerging-science.md
  • Yang T, et al. Causal effect of hypertension and blood pressure on aortic diseases: evidence from Mendelian randomization. Hypertens Res. 2023;46:2203-2212. PMID 37443259 — tags: [mendelian-randomization, hypertension] — cited by: omics-and-emerging-science.md
  • Zhou T, et al. Remnant cholesterol and risk of aortic aneurysm and dissection: a prospective cohort Study from the UK biobank study and mendelian randomization analysis. Lipids Health Dis. 2025;24:53. PMID 39962497 — tags: [mendelian-randomization, lipids, TAA-null] — cited by: omics-and-emerging-science.md
  • Daskalopoulou A, et al. Targeted Proteomic Analysis of Patients with Ascending Thoracic Aortic Aneurysm. Biomedicines. 2023;11:1273. PMID 37238945 — tags: [proteomics, cross-sectional] — cited by: omics-and-emerging-science.md
  • Arndt N, et al. Thoracic aortic diseases: Identification of diagnostic biomarkers using proteomic analysis. Cardiovasc Pathol. 2025;80:107785. PMID 41061941 — tags: [proteomics, extracellular-vesicles] — cited by: omics-and-emerging-science.md
  • Kimura K, et al. Novel Aortic Dissection Model Links Endothelial Dysfunction and Immune Infiltration. Circ Res. 2025;137:26-42. PMID 40365676 — tags: [CRISPR-model, Fbn1, dissection-model] — cited by: omics-and-emerging-science.md
  • Kaw A, et al. Mosaicism for the smooth muscle cell (SMC)-specific knock-in of the Acta2 R179C pathogenic variant: Implications for gene editing therapies. J Mol Cell Cardiol. 2022;171:102-104. PMID 35878552 — tags: [gene-editing, ACTA2, mosaicism] — cited by: omics-and-emerging-science.md
  • Winter H, et al. Targeting long non-coding RNA NUDT6 enhances smooth muscle cell survival and limits vascular disease progression. Mol Ther. 2023;31:1775-1790. PMID 37147804 — tags: [antisense, lncRNA, preclinical] — cited by: omics-and-emerging-science.md

Red flags & safety concerns — 2026-08-28

New papers cited by wiki/red-flags-and-safety-concerns.md; papers it shares with other pages carry the tag on their existing lines above.

  • Park SW, et al. Association of painless acute aortic dissection with increased mortality. Mayo Clin Proc. 2004;79:1252-7. PMID 15473405 — tags: [dissection, painless, syncope, stroke, mortality, patient-safety] — cited by: red-flags-and-safety-concerns.md
  • Tolenaar JL, et al. Painless type B aortic dissection: insights from the International Registry of Acute Aortic Dissection. Aorta (Stamford). 2013;1:96-101. PMID 26798680 — tags: [dissection, type-b, painless, diagnostic-delay, patient-safety] — cited by: red-flags-and-safety-concerns.md
  • Nallamothu BK, et al. Syncope in acute aortic dissection: diagnostic, prognostic, and clinical implications. Am J Med. 2002;113:468-71. PMID 12427495 — tags: [dissection, syncope, tamponade, stroke, presentation] — cited by: red-flags-and-safety-concerns.md
  • Mehta RH, et al. Acute type A aortic dissection in the elderly: clinical characteristics, management, and outcomes in the current era. J Am Coll Cardiol. 2002;40:685-92. PMID 12204498 — tags: [dissection, elderly, atypical-presentation, irad, mortality] — cited by: red-flags-and-safety-concerns.md
  • Niclauss L, et al. Impact of preoperative central neurologic dysfunction on patients undergoing emergency surgery for type A dissection. Ann Vasc Surg. 2014;28:1227-35. PMID 24184458 — tags: [dissection, neurological-presentation, misdiagnosis, time-to-surgery] — cited by: red-flags-and-safety-concerns.md
  • Sakamoto Y, et al. Frequency and detection of Stanford type A aortic dissection in hyperacute stroke management. Cerebrovasc Dis. 2016;42:110-6. PMID 27070149 — tags: [dissection, stroke-mimic, thrombolysis-risk, d-dimer, patient-safety] — cited by: red-flags-and-safety-concerns.md
  • Rogers AM, et al. Sensitivity of the aortic dissection detection risk score, a novel guideline-based tool for identification of acute aortic dissection at initial presentation: results from the International Registry of Acute Aortic Dissection. Circulation. 2011;123:2213-8. PMID 21555704 — tags: [add-risk-score, diagnosis, irad, validation, patient-safety] — cited by: red-flags-and-safety-concerns.md
  • Nazerian P, et al. Diagnostic accuracy of the aortic dissection detection risk score plus D-dimer for acute aortic syndromes: the ADvISED prospective multicenter study. Circulation. 2018;137:250-258. PMID 29030346 — tags: [add-risk-score, d-dimer, rule-out, prospective, NCT02086136] — cited by: red-flags-and-safety-concerns.md
  • Loeys BL, et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet. 2010;47:476-85. PMID 20591885 — tags: [marfan, diagnostic-criteria, ectopia-lentis, FBN1, screening] — cited by: red-flags-and-safety-concerns.md [check: may already exist under syndromic-aortopathies.md]
  • Hibino M, et al. Blood pressure, hypertension, and the risk of aortic dissection incidence and mortality: results from the J-SHC study, the UK Biobank study, and a meta-analysis of cohort studies. Circulation. 2022;145:633-644. PMID 34743557 — tags: [hypertension, dose-response, meta-analysis, uk-biobank, prevention] — cited by: red-flags-and-safety-concerns.md
  • Kamel H, et al. Pregnancy and the risk of aortic dissection or rupture: a cohort-crossover analysis. Circulation. 2016;134:527-33. PMID 27492904 — tags: [pregnancy, dissection, population-based, cohort-crossover] — cited by: red-flags-and-safety-concerns.md
  • Martín CE, et al. Aortic events in pregnant patients with Marfan syndrome. Lessons from a multicenter study. Rev Esp Cardiol (Engl Ed). 2022;75:552-558. PMID 34481752 — tags: [pregnancy, marfan, aortic-events, third-trimester, multicenter] — cited by: red-flags-and-safety-concerns.md
  • Chang FC, et al. Maternal and neonatal outcomes in Marfan syndrome: a nationwide registry. Heart. 2025 (epub 2025-12-31). PMID 41475970 — tags: [pregnancy, marfan, nationwide, taiwan, maternal-mortality] — cited by: red-flags-and-safety-concerns.md
  • Smith K, Gros B. Pregnancy-related acute aortic dissection in Marfan syndrome: a review of the literature. Congenit Heart Dis. 2017;12:251-260. PMID 28371362 — tags: [pregnancy, marfan, dissection, small-diameter-events, review] — cited by: red-flags-and-safety-concerns.md
  • Stewart FM. Marfan's syndrome and other aortopathies in pregnancy. Obstet Med. 2013;6:112-119. PMID 27708702 — tags: [pregnancy, aortopathy, loeys-dietz, vEDS, turner, review] — cited by: red-flags-and-safety-concerns.md
  • Tadakawa M, et al. Fatal aortic rupture at term pregnancy caused by vascular Ehlers-Danlos syndrome diagnosed by postmortem genetic testing using formalin-fixed, paraffin-embedded tissue. J Obstet Gynaecol Res. 2026;52:e70299. PMID 42092737 — tags: [pregnancy, vEDS, COL3A1, maternal-death, case-report] — cited by: red-flags-and-safety-concerns.md
  • Greve D, et al. Cocaine-related aortic dissection: what do we know? Braz J Cardiovasc Surg. 2020;35:764-769. PMID 33118742 — tags: [cocaine, stimulants, dissection, case-series, young-patients] — cited by: red-flags-and-safety-concerns.md
  • Matabele M, et al. Role of illicit drug use in type B aortic dissection and trial participation. Semin Vasc Surg. 2026;39:151-156. PMID 42285641 — tags: [stimulants, cocaine, amphetamine, type-b, trial-exclusion, review] — cited by: red-flags-and-safety-concerns.md
  • Mosquera VX, et al. Blunt traumatic aortic injuries of the ascending aorta and aortic arch: a clinical multicentre study. Injury. 2013;44:1191-7. PMID 23294894 — tags: [trauma, deceleration, blunt-aortic-injury, mortality] — cited by: red-flags-and-safety-concerns.md
  • Patel HJ, et al. Five-year outcomes from the United States pivotal trial of Valiant Captivia stent graft for blunt aortic injury. Ann Thorac Surg. 2020;110:815-820. PMID 31991136 — tags: [trauma, blunt-aortic-injury, TEVAR, RESCUE, outcomes] — cited by: red-flags-and-safety-concerns.md
  • Fang XX, et al. Blunt aortic injury — traumatic aortic isthmus pseudoaneurysm with right iliac artery dissection aneurysm: a case report. World J Clin Cases. 2022;10:4998-5004. PMID 35801016 — tags: [trauma, missed-diagnosis, pseudoaneurysm, case-report] — cited by: red-flags-and-safety-concerns.md
  • Lareyre F, et al. High neutrophil to lymphocyte ratio is associated with symptomatic and ruptured thoracic aortic aneurysm. Angiology. 2018;69:686-691. PMID 29334754 — tags: [symptomatic-aneurysm, rupture, inflammation, biomarker, small-series] — cited by: red-flags-and-safety-concerns.md
  • Agarwal T, et al. Cardiovocal syndrome secondary to thoracic aortic aneurysm: an old sign revisited. Cureus. 2020;12:e10087. PMID 33005510 — tags: [compression-syndrome, hoarseness, dysphagia, ortner, case-report] — cited by: red-flags-and-safety-concerns.md
  • Dueppers P, et al. Long-term results of total endovascular repair of arch-involving aortic pathologies using parallel grafts for supra-aortic debranching. J Vasc Surg. 2022;75:813-823. PMID 34606961 — tags: [endoleak, TEVAR, arch, reintervention, surveillance] — cited by: red-flags-and-safety-concerns.md
  • Lyons OTA, et al. Diagnosis of aortic graft infection: a case definition by the Management of Aortic Graft Infection Collaboration (MAGIC). Eur J Vasc Endovasc Surg. 2016;52:758-763. PMID 27771318 — tags: [graft-infection, MAGIC-criteria, case-definition] — cited by: red-flags-and-safety-concerns.md
  • Anagnostopoulos A, et al. Editor's Choice — Validation of the Management of Aortic Graft Infection Collaboration (MAGIC) criteria for the diagnosis of vascular graft/endograft infection: results from the prospective Vascular Graft Cohort study. Eur J Vasc Endovasc Surg. 2021;62:251-257. PMID 34140225 — tags: [graft-infection, MAGIC-criteria, validation, VASGRA, thoracic-grafts] — cited by: red-flags-and-safety-concerns.md
  • Treasure T, Takkenberg JJM, Pepper J. Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms. Heart. 2014;100:1571-6. PMID 24986892 — tags: [root-replacement, mechanical-valve, anticoagulation, valve-sparing, PEARS] — cited by: red-flags-and-safety-concerns.md [note: a republished duplicate exists as PMID 26811510, Postgrad Med J 2016;92:112-7 — cite the Heart original]
  • (Registry note for guidelines/REGISTRY.md: a third simultaneous publication of the same document exists — J Thorac Cardiovasc Surg. 2023;166:e182-e331, PMID 37389507, verified this session. Same text, third journal; worth recording so it is not mistaken for a separate guideline.)
  • All PMIDs above were confirmed by get_article_metadata or search_articles calls on 2026-08-28.
  • One claim on the wiki page is deliberately marked [unverified]: the commonly quoted rule that a symptomatic thoracic aortic aneurysm warrants intervention regardless of diameter. The 2022 guideline abstract confirms the document is organised by presentation subset (asymptomatic / stable symptomatic / acute aortic syndrome), but the specific class-of-recommendation text was not retrieved this session.
  • Pre-pregnancy diameter thresholds from the 2022 guideline are likewise referenced only at the level verified (emphasis on shared decision making before and during pregnancy); the numeric thresholds should be confirmed during a guidelines.md pass.
  • No indexed primary evaluation of the THINK AORTA campaign was found in PubMed this session; the campaign is cited via Holmes 2021 (PMID 34838743), which names it, and via the patient-voice layer.

Trial readouts & safety — 2026-08-28

  • Lacro RV, Dietz HC, Sleeper LA, et al. Atenolol versus losartan in children and young adults with Marfan's syndrome. N Engl J Med. 2014;371:2061-2071. PMID 25405392 — tags: [marfan, RCT, losartan, atenolol, z-score-surrogate, safety] — cited by: clinical-trials-landscape
  • Milleron O, Arnoult F, Ropers J, et al. Marfan Sartan: a randomized, double-blind, placebo-controlled trial. Eur Heart J. 2015;36:2160-2166. PMID 25935877 — tags: [marfan, RCT, losartan, placebo-controlled, null-result] — cited by: clinical-trials-landscape
  • Nienaber CA, Kische S, Rousseau H, et al. Endovascular repair of type B aortic dissection: long-term results of the randomized investigation of stent grafts in aortic dissection trial. Circ Cardiovasc Interv. 2013;6:407-416. PMID 23922146 — tags: [type-B-dissection, TEVAR, RCT, INSTEAD-XL, 5-year-outcomes, NCT01415804] — cited by: clinical-trials-landscape
  • Baxter BT, Matsumura J, Curci JA, et al. Effect of doxycycline on aneurysm growth among patients with small infrarenal abdominal aortic aneurysms: a randomized clinical trial. JAMA. 2020;323:2029-2038. PMID 32453369 — tags: [AAA, doxycycline, N-TA3CT, RCT, null-result, NCT01756833] — cited by: clinical-trials-landscape
  • Szeto WY, Fukuhara S, Fleischman F, et al. A novel hybrid prosthesis for open repair of acute DeBakey type I dissection with malperfusion: early results from the PERSEVERE trial. J Thorac Cardiovasc Surg. 2025;170:114-123.e3. PMID 39116932 — tags: [AMDS, PERSEVERE, DeBakey-I, 30-day-outcomes, DANE, NCT05174767] — cited by: clinical-trials-landscape
  • Szeto WY, Fukuhara S, Fleischman F, et al. One-year results of novel aortic arch hybrid prosthesis for repair of acute DeBakey type I dissection with malperfusion: PERSEVERE study. Ann Thorac Surg. 2026;121:1069-1079. PMID 41238072 — tags: [AMDS, PERSEVERE, 1-year-outcomes, aortic-remodeling, NCT05174767] — cited by: clinical-trials-landscape
  • Coselli JS, Roselli EE, Preventza O, et al. Total aortic arch replacement using a frozen elephant trunk device: results of a 1-year US multicenter trial. J Thorac Cardiovasc Surg. 2024;167:1680-1692.e2. PMID 36253292 — tags: [frozen-elephant-trunk, Thoraflex, IDE-trial, 1-year-outcomes, NCT02724072] — cited by: clinical-trials-landscape
  • Orozco-Sevilla V, Coselli JS, Green SY, et al. Total aortic arch replacement using the Thoraflex Hybrid device: evolution from investigational to federally approved use in the United States. Ann Cardiothorac Surg. 2025;14:279-290. PMID 40808788 — tags: [Thoraflex, FDA-approval-2022, total-arch-replacement, single-centre-series] — cited by: clinical-trials-landscape
  • Holmes DR, Farb A, Dib N, et al. The medical device development ecosystem: current regulatory state and challenges for future development: a review. Cardiovasc Revasc Med. 2023;60:95-101. PMID 37778922 — tags: [regulatory, early-feasibility-study, FDA, device-development, review] — cited by: clinical-trials-landscape