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Acute Aortic Syndrome (AAS): A High-Risk Missed Diagnosis in the Emergency Department

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Abstract

Acute aortic syndrome (AAS) is a pathological condition including aortic dissection (AD), intramural hematoma (IMH), and penetrating aortic ulcer (PAU).

These three pathologies have similar clinical presentations, with chest pain as the main symptom, and they are associated to an high risk of aortic rupture, with an high morbidity and mortality.

The incidence of AAS is relatively low (2.6–3.5 cases/100,000/year), but their higher mortality and their frequent misdiagnosis or delay in diagnosis make necessary to know and to apply in the emergency department specific diagnostic and surgical algorithms. CT scan is the exam of choice in emergency to reach the diagnosis and to choose the appropriated treatment for patients, according to his peculiar clinical, anatomical, and radiological features.

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Notes

  1. 1.

    Patients with congenital disease can show the onset of AAS symptoms at very praecox age: in Loeys–Dietz syndrome, the middle age for the first aortic dissection is 26, in Ehlers–Danlos, the first vascular signs can be found at an average of 23. Other congenital diseases (such Turner or Marfan syndrome) show the first pathologic signs in aorta lately, but always decades before people with atheromasic etiology of disease [7].

  2. 2.

    Chest trauma with rapid deceleration, typical of motor vehicle accidents, can produce aortic injury or even complete rupture. The aortic isthmus is generally involved because here ascending aorta and arch become relatively fixed to the thoracic cage [6, 8].

  3. 3.

    “Classical ADs”: aortic dissection not associated to connective congenital degenerative disease.

  4. 4.

    Park et al. found that 73% of patients treated in open surgery for an IMH in the proximal aorta and diagnosed by CT scan were affected by ADs, with a small entry tear that spontaneously had sealed [12, 19].

  5. 5.

    It is necessary to distinguish between blood pools and Ulcers Like Projection (ULP). The former has a very narrow intimal orifice, usually communicate with a lumbar or intercostal artery, and have no communication with the true lumen; the latter appear at CT scan as localized contrast filled pouch that communicates with the true lumen [20].

  6. 6.

    International Registry of Acute Aortic Dissections.

  7. 7.

    There is no strong evidence suggesting the best blood pressure target for dissections or IMH, but most guidelines propose a SBP target of 100–120 mmHg.

  8. 8.

    Flow mainly perpendicular to the direction of bulk flow in the descending thoracic aorta.

  9. 9.

    dP/dT: rate of rise of left ventricular pressure; dP/dt(max): is the maximal rate of rise of (usually) left ventricular pressure (LVP), but it is determined by myocardial contractility and the loading conditions on the ventricle, thus it is an imperfect and sometimes incorrect predictor of the inotropic state.

References

  1. Clough RE, Nienaber CA. Management of acute aortic syndrome. Nat Rev Cardiol. 2015;12:103–14.

    Article  CAS  PubMed  Google Scholar 

  2. Duran ES, Ahmad F, Elshikh M, Masood I, Duran C. Computed tomography imaging findings of acute aortic pathologies. Cureus. 2019;11(8):e5534.

    PubMed  PubMed Central  Google Scholar 

  3. Pereira AH. Intramural hematoma and penetrating atherosclerotic ulcers of the aorta: uncertainties and controversies. J Vasc Bras. 2019;18:e20180119.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Dudznski DM, Isselbacher EM. Diagnosis and management of thoracic aortic disease. Curr Cardiol Rep. 2015;17:106.

    Article  Google Scholar 

  5. Ridge CA, Litmanovich DE. Acute aortic syndromes current status. J Thorac Imaging. 2015;30:193–201.

    Article  PubMed  Google Scholar 

  6. Baliga R, Nienaber CA, Bossone E, Oh JK, IsselbacherE M, Sechtem U, Fattori R, Raman SV, Eagle KA. The role of imaging in aortic dissection and related syndromes. JACC Cardiovasc Imaging. 2014;7:406–24.

    Article  PubMed  Google Scholar 

  7. Riambau V, Böckler D, Brunkwall J, Cao P, Chiesa R, Coppi G, et al. Editor’s choice—management of descending thoracic aorta diseases, clinical practice guidelines of the European Society for Vascular Surgery (ESVS). Eur J Vasc Endovasc Surg. 2017;53:4e52.

    Article  Google Scholar 

  8. Bonaca MP, O'Gara PT. Diagnosis and management of acute aortic syndromes: dissection, intramural hematoma, and penetrating aortic ulcer. Curr Cardiol Rep. 2014;16:536.

    Article  PubMed  Google Scholar 

  9. Corvera JS. Acute aortic syndrome. Ann Cardiothoracic Surg. 2016;5:188–93.

    Article  Google Scholar 

  10. Bruce MC, Honaker CE. Transcriptional regulation of tropoelastin expression in rat lung fibroblasts: changes with age and hyperoxia. Am J Phys. 1998;274(6):L 940-50.

    Google Scholar 

  11. Fritze O, Romero B, Schleicher M, Jacob MP, Oh D, Starcher B. Age-related changes in the elastic tissue of the human aorta. J Vasc Res. 2012;49(1):7786.

    Article  Google Scholar 

  12. Howard DPJ, Banerjee A, Fairhead JF, Perkins J, Silver LE, Rothwell PM. 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.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Clift P, Cervi E. A review of thoracic aortic aneurysm disease. Ecoresearch Practice. 2020;7(1):R1–R10.

    Google Scholar 

  14. Salmasi MY, Al-Saadi N, Hartley P, Jarral OA, Raja S, Hussein M. The risk of misdiagnosis in acute thoracic aortic dissection: a review of current guidelines. Heart. 2020;106:885–91.

    Article  PubMed  Google Scholar 

  15. Booher AM, Isselbacher EM, Nienaber CA, Trimarchi S, Evangelista A, Montgomery DG, et al. The IRAD classification system for characterizing survival after aortic dissection. Am J Med. 2013;126(730):e19–24.

    Google Scholar 

  16. Marshall LM, Carlson EJ, O’Malley J, Snyder CK, Charbonneau NL, Hayflick SJ, et al. Thoracic aortic aneurysm frequency and dissection are associated with fibrillin-1 fragment concentrations in circulation. Circ Res. 2013;113:1159–68.

    Article  CAS  PubMed  Google Scholar 

  17. Bossone E, Evangelista A, Isselbacher E, Trimarchi S, Hutchinson S, Gilon D, et al. Prognostic role of transesophageal echocardiography in acute type A aortic dissection. Am Heart J. 2007;153:1013–20.

    Article  PubMed  Google Scholar 

  18. Bosma MS, Quint LE. Ulcerlike projections developing in non communicating aortic dissections: CT findings and natural history. AJR Am J Roentgenol. 2009;193(3):895–905.

    Article  PubMed  Google Scholar 

  19. Park KH, Lim C, Choi JH, Sung K, Kim K, Lee YT, et al. Prevalence of aortic intimal defect in surgically treated acute type A intramural hematoma. Ann Thorac Surg. 2008;86:1494–500.

    Article  PubMed  Google Scholar 

  20. Abbas A, Brown IW, Peebles CR, Harden SP, Shambrook JS. The role of multidetector-row CT in the diagnosis, classification and management of acute aortic syndrome. Br J Radiol. 2014;87

    Google Scholar 

  21. Nienaber CA, Von Kodolitsch Y, Petersen B, Loose R, Helmchen U, Haverich A, et al. Intramural hemorrage of the thoracic aorta. Diagnostic and therapeutic implications. Circulation. 1995;92:1465–72.

    Google Scholar 

  22. Song JK, Yim JH, Ahn JM, Kim DH, Kang JW, Lee TY, et al. Outcomes of patients with acute type a aortic intramural hematoma. Circulation. 2009;120:2046–52.

    Article  PubMed  Google Scholar 

  23. Kitai T, Kaji S, Yamamuro A, Tani T, Tamita K, Kinoshita M, et al. Clinical outcomes of medical therapy and timely operation in initially diagnosed type a aortic intramural hematoma: a 20-year experience. Circulation. 2009;120:S292–8.

    Article  PubMed  Google Scholar 

  24. Robbins RC, McManus RP, Mitchell RS, Latter DR, Moon MR, Olinger GN, et al. Management of patients with intramural hematoma of the thoracic aorta. Circulation. 1993;88:1–10.

    Google Scholar 

  25. Tittle SL, Lynch RJ, Cole PE, Singh HS, Rizzo JA, Kopf GS, et al. Midterm follow-up of penetrating ulcer and intramural hematoma of the aorta. J Thorac Cardiovasc Surg. 2002;123:1051–9.

    Article  PubMed  Google Scholar 

  26. Song JK, Kim HS, Song JM, Kang DH, Ha JW, Rim SJ, et al. Outcomes of medically treated patients with aortic intramural hematoma. Am J Med. 2002;113:181187.

    Article  Google Scholar 

  27. Choi YJ, Son JW, Lee SH, Kim U, Shin DG, Kim YJ, et al. Treatment patterns and their outcomes of acute aortic intramural hematoma in real world: multicenter registry for aortic intramural hematoma. BMC Cardiovasc Disord. 2014;14:103. https://doi.org/10.1186/1471-2261-14-103.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Asha SE, Miers JW. A systematic review and meta-analysis of d-dimer as a rule-out test for suspected aortic dissection. Ann Emerg Med. 2015;66:368–78.

    Google Scholar 

  29. Rybicki FJ, Udelson JE, Peacock WF, Goldhaber SZ, Isselbacher EM, Kazerooni E, et al. 2015 ACR/ACC/AHA/AATS/ACEP/ASNC/NASCI/SAEM/SCCT/SCMR/SCPC/SNMMI/STR/STS appropriate utilization of cardiovascular imaging in emergency department patients with chest pain. J Am Coll Cardiol. 2016;67:853–79.

    Article  PubMed  Google Scholar 

  30. Baliga R, Nienaber CA, Bossone E, Oh JK, Isselbacher EM, Sechtem U, et al. Role of imaging in aortic dissection. JACC Cardiovasc Imaging. 2014;7:406–24.

    Article  PubMed  Google Scholar 

  31. Evangelista A, Mukherjee D, Mehta RH, O'Gara PT, Fattori R, Cooper JV, et al. Acute intramural hematoma of the aorta: a mistery in evolution. Circulation. 2005;111:1063–70.

    Article  PubMed  Google Scholar 

  32. Bossone E, Czerny C, Lerakis S, Rodriguez-Palomares J, Kukar N, Ranieri B, et al. Imaging and biomarkers in acute aortic syndromes: diagnostic and prognostic implications. Curr Probl Cardiol. 2020:100654.

    Google Scholar 

  33. Boodhwani M, . Andelfinger G, Leipsic J, Lindsay T, McMurtry M S, Therrien J et al., Canadian Cardiovascular Society position statement on the management of thoracic aortic disease. Can J Cardiol, 2014; 30: 577-589.

    Article  PubMed  Google Scholar 

  34. Nienaber CA, Kische S, Rousseau H, Eggebrecht H, Rehders TC, Kundt G, 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.

    Article  CAS  PubMed  Google Scholar 

  35. Afifi RO, Sandhu HK, Leake S, Rice RD, Azzizadeh A, Charlton-Ouw KM, et al. Determinants of operative mortality in patients with ruptured acute type A aortic dissection. Ann Thorac Surg. 2016;101:64–71.

    Google Scholar 

  36. Nienaber CA. The art of stratifying patients with type B aortic dissection. J Am Coll Cardiol. 2016:2843–5.

    Google Scholar 

  37. Suzuki T, Mehta RH, Ince H, Nagai R, Sakomura Y, Weber F, et al. Clinical profiles and outcomes of acute type B aortic dissection in the current era: lessons from the International Registry of Aortic Dissection (IRAD). Circulation. 2003;108:312–7.

    Article  Google Scholar 

  38. Kamman AV, Brunkwall J, Verhoeven EL, Heijmen RH, Trimarchi S. Predictors of aortic growth in uncomplicated type B aortic dissection from the acute dissection stent grafting or best medical treatment (ADSORB) database. J Vasc Surg. 2017;65:964–71.

    Article  PubMed  Google Scholar 

  39. Trimarchi S, Eagle KA, Nienaber CA, Pyeritz RE, Jonker FHW, Suzuki T, et al. Importance of refractory pain and hypertension in acute type B aortic dissection: insights from the International Registry of Acute Aortic Dissection (IRAD). Circulation. 2010;122:1283–9.

    Article  PubMed  Google Scholar 

  40. Kan CB, Chang RY, Chang JP. Optimal initial treatment and clinical outcome of type A aortic intramural hematoma: a clinical review. Eur J Cardiothorac Surg. 2008;33:1002–6.

    Article  PubMed  Google Scholar 

  41. Ho H, Cheung CW, Jim MH, Miu KM, Siu CW, Lam YM, et al. Type A aortic intramural hematoma: clinical features and outcomes in Chinese patients. Clin Cardiol. 2011;34:E1–5.

    Article  PubMed  PubMed Central  Google Scholar 

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Tracanelli, P., Aseni, P. (2023). Acute Aortic Syndrome (AAS): A High-Risk Missed Diagnosis in the Emergency Department. In: Aseni, P., Grande, A.M., Leppäniemi, A., Chiara, O. (eds) The High-risk Surgical Patient. Springer, Cham. https://doi.org/10.1007/978-3-031-17273-1_19

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  • DOI: https://doi.org/10.1007/978-3-031-17273-1_19

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