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.
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.
- 3.
“Classical ADs”: aortic dissection not associated to connective congenital degenerative disease.
- 4.
- 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.
International Registry of Acute Aortic Dissections.
- 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.
Flow mainly perpendicular to the direction of bulk flow in the descending thoracic aorta.
- 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
Clough RE, Nienaber CA. Management of acute aortic syndrome. Nat Rev Cardiol. 2015;12:103–14.
Duran ES, Ahmad F, Elshikh M, Masood I, Duran C. Computed tomography imaging findings of acute aortic pathologies. Cureus. 2019;11(8):e5534.
Pereira AH. Intramural hematoma and penetrating atherosclerotic ulcers of the aorta: uncertainties and controversies. J Vasc Bras. 2019;18:e20180119.
Dudznski DM, Isselbacher EM. Diagnosis and management of thoracic aortic disease. Curr Cardiol Rep. 2015;17:106.
Ridge CA, Litmanovich DE. Acute aortic syndromes current status. J Thorac Imaging. 2015;30:193–201.
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.
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.
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.
Corvera JS. Acute aortic syndrome. Ann Cardiothoracic Surg. 2016;5:188–93.
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.
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.
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.
Clift P, Cervi E. A review of thoracic aortic aneurysm disease. Ecoresearch Practice. 2020;7(1):R1–R10.
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.
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.
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.
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.
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.
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.
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
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Nienaber CA. The art of stratifying patients with type B aortic dissection. J Am Coll Cardiol. 2016:2843–5.
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.
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.
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.
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.
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.
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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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