AKUT AORT SENDROMLARI

AkutAortSendromlariKapak

DOĞAL SEYİR VE PROGNOZ

Hakan Usta1 Hakan Baltacı2

1Hakkari Devlet Hastanesi, Kalp Damar Cerrahisi Kliniği, Hakkari, Türkiye
2Elazığ Fethi Sekin Şehir Hastanesi, Kalp Damar Cerrahisi Kliniği, Elazığ, Türkiye

Usta H, Baltacı H. Doğal Seyir ve Prognoz. Ünlü Y, editör. Akut Aort Sendromları. 1. Baskı. Ankara: Türkiye Klinikleri; 2025. p.131-139.

ÖZET

Akut Aortik Sendrom (AAS) kavramı, aortla ilgili bakış açımızın aortik diseksiyonla kısıtlı olmadığı, daha geniş perspektif sağlayan bir tanımlamadır. AAS bileşenleri olan aort diseksiyonları, intramural hematom (IMH) ve penetran aterosklerotik ülserler (PAU) her biri farklı klinik durumlar olup hastalığın doğal akışı esnasında bir diğerine ilerleyebildiği gibi, daha sessiz bir tablo ile de seyredebilir. Hastalığın seyri ve prognostik özellikleri, ilgili lezyonun aortta yerleştiği segmente, hastalığın görülme yaşına, altta yatan nedenlere ve eşlik eden komorbidetelere bağlı olarak değişmektedir. Medikal/cerrahi tedavilerin etkinlikleri, erken ve geç dönem mortalite ve morbiditeleri birbirinden farklı olmaktadır.

Tutulum yeri temel belirleyici özelliklerden olup Tip A (proksimal) tutulum varlığında özellikle en yüksek erken mortalite riski mevcuttur. Tip B (distal) tutulumlarda ise başlangıç riski daha düşük olmasına rağmen komplikasyonlar (rüptür, organ iskemisi vb.) eşlik ettiği durumda ölümcül olabilir. IMH’larda seyir değişkendir. Kendiliğinden düzelebilir, stabil seyredebilir veya diseksiyona ilerleyebilir. Proksimal yerleşim, büyük hematom kalınlığı, başlangıç aort çapının yüksek oluşu, aort çapında hızlı progresyon görülmesi, eşlik eden plevral efüzyon varlığı kötü prognoza işaret eder. AAS alt gruplarının görüldüğü hasta gruplarında da farklılıklar olabilir. PAU genellikle daha yaşlı ve aterosklerotik risk faktörleri fazla olan hastalarda görülür. Hastalar çoğunlukla asemptomatiktir ve asemptomatik hastalarda seyir daha ılımlıdır. Ülserin derin ve geniş olması, hızlı büyüme, plevral efüzyonun varlığı ve medikal tedaviye dirençli ağrı olması kötü prognozla ilişkilidir.

AAS’de prognoz sadece tanıya bağlı değildir. AAS bileşeninden farklı olarak komplikasyon varlığı (rüptür, organ iskemisi ve şok en önemli ölüm nedenleridir), hasta özellikleri (ileri yaş, hipertansiyon, böbrek yetmezliği, kronik obstrüktif akciğer hastalığı hem cerrahi riski arttırır hem de uzun dönem sağkalımı sınırlar), erken tanı ve tedaviye ulaşma süresi, merkezin deneyimi (yüksek volümlü aort merkezlerinde yapılan girişimlerde sonuçlar belirgin olarak daha iyidir) prognozu doğrudan etkilemektedir. Tedavi hastalık ve hasta özelliklerine bağlı olarak cerrahi tedaviler, endovasküler onarımlar ve medikal yaklaşımlar olabilmektedir. Endovasküler yaklaşımlarda erken dönem mortalite ve morbidite, cerrahiye kıyasla daha düşük olmasına rağmen tekrar girişim ihtiyaçları olabilmektedir. Medikal tedavilerde ise uzun vadede hastaların önemli bir kısmında aortta genişleme devam edebilir ve cerrahi tedavi, endovasküler onarım gibi ileri tedavilere gereksinim duyulabilir.

Anahtar Kelimeler: Akut aortik sendrom; Aort diseksiyonu; Aortik intramural hematom; Penetran aterosklerotik ülser

Referanslar

  1. Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U, et al. Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. Circulation. 2018;137(17):1846-1860. [Crossref]  [PubMed]
  2. Filippone G, Caruana G, Calia C, Moscaritolo V, Argano V. Evidence of intimal tear in type A intramural hematoma of the aorta: A case series. International Journal of Surgery Case Reports. 2017;42:179.-181 IJSCR.2017.12.018 [Crossref]
  3. Hamilton M. Pathophysiology of Aortic Dissection and Connective Tissue Disorders. Mechanisms of Vascular Disease: A Reference Book for Vascular Specialists. 2011:255-276. [Crossref]  [PubMed]
  4. Levy D, Sharma S, Grigorova Y, Farci F, Le JK. Aortic Dissection. Peripheral Arterial Disease Handboo. 2024:283-297. [Crossref]
  5. Fukui T. Management of acute aortic dissection and thoracic aortic rupture. Journal of Intensive Care. 2018;6(1). [Crossref]  [PubMed]  [PMC]
  6. Erbel R, Aboyans V, Boileau C, Bossone E, Di Bartolomeo R, Eggebrecht H, et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). European Heart Journal. 2014;35(41):28732926. [Crossref]  [PubMed]
  7. Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE, 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: Executive summary: A report of the american college of cardiology foundation/american heart association task force on practice guidelines, american association for thoracic surgery, american college of radiology, american stroke association. Circulation. 2010;121(13):266-369. [Crossref]  [PubMed]
  8. Norton EL, Khaja MS, Williams DM, Yang B. Type A Aortic Dissection Complicated by Malperfusion Syndrome. Current Opinion in Cardiology. 2019;34(6):610. [Crossref]  [PubMed]
  9. De Freitas S, Rossi MJ, Abramowitz SD, Fatima J, Kiguchi MM, Vallabhaneni R, et al. Systematic review and meta-analysis of endovascular interventions for Stanford type A aortic dissection. Journal of Vascular Surgery. 2021:74(5);17211731.e4. [Crossref]  [PubMed]
  10. Williams DM, Lee DY, Hamilton BH, Marx MV, Narasimham DL, Kazanjian SN, et al. The dissected aorta: percutaneous treatment of ischemic complications--principles and results. Journal of Vascular and Interventional Radiology : JVIR. 1997;8(4):605-625. [Crossref]  [PubMed]
  11. Tsagakis K, Jánosi RA, Frey UH, Schlosser T, Chiesa R, Rassaf T, Jakob H. True Lumen Stabilization to Overcome Malperfusion in Acute Type I Aortic Dissection. Semin Thorac Cardiovasc Surg. 2019;31(4):740-748. [Crossref]  [PubMed]
  12. Goel NJ, Kelly JJ, Patrick WL, Zhao Y, Bavaria JE, Ouzounian M, et al. Malperfusion in Patients With Acute Type A Aortic Dissection: A Nationwide Analysis. Annals of Thoracic Surgery. 2025;119(5):980-989. [Crossref]  [PubMed]
  13. Zindovic I, Gudbjartsson T, Ahlsson A, Fuglsang S, Gunn J, Hansson EC, et al. Malperfusion in acute type A aortic dissection: An update from the Nordic Consortium for Acute Type A Aortic Dissection. Journal of Thoracic and Cardiovascular Surgery. 2019;157(4):1324-1333.e6. [Crossref]  [PubMed]
  14. Pacini D, Leone A, Belotti LM, Fortuna D, Gabbieri D, Zussa C, Contini A, et al. Acute type A aortic dissection: significance of multiorgan malperfusion. Eur J Cardiothorac Surg. 2013;43(4):820-6. [Crossref]  [PubMed]
  15. Czerny M, Schoenhoff F, Etz C, Englberger L, Khaladj N, Zierer A, et al. The Impact of Pre-Operative Malperfusion on Outcome in Acute Type AAortic Dissection: Results From the GERAADA Registry. J Am Coll Cardiol. 2015;65(24):26282635. [Crossref]  [PubMed]
  16. Hirst AE Jr, Johns VJ Jr, Kime SW Jr. Dissecting aneurysm of the aorta: a review of 505 cases. Medicine (Baltimore). 1958;37(3):217-79. [Crossref]  [PubMed]
  17. Kreibich M, Bavaria JE, Branchetti E, Brown CR, Chen Z, Khurshan F, et al. Management of Patients With Coronary Artery Malperfusion Secondary to Type A Aortic Dissection. Ann Thorac Surg. 2019;107(4):1174-1180. [Crossref]  [PubMed]
  18. Imoto K, Uchida K, Karube N, Yasutsune T, Cho T, Kimura K, et al. Risk analysis and improvement of strategies in patients who have acute type A aortic dissection with coronary artery dissection. Eur J Cardiothorac Surg. 2013;44(3):419-24. [Crossref]  [PubMed]
  19. Czerny M, Grabenwöger M, Berger T, Aboyans V, Della Corte A, Chen EP, et al. EACTS/STS Scientific Document Group. EACTS/STS Guidelines for diagnosing and treating acute and chronic syndromes of the aortic organ. Eur J Cardiothorac Surg. 2024;65(2):ezad426. [Crossref]  [PubMed]
  20. Bossone E, Corteville DC, Harris KM, Suzuki T, Fattori R, Hutchison S, et al. Stroke and outcomes in patients with acute type A aortic dissection. Circulation. 2013;128(11 Suppl 1):S175-9. [Crossref]  [PubMed]
  21. Di Eusanio M, Patel HJ, Nienaber CA, Montgomery DM, Korach A, Sundt TM, et al. Patients with type A acute aortic dissection presenting with major brain injury: Should we operate on them? Journal of Thoracic and Cardiovascular Surgery, 2023;145(3 SUPPL.): S213-S221.e1. [Crossref]  [PubMed]
  22. He, K., Qin, X., Li, M., Bian, L., Yue, H., Liang, W., & Wu, Z. (2024). Two surgical strategies (early carotid reperfusion vs. Central aortic repair-first) of acute type a aortic dissection complicated with cerebral malperfusion syndrome: a meta-analysis and systematic review. BMC Cardiovascular Disorders, 24(1), 1-11.
  23. Tsagakis K, Konorza T, Dohle DS, Kottenberg E, Buck T, Thielmann M, et al. Hybrid operating room concept for combined diagnostics, intervention and surgery in acute type A dissection. Eur J Cardiothorac Surg. 2013;43(2):397-404. [Crossref]  [PubMed]
  24. Geirsson A, Ahlsson A, Franco-Cereceda A, Fuglsang S, Gunn J, Hansson EC, et al. Hospital volumes and later year of operation correlates with better outcomes in acute Type A aortic dissection. Eur J Cardiothorac Surg. 2018;53(1):276-281. [Crossref]
  25. Vallabhajosyula P, Gottret JP, Menon R, Sultan I, Abbas Z, Siki M, et al. Central Repair With Antegrade TEVAR for Malperfusion Syndromes in Acute Debakey I Aortic Dissection. Ann Thorac Surg. 2017;103(3):748-755. [Crossref]  [PubMed]
  26. Di Eusanio M, Trimarchi S, Patel HJ, Hutchison S, Suzuki T, Peterson MD, 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(2):385-390.e1. [Crossref]  [PubMed]
  27. Wolfe SB, Sundt TM 3rd, Isselbacher EM, Cameron DE, Trimarchi S, Bekeredjian R, et al. Survival after operative repair of acute type A aortic dissection varies according to the presence and type of preoperative malperfusion. J Thorac Cardiovasc Surg. 2024;168(1):37-49.e6. [Crossref]  [PubMed]
  28. Yang B, Norton EL, Rosati CM, Wu X, Kim KM, Khaja MS, et al. Managing patients with acute type A aortic dissection and mesenteric malperfusion syndrome: A 20-year experience. J Thorac Cardiovasc Surg. 2019;158(3):675-687.e4 [Crossref]
  29. Patel HJ, Williams DM, Dasika NL, Suzuki Y, Deeb GM. Operative delay for peripheral malperfusion syndrome in acute type A aortic dissection: a long-term analysis. J Thorac Cardiovasc Surg. 2008;135(6):1288-95. [Crossref]  [PubMed]
  30. Deeb GM, Williams DM, Boiling SF, Quint LE, Monaghan H, Sievers J, et al. Surgical delay for acute type A dissection with malperfusion. Annals of Thoracic Surgery, 1997;64(6):1669-1677. [Crossref]  [PubMed]
  31. Gilon D, Korach A, Carbone A, Evangelista A, Brinster DR, Estrera AL, et al. Cardiac Tamponade Complicating Type A Acute Aortic Dissection: Insights From 25 Years of Registry Research. JACC: Advances. 2025;4(4):101632. [Crossref]  [PubMed]  [PMC]
  32. Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL, et al. The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. JAMA. 2000;283(7):897-903. [Crossref]  [PubMed]
  33. Howard DP, Banerjee A, Fairhead JF, Perkins J, Silver LE, Rothwell PM, 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(20):2031-7. [Crossref]  [PubMed]
  34. Harris KM, Nienaber CA, Peterson MD, Woznicki EM, Braverman AC, Trimarchi S, et al. Early Mortality in Type A Acute Aortic Dissection: Insights From the International Registry of Acute Aortic Dissection. JAMA Cardiol. 2022;7(10):1009-1015. [Crossref]  [PubMed]  [PMC]
  35. Geirsson A, Shioda K, Olsson C, Ahlsson A, Gunn J, Hansson EC, et al. Differential outcomes of open and clamp-on distal anastomosis techniques in acute type A aortic dissection. J Thorac Cardiovasc Surg. 2019;157(5):1750-1758. [Crossref]  [PubMed]
  36. Bossone E, Eagle KA. Epidemiology and management of aortic disease: aortic aneurysms and acute aortic syndromes. Nat Rev Cardiol. 2021;18(5):331-348. [Crossref]  [PubMed]
  37. Weiss G, Wolner I, Folkmann S, Sodeck G, Schmidli J, Grabenwöger M, et al. The location of the primary entry tear in acute type B aortic dissection affects early outcome. Eur J Cardiothorac Surg. 2012;42(3):571-6. [Crossref]  [PubMed]
  38. Trimarchi S, Jonker FH, van Bogerijen GH, Tolenaar JL, Moll FL, Czerny M, et al. Predicting aortic enlargement in type B aortic dissection. Ann Cardiothorac Surg. 2014;3(3):285-91. [Crossref]  [PubMed]
  39. Evangelista A, Salas A, Ribera A, Ferreira-González I, Cuellar H, Pineda V, et al. Long-term outcome of aortic dissection with patent false lumen: predictive role of entry tear size and location. Circulation. 2012;125(25):3133-41. [Crossref]  [PubMed]
  40. Codner JA, Lou X, Duwayri YM, Chen EP, Binongo JN, Moon R, et al. The distance of the primary intimal tear from the left subclavian artery predicts aortic growth in uncomplicated type B aortic dissection. J Vasc Surg. 2019;69(3):692700. [Crossref]  [PubMed]  [PMC]
  41. Evangelista A, Pineda V, Guala A, Bijnens B, Cuellar H, Rudenick P, et al. False Lumen Flow Assessment by Magnetic Resonance Imaging and Long-Term Outcomes in Uncomplicated Aortic Dissection. J Am Coll Cardiol. 2022;79(24):2415-2427. [Crossref]  [PubMed]
  42. Ante M, Mylonas S, Skrypnik D, Bischoff MS, Rengier F, Brunkwall J, et al. Prevalence of the Computed Tomographic Morphological DISSECT Predictors in Uncomplicated Stanford Type B Aortic Dissection. Eur J Vasc Endovasc Surg. 2018;56(4):525-533. [Crossref]  [PubMed]
  43. Song JM, Kim SD, Kim JH, Kim MJ, Kang DH, Seo JB, et al. Long-term predictors of descending aorta aneurysmal change in patients with aortic dissection. J Am Coll Cardiol. 2007;50(8):799-804. [Crossref]  [PubMed]
  44. Weissler EH, Osazuwa-Peters OL, Greiner MA, Hardy N C, Kougias P, O’Brien SM, et al. Initial Thoracic Endovascular Aortic Repair vs Medical Therapy for Acute Uncomplicated Type B Aortic Dissection. JAMA Cardiology, 2022;8(1):44. [Crossref]  [PubMed]  [PMC]
  45. Pape LA, Awais M, Woznicki EM, Suzuki T, Trimarchi S, Evangelista A, et al. Presentation, Diagnosis, and Outcomes of Acute Aortic Dissection: 17-Year Trends From the International Registry of Acute Aortic Dissection. Journal of the American College of Cardiology. 2015;66(4):350-358. [Crossref]  [PubMed]
  46. Fattori R, Tsai TT, Myrmel T, Evangelista A, Cooper JV, Trimarchi S, et al. Complicated acute type B dissection: is surgery still the best option?: a report from the International Registry of Acute Aortic Dissection. JACC Cardiovasc Interv. 2008;1(4):395-402. [Crossref]  [PubMed]
  47. Tsai TT, Isselbacher EM, Trimarchi S, Bossone E, Pape L, Januzzi JL, et al. Acute type B aortic dissection: Does aortic arch involvement affect management and outcomes? Insights from the International Registry of Acute Aortic Dissection (IRAD). Circulation,. 2007;116(11 SUPPL. 1). [Crossref]  [PubMed]
  48. Nauta FJ, Tolenaar JL, Patel HJ, Appoo JJ, Tsai TT, Desai ND, et al. Impact of Retrograde Arch Extension in Acute Type B Aortic Dissection on Management and Outcomes. Ann Thorac Surg. 2016;102(6):2036-2043 org/ [Crossref]
  49. Yen HT, Wu CC, Lee YW, Lo CM, Chen YY. CT characteristics and clinical implications of acute type A aortic intramural hematoma. Frontiers in Cardiovascular Medicine. 2023;9:1041796. [Crossref]  [PubMed]  [PMC]
  50. Estrera A, Miller C, Lee TY, De Rango P, Abdullah S, Walkes JC, et al. Acute type A intramural hematoma analysis of current management strategy. Circulation. 2009;120(SUPPL. 1). [Crossref]  [PubMed]
  51. Chou AS, Ziganshin BA, Charilaou P, Tranquilli M, Rizzo JA, Elefteriades JA. Long-term behavior of aortic intramural hematomas and penetrating ulcers. J Thorac Cardiovasc Surg. 2016;151(2):361-72, 373.e1. [Crossref]  [PubMed]
  52. Sadamatsu K, Takase S, Sagara S, Ohe K, Nishi JI, Tashiro H, et al. Initial medical management in acute type A aortic dissection patients with a thrombosed false lumen in the ascending aorta combining intramural hematoma and retrograde dissection from the descending to the ascending aorta. Eur Heart J Acute Cardiovasc Care. 2020;9(3_suppl):S13-S20. [Crossref]  [PubMed]
  53. Wee I, Varughese RS, Syn N, Choong AMTL. Non-operative Management of Type A Acute Aortic Syndromes: A Systematic Review and Meta-Analysis. European Journal of Vascular and Endovascular Surgery. 2019;58(1):41-51. [Crossref]  [PubMed]
  54. Brinster DR. Endovascular Repair of the Descending Thoracic Aorta for Penetrating Atherosclerotic Ulcer Disease. Journal of Cardiac Surgery. 2009;24(2):203-208. [Crossref]  [PubMed]
  55. Hirst AE Jr, Barbour BH. Dissecting aneurysm with hemopericardium; report of a case with healing. N Engl J Med. 1958;258(3):116-20. [Crossref]  [PubMed]
  56. Cho KR, Stanson AW, Potter DD, Cherry KJ, Schaff HV, Sundt TM, et al. Penetrating atherosclerotic ulcer of the descending thoracic aorta and arch. Journal of Thoracic and Cardiovascular Surgery. 2004;127(5):1393-1401. [Crossref]  [PubMed]