4. Nienaber CA, Powell J. Management of Acute Aortic Syndromes. European Heart Journal 2012; 33:26-35

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1 The Acute aortic syndrome what do we know and what should we know? Kim Houlind Dept of Vascular Surgery, Kolding Hospital Institute of Regional Health Research, University of Southern Denmark Aortic dissection (AD)was first described in Treatment options have been medical and surgical and are guided by the Stanford and De Bakey classifications based on the location of intimal tears and false lumens. More recently, endovascular treatment options have been suggested. Penetrating aortic ulcers (PAU) were first described in 1934 as a focal, atherosclerotic plaque that corrodes a variable depth through the internal elastic lamina into the media. It is predominantly found in elderly men, closely related to atherosclerosis, and most often located in the descending thoracic aorta. Intramural hematomas (IMH) are often found on CT-scans in patients with typical aortic pain. They are characterized by the presence of a hematoma in the media, but the absence of flow in the false lumen and the absence of a primary intimal tear. An intimal tear may, however, occur secondary to the IMH, complicating the distinction between an IMH and a thrombosed false lumen of an AD. In 2000 Shimizu et al(1) published a series of 96 patients admitted on the diagnosis Aortic dissection. On CT evaluation 51 of these turned out to have an IMH without an intimal tear. Interestingly, this subgroup of patients had a much better survival rate than the patients with true AD. This observation prompted Vilacosta in 2001 to introduce the term Acute Aortic Syndrome (AAS) (2), including AD, PAU, and IMH. While later observations from Asian populations confirmed the finding of IMH as a relatively common and benign condition, this does not seem to be the case in Western populations, where the incidence is relatively lower and the prognosis closer to that of patients with AD (3). In a recent review, Nienaber and Powell added the clinical entities of leaking thoracic aneurysm and traumatic dissection or rupture to the definition of AAS (4). The concept of AAS may have a justification by reminding clinicians that not all patients presenting with aortic pain have AD. It should, however, not lead us to believe that a treatment scheme that is based on evidence from treating one subgroup of patients with AAS is necessarily applicable to other subgroups. 1. Shimizu H, Yoshino H, Udagawa H, et al. Prognosis of Aortic Intramural Hemorrage compared With Classic Aortic Dissection. Am J Cardiol 2000;85: Vilacosta I, San Román JA. Acute Aortic Syndrome. Heart 2001;85: Pelzel JM, Braverman AC, Hirsch AT, et al. International heterogeneity in diagnostic frequency and clinical outcomes of ascending aortic intramural hematoma. J Am Soc Echocardiogr 2007;20:1260-8

2 4. Nienaber CA, Powell J. Management of Acute Aortic Syndromes. European Heart Journal 2012; 33:26-35

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4 Tales From the Other Side How the aorta can be treated medically Niels Holmark Andersen Medicinsk behandling af aortadilatation har været omdiskuteret i årevis, og der har været håb om speciel gevinst, ved målrettet behandling, til særligt udsatte patienter såsom Marfan Syndrom og Turner Syndrom. Desværre har resultaterne været ret tvivlsomme. Losartan var oprindelig udnævnt som præparatet, der ville løse problemet med aortadilatation ved Marfan syndrom, men desværre var to større undersøgelser fra aldeles neutrale (1, 2). I større befolkningsundersøgelser, er aortarodsdilatation associeret med højt diastolisk blodtryk, alder, rygning, overvægt og mandligt køn (3). Tilgangen til patienten med aortarodsdilatation vil derfor stadig være blodtryksreduktion, rygeophør og vægttab, samt tæt kontrol og forebyggende behandling i god tid. I lyset af den beskedne effekt af Losartan, synes betablokker-behandling at genvinde status som det foretrukne medicinske præparat, men evidensen er beskeden. Vidunderpræparatet lader således endnu vente på sig. 1) Milleron O. et al. Marfan Sartan: a randomized, double-blind, placebocontrolled trial. Eur Heart J Aug 21;36(32): ) Lacro RV et. al. Atenolol versus losartan in children and young adults with Marfan's syndrome. N Engl J Med Nov 27;371(22): ) de Simone G et al. Hemodynamic Correlates of Abnormal Aortic Root Dimension in an Adult Population: The Strong Heart Study. J Am Heart Assoc Sep 28;4(10)

5 Genetic aorta tales - the genetics of aortic disease? Kristian A Groth, MD, Kardiologisk afdeling AUH. Aortadilatation i proksimale aorta er ofte betinget af en genetisk årsag 1. Genetisk betinget aortadilatation ses specielt i den yngre patient population. De genetiske årsager der betinger aortadilatation er mange facettet og vores viden om genetikken bag aortadilatation udvikles enormt hurtigt i disse år 2, Caglayan AO, Dundar M. Inherited diseases and syndromes leading to aortic aneurysms and dissections. Eur J Cardiothorac Surg. 2009;35(6): Pyeritz RE. Heritable thoracic aortic disorders. Curr Opin Cardiol. 2014;29(1): Jondeau G, Boileau C. Genetics of Thoracic Aortic Aneurysms. Curr Atheroscler Rep

6 Limitations and pitfalls in treating the world s greatest tissue with a stent Kevin Mani, Associate professor, Department of surgical sciences, Uppsala University, Sweden Treatment of aortic disease has been transformed over the past decades, with introduction of endovascular techniques. Endovascular aortic repair (EVAR) is currently the most utilised technique in the western world for treatment of pathologies in the abdominal aorta, as well as for acute aortic syndromes in the descending aorta 1, 2. Despite this development, challenges remain which sometimes prohibit stentgraft treatment of aortic disease, or affect the long-term outcome of endovascular treatment negatively. These challenges can be categorised into three areas: difficulties and complications related to iliac access, aortic anatomy and disease pathology. Iliac access remains a limitation for endovascular treatment of aortic pathology in 30-50% of the patients 3. Thanks to device development, the access size required for stentgraft insertion has been reduced significantly over the past years with introduction of low profile devices, specially in the infrarenal EVAR segment. Iliac access however still remains an issue in treatment of thoracic aortic disease, and complex endovascular aortic repair, when iliac vessel diameter of >7-8mm is frequently required. In addition, fenestrated stentgraft insertion often requires bilateral large sheet access. Compared to infrarenal aortic disease, thoracic and thoracoabdominal repair is more often present in a female smoker population with atherosclerotic disease, making access a significant issue in this patient cohort. Ongoing development, with introduction of complex stentgrafts on low profile platform and pre-loaded devices, will hopefully reduce this problem with time. Aortic anatomy poses another challenge. Tortuosity and angulation in the diseased aorta can make stentgraft insertion difficult, and may threaten the efficacy of endovascular treatment of aortic disease. In pathologies affecting the thoracic aorta, the arch anatomy with presence of acute angulation distal to the subclavian artery may increase risk for bird beaking phenomenon. Lack of adequate stentgraft apposition in the arch results in higher risk for endoleak, and increased risk for stentgraft fatigue and collapse 4. The major challenge to stentgraft treatment of of aortic disease is however related to aortic pathology. The stentgraft technique is used in various acute and elective aortic pathologies, with different natural courses of and disease properties. The fragility of the aorta in an acute dissection setting results in the risk for conversion of a type B dissection to a retrograde type A dissection, with massive risk for mortality for the patient 5. In the chronic dissection aneurysm, stentgraft treatment primarily can reduce pressure in the thoracic false lumen, but does not cure the patient from thoracoabdominal dilatation unless complex treatment is undertaken 6. Techniques are being developed for treatment of arch pathology, but neurological complications remain a concern 7. Further customisation of stentgrafts to specific disease properties may increase applicability of endovascular techniques for various aortic pathologies in the future.

7 1. Mani K, Lees T, Beiles B, Jensen LP, Venermo M, Simo G, et al. Treatment of abdominal aortic aneurysm in nine countries : a vascunet report. Eur J Vasc Endovasc Surg 2011;42(5): Clough RE, Mani K, Lyons OT, Bell RE, Zayed HA, Waltham M, et al. Endovascular Treatment of Acute Aortic Syndrome. J Vasc Surg 2011;54(6): Sweet MP, Fillinger MF, Morrison TM, Abel D. The influence of gender and aortic aneurysm size on eligibility for endovascular abdominal aortic aneurysm repair. J Vasc Surg 2011;54(4): Steuer J, Bjorck M, Sonesson B, Resch T, Dias N, Hultgren R, et al. Editor's Choice - Durability of Endovascular Repair in Blunt Traumatic Thoracic Aortic Injury: Long-Term Outcome from Four Tertiary Referral Centers. Eur J Vasc Endovasc Surg 2015;50(4): Williams JB, Andersen ND, Bhattacharya SD, Scheer E, Piccini JP, McCann RL, et al. Retrograde ascending aortic dissection as an early complication of thoracic endovascular aortic repair. J Vasc Surg 2012;55(5): Mani K, Clough RE, Lyons OT, Bell RE, Carrell TW, Zayed HA, et al. Predictors of Outcome after Endovascular Repair for Chronic Type B Dissection. Eur J Vasc Endovasc Surg 2012;43: Haulon S, Greenberg RK, Spear R, Eagleton M, Abraham C, Lioupis C, et al. Global experience with an inner branched arch endograft. J Thorac Cardiovasc Surg 2014;148(4):

8 The Impossible: Open Surgery in the Ailing Aorta. Åben kirurgi, er det nødvendigt? Jacob Budtz Lilly, MD AUH Karkirurgisk afdeling T Akut aortasyndrom (AAS) er et vigtigt, men relativt nyt koncept inden for karkirurgien. Der er stor variation i måden hvorpå AAS præsenterer sig klinisk og ofte er progression af sygdommen uforudsigelig. Det kan derfor være vanskeligt at afgøre hvad der er den optimale behandling. Selvom endovaskulære interventioner anvendes i stigende grad, skal det bemærkes, at åben kirurgi stadig har en hovedrolle, navnligt for akut type A dissektion. Studier der omhandler AAS er vanskelige at fortolke, hovedsageligt på grund af ikkesammenlignelige patientpopulationer, men også på grund af ændringen af behandlingsalgoritmer over tid. Åben kirurgis plads i behandlingen af AAS er vanskelig at definere. Årsagen skyldes i høj grad en kombination af fem vigtige faktorer: 1. Kendskabet til AAS er fortsat lav og korrekt diagnosticering forekommer alt for sporadisk. 2. Henvisningsproceduren varierer i høj grad. 3. Forløb og natural history er uforudsigeligt. 4. Behandlingsvinduet er smalt. 5. Graden af comorbiditet i denne patientgruppe er meget høj På grund af disse faktorer, er karkirurgen vigtigt. Den niche som åben kirurgi opfylder i den akutte situation kan være livsredende, og rettidige beslutninger er afgørende. Dette illustreres i foredraget med flere cases og eksempler. Herudover diskuteres det, hvordan nogle patientgrupper med en syg aorta er bedre tjent med åben kirurgi. Referencer: 1. Clough RE, Nienaber CA. Management of acute aortic syndrome. Nat Rev Cardiol 2015;12: Coady MA, Rizzo JA, Elefteriades JA. Pathologic variants of thoracic aortic dissections. Penetrating atherosclerotic ulcers and intramural hematomas. Cardiol Clin 1999; 17: Evangelista A, Mukherjee D, Mehta RH, et al. Acute intramural hematoma of the aorta: a mystery in evolution. Circulation 2005; 111: Odero A, Arici V, Bozzani A. Clinical presentation and evidence-based indications to treatment. In: Chiesa R, Melissano G, Zangrillo A, editors. Thoraco-Abdominal Aorta: Surgical and Anesthetic Management. Milan: Springer; pp Waterman AL, Feezor RJ, Lee WA. Endovascular treatment of acute and and chronic pathology in patients with Marfan syndrome. J Vasc Surg 2012;55: