Diagnosing and Treating Acute and Chronic Syndromes of the Aortic Organ

Publication Date: February 25, 2024
Last Updated: February 29, 2024

The aortic organ

  • It is recommended to view, interpret and treat the aorta in the context of an organ, whereby diagnosis, treatment and surveillance should be approached with this perspective. I, C

Nomenclature and risk stratification

  • In patients with aortic dissection, Ishimaru zones are recommended for use as a reporting standard of disease extent. I, C -
  • The use of the TEMd classification should be considered in any acute aortic syndrome to determine the type of disease and an initial treatment strategy. IIa, C
  • The use of the GERAADAe score should be considered in patients with acute type A aortic dissection undergoing surgery to determine 30-day mortality. IIa, C

Aortic teams and healthcare implications

  • Shared decision-making for the optimal treatment of aortic pathologies by a multidisciplinary aortic team is recommended. I, C
  • In patients with multisegmental aortic disease, treatment is recommended in aortic centres providing open and endovascular cardiac and vascular surgery on site. I, C
  • Transfer to an aortic centre should be considered for patients with complex aortic pathologies. IIa, B
  • For endovascular aortic procedures, a hybrid operating room, including an integrated imaging system, is recommended. I, C

Diagnostic work-up and imaging

Clinical risk assessment

  • Preoperative risk assessment is recommended. I, B
  • TTE is recommended as part of the preoperative work-up. I, B
  • Coronary evaluation/assessment should be considered to rule out coronary artery disease. IIa, B

Imaging—morphology and function

  • As the first line diagnostic modality, CTA from the common carotid to the common femoral arteries is recommended for imaging the aorta and its pathologies. I, C
  • For repetitive imaging of the aorta, it is recommended that the same imaging modality be used, with a similar method of measurement to assess change in diameter. I, C
  • Assessment of patency and morphology of the circle of Willis should be considered prior to total aortic arch replacement. IIa, C
  • In patients with known or suspected aortic disease, it is recommended that aortic diameters be measured at reproducible anatomical landmarks (and its maximal diameter) perpendicular to the axis of blood flow. I, C
  • In asymmetric or oval contour cases, reporting the longest diameter and its perpendicular diameter should be considered. IIa, C
  • When performing CT or MRI, one should consider measuring the aorta from the outer edge to the outer edge, if the vessel wall is visible. IIa, C
  • One should consider measuring the aortic root from sinus to sinus, and the largest diameter measured should be considered as a reference value. IIa, C
  • Assessment of the risk of radiation exposure is recommended, especially in younger adults and in those undergoing repetitive imaging. I, C
  • For CT or MRI techniques involving the entire aorta, it is recommended that motion artefacts of the ascending aorta be minimized and that the entire aorta should be acquired in a single data set. I, C

Acute aortic diseases: Type A aortic dissection

  • Initiation of emergency surgery is recommended in patients presenting with acute type A aortic dissection. I, B
  • A tear-oriented approach with exclusion or resection of the primary entry tear in the ascending aorta and arch is recommended. I, B
  • Inspection and coverage of communications between lumina in the proximal descending aorta may be considered in specialized centres for prognostic reasons. IIb, C
  • Despite preoperative neurologic dysfunction or non-haemorrhagic stroke, open repair should be considered. IIa, B
  • In case of clinical and imaging evidence of visceral malperfusion, revascularization may be considered prior to aortic repair. IIb, C
  • Antegrade systemic perfusion via axillary or direct aortic cannulation should be considered. IIa, B
  • An open distal anastomosis during lower body hypothermic circulatory arrest is recommended. I, B

Acute aortic diseases: non-A non-B aortic dissection

  • In patients with complicated non-A non-B aortic dissection with arch entry tear, repair via the FET technique should be considered. IIa, C
  • In patients with anatomical feasibility to cover the primary entry tear, a stent graft implantation may be considered. IIb, C

Acute aortic diseases: type B aortic dissection

  • In patients with complicated acute type B aortic dissection and suitable anatomy, TEVAR is recommended. I, B
  • In patients with acute complicated type B aortic dissection with unsuitable anatomy for TEVAR, FET repair should be considered. IIa, B
  • In acute type B aortic dissection with high-risk features, TEVAR should be considered in the subacute phase. IIa, C
  • In patients with acute type B aortic dissection without high-risk features, optimal medical therapy, close monitoring and follow-up is recommended for emerging high-risk features. I, B

Acute aortic diseases: type A intramural haematoma

  • In patients with acute type A IMH with complications or high-risk features, emergency surgery is recommended. I, B
  • Optimal medical therapies and serial imaging may be considered in patients with type A IMH in the absence of high-risk features. IIb, C
  • In selected patients with acute type A IMH without high-risk features but a tear in the descending aorta, TEVAR may be considered in addition to OMT in specialized centres. IIb, C

Acute aortic diseases: type B intramural haematoma

  • In patients with acute complicated type B IMH, urgent aortic repair is recommended. I, B
  • In patients with acute type B IMH with high-risk features, TEVAR should be considered if the patient is anatomically suitable. IIa, C
  • In patients with acute type B IMH without high-risk features, OMT and serial imaging are recommended. I, B
  • In patients with acute type B IMH without high-risk features, OMT, close monitoring and follow-up are recommended. I, C

Acute aortic diseases: penetrating atherosclerotic ulcer

  • In patients with PAUs in the ascending aorta and the presence of IMH or rupture, urgent aortic repair is recommended. I, B
  • In patients with high-risk PAUs located in the distal arch or descending aorta, TEVAR should be considered if anatomically suitable. IIa, B
  • In patients with high-risk PAUs located in the distal arch or descending aorta unsuitable for TEVAR, open surgical repair should be considered after careful evaluation of operative risk. IIa, B

Acute aortic diseases: blunt traumatic aortic injury

  • In patients with BTAI grade I, nonoperative management, including close follow-up imaging, is recommended. I, C
  • In patients with BTAI grade II and high-risk imaging features, TEVAR should be considered. IIa, C
  • In patients with BTAI grade II without high-risk imaging features, nonoperative management and close follow-up imaging may be considered. IIb, C
  • In patients with BTAI grades III–IV and suitable anatomy, TEVAR is recommended. I, C

Chronic aortic diseases: root and ascending aorta

  • Surgery for an ascending aortic aneurysm located at the root or tubular tract, either with TAV or BAV, is recommended when the maximum aortic diameter is ≥55 mm. I, B
  • Surgery for BAV-related aortopathy with ‘root phenotype’ is recommended when the maximum aortic diameter is ≥50 mm. I, B
  • Surgery for TAV-associated aneurysms with ‘root phenotype’ should be considered when the maximum aortic diameter is ≥50 mm in a low-surgical-risk setting. IIa, B
  • In patients with low surgical riske and ‘ascending phenotype’ dilatation, both with TAV and BAV, surgical treatment should be considered when the maximum aortic diameter is >52 mm. IIa, C
  • In patients with low surgical risk and ‘ascending phenotype’ BAV-related aortopathy, surgery should be considered at a maximum diameter ≥50 mm if any of the following is present:
    • age <50 years
    • short stature (<1.69 m)f
    • ascending aortic length >11 cmg
    • aortic diameter growth rate >3 mm/year
    • family history of the acute aortic syndrome
    • aortic coarctation
    • refractory hypertension
    • shared decision with the patienth
    • concomitant non-aortic valve cardiac surgery
    • IIa, C
  • In patients with non-syndromic TAV with ‘ascending phenotype’, in a low-surgical-risk setting,d surgery may be considered at a maximum diameter ≥50 mm if any of the following is present:
    • age <50 years
    • short stature (<1.69 m)f
    • ascending aortic length >11 cmg
    • aortic diameter growth rate >3 mm/year
    • refractory hypertension
    • shared decision with the patienth
    • IIb, C
  • Surgery for aortic dilatation in TAV patients undergoing non-aortic valve cardiac surgery should be considered at a root or ascending diameter ≥50 mm. IIa, C
  • Surgery for aortic dilatation in patients undergoing aortic valve surgery should be considered at a root or ascending diameter ≥45 mm. IIa, C
  • Surgery for aortic root dilatation in patients undergoing surgery for ascending aortic aneurysm should be considered at an aortic root diameter ≥45 mm. IIa, C
  • In patients with young age, a family history of the acute type A aortic dissection or known HTAD who are undergoing ascending replacement, a lower threshold than 45 mm may be considered for concomitant root replacement on an individual basis. IIb, C

Chronic aortic diseases: maximum diameter and maximum length

  • Surgery should be considered for a symptomatic aneurysm of the ascending aorta, almost independently of size (once non-aortic causes have been eliminated). IIa, C
  • For individuals of small body size, height nomograms should be considered in the decision about indications for surgery at various aortic dimensions. IIa, B
  • An ascending aortic length exceeding 110 mm should be considered as a risk factor for aortic events when indicating elective surgery for aortic aneurysms. IIa, B
  • Despite metrics and precision criteria, the benefits and risks of surgical intervention versus nonoperative management should be considered on a case-by-case basis. IIa, C
  • Other non-diameter criteria that also bear on the risk of aortic events may be considered in the decision-making. IIb, C

Chronic aortic diseases: aortic arch

  • In patients with symptomatic aortic arch pathologies, aortic arch repair is recommended irrespective of diameter. I, C
  • In patients with asymptomatic isolated aortic arch aneurysms ≥55 mm, aortic repair should be considered. IIa, B
  • In patients with arch pathologies and diseased aortic segments distal to zone 2, FET repair should be considered. IIa, B
  • In patients with an indication for aortic repair unsuitable for open surgery, hybrid or endovascular aortic repair may be considered. IIb, C
  • Hybrid or endovascular aortic repair is recommended to be performed in experienced centres with an adequate volume of both open and endovascular aortic repairs. I, C

Chronic aortic diseases: chronic aortic dissection

  • Intervention is recommended in patients with chronic aortic dissection at a maximum aortic diameter of ≥55 mm without involvement of the ascending aorta. I, B
  • In patients with HTAD with chronic aortic dissection, intervention at diameters <55 mm should be considered if the multidisciplinary aortic team makes the decision depending on the genotype, growth rate, family history and other individual patient risk factors. IIa, C
  • In patients with dSINE, treatment is recommended to prevent diameter progression. I, C
  • Intervention at >50 mm should be considered in patients with chronic aortic dissection if the treatment includes a multistep procedure, such as arch replacement with FET followed by TEVAR. IIa, C

Heritable thoracic aortic disease

  • Genetic testing is recommended in patients with thoracic aortic disease <60 years of age, family history of TAD, arterial aneurysms in other segments and those with syndromic features. I, B
  • Testing of family members is recommended by simpler, more cost-efficient Sanger sequencing of only the suspect genetic area. I, C

Marfan syndrome

  • In patients with Marfan syndrome, surgery on the aortic root or ascending aorta is recommended at a diameter of ≥50 mm. I, B
  • In patients with Marfan syndrome and high-risk features,* surgery on the aortic root or ascending aorta should be considered at a diameter of ≥45 mm. IIa, B
  • In patients with Marfan syndrome without high-risk features with a high likelihood of undergoing valve-sparing aortic root replacement and very low surgical risk, surgery on the aortic root or ascending aorta may be considered at a diameter of ≥45 mm when performed by an experienced aortic team. IIb, C
  • In patients with Marfan syndrome, surgery of the aortic arch, descending thoracic aorta or abdominal aorta should be considered at a diameter of ≥50 mm of the respective aortic segment. IIa, C

Loeys–Dietz syndrome

  • In patients with Loeys–Dietz syndrome, indication for surgery is recommended based on the specific genetic variant, aortic diameter, aortic growth rate, family history, history of aortic events, patient age and other individual patient-related factors and discussed by a multidisciplinary aortic team. I, C
  • In patients with Loeys–Dietz syndrome attributable to a pathogenic variant in TGFBR1 or TGFBR2, surgery on the aortic root or ascending aorta is recommended at a diameter of ≥45 mm. I, C
  • In patients with Loeys–Dietz syndrome attributable to a pathogenic variant in TGFBR1 and high-risk features,* surgery on the aortic root or ascending aorta may be considered at a diameter of ≥40 mm. IIb, C
  • In patients with Loeys–Dietz syndrome attributable to a pathogenic variant in TGFBR2 and high-risk features,# surgery on the aortic root or ascending aorta should be considered at a diameter of ≥40 mm. IIa, C
  • In patients with Loeys–Dietz syndrome attributable to a pathogenic variant in TGFB3, surgery on the aortic root or ascending aorta may be considered at a diameter of ≥50 mm. IIb, C
  • In patients with Loeys–Dietz syndrome attributable to a pathogenic variant in SMAD3, surgery on the aortic root or ascending aorta should be considered at a diameter of ≥45 mm. IIa, C
  • In patients with Loeys–Dietz syndrome attributable to a pathogenic variant in TGFBR1, TGFBR2 or SMAD3, surgery to replace the intact aortic arch, descending aorta or abdominal aorta at a diameter of ≥45 mm may be considered. IIb, C
  • In patients with Loeys–Dietz syndrome attributable to a pathogenic variant in SMAD2 or TGFB2, surgery on the aortic root or ascending aorta may be considered at a diameter of ≥45 mm. IIb, C

Chronic aortic diseases: non-syndromic heritable thoracic aortic disease

  • In patients with HTAD attributable to a pathogenic variant in ACTA2, surgery on the aortic root or ascending aorta should be performed at a diameter of ≥45 mm. IIa, C
  • In patients with HTAD attributable to a pathogenic variant in ACTA2 and high-risk features,* surgery on the aortic root or ascending aorta may be considered at a diameter of ≥42 mm. IIb, C
  • In patients with HTAD attributable to a pathogenic variant in PRKG1, surgery on the aortic root or ascending aorta may be considered at a diameter of ≥42 mm. IIb, C
  • In patients with HTAD attributable to a pathogenic variant in PRKG1 and high-risk features,* surgery on the aortic root or ascending aorta may be considered at a diameter of ≥40 mm. IIb, C
  • In patients with HTAD attributable to a pathogenic variant in MYLK or MYH11, surgery on the aortic root or ascending aorta may be considered at a diameter of ≥45 mm. IIb, C

Chronic aortic disease: descending aorta

  • In patients with asymptomatic descending aortic aneurysms ≥55 mm, aortic repair is recommended. I, C
  • In patients with asymptomatic descending aortic aneurysms with high-risk features, repair should be considered in diameters <55 mm. IIa, C

Chronic aortic disease: thoraco-abdominal

  • In patients with asymptomatic thoraco-abdominal aneurysms ≥55 mm, aortic repair is recommended. I, C
  • In patients with asymptomatic thoraco-abdominal aneurysms with high-risk features, aortic repair should be considered with diameters <55 mm. IIa, C

Chronic aortic diseases: abdominal aorta

  • In patients with ruptured AAA, emergency surgical or endovascular treatment is recommended. I, C
  • In patients with symptomatic, unruptured AAA, emergency repair is recommended, irrespective of diameter. I, B
  • In patients with asymptomatic AAA, repair is recommended when the AAA exceeds 55 mm. I, A
  • In women with asymptomatic AAA, repair should be considered when the AAA exceeds 50 mm. IIa, C
  • AAA repair should be considered if aneurysm growth exceeds 10 mm/year. IIa, C
  • In AAA patients with high-risk features, earlier intervention at lower diameters may be considered. IIb, C
  • In asymptomatic patients with AAA below the threshold for intervention, lifestyle modification, OMT and continuous surveillance are recommended. I, C
  • In elective patients undergoing AAA repair, preoperative cardiac evaluation is recommended. I, C
  • In elective patients undergoing AAA repair, preoperative cardiac echocardiography and/or screening for CAD (coronary artery angiography or coronary CTA) may be considered. IIb, C

How to monitor end-organ function and how to avoid end-organ injury

Perfusion

  • In patients undergoing aortic surgery with involvement of the aortic arch, the preference for antegrade arterial inflow cannulation as compared to retrograde inflow cannulation should be considered. IIa, B
  • Axillary artery cannulation should be considered for an antegrade inflow cannulation site for patients undergoing aortic surgery with involvement of the aortic arch. IIa, B
  • Multiple arterial pressure monitoring lines (upper and lower body) are recommended for aortic arch and/or thoraco-abdominal aortic repair. A femoral arterial line is recommended for lower body perfusion-pressure monitoring. I, C

Temperature management

  • Nasopharyngeal and core temperature (rectal/bladder) measurements are recommended in patients undergoing aortic arch surgery in lower body HCA. I, C
  • Considering the core temperature (rectal/bladder) as the reference value for the determination of the level of lower body HCA is recommended. I, C
  • A target hypothermic circulatory arrest temperature should be determined based on the anticipated extent of repair, expected duration of lower body HCA and presence of preoperative malperfusion. IIa, C
  • The use of the term ‘mild hypothermia’ is recommended for core temperatures >28°C. I, C
  • The use of the term ‘high moderate hypothermia’ is recommended for core temperatures between 24.1°C and 28°C. I, C
  • The use of the term ‘low moderate hypothermia’ is recommended for core temperatures between 20.1°C and 24°C. I, C
  • The use of the term ‘deep hypothermia’ is recommended for core temperatures ≤20°C. I, C
  • High-moderate lower body HCA in combination with cerebral perfusion for hemiarch replacement is recommended. I, C
  • High-moderate lower body HCA in combination with selective antegrade cerebral perfusion for complex aortic arch procedures should be considered. IIa, B
  • Target lower body HCA temperature, as well as the cerebral protection method, should be determined based on the anticipated extent of repair, expected duration of lower body HCA and the presence of preoperative malperfusion. IIa, C

Organ protection

  • Retrograde cardioplegia should be considered to facilitate repetitive cardioplegia applications without interrupting the procedure. IIa, C
  • Non-cardioplegic myocardial perfusion may be considered during extended aortic arch repair to reduce myocardial ischaemia time. IIb, C
  • During surgery for aortic arch repair, the use of bilateral NIRS-based cerebral oximetry combined with an algorithmic approach to intervention for cortical Hb-desaturation is recommended. I, C
  • In a patient with an incomplete circle of Willis diagnosed in preoperative cerebral CTA, trilateral antegrade perfusion should be considered via additional cannulation or intubation of the left subclavian artery. IIa, C
  • CSF drainage for spinal cord protection is recommended in open TAAA replacement. I, B
  • CSF drainage should be considered in total aortic arch replacement in patients at risk of SCI. IIa, C
  • CSF drainage may be considered in type A aortic dissection patients with preoperative symptomatic SCI. IIb, C
  • CSF drainage is not recommended as a routine measure in FET procedures if coverage of ICAs is limited due to the operative strategy. III, C
  • For patients at an increased risk of spinal cord injury undergoing endovascular treatment of thoracic or thoraco-abdominal aortic disease, prophylactic CSF drainage should be considered. IIa, C
  • The 4-territory concept should be considered during planning to reduce procedure-induced deprivation of antegrade arterial spinal cord supply to a minimum. IIa, C
  • A critical appraisal of the contribution of thoracic and lumbar segmental arteries to spinal cord perfusion should be considered before surgery to determine the need for reimplantation. IIa, C
  • Prevention of steal in particular from segmental arteries to avoid SCI is recommended. I, C
  • In patients undergoing aortic arch surgery repair, early reinstitution of distal aortic perfusion should be considered to reduce postoperative visceral, renal and spinal ischaemia. IIa, B
  • Selective visceral and renal perfusion is recommended in open TAAA repair I, B
  • In TAAA surgery, maintenance of lower limb perfusion to avoid rhabdomyolysis and AKI is recommended. I, C
  • Deep HCA should be considered in patients undergoing open thoraco-abdominal aorta repair based on surgical and institutional experience. IIa, C
  • A period of hypothermic perfusion at the desired target core temperature before hypothermic lower body circulatory arrest should be considered to ensure appropriate thermal distribution throughout the body. IIa, C
  • A period of hypothermic perfusion before initiation of the rewarming phase should be considered to decrease end-organ injury. IIa, C
  • Arterial CPB outlet temperature of <37°C during rewarming to avoid hyperthermic perfusion is recommended. I, C

Therapeutic options: aortic root

  • For aortic dilatations and aneurysms involving the aortic root with a structurally diseased aortic valve, replacement of the aortic valve and sinuses with coronary ostia direct reimplantation (modified Bentall operation) is recommended. I, B
  • Valve-sparing root replacement should be considered for patients with a non-diseased tricuspid aortic valve and dilated root, especially young patients, if performed by experienced surgeons. IIa, B
  • Valve-sparing root replacement may be considered for patients with a non-diseased bicuspid aortic valve and dilated root if performed by surgeons with specific expertise in aortic valve repair. IIb, B

Ascending aorta with or without proximal arch

  • In ascending aortic surgery, supracommissural isolated ascending aorta replacement is recommended in patients without adjunct aortic root and aortic arch dilatation. I, B
  • It is recommended that in any open proximal thoracic aortic operation, ascending/hemiarch replacement has to be extensive, and short ascending grafts should be avoided to prevent disease progression and to anticipate a future endovascular modular distal extension. I, C
  • In ascending aortic surgery with a diameter larger than 45 mm of the proximal aortic arch, hemiarch or more extensive aortic arch replacement may be considered. IIb, B

Therapeutic options: aortic arch

  • In patients with an intended one-stage aortic arch treatment, the FET technique should be considered. IIa, B
  • Aortic arch replacement, including the creation of a sufficient landing zone, is recommended in the treatment of multisegmental aneurysms of the thoracic aorta in order to facilitate the further downstream repair. I, C
  • When performing the frozen and conventional elephant trunk techniques, a distal anastomosis in arch zone 2 should be considered. IIa, C
  • In the case of an elephant trunk implant, the polyester trunk component should be considered to be accessible in zone 4. IIa, C
  • Hybrid procedures may be considered for aortic arch repair. IIb, C
  • In any TEVAR involving zone 2, left subclavian artery revascularization is recommended to reduce the risk of neurologic complications such as stroke and spinal cord ischaemia. I, B

Therapeutic options: descending aorta

  • In patients undergoing endovascular aortic repair with suitable femoral artery anatomy, ultrasound-guided percutaneous access is recommended. I, B
  • TEVAR is recommended as the first-choice therapy for acute complicated descending aortic syndromes. I, B
  • If LSA-to-LCCA bypass/transposition or double transposition cannot create a sufficient proximal landing zone, the FET technique should be considered. IIa, C
  • If concomitant valvular or coronary disease requiring treatment is present together with acute and chronic thoracic aortic pathology involving the aortic arch, the FET technique should be considered. IIa, B
  • Stent-graft oversizing in degenerative/atherosclerotic aneurysms or PAUs should be considered <15–20% of the proximal and distal landing zone diameters. I, C
  • Stent-graft oversizing in acute aortic dissection/IMH should be considered <10% of the proximal landing zone diameter. I, C
  • It should be considered that the landing zone diameter should not exceed 38 mm in diameter. IIa, B
  • It should be considered that the proximal landing zone length be at least 25 mm. IIa, C
  • It should be considered that the distal landing zone length be at least 25 mm. IIa, C
  • In TEVAR for type B aortic dissection, distal tapering for the prevention of dSINE should be considered according to the TL diameter. IIa, C
  • The PETTICOAT technique should be considered in acute aortic dissections as a distal adjunct to TEVAR in case adequate true lumen decompression cannot be established by TEVAR alone. IIa, B
  • The STABILISE technique may be considered in particular scenarios but preferably under controlled study conditions. IIb, C

Therapeutic options: thoraco-abdominal aorta

  • For patients with low-to-moderate perioperative risk, open repair as well as endovascular treatment of pararenal and TAAA should be considered. IIa, C
  • For patients unfit for open repair, a B/FEVAR procedure should be considered the first-line treatment. IIa, B
  • A hybrid approach may be considered for patients unfit for open repair and anatomically unsuitable for a B/FEVAR procedure. IIb, C
  • A ‘distal-shifting’ strategy may be considered in any TAAA to reduce left lung manipulation to a minimum. IIb, C
  • For patients at high risk for SCI undergoing endovascular treatment of type I, II, III or V thoraco-abdominal aortic aneurysms, a staged TEVAR–B/FEVAR approach should be considered. IIa, C
  • In open TAAA repair, proximal clamping before the full establishment of CPB to avoid retrograde embolization of parietal thrombi should be considered. IIa, C
  • In patients undergoing open descending or thoraco-abdominal aorta repair, cryoablation of multiple intercostal spaces (temporary nerve blockage) may be considered for pain control as an adjuvant strategy. IIb, C
  • Left heart bypass or partial femoral–femoral bypass for open thoraco-abdominal aorta repair should be considered based on surgical and institutional experience. IIa, C

Therapeutic options: infrarenal

  • In patients with complicated PAU, dissection or intramural haematoma and in patients with mycotic aneurysm and pseudoaneurysm in the abdominal aorta, repair is recommended. I, C
  • In patients with complicated PAU, dissection intramural haematoma, or pseudoaneurysm with suitable anatomy of the abdominal aorta, endovascular repair should be considered as the first option. IIa, C
  • In patients with intact AAA with suitable anatomy for either open or EVAR, a shared decision-making process for each approach, including life expectancy, is recommended. I, C
  • In patients with long life expectancy, open AAA repair should be considered the preferred treatment modality. IIa, B
  • In patients with suitable anatomy and reasonable life expectancy, endovascular AAA repair should be considered the preferred treatment modality. IIa, B
  • In patients with ruptured AAA undergoing endovascular treatment, a bifurcated device should be considered as preferable over an aorta-uni-iliac device if anatomically suitable. IIa, C
  • Tubular EVAR without an iliac bifurcation stent graft implant is not recommended. III, C

First branch vessels

  • In patients with involvement of the iliac axis in the disease process, the preservation of blood flow to at least 1 hypogastric artery is recommended. I, B
  • In patients with isolated iliac artery aneurysms, elective treatment should be considered at a diameter threshold of a minimum of 35 mm. IIa, B
  • In patients with isolated iliac artery aneurysms, endovascular aneurysm repair may be considered. IIb, B

Therapeutic options: endoleaks

  • Type I and type III endoleaks are regarded as treatment failures, and reintervention is recommended. I, C
  • In the presence of a type II endoleak and aneurysm growth ≥10 mm, treatment should be considered in both thoracic and abdominal pathologies. IIa, C
  • In the presence of a type V endoleak (endotension without depictable endoleak on standard follow-up imaging), treatment should be considered in both thoracic and abdominal pathologies. IIa, C

Rare conditions: graft infections

  • It should be considered that patients with vascular graft infections be managed by a multidisciplinary team, including cardiovascular and vascular surgeons, imaging specialist, infectious diseases and microbiology specialist, in a facility with emergency access to these services. IIa, C
  • In patients with a prosthetic vascular graft, with positive blood cultures without a clear source, multimodality imaging (CTA, 18F-FDG PET/CT) to evaluate potential graft infection is recommended. I, B
  • In patients with vascular graft infection who are fit for surgery, open surgical repair is recommended. I, C
  • In patients with vascular graft infection and new-onset pseudoaneurysm or ongoing bleeding, emergency operative management is recommended. I, C
  • In patients with vascular graft infection and active bleeding into the airway or gastrointestinal tract, endovascular treatment may be considered as a bridging strategy. IIb, C
  • In patients with vascular graft infection who are haemodynamically stable without vascular discontinuity, scheduled open surgery should be considered. IIa, C

Surgical management

  • Surgical management of vascular graft infection, including excision of prosthetic material, local debridement and preferably orthotopic vascular reconstruction, is recommended. I, C
  • In patients with suspected vascular graft infection undergoing open surgical repair, multiple samples for obtaining conventional culture, 16S RNA genomic analysis and pathological examination are recommended. I, C
  • For surgical vascular graft infection management, liberal use of adjunct therapies such as delayed cavity closure, use of coverage flaps and continuous irrigation may be considered based on individual characteristics and local expertise. IIb, C

Postsurgical management

  • In patients who were operated on for vascular graft infection, a postoperative antimicrobial regimen for at least 6 weeks is recommended, if the tissue samples from retrieved specimens are culture positive. I, C
  • In patients with intraoperative purulence, MRSA, or infection caused by a multidrug-resistant microorganism or Candida spp, a 6-month regimen of antimicrobial therapy is recommended. In selected patients, life-long antibiotic therapy is recommended. I, C
  • After surgical treatment of vascular graft infection caused by fastidious intracellular microbiological agents (e.g. Coxiella brunetti, Mycobacterium spp, Mycoplasma spp, Bartonella spp) extending the postoperative antimicrobial course beyond 6 weeks may be considered. IIb, C
  • 18F-PET/CT should be considered to decide on the duration of prolonged antimicrobial course for vascular graft infection, particularly in patients with a retained vascular graft. IIa, C
  • In patients with vascular graft infection due to a low-virulence causative agent and favourable clinical/imaging features, medical management alone and close follow-up may be considered. IIb, C

Rare conditions: Kommerell diverticulum

  • In symptomatic patients with a high suspicion (dysphagia) of an aberrant subclavian artery and/or KD, CTA or MRI is recommended. I, C
  • It is recommended to offer multiple measurements of the KD and the adjacent aorta to improve surveillance and facilitate operative decision-making. I, C
  • In symptomatic patients with aberrant subclavian artery and/or KD, treatment is recommended. I, C
  • In patients with aberrant subclavian artery and/or KD, treatment may be considered when the diverticulum orifice is ≥30 mm and/or when the combined diameter of the diverticulum and the adjacent aorta (DAW) is ≥50 mm and/or when the diameter of the aberrant artery is ≥30 mm. IIb, C
  • In patients with a symptomatic aberrant subclavian artery, transsection of the vascular ring and transposition or bypass is recommended to release compression and to re-establish arterial circulation. I, C
  • In young patients without significant comorbidities, open surgical treatment (via a thoracotomy and carotid-to-subclavian artery bypass) of KD is recommended. I, C
  • In patients with an emergency presentation or who might not tolerate open surgery, hybrid (closed-chest) repair of the KD is recommended. I, C
  • In patients with KD and indication for treatment, open surgical resection and endovascular repair with subclavian debranching may be considered depending on the patient’s anatomical characteristics, age and general status. IIb, C

Rare conditions: coarctation of the aorta

  • In patients with a diagnosis of CoA, primary or recurrent and with clinical significance, treatment is recommended. I, C
  • Asymptomatic patients with a diagnosis of CoA but in whom there is a systolic gradient across the stenosis at rest (>20 mmHg) and/or SBP difference above 10 mmHg combined with coarctation-attributed HF and/or proximal hypertension should be considered for open or endovascular repair. IIa, C
  • Regardless of the pressure gradient, patients with documented HTA with >50% aortic luminal narrowing in relation to the aortic diameter at the level of the diaphragm (based on MRI, CT or invasive angiography measurements) should be considered for treatment. IIa, C
  • For adult patients with a diagnosis of CoA, referral to a specialized aortic centre should be considered. IIa, C
  • In patients with a diagnosis of coarctation of the native thoracic aorta as well as after previous repair and suitable anatomy, endovascular repair should be considered as an alternative to open surgical repair. IIa, C
  • In patients requiring coarctation re-repair, extra anatomical bypass from the ascending to the descending aorta may be considered at the time of concomitant cardiac repair. IIb, C

Aortic disease in women: pregnancy and post partum

Delivery in pregnant patients with aortopathy

  • In pregnant patients with a history of aortic dissection, C-section is recommended. I, C
  • In pregnant patients with an aortic diameter of <40 mm, vaginal delivery is recommended. I, C
  • C-section should be considered in pregnant patients with a diameter ≥45 mm of the aortic root or ascending aorta. IIa, C

Surgery before pregnancy in women with aortic disease

  • Aortic repair before pregnancy is recommended in patients with Marfan’s syndrome and aortic root diameter ≥45 mm. I, C
  • With an aortic root diameter of 40–45 mm, aortic repair before pregnancy may be considered in the presence of risk factors for aortic dissection (family history or rapid growth > 3 mm/year). IIb, C

Pregnancy in patients with aortopathy: dissection and surgery in pregnancy

  • Urgent aortic repair is recommended with foetal monitoring in patients with acute type A aortic dissection during the first 2 trimesters. I, C
  • In patients with acute type A aortic dissection during the third trimester, urgent caesarean delivery immediately followed by aortic surgery is recommended. I, C
  • OMT is recommended in patients with acute type B aortic dissection during pregnancy unless complications develop. I, C

Radiation protection

  • During endovascular aortic repair, the following radiation dose reduction strategies to protect the patient are recommended:
    • Positioning the image intensifier as close to the patient as possible
    • Using digital subtraction acquisitions and lateral angulations
    • Minimizing the time of exposure to radiation
    • I, C
  • During endovascular aortic repair, the following radiation dose reduction strategies to protect the physician are recommended:
    • Keep the personnel the greatest possible distance from the source of the radiation
    • Wear personal lead shields (apron, thyroid, shins and goggles)
    • Use mobile lead shields
    • I, C

Intensive care unit postoperative management of patients undergoing aortic surgery

  • The enhanced recovery after surgery concept is recommended in stable patients undergoing aortic repair. I, C
  • Normothermia is recommended in the early postoperative phase. I, C
  • The femoral artery should be considered as preferred for arterial pressure monitoring postoperatively. IIa, C
  • Close monitoring of lactate levels as a surrogate for postoperative malperfusion is recommended. I, C
  • Point-of-care monitoring, in addition to regular laboratory coagulation parameters, should be considered in the early postoperative phase after open aortic repair. IIa, B
  • Rescue protocols for postoperative SCI after aortic arch surgery procedures that include CSF drainage and blood pressure elevation are recommended. I, C
  • Immediate CSF drainage in patients with new onset of paraplegia after TEVAR or open TAAA replacement is recommended. I, C
  • For patients with ILT after FET procedures, therapeutic anticoagulation is recommended. I, C
  • Early TEVAR extension may be considered in patients with ILT after a FET implant. IIb, C

Living with aortic disease

Exercise and sports

  • Thorough education regarding the individual risks and benefits of exercise is recommended. I, C
  • For patients with adequately controlled BP, 30–60 min of mild-to-moderate dynamic exercise at least 3–4 days per week is recommended. I, C
  • Intense static exercise (e.g. heavy weightlifting or activities requiring the Valsalva manoeuvre) and collision sports are not recommended. III, C

Cardiac rehabilitation

  • In patients after invasive treatment of aortic pathologies, an individual cardiac rehabilitation programme under medical supervision is recommended. I, C

Surveillance

  • In any patients receiving treatment of aortic pathologies, a disease- and treatment-based individual surveillance programme in a specialized aortic centre with a dedicated outpatient clinic is recommended. I, C
  • Imaging-based quality control after every open or endovascular aortic procedure is recommended, irrespective of the treated segment before discharge. I, C
  • TTE is the recommended imaging modality after any kind of root surgery. I, C
  • In patients after treatment for acute aortic dissection or IMH irrespective of treatment modality, CTA surveillance is recommended after 6 months and 12 months and, in case of stable conditions, annually thereafter for 5 years. I, C
  • In patients after endovascular treatment irrespective of the underlying aortic disease, CTA surveillance is recommended after 6 months and 12 months and, in case of stable conditions, annually thereafter for 5 years. I, C
  • In patients after open surgical treatment for non-aortic dissection or IMH pathologies, imaging surveillance is recommended after 12 months and 24 months and, in case of stable conditions, should be extended thereafter. I, C
  • In patients with stable aortic conditions, extended surveillance intervals should be considered after 5 years based on an individual protocol. IIa, C
  • In case of non-operability, no option or informed consent of refraining from treatment, stopping surveillance is recommended. I, C

Recommendation Grading

Disclaimer

The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.

Overview

Title

Diagnosing and Treating Acute and Chronic Syndromes of the Aortic Organ

Authoring Organization

Society of Thoracic Surgeons

Publication Month/Year

February 25, 2024

Last Updated Month/Year

April 1, 2024

Document Type

Guideline

Country of Publication

US

Inclusion Criteria

Male, Female, Adult, Older adult

Health Care Settings

Ambulatory, Hospital, Outpatient, Operating and recovery room

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Diagnosis, Assessment and screening, Treatment, Management

Diseases/Conditions (MeSH)

D001018 - Aortic Diseases

Keywords

aortic disease, aortic organ

Source Citation

Martin Czerny, Martin Grabenwöger, Tim Berger, Victor Aboyans, Alessandro Della Corte, Edward P Chen, Nimesh D Desai, Julia Dumfarth, John A Elefteriades, Christian D Etz, Karen M Kim, Maximilian Kreibich, Mario Lescan, Luca Di Marco, Andreas Martens, Carlos A Mestres, Milan Milojevic, Christoph A Nienaber, Gabriele Piffaretti, Ourania Preventza, Eduard Quintana, Bartosz Rylski, Christopher L Schlett, Florian Schoenhoff, Santi Trimarchi, Konstantinos Tsagakis, EACTS/STS Scientific Document Group , EACTS/STS Guidelines for diagnosing and treating acute and chronic syndromes of the aortic organ, European Journal of Cardio-Thoracic Surgery, Volume 65, Issue 2, February 2024, ezad426, https://doi.org/10.1093/ejcts/ezad426