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Acute Aortic Syndromes

A multidisciplinary approach to acute aortic dissection

21 November 2024
Dr Katherine Neas, Clinical Geneticist | Prof Sean Galvin, Cardiothoracic Surgeon
Dr Lupe Taumoepeau, Vascular Surgeon | Dr Sheila Hart, Cardiothoracic Anaesthetist

OVERVIEW


CERC 01 was the inaugural meeting of the Cardiothoracic Education & Research Collaboration. It brought together cardiac surgeons, vascular surgeons, anaesthetists, and a clinical geneticists to provide a comprehensive, MDT overview of acute aortic syndromes.


We covered:

  • The genetic basis of thoracic aortic disease: which genes to test, who to refer, and how to interpret results in a New Zealand context.

  • The anatomy of dissection, evolution of arch replacement techniques, and the challenge of malperfusion.

  • Endovascular management of malperfusion syndromes, including the emerging endo-first strategy for visceral ischaemia.

  • A practical anaesthetic guide to getting patients safely to - and through - their operation.


A huge thank-you to our first speakers and to everyone who tuned in!

KEY LEARNING POINTS


All Clinicians

  • Acute aortic syndromes encompass intramural haematoma, penetrating aortic ulcer, and aortic dissection – each with distinct but overlapping management implications.

  • Aortic dissection can present as abdominal pain, limb ischaemia, stroke, or chest pain mimicking ACS. High vigilance is essential.

  • Type A dissection: mortality rises rapidly in the first 48 hours without surgery. Current ANZ 30-day surgical mortality is 10–25%.

  • Malperfusion involving a single organ carries approximately 30% mortality; each additional organ bed worsens outcomes significantly.

  • Non-haemorrhagic stroke is NOT a contraindication to surgery – prompt repair may reduce infarct size.

  • CPR in the setting of tamponade (reversible cause): proceed. Prolonged CPR >10–15 minutes outside the OR: surgical outcomes are very poor.


Surgeons

  • Open distal anastomosis is standard of care – clamping the aorta in Type A dissection risks arch aneurysm formation and re-entry tears.

  • Primary intimal tear is in the arch or descending aorta in ~40% of cases; multiple tears are common. Ascending repair alone does not address these.

  • Root decision: resuspension is adequate if root ≤35–40mm with no circumferential dissection or coronary involvement. Replace (Bentall) if root ≥45–50mm or extensive dissection.

  • Zone 2 arch replacement with antegrade cerebral perfusion (ACP) avoids deep hypothermic arrest and its coagulopathic complications.

  • Frozen elephant trunk: use selectively for circumferential arch dissection, large arch fenestration, retrograde Type B, or to create a TEVAR landing zone.

  • Up to 90% of patients have persistent false lumen flow after ascending-only repair. Plan for completion TEVAR and long-term imaging surveillance.

  • Approximately one in four patients will require re-intervention by 10 years.


Anaesthetists

  • Haemodynamic goals at induction: target SBP 100–120 mmHg, avoid tachycardia and hypertension. Use beta-blockers and/or opiates.

  • Bilateral radial arterial lines if antegrade cerebral perfusion planned; femoral arterial line when cerebral and systemic circuits are separated.

  • TOE at induction: confirm dissection, assess AR mechanism and severity, identify LV function and regional wall motion abnormalities.

  • True lumen on TOE: more circular, expands in systole, no spontaneous echo contrast. False lumen: thrombus/smoke, does not expand in systole.

  • Circumferential STJ intimal flap prolapse: can cause unrecognised coronary malperfusion when pulsatile flow is lost on bypass. Watch the TOE. ECG changes or declining cardiac output – check TOE and consider briefly restoring pulsatile flow.

  • Cerebral oximetry: useful for unilateral drops (cannula position, incomplete circle of Willis). Bilateral desaturation – evidence for intervention is limited; optimise haemoglobin, oxygenation, and pressure.

  • Coagulopathy is predictable and severe. Get all products into the room early. Guide treatment with TEG/ROTEM.


ICU

  • Anticipate bleeding, multi-organ failure, and neurological complications.

  • Early post-operative CT (day 3–7), then 3 months, 6 months, and annually. False lumen surveillance is lifelong.

  • Endo-first approach for mesenteric malperfusion: endovascular fenestration and stenting followed by delayed central repair (~24–48 hours). Allows reperfusion before the insult of open surgery.

  • Frank gut ischaemia at presentation carries close to 100% mortality regardless of approach – the endo-first strategy avoids futile central repair in this group.

  • Spinal cord malperfusion: lumbar drains and spinal perfusion pressure optimisation. Neurological recovery is possible with time.


Cardiologists and Echocardiographers

  • Genetic testing yield is higher with syndromic features (Marfan, Loeys-Dietz), age <50 at presentation, family history of aortic disease, and ascending aorta involvement.

  • Most patients should receive a gene panel rather than single-gene FBN1 testing – unless there is a clear Marfan phenotype meeting revised Ghent criteria.

  • Variant interpretation: population-specific databases matter. NZ Māori population-specific variant frequencies must be considered when classifying variants.

  • Patent false lumen on follow-up CT is associated with reduced survival and increased re-intervention. True lumen remodelling and false lumen regression are favourable.

  • Refer patients to genetics: syndromic features, aortic disease <50 years of age, or strong family history. HealthPathways is the standard NZ referral route.

Dr Katherine Neas
Kate is a clinical geneticist based in Wellington and the National Clinical Director of Genetic Health Service NZ. Her practice includes the assessment and management of patients with heritable thoracic aortic disease, including Marfan syndrome, Loeys-Dietz syndrome, and familial thoracic aortic aneurysm and dissection. She works closely with cardiothoracic teams to develop shared care pathways for genetic testing and cascade screening in families with identified pathogenic variants.


Professor Sean Galvin
Sean is a cardiothoracic surgeon based in Wellington. His post-fellowship training focused on surgery of the thoracic aorta,  the use of arterial conduits in coronary surgery and both general and minimally invasive thoracic surgical techniques. He is actively involved in clinical research and teaching with honorary appointments at a number of academic institutes. He is the Cardiothoracic Surgical Supervisor of Training at Wellington Hospital.


Dr Lupe Taumoepeau
Lupe is a consultant vascular, endovascular and transplant surgeon at Wellington Hospital. Her practice encompasses the full range of open and endovascular aortic interventions, and she has particular expertise in the management of malperfusion syndromes complicating aortic dissection.


Dr Sheila Hart
Dr Sheila Hart is a consultant cardiothoracic anaesthetist, Clinical Director of the Department of Anaesthesia and Pain at Wellington Hospital, and a past President of the New Zealand Society of Anaesthetists. She has extensive experience in the anaesthetic management of emergency aortic surgery and complex cardiothoracic procedures.

References


  • Loeys BL, Dietz HC, Braverman AC, et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet. 2010;47(7):476–485. PMID: 20591885

  • Ostberg NP, Zafar MA, Ziganshin BA, Elefteriades JA. The Genetics of Thoracic Aortic Aneurysms and Dissection: A Clinical Perspective. Biomolecules. 2020;10(2):182. PMID: 31991693

  • Isselbacher EM, Preventza O, Hamilton Black J, et al. 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease. Circulation. 2022;146(24):e334–e482. PMID: 36322642

  • Evangelista A, Isselbacher EM, Bossone E, 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. PMID: 29685932

  • Leshnower BG, Keeling WB, Duwayri YM, Jordan WD, Chen EP. The "thoracic endovascular aortic repair-first" strategy for acute type A dissection with mesenteric malperfusion. J Thorac Cardiovasc Surg. 2019;158(6):1516–1524. PMID: 30853232


Online Resources


  • Genomics England PanelApp – Thoracic Aortic Aneurysm or Dissection: panelapp.genomicsengland.co.uk

  • International Registry of Acute Aortic Dissection (IRAD): iradonline.org

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