2014 CCS Thoracic Aortic Disease Guideline Summary

Guideline

Boodhwani et al (2014)  “Canadian Cardiovascular Society Position Statement on the Management of Thoracic Aortic Disease” Can J Cardiol 30:577-589
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Introduction

  • Generally asymptomatic until a catastrophic event (dissection/rupture)
  • Decision on intervention must be individualized by balancing risks of aortic complications vs. risk of surgical intervention:
Factors associated with increased risk of aortic complications Factors associated with increased risk of surgical intervention
  • ***Aortic diameter***
  • Connective tissue disorder
  • Family history of aortopathy
  • Bicuspid aortic valve
  • Aneurysm related symptoms
  • Rapid growth (>0.5 cm/y)
  • Concomitant aortic valve disease
  • Uncontrolled hypertension
  • Aortic arch pathology
  • Descending thoracic aortic pathology
  • COPD
  • Renal dysfunction
  • Previous cardiac surgery
  • Advanced age
  • LV Dysfunction 

Intervention Thresholds

Table: Recommended size thresholds for intervention of asymptomatic thoracic aortic aneurysms
  Aortic Root Ascending Arch Descending
Degenerative 5.5 cm 5.5 cm 6.0 cm 6.5 cm
Bicuspid Aortic Valve 5.0-5.5 cm 5.0-5.5 cm 5.5 cm 6.5 cm
Marfan Syndrome 5.0 cm * 5.0 cm 5.5-6.0 cm 5.5-6.0 cm
Familial Aortopathy 4.5-5.0 cm 4.5-5.0 cm 5.5-6.0 cm 5.5-6.0 cm
Other Genetic Syndromes 4.0-5.0 cm 4.2-5.0 cm 5.5-6.0 cm 5.5-6.0 cm
Undergoing CVSx — 4.5 cm — —
* For women anticipating pregnancy, threshold is 4.1-4.5 cm

Genetic Causes

Aortopathy Gene(s) Inheritance Aorta Notes
Marfan Syndrome FBN1 Autosomal D. Asc++, Arch+, Desc+ – Ghent Criteria
Loeys-Dietz Syndrome TGFBR1, TGFBR2, SMAD3, TGFB2 AD var. expr.

Asc++, Arch+, Desc+

  
Aneurysm-osteoarthritis Syndrome SMAD3 Autosomal D Asc++, Desc+  
Ehlers-Danlos Type IV COL3A1 Autosomal D Asc+, Desc+  
Bicuspid Ao Valve Multiple Comlex, Familial Cluster Asc++, Arch+, Desc-  
Familial Thoracic Ao Aneurysm TGFB2, TGFBR1, TGFBR2, MYH11, SMAD3, ACTA2 Autosomal D with reduced penetrance, var expr. Asc++, Arch+, Desc+  

Management

  • Risk of repair: 1% complications in experienced centres
  • Aneurysms involving arch and descending aorta have a higher risk

Follow-Up for dilated aorta:

  • Echocardiogram at diagnosis and at 6mo to determine rate of progression
    • Once stable, yearly echo (more frequent if > 4.5cm)
  • Genetic Testing if not Bicuspid AoV
  • Screen 1st degree relatives if
    • Genetic cause of aortic disease
    • Bicuspid aortic valve
Marfans Patients With Dilated Aorta
CCS Position Statement 2014 – Thoracic Aortic Disease
  • Target BP < 140/90 (Diabetes < 130/90)
  • B-Blocker and ARB 1st line to reduce dilation rate
    • Can add ACE/ARB/B-Blocker
  • Aggressive CV RF modification (smoking, exercise, etc.)
  • Avoid strenuous isometric exercise
  • Women with Marfan’s planning pregnancy should get root+aortic replacement at 4.1-4.5cm
 After Surgical Intervention
CCS Position Statement 2014 – Thoracic Aortic Disease
  • Image entire aorta every 3-5y
    • Preferred modality: MRI if < 50 yo; CT if > 50 yo
  • Resume private driving 6 weeks and commercial 3 months after open repair
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