Coarctation of the Aorta Flashcards

1
Q

What is CoAo of the Aorta?

A
  • Characterised by discrete narrowing in descending aorta, just distal to left subclavian artery and adjacent to ligament arteriosum
  • Accounts for around 6-8% of CHD
  • Anatomic variation include location and type of narrowing
  • CoAo may occur a a long segment stenosis or diffuse hypoplasia, and rarely may occur in ascending or abdominal aorta
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2
Q

Common associated CHDs with CoAo?

A
  • BAV (50% CoAo)
  • Congenital AS (any level)
  • VSD
  • PDA
  • Part of Shone syndrome
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3
Q

Common associated syndromes with CoAo?

A
  • Goldenhar syndrome
  • Turner syndrome
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4
Q

What are the two proposed mechanisms for the aetiology of CoAo?

A
  1. Ectopic Ductal Tissue Theory
  2. Reduced-Flow (Haemodynamic) Theory
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5
Q

Mechanism for CoAo based on ectopic ductal tissue theory?

A
  • Muscle tissue of ductus arteriosus ‘invades’ wall of aorta just distal to aortic isthmus
  • When DA contracts after birth, ductal muscle in aorta also contracts, forming a shelf or indentation in descending thoracic aorta
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6
Q

Mechanism for CoAo based on reduced flow theory?

A
  • CoAo occurs due to reduced flow in aortic isthmus during foetal circulation
  • Failure or aortic isthmus to enlarge after birth = narrowing persists as a coarctation
  • Following closure of PDA, isthmus normally enlarges until it is the same diameter as aorta on either side of it, however, if this enlargement doesn’t occur –> narrowing persists as a coarctation
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7
Q

Sites of CoAo?

A
  1. Pre-ductal (before ductus arteriosus)
  2. Juxta-ductal (at ligament arteriosum)
  3. Post-ductal (after ductus arteriosus)
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8
Q

Types of CoAo?

A
  1. Discrete stenosis (most common)
  2. Tubular hypoplasia (associated with LVOTOB or VSD)
  3. Pseudo-coarct (non-obstructive kinking or buckling of aorta distal to origin of left subclavian artery; usually seen on CT or CMR)
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9
Q

Diagnosing CoAo with echo?

A
  • CoAo diagnosis base on visualisation of shelf-like narrowing of aorta or tissue ridge extending into aortic lumen
  • Best visualised from suprasternal window
  • CFI confirms CoAo; turbulent flow through region of narrowing
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10
Q

Assessing severity of CoAo?

A
  • Measure peak velocity and maximum PG across narrowing using CW
  • Simplified Bernoulli equation: PG = 4V^2 (can only use when velocity of flow proximal to the narrowing, V1, is < 1.2m/s)
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11
Q

Expanded Bernoulli Equation?

A
  • Pressure gradient = 4(V2^2 - V1^2)
  • Use when V1 ≥ 1.2m/s
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12
Q

Limitation of CoAo Pressure Gradient: Collaterals?

A
  • Collateral circulation: blood flow diverted to internal thoracic and intercostal arteries
  • Therefore, blood flow effectively bypasses CoAo
  • Pressure gradient across CoAo reduced and severity may be underestimated
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13
Q

Limitation of CoAo Pressure Gradient: PDA?

A
  • PDA: blood shunted from aorta to PA
  • Therefore, blood flow across CoAo diminished
  • Pressure gradient across CoAo reduced and severity may be underestimated
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14
Q

False Negatives and False Positives: CoAo?

A
  • False positive: aorta images from oblique plane - ‘pseudo shelf’
  • False negative: inability to image distal descending thoracic aorta (CoAo site missed)
  • Incidence of false +/-ives reduced by using CW Doppler and CFI to interrogate descending aorta
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15
Q

Severe CoAo: CW Descending Aorta?

A
  • Persistent PG across narrowing over both systole and diastole
  • Diastolic tail/saw-tooth pattern suggests severe stenosis and collateral circulation which is clinically significant irrespective of peak velocity
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16
Q

Normal vs Severe CoAo: PW Abdominal Aorta?

A
  • Normal: rapid upstroke in early systole with flow terminating at end T-wave on ECG
  • Severe CoAo: forward flow velocity blunted, slow upstroke, so time to peak systolic velocity is delayed, and persistent forward flow throughout diastole
  • Note: diastolic flow can also be seen with CFI
17
Q

CoAo is significant when?

A

Mean Doppler systolic gradient:
- > 20mmHg
- > 10mmHg + either deceased LV systolic function or AR
- > 10mmHg with collateral flow

  • These findings in conjunction with systemic HTN, upper extremity to lower extremity BP differences and anatomic evidence of CoAo provide best evidence for intervention and surgery
18
Q

Percutaneous repair of CoAo?

A
  • Stent
  • Balloon angioplasty
19
Q

Surgical repair of CoAo?

A
  • End to end anastomosis
  • Patch repair
20
Q

Post repair CoAo echo needs to assess?

A
  • LV size, systolic function, wall thickness and evaluation of repair site
  • Identify potential post-op complications such as re-coarctation, and formation of an aortic aneurysm (best evaluated by CMR)
  • Consider aortic arch remodelling
21
Q

Types of aortic arch remodelling?

A
  1. Gothic arch: angular geometry (Doppler assessment difficult due to acute angle of arch - parallel alignment not possible)
  2. Crenel arch: rectangular shape
  3. Romanesque arch: smooth round shape similar to normal arch anatomy