Aortic coarctation

Question 1

What is the most common abnormality that accompanies Aortic Coarctation?

Some others?

Answer 1

Bicuspid aortic valve, in 85%

Turner’s syndrome
Williams syndrome
Retroesophageal right sublcavian artery

Question 2

Heart consequences of aortic coarctation

Answer 2

LV hypertrophy, subsequent dysfunciton.

Cystic medial necrosis of the preceeding ascending aorta and the coarcted segment (raised tension prevents flow into the vessels supplying aorta)

Question 3

Extracardiac consequences of aortic coarctation

Answer 3

Collateral criculation development, increased size of internal mammary/thoracic, intercostal arteries.

Assymetric blood pressure

Gradient more than 20mmHg difference beween upper and lower limbs. —This gradient is not always seen due to the compensating collateral arteries developed.

Radial/Femoral pulse delay

Question 4

Prognosis of aortic coarctation

Answer 4

1.6% mortality per year ages 10-20
7% per year over age 60.

25% by 20
50% by 32
75% by 46
92% by 60

Question 5

Adulthood consequences of CoA

Answer 5

Late diagnosed coarctation
Re-coarctation - occurs in about 1/3rd even after balloon angioplasty or surgical repair.

Hypertension - The earlier it is diagnosed and repaired, the lower chance of hypertension later in life.
20-40% are hypertensive by Teenage years.
70% by age 30. (even if it is repaired in childhood)

Aneurysm at coarctation site, aortic dissections or ruptures.

Aortic stenosis - from bifid aortic valve

Berry aneurysms and hemmorhagic strokes. BA in 7-10%. CoA patients should be screened at least once.
Severe risk for aneurysms abouve 7mm.

Premature coronary artery disease/ischemic heart disease. Main cause of death is MI. 40% develop CAD.

Abnormal blood pressure response to exercise in 20%

Question 6

Gold standard for Coarctation diagnosis

Answer 6

Cardiac catheterization and coronary/aortic angiography.

MRI/CT scans.

Question 7

Criteria for significant CoA

Answer 7

> 20mmHg limb asymmetry

Coarctation is 50% or smaller than the aortic diameter at the diaphragm.

Aneurysm formation

Recurrent coarctation

Question 8

Treatment for CoA in adults

Answer 8

For a newly diagnosed native coarctation, Surgery preferred.
For native repair, the risk of aneurysm after stenting/ballooning is higher

Re-coarctation - Percutaneous Stenting is preferred.

Ballooning has only 18% early complications, but about 25% chance of Re-CoA and aneurysm.

Stenting has high success and only 9% aneurysm late.

Stenting with a coated stent, hydrophobic covered polymer, is now the preferred method. (Not a DES)

Resection and interposition grafting - All will develop graft dilation, ~8% with false aneurysm
But, restenosis is very rare.

Question 9

Potential complications of stenting

Answer 9

Recoartcation

Pseudoaneurysm formation (bleeding out of an artery that is contained by surrounding tissue)

Aortic Rupture

Femoral artery thrombosis/ischemia

Question 10

Complications after repair of native CoA

Answer 10

Spinal cord ischemia due to underdeveloped collaterals

Rebound hypertension

Recrrent laryngeal nerve damage

Phrenic nerve damage, diaphragm paralysis

Aneurysm

Arm claudication/ischemia

Question 11

Post op/repair follow ups for CoA

Answer 11

At least one MRI or CT to screen for aneurysms.
-Every 3-5 years with resection graft repair

Any significant new onset headache, re-screen.

Regular followups for cath/angiography

Regular monitor blood pressure.