Aortic coarctation is a narrowing of the aorta that commonly occurs near the insertion of the ductus arteriosus. It accounts for 6-8% of congenital heart defects. Diagnosis involves physical exam findings like hypertension and diminished pulses as well as imaging tests like echocardiogram and CT/MRI angiography. Treatment depends on age and severity, ranging from surgical repair for critical cases to balloon angioplasty or stenting for older children with non-critical coarctation. Long term follow up is needed due to risks of complications like recoarctation and hypertension.
2. Aim of the
Presentation
• Define the disease and the classifications
• Mention the epidemiology
• Discuss the etiology
• Discuss the diagnostic approach and differential diagnosis
• Discuss the treatment approach
• Discuss the recommended follow-up and possible
complications
• Prevention?
3. Definition
• Narrowing of the aorta
• Mostly at side of DA insertion
(juxtaductal)
• Less like preductal, abdominal
or infrarenal
• 6-8% of CHD
Am J Med Genet A. 2005;134A:180-186.
• Males > females
• Mostly sporadic but could be familial
Am J Med Genet A. 2005;134A:171-179.
• Could be associated with others sydromes or CHD
4. Etiology
• Incompletely understood
∀ ↓ blood flow to the CoA site in the fetal life → under-
development
• Ductal tissue extends into the thoracic aorta, and, when the
ductus arteriosus constricts and closes postnatally, the
thoracic aorta is constricted.
Am J Cardiol. 1972;30:514-525
8. History and examination
Diagnostic factors Risk factors
Common Strong
presence of risk factors
hypertension presenting at a
young age or resistant to treatment
diminished lower extremity pulses
differential upper and lower
extremity BPs
systolic ejection murmur
male
young age
Turner's syndrome
DiGeorge's syndrome
hypoplastic left heart syndrome
Shone's complex
PHACE syndrome
Uncommon Weak
claudication
headache
systolic ejection click
Positive family history
10. Diagnostic Tests
1st tests to order
Test Result
Echocardiogram
discrete narrowing in the thoracic aorta;
pressure gradient across narrowing
CXR
age and severity dependent; may be
normal, have cardiomegaly, or show
posterior rib notching
ECG may be normal; show RVH or LVH
11. Diagnostic Tests
Tests to consider
Test Result
CT angiography abnormal anatomy of aortic arch
magnetic resonance angiography abnormal anatomy of aortic arch
cardiac catheterisation
abnormal gradient across narrowing;
therapeutic intervention possible
14. Differential Diagnosis
Condition
Differentiating
signs/symptoms
Differentiating tests
Aortic stenosis (AS)
With mild AS, the patient may
be asymptomatic with a
harsh systolic ejection
quality murmur at the
upper right sternal border
with radiation to the
carotids on physical
examination. In moderate
or severe AS, the patient
may have significant SOB,
especially on exertion.
• Differentiated from
coarctation of the aorta by
echocardiogram.
• Two-dimensional
echocardiogram of the
aortic valve shows an
abnormally narrowed or
dysplastic aortic valve.
• Patients with aortic
coarctation with
associated bicuspid aortic
valve may also have AS.
15. Differential Diagnosis
Condition
Differentiating
signs/symptoms
Differentiating tests
Left ventricular
outflow tract
obstruction
Depending on the level and
severity of the obstruction,
patients may be
asymptomatic or may
present with SOB,
especially on exertion.
• An echocardiogram will
differentiate the level of
the obstructed or
narrowed area in the
aorta. Using colour and
pulse Doppler, any
narrowing in the left
ventricular outflow tract
can be localised to the
valvar, sub-valvar, or
supra-valvar area, or
across the aortic arch.
16. Differential Diagnosis
Condition
Differentiating
signs/symptoms
Differentiating tests
Essential
hypertension
Similar clinical presentation.
Lower extremity pulses usually
normal unless peripheral
vascular disease present.
Four-extremity BP shows no
gradient. Echocardiogram
shows normal flow across the
aortic arch and normal Doppler
of the abdominal aorta.
Renal artery stenosis
• Similar clinical
presentation.
• Renal artery Doppler
shows renal artery
stenosis.
• Normal echocardiogram.
17. Treatment Approach
• Critical Coarctation
• Non-critical coarctation < 1 year of age
• Non-critical coarctation > 1 year of age
18. Treatment Approach
Patient Treatment
Critical CoA Keep PDA PLUS surgical repair
Non-critical < 1 year Surgical repair
Non-critical > 1 year Surgical OR percutaneus repair
Recurrent Percutaneus ballon angioplasty
21. Percutaneus repair
• For children > 1 year of age or recurrent coarctation
• By ballon angioplasty with or without stent implantation
• Stent implantation only in older childrens
23. Prevention
• routine palpation of femoral pulses starting with the first
nursery visit.
• Any question of a differential in the pulses or an elevated
upper extremity BP should prompt measurement of BP in all 4
extremities
• Adults with a diagnosis of an intracranial berry aneurysm
should have their BP measured
• genetic evaluation is also warranted when there are
dysmorphic features, multiple organ abnormalities or
additional intracardiac or vascular abnormalities
• Prophylaxis for infective endocarditis is recommended for 6
months after intervention or surgery is performed
Circulation. 2007;116:1736-1754
24. conclusion
• Aortic CoA is a common CHD
• A careful nursery physical exam could prevent fatal
complications
• Treatment options depend on the severity and length of the
narrowing, the age of patient.
• Post-op complications is uncommon but could be dangerous or
could affect the further quality of life
• Close follow-up with pediatrics cardiologist is required for all
patients
