Aortic CoarctationAortic CoarctationKhaled Ghanem, M.DKhaled Ghanem, M.D

Aim of the PresentationAim 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?

Definition

• Narrowing of the aorta

• Mostly at side of DA insertion (juxtaductal)

• Less like preductal, abdominal or infrarenal

• 6-8% of CHDAm J Med Genet A. 2005;134A:180-186.

• Males > females

• Mostly sporadic but could be familialAm J Med Genet A. 2005;134A:171-179.

• Could be associated with others sydromes or CHD

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

Etiology

Diagnostic Approach

• History and examination

• Diagnostic tests

• Differential diagnosis

Diagnostic Approach

• History and examination

• Diagnostic tests

• Differential diagnosis

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

Diagnostic Approach

• History and examination

• Diagnostic tests

• Differential diagnosis

Diagnostic Tests

1st tests to orderTest Result

Echocardiogramdiscrete narrowing in the thoracic aorta; pressure gradient across narrowing

CXRage and severity dependent; may be normal, have cardiomegaly, or show posterior rib notching

ECG may be normal; show RVH or LVH

Diagnostic Tests

Tests to consider

Test Result

CT angiography abnormal anatomy of aortic arch

magnetic resonance angiography

abnormal anatomy of aortic arch

cardiac catheterisationabnormal gradient across narrowing; therapeutic intervention possible

Diagnostic Tests

Diagnostic Approach

• History and examination

• Diagnostic tests

• Differential diagnosis

Differential Diagnosis

ConditionDifferentiating

signs/symptomsDifferentiating 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.

Differential Diagnosis

ConditionDifferentiating

signs/symptomsDifferentiating 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.

Differential Diagnosis

ConditionDifferentiating

signs/symptomsDifferentiating 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.

Treatment Approach

• Critical Coarctation

• Non-critical coarctation < 1 year of age

• Non-critical coarctation > 1 year of age

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

Surgical Repair - 1

• For short segment narrowing

Surgical Repair - 2

• For medium-length and long narrowing

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

Complications

• Post-op• HTN• recurrent laryngeal nerve and phrenic nerve injury • Ischemic mesenteric enteropathy• Long-term recoarctation• Paraplegia in patients with inadequate collateral

circulation• Femoral artery access-related complications• Aneurysm formation• Aortic dissection• stroke

• Others • Coronary artery disease

Am J Cardiol 2002 ;8 9:541-547

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

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

Thank you