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Aortic Coarctation

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					Aortic Coarctation
   Khaled Ghanem, M.D
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?
               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
               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             male
 hypertension presenting at a         young age
young age or resistant to treatment    Turner's syndrome
 diminished lower extremity pulses    DiGeorge's syndrome
 differential upper and lower         hypoplastic left heart syndrome
extremity BPs                          Shone's complex
 systolic ejection murmur             PHACE syndrome


Uncommon                              Weak
 claudication                         Positive family history
 headache
 systolic ejection click
               Diagnostic Approach

•   History and examination

• Diagnostic tests

•   Differential diagnosis
 Diagnostic Tests

                 1st tests to order
     Test                                   Result
                          discrete narrowing in the thoracic aorta;
Echocardiogram
                          pressure gradient across narrowing

                          age and severity dependent; may be
    CXR                   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


                                 abnormal gradient across narrowing;
    cardiac catheterisation
                                 therapeutic intervention possible
Diagnostic Tests
              Diagnostic Approach

•   History and examination

•   Diagnostic tests

• Differential diagnosis
                 Differential Diagnosis

                              Differentiating
     Condition                                              Differentiating tests
                             signs/symptoms

                                                           Differentiated from
                       With mild AS, the patient may        coarctation of the aorta by
                           be asymptomatic with a           echocardiogram.
                           harsh systolic ejection
                                                           Two-dimensional
                           quality murmur at the
                                                            echocardiogram of the
                           upper right sternal border
                                                            aortic valve shows an
Aortic stenosis (AS)       with radiation to the
                                                            abnormally narrowed or
                           carotids on physical
                                                            dysplastic aortic valve.
                           examination. In moderate
                           or severe AS, the patient       Patients with aortic
                           may have significant SOB,        coarctation with
                           especially on exertion.          associated bicuspid aortic
                                                            valve may also have AS.
                   Differential Diagnosis

                                  Differentiating
       Condition                                                 Differentiating tests
                                 signs/symptoms

                                                                An echocardiogram will
                                                                 differentiate the level of
                                                                 the obstructed or narrowed
                           Depending on the level and
                                                                 area in the aorta. Using
                              severity of the obstruction,
                                                                 colour and pulse Doppler,
Left ventricular outflow      patients may be
                                                                 any narrowing in the left
   tract obstruction          asymptomatic or may
                                                                 ventricular outflow tract
                              present with SOB,
                                                                 can be localised to the
                              especially on exertion.
                                                                 valvar, sub-valvar, or
                                                                 supra-valvar area, or
                                                                 across the aortic arch.
                   Differential Diagnosis

                                Differentiating
       Condition                                            Differentiating tests
                               signs/symptoms
                                                        Four-extremity BP shows no
                         Similar clinical presentation.
                                                        gradient. Echocardiogram
                         Lower extremity pulses usually
Essential hypertension   normal unless peripheral
                                                        shows normal flow across the
                                                        aortic arch and normal Doppler
                         vascular disease present.
                                                        of the abdominal aorta.



                                                           Renal artery Doppler
                            Similar clinical               shows renal artery
Renal artery stenosis        presentation.                  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

				
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posted:4/12/2013
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