1. Aortic Coarctation Khaled Ghanem, M.D

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

5. Etiology

6. Diagnostic Approach History and examination Diagnostic tests Differential diagnosis

7. Diagnostic Approach History and examination Diagnostic tests Differential diagnosis

8. History and examination Diagnostic factorsRisk factors CommonStrong  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 UncommonWeak  claudication  headache  systolic ejection click  Positive family history

9. Diagnostic Approach History and examination Diagnostic tests Differential diagnosis

10. Diagnostic Tests 1st tests to order TestResult 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 TestResult CT angiography abnormal anatomy of aortic arch magnetic resonance angiography abnormal anatomy of aortic arch cardiac catheterisation abnormal gradient across narrowing; therapeutic intervention possible

12. Diagnostic Tests

13. Diagnostic Approach History and examination Diagnostic tests Differential diagnosis

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 PatientTreatment Critical CoAKeep PDA PLUS surgical repair Non-critical < 1 yearSurgical repair Non-critical > 1 yearSurgical OR percutaneus repair RecurrentPercutaneus ballon angioplasty

19. Surgical Repair - 1 For short segment narrowing

20. Surgical Repair - 2 For medium-length and long narrowing

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

22. 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

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

25. Thank you