Overview
Definition
Congenital narrowing of the aorta, typically occurring near the insertion of the ductus arteriosus.
Epidemiology
Accounts for 5-8% of congenital heart defects. Strongly associated with Turner Syndrome.
Etiology & Risk Factors
- Abnormal development of aortic arch during embryogenesis
- Association with bicuspid aortic valve in 50-80% of cases
Clinical Symptoms
- Hypertension in arms with low BP in legs
- Delayed or weak femoral pulses ('radial-femoral delay')
- Cold feet
- Headaches
- Leg claudication on exertion
Clinical Approach
Diagnosis
- Echocardiography
- Chest X-Ray showing '3 sign' and rib notching (from collateral intercostal flow)
- MRA/CTA
Management
- Surgical repair (resection and end-to-end anastomosis)
- Balloon angioplasty / Stenting in older children/adults
Complications
- Severe Hypertension
- Cerebral berry aneurysm rupture / Stroke
- Aortic rupture
- Infective Endocarditis