Overview
Coarctation of the aorta (CoA) describes congenital narrowing of the aorta, most commonly where the ductus arteriosus inserts. If the narrowing is severe, it can present with shock at birth once the ductus arteriosus closes.
Risk Factors
- Associated with Turner’s syndrome
- Associated with DiGeorge’s syndrome
- More common in males, despite its association with Turner’s syndrome
Presentation
The presence and severity of symptoms depend on the severity of the narrowing of the aorta. Some features include:
- In infants: poor feeding, lethargy, tachypnoea, and heart failure
- In adults: hypertension at a young age or resistant to treatment
- Diminished/absent lower limb pulses
- A higher blood pressure in the upper limbs compared to the lower limbs
- Claudication due to reduced blood flow in the lower limbs
- An ejection systolic click if associated with a bicuspid aortic valve
Investigations
Investigations include:
- ECG:
- May show right ventricular or left ventricular hypertrophy
- Chest x-ray:
- May show cardiomegaly
- May show posterior rib notching – seen in older children/adults due to collateral vessel enlargement
- Echocardiography – diagnostic test of choice:
- Visualises CoA
Management
Overview
Management may involve:
- In neonates with critical coarctation:
- Prostaglandin E1 (e.g. alprostadil) to keep the ductus arteriosus open to buy time for surgery
- Non-critical coarctation:
- Surgical/percutaneous repair
Complications
Overview
Some complications include:
- Hypertension
- Congestive cardiac failure
- Recurrent coarctation
Prognosis
- If untreated, the prognosis is very poor and few people survive beyond 50 years of age
