- Congenital narrowing at the junction of the aortic arch and descending aorta, secondary to a fibrous ridge protruding into the aortic lumen.
- 80% male.
- 50% associated with other congenital anomalies:
- Bicuspid aortic valve.
- Turner’s syndrome.
- PDA, VSD.
- Cerebral Berry aneurysms.
- Second most common cause of cardiac failure in neonates.
- May remain undetected well into adult life.
- Symptomatic presentation in neonates includes tachypnoea, cyanosis and generalised oedema. Patients require urgent corrective surgery. Most cases are now identified on neonatal screening.
- Delayed presentation in adults includes hypertension, headaches, claudication, cardiac failure and renal impairment.
- Patients may have cardiac systolic heart murmurs sometimes heard over the back and ‘pistol shot’ femoral pulses.
- Neonates – cardiomegaly and pulmonary plethora.
- Adults – CXR
- Inferior rib notching (ribs 3–9), may be unilateral depending on where the right subclavian branches from the aorta. Only visible after 7 years of age.
- Small aortic knuckle.
- Figure-3 indentation on the left lateral wall of the aortic arch. The reverse-3 is present on barium studies.
- Elevated left ventricular apex secondary to hypertrophy.
- MRI is very good at characterising the coarctation and associated cardiac anomalies.
- The extent of the stenosis is very difficult to assess on imaging.
- Pseudo-coarctation – no pressure gradient across a narrowing in the aortic calibre – no obstruction.
- Inflammatory conditions such as Takayasu’s arteritis can have similar radiological features.
- Chronic SVC, IVC or subclavian artery obstruction may have similar radiological features.
- Angioplasty and stenting.
- Surgical repair.
- Hypertension control.