Coarctation of the aorta in the newborn

What it is

Coarctation of the aorta is a narrowing of the aorta at the junction of the arch with the descending aorta, preductal or postductal (98%). In the classification of congenital heart disease it is non-cyanotic, and within that it belongs to the outflow obstruction group alongside AS and PS, not to the left to right shunts.

Causes and risk

It is one of the CHD of Turner syndrome (45 XO), listed there as bicuspid valve and coarctation of aorta.

How it presents

On the first day of life it can appear as sudden onset tachypnea and poor feeding, with poor perfusion, weak femoral pulses, and a significant discrepancy between upper and lower limbs blood pressure. When it is critical the baby is sick, with severe heart failure, absent femoral pulses (radio femoral delay) and severe metabolic acidosis.

The other manifestations are:

• Radio-femoral delay.
• Weak pulse in the lower extremities.
• Fall in systolic BP in the lower extremities compared to the upper extremities.
• Systemic HTN in the upper extremities.
• Loud aortic S2.
• Systolic ejection murmur, and a systolic ejection click.

The main clinical features are the pulses and the upper extremity HTN. The murmur is systolic and loud in the back, so upper limb hypertension with a murmur between the scapulae should bring coarctation to mind first.