Ten key facts about coarctation of the aorta

Coarctation of the aorta is a narrowing of the aorta, classically (but not always) just distal to the origin of the left subclavian artery. Here are ten key facts about coarctation of the aorta to be aware of in your cardiology clinic.

MR aortogram showing coarctation of the aorta plus collaterals

1. Coarctation of the aorta accounts for 5-8% of congenital heart defects, with a prevalence of around 0.03% of live births
2. The location of coarctation of the aorta is most commonly at the insertion point of the ductus arteriosus (just distal to the origin of the left subclavian artery)
3. Ectopic coarctation can sometimes occur in the ascending aorta, the descending aorta or even the abdominal aorta
4. The region of coarctation is most commonly discrete, but it can sometimes extend over a long segment of the aorta. There is often some dilatation of the aorta immediately beyond the coarctation
5. Bicuspid aortic valve is a frequent association with coarctation of the aorta, reported in up to 85% of cases. Other associations with coarctation of the aorta include Turner syndrome, aortic stenosis (subvalvular, valvular or supravalvular), Shone syndrome and neurofibromatosis
6. Mild cases of coarctation of the aorta may not present until adulthood. Common presentations include incidental hypertension (affecting the upper body) or a murmur
7. A blood pressure gradient >20mmHg between upper and lower limbs indicates significant coarctation of the aorta
8. Chest X-ray findings include rib notching (caused by collaterals) and a double contour of the descending aorta ('figure 3 sign')
9. Both MRI (see image above) and cardiac CT are useful non-invasive methods for imaging the anatomy of coarctation of the aorta, and the presence of collateral vessels
10. Treatment options include stenting, resection with end-to-end anastomosis, and resection with interposition of a graft

After primary repair of coarctation of the aorta, long-term survival remains below that of normal controls, and the need for re-operation is relatively common. Patients should therefore remain under lifelong surveillance after surgical repair.

For further reading about the importance of ongoing surveillance after surgical repair, read the following reference:

Brown ML, et al. Coarctation of the aorta: Lifelong surveillance is mandatory following surgical repair. J Am Coll Cardiol 2013; 62: 1020-1025.

Ten key facts about bicuspid aortic valve disease

Bicuspid aortic valve disease (BAVD) is a common problem in which the aortic valve has two, rather than three, cusps. BAVD is frequently picked up as an incidental finding during cardiac imaging. Here are Ten Key Facts about BAVD that you should be aware of in your cardiology clinic:

Transoesophageal echo showing bicuspid aortic valve en face

1. BAVD affects about 2% of the population
2. This means that BAVD is the most common congenital cardiac abnormality
3. BAVD is around 2x commoner in males
4. BAVD has been associated with an abnormality of the NOTCH1 gene
5. The most frequent complication of BAVD is aortic stenosis
6. Around one-third of patients with BAVD will experience a serious complication of the condition (aortic stenosis or regurgitation, infective endocarditis, aortic aneurysm or dissection)
7. BAVD is not just an abnormality of the aortic valve, but is a disease of the aortic root and ascending aorta as well
8. Patients with BAVD have a 9x greater risk of aortic dissection than those with a normal aortic valve
9. BAVD is present in around 50% of patients with coarctation of the aorta
10. Once diagnosed, patients with BAVD should remain under long-term surveillance which regular imaging assessment of their aortic valve function and aortic root/ascending aortic dimensions

A detailed overview of BAVD, which includes key references, can be accessed from the European Society of Cardiology by clicking here.