Coarctation of the Aorta and Hypertension

Pathophysiology and Presentation

Coarctation of the aorta manifests as a discrete stenosis or hypoplastic segment typically located at the insertion of the ductus arteriosus, causing obstruction to blood flow 1
The natural course of CoA is largely driven by hypertension-related complications, including heart failure, intracranial hemorrhage, premature coronary/cerebral artery disease, and aortic rupture/dissection 1
Symptoms of CoA reflect pre-stenotic hypertension and post-stenotic hypoperfusion 1
Mild cases may only become evident in adulthood when hypertension is detected 2

Hypertension remains an important complication even after successful treatment of CoA, and is more common when initial repair is performed in adulthood 2
The mechanism of hypertension in CoA is multifactorial and includes mechanical obstruction in native or recurrent coarctation 1, 3

Blood pressure measurements at both arms and one lower extremity are recommended to detect pressure gradients 1, 3
Significant CoA is indicated by a non-invasive right arm-to-leg gradient >35 mmHg peak or >20 mmHg mean 1
Significant CoA is also indicated by >50% narrowing on any imaging modality 1
Invasive peak-to-peak gradient >20 mmHg is also an indicator of significant CoA 1, 3
Imaging with CMR or CTA is recommended for initial and follow-up evaluation of CoA 3, 4
Ambulatory blood pressure monitoring can be useful for diagnosis and management of hypertension in CoA patients 3, 4

Coarctation or re-coarctation repair is indicated in patients with hypertension with an increased gradient between upper and lower limbs confirmed with invasive measurement 1, 3
Endovascular treatment with stenting is the first-choice treatment for native CoA and re-coarctation in adults when technically feasible 3, 2
The European Society of Cardiology recommends treating hypertension in patients with CoA according to hypertension guidelines 1, 5

All CoA patients require lifelong follow-up, even after successful repair 1, 3
Regular imaging of the aorta with CMR/CCT every 3–5 years is recommended 1, 3
Follow-up is essential to document post-repair or post-interventional complications such as re-coarctation, aneurysms, or pseudoaneurysms 1, 2
The American College of Cardiology recommends screening for intracranial aneurysms by magnetic resonance angiography or CTA in adults with CoA 1, 3

Aortic dissection has been reported in association with coarctation of the aorta, occurring both above and below the coarctation site 6
This complication is not typically seen during childhood but develops as a complication of untreated coarctation—usually in the third decade of life or later 7
Hypertension is a critical factor in the development of aortic dissection in patients with coarctation history 8

Untreated coarctation poses a particularly high risk, with complications typically emerging in the third decade or later 7
Pregnancy represents a period of increased risk, with dissection involving the descending aorta below the coarctation site reported as a complication 6

Dissection can occur both proximal and distal to the coarctation site 6, 7
The risk increases with age, with most cases occurring in adulthood rather than childhood 8
According to the European Heart Journal guidelines, this complication is not seen in childhood but develops in adulthood, particularly in association with hypertension 6

According to the law of La Place, wall stress is directly proportional to pressure and radius and inversely proportional to vessel wall thickness, making hypertension a significant factor in aortic wall weakening 6
Primary balloon dilatation of aortic coarctation and dilatation of re-coarctation may produce damage to the aorta with subsequent development of aneurysms 6
A degree of intimal tearing and dissection can occur during interventional procedures 6