Can coarctation of the aorta cause hypertension?

Coarctation of the Aorta and Hypertension

Yes, coarctation of the aorta is a significant cause of hypertension, with hypertension being the most common sequela of coarctation whether repaired or unrepaired. 1

Pathophysiology and Presentation

• Coarctation of the aorta (CoA) 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 (headache, nosebleeds) and post-stenotic hypoperfusion (abdominal angina, claudication) 1
• Mild cases may only become evident in adulthood when hypertension is detected 1

Hypertension in CoA

• Hypertension remains an important complication even after successful treatment of CoA, and is more common when initial repair is performed in adulthood 1
• Studies show that the prevalence of hypertension after CoA repair ranges from 20-70%, with a mean of 47.3% 2
• When 24-hour blood pressure monitoring is used for assessment, the incidence of hypertension rises to 57.8% 2
• The mechanism of hypertension in CoA is multifactorial and includes:
  • Mechanical obstruction in native or recurrent coarctation 1
  • Vascular dysfunction and reduced arterial compliance 3
  • Abnormal central aortic hemodynamics that persist even after repair 2

Diagnosis of Significant CoA

• Blood pressure measurements at both arms and one lower extremity are recommended to detect pressure gradients 1
• Significant CoA is indicated by:
  • Non-invasive right arm-to-leg gradient >35 mmHg peak or >20 mmHg mean 1

  • 50% narrowing on any imaging modality 1

  • Invasive peak-to-peak gradient >20 mmHg 1
• Imaging with CMR or CTA is recommended for initial and follow-up evaluation of CoA 1
• Ambulatory blood pressure monitoring can be useful for diagnosis and management of hypertension in CoA patients 1

Management of Hypertension in CoA

Interventional Treatment

• Coarctation or re-coarctation repair (either surgical or endovascular) is indicated in patients with hypertension with an increased gradient between upper and lower limbs confirmed with invasive measurement (peak-to-peak >20 mmHg) 1
• Endovascular treatment with stenting is the first-choice treatment for native CoA and re-coarctation in adults when technically feasible 1
• Surgical repair is recommended when stenting is less suitable 1
• Endovascular treatment should be considered in patients with hypertension with >50% narrowing relative to the aortic diameter at the diaphragm, even if the invasive peak-to-peak gradient is <20 mmHg 1

Medical Management

• It is recommended to treat hypertension in patients with CoA according to hypertension guidelines 1
• Medical therapy for hypertension due to CoA parallels treatment of essential hypertension with beta blockers, angiotensin-converting enzyme inhibitors, and angiotensin receptor blockers as first-line agents 4
• Right arm 24-hour ambulatory blood pressure measurement or exercise tests better detect hypertension in CoA patients 1

Follow-up

• All CoA patients require lifelong follow-up, even after successful repair 1
• Regular imaging of the aorta with CMR/CCT every 3–5 years is recommended, adjusted to previous imaging findings and type of intervention 1
• Follow-up is essential to document post-repair or post-interventional complications such as re-coarctation, aneurysms, or pseudoaneurysms 1
• Patch repairs are at particular risk of repair-site para-anastomotic aneurysms or pseudo-aneurysms 1

Clinical Pearls and Pitfalls

• Even with excellent repair, hypertension remains common and predisposes to later myocardial infarction, stroke, and heart failure 1
• Peripheral blood pressure in CoA patients has a poor correlation with central aortic pressure, which may lead to underestimation of the true hemodynamic burden 2
• Hypertension in CoA has a progressive character, making long-term surveillance crucial 2
• Screening for intracranial aneurysms by magnetic resonance angiography or CTA may be reasonable in adults with CoA, as they occur in approximately 10% of patients 1