What is the recommended treatment for a patient with coarctation of the aorta?

Coarctation of the Aorta: Treatment Recommendations

For patients with coarctation of the aorta, endovascular stent placement is the first-choice treatment in adults and older children (>25 kg) when technically feasible, indicated by hypertension with an invasive peak-to-peak gradient >20 mmHg or >50% aortic narrowing. 1, 2

Diagnostic Confirmation

Before any intervention, confirm the diagnosis with specific measurements:

• Measure blood pressure in both arms and one lower extremity to document the characteristic upper-to-lower extremity gradient 1, 3
• Look for diminished or delayed femoral pulses compared to brachial pulses, systolic murmur over the back, and upper extremity hypertension with lower extremity hypotension 4
• Obtain CMR or cardiac CT of the entire thoracic aorta to define coarctation anatomy, assess for arch hypoplasia, identify collateral vessels, detect aneurysms, and screen for associated bicuspid aortic valve 2, 4
• Confirm hemodynamic significance with invasive catheterization showing peak-to-peak gradient >20 mmHg 1, 2

Indications for Intervention

Intervention is indicated in the following scenarios:

• Hypertension (>140/90 mmHg in adults) PLUS invasive peak-to-peak gradient >20 mmHg 1, 2
• Hypertension PLUS >50% aortic narrowing relative to diameter at the diaphragm level, even if invasive gradient is <20 mmHg 1, 2
• Normotensive patients with invasive peak-to-peak gradient >20 mmHg 1, 2
• Peak-to-peak gradient <20 mmHg but with anatomic imaging evidence of significant coarctation AND radiological evidence of significant collateral flow 1

Treatment Approach by Patient Characteristics

Adults and Older Children (>25 kg)

Transcatheter treatment with covered stent placement is the first-choice treatment 1, 2, 4. The 2024 ESC guidelines give preference to stenting when technically feasible, with a 98.6% success rate and low complication rates (11.7% total complications, 0.3% mortality) 1, 5

Recurrent/Residual Coarctation After Prior Repair

Percutaneous catheter intervention with stenting is indicated for discrete recoarctation with peak-to-peak gradient ≥20 mmHg 1. This has become the gold standard for post-surgical recoarctation 1

When Endovascular Treatment is Not Feasible

Surgery is indicated for:

• Long recoarctation segments 1
• Concomitant hypoplasia of the aortic arch 1
• Women of childbearing age 1
• Associated aneurysm or transverse arch hypoplasia 1

Surgical technique: Excision of narrowed segment with interposition graft is the preferred approach in adults 1

Medical Management of Hypertension

All patients with coarctation require aggressive blood pressure control:

• Beta-blockers are the most useful first-line agents 1, 4
• ACE inhibitors or angiotensin-receptor blockers as standard second-line therapy 1, 4
• The choice between beta-blockers and vasodilators may be influenced by aortic root size and presence of aortic regurgitation 1
• Treat hypertension according to standard ESC hypertension guidelines 1, 2, 4

Important Caveat on Hypertension Diagnosis

Use 24-hour ambulatory blood pressure monitoring to confirm true resistant hypertension, as white-coat hypertension accounts for approximately 50% of apparent resistant cases 4. Exercise testing may also better detect hypertension than office measurements alone 2, 4

Lifelong Surveillance Requirements

All coarctation patients require lifelong follow-up regardless of treatment success:

• CMR or cardiac CT every 3-5 years to monitor for recoarctation, aneurysms, pseudoaneurysms, and dissection 1, 3, 2, 4
• At least yearly cardiology evaluations for all patients who have had repair 1
• Monitor closely for resting or exercise-induced systemic arterial hypertension, which should be treated aggressively after recoarctation is excluded 1
• Surveillance for bicuspid aortic valve dysfunction and ascending aortic dilation, present in 30-85% of coarctation patients 1, 2

Screening for Intracranial Aneurysms

The 2024 ESC guidelines state there is no evidence supporting routine screening for intracerebral aneurysms in asymptomatic patients 1. However, the ACC suggests screening by MRA or CTA may be reasonable given the 10% prevalence 1, 2. This remains controversial and should be discussed with patients individually.

Critical Prognostic Information

The natural course of untreated coarctation is poor:

• Unoperated survival averages 35 years of age 1
• 75% mortality by 46 years of age 1
• Complications include heart failure, intracranial hemorrhage, premature coronary/cerebral artery disease, and aortic rupture/dissection 1, 3

Hypertension remains common even after successful repair, particularly when initial repair is performed in adulthood 3. This underscores the critical importance of lifelong blood pressure monitoring and aggressive treatment.

Common Pitfalls to Avoid

• Do not rely solely on office blood pressure measurements – use ambulatory monitoring to detect true hypertension 2, 4
• Do not assume successful repair means cure – recoarctation, aneurysm formation, and persistent hypertension are common long-term complications requiring lifelong surveillance 1, 3
• Do not overlook associated cardiac lesions, particularly bicuspid aortic valve (present in 30-85% of cases) 1, 2
• Do not use balloon angioplasty alone in adults – stenting has superior outcomes with lower recurrence rates and aneurysm formation compared to balloon angioplasty alone 1, 2, 5