What is the treatment for coarctation of the aorta?

Treatment of Coarctation of the Aorta

For adults and older children (>25 kg) with coarctation of the aorta, transcatheter treatment with covered stent placement is the first-choice treatment when technically feasible, with intervention indicated when there is hypertension plus a gradient >20 mmHg or >50% narrowing on imaging. 1

Indications for Intervention

Intervention is recommended in the following circumstances:

• Peak-to-peak gradient ≥20 mmHg on invasive catheterization, which remains the gold standard for confirming hemodynamic significance 2, 1
• Gradient <20 mmHg but with >50% aortic narrowing relative to the diameter at the diaphragm level in hypertensive patients (>140/90 mmHg in adults) 1
• Significant collateral vessels visible on imaging even with lower measured gradients, as collaterals can mask the true severity of obstruction 2

Treatment Approach by Clinical Scenario

Native Coarctation in Adults and Older Children

Covered stent placement is the preferred first-line treatment for patients who can receive a stent expandable to adult size (minimum 2 cm diameter) 2, 1. The choice between percutaneous intervention versus surgical repair should be determined by consultation with a multidisciplinary team at an adult congenital heart disease (ACHD) center 2.

Surgical repair remains the gold standard and may be preferred for:

• Women of childbearing age due to concerns about tissue integrity during pregnancy 2
• Long-segment or tortuous coarctation where stenting is less suitable 2
• Complex anatomy with arch hypoplasia 2

Surgical techniques include extended resection with end-to-end anastomosis, subclavian flap repair, or patch aortoplasty 2.

Recurrent/Residual Coarctation (After Prior Surgical Repair)

Percutaneous catheter intervention (balloon angioplasty with or without stent) is the preferred treatment for discrete recoarctation with a gradient ≥20 mmHg 2. This has been shown to be the best therapeutic option with lower morbidity than reoperation 2.

Surgery is reserved for:

• Long recoarctation segments 2
• Concomitant hypoplasia of the aortic arch 2
• Presence of aneurysm or pseudoaneurysm formation 2
• Failed percutaneous treatment 2

Neonates and Infants

Neonates presenting with ductal-dependent coarctation require prostaglandin infusion and supportive measures until definitive treatment 2. Surgical repair is most commonly performed in infancy and early childhood 2.

Balloon angioplasty may be considered as a palliative measure in critically ill infants with severely depressed ventricular function, severe mitral regurgitation, or low cardiac output 2. For young patients (1-6 months) with discrete narrowing and no arch hypoplasia, balloon angioplasty can be performed, though recurrence rates are higher in this age group 2.

Critical Pre-Intervention Workup

Before any intervention, obtain:

• Blood pressure measurements in both arms and one lower extremity to document gradients 1, 3
• CMR or cardiac CT to visualize the entire thoracic aorta, define coarctation anatomy, assess for arch hypoplasia, identify collateral vessels, and detect aneurysms 2, 1
• Invasive catheterization with manometry to confirm peak-to-peak gradient >20 mmHg 1
• Echocardiography to evaluate for associated lesions, particularly bicuspid aortic valve (present in >50-85% of cases) 2

Medical Management of Hypertension

Hypertension should be controlled with beta blockers, ACE inhibitors, or angiotensin-receptor blockers as first-line medications 2, 1. Beta blockers may be particularly useful to prevent rebound hypertension after repair 2. The choice may be influenced by aortic root size and presence of aortic regurgitation 2.

Lifelong Surveillance Requirements

All coarctation patients require lifelong follow-up regardless of treatment success 1, 3. This is critical because:

• Hypertension persists or recurs in many patients even after successful repair, particularly when initial repair is performed in adulthood 2, 3, 4
• Recoarctation occurs in approximately 7% after balloon angioplasty 2
• Aneurysm or pseudoaneurysm formation can develop at the repair site 2, 4
• Aortic dissection risk increases, particularly in the third decade of life or later in untreated or inadequately treated patients 3

Surveillance protocol includes:

• CMR or cardiac CT every 3-5 years to monitor for recoarctation, aneurysms, pseudoaneurysms, and dissection 1, 3
• 24-hour ambulatory blood pressure monitoring or exercise testing to detect hypertension more accurately than office measurements 1
• Echocardiographic surveillance for bicuspid aortic valve dysfunction and ascending aortic dilation 1
• Consider screening for intracranial aneurysms by MRA or CTA, though routine screening in asymptomatic patients remains controversial 1, 3

Common Pitfalls to Avoid

Do not rely solely on gradient measurements when significant collaterals are present, as femoral pulses may be less diminished and catheter-based/Doppler gradients may underestimate the true degree of obstruction 2. In these cases, anatomic imaging evidence of >50% narrowing with radiological evidence of collateral flow warrants intervention even with gradients <20 mmHg 2, 1.

Do not assume successful repair eliminates future cardiovascular risk. Coarctation is an aortopathy with abnormal vascular properties characterized by stiffness and impaired relaxation that persists despite anatomic correction 5. Patients remain at heightened risk for premature coronary and cerebral artery disease, heart failure, and aortic complications 3, 4.

Early mortality for primary operation is <1%, but reoperation carries 1-3% mortality (up to 5-10% with significant comorbidities or LV dysfunction) 2. All interventions should be performed at centers with expertise in congenital heart disease 2.