What is the treatment for coarctation of the aorta?

Treatment of Coarctation of the Aorta

For adults and older children (>25 kg) with native or recurrent coarctation, covered stent placement is the first-line treatment when technically feasible; surgical repair with interposition tube graft is reserved for cases unsuitable for stenting. 1

Indications for Intervention

Intervention is indicated when specific hemodynamic and anatomic criteria are met:

• Hypertension (>140/90 mmHg in adults) PLUS a non-invasive arm-to-leg gradient >20 mmHg (confirmed by invasive peak-to-peak gradient >20 mmHg) warrants repair, with preference for stenting when technically feasible 1

• Hypertensive patients with >50% aortic narrowing relative to the diameter at the diaphragm level should be considered for endovascular treatment even if the invasive gradient is <20 mmHg 1

• Normotensive patients with invasive peak-to-peak gradient >20 mmHg should be considered for endovascular treatment when technically feasible 1

Additional hemodynamic markers suggesting significant coarctation include: peak gradient >35 mmHg, mean gradient >20 mmHg, abdominal antegrade diastolic flow on Doppler, or diastolic run-off in the descending thoracic aorta 1

Treatment Approach by Age and Anatomy

Neonates, Infants, and Young Children (<25 kg)

Surgical resection is the treatment of choice in this population 2. Surgical techniques include:

• Extended end-to-end anastomosis (preferred for discrete coarctation) 1
• Subclavian flap repair 1
• Patch aortoplasty 1
• Interposition tube graft for complex anatomy 1

Older Children (>25 kg) and Adults

Transcatheter treatment with covered stent placement is the first-choice treatment 1, 2. The rationale for stenting in this population includes:

• Superior short-term outcomes compared to balloon angioplasty alone 1
• Ability to use stents expandable to adult size (minimum 2 cm diameter) 1
• Lower risk of aneurysm formation compared to balloon angioplasty alone 1

Balloon angioplasty without stenting may be considered if stent placement is not feasible and surgical intervention is not an option, though it carries higher recurrence rates and risk of aneurysm formation 1

Recurrent Coarctation

Balloon angioplasty with or without stenting is the best therapeutic option for discrete recurrent/residual coarctation after surgical repair 1. Covered stent placement is preferred as first-line treatment for re-coarctation 1

Critical Diagnostic Work-Up Before Intervention

Before any intervention, obtain:

• Blood pressure measurements in both arms and one lower extremity to document gradients 1
• CMR or cardiac CT to visualize the entire thoracic aorta, define the coarctation anatomy, assess for arch hypoplasia, identify collateral vessels, and detect aneurysms 1
• Invasive catheterization with manometry remains the gold standard for confirming hemodynamic significance (peak-to-peak gradient >20 mmHg) before intervention 1
• Echocardiography to assess for associated lesions (bicuspid aortic valve in 50-85%, left ventricular hypertrophy, ventricular function) 1

Lifelong Surveillance Requirements

All coarctation patients require lifelong follow-up regardless of treatment success 1. This is non-negotiable because:

Imaging Surveillance

• CMR or cardiac CT every 3-5 years (adjusted to clinical status and previous findings) to monitor for re-coarctation, aneurysms, pseudoaneurysms, and dissection 1
• Patients with patch repairs are at particular risk for para-anastomotic aneurysms 1

Blood Pressure Monitoring

• Hypertension remains common even after successful repair, particularly when initial repair occurs in adulthood 1, 3
• 24-hour ambulatory blood pressure monitoring or exercise testing better detects hypertension than office measurements alone 1
• Treat hypertension according to standard hypertension guidelines 1
• Exercise testing to evaluate for exercise-induced hypertension is reasonable in patients who exercise 1

Associated Conditions Requiring Monitoring

• Bicuspid aortic valve (present in 50-85% of cases) requires surveillance for valve dysfunction and ascending aortic dilation 1
• Screening for intracranial aneurysms by MRA or CTA may be reasonable, though routine screening in asymptomatic patients is not currently evidence-based 1
• Premature coronary artery disease risk is elevated due to chronic hypertension 1, 3

Common Pitfalls to Avoid

Do not rely solely on Doppler gradients by echocardiography to quantify severity in native or post-operative coarctation—they are unreliable in the presence of collaterals or decreased ventricular function 1. A diastolic run-off phenomenon is more reliable for detecting significant stenosis 1

Do not assume successful repair eliminates future risk—the natural history is driven by hypertension-related complications including heart failure, intracranial hemorrhage, premature coronary/cerebral artery disease, and aortic rupture/dissection even after intervention 1, 3

Do not delay treatment in symptomatic patients—delay in treatment impacts late morbidity and mortality 4