Approach

The main goal in treatment is complete repair of the coarcted segment with minimal complications and no residual gradient across the narrowed segment. The type of repair depends on the age of the patient at diagnosis and the severity of the coarctation. There is no clear evidence to support the superiority of either surgical- or catheter-based treatment for coarctation of the aorta.[29][30]

Critical coarctation

Neonates with critical coarctation should be given alprostadil (prostaglandin E1) to maintain ductal patency. Surgical repair is the preferred choice in neonates, as it is associated with fewer reinterventions than transcatheter repair.[31]

Surgical repair

Repair of coarctation is performed on diagnosis to prevent the complications of longstanding hypertension. Indications for urgent repair are congestive heart failure, systolic hypertension, or a peak pressure gradient >20 mmHg across the coarctation measured by Doppler echocardiography or catheterization.[6][9][16]

There are multiple surgical techniques depending on the length of the narrowing.

1. Short segment narrowing

  • The narrowed segment is resected with an extended end-to-end anastomosis of the aorta.[32]

2. Medium-length segment narrowing

  • It may be necessary to perform a more extensive arch reconstruction with placement of a patch or subclavian flap aortoplasty, in which the left subclavian artery is brought down as a flap to widen the narrowed segment. While subclavian flap aortoplasty can significantly affect the development of the left upper extremity and has a higher long-term mortality, a long-term follow-up of a cohort repaired at a single institution found no lifestyle limitations.[33]

3. Long segment narrowing (rare)

  • If the segment is considered too long for either of the above procedures, a bypass graft across the area of coarctation may be necessary.

When the surgical approach is appropriately tailored to the individual patient and their anatomy, the residual or recoarctation rate is approximately 6%.[32]

Death from surgical repair is rare and significant improvements in hypertension are typically observed.[34] Complications from surgery are also unusual and include postoperative hypertension, recurrent laryngeal nerve and phrenic nerve injury, postoperative chylothorax from lymphatic duct disruption, long-term recoarctation, and, rarely, paraplegia in patients with inadequate collateral circulation. Use of left atrium-to-descending aortocardiopulmonary bypass during surgical repair is thought to protect the spinal cord in patients with inadequate collateral circulation.[35]

Percutaneous repair

In children and adults with a discrete narrowing, percutaneous balloon angioplasty with or without stent implantation is an alternative to surgery.[36][37][38] Percutaneous stent implantation has a 98% success rate in repair of aortic coarctation.[39] Some authors have suggested that balloon angioplasty may be superior to surgery, but this remains highly controversial and the level of evidence is poor.[40][41][42]

In older children, some authors suggest that percutaneous stent implantation may be superior to balloon angioplasty alone, as it has a lower restenosis rate and incidence of aortic wall abnormalities.[43]

The overall complication rate of stent implantation is around 12%, including femoral artery access-related complications, aneurysm formation, aortic dissection, and stroke.[39]

Percutaneous repair is generally not recommended for children <1 year old, as recurrence rates are high. Additionally, stent placement is limited both by patient size and by the rapid growth seen during infancy.

Following the placement of artificial materials, such as stents, during interventions for aortic coarctation, it is recommended to prescribe antiplatelet agents (e.g., aspirin) for at least 6 months to prevent thrombotic complications.[15]​

Recurrent coarctation

Recurrent coarctation occurs in approximately 10% to 20% of patients.[44] Stent implantation has a higher rate of restenosis compared with surgical repair.[45] However, in general for patients with a recurrent coarctation, repeat surgery is more complicated than an initial intervention. Recurrent coarctation is often treated with balloon angioplasty or stent placement depending on age and anatomy.

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