What are the clinical practice guidelines for coarctation of the aorta?

From the Guidelines

Coarctation of the aorta should be managed with early detection and intervention, including blood pressure measurement in both arms and legs, echocardiography, and advanced imaging (CT or MRI) to define anatomy, with surgical repair or endovascular stenting recommended for significant native or recurrent coarctation and hypertension, as stated in the 2022 ACC/AHA guideline 1.

Key Recommendations

• Initial, surveillance, and follow-up aortic imaging should be performed with MRI or CT in patients with coarctation of the aorta, including those who have undergone surgical or endovascular intervention 1.
• Blood pressure should be measured in both arms and one of the lower extremities in patients with coarctation of the aorta 1.
• Endovascular stenting or open surgical repair of the coarctation is recommended for patients with significant native or recurrent coarctation and hypertension 1.
• Guideline-directed medical therapy is recommended for the treatment of hypertension in patients with coarctation of the aorta 1.

Management Considerations

• The choice of percutaneous catheter intervention versus surgical repair of native discrete coarctation should be determined by consultation with a team of ACHD cardiologists, interventionalists, and surgeons at an ACHD center 1.
• Percutaneous catheter intervention is indicated for recurrent, discrete coarctation and a peak-to-peak gradient of at least 20 mm Hg 1.
• Surgical repair is recommended for infants and young children, while older children and adults may be candidates for balloon angioplasty with or without stent placement 1.

Long-term Follow-up

• Regular blood pressure monitoring, echocardiography every 1-2 years, and advanced imaging every 3-5 years are essential for assessing restenosis, aneurysm formation, or associated cardiac abnormalities 1.
• Lifelong antihypertensive therapy may be necessary even after successful repair, with beta-blockers or ACE inhibitors as first-line agents 1.

From the Research

Clinical Practice Guidelines for Coarctation of the Aorta

• Coarctation of the aorta is a congenital heart defect that requires lifelong surveillance following surgical repair 2
• The goal of treatment is to relieve the obstruction and prevent long-term complications such as recurrent coarctation, aneurysm formation, and vascular disease 3
• Treatment options include surgical repair, endovascular treatment with aortic balloon dilation and stent placement, and transcatheter-based therapies 4, 5, 6
• Patients with prior coarctation repair are at risk of long-term complications and require regular follow-up with aortic imaging and surveillance and management of hypertension to prevent vascular disease 3, 5
• Endovascular management, including stent implantation, is a preferred strategy for adult patients with coarctation of the aorta 6
• Anesthetic management for coarctation of the aorta repair requires careful consideration of the patient's pathophysiology and clinical presentation 4

Evaluation and Management

• Evaluation of coarctation of the aorta includes assessment of the degree of obstruction, associated lesions, and overall cardiac function 3, 5
• Management strategies include surgical repair, endovascular treatment, and medical therapy to control hypertension and prevent vascular disease 3, 5, 6
• Patients with unrepaired coarctation of the aorta require careful evaluation and management to prevent long-term complications 5

Long-term Outcomes

• Long-term survival after coarctation of the aorta repair is reduced compared to an age- and sex-matched population 2
• Patients with coarctation of the aorta are at risk of long-term complications, including recurrent coarctation, aneurysm formation, and vascular disease 2, 3, 5
• Regular follow-up and surveillance are essential to prevent and manage these complications 2, 3, 5