Highlights & Basics
- Aortic coarctation is characterized by a blood pressure differential between upper and lower extremities (upper >lower).
- In neonates, may present with shock if there is inadequate systemic perfusion once the ductus arteriosus closes.
- Physical exam may show diminished, absent, or delayed lower extremity pulses compared with upper extremity pulses.
- Diagnosis is made by demonstration of aortic arch narrowing, typically by echocardiography.
- Treatment may be surgical repair or percutaneous balloon dilation or stent placement.
- Long-term complications include persistent hypertension or aneurysm formation at site of surgery or balloon.
Quick Reference
History & Exam
Key Factors
hypertension presenting at a young age or resistant to treatment
diminished lower extremity pulses
differential upper and lower extremity blood pressure (BP)
Other Factors
systolic ejection murmur
male sex
genetic syndrome
claudication
headache
systolic ejection click
other cardiac anomalies
Diagnostics Tests
1st Tests to Order
electrocardiogram (ECG)
chest x-ray (CXR)
echocardiogram
Other Tests to consider
computed tomography (CT) angiography
magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA)
cardiac catheterization
Treatment Options
acute
critical coarctation
maintenance of ductal patency
surgical repair
ongoing
noncritical coarctation
≤1 year old
>1 year old
Definition
Classifications
Site of coarctation
- Most common
- Narrowing is at the insertion site of the ductus arteriosus.
- Less common
- Narrowing can be proximal to the left subclavian artery or in the abdominal aorta.
- Rare
- Long segment of narrowing.
Exact location of the narrowing in relationship to the ductus arteriosus
- Narrowing at the ductus is termed juxtaductal, which has also been termed an infantile coarctation.
- Narrowing proximal to the ductal insertion is termed a preductal coarctation and is typically associated with hypoplasia of the transverse arch.
- Postductal coarctations occur distal to the ductal insertion.
- An abdominal coarctation occurs in the abdominal aorta and may be associated with narrowing of the abdominal aortic vessels.Image
Presence of associated abnormalities
- Type I: coarctation with or without patent ductus arteriosus (PDA)
- IA: with ventricular septal defect (VSD)
- IB: with other major cardiac defects.
- Type II: coarctation with isthmus hypoplasia, with or without PDA
- IIA: with VSD
- IIB: with other major cardiac defects.
- Type III: coarctation with tubular hypoplasia of the isthmus and segment between left carotid and subclavian arteries, with or without PDA
- IIIA: with VSD
- IIIB: with other major cardiac defects.
Vignette
Common Vignette 1
Common Vignette 2
Other Presentations
Epidemiology
Etiology
- The narrowed segment is underdeveloped during fetal life due to reduced blood flow across the developing arch. This is particularly thought to be responsible when associated with additional left-sided lesions such as hypoplastic left heart syndrome.
- Ductal tissue extends into the thoracic aorta, and when the ductus arteriosus constricts and closes postnatally, the thoracic aorta is constricted.[6]
Pathophysiology
Images
Abdominal coarctation
Two-dimensional suprasternal notch view of the aortic arch. Focal narrowing of the aortic arch (arrow) in the typical juxtaductal region
Addition of color Doppler shows turbulent higher velocity flow after the obstructed area
Three-dimensional computed tomographic reconstruction visualized from posterior to anterior. The area of coarctation is well seen (arrow)
Angiography in the ascending aorta shows a focal area of narrowing after the left subclavian artery
Two-dimensional suprasternal notch view of the aortic arch. Focal narrowing of the aortic arch (arrow) in the typical juxtaductal region
Addition of color Doppler shows turbulent higher velocity flow after the obstructed area
Three-dimensional computed tomographic reconstruction visualized from posterior to anterior. The area of coarctation is well seen (arrow)
Angiography in the ascending aorta shows a focal area of narrowing after the left subclavian artery
After stent placement, the narrowed area is markedly improved
Aortic angiography in an infant after surgical reconstruction of the aortic arch shows marked narrowing at the distal aspect of the surgical repair
Balloon angioplasty of the narrowed region leads to complete resolution of the obstruction
Diagnostic Approach
History
Physical exam
Investigations
Risk Factors
History & Exam
Tests
Differential Diagnosis
Aortic stenosis (AS)
Differentiating Signs/Symptoms
- Presenting features vary depending on the severity of stenosis. With mild AS, the patient may be asymptomatic with a harsh systolic ejection quality murmur at the upper right sternal border with radiation to the carotids on physical exam. In moderate or severe AS, the patient may have significant shortness of breath, especially on exertion.
Differentiating Tests
- Differentiated from coarctation of the aorta by echocardiogram.
- Two-dimensional echocardiogram of the aortic valve shows an abnormally narrowed or dysplastic aortic valve.
- Color Doppler and pulse Doppler across the aortic valve show turbulent blood flow across the valve and are used to measure the gradient.
- Patients with aortic coarctation with associated bicuspid aortic valve may also have AS.
Differentiating Signs/Symptoms
- Presents as shock in neonates. Unable to clinically differentiate between interruption of the aortic arch and critical coarctation of the aorta.
Differentiating Tests
- Echocardiogram will differentiate the severe narrowing seen in critical coarctation from a frank interruption.
Differentiating Signs/Symptoms
- Depending on the level and severity of the obstruction, patients may be asymptomatic or may present with shortness of breath, especially on exertion.
Differentiating Tests
- An echocardiogram will differentiate the level of the obstructed or narrowed area in the aorta. Using color and pulse Doppler, any narrowing in the left ventricular outflow tract can be localized to the valvar, subvalvar, or supravalvar area, or across the aortic arch.
Essential hypertension
Differentiating Signs/Symptoms
- Similar clinical presentation.
- Lower extremity pulses are usually normal unless peripheral vascular disease present.
Differentiating Tests
- Four-extremity blood pressure shows no gradient.
- Echocardiogram shows normal flow across the aortic arch and normal Doppler of the abdominal aorta.
Renal artery stenosis
Differentiating Signs/Symptoms
- Similar clinical presentation.
Differentiating Tests
- Renal artery Doppler shows renal artery stenosis.
- Normal echocardiogram.
Pheochromocytoma
Differentiating Signs/Symptoms
- Hypertension with associated symptoms such as palpitations and flushing.
Differentiating Tests
- A 24-hour urine collection is positive for elevated urine creatinine, metanephrines, and catecholamines.
Criteria
- Collateral blood vessels often enlarge and provide a route for blood to bypass the narrowed segment of the aorta.
- May be asymptomatic or present with hypertension.
- Well tolerated during fetal life because 90% of blood is shunted across the ductus arteriosus.
- After birth, the infant will present with low cardiac output and shock once the ductus arteriosus closes.
- Evidence of upper extremity hypertension (at rest, on ambulatory blood pressure [BP] monitoring, or with pathologic BP response to exercise) or left ventricular hypertrophy and evidence for one of the following gradient measurements:
- A noninvasive blood pressure difference of >20 mm Hg between the upper and lower extremities.
- A peak-to-peak gradient of >20 mm Hg across the coarct by catheterization; or a peak-to-peak gradient of >10 mm Hg across the coarct by catheterization in the setting of decreased left ventricular systolic function or significant collateral flow
- A mean gradient of >20 mm Hg across the coarct by Doppler echocardiography; or a mean gradient of >10 mm Hg across the coarct by Doppler echocardiography in the setting of decreased left ventricular systolic function or significant collateral flow.
Screening
Treatment Approach
Critical coarctation
Surgical repair
- The narrowed segment is resected with an extended end-to-end anastomosis of the aorta.[28]
- 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.[29]
- If the segment is considered too long for either of the above procedures, a bypass graft across the area of coarctation may be necessary.
Percutaneous repair
Recurrent coarctation
Treatment Options
critical coarctation
maintenance of ductal patency
Primary Options
- alprostadil
0.02 to 0.1 micrograms/kg/min intravenously
- alprostadil
Comments
- Prostaglandin E1 (alprostadil) maintains patency of the ductus arteriosus in neonates.
surgical repair
Comments
- Surgical repair is the preferred choice in neonates, as it is associated with few repeat interventions.[27]
noncritical coarctation
≤1 year old
surgical repair
Comments
- Surgical repair is the preferred choice in neonates, as it is associated with fewer reinterventions than transcatheter repair.[27]
- Type of surgical repair depends on length of narrowing and may be an end-to-end anastomosis, arch reconstruction with placement of patch, or bypass graft.
- 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.
>1 year old
surgical or percutaneous repair
Comments
- Type of surgical repair depends on length of narrowing and may be an end-to-end anastomosis, arch reconstruction with placement of patch, or bypass graft.
- In children and adults with a discrete narrowing, percutaneous angioplasty with or without stent implantation is an appropriate alternative to surgery.[32] [33] [34] Percutaneous stent implantation has a 98% success rate in repair of aortic coarctation.[35] Some authors have suggested that balloon angioplasty may be superior to surgery, but this remains highly controversial and the level of evidence is poor.[36] [37] [38] Percutaneous stent implantation may be superior to balloon angioplasty alone, as it has a lower restenosis rate and incidence of aortic wall abnormalities.[39] The overall complication rate of stent implantation is around 12%, including femoral artery access-related complications, aneurysm formation, aortic dissection, and stroke.[35]
recurrent coarctation
percutaneous balloon angioplasty or stent placement
Comments
- Recurrent coarctation occurs in approximately 10% to 20% of patients.[40] It may be treated with balloon angioplasty or stent placement depending on age and anatomy.
Emerging Tx
Bioabsorbable stents
Prevention
Secondary Prevention
Follow-Up Overview
Prognosis
Monitoring
Complications
Citations
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Writing Committee Members, Isselbacher EM, Preventza O, et al. 2022 ACC/AHA Guideline for the diagnosis and management of aortic disease: A report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2022 Dec 13;80(24):e223-e393.[Abstract][Full Text]
Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J. 2020 Aug 29;ehaa554.[Full Text]
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on clinical practice guidelines. J Am Coll Cardiol. 2019 Apr 2;73(12):e81-e192.[Abstract][Full Text]
Writing Committee; Riambau V, Böckler D, Brunkwall J, et al. Editor's choice - management of descending thoracic aorta diseases: clinical practice guidelines of the European Society for Vascular Surgery (ESVS). Eur J Vasc Endovasc Surg. 2017 Jan;53(1):4-52.[Abstract][Full Text]
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26. Forbes TJ, Kim DW, Du W, et al; CCISC Investigators. Comparison of surgical, stent, and balloon angioplasty treatment of native coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular Interventional Study Consortium). J Am Coll Cardiol. 2011 Dec 13;58(25):2664-74.[Abstract]
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Key Articles
Referenced Articles
Guidelines
Diagnostic
Summary
Includes recommendations for imaging of patients with suspected aortic coarctation with inadequate evaluation after transthoracic echocardiography.Published by
American College of Radiology
Published
2023
Summary
Includes recommendations for the diagnosis and management of aortic coarctation.Published by
American College of Cardiology; American Heart Association
Published
2022
Summary
Guidelines for the diagnosis and management of adults with congenital heart disease, including aortic coarctation.Published by
American Heart Association; American College of Cardiology
Published
2018
Summary
Guidelines for the diagnosis and management of adult congenital heart disease, including aortic coarctation.Published by
European Society of Cardiology
Published
2020
Summary
This guidance includes diagnostic methods in descending thoracic aortic disease.Published by
European Society of Vascular Surgery
Published
2017
Summary
Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. Contains detailed information on assessment of the aorta.Published by
European Society of Cardiology
Published
2014
Treatment
Summary
Includes recommendations for the diagnosis and management of aortic coarctation.Published by
American College of Cardiology/American Heart Association
Published
2022
Summary
Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. Contains detailed information on treatment options.Published by
European Society of Cardiology
Published
2014
Summary
Guidelines for the diagnosis and management of adult congenital heart disease, including aortic coarctation.Published by
European Society of Cardiology
Published
2020
Summary
Guidelines for the diagnosis and management of adults with congenital heart disease, including aortic coarctation.Published by
American Heart Association; American College of Cardiology
Published
2018
Summary
Guidelines for cardiovascular intervention in adults with congenital heart disease, including aortic coarctation.Published by
Canadian Cardiovascular Society
Published
2022