Cardio · adult-congenital-heart-disease
Coarctation of the aorta in adults
Fellowship-level guide to coarctation of the aorta in adults under the 2020 ESC and 2025 ACC/AHA adult congenital heart disease guidelines, the 2024 ESC and 2022 ACC/AHA aortic guidelines and the 2025 ESC pregnancy guideline: anatomy and associated lesions, presentation in the hypertension work-up, criteria for significance, stent or surgical repair, hypertension after repair, lifelong imaging, bicuspid valve and Turner syndrome, pregnancy and sport.
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Target exams
- EECC
- ABIM Cardiovascular Disease Certification
Red flags
- Aneurysms of the ascending aorta or at the intervention site present a risk of rupture and death (ESC 2020)
- A low gradient does not exclude significant coarctation: with extensive collaterals gradients are often underestimated (ESC 2020), and with collaterals or decreased LV function gradients or ABI may underestimate severity (ESC 2024)
- Women with unrepaired coarctation, and those after repair with hypertension, residual coarctation or aortic aneurysms, have an increased risk of aortic rupture and cerebral aneurysm rupture during pregnancy and delivery (ESC 2020)
- Imaging of the intracerebral vessels is indicated in the case of symptoms and/or clinical manifestations of aneurysms or rupture (ESC 2020)
ACC/AHA 2025 says coarctation is mostly diagnosed and repaired in infancy and early childhood.[2] Yet it may first appear in an adult with hypertension, and hypertension is common even after successful repair.[1][2]
- This page covers recognition, grading of severity, repair, blood pressure, lifelong imaging, pregnancy and sport; the general principles of adult congenital care are in ACHD: anatomy segments and general principles.[3][2][7][1]
What coarctation is
ESC 2020 regards coarctation as part of a generalised arteriopathy, not only as narrowing of the aorta.[1] It occurs as a discrete stenosis or as a long, hypoplastic aortic (arch) segment.[1] Typically it lies where the ductus arteriosus inserts, and only in rare cases ectopically (ascending, descending or abdominal aorta).[1]
- ACC/AHA 2022 places the narrowing most often just distal to the left subclavian artery, typically with an aneurysmal segment immediately beyond the stenosis, but says variants are frequent.[4]
ACC/AHA 2025 characterises coarctation by aortic isthmus stenosis.[2] ESC 2024 adds that more distal locations are known as mid-aortic syndrome and require dedicated management.[3]
Where coarctation sits in the complexity classifications
ESC 2020 Table 4 lists coarctation of the aorta in its moderate complexity group (repaired or unrepaired).[1] ACC/AHA 2025 Table 4 lists coarctation of the aorta in anatomic group II: Moderate Complexity.[2] Patients are classified according to the "highest" relevant anatomic or physiological feature.[2]
[1] [4] [3]Associated lesions and syndromes
Associated lesions, extracardiac vascular anomalies and syndromes reported in the held guidelines
| Association | What the guidelines report |
|---|---|
| Bicuspid aortic valve | ESC 2020: up to 85%; ESC 2024: up to 50%–85%; ACC/AHA 2025: more than 60% of patients with coarctation; ACC/AHA 2022: coexists in at least 50% of cases |
| Coarctation in bicuspid valve disease | ACC/AHA 2025: coarctation is present in 4% to 10% of cases of BAV |
| Intracerebral aneurysms | ESC 2020: an extracardiac vascular anomaly, in up to 10%; ESC 2024: 10%; ACC/AHA 2022: screening studies suggest a 10% prevalence in adults with coarctation, compared with 2% in the normal adult population |
| Ascending aortic aneurysm | Listed as an associated lesion by ESC 2020 and ESC 2024 |
| Left heart lesions | ESC 2020: subvalvular or supravalvular aortic stenosis, (supra)mitral valve stenosis including parachute mitral valve, Shone complex; ACC/AHA 2025: aortic arch hypoplasia, LVOT disease (subaortic stenosis) and LV inflow disease (supravalvar mitral stenosis, mitral valve stenosis and parachute mitral valve) |
| Complex congenital heart defects | Listed by ESC 2020; ACC/AHA 2022 says coarctation may associate with complex congenital heart disease |
| Anomalous origin of the right subclavian artery | An extracardiac vascular anomaly reported in 4–5% of cases (ESC 2020) |
| Collateral arterial circulation | An extracardiac vascular anomaly reported by ESC 2020 |
| Turner syndrome | ESC 2020 and ESC 2024: an associated syndrome; ESC 2024: up to 12.6% of females diagnosed with coarctation also have Turner syndrome, and coarctation is observed in 7%–18% of patients with Turner syndrome |
| Williams–Beuren syndrome | Associated syndrome (ESC 2020) |
Pathophysiology: a pressure-loaded ventricle and an abnormal aorta
ACC/AHA 2025 explains the adult disease in two layers. First, the anatomic lesions (LVOT disease and aortic isthmus stenosis) cause LV pressure overload.[2] That leads to LV remodeling: hypertrophy, fibrosis, impaired relaxation and compliance, and impaired contractility.[2]
Second, coarctation carries physiological abnormalities such as endothelial dysfunction, abnormal smooth-muscle reactivity and sympathetic hyperactivity.[2] These raise aortic stiffness and aortic pressure wave reflection, which in turn exacerbate the remodeling caused by the anatomic lesions.[2]
How ACC/AHA 2025 links the lesion to its late consequences
- 1
Pressure overload
Anatomic lesions (LVOT disease and aortic isthmus stenosis) cause LV pressure overload, which leads to LV remodeling
- 2
Early remodeling
Initially asymptomatic LV diastolic and systolic dysfunction, as well as left atrial dysfunction
- 3
Late disease
LV remodeling may eventually progress to overt heart failure (mostly heart failure with preserved ejection fraction), pulmonary hypertension and atrial fibrillation
- The guideline adds that LV hypertrophy is a normal physiological response that maintains wall stress, but chronic pressure overload ultimately leads to myocardial ischaemia with replacement fibrosis, impaired relaxation and compliance, and impaired contractility.[2]
Hypertension underpins the cardiovascular remodeling that ultimately leads to cardiovascular morbidity and mortality in this population.[2] Coarctation is also associated with premature coronary artery disease and an increased risk for stroke.[2]
How adults present
ESC 2020 says signs and symptoms depend on the severity of coarctation.[1] Severe coarctation usually presents early in life.[1] Particularly mild cases may not become evident until adulthood, when coarctation is detected in the work-up of arterial hypertension.[1]
ACC/AHA 2025 agrees that diagnosis and repair mostly occur in infancy and early childhood, but coarctation can be missed in childhood.[2] The adult diagnosis then usually comes during evaluation for refractory hypertension.[2]
ESC 2020: key symptoms
- Key symptoms may include headache, nosebleeds, dizziness, tinnitus, shortness of breath, abdominal angina, claudication and cold feet
ESC 2024: two mechanisms
- Pre-stenotic hypertension, for example headache and nosebleeds
- Post-stenotic hypoperfusion, for example abdominal angina and claudication
Natural history
ESC 2020 reports very good long-term survival up to age 60 years in patients who reach adolescence.[1] Long-term morbidity is common, however, largely related to aortic complications and longstanding hypertension.[1]
- ESC 2020: the natural course may be complicated by left heart failure, intracranial haemorrhage (from berry aneurysm), endocarditis, aortic rupture or dissection, premature coronary and cerebral artery disease, and associated heart defects.[1]
- ESC 2024: the natural course is largely driven by hypertension-related complications, including heart failure, intracranial haemorrhage, premature coronary or cerebral artery disease, and aortic rupture or dissection.[3]
- ACC/AHA 2022: untreated coarctation may be complicated by aortic dissection, heart failure, ruptured cerebral aneurysm, distal hypoperfusion, or the consequences of significant hypertension.[4]
- ESC 2024 hypertension guideline: when coarctation is not treated, patients often develop severe hypertension and hypertension-mediated organ damage, especially LV hypertrophy and LV dysfunction, aortopathy and cerebrovascular complications.[5]
Coarctation in the hypertension work-up
- Both hypertension guidelines list coarctation among the secondary causes, and AHA/ACC 2025 names a young adult with hypertension (age <30 y) as its indication for testing; the wider work-up is in Secondary hypertension.[6][5]
Secondary hypertension tables: the coarctation rows
| Guideline | Coarctation entry |
|---|---|
| ESC 2024 hypertension, Table 13 (optional screening tests when signs, symptoms or history suggest a secondary cause) | Screening tests: echocardiogram; aortic CT angiogram |
| AHA/ACC 2025 blood pressure, Table 10 (uncommon causes) | Aortic coarctation (undiagnosed or repaired): prevalence 0.1%; indication for additional testing, a young adult with hypertension (age <30 y); examination, BP higher in the upper than the lower extremities, absent femoral pulses, continuous murmur over the back or chest or an abdominal bruit, or a left thoracotomy scar (postoperative); screening test, echocardiogram; confirmatory tests, thoracic and abdominal CT angiogram or magnetic resonance arteriography |
ESC 2024 notes that significant inter-arm BP differences may reflect arterial stenosis or coarctation of the aorta, which may require investigation.[5] If systolic BP differs by more than 10 mmHg between arms, subsequent measurements use the arm with the higher value.[5] ACC/AHA 2022 adds that the location of the coarctation will inform any BP differential between the left and right arms.[4]
Bedside assessment
ESC 2020 makes office blood pressure in the upper and lower extremities the primary study required in all patients with coarctation.[1] A systolic gradient of ≥20 mmHg between upper and lower extremities indicates significant coarctation.[1] Weak or absent lower-limb pulses, or radiofemoral pulse delay, also indicate significant coarctation.[1]
-
ESC 2024 says a systolic non-invasive gradient between upper and lower extremities, an abnormal ABI, or an invasive peak-to-peak gradient ≥20 mmHg indicates significant coarctation.[3]
-
ACC/AHA 2025 text: measure blood pressure in all 4 extremities at the initial evaluation, then in the right arm and leg during routine evaluation to screen for hemodynamically significant residual coarctation.[2]
-
Blood pressure should preferably be measured in the right arm (except with aberrant origin of the right subclavian artery) at every clinical encounter, to screen for hypertension or monitor response to treatment (ACC/AHA 2025).[2]
-
ACC/AHA 2022: physical examination may reveal a delay in timing and a decreased amplitude of the femoral pulse; upper extremity hypertension and lower extremity hypoperfusion are the hallmarks of coarctation.[4]
-
ESC 2020: other findings are a suprasternal thrill (proximal obstruction), an interscapular systolic murmur, or continuous murmurs from collateral vessels; with a pinpoint coarctation, murmurs may be completely absent.[1]
-
ESC 2020: the chest X-ray may show rib notching of the third and fourth (to the eighth) ribs due to the collaterals.[1]
Blood pressure measurement rows
| Row | Class | Level / LOE |
|---|---|---|
| ACC/AHA 2025: in adults with coarctation, blood pressure measurement at rest in the upper and lower extremities is recommended as part of routine clinical assessment to screen for hypertension and residual/recurrent coarctation | COR 1 | B-NR |
| ESC 2024: in patients with coarctation, BP measurements at both arms and one lower extremity are recommended | I | C |
| ACC/AHA 2022 (dated; the ACC/AHA 2025 row above is newer): in patients with coarctation, BPs should be measured in both arms and one of the lower extremities | COR 1 | C-EO |
Investigations: confirming and grading the narrowing
Echocardiography and its Doppler traps
- ESC 2020 says echocardiography shows the site, structure and extent of coarctation, LV function and hypertrophy, associated cardiac abnormalities, and aortic and supra-aortic vessel diameters.[1]
ESC 2024 adds that TTE detects LV hypertrophy, which is a marker of disease.[3] ACC/AHA 2022 finds TTE useful for the gradient across the coarctation and for identifying a coexisting bicuspid valve and other congenital defects.[4]
Gradient underestimated
- ESC 2020: with extensive collateral arteries, gradients are not reliable and are often underestimated
- ESC 2024: with collaterals or decreased LV function, gradients or ABI may underestimate severity
- ACC/AHA 2025 Table 25: gradients may be lower with LV dysfunction, severe aortic stenosis or extensive collateral arteries
Gradient overestimated
- ESC 2020: after surgical repair or stenting, increased systolic flow rates may develop even without significant narrowing, because of decreased or absent aortic compliance and Doppler pressure recovery; the gradient is then overestimated
- ACC/AHA 2025 Table 25: in repaired coarctation, Doppler gradients may be elevated, even without narrowing, because of poor arterial compliance and pressure recovery
The two guideline bodies read Doppler differently.[1][2] ESC 2020 says Doppler gradients are not useful for quantification, in native or post-operative coarctation.[1]
- ACC/AHA 2025 lists Doppler gradients among its features suggestive of hemodynamic significance (Table 25), and says the mean gradient best approximates the catheter peak-to-peak gradient.[2]
Both treat a diastolic tail in the descending aorta with diastolic forward flow in the abdominal aorta as a sign of significant (re-)coarctation.[1][2]
CMR, CT and catheterisation
ESC 2020 makes CMR and CCT, including 3D reconstruction, the preferred non-invasive techniques for the entire aorta in adolescents and adults.[1]
- Both show the narrowing, the arch and head and neck vessels, the pre- and post-stenotic aorta and collaterals, and detect aneurysms, false aneurysms, restenosis or residual stenosis.[1]
ESC 2024 calls CMR and CCT the preferred techniques for interventional decision-making.[3]
- ACC/AHA 2025 text: a new or suspected diagnosis should prompt TTE and cross-sectional imaging (CMR or CT angiography) to determine severity, arch anatomy and ascending aorta dimensions, and to rule out associated intracardiac lesions.[2]
- If the severity of coarctation or aortic aneurysm at baseline does not warrant intervention, ongoing monitoring for progression with CMR or CT angiography may be required, at a frequency that depends on lesion severity (ACC/AHA 2025).[2]
- ESC 2020: cardiac catheterisation with manometry (peak-to-peak gradient ≥20 mmHg) indicates a haemodynamically significant coarctation in the absence of well-developed collaterals, and is performed in the setting of interventional treatment; under general anaesthesia the invasive gradient may be underestimated.[1]
Cross-sectional imaging rows
| Row | Class | Level / LOE |
|---|---|---|
| ACC/AHA 2025: in adults with unrepaired coarctation, cross-sectional imaging is recommended to determine coarctation severity and delineate anatomy of the thoracic aorta | COR 1 | B-NR |
| ACC/AHA 2022 (dated; the ACC/AHA 2025 row above is newer): in patients with coarctation, including those who have undergone surgical or endovascular intervention, an MRI or CT is recommended for initial, surveillance and follow-up aortic imaging | COR 1 | B-NR |
Criteria for significance, guideline by guideline
How the guidelines define a significant coarctation
| Source | Criteria as printed |
|---|---|
| ESC 2020 text | Upper-to-lower extremity systolic gradient ≥20 mmHg; weak or absent lower-limb pulses or radiofemoral delay; catheter peak-to-peak gradient ≥20 mmHg in the absence of well-developed collaterals; diastolic tail in the descending aorta and diastolic forward flow in the abdominal aorta |
| ESC 2024 text | A systolic non-invasive gradient between upper and lower extremities, an abnormal ABI, or an invasive peak-to-peak gradient ≥20 mmHg; a diastolic tail in the descending thoracic aorta or abdominal diastolic antegrade flow by TTE is suggestive of significant narrowing |
| ACC/AHA 2022 Table 37 | Upper extremity hypertension (at rest, on ambulatory monitoring or with a pathological response to exercise) or LV hypertrophy, plus 1 of: a noninvasive upper-to-lower extremity difference >20 mm Hg; a catheter peak-to-peak gradient >20 mm Hg, or >10 mm Hg with decreased LV systolic function or significant collateral flow; a Doppler mean gradient >20 mm Hg, or >10 mm Hg with decreased LV systolic function or significant collateral flow |
| ACC/AHA 2025 Table 25 | Upper-to-lower extremity systolic gradient >20 mm Hg; TTE Doppler mean gradient >20 mm Hg, corrected maximum instantaneous gradient >20 mm Hg, and a diastolic tail in the descending aorta with diastolic forward flow in the abdominal aorta; catheter peak-to-peak gradient >20 mm Hg; MRA/CTA aortic isthmus ratio <0.5–0.7 and collateral arteries on angiogram |
ACC/AHA 2025 defines the aortic isthmus ratio as the smallest coarctation diameter divided by the descending aorta at the level of the diaphragm.[2] It says expert consensus generally uses <0.5 for anatomic significance, while recent empirical data suggest <0.7 may be optimal for recommending repair.[2] Anatomic severity can be used when the gradient may underestimate severity, for example with severe LV systolic dysfunction, severe aortic stenosis or extensive collaterals.[2]
[2] [1] [3]Blood pressure testing beyond the clinic
ESC 2020 recommends right-arm ambulatory blood pressure to detect or confirm hypertension, defined as a 24-h mean systolic >130 mmHg and/or diastolic >80 mmHg.[1] ACC/AHA 2025 says office and home blood pressure are the baseline screening tests but can sometimes miss hypertension in coarctation.[2] Ambulatory measurement also detects isolated nocturnal hypertension (absence of nocturnal dipping), a marker of vascular dysfunction and future events.[2]
- ACC/AHA 2025 generally defines exercise-induced hypertension as a peak-exercise systolic pressure >210 mm Hg in males and >190 mm Hg in females, and says it is common in coarctation.[2]
It is associated with LV hypertrophy and higher cardiovascular risk, independent of resting blood pressure and other clinical characteristics.[2]
Ambulatory, exercise and coronary testing rows
| ACC/AHA 2025 row | COR | LOE |
|---|---|---|
| In adults with coarctation, ambulatory blood pressure measurement is reasonable to diagnose hypertension and monitor response to antihypertensive therapy | 2a | B-NR |
| In adults with coarctation, exercise testing is reasonable to screen for exercise-induced hypertension | 2a | B-NR |
| In adults with coarctation who present with exertional symptoms, evaluation for coronary artery disease is reasonable to guide management (marked New in the 2025 guideline) | 2a | B-NR |
- Premature coronary artery disease is common in coarctation, with a higher incidence of myocardial infarction at a younger age than in patients without coarctation (ACC/AHA 2025).[2]
The guideline attributes it to a high prevalence of risk factors (hypertension, hyperlipidaemia and type 2 diabetes) and, possibly, an inherent vascular dysfunction.[2] Data to guide screening are limited, but coronary disease should be considered in a patient with angina and exertional dyspnoea.[2]
Intracranial aneurysm screening: the guidelines disagree
Screening for intracranial aneurysms
| Source | Position | Class / LOE |
|---|---|---|
| ESC 2020 text | Imaging of intracerebral vessels is indicated in the case of symptoms and/or clinical manifestations of aneurysms or rupture; for berry aneurysms of the circle of Willis, routine screening in asymptomatic patients is not recommended | No class or level given |
| ESC 2024 text | Presently, there is no evidence supporting screening for intracerebral aneurysms in asymptomatic patients | No class or level given |
| ACC/AHA 2022 row (dated; the 2025 row below is newer) | In adult patients with coarctation, screening for intracranial aneurysms by MRI or CT may be reasonable | COR 2b, LOE B-NR |
| ACC/AHA 2025 row | In adults with coarctation, screening for intracranial aneurysms by brain MR or CT angiography may be considered to guide management | COR 2b, LOE B-NR |
- ACC/AHA 2025 says adults with coarctation may be at elevated risk for intracranial aneurysms, although the literature supporting this association remains controversial.[2]
- Studies of older patients with a higher prevalence of hypertension showed more intracranial aneurysms than a general population of similar age, whereas studies of younger patients showed no association (ACC/AHA 2025).[2]
- The limited data suggest that most aneurysms found are generally small, remain stable over time and carry a low risk of rupture, so the role of routine screening remains unclear (ACC/AHA 2025).[2]
- ACC/AHA 2022 cites a cost-effectiveness analysis that supports screening but notes that preferred strategies remain unknown, and says shared decision-making about screening may be informed by age, risk factors and anticoagulation considerations, because many detected aneurysms will be very small and not need treatment.[4]
When to act quickly
Who needs repair
The ESC rows split patients by blood pressure, then by gradient or degree of narrowing.[1]
- The ACC/AHA 2025 row asks whether the narrowing is anatomically and hemodynamically significant; the ACC/AHA 2022 row asks for significant coarctation by its Table 37 criteria together with hypertension.[2][4]
ESC intervention rows for coarctation and re-coarctation (2020 and 2024)
| Situation | ESC 2020 (dated) | ESC 2024 Recommendation Table 69 |
|---|---|---|
| Hypertensive, with an increased non-invasive upper-to-lower limb gradient confirmed invasively | Repair (surgical or catheter based) is indicated when the invasive peak-to-peak gradient is ≥20 mmHg, with preference for catheter treatment (stenting) when technically feasible: Class I, Level C | Repair (surgical or endovascular) is indicated when the increased gradient (decreased ABI) is confirmed with an invasive peak-to-peak gradient >20 mmHg, with a preference for stenting when technically feasible: Class I, Level C |
| Hypertensive, with narrowing relative to the aortic diameter at the diaphragm, even if the invasive peak-to-peak gradient is <20 mmHg | Stenting should be considered with ≥50% narrowing, when technically feasible: Class IIa, Level C | Endovascular treatment should be considered with >50% narrowing, when technically feasible: Class IIa, Level C |
| Normotensive, with an increased non-invasive gradient confirmed invasively | Stenting should be considered when the invasive peak-to-peak gradient is ≥20 mmHg, when technically feasible: Class IIa, Level C | Endovascular treatment should be considered when the invasive peak-to-peak gradient is >20 mmHg, when technically feasible: Class IIa, Level C |
| Normotensive, with ≥50% narrowing relative to the aortic diameter at the diaphragm, even if the invasive peak-to-peak gradient is <20 mmHg | Stenting may be considered, when technically feasible: Class IIb, Level C | No row in Recommendation Table 69 for this situation |
- ESC 2020 footnote to its intervention table: right-arm ambulatory blood pressure monitoring should be considered for the diagnosis of hypertension.[1]
- ESC 2024 Figure 44 footnote: the diagnosis of hypertension may require confirmation with ambulatory blood pressure and should also be considered with exercise-induced hypertension and/or LV hypertrophy on TTE.[3]
- ESC 2024 says this topic is extensively discussed in the ESC 2020 adult congenital heart disease guideline; among the ESC guidelines checked here, the normotensive ≥50% narrowing row exists only in the 2020 table.[3][1]
ACC/AHA repair rows
| ACC/AHA row | COR | LOE |
|---|---|---|
| 2025: in adults with native or repaired coarctation, surgical or transcatheter stent therapy is recommended for anatomically and hemodynamically significant coarctation to prevent irreversible LV dysfunction from chronic pressure overload (Table 25 gives the features suggestive of hemodynamic significance) | 1 | B-NR |
| 2022 (aortic disease; dated, the 2025 row above is newer): in patients with significant native or recurrent coarctation (Table 37) and hypertension, endovascular stenting or open surgical repair of the coarctation is recommended | 1 | B-NR |
ACC/AHA 2025 says repair should be performed by an operator with ACHD expertise.[2] ESC 2020 says treatment should be performed in centres with extensive experience in congenital heart disease.[1]
[1] [3] [2]Associated lesions that may need their own intervention
ESC 2020 reminds the reader that coarctation is not a localised disease of the aorta.[1] Its list of associated lesions that may require structural intervention has four items.[1]
- Associated significant aortic valve stenosis or regurgitation (bicuspid aortic valve).[1]
- Aneurysm of the ascending aorta with a diameter >50 mm or rapid progression of diameter.[1]
- Aneurysm and false aneurysms at the previous coarctation site.[1]
- Symptomatic or large aneurysms of the circle of Willis.[1]
Stent or surgery
ESC 2020 and ESC 2024
- ESC 2020: in native coarctation and re-coarctation with appropriate anatomy, stenting has become the first-choice treatment in many ACHD centres
- ESC 2020: covered stents are preferred because of lower short- and long-term complication rates
- ESC 2024: in native coarctation and re-coarctation, covered stenting is the first-choice treatment, and interposition of a tube graft is the preferred surgical therapy if stenting is less suitable
- ESC 2020 key message: stenting is the treatment of choice when technically feasible, and the decision to (re)intervene depends on blood pressure, gradient and stenosis morphology
ACC/AHA 2022 and 2025
- ACC/AHA 2022: with native or recurrent coarctation and appropriate anatomy, endovascular stenting is typically performed
- ACC/AHA 2025: surgical repair and transcatheter stent therapy give similar immediate hemodynamic results (postintervention residual gradient)
- ACC/AHA 2025: long-term outcome studies are limited but suggest a higher risk of reintervention (stent fracture, restenosis, aneurysm or pseudoaneurysm) and less complete LV reverse remodeling after stenting than after surgery
- ACC/AHA 2025: therapy should be individualised according to thoracic aorta anatomy, procedural risk and institutional expertise
- Balloon angioplasty in adults is only indicated for re-dilatation of previously stented aortas (ESC 2020).[1]
- Of the paediatric surgical techniques (resection with end-to-end or extended end-to-end anastomosis, prosthetic patch aortoplasty, subclavian flap aortoplasty, interposition of a tube graft and bypass tube or jump grafts), ESC 2020 says only the last two are generally feasible in adults; ascending-to-descending aorta conduits may be preferable with difficult anatomy.[1]
- ACC/AHA 2022 says open repair may include subclavian flap aortoplasty, resection and end-to-end anastomosis, interposition grafting or bypass grafting, chosen on patient- and anatomic-specific characteristics.[4]
- ESC 2020: the surgical risk in simple coarctation may currently be below 1%, but it increases significantly beyond the age of 30–40 years; spinal cord injury has become extremely rare.[1]
- ACC/AHA 2022: after a previous open repair, an endovascular approach to recoarctation, aneurysm or pseudoaneurysm avoids reoperation, assuming adequate iliofemoral access and no involvement of the supra-aortic trunks.[4]
Hypertension: the long tail of coarctation
Repair does not end the hypertension problem.[2][1] ACC/AHA 2025 says more than half of adults with repaired coarctation have systemic hypertension, which can occur even without significant residual narrowing.[2] ESC 2024 says hypertension is more common when the initial repair is performed in adulthood.[3] ACC/AHA 2022 says it is common after repair, especially during exercise, and when repair is performed in adults.[4]
- ESC 2020: hypertension at rest or during exercise is common even after successful treatment, and is an important risk factor for premature coronary artery disease, ventricular dysfunction and rupture of aortic or cerebral aneurysms.[1]
- ESC 2020: arch geometry (gothic, crenel, normal) and a smaller aortic size in the stented region may play a role in developing hypertension.[1]
- ESC 2020: recurring or residual coarctation may induce or aggravate systemic hypertension and its complications.[1]
- ESC 2024 hypertension guideline: aortic coarctation is associated with cardiovascular disease in the long term, even after early surgical or percutaneous treatment, and its most common complications are associated with hypertension.[5]
Treating hypertension: the rows
| Row | Class | Level / LOE |
|---|---|---|
| ACC/AHA 2025: in adults with native or repaired coarctation and hypertension without hemodynamically significant coarctation, GDMT for hypertension is recommended to maintain controlled resting and ambulatory blood pressure | COR 1 | B-NR |
| ESC 2024 (aortic): it is recommended to treat hypertension in patients with coarctation according to ESC hypertension guidelines | I | C |
| ACC/AHA 2022 (dated; the ACC/AHA 2025 row above is newer): in patients with coarctation, guideline-directed medical therapy is recommended for the treatment of hypertension | COR 1 | C-EO |
- ACC/AHA 2025 text says hypertension should be treated aggressively once detected, with a target systolic pressure <130 mm Hg, given the high prevalence of other risk factors.[2]
Office or home readings can underestimate systolic pressure and lead to undertreatment.[2]
- Ambulatory blood pressure and exercise testing may be considered to monitor control, because on-treatment systolic pressure correlates directly with LV hypertrophy, LV dysfunction and cardiovascular events.[2]
The ESC 2024 hypertension guideline notes that no formal RCTs have defined optimal medical treatment of hypertension in aortic coarctation.[5] Patients not suitable for, or having undergone, intervention should therefore be treated with the core algorithm for the general population.[5] ESC 2020 had pointed to the 2018 ESC/ESH hypertension guidelines, noting that the evidence for treating repaired coarctation like general hypertension is lacking.[1]
Lifelong follow-up and imaging
ESC 2020, ESC 2024, ACC/AHA 2022 and ACC/AHA 2025 all call for continuing follow-up after repair.[1][3][4][2] ESC 2020 says all patients require regular follow-up at least every year.[1] ESC 2024 says all patients require lifelong follow-up.[3]
Surveillance imaging rows
| Row | Class | Level / LOE |
|---|---|---|
| ESC 2024: in patients with native or repaired coarctation, lifelong follow-up is recommended, including regular imaging of the aorta with CCT/CMR every 3–5 years (adapted to clinical status and previous imaging findings) | I | B |
| ACC/AHA 2025: in adults with repaired coarctation, periodic cross-sectional imaging is recommended to screen for recurrent coarctation, aortic root or ascending aorta aneurysms, and vascular complications of repair (focal aortic aneurysm, pseudoaneurysm, dissection and stent fracture) | COR 1 | B-NR |
| ACC/AHA 2022 (dated; the ACC/AHA 2025 row above is newer): MRI or CT is recommended for initial, surveillance and follow-up aortic imaging in coarctation, including after surgical or endovascular intervention | COR 1 | B-NR |
- ESC 2020 text: imaging of the aorta, preferably with CMR, documents post-repair or post-interventional anatomy and complications (restenosis, aneurysm, false aneurysm); intervals are commonly every 3–5 years but depend on baseline pathology.[1]
- ESC 2024 text: CMR/CCT every 3–5 years, adjusted to previous imaging findings and type of intervention.[3]
- ACC/AHA 2025 text: CMR is preferred because it avoids radiation, but CT angiography should be used after coarctation stent implantation (stent artefacts on CMR) and may also screen for coronary disease.[2]
- ACC/AHA 2025 text: without aortic aneurysm or residual coarctation, CMR or CT angiography may be performed every 5 years; with aneurysm or residual coarctation, frequency depends on lesion severity.[2]
- ACC/AHA 2022 text: the optimal frequency after repair is not well established and is best individualised; after stable imaging is established, surveillance is often every 3 to 5 years, and arch and descending complications are better seen on MRI or CT than TTE.[4]
ACC/AHA 2025 Table 24: routine follow-up and testing intervals in coarctation, by physiological stage
| ACC/AHA 2025 Table 24 | Stage A | Stage B | Stage C | Stage D |
|---|---|---|---|---|
| Outpatient ACHD cardiologist (months) | 24 | 24 | 12 | 6 |
| Electrocardiogram (months) | 24 | 24 | 12 | 6 |
| Transthoracic echocardiogram (months) | 24 | 24 | 12 | 12 |
The table notes refer exercise testing to recommendation 5 and CMR or CT timing to the supportive text.[2]
[2] [3]Complications after repair and common pitfalls
- ACC/AHA 2025: adults with repaired coarctation are at risk of re-coarctation (restenosis of the isthmus), aortic wall injury and remodeling at the repair site leading to aneurysms or pseudoaneurysms, and dissection of any part of the thoracic aorta.[2]
- ESC 2020: patch repairs (for example with Dacron) are at particular risk of repair-site aneurysms, and interposition grafts of false aneurysms; both should be imaged regularly.[1]
- ESC 2024: patch repairs are at particular risk of para-anastomotic aneurysms or pseudo-aneurysms, the latter possibly occurring after interposition grafts as well.[3]
- ACC/AHA 2022: late complications after surgical or endovascular repair may include undersized grafts, recurrent stenosis, aneurysm or pseudoaneurysm and rupture, typically treated with endovascular procedures unless anatomy dictates open or hybrid surgery.[4]
- ACC/AHA 2022: recoarctation occurs in about 10% after surgical repair and about 8% after balloon dilation.[4]
- ESC 2020: an increased upper-to-lower limb systolic gradient (≥20 mmHg) indicates re-coarctation and warrants invasive assessment for confirmation and treatment.[1]
- ESC 2020: attention is required for the bicuspid valve, mitral valve disease, premature coronary disease and berry aneurysms of the circle of Willis (routine screening for the last in asymptomatic patients is not recommended).[1]
Prognosis
- ESC 2020 reports very good long-term survival up to age 60 years in patients who reach adolescence, but common long-term morbidity from aortic complications and longstanding hypertension.[1]
The ESC 2024 hypertension guideline says coarctation is associated with cardiovascular disease in the long term, even after early treatment.[5] ACC/AHA 2022 says patients undergo lifelong follow-up and imaging because of the associated cardiovascular risks and the potential need for repeat intervention.[4]
Special populations
The bicuspid aortic valve
- The two lesions are linked in both directions: ACC/AHA 2025 says to screen adults with a BAV for coarctation, and ACC/AHA 2022 indicates an echocardiogram in coarctation because a BAV coexists in at least 50% of cases.[2][4]
ESC 2024 hypertension guideline: the bicuspid valve is the most common congenital heart disease and is sometimes associated with aortopathy or aortic coarctation.[5]
Looking for coarctation in bicuspid valve disease
| Row or statement | Class / LOE |
|---|---|
| ACC/AHA 2025: adults with a BAV should be screened for the presence of coarctation and aortic aneurysm to obtain early diagnosis and determine a management plan | COR 1, LOE B-NR |
| ACC/AHA 2022 (dated; the ACC/AHA 2025 row above is newer): in patients with a BAV, TTE is indicated to evaluate valve morphology and function, the diameter of the aortic root and ascending aorta, and aortic coarctation and other associated cardiovascular defects | COR 1, LOE B-NR |
| ESC 2024 Recommendation Table 68: when a BAV is first diagnosed, initial TTE to assess diameters of the aorta at several levels is recommended | Class I, Level B |
| ESC 2024 Recommendation Table 68: CCT or CMR of the entire thoracic aorta is recommended at first diagnosis, when important discrepancies in measurements are found between subsequent TTE controls during surveillance, or when the aortic diameter exceeds 45 mm | Class I, Level C |
| ESC 2024 text: when TTE detects BAV-associated aortic dilatation, CCT or CMR is recommended to confirm measurements, exclude coarctation and record baseline diameters | No class or level given |
Coarctation also counts as a risk factor in the bicuspid aortopathy rows.[2][4][3]
- ACC/AHA 2025: adults with a BAV and an aortic diameter >4 cm at the sinuses or ascending aorta, or with risk factors for dissection, should undergo lifelong surveillance to improve early detection of aortic growth (COR 1, LOE B-NR); its listed risk factors are family history of aortic dissection, aortic growth rate ≥0.3 cm per year, coarctation and "root phenotype" aortopathy.[2]
- ACC/AHA 2022: with a BAV, a root or ascending diameter of 5.0 cm to 5.4 cm and an additional risk factor for dissection (its Table 14: family history of aortic dissection, aortic growth rate ≥0.3 cm/y, aortic coarctation, "root phenotype" aortopathy), surgery to replace the root, ascending aorta or both is reasonable when performed by experienced surgeons in a Multidisciplinary Aortic Team (COR 2a, LOE B-NR).[4]
- ESC 2024: in patients with low surgical risk and ascending phenotype bicuspid aortopathy (tubular diameter > sinus diameter), surgery should be considered at a maximum diameter ≥50 mm if any of these applies: age <50 years, shorter stature (height 1.50–1.69 m), ascending aortic length ≥11 cm, growth ≥3 mm per year, family history of acute aortic syndrome, aortic coarctation, resistant hypertension, concomitant non-aortic-valve cardiac surgery, or desire for pregnancy (Class IIa, Level C).[3]
- ACC/AHA 2022 notes that BAV with coarctation has been reported to carry an increased dissection risk, although a report of 499 patients with BAV (mean age 40±16 years; 24% with coarctation) found no difference in adverse aortic events with or without coarctation.[4]
- Thresholds for the aortic root and ascending aorta are covered in Thoracic and abdominal aortic aneurysm: surveillance and repair thresholds and Aortic regurgitation.
Turner syndrome
ACC/AHA 2022 says approximately 50% of patients with Turner syndrome have cardiovascular defects, including aortic coarctation in 7%–18%.[4] ACC/AHA 2025 Table 9 lists coarctation among the congenital lesions associated with Turner syndrome (45,X).[2]
- ACC/AHA 2022: in patients with Turner syndrome, TTE and cardiac MRI are recommended at the time of diagnosis to evaluate for BAV, aortic root and ascending dilation, aortic coarctation and other congenital heart defects (COR 1, LOE B-NR).[4]
- ESC 2024 lists aortic dilatation, BAV, coarctation and arterial hypertension as risk factors for aortic dissection in Turner syndrome; ESC 2025 lists hypertension, BAV and coarctation.[3][7]
Pregnancy
ESC 2020 says many women tolerate pregnancy without major problems after successful treatment of coarctation.[1]
- Women with unrepaired coarctation, and those after repair with hypertension, residual coarctation or aortic aneurysms, have an increased risk of aortic rupture and of cerebral aneurysm rupture during pregnancy and delivery.[1]
An excess of miscarriages and hypertensive disorders has been reported.[1]
ESC 2025 Table 12: risks, monitoring and management in pregnancy (coarctation row)
| ESC 2025 Table 12 column | Coarctation of the aorta |
|---|---|
| Maternal risk | Higher complication risk with residual obstruction (gradient >20 mmHg, aortic lumen <12 mm), clinical signs of heart failure, LVEF <40% or NYHA class >1; higher risk of aortic dissection if an aneurysm is present; uncontrolled hypertension |
| Obstetric and foetal risk | Higher miscarriage rate; pre-term birth and low birth weight in 9% |
| Monitoring | Close BP monitoring, also early post-partum |
| Pregnancy management and delivery | Pre-pregnancy CMR and treatment of residual lesions; treat hypertension; consider bed rest, hospital admission and stenting in severe symptomatic (re)coarctation (including refractory hypertension or maternal or foetal compromise); vaginal delivery preferred unless aneurysm, heart failure or severe hypertension |
- ESC 2025 also notes that women with serial left heart obstructive lesions have higher maternal cardiovascular event rates.[7]
- ESC 2025 Table 6 (mWHO 2.0) lists both "Repaired coarctation" and "Severe (re)coarctation" in its aortopathy row; the held text runs the five class columns together, so this page does not state which class each entry is in.[7]
- Dated history: ESC 2020 Table 10 placed severe (re-)coarctation in the extremely high risk group (mWHO class IV), with the footnote that pregnancy should definitely be avoided.[1]
- ACC/AHA 2025 Table 10 lists severe native coarctation or recoarctation among the congenital conditions considered significantly high risk for maternal complications in pregnancy.[2]
- ESC 2025: in women with aortic dilatation related to BAV, imaging of the aortic root, ascending aorta and descending aorta (to rule out coarctation) is recommended before pregnancy, with TTE and CMR/CT if needed (Class I, Level C).[7]
- General pregnancy care for heart disease is covered in Cardiovascular disease in pregnancy.
Exercise and competitive sport
ESC 2020 says patients without residual obstruction who are normotensive at rest and with exercise can usually lead normally active lives without restriction.[1] Patients with hypertension, residual obstruction or other complications should avoid heavy isometric exercise in proportion to the severity of their problems.[1]
Competitive sport: the AHA/ACC 2025 scientific statement (no class or level given)
| AHA/ACC 2025 competitive sports statement, Table 8 (aortic arch obstruction) | Content |
|---|---|
| Diagnoses | Coarctation after surgical repair, after stent angioplasty, or unrepaired; interrupted aortic arch after repair; can be seen with VSD, DORV or other congenital repairs |
| Additional clinical evaluations | Upper and lower extremity BPs; CPET (or exercise stress test); CTA or CMR; hypertension should be treated before exercise |
| Reassuring findings | Normotensive at rest, treated or untreated; no or minimal resting upper-to-lower extremity BP gradient; normal BP response to exercise; no aortic aneurysms |
| Concerning findings | Resting hypertension or hypertensive response to exercise; aortic aneurysm, ascending or at the repair site; LV hypertrophy inconsistent with exercise-induced remodeling; substantial residual coarctation; complex arrhythmias at rest or stress |
| Consideration without concerning findings | It is reasonable to consider competitive sports participation after shared decision-making with the athlete and family; participation in these groups is not well studied and needs careful assessment of anatomy and physiology at rest and with exercise, focusing on the physiology of the intended sport |
- The statement adds that patients with aortic arch obstruction may be at risk of cardiovascular events even after successful repair, so competitive athletes should continue longitudinal surveillance of blood pressure control, imaging and exercise testing.[9]
- The ESC 2020 sports cardiology guideline, in its aortic assessment of athletes with congenital heart disease, says ascending aorta dilatation should lead to assessment for coarctation, as this can be associated with severe coarctation, which may be missed on clinical assessment but may cause severe exercise-related hypertension.[8]
Evidence, guidelines and regional differences
Where the two sets of guidelines differ
| Question | ESC | ACC/AHA |
|---|---|---|
| Gradient threshold | ESC 2020: ≥20 mmHg (limb and catheter); ESC 2024 text: invasive peak-to-peak ≥20 mmHg indicates significant coarctation, while its Recommendation Table 69 rows use invasive peak-to-peak >20 mmHg | ACC/AHA 2022 and 2025 tables: >20 mm Hg |
| Stent versus surgery | ESC 2024: covered stenting is the first-choice treatment; tube graft if stenting is less suitable | ACC/AHA 2025: similar immediate results; limited long-term data suggest more reintervention and less complete LV reverse remodeling after stenting; individualise |
| Intracranial aneurysm screening | ESC 2020 and 2024 text: routine screening in asymptomatic patients is not recommended / not supported by evidence | ACC/AHA 2022 and 2025: may be reasonable / may be considered (COR 2b, LOE B-NR) |
| Aortic imaging interval | ESC 2024 row: lifelong follow-up is recommended, with regular CCT/CMR every 3–5 years adapted to clinical status and previous imaging (Class I, Level B) | ACC/AHA 2025 text: CMR/CT angiography may be performed every 5 years without aneurysm or residual coarctation; ACC/AHA 2022 text: often every 3 to 5 years once stable |
| Blood pressure target | ESC 2024 hypertension guideline: core algorithm for the general population | ACC/AHA 2025 text: systolic <130 mm Hg |
In Australia and New Zealand
- No Australian or New Zealand guideline on coarctation of the aorta or adult congenital heart disease was found in the PubMed census for this topic (census 2026-10-24).
- The 2024 CSANZ Position Statement on Indications, Assessment and Monitoring of Structural and Valvular Heart Disease With Transthoracic Echocardiography in Adults (PMID 38749800) is not held as text in its version of record, so it was not checked for this topic; only its PubMed abstract is quotable.
- In the absence of an Australian or New Zealand guideline in this census, this page quotes the ESC and ACC/AHA guidelines, for example the ESC 2024 and ACC/AHA 2025 repair rows.[3][2]
Guidelines checked for this topic
-
Rows on this page come from the ESC 2020 adult congenital heart disease guideline, the ESC 2024 peripheral arterial and aortic diseases guideline and the ESC 2025 pregnancy guideline, with narrative from the ESC 2024 hypertension and ESC 2020 sports cardiology guidelines.[1][3][7][5][8]
-
ACC/AHA rows come from the ACC/AHA/HRS/ISACHD/SCAI 2025 adult congenital heart disease guideline and the ACC/AHA 2022 aortic disease guideline, with tables from the AHA/ACC 2025 blood pressure guideline (JACC PMID 40815242; Circulation PMID 40811497) and the 2025 AHA/ACC competitive sports scientific statement (JACC PMID 39976316; Circulation PMID 39973614).[2][4][6][9]
-
Rows marked "dated" have the newer row from the same body beside them; for the other rows, the same-month and newer guidelines in the census below were checked and give no revising row (census 2026-10-24).
-
The documents in this list are not used; each line gives the held status recorded in the census for this topic.
-
The PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD) (PMID 24814377) is not held as text in the evidence caches, so it was not checked for this topic.
-
The PACES/HRS expert consensus statement on the use of catheter ablation in children and patients with congenital heart disease: Developed in partnership with the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American Academy of Pediatrics (AAP), the American Heart Association (AHA), and the Association for European Pediatric and Congenital Cardiology (AEPC) (PMID 26899545) is not held as text in the evidence caches, so it was not checked for this topic.
-
Only the PubMed abstract of the Guideline for the diagnosis and management of hypertension in adults - 2016 (PMID 27456450) is held; its full text is not held and was not checked for this topic.
-
The NHFA/CSANZ Australian Clinical Guidelines for the Diagnosis and Management of Atrial Fibrillation 2018 (PMID 30077228) are not held as text in their version of record, so they were not checked for this topic.
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The 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease (PMID 30121239) is not held as text in the evidence caches, so it was not checked for this topic.
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The 2018 ESC/EACTS Guidelines on myocardial revascularization (PMID 30165437) are not held as text in the evidence caches, so they were not checked for this topic.
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The 2019 ESC Guidelines for the diagnosis and management of chronic coronary syndromes (PMID 31504439) are not held as text in the evidence caches, so they were not checked for this topic.
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The Hypertension Canada's 2020 Evidence Review and Guidelines for the Management of Resistant Hypertension (PMID 32389336) are not held as text in the evidence caches, so they were not checked for this topic.
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The Recommendations for participation in competitive sport in adolescent and adult athletes with Congenital Heart Disease (CHD): position statement of the Sports Cardiology & Exercise Section of the European Association of Preventive Cardiology (EAPC), the European Society of Cardiology (ESC) Working Group on Adult Congenital Heart Disease and the Sports Cardiology, Physical Activity and Prevention Working Group of the Association for European Paediatric and Congenital Cardiology (AEPC) (PMID 32845299) is not held as text in the evidence caches, so it was not checked for this topic.
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The 2020 ESC Guidelines for the management of acute coronary syndromes in patients presenting without persistent ST-segment elevation (PMID 32860058) are not held as text in the evidence caches, so they were not checked for this topic.
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The 2020 ESC Guidelines for the diagnosis and management of atrial fibrillation (PMID 32860505) are not held as text in the evidence caches, so they were not checked for this topic.
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Only the PubMed abstract of the 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary (PMID 33332149) is held; its full text is not held and was not checked for this topic.
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The Recommendations for participation in leisure-time physical activity and competitive sports of patients with arrhythmias and potentially arrhythmogenic conditions. Part 2: ventricular arrhythmias, channelopathies, and implantable defibrillators (PMID 32596731) is not held as text in the evidence caches, so it was not checked for this topic.
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Only the PubMed abstract of the Recommendations for participation in leisure-time physical activity and competitive sports in patients with arrhythmias and potentially arrhythmogenic conditions: Part 1: Supraventricular arrhythmias. A position statement of the Section of Sports Cardiology and Exercise from the European Association of Preventive Cardiology (EAPC) and the European Heart Rhythm Association (EHRA), both associations of the European Society of Cardiology (PMID 32597206) is held; its full text is not held and was not checked for this topic.
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Only the PubMed abstract of the Echocardiographic assessment of aortic stenosis: a practical guideline from the British Society of Echocardiography (PMID 33709955) is held; its full text is not held and was not checked for this topic.
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The European Society of Hypertension position paper on renal denervation 2021 (PMID 34261957) is not held as text in the evidence caches, so it was not checked for this topic.
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Only the PubMed abstract of the Update on pulmonary embolism in pregnancy and post-partum: The Society of Obstetric Medicine of Australia and New Zealand Position Statement on Pulmonary Embolism in Pregnancy and Post-partum (PMID 34435660) is held; its full text is not held and was not checked for this topic.
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The 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure (PMID 34447992) are not held as text in the evidence caches, so they were not checked for this topic.
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The 2021 ESC/EACTS Guidelines for the management of valvular heart disease (PMID 34453165) are not held as text in the evidence caches, so they were not checked for this topic.
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The 2021 ACC/AHA/SCAI Guideline for Coronary Artery Revascularization (PMID 34882435) is not held as text in the evidence caches, so it was not checked for this topic.
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The Multi-modality imaging assessment of native valvular regurgitation: an EACVI and ESC council of valvular heart disease position paper (PMID 35292799) is not held as text in the evidence caches, so it was not checked for this topic.
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The 2022 ESC Guidelines on cardio-oncology (PMID 36017568) are not held as text in the evidence caches, so they were not checked for this topic.
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Only the PubMed abstract of the Update from the National Hypertension Taskforce of Australia: prevalence, treatment and control rates of hypertension between 2022 and 2024 - implications and future directions (PMID 42223442) is held; its full text is not held and was not checked for this topic.
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Only the PubMed abstract of the Renal denervation in the management of hypertension in adults. A clinical consensus statement of the ESC Council on Hypertension and the European Association of Percutaneous Cardiovascular Interventions (EAPCI) (PMID 36790101) is held; its full text is not held and was not checked for this topic.
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Only the PubMed abstract of the 2023 HRS expert consensus statement on the management of arrhythmias during pregnancy (PMID 37211147) is held; its full text is not held and was not checked for this topic.
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Only the PubMed abstract of the 2023 ESH Guidelines for the management of arterial hypertension (PMID 37345492) is held; its full text is not held and was not checked for this topic.
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The Multi-modality imaging in aortic stenosis: an EACVI clinical consensus document (PMID 37395329) is not held as text in the evidence caches, so it was not checked for this topic.
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The 2023 Focused Update of the 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure (PMID 37622666) are not held as text in the evidence caches, so they were not checked for this topic.
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Only the PubMed abstract of the 2023 Australian guideline for assessing and managing cardiovascular disease risk (PMID 38623719) is held; its full text is not held and was not checked for this topic.
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Only the PubMed abstract of the A summary of the 2023 Society of Obstetric Medicine of Australia and New Zealand (SOMANZ) hypertension in pregnancy guideline (PMID 38763516) is held; its full text is not held and was not checked for this topic.
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The SCAI Position Statement on Renal Denervation for Hypertension: Patient Selection, Operator Competence, Training and Techniques, and Organizational Recommendations (PMID 39129887) is not held as text in the evidence caches, so it was not checked for this topic.
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Only the PubMed abstract of the 2024 ACC/AHA/AACVPR/APMA/ABC/SCAI/SVM/SVN/SVS/SIR/VESS Guideline for the Management of Lower Extremity Peripheral Artery Disease (PMID 38743805) is held; its full text is not held and was not checked for this topic.
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The 2024 CSANZ Position Statement on Indications, Assessment and Monitoring of Structural and Valvular Heart Disease With Transthoracic Echocardiography in Adults (PMID 38749800) is not held as text in its version of record, so it was not checked for this topic; only its PubMed abstract is quotable.
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Only the PubMed abstract of the 2024 HRS expert consensus statement on arrhythmias in the athlete: Evaluation, treatment, and return to play (PMID 38763377) is held; its full text is not held and was not checked for this topic.
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Only the PubMed abstract of the Renal Denervation for the Treatment of Hypertension (PMID 39101202) is held; its full text is not held and was not checked for this topic.
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Only the PubMed abstract of the Pulmonary hypertension associated with left heart disease (PMID 39209478) is held; its full text is not held and was not checked for this topic.
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Only the PubMed abstract of the Investigation and management of resistant hypertension: British and Irish Hypertension Society position statement (PMID 39653728) is held; its full text is not held and was not checked for this topic.
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The role of multi-modality imaging in multiple valvular heart diseases: a clinical consensus statement of the European Association of Cardiovascular Imaging of the European Society of Cardiology (PMID 39874243) is not held as text in the evidence caches, so it was not checked for this topic.
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Only the PubMed abstract of the Guidelines for the Echocardiographic Assessment of the Right Heart in Adults and Special Considerations in Pulmonary Hypertension: Recommendations from the American Society of Echocardiography (PMID 40044341) is held; its full text is not held and was not checked for this topic.
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The NHFA/CSANZ Comprehensive Australian Clinical Guideline for Diagnosing and Managing Acute Coronary Syndromes 2025 (PMID 40180468) is not held as text in its version of record, so it was not checked for this topic.
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Only the PubMed abstract of the Automated office blood pressure measurement: a Hypertension Australia and National Hypertension Taskforce of Australia position statement (PMID 40534535) is held; its full text is not held and was not checked for this topic.
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The Management of aortic stenosis and chronic heart failure: A clinical consensus statement of the Heart Failure Association (HFA) and the European Association of Percutaneous Cardiovascular Interventions (EAPCI) of the ESC (PMID 40948025) is not held as text in the evidence caches, so it was not checked for this topic.
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The JCS/JPCPHS 2025 Guideline on Pulmonary Hypertension and Pulmonary Embolism/Deep Vein Thrombosis (PMID 42386545) is not held as text in the evidence caches, so it was not checked for this topic.
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The HRS/ISACHD/PACES scientific statement on provider and institutional best practices for comprehensive adult congenital heart disease electrophysiology programs (PMID 41679448) is not held as text in the evidence caches, so it was not checked for this topic.
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The ACR Appropriateness Criteria® Renovascular Hypertension: Update 2026 (PMID 41817473) is not held as text in the evidence caches, so it was not checked for this topic.
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The Masters athletes with abnormal cardiovascular findings: a clinical consensus statement of the European Association of Preventive Cardiology of the ESC and the American College of Cardiology (PMID 41947397) is not held as text in the evidence caches, so it was not checked for this topic.
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The Correction to "Screening for Endocrine Hypertension: An Endocrine Society Scientific Statement" (PMID 42555025) is not held as text in the evidence caches, so it was not checked for this topic.
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The 2026 ESC Guidelines for the management of cardiovascular disease and chronic kidney disease, in collaboration with the European Renal Association (ERA) (PMID 42661426) are not held as text in their version of record, so they were not checked for this topic.
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The Clinical Considerations for the Care of the Tactical Athlete With Cardiovascular Abnormalities (PMID 42691142) is not held as text in the evidence caches, so it was not checked for this topic.
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Only the PubMed abstract of the European Respiratory Society clinical practice guidelines update for the treatment of pulmonary arterial hypertension (PMID 42705705) is held; its full text is not held and was not checked for this topic.
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Only the PubMed abstract of the 2026 AHA/ACC/ACS/ASNC/HRS/SCA/SCCT/SCMR/SVM Guideline for Perioperative Cardiovascular Management for Noncardiac Surgery (PMID 42804570) is held; its full text is not held and was not checked for this topic.
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The Optimizing Blood Pressure Care in Patients With Resistant Hypertension: 2026 ACC Expert Consensus Decision Pathway (PMID 42831847) is not held as text in the evidence caches, so it was not checked for this topic.
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The Abnormal electrocardiogram findings in athletes (PMID 40996312) is not held as text in the evidence caches, so it was not checked for this topic.
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The Correction to: Abnormal electrocardiogram findings in athletes: A consensus statement of the European Association of Preventive Cardiology of the European Society of Cardiology (PMID 41395827) is not held as text in the evidence caches, so it was not checked for this topic.
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The Cardiac evaluation of paediatric athletes (PMID 41967042) is not held as text in the evidence caches, so it was not checked for this topic.
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The Opposing Legislative Mandates for ECG Screening in Competitive Athletes: A Report of the American College of Cardiology Solution Set Oversight Committee (PMID 41686106) is not held as text in the evidence caches, so it was not checked for this topic.
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Only the PubMed abstract of the Canadian Cardiovascular Society 2022 guidelines for cardiovascular interventions in adults with congenital heart disease (PMID 35460862) is held; its full text is not held and was not checked for this topic.
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The PubMed census for this topic also found the 2018 AHA scientific statement on cardiovascular health in Turner syndrome (PMID 30354301) and the 2024 PICS/AEPC/APPCS/CSANZ/SCAI/SOLACI expert consensus statement on cardiac catheterization for pediatric patients and adults with congenital heart disease (PMID 38099915); neither is held as text in the evidence caches, so neither was checked for this topic.
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The held guidelines below mention coarctation, and no row from them is used here; each line gives the reason.
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ACC/AHA/HRS 2017 syncope (PMID 28280231): coarctation appears only as an entry in a table of syncope causes; no coarctation row.
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AHA/ACC/HRS 2017 ventricular arrhythmias (PMID 29084731): coarctation appears in a congenital sudden-death risk table and in its definition of moderate-complexity disease; sudden death risk is outside this page and no coarctation row is given.
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2019 ESC/EAS dyslipidaemias (PMID 31504418): the text-search hits are "HMG-CoA", not coarctation.
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ACC/AHA 2020 valvular heart disease (PMID 33332150): its bicuspid valve recommendations and supporting text that name coarctation are older than the ACC/AHA 2022 aortic and ACC/AHA 2025 congenital rows on the same questions, which are given above.
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AHA/ACC/HFSA 2022 heart failure (PMID 35363499): coarctation appears only in a reference title.
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ESC 2022 non-cardiac surgery (PMID 36017553): coarctation is named only as an example when choosing arterial line location during anaesthesia; perioperative care is outside this page.
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ESC 2023 cardiomyopathies (PMID 37622657): coarctation is named only among causes to rule out in infants with dilated cardiomyopathy.
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AHA/ACC 2024 perioperative (PMID 39316661): coarctation is listed among obstructive left-sided lesions in a congenital risk table; perioperative care is outside this page.
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2025 ESC/EAS dyslipidaemias focused update (PMID 40878289): the text-search hit is "acyl-CoA", not coarctation.
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NHFA/CSANZ 2025 ACS guideline summary in Med J Aust (PMID 41693087): the text-search hits are "HMG-CoA", not coarctation.
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ACC/AHA 2026 dyslipidemia (PMID 41824552): the text-search hits are "HMG-CoA" and "acyl-CoA", not coarctation.
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These held documents were searched and contain no mention of coarctation: 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC)Endorsed by: The European Association for Cardio-Thoracic Surgery (EACTS) (PMID 26320112); 2015 ACC/AHA/HRS Guideline for the Management of Adult Patients With Supraventricular Tachycardia: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society (PMID 26399663); Spontaneous Coronary Artery Dissection: Current State of the Science: A Scientific Statement From the American Heart Association (PMID 29472380); European Society of Cardiology, acute cardiovascular care association, SCAD study group: a position paper on spontaneous coronary artery dissection (PMID 29481627); Practical Instructions for the 2018 ESC Guidelines for the diagnosis and management of syncope (PMID 29562291); 2018 ESC Guidelines for the diagnosis and management of syncope (PMID 29562304); National Heart Foundation of Australia and Cardiac Society of Australia and New Zealand: Australian clinical guidelines for the management of heart failure 2018 (PMID 30067937); National Heart Foundation of Australia and Cardiac Society of Australia and New Zealand: Guidelines for the Prevention, Detection, and Management of Heart Failure in Australia 2018 (PMID 30077227); 2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society (PMID 30412709); Fourth Universal Definition of Myocardial Infarction (2018) (PMID 30571511); 2019 ACC/AHA Guideline on the Primary Prevention of Cardiovascular Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines (PMID 30879355); 2019 ESC Guidelines for the management of patients with supraventricular tachycardiaThe Task Force for the management of patients with supraventricular tachycardia of the European Society of Cardiology (ESC) (PMID 31504425); 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism developed in collaboration with the European Respiratory Society (ERS) (PMID 31504429); 2021 Guideline for the Prevention of Stroke in Patients With Stroke and Transient Ischemic Attack: A Guideline From the American Heart Association/American Stroke Association (PMID 34024117); Mechanical Complications of Acute Myocardial Infarction: A Scientific Statement From the American Heart Association (PMID 34126755); 2021 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy (PMID 34455430); 2021 ESC Guidelines on cardiovascular disease prevention in clinical practice (PMID 34458905); 2021 AHA/ACC/ASE/CHEST/SAEM/SCCT/SCMR Guideline for the Evaluation and Diagnosis of Chest Pain: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines (PMID 34709879); 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension (PMID 36017548); 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death (PMID 36017572); 2023 AHA/ACC/ACCP/ASPC/NLA/PCNA Guideline for the Management of Patients With Chronic Coronary Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines (PMID 37471501); 2023 ESC Guidelines for the management of acute coronary syndromes (PMID 37622654); 2023 ESC Guidelines for the management of endocarditis (PMID 37622656); 2023 ESC Guidelines for the management of cardiovascular disease in patients with diabetes (PMID 37622663); 2023 ACC/AHA/ACCP/HRS Guideline for the Diagnosis and Management of Atrial Fibrillation: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines (PMID 38033089); 2024 Australia-New Zealand Expert Consensus Statement on Cardiac Amyloidosis (PMID 38570258); 2024 European Heart Rhythm Association/Heart Rhythm Society/Asia Pacific Heart Rhythm Society/Latin American Heart Rhythm Society expert consensus statement on catheter and surgical ablation of atrial fibrillation (PMID 38587017); 2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines (PMID 38718139); Definition, classification and diagnosis of pulmonary hypertension (PMID 39209475); Treatment algorithm for pulmonary arterial hypertension (PMID 39209476); 2024 ESC Guidelines for the management of chronic coronary syndromes (PMID 39210710); 2024 ESC Guidelines for the management of atrial fibrillation developed in collaboration with the European Association for Cardio-Thoracic Surgery (EACTS) (PMID 39210723); 2024 Guideline for the Primary Prevention of Stroke: A Guideline From the American Heart Association/American Stroke Association (PMID 39429201); 2024 ACC Expert Consensus Decision Pathway on Strategies and Criteria for the Diagnosis and Management of Myocarditis: A Report of the American College of Cardiology Solution Set Oversight Committee (PMID 39665703); Adrenal Vein Sampling for Primary Aldosteronism: Recommendations From the Australian and New Zealand Working Group (PMID 39360599); 2025 ACC/AHA/ACEP/NAEMSP/SCAI Guideline for the Management of Patients With Acute Coronary Syndromes: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines (PMID 40014670); 2025 ESC/EACTS Guidelines for the management of valvular heart disease (PMID 40878295); 2025 ESC Guidelines for the management of myocarditis and pericarditis (PMID 40878297); Part 10: Adult and Pediatric Special Circumstances of Resuscitation: 2025 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care (PMID 41122889); 10. Cardiovascular Disease and Risk Management: Standards of Care in Diabetes-2026 (PMID 41358899); 2026 AHA/ACC/ACCP/ACEP/CHEST/SCAI/SHM/SIR/SVM/SVN Guideline for the Evaluation and Management of Acute Pulmonary Embolism in Adults: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines (PMID 41712677); 2026 AHA/ACC/ADA/ASN Guideline for the Prevention, Detection, Evaluation, and Management of Cardiovascular-Kidney-Metabolic Syndrome: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines (PMID 42263157); 2026 ESC Guidelines on cardiac rehabilitation (PMID 42661418); 2026 ESC Guidelines for the management of heart failure (PMID 42661420); Fifth Universal Definition of Myocardial Infarction (2026) (PMID 42661425); Guideline 11.10 – Resuscitation in Special Circumstances (approved July 2026) (anzcor-11-10).
Exam pearls
- ESC 2020 sees coarctation as part of a generalised arteriopathy, not only a narrowing of the aorta.[1]
- Upper-to-lower limb systolic gradient: ≥20 mmHg in ESC 2020 text, >20 mm Hg in ACC/AHA 2025 Table 25.[1][2]
- Aortic isthmus ratio: smallest coarctation diameter over the descending aorta at the diaphragm; expert consensus generally takes <0.5 as the threshold for hemodynamic (anatomic) significance, and recent empirical data suggest <0.7 may be optimal for recommending repair (ACC/AHA 2025).[2]
- Exercise-induced hypertension, as ACC/AHA 2025 generally defines it: peak systolic >210 mm Hg in males and >190 mm Hg in females.[2]
- Blood pressure preferably in the right arm (except with an aberrant right subclavian artery) at every clinical encounter (ACC/AHA 2025).[2]
- CT rather than CMR after a coarctation stent, because of stent artefacts on CMR (ACC/AHA 2025).[2]
- Balloon angioplasty in adults only for re-dilatation of previously stented aortas (ESC 2020).[1]
- Repaired coarctation, asymptomatic and normotensive with normal aorta and exercise capacity: AP class IIA (ACC/AHA 2025).[2]
References9ShowHide
- [1]Baumgartner H, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J, 2021.PMID 32860028
- [2]Gurvitz M, et al. 2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation, 2026.PMID 41411375
- [3]Mazzolai L, et al. 2024 ESC Guidelines for the management of peripheral arterial and aortic diseases. Eur Heart J, 2024.PMID 39210722
- [4]Isselbacher EM, 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. Circulation, 2022.PMID 36322642
- [5]McEvoy JW, et al. 2024 ESC Guidelines for the management of elevated blood pressure and hypertension. Eur Heart J, 2024.PMID 39210715
- [6]Jones DW, et al. 2025 AHA/ACC/AANP/AAPA/ABC/ACCP/ACPM/AGS/AMA/ASPC/NMA/PCNA/SGIM Guideline for the Prevention, Detection, Evaluation, and Management of High Blood Pressure in Adults: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol, 2025.PMID 40815242
- [7]De Backer J, et al. 2025 ESC Guidelines for the management of cardiovascular disease and pregnancy. Eur Heart J, 2025.PMID 40878294
- [8]Pelliccia A, et al. 2020 ESC Guidelines on sports cardiology and exercise in patients with cardiovascular disease. Eur Heart J, 2021.PMID 32860412
- [9]Kim JH, et al. Clinical Considerations for Competitive Sports Participation for Athletes With Cardiovascular Abnormalities: A Scientific Statement From the American Heart Association and American College of Cardiology. J Am Coll Cardiol, 2025.PMID 39976316