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OverviewAetiologyPathophysiologyClinical features and red flagsInvestigationsDifferential diagnosisManagementComplicationsMonitoring and follow-upSpecial situationsCommon pitfallsQuestionsSources
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Educational draft · awaiting clinical reviewThe full textbook explains uncertainty but does not replace live national or local guidance, specialist advice, or current prescribing information.

Coarctation of the aorta

Cardiology · UK scope · Sources checked 27 Aug 2026 · Clinical review pending

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Time-critical presentation

Acute severe chest or back pain, neurological deficit, haemodynamic collapse or rapidly worsening aortic regurgitation in coarctation may indicate aortic dissection/rupture and needs immediate aortic imaging and specialist transfer.

Overview

Detect native or recurrent coarctation, assess whole-aorta complications and maintain lifelong blood-pressure and ACHD surveillance.

Most coarctation lies near the ductal insertion. Adults may present with resistant hypertension, headache, exertional leg fatigue or an incidental arm-leg pressure difference; previously repaired patients may develop recoarctation or aneurysm.

Assessment belongs in an ACHD/aortic service. The team integrates blood pressure, collateral flow, anatomy, ventricular response and associated valve/aortic disease when choosing stent, surgery or surveillance.

Key points

  • Measure blood pressure in both arms and at least one leg; upper-limb hypertension with a lower leg pressure and radiofemoral delay is the bedside clue.
  • A systolic murmur over the left infraclavicular area or back and collateral bruits may be present.
  • Coarctation is an aortopathy, not simply a focal narrowing; inspect the arch, repair site and entire thoracic aorta.
  • Bicuspid aortic valve and ascending-aortic dilatation are common associated lesions.
  • CMR is preferred for serial anatomy and flow; CT is valuable for stents, calcification and emergency detail.
  • Intervention is generally indicated for significant obstruction with upper-limb hypertension, particularly when an invasive peak-to-peak gradient is at least 20 mmHg.
  • Hypertension and aortic complications can persist after an anatomically successful repair, so follow-up is lifelong.

Aetiology

01

Congenital juxtaductal narrowing

Abnormal development of the aortic arch and tissue near the ductal insertion produces a fixed narrowing, usually just beyond the left subclavian artery. Severity varies considerably.

02

Associated congenital aortopathy

Bicuspid aortic valve, ascending-aortic dilatation and ventricular septal defect occur more often in affected people. These associated lesions influence presentation, surveillance and complication risk.

03

Residual or recurrent obstruction

After childhood surgery or catheter treatment, residual narrowing, scar-related restenosis or altered growth may recreate obstruction. Repair sites can also develop aneurysmal change.

Pathophysiology

  1. 1
    Fixed outflow obstruction

    The narrowed aortic segment increases resistance between the upper and lower body. Pressure rises proximally while distal pulse amplitude and perfusion pressure fall.

  2. 2
    Ventricular and vascular adaptation

    The left ventricle hypertrophies in response to increased afterload. Neurohormonal and vascular dysfunction can sustain upper-body hypertension even when the anatomical obstruction is later relieved.

  3. 3
    Collateral circulation develops

    Intercostal and other arterial collaterals enlarge to bypass the narrowing. They may produce posterior bruits or rib notching and can mask the resting arm–leg pressure difference.

  4. 4
    Aortic wall stress persists

    Abnormal flow, hypertension and associated aortopathy expose the ascending aorta and repair site to continuing stress. Dilatation, aneurysm or dissection may occur despite previous intervention.

Clinical features and red flags

Arm-leg discrepancy

Raised arm systolic pressure with lower leg pressure, weak/delayed femoral pulses and radiofemoral delay.

Collateral circulation

Intercostal collateral bruits, scapular pulsation or rib notching on chest radiograph may accompany longstanding obstruction.

Adult symptoms

Headache, epistaxis, exertional leg fatigue, cold feet, chest pain or dyspnoea; many people are asymptomatic despite hypertension.

Associated disease

Bicuspid aortic valve, ascending-aortic dilatation, VSD and intracranial aneurysm risk should be considered.

Aortic emergencyRed flag

Sudden tearing chest/back pain, neurological signs or shock suggests dissection or rupture.

Investigations

  1. 01
    Four-limb blood pressure and pulse examinationFirst step
    Why
    Screen for haemodynamic obstruction and hypertension.
    Interpretation and limitations
    A resting systolic arm-leg difference of 20 mmHg or more supports significant coarctation, but collateral flow can mask the gradient.
  2. 02
    Transthoracic echocardiography
    Why
    Assess Doppler flow, LV hypertrophy/function, bicuspid valve and proximal aorta.
    Interpretation and limitations
    Doppler gradients may overestimate or underestimate catheter gradients; look for diastolic run-off and whole physiology.
  3. 03
    CMR angiography
    Why
    Define arch, narrowing, collateral flow, repair site and aortic dimensions without radiation.
    Interpretation and limitations
    Use serial measurements with the same technique when possible.
  4. 04
    CT angiography
    Why
    Provide high-resolution aortic and stent anatomy, especially in emergencies or when CMR is unsuitable.
    Interpretation and limitations
    Balance diagnostic value against radiation and contrast exposure.
  5. 05
    Ambulatory BP and exercise testing
    Why
    Detect masked or exercise-induced hypertension and assess functional response.
    Interpretation and limitations

Differential diagnosis

01

Essential hypertension

Primary hypertension is common but does not usually cause radiofemoral delay, a consistent arm–leg pressure difference or an interscapular murmur. Four-limb assessment helps separate the patterns.

02

Renovascular or endocrine hypertension

Renal artery disease and endocrine disorders can cause early or resistant hypertension without focal arch obstruction. Biochemical clues and renal imaging guide evaluation when limb findings are absent.

03

Lower-limb peripheral arterial disease

Aortoiliac or leg arterial disease may lower ankle pressure and weaken femoral pulses. Asymmetry, distal vascular signs and imaging below a normal aortic arch favour acquired peripheral disease.

04

Large-vessel arteritis

Inflammatory aortic disease can create stenoses, pulse differences and hypertension. Systemic inflammatory features, multifocal arterial involvement and vessel-wall imaging distinguish it from a congenital focal lesion.

05

Subclavian disease or measurement artefact

Proximal arm-artery stenosis or an inaccurate cuff reading can distort the apparent arm–leg gradient. Repeated four-limb measurements, pulse examination and arch imaging clarify the pattern.

Management

01FirstSuspected native or recurrent coarctationFirst stepYoung or resistant hypertension, arm-leg BP difference, radiofemoral delay or previous repair.+
  1. 1Measure both-arm and leg blood pressures, examine femoral pulses and listen over the precordium and back.
  2. 2Obtain ECG and expert echo for LV, valve and Doppler assessment.
  3. 3Refer to an ACHD/aortic service and obtain CMR or CT of the whole thoracic aorta.
  4. 4Use ambulatory BP and, when appropriate, exercise testing to define the true hypertensive burden.
02NextIntervention assessmentSignificant anatomical narrowing with hypertension, symptoms, LV effects or a meaningful invasive gradient.+
  1. 1Confirm anatomy, collaterals and associated aortic/valve disease in the ACHD multidisciplinary team.
  2. 2Use catheter haemodynamics when needed; a peak-to-peak gradient of at least 20 mmHg supports intervention in a hypertensive patient.

Complications

01

Persistent systemic hypertension

Upper-body hypertension may continue after technically successful repair because vascular dysfunction persists. It increases risk of left-ventricular hypertrophy, heart failure, coronary disease and stroke.

02

Aortic aneurysm or dissection

Hypertension, intrinsic aortopathy and repair-site injury can weaken the aortic wall. Aneurysm, rupture or dissection may present with abrupt chest or back pain and collapse.

03

Recoarctation

Residual tissue, scarring or vessel growth can restore a clinically important obstruction. Recurrent arm–leg pressure disparity, hypertension or exercise intolerance should prompt anatomical reassessment.

04

Bicuspid aortic valve disease

Associated bicuspid valve may develop stenosis or regurgitation, while the ascending aorta can dilate. These processes add ventricular load independently of the coarctation gradient.

05

Cerebrovascular complication

Longstanding hypertension and associated intracranial aneurysm susceptibility increase risk of ischaemic or haemorrhagic neurological events. New severe headache or focal deficit warrants urgent assessment.

Monitoring and follow-up

  • Lifelong ACHD follow-up with arm and leg blood pressure at every clinical review.
  • Use ambulatory BP periodically because masked hypertension is common after repair.
  • Perform serial CMR or CT of the arch, repair/stent and thoracic aorta at specialist-defined intervals.
  • Monitor bicuspid valve function, ascending-aortic size and LV hypertrophy/function by echo.
  • Provide pre-pregnancy aortic imaging and Pregnancy Heart Team review when residual obstruction, hypertension or aortic dilatation is present.

Special situations

Check the right arm

A lesion involving the left subclavian origin can alter the left-arm pressure; both arms reduce misclassification.

Collateral flow masks gradients

Severe anatomical narrowing may coexist with a modest resting pressure difference when collaterals are extensive.

Repair is not cure

Vascular dysfunction sustains lifelong hypertension and premature vascular risk even after relief of obstruction.

Whole-aorta imaging

The repair site can develop aneurysm while the ascending aorta may dilate because of associated bicuspid-valve aortopathy.

Exercise BP adds information

An exaggerated response can reveal clinically relevant physiology not apparent at rest.

Common pitfalls

  1. 01

    Measuring blood pressure in one arm only and never checking a leg.

  2. 02

    Excluding significant coarctation because the resting Doppler or cuff gradient is below 20 mmHg.

  3. 03

    Imaging only the focal narrowing instead of the entire thoracic aorta.

  4. 04

    Stopping follow-up after childhood repair.

  5. 05

    Allowing uncontrolled hypertension before pregnancy or high-static exercise without specialist assessment.

Practice

Two practice questions

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Question 1 of 2
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CardiologyOriginal SBA

Bedside clue

A 29-year-old has upper-limb hypertension and exertional leg fatigue. Which finding most supports coarctation?

Choose one answer, then submit
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Sources and review status4 sources · checked 27 Aug 2026 · clinical review pending+
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Apply principles in context and verify current guidance when a decision affects care. Source check completed 27 Aug 2026; clinical approval remains outstanding.

  • ESC 2020 adult congenital heart disease guidelineCoarctation diagnosis, intervention and follow-up.
  • NICE HTG45: balloon angioplasty with or without stentingUK interventional procedure context.
  • NHS England adult congenital heart servicesACHD network care.
  • ESC 2024 peripheral arterial and aortic diseases guidelineAcute and chronic aortic-disease framework.

Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes

Authoring stateComplete draftClinical stateAwaiting reviewJurisdictionUnited Kingdom
Normal clinic pressure does not exclude a clinically important hypertensive burden after repair.
  • 3Prefer stenting in anatomically suitable adults; use surgery for anatomy not suited to catheter treatment or complex associated disease.
  • 4Image the repair for residual obstruction, dissection or aneurysm and establish lifelong surveillance.
    • 03EscalationSuspected aortic complicationEscalationAcute chest/back pain, collapse, new neurological deficit or rapidly changing aortic signs.+
    1. 1Activate an emergency aortic-syndrome pathway with ABC management and senior cardiothoracic/aortic input.
    2. 2DefinitiveObtain immediate ECG, blood tests and definitive CT aortography unless instability requires direct operative action.
    3. 3DefinitiveControl heart rate and blood pressure under specialist emergency protocols while avoiding delay to definitive treatment.
    4. 4Transfer to an aortic centre for endovascular or surgical management as anatomy dictates.