MioAtlas
G4: Congenital heart disease

Aortic coarctation

Congenital stenosis or luminal narrowing of the thoracic aorta characteristically located in the region of the ductus or ligamentum arteriosum insertion (isthmic area), immediately distal to origin of left subclavian artery.

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Definition

Congenital stenosis or luminal narrowing of the thoracic aorta characteristically located in the region of the ductus or ligamentum arteriosum insertion (isthmic area), immediately distal to origin of left subclavian artery. It creates a fixed mechanical obstruction to left ventricular ejection, generating proximal arterial hypertension (upper extremities and cerebral circulation), distal hypoperfusion (lower extremities), concentric left ventricular hypertrophy, and development of a collateral bypass vascular network (internal mammary, intercostal, and scapular arteries) [C5] [C6] [G4-09].

Severity criteria

  • Mild (L): Mild isthmic stenosis with arm-leg systolic gradient < 10 mmHg at rest, without appreciable radio-femoral delay or significant hypertension [C5] [C6].
  • Moderate (M): Moderate isthmic stenosis with arm-leg pressure gradient 10–19 mmHg at rest, or manifest arterial hypertension on exercise testing [C4] [C5].
  • Severe / Significant / Indication for intervention (G):
    • Arm-leg systolic gradient ≥ 20 mmHg at rest measured by sphygmomanometry or catheterization (peak-to-peak gradient) [C5] [C6].
    • Upper-extremity arterial hypertension (SBP ≥ 140 mmHg) with diminished, thready, and delayed femoral pulses (pathognomonic radio-femoral delay) [C5] [G4-09].
    • Descending aorta Doppler showing high-velocity systolic acceleration (Vmax > 3.0 m/s) with persistence of anterograde flow throughout diastole (diastolic tail or sawtooth flow pattern) [C5] [C6].

Findings by layer

Hemodynamics

  • Arm-leg systolic pressure gradient (arm-leg pressure gradient): L < 10 mmHg; M 10–19 mmHg; G ≥ 20 mmHg [C5] [C6].
  • Upper extremity blood pressure: Proximal arterial hypertension (L normal/130–139, M 140–159, G ≥ 160 mmHg) [C5] [C6].
  • Trans-coarctation aortic gradient (mean transaortic gradient / peak transaortic velocity): Increased systolic velocity with continuous anterograde diastolic continuation [C5] [C6].

Rhythm

  • Normal sinus rhythm habitual [C5].
  • Atrial fibrillation in adults due to chronic hypertensive heart disease [C4] [C5].

ECG

  • Concentric left ventricular hypertrophy from pressure overload (left ventricular hypertrophy voltage criteria, LV systolic overload pattern), with positive Sokolow-Lyon and Cornell voltage criteria accompanied by ST-segment depression and asymmetric T-wave inversion in lateral precordial leads (I, aVL, V5–V6) in moderate and severe forms [E1] [G4-09].

Heart sounds and murmurs

  • Interscapular systolic / continuous murmur (back area): Midsystolic or late-systolic ejection murmur, audible in anterior left infraclavicular fossa but with maximal intensity in posterior left interscapular region (at level of T4–T6 over descending thoracic aorta) [G1-14] [G4-09].
  • In severe forms with collaterals: Diffuse continuous or early systolic murmurs over dorsal and axillary chest wall due to flow through dilated intercostal arteries [G1-14] [G4-09].
  • Second heart sound (S2): Accentuated aortic component (A2) due to high diastolic pressure in proximal aortic root [G1-11] [G4-09].
  • Ejection click: Frequent at apex or aortic area if bicuspid aortic valve coexists (present in 50–70% of cases) [G1-16] [C5].
  • Fourth heart sound (S4): Atrial gallop in severe forms with stiff left ventricular hypertrophy [G1-13].

Arterial pulse

  • Radio-femoral delay and femoral pulsus parvus: Pulse in femoral arteries is markedly reduced in amplitude (parvus), slow-rising, and delayed in time relative to simultaneous palpation of radial or brachial pulse [B4] [G4-09].
  • Hyperdynamic and bounding pulses in upper extremities [B4].

Jugular venous pulse

  • Generally normal; prominent a wave if LV septal hypertrophy impairs RV filling (Bernheim effect) [B3].

Maneuvers

  • Isometric handgrip: Increases proximal afterload and increases systolic gradient and interscapular aortic murmur intensity [B1].
  • Dynamic exercise: Produces disproportionate hypertensive rise in upper extremity systolic blood pressure and widens arm-leg gradient [C5] [C6].

Simulator severity grades

Simulator severity grades: Mild, Moderate, Severe
Baseline rhythm: Normal Sinus Rhythm

Also known as: Aortic coarctation · Coarctation of the aorta · Aortic isthmic stenosis · CoA

Other pathologies in this group

Sources

  1. [B1] Bedside diagnosis of systolic murmurs . N Engl J Med (1988) . DOI: 10.1056/NEJM198806163182404 PubMed: 2897627
  2. [B3] The jugular venous pressure revisited . Cleve Clin J Med (2013) . DOI: 10.3949/ccjm.80a.13039 PubMed: 24085809
  3. [B4] The Carotid Pulse . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250154
  4. [C4] 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) . DOI: 10.1161/CIR.0000000000001402 PubMed: 41411375
  5. [C5] 2020 ESC Guidelines for the management of adult congenital heart disease . Eur Heart J (2021) . DOI: 10.1093/eurheartj/ehaa554 PubMed: 32860028
  6. [C6] 2024 ESC Guidelines for the management of peripheral arterial and aortic diseases . Eur Heart J (2024) . DOI: 10.1093/eurheartj/ehae179 PubMed: 39210722
  7. [E1] AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram: part V: electrocardiogram changes associated with cardiac chamber hypertrophy: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society: endorsed by the International Society for Computerized Electrocardiology . Circulation (2009) . DOI: 10.1161/CIRCULATIONAHA.108.191097 PubMed: 19228820
  8. [E2] AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram: part III: intraventricular conduction disturbances: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society: endorsed by the International Society for Computerized Electrocardiology . Circulation (2009) . DOI: 10.1161/CIRCULATIONAHA.108.191095 PubMed: 19228822
  9. [G1-11] The Second Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250183
  10. [G1-13] The Fourth Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250185
  11. [G1-14] Systolic Murmurs . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250186
  12. [G1-16] Ejection Clicks . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250188
  13. [G4-09] Congenital heart disease in adults. First of two parts . N Engl J Med (2000) . DOI: 10.1056/NEJM200001273420407 PubMed: 10648769