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Key Features

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Essentials of Diagnosis
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  • Absent or diminished femoral pulses

  • Upper to lower extremity systolic blood pressure gradient of > 20 mm Hg

  • Blowing systolic murmur in the back or left axilla

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General Considerations
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  • Defined as narrowing in the aortic arch that usually occurs in the proximal descending aorta near the takeoff of the left subclavian artery near the ductus arteriosus

  • Accounts for about 6% of all congenital heart disease

  • Three times as many males as females are affected

  • Presents insidiously in 60% of children with no symptoms in infancy

  • Many affected females have Turner syndrome

  • Incidence of associated bicuspid aortic valve with coarctation is 80–85%

  • The abdominal aorta is rarely involved

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Clinical Findings

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Symptoms and Signs
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  • Decreased or absent femoral pulses

  • Infants with severe coarctation have equal upper and lower extremity pulses from birth until the ductus arteriosus closes

  • Heart failure in first month of life may be caused by

    • Coarctation alone

    • Coarctation combination with ventricular septal defect, atrial septal defect, or other congenital cardiac anomalies

  • Pulse and blood pressure (> 15 mm Hg) discrepancy between the arms and legs

  • Pulses in the legs are diminished or absent

  • The left subclavian artery is occasionally involved in the coarctation, in which case the left brachial pulse is also weak

  • Pathognomonic murmur is heard in the left axilla and the left back

  • Systolic ejection murmur is often heard at the aortic area and the lower left sternal border along with an apical ejection click if there is an associated bicuspid aortic valve

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Diagnosis

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Imaging
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  • Chest radiography

    • May show a normal-sized heart or more often some degree of left ventricular (LV) enlargement

    • Aorta proximal to the coarctation is prominent

    • Aortic outline may indent at the level of the coarctation

    • Poststenotic segment is often dilated

    • This combination of abnormalities results in the "figure 3" sign

    • Notching of the ribs caused by marked enlargement of the intercostal collaterals

    • Marked cardiac enlargement and pulmonary venous congestion occur in patients with severe coarctation and associated heart failure

  • Echocardiography

    • Two-dimensional echocardiography and color-flow Doppler are used to visualize the coarctation directly

    • Continuous-wave Doppler estimates the degree of obstruction

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Diagnostic Procedures
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  • Electrocardiography

    • May be normal or may show LV hypertrophy in older children

    • Usually shows right ventricular hypertrophy in infants with severe coarctation because the right ventricle serves as the systemic ventricle during fetal life

  • Cardiac catheterization and angiocardiography

    • Rarely performed for diagnosis in infants or children with coarctation

    • Used, however, when transcatheter intervention is planned

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Treatment

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  • Resuscitative measures include PGE2 infusion (0.05–0.1 μg/kg/min) to reopen the ductus arteriosus

  • Inotropic support is frequently needed for end-organ damage distal to the coarctation

  • Once stabilized, the infant should undergo corrective repair

  • Balloon angioplasty

    • Some performed as palliative ...

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