Aortic Coarctation

Aka: Aortic Coarctation, Coarctation of the Aorta

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II. Epidemiology

  1. Incidence: 6% of Congenital Heart Disease
  2. More common in Males by a ratio of 1.7 to 1

III. Associated Conditions

  1. Turner's Syndrome
  2. Bicuspid aortic valves (50%)
  3. Aortic Coarctation Syndrome
    1. Patent Ductus Arteriosus (PDA)
    2. Tubular hypoplasia the aortic isthmus
    3. Ventricular Septal Defect (VSD)
    4. Congestive Heart Failure

IV. Pathophysiology

  1. Constriction of aorta near isthmus
    1. Ductus arteriosus insertion
    2. Occurs below origin of left subclavian

V. Etiology

  1. Congenital malformation
  2. Atherosclerosis
  3. Thrombosis
  4. Aortic aneurysm
  5. External compression

VI. Symptoms (May be asymptomatic)

  1. Epistaxis
  2. Intermittent Claudication
  3. Dizziness
  4. Headaches
  5. Tinnitus

VII. Signs

  1. Arterial Hypertension
  2. Upper extremity Hypertension
    1. Arm to leg systolic Blood Pressure difference >20 mmHg
  3. Diminished or delayed femoral pulses
    1. Femoral pulses lag brachial pulses (Brachial-femoral delay)
    2. Low or unobtainable Blood Pressure in the lower extremities
  4. Palpable pulsating arteries about Scapula and axilla

VIII. Diagnostics: Electrocardiogram

  1. Right Ventricular Hypertrophy (Infants)
  2. Left Ventricular Hypertrophy (older child and adults)

IX. Imaging

  1. Chest XRay
    1. First-line initial screening study
    2. Older Children, adolescents or young adults
      1. Notching of the lower rib borders (figure 3 sign)
      2. Displaced esophageal shadow rightward
    3. Infants with Congestive Heart Failure signs
      1. Cardiomegaly
      2. Pulmonary venous congestion
  2. Transthoracic Echocardiogram
    1. Preferred diagnostic test in children
    2. Findings
      1. Dilated Right Ventricle
      2. Hypoplastic Left Ventricle (Infants)
      3. Coarctation site
  3. MR Angiogram Chest
    1. Preferred diagnostic test in adults

X. Management

  1. Infant with Congestive Heart Failure
    1. PGE1 to open ductus arteriosus (Neonates)
    2. Surgical Repair

XI. Management: Surgical Repair

  1. Indications
    1. Transcoarctation pressure gradient >30 mmHg
    2. Routine repair between ages 2 and 4
    3. Urgent repair
      1. Circulatory shock
      2. Cardiomegaly
      3. Severe Hypertension
      4. Severe Congestive Heart Failure
  2. Techniques
    1. Subclavian flap repair
    2. End to end anastomosis with resection
    3. Balloon Angioplasty (for recoarctation)

XII. References

  1. Cyran (1998) PREP review lecture, October, Phoenix
  2. Merenstein (1994) Pediatrics, Lange
  3. Charles (2017) Am Fam Physician 96(7): 453-61 [PubMed]
  4. Saenz (1999) Am Fam Physician 59(7):1857-66 [PubMed]

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