Coarctation of the aorta
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Introduction

Coarctation of the aortaL-to-R shunt – ACYANOTIC
  • Accounts for up to 10% of congential heart defects
  • Male:female ratio is 2:1
  • Affects 10-20% of patients with Turner’s Syndrome
  • Almost always affects the thoracic aorta, virtually never the abdominal
Coarctation of the aorta
Coarctation of the aorta

Clinical features

  • Collapse at 3 days of age when the PDA naturally closes. The closing of the PDA suddenly creates lots of Afterload.
  • Grey baby
  • Upper body hypertension, lower body hypotension
  • If very severe:
  • If minor
    • May be asymptomatic in young children. Signs may appear as the child grows, such as:
    • Headaches, chest pain, fatigue, claudication in the legs (particularly during physical activity)
    • Heart failure is unlikely to occur if it has not appeared during the neonatal period

Pathology

Coarctation of the aorta is where there is a narrowing of the aorta in the region of the ductus arteriosus. it can be divided into three types:
Narrowing is proximal to ductus arteriosus
  • A PDA can be life-saving (consider IV prostaglandins)
  • Usually the result of abnormally low blood flow in the left side of the heart during fetal life, resulting in hypoplasia of the aorta

Narrowing at the ductus arteriosus

  • Occurs when the DA closes

Narrowing distal to the ductus arteriosus

Examination Findings

  • Hypertension in the upper limbs
  • Reduced / absent femoral pulses
  • Low BP in the lower limbs
  • Murmur
    • Usually grade 2/3
    • Left sterna edge
    • Murmurs in the intercostals spaces – may be heard due to dilation of the intercostal arteries

Signs of Turner’s syndrome in females:

  • Webbed neck
  • Lymphoedema in the feet
  • Square chest
  • Widely spaced nipples

Diagnosis

CXRthe ‘3’ sign in the upper left of the chest. After age 5 there may be rib notching (especially 3rd and 8th ribs) due to intercostals hypertension
BP measurements – upper and lower limbs
Echo with Doppler flow
ECG

  • In neonates – RBBB, RVH
  • In older children – LVH

Prognosis

Can result in:

Treatment

The sick neonate:
  • Correct acidosis
  • Fluids + frusemide
  • High pressure ventilation
  • IV prostaglandins
  • Correct hypertension
    • ACE inhibitors should be avoided
    • Consider beta-blockers
Then consider surgery:
Surgical balloon treatment +stenting
  • 80-90% effective
  • Further balloon treatment as the child grows can help dilate the stent

Surgical repair

  • Less common since advent of balloon treatment
  • 5% mortality in symptomatic children
  • 1% mortality in asymptomatic children
  • Only 65-90% effective
  • Rarely can cause paraplegia due to aortic clamping during the surgery

All patients need prophylaxis for infective endocarditis before surgery, e.g.:

  • Amoxicillin – PO 1 hr before surgery – 50mg/Kg, or, if allergic to penicillin:
  • Clindamycin – 20mg / Kg

References

  • Murtagh’s General Practice. 6th Ed. (2015) John Murtagh, Jill Rosenblatt
  • Oxford Handbook of General Practice. 3rd Ed. (2010) Simon, C., Everitt, H., van Drop, F.
  • Beers, MH., Porter RS., Jones, TV., Kaplan JL., Berkwits, M. The Merck Manual of Diagnosis and Therapy

Read more about our sources

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Dr Tom Leach

Dr Tom Leach MBChB DCH EMCert(ACEM) FRACGP currently works as a GP and an Emergency Department CMO in Australia. He is also a Clinical Associate Lecturer at the Australian National University. After graduating from his medical degree at the University of Manchester in 2011, Tom completed his Foundation Training at Bolton Royal Hospital, before moving to Australia in 2013. He started almostadoctor whilst a third year medical student in 2009. Read full bio

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