Congenital Heart Defects (acyanotic)

Coarctation of aorta


  • Narrowing of aorta post arch

  • MC in ♂ but ↑risk in ♀ with Turner syndrome

  • Also seen with other defects like bicuspid AV, VSD, PDA

  • Narrowing ➔ ↑ pressure proximal to coarctation ➔ ↑ flow to upper ext and head

    • ↓ pressure distal to coarctation ➔ ↓ flow to lower ext and organs

  • Shunting ➔ cyanosis in lower extremities (new borns)

    • As ductus arteriosus closes ➔ post-stenotic end-organ failure in newborns

  • Adults will have HTN in upper extremities/head and hypotension in lower extremities/organs


  • Cyanosis in lower extremities of new borns

    • Once ductus closes ➔ HF sx at ~1-2 weeks old

  • HTN in upper extremities and Hypotension in lower extremities

    • Brachial/radial-femoral pulse delay

    • Head HTN ➔ tinnitus, epistaxis, headache

    • Prolonged cap refill and claudication in lower extremities that ↑ with exercise

    • Unequal BP (LUE vs RUE) if left subclavian artery is distal to coarctation

  • Murmurs from other cardiac abn (PDA, AS, VSD) or systolic ejection murmur radiating to posterior


  • BP measurement ➔ brachial-femoral delay

  • CXR shows notching of ribs (3&4) and figure 3 sign of aorta

  • ECHO confirms coarctation, location/degree of stenosis and other defects

  • Angiography best to assess severity and location, also evaluates collateral vessels

    • Definitely for adults to evaluate for aneurysms

Rib notching

  • Above coarctation with ↑ pressure

    • Anterior intercostal arteries supply anterior ribs 1-12 and posterior intercostal arteries 1-2

  • Below coarctation with ↓ pressure

    • Posterior intercostal arteries coming directly off aorta to supply posterior ribs 3-12

  • Reverse flow in posterior intercostal arteries (anterior ➔ posterior) ➔ posterior intercostal artery dilation

  • Pulsation of dilated arteries ➔ rib notching (mainly at ribs 3 & 4)

    • can be at ribs 3-9, never at ribs 1-2


  • PG infusion in newborns until surgical repair (resection with end-to-end anastomosis) 

  • Balloon angioplasty (children > 4mo and < 25kg)

  • Adults get balloon and/or stent