Coarctation of the aorta- COLLAPSE/ SHOCK

Question 1

Presenting features and diagnosis of coarctation of the aorta

Answer 1

Presenting features

• acute cicrulatory collapse day 2 of life (when DA closes)
• sick baby
• heart failure
• absent femoral pulses
• severe metabolic acidosis

Diagnosis

• normal or ejection systolic murmur between shoulder blades
• CXR: normal or cardiomegaly from HF and shock. Rib notching from aortic-aortic collateral arteries in teenagers and adults
• ECG: V2- deep S wave and tall R wave upright T wave. V6- downward T wave

Question 2

Bicuspid aortic valve

What is it?

What common syndromes are associated?

Answer 2

• A normal aortic valve has three cusps, whereas a bicuspid valve has only two.
• 1-2% of the population have bicuspid aortic valves,
  
  • M:F 2:1
• Majority will cause no problems
• In later life, a bicuspid aortic valve may become calcified, which may lead to varying degrees of severity of aortic stenosis and aortic reguritation, which will manifest as murmurs
• Syndromes associated
  
  • left heart obstruction (hypoplastic left heart syndrome, aortic stenosis, coarctation of the aorta or interrupted aortic arch (>50% of patients with these lesions)
  • Williams syndrome (bicuspid aortic valve associated with supravalvular aortic stenosis occurs in 11.6% of cases)
  • Patent ductus arteriosus, also associated with hand anomalies
  • Erdheim cystic medial necrosis (familial aortic dissection)
  • Turner syndrome (bicuspid aortic valve occurs in 30% of patients)

Question 3

Surgical management of coarctation of the aorta

Answer 3

• Stent insertion- older children as stents don’t grow!
• Surgery performed soon after diagnosis
  
  • cont. IV prostaglandin (PGE-1), is used to open DA allowing blood flow to the body beyond the coarctation
  • IV meds to improve the contraction of the myocardium
  • Babies will almost always need to be placed on a ventilator before surgery
  • Symptomatic newborns with coarctation, surgical repair is usually done on an urgent basis following initial stabilisation
  • End-to-end anastomosis: resection of the narrowed area with anastomosis of the two ends to each other
  • arch advancement: taking the aorta after the area of narrowing and anastomosing it with the ascending aorta. indicated with more diffuse hypoplasia
  • Subclavian flap aortoplasty: the narrowing may be opened with a patch, or a portion of an artery may be used as a flap to expand the area