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Article No. 119


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Ductal-dependent congenital heart defects

Ductal-dependent congenital heart defects

Table of Contents

Ductal-dependent lesions (i.e. requiring the ductus arteriosus for adequate circulation) usually present in the neonate, around 1 week to 1 month old (when the ductus arteriosus closes, thus unmasking the lesion)

2 types:

  • Left-sided/Ductal-dependent pulmonary circulation: presents with cyanosis that is not really improved with oxygen because blood can’t get from the left heart to the body
    • Coarctation of aorta/critical aortic stenosis/interrupted aortic arch
    • Hypoplastic left heart
  • Right-sided/Ductal-dependent systemic circulation: presents with shock/acidosis that is often initially confused for sepsis but doesn’t really improve (or gets worse) with fluid because blood can’t get from the right heart to the lungs
    • Tetralogy of Fallot
    • Tricuspid atresia
    • Pulmonary atresia/pulmonic stenosis
    • Severe Ebstein’s anomaly
  • Treatment in acute setting
    • Empiric prostaglandin E1 drip at up to 0.1 mcg/kg/min (which can be given peripherally) if there is suspicion for this, even without confirmatory diagnosis
      • Strong vasodilating effects which act on the smooth muscle of the ductus arteriosus
      • Need close airway monitoring due to side effect of apnea
    • Also treat empirically for sepsis with antibiotics, glucose
    • Be careful with too much supplemental O2 which can make the ductus close more quickly; Also be careful with giving too much fluid
    • Ultimately, the patient needs an echo and a pediatric cardiac surgeon emergently

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