Increase rate to 10nanograms/kg/min

You increase the infusion rate to 10ng/kg/min as part of the management plan suggested by the cardiology team. However, Bella’s hypoxia continues to worsen and the consultant is called by the senior nursing staff. The consultant arrives shortly afterwards to review baby. Together you check that baby is being ventilated effectively, and that drugs have been prescribed correctly and have been administered.

The consultant picks up that prostacyclin was prescribed on the drug chart, and not prostaglandin. Prostacyclin (also known as Epoprostenol or Flolan) is used as a pulmonary vasodilator, NOT to maintain ductal patency. The infusion is stopped and the baby is started on prostaglandin infusion. Within 5 minutes the oxygen saturations improve to above 70%.

The consultant comments that there has been a prolonged period of critical hypoxaemia, as a result of the delay in starting prostaglandin infusion.

Bella is transferred to the cardiac referral centre, where she is a diagnosed with Fallot’s tetralogy with critical pulmonary stenosis. She undergoes an emergency balloon valvotomy, before having a modified Blalock-Taussig shunt.

Bella is reviewed in the local developmental clinic at 12 months of age.

She has severely delayed gross motor milestones. Her mother had been told that this may have been due to brain damage as a result of low oxygen levels. However, she is well from a cardiac perspective.

Outcome: severe learning difficulties