Acute Management

A hypercyanotic attack is a medical emergency and requires prompt

management to break the hypoxic cycle.

Call for help early and inform the cardiologist in charge.

Depending on the severity of attack, institute one or more of the following.

Try to calm the infant.

Have the parent hold the infant over the parents shoulder, or place the child in a
knee-chest position.

Administration of oxygen (although this will not reverse cyanosis due to

intracardiac shunting). Avoid if such attempts further aggravate the child.

Drugs (in order or preference, unless contraindicated)

IV sodium bicarbonate is necessary to correct metabolic acidosis. The
dosage is 1-2 meq/kg as 1-2 ml/kg of 8.4% NaHCO3 slow IV bolus. For infants less
than 3 months of age, administer as 4.2% NaHCO3 (dilute the 8.4% NaHCO3 1:1 with
normal saline). Ensure the intravenous access is secure before administration as
extravasation can lead to severe tissue injury.

-adrenergic blockade with intravenous propranolol (0.15 - 0.25

mg/kg given slowly over 5 - 10 minutes; dose can be repeated once). In the acute
attack, propranolol slows the heart rate and reduces the right ventricular outflow
obstruction; it also has a sedative effect. IV Esmolol (0.5 mg/kg over 1 minute and can
be given as an infusion at 50 mcg/kg/min) is an alternative.
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-agonists: phenylephrine (0.1 mg/kg SC or IM, 0.01 mg/kg IV, or as an

infusion 0.1 - 0.5 mcg/kg/min) or metaraminol (Aramine)(0.01 mg/kg IV and repeated

PRN, can be given as an infusion 0.1-1.0 mcg/kg/min), increases systemic vascular
resistance (SVR) and reduces right to left shunting.

Ketamine (1-2 mg/kg IV or 5-10 mg/kg IM) is a drug, which

simultaneously increases the SVR, sedates the patient. Both effects are known to
terminate the spell.

Morphine (0.1 mg/kg IV or SC). Mechanism of action is via suppression of

the respiratory centre and abolishing hyperpnoea. However, its disadvantages include
slow onset and respiratory depression. Be ready to intubate.

Ventilatory support if necessary.

General anaesthesia or emergency Blalock-Taussig shunt in intractable cases.

Maintenance therapy

Propranolol and sedation as required.

Propranolol acts by its peripheral actions of stabilizing the reactivity of the

systemic arteries, thereby preventing a sudden decrease in the systemic vascular
resistance. Oral dose is 0.2-0.5 mg/kg/dose 6-12 hourly, and can be slowly increased to
maximum of 1 mg/kg/dose 6 hourly as needed.

Sedation may be with chloral hydrate or diazepam. However, avoid midazolam,

which reduces systemic vascular resistance further.

Treat fever aggressively and ensure adequate hydration.

Avoid epinephrine, dopamine, dobutamine, digitalis, and digoxin, which have

positive inotropic effects and may therefore, worsen Tet spells.