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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.

• ave the parent hold the infant over the parent!s 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$

o IV sodium bicarbonate is necessary to correct metabolic acidosis. The

dosage is %"& meq'kg as %"& ml'kg of (.)* +aC- slow / bolus. 0or infants less

than - months of age, administer as ).&* +aC- #dilute the (.)* +aC- %1% with

normal saline$. 2nsure the intravenous access is secure before administration as

extravasation can lead to severe tissue in3ury.

o   -adrenergic blockade with intravenous propranolol #4.%5 " 4.&5

mg'kg given slowly over 5 " %4 minutes6 dose can be repeated once$. n the acute

attack, propranolol slows the heart rate and reduces the right ventricular outflow

obstruction6 it also has a sedative effect. IV Esmolol  #4.5 mg'kg over % minute and can

be given as an infusion at 54 mcg'kg'min$ is an alternative.

o   -agonists: phenylephrine #4.% mg'kg 7C or 8, 4.4% mg'kg /, or as an

infusion 4.% " 4.5 mcg'kg'min$ or metaraminol (Aramine®)#4.4% mg'kg / and repeated

9:+, can be given as an infusion 4.%"%.4 mcg'kg'min$, increases systemic vascular

resistance #7/:$ and reduces right to left shunting.

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o etamine #%"& mg'kg / or 5"%4 mg'kg 8$ is a drug, which

simultaneously increases the 7/:, sedates the patient. ;oth effects are known to

terminate the spell.

o !orphine #4.% mg'kg / or 7C$. 8echanism of action is via suppression of the respiratory centre and abolishing hyperpnoea. owever, its disadvantages include

slow onset and respiratory depression. ;e ready to intubate.

• Ventilatory support if necessary.

• <eneral anaesthesia or emergency ;lalock"Taussig shunt in intractable cases.

Maintenance therapy

• 9ropranolol and sedation as required.

• 9ropranolol acts by its peripheral actions of stabili=ing the reactivity of the

systemic arteries, thereby preventing a sudden decrease in the systemic vascular

resistance. ral dose is 4.&"4.5 mg'kg'dose >"%& hourly, and can be slowly increased to

maximum of % mg'kg'dose > hourly as needed.

• 7edation may be with chloral hydrate or dia=epam. owever, avoid mida=olam,

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@.