How do you manage Hypercyanotic spells?

Place infants with hypercyanotic spells in the knee-chest position and give oxygen; sometimes, opioids (morphine or fentanyl), volume expansion, sodium bicarbonate, beta-blockers (propranolol or esmolol), or phenylephrine may help. Repair surgically at 2 to 6 months or earlier if symptoms are severe.

What is a Hypercyanotic spell?

Hypercyanotic spells are sudden episodes of profound cyanosis and hypoxia that may be triggered by a fall in oxygen saturation (eg, during crying, defecating), decreased systemic vascular resistance (eg, during playing, kicking legs), or sudden tachycardia or hypovolemia.

Why is morphine spelled in Tet?

If these maneuvers are unsuccessful, the most common subsequent treatment is to administer morphine, which calms the patient, resolves the hyperpnea, nor- malizes the systemic venous return, and increases the partial pressure of oxygen in the blood.

How does knee-chest position help in TOF?

Bring the child’s knees up tight against his or her chest (this is called the knee-chest position) or have your child squat down. This will increase blood flow to the lungs.

Why does crying cause Tet spell?

Sometimes, babies who have tetralogy of Fallot will suddenly develop deep blue skin, nails and lips after crying or feeding, or when agitated. These episodes are called tet spells. Tet spells are caused by a rapid drop in the amount of oxygen in the blood.

What triggers Tet spell?

Tet spells are caused by a rapid drop in the amount of oxygen in the blood. Tet spells are most common in young infants, around 2 to 4 months old. Toddlers or older children might instinctively squat when they’re short of breath. Squatting increases blood flow to the lungs.

Why does squatting relieve tet spells?

Squatting is a compensatory mechanism, of diagnostic significance, and highly typical of infants with tetralogy of Fallot. Squatting increases peripheral vascular resistance (PVR) and thus decreases the magnitude of the right-to-left shunt across the ventricular septal defect (VSD).

Why is morphine given in TOF?

Morphine has been recommended primarily as a sedative for the treatment of TOF patients with hypercyanotic spells. However, morphine causes significant vasodilation in both venous and arterial beds, resulting in significant reduction of cardiac preload and systemic vascular resistance.

Are tet spells serious?

Some babies with tetralogy of Fallot have episodes called tet spells, when they suddenly turn bluish and may faint. These spells are serious. A tet spell may be caused by activities that change the pressure in your baby’s heart and increase the flow of oxygen-poor blood to their body.

What is cyanotic spell?

Cyanosis is a bluish tinge, mostly noticeable on the lips and nail beds. During a cyanotic spell, your baby’s lips and skin will appear more blue than usual and their breathing may be deeper and faster. Your child may initially be very irritable, then may become grey, floppy and unresponsive.

How does squatting help in TOF?

What to do if a hypercyanotic spell continues?

Spells will often settle with simple manoeuvres. If hypercyanotic spells continue: Give intranasal fentanyl 1 microgram/kg. If hypercyanotic spells continue: Give morphine sulphate 0.1 mg/kg – administer this subcutaneously unless IV access is already available (don’t delay).

How to treat hypercyanotic spells with morphine sulphate?

If hypercyanotic spells continue: Give morphine sulphate 0.1 mg/kg – administer this subcutaneously unless IV access is already available (don’t delay). Fentanyl and morphine suppresses the respiratory centre, thereby reducing the hyperapnoea and the resultant systemic venous overload which contributes to the right-to-left shunt.

What to do if you have a hypercyanotic crisis?

To correct any metabolic acidosis, if present. AVOID Epinephrine and isoproterenol – as these will decrease SVR! Van Roeken s CN, Zuckerberg AL. Emergency management of hypercyanotic crises in tetralogy of Fallot.