PEM GUIDE - TETRALOGY OF FALLOT SPELL

COMPONENTS
1.
RV Outflow Tract obstruction
2.
VSD
3.
Right Ventricular Hypertrophy
4.
Overriding aorta
CLINICAL FINDINGS
Hypoxia in TOF is due to R-to-L shunting through a large VSD that equalizes right
and left ventricular pressures. Complete venous mixing in these patients usually
sustains O2 sat at around 80%. Degree of shunting depends on the fine balance
between pulmonary vascular resistance (PVR) / PS/ RVOT obstruction and systemic
vascular resistance (SVR). Any condition that increases the PVR / RVOT
obstruction, such as pneumonia, bronchiolitis or tet spell will result in increased
shunting R-to-L. On the other hand, any condition that decreases the SVR will also
result in increased shunting through the VSD dehydration, fever (tachycardia),
medications etc.

HYPERCYANOTIC SPELL
TET or hypercyanotic spell in TOF is a sudden exacerbation of the existing
cyanosis and is a true emergency. This is the manifestation of an increased right to
left shunt, increasing cyanosis. The complete pathophysiology is poorly understood.
It appears to be probably due to upregulation of the beta-receptors in the RVOT
which are much more numerous than in other parts of the myocardium in these
children. This typically develops in 2-3 month old infant with TOF, and pink TOF
patients may have it as well. It usually happens in the morning (when SVR is low)
upon awakening, after feeding, bowel movement or bathing, or during periods of
acute agitation (such as invasive procedures). Presenting signs are irritability,
persistent crying and worsening cyanosis (and for the pink TETs sudden onset
of cyanosis). On examination one would detect absence of the usual PS murmur
which is indicative of the dramatically decreased pulmonary blood flow. If left
untreated, TET spell may result in seizures from hypoxia, CVA or even death.

MANAGEMENT
1.
Calm the infant quite environment, low light, parents are best at this
2.
Knee-chest position increases SVR
3.
Oxygen - blow-by in non-threatening manner, doesnt do much, but if
tolerated use it. It may decrease hypoxemia sufficiently to prevent arterial
vasodilatation.
4.
Medications
a.
Morphine 0.1-0.2 mg/kg SQ, IM, IV calms the infants, decreases
PVR. Blunts hyperpneic drive, decreasing the circulating
catecholamines and infundibular tone.
b.
Propranolol 0.15-0.25 mg/kg/dose IM, or slow IV push relaxes
RVOT.
c.
NS bolus 10-20 cc/kg, increases the SVR, especially if child is
dehydrated. May increase right ventricular pre-load & may decrease
the hypertrophic right ventricular outflow obstruction.
d.
Na bicarbonate 1mEq/kg, decreases acidosis, hyperventilation
consider in protracted cyanotic spell acidosis may prolong the
hyperpneic cycle
e.
Ketamine 1 mg/kg, sedative and mild sympathomimetic increases
SVR
f.
Alpha-agonist Phenylephrine 0.1mg/kg bolus, followed by a drip 2-10
mcg/kg/min. This will increase the systemic vascular resistance &
increase arterial oxygen saturation. It will also help to reverse
intracardiac shunting.
5..
Surgery - Emergent modified Blalock-Taussig shunt palliative subclavian
artery to right PA shunt.
The listed interventions should be used in this order.
Most of the spells will abort with the first 2-3 interventions.
Avoid agitating the infant initially with procedures such as blood drawing, placing an
IV, etc. Try to keep the child with the parents, who can best calm and comfort him.
Frequent Tet spells are indication for BT shunt placement until infant is big enough
to undergo definitive surgery by 1 year of age.
After the initial assessment, contact the cardiology consult, since the patient may
require more intensive therapy and even emergent surgery.

CXR of a child with Tetralogy of Fallot (note the boot-shaped heart)

EKG of a pre-operative TET (note the right sided dominance seen by positive Twaves in leads V1 V3 and the axis noted by QRS direction in leads I and AVF)