RV Outflow Tract obstruction
Right Ventricular Hypertrophy
Overriding aorta
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.

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

who can best calm and comfort him. This will increase the systemic vascular resistance & increase arterial oxygen saturation. c. b. Morphine – 0. followed by a drip 2-10 mcg/kg/min.blow-by in non-threatening manner. parents are best at this 2. decreases acidosis.. The listed interventions should be used in this order. NS bolus – 10-20 cc/kg. decreasing the circulating catecholamines and infundibular tone. increases the SVR. placing an IV. 5.15-0.25 mg/kg/dose IM. Propranolol – 0. low light. Alpha-agonist – Phenylephrine 0. IM. 4. or slow IV push – relaxes RVOT. Surgery . Most of the spells will abort with the first 2-3 interventions. d. IV – calms the infants. etc. Oxygen . since the patient may require more intensive therapy and even emergent surgery. Na bicarbonate – 1mEq/kg. Frequent Tet spells are indication for BT shunt placement until infant is big enough to undergo definitive surgery by 1 year of age. May increase right ventricular pre-load & may decrease the hypertrophic right ventricular outflow obstruction. Calm the infant – quite environment. hyperventilation – consider in protracted cyanotic spell – acidosis may prolong the hyperpneic cycle e.1-0. . decreases PVR. It may decrease hypoxemia sufficiently to prevent arterial vasodilatation. contact the cardiology consult.MANAGEMENT 1.Emergent modified Blalock-Taussig shunt – palliative subclavian artery to right PA shunt.2 mg/kg SQ. Medications a. sedative and mild sympathomimetic – increases SVR f. Avoid agitating the infant initially with procedures such as blood drawing. Try to keep the child with the parents.1mg/kg bolus. Knee-chest position – increases SVR 3. but if tolerated use it. Ketamine – 1 mg/kg. After the initial assessment. Blunts hyperpneic drive. especially if child is dehydrated. It will also help to reverse intracardiac shunting. doesn’t do much.

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) .