Cyanotic Spell

dr. Shirley L A, Sp. A

12 April 2011

Cyanotic spell
= hypoxic spell, tet spell or hypercyanotic spell
occurs in young infants with TOF
It consists of hyperpnea, worsening cyanosis, &

disappearance of the heart murmur

Any event such as crying, defecation, or increased

physical activity that suddenly lowers the SVR or

produces a large right-to-left ventricular shunt may
initiate the spell and, if not corrected, establishes a
vicious circle of hypoxic spells

Fall in arterial Po2, increase in

Pco2and a fall in pH, stimulates the

respiratory center and produces
hyperpnea
The hyperpnea, in turn, makes the

negative thoracic pump more

efficient and results in an increase in
the systemic venous return to the RV
In the presence of fixed resistance at

the RVOT (i.e., pulmonary resistance)

or decreased SVR, the increased
systemic venous return to the RV
must go out the aorta
This leads to a further decrease in

the arterial oxygen saturation, which

establishes a vicious circle of
hypoxic spells

Treatment of hypoxic spells

1.knee-chest position traps systemic venous

blood in the legs temporarily systemic venous

return & helping to calm the baby
The knee-chest position may also SVR by
reducing arterial blood flow to the lower
extremities

2.Morphine sulfate suppresses the respiratory

center & abolishes hyperpnea

7.Propranolol has been used successfully in some cases of

hypoxic spell, both acute and chronic
Mechanism of action ?
- may slow HR and perhaps reduce the spasm of the RVOT
- may increase SVR by antagonizing the vasodilating
effects of
-adrenergic stimulation
The successful use of propranolol in the prevention of
hypoxic spell is more likely the result of the drug's
peripheral action
The drug may stabilize vascular reactivity of the systemic
arteries, thereby preventing a sudden decrease in SVR.

Squatting
What is the mechanism of recovery from these symptoms

during squatting?
same as the knee-chest position
3 mechanisms may be involved :

1. reduction of the systemic venous return by trapping

venous blood in the lower extremities reduces RL
shunt at the ventricular level
2. reduced arterial blood flow to the legs reduces venous
washout from leg muscles
3. may also increase SVR, a known mechanism to reduce
R L ventricular shunt