Review Article

HYPERCYANOTIC SPELLS

A TAKSANDE*, V. GAUTAMI**, S PADHI***, K BAKSHI****

Introduction progressive cyanosis. The resulting fall in arterial

The 'Tet spell' (also called 'hypoxic spell',
'cyanotic spell', 'hypercyanotic spell', 'paroxysmal
dyspnea') is an episodic central cyanosis due to
total occlusion of right ventricle outflow in a
patient with a congenital heart disease, such as
Tetralogy of Fallot (TOF). Hypercyanotic spell
characterized by paroxysm of hyperpnea (rapid
and deep respirations),irritability and prolonged
cry, increased cyanosis and decreased intensity
of heart murmur. If not treated in time it may
lead to limpness, seizures, neurological deficit
and death. A spell is most likely to be seen a child
less than 2 year old, upon waking up in the
morning and following a crying episode(1). Weng
YM et al reported cyanotic spell in a 29-year-old
man of TOF also(2).
PO2 in addition to an increase in PCO2 and fall
in pHà stimulate the respiratory centre à Increased
rate and depth of breathing i.e. hyperpnoea à
increase in the systemic venous return to the
RVàIn the presence of fixed resistance at the
right ventricular outflow tract or decreased SVR,
the increased systemic venous return to the RV
must go out the aorta à Further decrease in the
arterial oxygen saturation à Right to left shunt
of deoxygenated blood, thus leading to a vicious
cycle of hypoxic spells (fig 1).

Pathophysiology
In TOF, The level of cyanosis and onset
of cyanotic spell is determined the SVR & level
of PS component. In case of mild PS, the RV
pressures are usually less than the left ventricle
and hence the shunt is usually left to right. If
Figure 1. Mechanism of Hypoxic Spell
Severe PS then the RV after load becomes high
and hence the RV pressures become high. If the Five mechanisms are involved in the
SVR is low (TOF with cyanotic spells) then the pathogenesis of fallot spells 1) An acceleration
shunt flow becomes right to left. This results in in heart rate 2) An increase in cardiac output and
*M.D., (Fellow) **M.D.,D.M, Head and Consultant venous return 3) An increase in right to left shunt
***M.D., D.M,(Consultant),****M.D., FNB,(Consultant) 4) Vulnerable respiratory control centers and 5)
Address For Correspondence : Dr. Amar Taksande, Infundibular contraction. Manual compression
Innova Children Heart Hospital And Research
Centre, White House, Tarnaka, Hyderabad, A.P. 500017 of abdominal aorta can abort spell by decreasing
E mail : amar_bharti2000@yahoo.co.uk cardiac output and decreasing cardiac output(3-4).

J MGIMS, September 2009, Vol 14, No (ii), 7 - 9

Hypercyanotic Spells
Certain theories have also been postulated
as possible explanation for the cause of cyanotic
spells. These theories have basically compared
the onset of cyanotic spells to exercise in normal
individuals where there is fall in systemic arterial
oxygen saturation during exercise and which
reverses once exercise stops. In cyanotic spells
this process of low systemic oxygen saturation
continues and ultimately leads to progressive
metabolic acidosis. These theories are : Woods
et al(5) reported that hypoxemic spells are caused
by spasm of the infundibulum of the RV, à
progressively increasing right to left shunting
and metabolic acidosis. Surge in Catecholamine
release leads to increased myocardial contractility
and infundibular stenosis. Guntheroth et al(6)
reported that episodes of paroxysmal hyperpnea
are the cause rather than the effect of cyanotic
spells. Hyperpnea increases the systemic venous
return leading to right to left shunt as well as
oxygen consumption through increase work of
breathing. Kothari SS(7) argued against the views
mentioned previously and suggested the role of
stimulation of mechanoreceptors in the RV to
be the cause of spells. Increased contractility
(due to catecholamine) and decreased right
ventricular size (due to various factors) can trigger
a reflex resulting in hyperventilation, some
peripheral vasodilation without bradycardia, and
this may initiate a spell.
Figure 2. Various postures assumed for relief of dyspnea in TOF 1) squatting, 2) sitting with legs drawn underneath
(squatting equivalent), 3) legs crossed while standing, 4) infant held with legs flexed on its abdomen, and 5) lying down.
Clinical Manifestation
Typically cyanotic spells occur early in the
morning. The possible triggers are anxiety, fever,
anemia, sepsis or even spontaneously without any
cause. The spell are typically initiated by the stress
of feeding, crying or bowel movement, particularly
after an infant awakens from a long deep sleep.
A typical infant with cyanotic spell would appear
fussy, inconsolable and thereafter progresses to
increasing cyanosis, hyperpnea that is typical of
a spell. The older child experiencing a hypoxic
spell will often squat to recover. Squatting
compresses the superior vena cava and increases
systemic vascular resistance, directing blood
through the pulmonary stenosis and into the
lungs8.
Cardiac causes other than TOF that may
present with cyanotic spells are tricuspid atresia
with pulmonary stenosis (PS), Transposition of
great vessels with PS, Single ventricle physiology
with PS or pulmonary atresia.
Management
Squatting' / 'Knee-to-chest' : Placing the child
in the knee-chest position either lying supine or
over the parent's shoulder. This calms the infant,
reduces systemic venous return and increases
systemic vascular resistance. Various postures (1)
assumed for relief of dyspnoea (fig2).
J MGIMS, September 2009, Vol 14, No (ii), 7 - 9

There is very limited benefit to administer
oxygen, since the problem is reduced pulmonary
blood flow, not the ability to deliver oxygen to
the lungs. Administer morphine sulfate 0.1 mg/
kg IV or IM. It depresses respiratory center and
lead to decreases in systemic venous return.
Correct acidosis : Obtain pH, give Inj. Soda
bicarbonate(1-2 meq/kg IV). It reduces the
respiratory stimulation by metabolic acidosis,
and may diminish the increase in pulmonary
vascular resistance caused by hypoxia and acidosis.
Propranolol, 0.1 mg/kg slow IV push. May be
repeated in 15 minutes. By decreasing cardiac
contractility, propranolol may decrease
infundibular obstruction of right ventricular
outflow. Given orally at 2-4 mg/kg/day PO to
prevent spells. When used chronically, have the
beneficial effect of stabilizing peripheral vascular
reactivity. or Inj Esmolol(0.5mg/kg over 1 min
then 50mcg/kg/min over 4 min. or Inj
Metoprolol- 0.1mg/kg over 5 min, repeat every 5
min to max 3 doses , then start infusion 1-5 mcg/
kg/min. Phenylephrine 5-20 mcg/kg IV every
10-15 minutes. Increases the SVR, forcing more
blood flow to the lungs. Continuous phenylephrine
infusion to maintain adequate pulmonary
blood flow to keep oxygen saturations in the 90.
A phenylephrine drip may be run at 0.1-0.5 mcg/
kg/min, titrated to desired effect. It is a potent
vasoconstrictor that will result in reduced renal
and mesenteric perfusion as well. Ketamine- 0.25
- 1.0 mg/kg. IV or IM? has dual benefit causes
sedation and increase SVR. Methoxamine - 0.10mg/
kg IV over 5-10 min.--> Leads to increase SVR.
Intravenous fluids - preferably initially as bolus
of 10-20cc/kg à 60cc/kg. Crystalloid or colloid
fluid bolus: This maximises preload and should
be given prior to the following drugs which may
A Taksande, et al
induce hypotension. Correct anemia and con-
sider operation9.
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