mangement of cynotic spells in tof

1. DR SHOAIB AHMED MAGSI

2. Tetrollogy of fallot

3. Clinical features
 SYMPTOMS
 Difficulty with feeding
 Failure to thrive
 Episodes of bluish pale skin during crying or feeding
(ie, "Tet" spells)
 Exertional dyspnea, usually worsening with age
 Squatting during excercise

4.  SIGNS
 Most infants are smaller than expected for age
 Cyanosis
 Clubbing
 A systolic thrill is usually present anteriorly along the left sternal
border
 A harsh systolic ejection murmur (SEM) is heard over the
pulmonic area and left sternal border
 During cyanotic episodes, murmurs may disappear

5. Cyanotic (Tet) spells
 Acute hypoxemic attacks represent a true emergency
 Usually, the underlying diagnosis is tetralogy of Fallot.
 In a Tet spell,
 increase in obstruction to pulmonary blood flow (either in heart or in
pulmonary circulation)
 if systemic perfusion is reduced, as with hypovolemia or the development of
a tachyarrhythmia

6. Clinical presentation and diagnosis
 They are characterised by:
Period of uncontrollable crying / panic,
Rapid and deep breathing (hyperpnoea),
Deepening of cyanosis,
Decreased intensity of heart murmur,
Limpness, convulsions and rarely, death.
 .

7. Prespitation
 common in the early morning
 Prolonged agitation and crying
 Noxious stimuli
 Exercise, bathing, or fever
 In such cases(tet spells), the absence of a heart
murmur is a worrisome indicator that pulmonary
blood flow is severely compromised

9. Workup
 Hemoglobin and hematocrit values are usually elevated in proportion to
the degree of cyanosis. Prolonged cyanosis causes reactive polycythemia
that increases the oxygen-carrying capacity. While in cyanosis due to
Anemia hb is 3-5g/dl
 ABG
 results show varying oxygen saturation, but pH and partial pressure of
carbon dioxide (pCO2) are normal, unless the patient is in extremis, such
as during a tet spell.
 Oximetry is particularly useful in a dark-skinned patient or an anemic
patient whose level of cyanosis is not apparent. Generally, cyanosis is not
evident until 3-5 g/dL of reduced hemoglobin is present.

10.  ECG
 Echocardiography
 Radiography

11. Emergency management
 Management is directed at manipulating the relative resistances of the
systemic and pulmonary
 vascular beds, as well as maintenance of appropriate circulating volume
and heart rate
 1. Knee-to-chest / Squatting:

12.  2. Oxygen (100%) can be administered which also increases systemic
resistance and may help enhance oxygen delivery
 but usually has minimal effect.
 3. Morphine: 0.1-0.2 mg/kg IM. (Caution in infants under 3 months).
 morphine may cause pulmonary vasodilatation and decreases vantilatory drive

13.  If the above procedures are ineffective or have suboptimal effect, the following
treatments may need to be given.

 4. Crystalloid or colloid fluid bolus: 10-20ml/kg by rapid IV push.
 give an IV fluid bolus of 20 mL/kg normal saline
 Sodium bicarbonate, 1–2 mEq/kg slowly IV
 5. . phenylephrine
 If cyanosis persists, give phenylephrine (10 mcg/kg by slow IV push)
 to pharmacologically increase the systemic vascular resistance

14.  5. beta blocker (e.g. propranolol or esmolol)
 In severe episodes, propranolol (Inderal) may be given
 Esmolol 500 mcg/kg over one minute IV, then maintenance of 50
mcg/kg/min can be increased in steps of 50mcg/kg/min to maximum dose of
300mcg/kg/min
 beta blocker, reduces dynamic muscular stenosis of the right ventricular
outflow tract and increasing pulmonary blood flow
 . Progressive hypoxemia and the occurrence of cyanotic spells are
indications for early surgery.