mangement of Tet spells

mangement of cynotic spells in tof

1. Cyanotic ( Tet ) spells 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 N oxious stimuli E xercise , 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

8. 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 (pCO 2 ) 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.

9. ECG Echocardiography Radiography

10. 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 :

11. 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

12. 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 S odium 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

13. 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 .