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mangement of Tet spells


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mangement of cynotic spells in tof

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mangement of Tet spells

  2. 2. Tetrollogy of fallot
  3. 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. 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. 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. 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. 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
  8. 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 (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.
  9. 9.  ECG  Echocardiography  Radiography
  10. 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. 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. 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  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
  13. 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.