What is Cyanotic Spells ?

Hypercyanotic or Cyanotic spell is a pediatric emergency, which requires prompt recognition, and intervention to prevent disabling cerebro-vascular insults and to save lives. A cyanotic spell needs to be taken seriously not just because of the immediate threat but also because it indicates the need for early operation.

How to recognize a spell?

Commonly seen below two years peaks between 2 months to 6 months. Onset is usually spointaneous and unpredictable. Occurs more often in early morning, although can occur at anytime in the day. Infant cries incessantly, is irritable and often inconsolable. Tachypnea is prominent and a cardinal feature. Typically these infants have a pattern of deep and rapid breathing without significant subcostal recession. Cyanosis deepens as the spell progresses. Later gasping respiration and apnea ensues, which leads to limpness and ultimately anoxic seizures. Can last from minutes to hours. Auscultation reveals softening or disappearance of pulmonary ejection murmur. Occasional patient can have profound bradycardia.

Cardiac lesions which produce spells
1.Tetralogy of fallot.
2 TOF with Pulmonary atresia.
3Tricuspida atresia and PS.
4 DORV with VSD and PS.
5 D-TGA or L-TGA wit11 VSD and PS.
6 Single ventricle with PS.
7 Atrioventricular septa1 defect with PS.

Mechanisms of spells
Cyanotic spells are due to an acute decease in pulmonary blood flow, increased light to left shunt and systemic desaturation due to various causes Infundibuslar spasm due to increased circulating catecholamines as a result of effort of feeding or crying .Activation of mechanic-receptors in RV due to decrease in systemic venous return or that in LV due to decreases in pulmonary blood flow, leading to peripheral vasodilatation and fall in systemic vascular resistance producing increased right-left shunt and systemic desaturation. Same mechanism can account for occasional episodes of bradycardia vaso-inhibitory response. Supra venbicular tachycardia as a cause of spells in pulmonary atresia.

Management of spells
1 Check airway and start oxygen.
If child is uncomfortable with mask or nasal cannula, deliver oxygen via tube whose end is held $4 - 1 inch away from nose. This corresponds to delivering 80 per cent oxygen.
2 Knee-chest position.
3 Sedate child with subcutaneous morphine 0.2 mg/kg dose or IIM ketamile [3-5 mg/kg/dose].
4 Obtain a reliable intravenous access.
5 Soda-bicarbonate 1-2 m/kg given as 1:1 dilution or can be diluted in 10 ml/kg of isolate P which is given bolus as the initial resuscitating fluid.
6 Correct hypovolemia (10mVkg fluid bolus of isolyte P or dextrose normal saline).
7 Keep the child warm.
8 Correct anemia by packed cell transfusion. Hemoglobin level < 12 gddl merit correction through a blood transfusion in children with cyanotic spells.
9 Start beta-blockade Beta blockade is fairly safe unless a specific contraindication like bronchial asthma or ventricular dysfunction exists. It should always be given with heart rate monitoring.

Medications and dosages: 

IV metoprolol0.1. mg/kg, given slowly over 5 rnin.
Can repeat every 5-nlin for a maximum of 3 doses.
Can be followed by infusion 1-2 mcg/kg/min.
Monitor saturation, heart rates and BP.
Aim to keep heart rate below 100Imin.
Other options

I/V esmolol: 500mcg/kg over 1 min as loading dose, 50 mcg/kg/min for 4 minutes; if desideration persists without a significant decrease in heart rate the loading dose will need to be repeated and the infusion rate can be increased in
50 mcg/kg/min increments until 300mcglkgmin; this infusion should be maintained at the rate that produces the desired result. Esmolol is relatively expensive but has the advantage of being very need to acting.

I/V propranolol [O. 1 mgkg] :  If desideration persists and there is still no significant trend towards improvement despite maximum beta blockage.
1 Start vasopressin infusion.
Methoxamine gave I/V at dose of 0.lmg-0.2 mg/kg /dose or I/M (0.1- 0.4mgkgldose).
Phenylepherine: 5ugkg as bolus and than 1-4 ug/kg/min as infusion.
2 If spells are persistent, consider paralysing the child, elective intubation and ventilation and plan for surgery, which can be corrective or palliative [BT shunt].
3 If convulsions occur consider IV diazepam 0.2 mg/kg or IV midazolam 0.1-0.2 mg /kg /dose, as slow push.
Appropriate and timely management of cyanotic spells can save lives and prevent CNS insults.