Hypoxic spells Medical Presentation

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Slide 1-

hypoxic spells Mohamed Abdullahi Sh. Mohamed
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What is Tet spell spells Tet spell (hypoxic spell/cyanotic spell) 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).
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Cont.. 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. Tet spell: A term for an episode of severe hypoxia and cyanosis in a child with tetralogy of Fallot.
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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.
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Pathophysiology of Tet spell
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Triggers The possible triggers are: anxiety fever anemia sepsis or even spontaneously without any cause. Prolonged agitation and crying noxious stimuli …. 
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Clinical presentation and diagnosis They are characterized by: Period of uncontrollable crying Rapid and deep breathing (hyperpnoea) Deepening of cyanosis Decreased intensity of heart murmur Limpness, convulsions and rarely, death.
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Workup Hemoglobin and hematocrit values are usually elevated in proportion to the degree… cyanosis due to Anemia hb is 3-5g/dl. ABG Oximetry: particularly useful in a dark-skinned patient or an anemic patient whose level of cyanosis is not apparent
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Echo: gold stander Radiography Workup
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Management Knee-to-chest / Squatting: This calms the infant, reduces systemic venous return and increases systemic vascular resistance Emergency management
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Cont… 2). Oxygen (100%)…. 3). Morphine: 0.1-0.2 mg/kg IM. 4. Crystalloid or colloid fluid bolus: 10-20ml/kg by rapid IV push. 5. . Phenylephrine….. 5. beta blocker (e.g. propranolol or esmolol)
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For chronic cyanotic congenital heart conditions  supportive treatment is all that can be done until a surgical or catheter-directed intervention can be accomplished.  Give supplemental oxygen, even though dramatic changes in saturation will not occur with oxygen alone.  Secure IV access and give fluid to maintain an adequate circulating volume.
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 Treat systemic acidosis once adequate ventilation is ensured.  Most of all, immediately consult with a cardiologist to arrange for more definitive treatment and to prevent unnecessary interventions.
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Differential Diagnoses  Aortic stenosis  Acute Respiratory Distress Syndrome  Apnea  Pediatric Patent Ductus Arteriosus Surgery  Pneumonia  Pneumothorax  Pulmonic Valvular Stenosis  Sickle Cell Anemia
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The END THANK YOU.
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Choice ONE answer Therapy of a "blue" or "tet" spell could include all of the following Except: A. Epinephrine B. Knee-chest position C. Oxygen D. Morphine E. Sodium bicarbonate
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Choice ONE answer Cyanosis is produced by the presence of deoxygenated hemoglobin of at least: a. 1-2 gm/dL b. 3-5 gm/dL c. 6-8 gm/dL d. 9-10 gm/Dl
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Choice ONE answer An infant with a marked cyanotic congenital heart defect with decreased pulmonary vascularity should be treated with: a. Digoxin b. Indomethacin c. Prostaglandin E1 d. Epinephrine
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Choice ONE answer A two day old cyanotic infant with a grade 3/6 ejection systolic murmur is noted to have decreased pulmonary vascular markings on chest x-ray and left axis deviation on EKG. The most likely diagnosis is: a. Tetralogy of Fallot b. Transposition of Great Vessels c. Truncus Arteriosus d. Tricuspid Atresia.
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