2. A 7 month old girl presents to the outpatient clinic because
of recurrent episodes of perioral and extremity cyanosis
over the preceding 2 days. Each episode lasts 2 to 3 hours,
occurs two or three times per day, and seems most
prominent on the hands and feet. There has been no
cyanosis of the chest or abdomen. Her skin colour varies
from blue to a sunburned pink. The girl has been afebrile,
with no cough, congestion, wheezing or symptoms of any
illnesses. She has been formula-fed for the preceding 5
weeks, drinking 6-8 Oz four to five times per day.
3. On physical examination, the infant is afebrile, has normal
vital signs. There is no respiratory distress and her lungs
are clear to auscultation. She has a regular cardiac rhythm
with no murmurs. Abdominal and neurological examinations
yield normal results. Diagnostics tests, including a complete
blood cell count, complete metabolic panel, chest
radiograph and echocardiography was done. Further
questioning and a laboratory test reveal the diagnosis.
4. CYANOSIS
DEFINITION:
Cyanosis specifically refers to a bluish tone visible in the mucous
membranes and skin when desaturated or abnormal hemoglobin is
present in the peripheral circulation.
Central cyanosis occurs when poorly oxygenated blood enters the
systemic circulation where there is “right-to-left” shunt and it may
occur within the heart or in the pulmonary circulation itself.
When there is primary parenchymal lung disease or neurologic
disease causing alveolar hypoventilation, an “intrapulmonary” right-
to-left shunt can occur.
5. CAUSES OF CYANOSIS
Abnormal hemoglobins may be fully saturated with oxygen, yet
unable to release it to the tissues like Methemoglobinemia.
Anemia when Hb= 3-5gm/dl cyanosis will be visible.
Typical cyanotic lesions are the “five Ts” of congenital heart
disease:
tetralogy of Fallot,
transposition of the great vessels,
total anomalous pulmonary venous return,
tricuspid atresia, and
truncus arteriosus but others may also be present.
6. Pulmonary diseases like :
upper airway obstructive problems (croup, epiglottitis)
lower airway diseases (bronchiolitis, asthma, cystic
fibrosis, pneumonia with lobar consolidation).
Foreign body
7. CYANOTIC SPELL
DEFINITION : Acute hypoxemic attack which represents a true
emergency and initial treatment is crucial to long term outcome.
Also called as Hyperpnoeic spell, Hypoxic spell, Anoxic or blue
spell, Hypercyanotic spell or Tet spell.
Usually occurs in cyanotic congenital heart diseases with
reduced pulmonary blood flow.
A pediatric emergency- a typical episode can lead to death.
Peak incidence between the age group of 2-6 months.
Episodes beyond the age of 2 years are rare.
8. About 40% of pts. with cyanotic congenital heart disease &
decreased blood flow develop this spell.
Usually, the underlying diagnosis is tetralogy of Fallot.
A typical episode begins with a progressive increase in rate &
depth of respiration, resulting in paroxysmal hyperpnoea,
deepening cyanosis, limpness & syncope, convulsions, CVA &
even death.
The spells are usually self-limited and last for about <15-30
mins duration.
9. CYANOTIC SPELLS are classically found in :-
Tetralogy of Fallot
Double Outlet Right Ventricle (DORV) with VSD with PS
Tricuspid Atresia
Single Ventricle with Pulmonary Stenosis
Transposition of great arteries
Eisenmenger’s Syndrome
10. Precipitating Factor
Common in the early morning, shortly after the patient
awakens or following any exertion.
Prolonged agitation and crying are also cited as precipitants.
Also, noxious stimuli such as phlebotomy or a bee sting or any
circumstance which leads to enhanced catecholamine output
can precipitate a spell in a susceptible child.
A decrease in systemic vascular resistance (SVR) during
exercise, bathing or fever, dehydration, tachypnoea,
tachycardia due to any cause potentiates a right-to-left shunt
and precipitates hypoxemia.
In such cases (tet spells), the absence of a heart murmur is a
worrisome indicator that pulmonary blood flow is severely
compromised.