Pediatric emergency medicine trisk 0505 0505
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is congenital heart disease. Although most newborns with cyanotic congenital
heart disease are recognized in utero or while in the newborn nursery, on
occasion, such a newborn will initially present to the emergency department (ED)
in the first few days or weeks of life with cyanosis. One condition particularly
prone to such late presentation is tetralogy of Fallot with pulmonary atresia.
When the ductus closes, profound cyanosis ensues. Rarely, an infant with mild
tetralogy of Fallot (or “pink tet”) may present with intermittent cyanosis during a
“tet” or “hypercyanotic” spell. These self-limited episodes are caused by
increased right-to-left shunting and decrease in pulmonary blood flow. The causes
of cyanotic congenital heart disease are listed in Table 21.1 (II,A) .
TABLE 21.3
LIFE-THREATENING CAUSES OF CYANOSIS
I. Respiratory
A. Decreased inspired O2 concentration
B. Upper airway obstruction/disruption
C. Chest wall immobility
D. Tension pneumothorax
E. Massive hemothorax
F. Lung disease leading to hypoxemia
II. Vascular
A. Cardiac
1. Cyanotic congenital defects
2. Congestive heart failure
3. Cardiogenic shock
B. Pulmonary
1. Pulmonary edema
2. Primary pulmonary hypertension of the newborn
3. Pulmonary embolism
4. Pulmonary hemorrhage
C. Peripheral
1. Septic shock
III. Other
A. Neurologic conditions leading to hypoxemia
B. Severe methemoglobinemia
Cyanosis may also be caused by pulmonary congestion from cardiac failure or
left-to-right shunt lesions leading to increased pulmonary blood flow and
