Pediatric emergency medicine trisk 1715 1715
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lesion involving RVOTO, experiences an event in which the muscular
infundibular portion of the RVOT becomes diminishingly small, preventing blood
flow to the lungs. All blood is then shunted right-to-left across the nonrestrictive
VSD. The pulmonary stenosis murmur disappears. Extreme cyanosis ensues
which can ultimately lead to death if severe.
Treatment of a hypercyanotic event follows a stepwise progression. Initially,
bring the knees to the chest, allowing the parent to comfort the patient in this
position. Monitor closely. Knee to chest position in infants, or squatting in older
children, increases SVR and decreases right-to-left shunting. Administer 100%
oxygen. If the spell persists, morphine (0.1 mg/kg IM or IV) can be used to calm
agitation. Normal saline bolus (10 mL/kg) ensures adequate preload and may be
repeated if the patient is dehydrated. If these steps are not successful, a
continuous IV infusion of phenylephrine, an alpha agonist (0.5 to 5 µg/kg/min),
may be titrated to increase SVR. Propranolol, a beta-blocker, may be used to
decrease heart rate and promote ventricular filling, but care should be exercised
when administering this drug as hypotension may occur. If the spell persists,
general anesthesia and emergent surgery for placement of a systemic–pulmonary
shunt or full repair is the next step. Ketamine 1 to 2 mg/kg IM or IV is an
excellent option for sedation for endotracheal intubation or other procedures.
Emergency surgical repair or palliation with an aortopulmonary shunt are options
if the hypercyanotic spell still cannot be corrected.
After stabilization the patient should be admitted to an intensive care unit
skilled in cardiac care. Chronic oral beta blocker therapy may be initiated in an
attempt to decrease RVOT infundibular reactivity and thus prevent further spells.
Elective surgical management provides definitive care.
Management/Diagnostic Testing of Infant Presenting With Left-to-Right Shunt
and Pulmonary Overcirculation. Diuretic therapy is the mainstay of acute
treatment for pulmonary overcirculation due to left-to-right shunt lesions. When
used with afterload reduction, the symptoms of overcirculation may be mitigated
until the time of surgical repair. Hospital admission may be necessary for
initiation of medical therapy, treatment of concurrent infections, or surgery if
medical management is not effective. IV fluids and oxygen should be avoided in
these patients, as either intervention will worsen pulmonary overcirculation.
Clinical Indications for Discharge or Admission. Any child newly diagnosed with
hemodynamically significant CHD should be admitted to the hospital.
Consultation with a pediatric cardiologist can guide this decision. Very ill
