Pediatric emergency medicine trisk 1716 1716
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patients, such as those requiring a PGE1 infusion, should be admitted to an
intensive care unit skilled in the care of sick neonates with heart disease. The
safest mode of transport is with an advanced pediatric transport team.
Congenital Heart Disease: Postoperative and Other Considerations. For a
reference on congenital heart defects, surgical repairs, common complications,
and sequelae, see Table 86.5 .
Some guiding principles will help EM providers navigate the specifics of a
patient with known CHD. Red flags in CHD include age less than 2 months,
especially with palliated or unrepaired defects, poor cardiac function, single
ventricle anatomy, and arrhythmias. Special consideration should also be given to
infants or older children with respiratory illness, especially respiratory syncytial
virus, with palliative surgery or infants with pulmonary overcirculation.
Aortopulmonary shunts (modified Blalock–Taussig [BT], central shunts, etc.)
may be larger or smaller than optimal, causing problematic over or under
circulation of the lungs. The size of the shunts can be judged by oxygen
saturation. Patients with excessive shunt flow will present with high saturations
(>95%) and pulmonary edema. Patients with inadequate shunt flow due to shunt
malfunction or decreased systemic blood pressure, or a small shunt present with
cyanosis (saturations <75%).
A patient with BT shunt obstruction will present with cyanosis that may
progress to cardiac arrest. History of CHD, worsening cyanosis, and lack of a
shunt murmur in the right or left infraclavicular area raise concerns for shunt
obstruction. Risk factors include shunt size <4 mm, very young age at the time of
shunt placement, and low weight. Shunt obstruction may be due to thrombus
formation, often in the setting of dehydration or kinking of the shunt.
Treatment of shunt stenosis or obstruction is administration of an IV normal
saline bolus (10 to 20 mL/kg), heparin, and emergent surgical and cardiology
consultation. Shunt obstruction can be confirmed with an echocardiogram.
Endotracheal intubation may be needed. Pressors may augment systemic blood
pressure to perfuse the shunt. Consider pulmonary hypertensive crisis in the
differential diagnosis of a patient who presents with cyanosis and no shunt
murmur. Pulmonary hypertensive crisis is treated with hyperventilation, oxygen,
correction of acidosis, and sedation. Ketamine is a good choice for sedation if
needed in these patients.
Infants with single ventricle anatomy who have undergone the first stage
(Norwood Stage I) palliation are at high risk for sudden cardiac death (SCD).
About 15% of these infants will die before the second palliative operation.
