I'm Blue and Bored - Review

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Sifting through the hoards of mucous and foreign objects in the nostrils of your city’s youth, you are ready for your mid-shift feeding in the peds ED. However, before stepping away to your trough, an EMS notification comes in for a hypoxic 10 week old child. You vomit and swallow, satisfying your hunger and head to the resuscitation room to prepare.

In comes a terrorized EMS team holding an agitated, grunting baby that is essentially blue, with an O2 saturation of 63% and a harsh systolic murmur.

What cyanotic congenital heart defect (CCHD) does our young agitator have?

Tetralogy of Fallot (hypercyanotic episode or “tet spell”).

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R ventricular tract obstruction
VSD
Overriding aorta
R ventricular hypertrophy

– Most common CCHD.

– Harsh systolic murmur.

– Only severe cases are ductus arteriosus-dependent (therefore, they can live to an older age).

– R->L shunt through large VSD causes cyanosis; the size depends on degree of pulmonary stenosis.

– Agitation increases peripheral vascular resistance (PVR) which increases R to L shunting and hypoxia-> hypoxia causes RVOT spasm and increases obstruction -> increase in R to L shunting.

After planning your career change to a hotel lobby pianist, what is your management?

In sequence of escalation:

Knee to chest position (increases PVR).
IV/IM/SC morphine (0.1-0.2mg/kg)
Fluid bolus (10-20cc/kg)
IV beta blockers, i.e. propranolol – decrease RVOT spasm
Phenylephrine – increase PVR.
Emergent surgical repair or shunt.

~ Neonates may need prostaglandins if they are dependent on their ductus arteriosus.

References

(1) Tintinalli, Judith E., Gabor D. Kelen, and J. Stephan. Stapczynski. Emergency Medicine: A Comprehensive Study Guide. New York: McGraw-Hill, Medical Pub. Division, 2004. Print.

(2) “Tetralogy of Fallor.” UpToDate. N.p., n.d. Web. 19 Jan. 2016.

(3) EM:RAP, Mizuho Files. April 2015.