Case Study #1: History

• We are called to the NICU for a one day old.

• Pertinent History: Full term, + prenatal care, g2 p3 mother,
+ fever at delivery, uneventful delivery. Baby at 12 hours of
life had worsening resp. distress, lethargy and decreasing
O2 sats to 60%. Patient has been intubated and a UAC is in
place. Maintenance fluids have been started, Amp/Gent
have been given.
• The patient has upper right extremity pulse oximetry reading
of 60%, upper left extremity of 40%. Overall perfusion is
very poor. Blood pressure taken on left arm is 55/35 (mean
48). Resp. rate is 45-55, heart rate 120 with a normal
tracing.
 Case Study #1: Physical Exam
• The patients exam as reported over the
phone is significant for:
• Obtundation with mild movement, resp.
failure, femoral pulses, pupils 3mm bilat.
Reactive, fontanel wnl, abd. Wnl, loud
systolic murmur at LSB.
 Case Study #1: Data
• CXR reveals large
heart. No pulm.
consolidation,effusion,
diaphragmatic hernia.
• Electrolytes WNL
• WBC 12.5, Hct 45. Nl.
Differential.
• ABG 6.8/35/45/-25
 Case Study #1:BEFORE you
leave
• What are the most likely diagnosis?

Thinking of the potential diagnosis allows

instructions to be given to the referring hospital
that may make a difference in the childs condition
while the team is en route. Always think through
the diagnosis. Your conclusion may be different
than the referring hospitals and therapies may also
differ. Discuss your concerns with the attending in
the PICU or NICU.
 Case Study #1: Diff. Dx.
• Sepsis. Always a consideration in the sick
newborn. It can be a concomitant diagnosis.
• The mother had a fever during delivery.
GBS status and other prenatal labs are
unknown.
• Broad spectrum antibiotics
(Amp/Cefotaxime) should be started.
 Case Study #1: Diff. Dx.
• Congenital Heart Disease
• The presence of a murmur, hypoxia, resp.
distress is suspicious for CHD.
• No Echo is available.
• Differential hypoxia suggest
Aortic Coarctation or other ductal
dependent malformations.
 Differential Diagnosis.
• Pulmonary Hypertension
• Hypoxia, resp failure, acidosis,
hypotension.
• If there is a large PDA and small foramen
ovale (ie small amount of mixing) there
could also be a pre and post ductal gradient.
 Case Study#1: Diff. Dx.
• Respiratory etiologies: Pneumonia,
pulmonary malformation, airway
malformation, AVM (pulmonary, cerebral,
intrabdominal).
• Inborn error of metabolism
 Case Study #1: Before leaving
• The most likely diagnosis are sepsis vs. co-
arctation.
• Recommend start PGE-1. Do not accept it if told
they will wait until the team arrives. All hospitals
have PGE-1 and can start the drip. If a patient is
not intubated impart that apnea and hypotension
are common effects of PGE-1 so they can prepare
for them.
• Infant is severely acidotic. Recommend
NaBicarbonate bolus and drip if needed
 Case Study #1: Arrival
• Upon arrival the patient is on the ventilator
pre-ductal pulse ox is 65%, post ductal
pulse ox is 45-50%. Patient’s heart rate is
60bpm, on mechanical vent 25/4 rate of 30,
100% FiO2. BP in right arm is 55/35.
Perfusion is very poor baby is cyanotic.
• Your first move?
 Case Study #1: Arrival
• BEGIN CPR. Patients heart rate is 60.
• .01 of 1/10000 Epi per UAC line.
• Take patient OFF vent and hand bag. The
vent setting may be inadequate. Assess if
tube is in correct place and functioning
(SEE Xray personally. NEVER take report
unless there is no alternative. Be polite but
insistent (I usually say “its just my habit”).
 Arrival
• Patient responds to Epi. HR 124 with normal
tracing.
• Perfusion remains poor.
• Your exam reveals infant moves with stimulation,
extremely poor perfusion, lung sounds clear equal,
equal nl femoral pulses, loud systolic murmur.
• You notice the pulse oximetry readings show
equal readings in the 65-70’s at times and a pre
and post ductal readings other times.
 Interventions
Identify the problem list and attack it in order of
ABC’s WHILE considering the diagnosis and
other potential diagnosis. Think through the other
differential possibilities when making
interventions to evaluate if your intervention
would be contraindicated with an alternative dx.
For example sepsis requires large amounts of fluid
while the same amount of fluid would worsen a
congenital heart malformation with failure.
 Interventions
• Co-arctation remains the leading diagnosis
however a variety of congenital heart
defects can give the same clinical picture.
The key is that they may also be ductal
dependant
– Pulmonary Atresia, tricuspid atresia, Tet. Of
Fallot, interrupted aortic arch, transposition of
the great vessels (with or without intact septum)
 PGE-1
• Before starting be prepared for the two major side
effects
– Apnea- prophylactic intubation if needed.
– Hypotension -usually transient
• Have referring hospital start. At times there may
be resistance secondary to unfamiliarity. Reassure,
educate but get the drip started rather than wait
until the team arrives.
 Interventions
• Patient’s saturations remain in the 60’s with
bagging.
• What are you options?
 Nitrous Oxide
• Nitrous Oxide. The patient may have a
degree of pulmonary hypertension (or
indeed ONLY pulmonary hypertension).
• Adverse effects if patient is co-arctation or
sepsis are low.
• Benefits could potentially be high.
 Maximize Ventilatory Efforts
• Mode of ventilation. This patient may need high
frequency ventilation with Nitrous oxide.
• Do NOT get stuck fiddling with the ventilator with
a sick patient. Hand bag and assess pressure,
inspiratory times and compliance.
• Switch to ventilator when hand bagging has given
best results and assess. Some patients require hand
bagging for the entire transport.
• LISTEN and incorporate the RT’s assessment and
recommendations.
 Interventions
• ABC’s
• You assess the tube, suction, breath sounds
are equal with good chest rise.
• You are trying Nitrous oxide and hand
bagging with little effect. 02 sats remain in
the 60-65 range. End tidal C02 is 30.
• Anything more?
 Moving on.
• You are maximizing your resp. intervention.
• Do not get stuck on one system. Maximize your
interventions and move on. The goal is to stabilize
the patient and commence transport. The airway is
patent you are ventilating well. The oxygenation
may be secondary to a cardiac defect or pulmonary
hypertension that will not be fixed on transport.
• Other interventions may help the resp. status.
• Onto the Circulation.
 Interventions
• Blood pressure is 50’s/30’s and stable.
Perfusion is poor and there is a possibility
of a Co-arctation. Of note there are femoral
pulses, the oxygen saturation is matching
pre and post ductal at times.
• Ensure access. Place UV line.
• Possible etiologies?
 Keep Thinking!
• The PDA could be so large that femoral
pulses are palpated even with a coarctation.
• OR the diagnosis is incorrect and the poor
perfusion is making the oxygen saturation
unreliable and misleading.
• Other possibilities?
 Differential Diagnosis
• Sepsis- make sure broad spectrum abx are
given
• Sepsis with pulmonary hypertension
• Pulmonary Hypertension alone
• Other congenital heart defect
 Circulation
• Ensure the patient has adequate perfusing
volume. Is the patient third spacing with
paralysis? Any urine output?
• Large amounts of fluid are contraindicated
in CHF however if the patient has
inadequate perfusing volume or if sepsis is
suspected a fluid bolus (10-20 cc/kg) may
be indicated.
 Interventions
• Dopamine, Dobutamine, Epinephrine, Nor-
Epinephrine are all options. Which one?
When?
• Generally if a patient has poor perfusion, is
hypotensive after ensuring there is adequate
perfusing volume pressors are indicated.
 Pressors
• Effects depend upon the pressor and the receptor.
• Alpha receptors
– Alpha 1 postsynaptic: vasoconstriction, mydriasis,
contraction of urethral sphincter
– Alpha-2 PRE synaptic. Decrease in noradrenaline
release
• Beta-1 (ONE heart). + ionotropic effect, increased
rate, increased conduction (esp. at high doses).
• Beta-2 (TWO lungs): vasoDILATION,
bronchodilation, (20% of heart B receptors are
type 2 so cardiac effects less)
 Dobutamine
• Causes increased contractility (Beta-1 effect) BUT
also can have Beta-2 effects with vasodilation.
• Good for cardiogenic shock but not used as a first
line for septic shock.
• Contraindicated in Atrial Fib/Flutter, or Idiopathic
Subaortic Stenosis (increased contractility causes
increased outflow obstruction)
 Dopamine
• Variable effects which are dose dependent.
• Recent studies indicating “renal dosing” may be
well intentioned but without real effect.
• Between 5-10 mcg/kg/min beta-1 effects lead to
increased cardiac output. Increased rate cause
some concern for increased oxygen consumption.
• Contraindicated in tachyarrythmias, ventricular
fibrillation, pheochromocytoma
 Milrinone
• Phosphodiesterase inhibitor
• Initial Bolus 50 mcg/kg slowly over 1-2 minutes.
• Maintenance 0.375-0.75 mcg/kg/min
• Ionotrope with little chronotropic activity. Usually
used for “cardiac kids”. Has pronounced
vasodilatory effect.
• Watch potassium especially in patients on
Digitalis. Know the K+ and correct it BEFORE
starting.
 Inamrinone (amrinone, Inocor)
• Phosphodiesterase inhibitor. + ionotrope but
also + chronotrope
• Initial Bolus 0.75mg/kg slowly over 1-2
minutes.
• Maintenance 5-10 mcg/kg/min
• Contraindicated any outlet tract obstruction
(worse with increased contractility)
 Epinephrine/Nor-Epinephrine
• Getting to the kitchen sink.
• Use once adequate perfusing volume is
assured and other methods are not working.
• Concern of severe peripheral
vasoconstriction, increased cardiac
requirements are usually overrode by
severity of case.
 Circulation
• Little urine output is noted. One 10cc/kg
bolus given.
• If patient paralyzed and third spacing
Albumin is a good choice for volume.
• Dopamine is started at 5 mcg/kg/min and
patients blood pressure remains stable,
perfusion improves.
 Sedation/Paralysis
• Do not rush to sedate and paralyze. Removing
sympathetic tone and potential third spacing can
cause severe blood pressure, cardiac output issues.
• Indications
– Fighting the vent
– All over the bed
– Very “touchy” with desaturation
– Possible pulmonary hypertension
• Contraindications
– limp patient with hypotension.
– Comfortable patient who may need neurological
assessments.
 Sedatives/Paralytics
• Versed
• Fentanyl
• Ketamine- yes for asthma, NO for glaucoma, head
trauma or seizure.
• Succinylcholine for induction. Not in chronic CP,
burns or crush injuries
• Vecuronium for maintenance
• DO NOT forget to re-dose and try to re-dose before
you get back to the ICU so there is not an
“awakening” on sign over.
 Re-Assess
• After maximizing oxygenation, ventilation,
circulation step back and reassess.
• Think out loud. Go over interventions with
the team elicit suggestions AND implement
them. If you do not think an intervention is
warranted explain why. It makes the plan
clear to the team as a whole.
 Should I stay or Should I go?
• Case by case but a few general guidelines.
• If there is a clear life saving therapy
(surgery, nitrous oxide) that can be offered
by transporting the patient severely ill
patients can be transported AFTER a clear
and informed consent is signed by the legal
guardians. Use clear language. Do not
couch the truth.
 Stay or go?
• If the patient is actively coding from a
etiology that will not be improved upon by
transport (sepsis, inborn error of
metabolism) then the patient is too ill to
transport.
• If you believe the patient too unstable call
the ICU attending and discuss the case
before leaving.
 Transport
• The mother was consented.
• On transport the patients oxygen saturation
improved to 98% both pre and postductal.
• Perfusion improved.
• Thoughts?
 Transport
• The patient may indeed NOT have congenital
heart disease.
• Sepsis, Sepsis/Pulmonary hypertension or
Pulmonary Hypertension alone may be at work.
• Abx are on board, Nitrous is still on, perfusing
volume is adequate.
• Make sure to discuss the evolution of the patient
with the accepting team.