Case Study

1
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.

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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.

3
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

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Case Study 1BEFORE 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.

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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.

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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.

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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.

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Case Study1 Diff. Dx.

• Respiratory etiologies Pneumonia, pulmonary
  malformation, airway malformation, AVM
  (pulmonary, cerebral, intrabdominal).
• Inborn error of metabolism

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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

10
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. Patients 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?

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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).

12
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 65-70s at times and a pre and
  post ductal readings other times.

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Interventions

• Identify the problem list and attack it in order
  of ABCs 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.

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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)

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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.

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Interventions

• Patients saturations remain in the 60s with
  bagging.
• What are you options?

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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.

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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 RTs assessment and
  recommendations.

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Interventions

• ABCs
• 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?

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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.

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Interventions

• Blood pressure is 50s/30s 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?

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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?

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Differential Diagnosis

• Sepsis- make sure broad spectrum abx are given
• Sepsis with pulmonary hypertension
• Pulmonary Hypertension alone
• Other congenital heart defect

24
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.

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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.

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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)

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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)

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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

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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.

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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)

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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.

32
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.

33
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.

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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.

35
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.

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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.

37
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.

38
Transport

• The mother was consented.
• On transport the patients oxygen saturation
  improved to 98 both pre and postductal.
• Perfusion improved.
• Thoughts?

39
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.