EM ReSCu Peds 4: Congenital Heart Lesion

Brief Narrative Description of Case

You are working a shift in your Emergency Department (ED) when you hear an overhead announcement that a neonate is being brought in by EMS who is in distress. The nurses turn to you and ask which room to bring the baby into. The anticipated interventions of the Emergency Medicine (EM) resident are designed to include the ACGME milestones listed by The Emergency Medicine Milestone Project, listed below in bold:

  1. Team management (ICS2): Call for Peds/NICU/CICU help to care for the infant; utilization of TeamSTEPPS (see Debrief section) or other techniques for effective teamwork and communication used by your specific institution
  2. Performance of Focused History and Physical (PC2): Assess the infant patient
  3. Emergency Stabilization (PC1) and Medical Knowledge (MK): Escalate care to address to following medical and procedural learning objectives
  4. Airway Management (PC10): Respiratory distress and hypoxemia requiring airway support and consideration of broad differential
    • Pharmacotherapy (PC5), General Approach to Procedures (PC9), and Other Diagnostic and Therapeutic Procedures (PC14): Closing of ductal dependent lesions that requires emergent opening with prostaglandin E1 (PGE1)
    • Patient Centered Communication (ICS1): Effectively and sensitively communicate with the guardian(s) that the infant is critically ill and will be transferred to the NICU or CICU for ongoing evaluation and management

A 10-day-old male who is brought in by EMS for concerns of fast breathing and poor feeding for the past 1-2 days. At first glance, the medical team may suspect an infant with bronchiolitis or sepsis but should soon entertain the diagnosis of a ductal-dependent cardiac lesion.

Overall topics included in this scenario include: Infant resuscitation, diagnosis and management of ductal dependent lesion presentation in infants, effective communication with parents, recognition of a sick child, basic airway maneuvers, including appropriate positioning based on pediatric anatomy, and bag-mask ventilation (BMV).

Primary Learning Objectives

At the end of this simulation, participants should be able to:

  1. Demonstrate early evaluation of a critically ill patient (application)
  2. Describe signs and symptoms concerning for a ductal dependent cardiac lesion (comprehension)
  3. Implement a plan to care for an ill infant with likely ductal dependent lesion (application)
  4. Demonstrate focused history taking from a caregiver (application)
  5. Explain diagnosis and management to caregivers (synthesis)
  6. Demonstrate teamwork and closed loop communication (application)
  7. Organize transfer to a higher level of care where needed resources are available (synthesis)

Critical Actions

General

  • Elicit a team to help with tasks, specifically: Team lead, monitors and survey, airway, access, labs/medication administration, runner for help
  • Acknowledge an ill infant in the ED with possible ductal dependent cardiac lesion and call for stat NICU/CICU/ pediatrics help
  • Administer PGE1 and recognize likely complications of its administration
  • Effectively communicate with consultants
  • Deliver the news to the parent that the infant is in critical condition and will be taken or transferred to a Cardiac Intensive Care Unit for ongoing management

Specific Roles

  • Team lead
    • Assign team roles (monitors and survey, airway, access, labs/medication administration, runner for help).
    • Elicit helper to bring newborn warmer bed (or warm blankets), if not already located in ED bay
    • Instruct airway role to perform maneuvers (i.e., reposition airway, suction, start PPV)
    • Instruct monitors/survey role to apply leads and communicate exam
    • Get more history from the guardian and learn that the infant is eating poorly
    • Given this history, in the setting of an infant in distress and poor perfusion, consider cardiac closing of ductal dependent lesion and administer PGE1. Discuss dosing with pharmacy/NICU/CICU. Recognize risk of apnea with higher dose and consider intubation and mechanical ventilation.
    • Maintain normothermia and euglycemia
    • Treat presumed sepsis with fluids (gingerly) and antibiotics
    • Effectively give consultants (NICU/CICU/pediatrics) a recap of patient presentation and discussion of current concerns
    • Sensitively communicate with the guardian that the infant is critically ill and will be transferred to the Cardiac Intensive Care Unit (CICU) for ongoing management
  • Monitors and survey
    • Place infant on bed (with radiant warmer if available), and fully expose the patient
    • Record infant’s weight from bed scale (if available), use length-based tools available in your institution, ask guardian last weight
    • Place pulse oximeter (pre- and post-ductal)
    • Apply cardiac monitors, evaluate pulses, obtain 4-extremity blood pressures
    • Place temperature probe
    • Report loudly to group their initial impression of the infant
  • Airway
    • Acknowledge respiratory distress and impending cardiorespiratory failure
    • Comment on patient’s respiratory effort and cardiac exam
    • Demonstrate appropriate airway/breathing maneuvers (jaw thrust, chin lift, suction/PPV, CPAP)
  • Access
    • Place IV or IO
  • Labs/Medication administration
    • Obtain heel stick blood sugar level
    • Order labs/imaging when requested, including venous point-of-care labs, VBG, lactate, BNP, blood and urine cultures, CBC, CRP, BMP, CXR
    • Order ECG and echocardiogram (“are pending”)
    • Administer PGE1, while identifying apnea risk and preparing for potential intubation
    • Administer normal saline
    • Administer antibiotics (ampicillin, gentamicin, acyclovir)
    • Order epinephrine 1:10,000, atropine, fentanyl, rocuronium or vecuronium
  • Runner for help
    • Call stat NICU/CICU/Peds help
    • Get infant warming materials (bed, chemical mattress, hat, blankets)
    • Call radiology for x-ray if/when requested

Case Creators

Authors

  • Khoon-Yen Tay, MD
  • Elizabeth Sanseau, MD, MS
  • Leah Carr, MD

Editors

  • Ilana Bank, MDCM, FRCPC, FAAP
  • Rebekah Burns, MD
  • Fabiana Ortiz-Figueroa, MD
  • David Rayburn, MD, MPH

Updated September 18, 2020

Setup

Chief complaint: Tachypnea, poor feeding
Patient age: 10 days old
Weight: 3.5 kg

Recommended Supplies

  • Manikin/Simulated actor:
    • Neonate manikin that can be bag-mask ventilated and tolerate chest compressions
    • Simulated patient actor to play laboring mother
  • Moulage: None
  • Resources:
    • PALS algorithm, length based tape (e.g., Broselow) or other weight-based equipment, sizing and medication dosing reference
  • Manikin set up:
    • Grey, lethargic, diaphoretic infant in respiratory distress
  • Equipment:
    • Infant warmer bed with scale
    • Warm blankets, hat
    • Diaper
    • Monitors: Pulse oximetry, cardiac, temperature, blood pressure cuff
    • Rectal thermometer
    • Heel stick sampling kit, including alcohol wipe, lancet, portable blood sugar level reader
    • Bag valve mask, cardiorespiratory (CR) monitoring including ETCO2, intubation supplies, code cart
    • Suction: Bulb and wall suction (set at 80-100 mmHg)
    • Oxygen source
    • IV/IO access equipment
  • Medications:
    • Normal saline, epinephrine 1:10,000, prostaglandin (PGE1), atropine, fentanyl, rocuronium or vecuronium, ampicillin, gentamicin, acyclovir

Supporting Files

  • Point-of-care labs
  • Chest x-ray

Participants/Roles

  • Participants/learners:
    • Team leader
    • Airway manager
    • Survey physician
    • Medication preparer
    • Medication giver
    • Family liaison/history taker
  • Embedded participant roles:
    • Bedside nurse: Gets patient on monitor
    • Mom: Provides history
    • EMS: States uneventful transport. Thought heard wheezing, but did not give albuterol because of age. Gave blow-by oxygen for comfort.

Team roles may need to be adjusted in order to suit local practices and norms

Prerequisite Knowledge

  • Faculty
    • NRP and PALS protocols
    • General knowledge of emergency medicine
    • Simulation implementation and debriefing experience
  • Emergency medicine residents
    • Any stage of training
    • Completed a required pediatric rotation in medical school

Case Alternatives

  • If the participants do not resuscitate the infant (oxygen, fluids) and give PGE1, the patient decompensates into cardiorespiratory failure and requires cardiopulmonary resuscitation.

Virtual Resus Room

This simulation case can be run virtually using Google Slides and Zoom from the Virtual Resus Room (Congenital Heart Disease) page.

Milestones

PC1. Emergency Stabilization
PC2. Performance of Focused History & Physical Exam
PC3. Diagnostic Studies
PC4. Differential Diagnoses and Management
PC5. Pharmacotherapy
PC6. Observation and Reassessment
PC7. Disposition
PC10. Airway Management
PC15. Medical Knowledge
ICS1. Patient Centered Communication
ICS2. Team Management

Resources

  1. Weiner GM, et al. Textbook of neonatal resuscitation (NRP). 2019.
  2. Wing R. The neonate in shock: When to think CARDIAC. Brown Emergency Medicine (blog). 2015.
ITEMFINDING
Overall AppearanceGrey, lethargic infant in distress on resuscitation bed
HPIA 10-day-old male, who was born full-term via precipitous vaginal delivery, presents to your local hospital. He had been feeding well initially, though taking longer to finish his bottle more recently per mom. Over the past day or two the mom has noticed that he seems to be breathing faster and having difficulty feeding. He last fed <5 minutes prior to the ambulance arriving at the ED. He has had no noted apnea or cyanosis, though mom notes in general his color seems “off.” He has not been vomiting. Normal stooling. Slightly fewer wet diapers. No fevers. Given his increased respiratory rate and poor color, mom called an ambulance, because she did not have a ride to the ED.
Past Medical/Surgical HistoryFull term, precipitous vaginal delivery, normal prenatal/ perinatal care, delivery at 38 6/7 weeks, GBS negative. Birth weight 7 pounds (3.18 kg).
MedicationsUnknown
AllergiesUnknown
Family HistoryNo known family history of congenital cardiac lesions
Social HistoryFirst infant to this single mother, no other caregivers in home

Initial Assessment

Start through assessment of pediatric triangle (color, respiratory effort and tone), followed by A, B, C, Ds

Critical Actions

  • Assess ABCs
  • Obtain vital signs
  • Obtain IV access
  • Place on oxygen
  • Ask for POC glucose, labs

Physical Exam

ITEMFINDING
Vital SignsT: 36.5oC, HR: 184, BP: bilateral upper extremities (BUE) 90/52; unable to obtain in lower extremities), RR: 78, SpO2: 93% on room air for BUE; not picking up if probe on LE’s
GeneralAwake infant in respiratory distress
HEENTEyes closed
NeckFloppy/poor tone
LungsAirway intact, course throughout, grunting, tachypneic, subcostal and sternal notch retractions
CardiovascularTachycardia, + murmur heard throughout, weak peripheral pulses (especially femoral)
AbdomenSoft, mildly distended, + hepatomegaly 3 cm below costal margin
NeurologicalMoving all extremities, poor tone
SkinMottled lower extremities, cool to touch, capillary refill 4-5 seconds in lower extremities
MusculoskeletalNo obvious skeletal abnormalities

Instructor Notes: Changes and Case Branch Points

INTERVENTION / TIME POINTCHANGE IN CASEADDITIONAL INFORMATION
Team takes newborn from nurse, finds and turns on warmer bed, brings neonate to warmer bedNeonate remains grey with increased work of breathing
Participants divide into rolesIf no roles are delineated in group, nurse demands to know who is in charge.
Team lead delegates nurse to call stat NICU or pediatric consultantsHelp is notified and is “en route.”
Patient weighed in warmer bed/scale; monitors applied topatient (pulse oximetry probe, cardiac monitors, temperature monitor, rectal temperature)Weight: 3.5 kg
Place on oxygenSpO2 does not increase with supplemental oxygen, if the probe is located on the LEs. It increases very slightly if the probe is on UEs.
Obtain IV accessIV placed on first attempt, if attempted in the UE. Unsuccessful in lower extremities.
Ask for labs (glucose, CBC, CRP, BMP, blood cx, urine cx, VBG, lactate, BNP)Glucose 74 mg/dL with other labs pending. Proceed to Stage 2.
Call for respiratory backup, pharmacy, x-ray (as is available at your institution)They are “en route.”

Initial Interventions

Completion of primary assessment through evaluation of chest x-ray

Critical Actions

  • Attach ECG monitors
  • Interpret POC labs
  • Order NS bolus
  • Order antibiotics
  • Order chest x-ray

Physical Exam

ITEMFINDING
Vital SignsT: 36.5oC, HR: 180, BP: 90/50 BUEs and 50/20 or unable to obtain in the BLEs, RR: 74, SpO2: 93% on 100% FiO2 via blow-by or NC, if probe on UEs, or SpO2 70% if on LEs
Exam Changes
  •  Continues to have increased work of breathing and prolonged capillary refill with weak pulses
  • HR unchanged 180s

Instructor Notes: Changes and Case Branch Points

INTERVENTION / TIME POINTCHANGE IN CASEADDITIONAL INFORMATION
Reassessment
  • Continued increased work of breathing, prolonged capillary refill, and weak pulses
VBG result available
  • pH 7.2
  • pCO2 30 mmHg
  • HCO3 15 mEq/L
  • BE -8
Order NS IV bolus, 5-10 mL/kg
  • No change in vital sign parameters if requested on pump or given slowly
  • If fluid bolus is pushed, will see slight improvement in vital signs in lower extremities, if pulse oximetry probe on LE and BP cuff taken on LE. If asked, perfusion slightly better in LE.
  • Goal for fluid bolus is to increase preload to overcome the obstruction from the coarctation.
  • If concerned about heart failure/cardiac lesion, reasonable to start with 5-10 mL/kg initial bolus to avoid precipitating significant pulmonary edema. Should always reassess.
  • At any point if 20 mL/kg of IV fluid are given, the patient’s oxygen saturation will drop to 89%. Increased preload will cause pulmonary edema and increase the liver size. BP will drop in UEs to 80/40.
Order broad spectrum antibiotics (ampicillin, gentamicin, acyclovir)
  • Antibiotics “being drawn up”
Place orogastric tube
  • Successfully placed
Order chest x-ray
  • Chest x-ray image provided and notable for cardiomegaly, decreased pulmonary markings, and OG tube in stomach. Proceed to Stage 3.

Initiation of PGE1

Review of CXR through initiation of PGE1 AND intubation

Critical Actions

  • Order prostaglandin (PGE1)
  • Request a stat cardiology consult
  • Repeat NS IV bolus

Physical Exam

ITEMFINDING
Vital SignsT: 36.2oC, HR: 175, BP: 74/36 (BUE), 50/20 (BLE), RR: 72, SpO2: 93% on 100% FiO2 (BUE); saturation reading is intermittent if problem is placed in the LEs because of poor perfusion
Exam ChangesMore tired in appearance, grey coloring, poor pulses

Instructor Notes: Changes and Case Branch Points

INTERVENTION / TIME POINTCHANGE IN CASEADDITIONAL INFORMATION
Ask if other labs have returnedCBC

  • WBC 8.9 x103/mm3
  • HgB 15.9 g/dL
  • Plt 255 x103/mm3

BMP

  • Na 140 mEq/L
  • K 3.8 mEq/L
  • Cl 100 mEq/L
  • CO2 16 mEq/L
  • BUN 40 mg/dL
  • Cr 1.0 mg/dL
  • Ca 9.0 mg/dL

Lactate: 4.9 mmol/L

LFT

  • ALT 50 U/L
  • AST 58 U/L
  • Total Bili 0.4 mg/dL
  • Direct Bili 0.2 mg/dL
  • Total Protein 7.0 g/dL
  • Albumin 4.0 g/dL
Order prostaglandin (PGE1)Should start at moderate end of dosing range and gradually increase until improvement in perfusion and BP and SpO2 after 10-15 min. If start at high end of dose, the patient will develop apnea and flushing.For any concern for a ductal dependent lesion where the duct closed, start PGE1 at a moderate dose (starting at 0.05 mcg/ kg/min) to open it. The top end of range is 0.1 mcg/kg/min. For reference, 0.01 mcg/kg/min is the dose for maintaining a patent duct (like in the delivery room).
Cardiology, Neonatology, or Pediatrics consultedLeader should give a comprehensive “one- liner” on the patient that indicates significant concern for patient and requesting expert consultation.
  • If verbalizes concern for congenital heart disease and/or ductal-dependent lesion, consultation should give appropriate recommendation for PGE1.
  • If verbalizes concern for sick neonate and has cardiac lesion on differential, would be appropriate for consult to discuss treatment with PGE1, as well as discussing treatment for other diagnoses on team’s differential.
  • If verbalizes concern for sick neonate but does not have congenital heart disease/ductal dependent lesion on differential, consult should ask team if they have considered that diagnosis.
Repeat NS IV bolus: 5-10 mL/kg
  • If prostaglandins started at moderate dose, bolus should result in continued improvement in VS parameters and perfusion.
  • BP in LE will become more similar to UEs (UEs 80s/40s and LEs 70s/30s)
1 minute after PGE1 started
  • Patient becomes apneic
  • SpO2 quickly goes to <70% if PPV is not initiated
  • HR decreases to <100 if PPV is not initiated within 30 seconds of becoming apneic
Team proceeds with BMVSpO2 will go back to >93% with 100% FiO2
If atropine 0.02 mg/kg givenIncrease in HR by 20 bpm from pre-administration HRAtropine is sometimes given to decrease secretions and blunt vagal response to intubation.
Sedative given for emergent intubation (e.g., fentanyl 1-2 mcg/kg/dose)No significant vital sign changes
Paralytic medication given, per institution (e.g., vecuronium 0.1 mg/kg)Patient becomes apneic and stops any spontaneous movementsInduction of paralysis time will depend on paralytic administered
Intubate with a 3.5 uncuffed ET tube using 0-1 laryngoscope blade to a depth of ~9-10.5 cm at the lip in coordination with medications given: +/-pre-med, sedative, paralyticVital signs post intubation:

  • T: 36.2oC (unchanged)
  • HR: 150
  • BP: 80s/40s UEs and 70s/30s LEs
  • RR: bagged rate
  • SpO2 >93%
Confirm presence of tube with end tidal CO2, mist in tube, good chest rise and auscultation. Repeat chest-xray.
Bedside cardiac ultrasound (if available)Normal cardiac anatomy; LV function decreased, mitral valve regurgitation jet if color Doppler used.
Team member updates mom on planMom verbalizes understanding and thanks team for care of her newborn. Proceed to Stage 4.

Case Conclusion

After fluid resuscitation and PGE1 administration and calling for NICU/CICU/Pediatrics help

Critical Actions

  • Discussion of patient with NICU and appropriate disposition stated

Physical Exam

ITEMFINDING
Vital SignsT: 36.2oC, HR: 170, BP: 80/40 (BUE), 70/30 (BLE), RR: 65, SpO2: 95% on 100% FiO2 upper and lower extremities
Exam ChangesImprovement in perfusion and BP and work of breathing after 10-15 min after initiation of PGE1

Instructor Notes: Changes and Case Branch Points

INTERVENTION / TIME POINTCHANGE IN CASEADDITIONAL INFORMATION
Call NICU/CICU/Peds; full summary givenConsultant accepts patientMakes recommendation to start patient on PGE1 infusion. Start at low dose initially (0.01 mcg/kg/min), can increase up to 0.05 mcg/kg/min in consultation with NICU/CICU up to max dose of 0.1 mcg/kg/min.

Demonstrate early evaluation of a critically ill patient (application)

Learners should approach a sick infant in a standardized fashion. The pediatric triangle and airway, breathing, and circulation should be assessed immediately. Interventions such as airway repositioning/ adjuncts, BMV, and CPR should be started concurrently, if required. After A, B, C have been addressed, the patient should be evaluated for disability and exposed for a thorough head to toe exam, and a blood sugar level should be obtained. Labs/imaging should be ordered and antibiotics and fluids administered in a timely fashion.

Describe signs and symptoms concerning for a ductal dependent cardiac lesion (comprehension)

Learners should recognize the symptoms of poor feeding and poor perfusion in a neonate as evidence of shock and to keep cardiogenic shock from a closing ductus arteriosus at the top of their differential.

Learners should recognize that different blood pressure values obtained pre-ductus arteriosus (usually right upper extremity) and post-ductus arteriosus (reliably the lower extremities) is suggestive of a coarctation.

Implement a plan to care for an ill infant with likely ductal dependent lesion (application)

The learners should promptly assess ABCDEs: Keep airway patent, supplement as appropriate with oxygen, ventilate the patient if necessary, support circulation by administering prostaglandin (PGE1) to reopen a closing ductus arteriosus and administer small fluid boluses with frequent reassessments between interventions, assess for disability, and maintain euglycemia and euthermia.

The learners should also identify appropriate consultants to discuss the case with and determine appropriate disposition based on their local resources.

The learners should also learn to anticipate adverse effects from the medications or interventions that are necessary for the care of an ill infant with a likely ductal-dependent lesion. They should consider interventions to mitigate these effects.

Demonstrate focused history taking from a caregiver (application)

The parental history should be focused during the initial evaluation on possible newborn etiologies of cardio-respiratory failure. Assign a participant to get a thorough history from the mother in a sensitive manner.

Explain diagnosis and management to caregivers (synthesis)

If personnel are available, assign one member of the team to stay with the mother to gather history and explain interventions. Update the mother on the baby’s status, interventions that have been done, and what next steps are (admission to the NICU/CICU). Avoid using medical jargon.

Demonstrate teamwork and closed loop communication (application)

Teams may use different frameworks to improve team dynamics and communication. Below are a few definitions that may be helpful to discuss, adapted from the AHRQ TeamSTEPPS Pocket Guide.

  • Brief: Short session prior to start of encounter to share the plan, discuss team formation, assign roles and responsibilities, establish expectations and climate, anticipate outcomes and likely contingencies
  • Huddle: Ad hoc team discussion to re-establish Situation Awareness; designed to reinforce plans already in place and assess the need to adjust the plan
  • Callout: A strategy used to communicate critical information during an emergent event. Helps the team prepare for vital next steps in patient care. (Example: Leader- “Airway status?”; Surveying provider- “Airway clear”; Leader- “Breath sounds?”; Surveying provider- “Breath sounds decreased on right”)
  • Check-back: A closed-loop communication strategy that requires a verification of information ensuring that information conveyed by the sender is understood by the receiver as intended. The sender initiates the message; the receiver accepts it and restates the message. In return, the sender verifies that the re-statement of the original message is correct or amends if not. (Example: Leader- “Give diphenhydramine 25 mg IV push”; Med Prep- “Diphenhydramine 25 mg IV push”; Leader- “That’s correct”)
  • SBAR: A framework for team members to structure information when communicating to one another.
    • S = Situation (What is going on with the patient?)
    • B = Background (What is the clinical background or context?)
    • A = Assessment (What do I think the problem is?)
    • R = Recommendation (What would I do to correct it?)
  • Situation monitoring: The process of continually scanning and assessing a situation to gain and maintain an understanding of what is going on around you.
  • Situation awareness: The state of “knowing what’s going on around you.”
  • Shared mental model: Result of each team member maintaining situation awareness and ensures that all team members are “on the same page.” An organizing knowledge structure of relevant facts and relationships about a task or situation that are commonly held by team members.
  • STEP: A tool for monitoring situations during complex situations. A systematic method to review Status of patient, Team members’ performance and status, Environment, and Progress towards goal.
  • Cross-monitoring: A harm error reduction strategy that involves 1. Monitoring actions of other team members 2. Providing a safety net within the team. 3. Ensuring that mistakes or oversights are caught quickly and easily. 4. “Watching each other’s back.”
  • CUS: Signal phrases that denote “I am Concerned,” “I am Uncomfortable,” and “This is a Safety Issue.” When spoken, all team members should understand clearly not only the issue but also the magnitude of the issue.

Develop a plan to transfer an infant to a higher level of care where needed resources are available (synthesis)

Give an organized and thorough patient sign-out to the transferring team (NICU/CICU/Peds/transport team). Be specific about the presenting scenario, initial evaluation, labs/imaging obtained, and what interventions were done with an update on how the patient has responded to said interventions.

Chest X-ray

Download Case 4 supporting files

Notes:

  • Chest x-ray interpretation: Post intubation film showing orogastric tube, leads, temperature probe, and high-riding ET tube. There are also some patchy opacities in the lung fields and possible cardiomegaly. Image from Dr. Leah Carr.

For the embedded participant playing the mother of her newborn baby, who is coming in with poor feeding and fast breathing

Case Background Information

The case is a 10-day-old male who is brought in by EMS for concerns of fast breathing and poor feeding for the past 1-2 days. The medical team entertains a broad differential including the diagnosis of a congenital heart lesion (i.e., closing of duct with dependent lesion), metabolic, sepsis, and pulmonary etiologies.

Who are the Learners?

Emergency Medicine interns and residents: They have little prior experience resuscitating babies, but do have significant experience in gathering information from patients and family members.

Standardized Patient Information

Over the past few days your 10-day-old son has been breathing faster and does not want to eat (breast milk or bottle). He last ate for <5 minutes just prior to the ambulance arrival. He seems tired. He has not stopped breathing or turned blue, but his color seems “off” to you. No vomiting, normal stooling, fewer wet diapers (3 per day instead of 6), no fevers. Given his fast breathing and poor color, you called an ambulance because you did not have a ride to the ED.

Meanwhile, the infant is being attended to by the medical team. The baby is grey and mottled with tachypnea. The patient is supported with airway and breathing maneuvers (including supplemental oxygen, positive pressure ventilation, and intubation), fluids, antibiotics, and a medication, PGE1, to treat the cardiac lesion before being signed out to the NICU team.

Patient Information

(Please remember not to offer any of this information, but when asked please respond while remaining in character.)

  • CHIEF COMPLAINT (your response to open-ended questions such as “what’s going on?” or “what can we do for you? Or “what happened?”): Fast breathing and difficulty with eating
  • AGE: 10 days old
  • ADDITIONAL HISTORY: Prenatal care limited to first trimester. You moved and did not find a new OB to get care with. Baby is estimated to be at or near term.
  • PAST MEDICAL HISTORY: Ex-full term, precipitous vaginal delivery, normal prenatal/ perinatal care, delivery at 38 6/7 weeks, GBS negative, birth weight 7 pounds
  • SOCIAL HISTORY: Lives with 2 parents, first baby
  • FAMILY HISTORY: Does not know
  • PAST SURGICAL HISTORY: None
  • MEDICATIONS: None
  • ALLERGIES: No known drug allergies
  • IMMUNIZATIONS: Up-to-date

Potential Dialogue

IMPORTANT: Do not offer unsolicited information. Please allow the learners to ask questions. Do not offer information unless they ask you.

Things you could say without being asked:

  • “How is my baby? I want to hold my baby. Can someone tell me what’s going on?”

Things you might say triggered by events in the scenario:

EVENTYOUR POTENTIAL RESPONSE
If participants do not recognize that the baby’s color is more grey“Why does his skin color look different?”
If participants do not recognize fast breathing“Why is he breathing so quickly?”

The patient will be brought back to the room. The nurse quickly asks for the physician to evaluate the patient because he doesn’t look “well.” The patient is noted to be tachypneic and tachycardic, and his color/perfusion is poor. He is awake, but not vigorous. The medical team should initially recognize an ill neonate and have on their differential diagnoses: sepsis, respiratory failure, cardiac disease, and metabolic disorder. On physical examination, they will note a murmur, decreased LE pulses, and hepatosplenomegaly. They should ask for IV access, general labs, a normal saline bolus, and a chest x-ray.

When considering congenital heart disease, the team should ask for an ECG, 4-extremity blood pressure measurements, chest x-ray, and pre- and post-ductal saturations. The team should recognize the potential for a ductal-dependent lesion and call for more assistance, either Cardiology/Neonatology/or Pediatrics, whichever is more likely. Either independently, or after consultation with experts, they should order prostaglandin E (PGE1; alprostadil). Their expert consultation should warn them that apnea is a PGE1 side effect and be ready to support breathing with BMV and intubation if necessary. If the team does not recognize apnea, the patient should go into cardiopulmonary arrest.

Disposition should be to the nearest NICU or CCU/CICU, depending on the discussion with the consultants.

Dosing for PGE1: Start at low dose initially (0.01 mcg/kg/min), can increase up to 0.05 mcg/kg/min in consultation with NICU/CICU up to max dose of 0.1 mcg/kg/min. Note that the team should start PGE1 empirically if there is a strong clinical suspicion for ductal-dependent congenital heart disease based on the initial evaluation. They should anticipate the side effect of apnea and learn that the risk increases with increasing doses of PGE1 infusion. The team should discuss intubation and mechanical ventilation early when ordering and administering PGE1.

Anticipated Management Mistakes

  1. Failure to consider a broad differential, including cardiac etiology: The patient will decompensate quickly if treated with aggressive fluid resuscitation (>30 mL/kg) and/or do not support airway, breathing, and circulation with PGE1. The differential in a persistently hypoxic newborn is broad and includes: sepsis, persistent pulmonary hypertension, and congenital anomalies (e.g., congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, tracheoesophageal fistula, and congenital cardiovascular anomalies).
  2. Failure to obtain access with IV: The participants must obtain access to administer PGE1 and resuscitation. If unable to obtain a peripheral IV, must obtain IO access.
  3. Failure to initiate PGE1 or only at the lower dosing range: Patient will continue to deteriorate.
  4. Initiating PGE1 at higher doses: This increases the risk of apnea. Team should discuss this and be prepared to intubate.
  5. Fluid resuscitation at 20mL/kg or above will increase preload: This will result in further cardiac failure and increase pulmonary edema and venous backup into liver, causing further enlargement of liver.

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