CDH Management
Protocol
Antepartum (Fetal Center)
• Level III ultrasound
• LHR - Routinely calculated –if survival estimate
less than ? % refer to PLUG center?
• O/E LHR - Routinely calculated up to 32 weeks
• Both LHR results will be listed on the bottom of
the front StarPanel page
• Cardiac echo - Routine
• Liver position – Determined and reported
• Multidisciplinary consults – MFM, NICU, Ped
Surg, Genetics, etc
Antepartum (Fetal Center)
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Fetal MRI – Not standard (QLI)
Follow-up – Monthly – BPP 2/wk at 34 wks
Timing of delivery – Induction at 39 wks
Antenatal steroids – For labor EGA < 34 wks
Calculate LHR or O/E LHR:
http://www.perinatology.com/calculators/
LHR.htm
Delivery Room
• Airway Management – No bag valve mask or CPAP.
Immediate ETT for respiratory distress or BBO2 if stable
• GI decompression – Replogle tube following airway
• Ventilatory Pressures - 20-25/5-6
• FiO2 (initial) – 100%
• Transport Vent - 20-25/5-6 x 40 It=0.35, FiO2=1
• SaO2 target - preductal increase no faster than NRP
guidelines, wean FiO2 when preductal SaO2 up to
>85%
• iNO – if baby requires FiO2 of 100%
NICU Stablilization
• SaO2 (preductal) - >70% x 1 hour, >85% by 2 hours, goal
90-95%
• Studies - Routine ECHO, HUS, cultures, PT/PTT, CBC, CRP,
state screen, cortisol, karyotype & microarray?
• Access – attempt single lumen UAC before peripheral a-line
or UAC cut down (consult surgeon)
– Single attempt UVC, if unsuccessful convert to emergent
position, discuss PICC vs. Cook vs. other with team based on
stability
• Sedation - fentanyl 1mcg/kg/hr – additional dose for
cardiac echo – add Versed as needed
• Analgesia - fentanyl 1mcg/kg/hr
• Paralysis - avoid
Initial Ventilation Strategy
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IMV - Initial settings PCV 22/5 x 40 It=.035
– Max RATE = 60
– Max PIP = 26
Oxygenation
– Preductal sat > 70%x 1 hour, by 2 hours >85% with adequate
delivery based on lactate, goal 90-95%
– Post ductal PaO2 >40 (consider >35 with adequate preductal
SaO2 and lactate)
Ventilation – Goal = pCO2 50-65
pH - Goal = 7.2 – 7.35
Perfusion – O2 delivery with lactate < 3 mmol/L; transiently (2 hours)
tolerable lactate >3, but <5
Weaning
– wean PIP first with adequate tidal volume, then rate to SIMV
when on low rate, volume based on PFT TV on prior setting,
target 4-5 cc/kg
– FiO2 to keep SaO2 90-95%
– Wean PEEP slowly (decrease by 0.5 q4h) if FiO2<0.60 with 8 rib
expansion
High Frequency Ventilation
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Criteria to Convert from CV to HFV
– PaCO2 > 65 with acidosis on PIP 26 and rate 60
– Pre-SaO2<70% or post-ductal PaO2<40 when IMV has
failed to achieve adequate (8-9 ribs) contralateral
recruitment
HFV initial settings
– HFOV MAP=IMV MAP + 2
– Increase MAP to achieve 8-9 rib expansion contralateral
to CDH
– Delta P = PIP, “adequate bounce”
– Starting frequency 10 Hz
Weaning
– Wean MAP slowly (decrease by 0.5 q4h) if FiO2<0.60 with
8 rib expansion (or 10 rib expansion?)
– Wean frequency first to 10, then delta P to PaCO2 50-65
– FiO2 to keep SaO2 90-95%
CDH Patient Management
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Systemic Hypotension - Criteria for treatment - Abnormal MAP for age
– NS bolus, pRBC’s if Hct<40, FFP for abnormal initial coagulation studies –
combined up to 40ml/kg in first 2 hours
– Dopamine and Dobutamine - begin at 5/5 and increase as needed
Pulmonary Hypertension - Criteria for treatment – Pre ductal SaO2<70% or
post-ductal PaO2<40 AND echocardiographic evidence of PH
– iNO
• iNO at 20ppm, wean when FiO2<0.6 and adequate oxygenation
– Prostacyclin
• Reserved for rescue post-ECMO or where ECMO contraindicated
• Consider inhaled for sustained hypoxemia on iNO if adequate
ventilation and adequate contralateral lung recruitment can be
achieved on conventional ventilator. Note: potential for
platelet/bleeding effect
– Catecholamines
• to correct systemic hypotension into normal range after volume
expansion and oxygen carrying capacity optimized
– Milrinone
• RV dysfunction/dilation and additional afterload reduction after iNO
– Prostaglandin
• Prostaglandin for RV overload with restrictive PDA
CDH Patient Management
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Fluid Management
- Initial 90 ml/kg with early protein
- Avoid fluid overload
- Furosemide for fluid overload when hemodynamically stable
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Laboratory Management
- Hematocrit > 40%
- Heparin assay (anti Xa) q6h, ATIII level QD (on ECMO)
- Platelet count > 100,000 perioperatively (on ECMO)
- TEG with clinical bleeding (on ECMO)
Antibiotics
- No specific indication for antibiotics with CDH alone
- Evaluate maternal risk factors, initial sepsis screen
- Start prior to cannulation
Sedation
- As clinically indicated
- Paralysis should be avoided if possible, use with caution
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Criteria for ECMO
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SaO2<85% on HFOV and iNO
HFOV MAP>17
OI>40 consistent (3 post-ductal BG over 2 hours)
Inadequate oxygen delivery, pH<7.20, lactate>5 despite adequate
volume expansion and pulmonary recruitment
Respiratory acidosis despite optimized HFOV pH<7.20,
PaCO2>70
Hypotension resistant to fluid and inotropic support with
UOP<0.5ml/kg/hr
Impending ventricular failure on ECHO with evidence of
inadequate oxygen delivery
Preductal sat <70 for 1 hour
• Attending to Attending Notification
ECMO Contraindications
• IVH Grade 2 or greater
• Lethal chromosomal
anomalies/syndromes
• Complex congenital heart disease
(single ventricle physiology)
• EGA < 35 wks
CDH ECMO
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Echocardiographic Surveillance:
– Cardiology to have Attending ECHO read upon arrival in NICU
– Serial exams with at least one additional ECHO at 48h on ECMO
ECMO Cannulation
– Routine use of VA ECMO in CDH
– Place 8 Fr arterial cannula
– 12 Fr venous cannula or smaller
Duration of ECMO Run
– Duration of ECMO based upon a multidisciplinary review of the course and
projected outcome / assessment of futility
– Periodic trial of lower flows/trial off with echo assessment of PH
Decannulation
– Consider when trial off-EMCO suggests native gas exchange and CV
function is sufficient
• Ionotropic and ventilatory support should be below ECMO cannulation
settings
– Consider targeting higher PaCO2 range for final 3-7 days of ECMO run
– Routine carotid artery repair unless contraindicated / unfeasible
– Routine Broviac placement
CDH Repair (no ECMO)
FiO2<0.5
Normal BP for EGA
Lactate <3
Pre-operative ECHO required demonstrating
improvement in pulmonary hypertension and
good right ventricular function
• UOP > 2ml/kg/hr
• Chest Tube – Consider no use of routine
chest tube when repaired off ECMO
• Location of Repair OFF ECMO:
- In NICU with Pediatric Anesthesiology
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CDH Repair (ECMO)
• Timing of repair will be based upon an ECHO after 48h on
ECMO (maintain inflation until ECHO)
– If there IS improvement in the pulmonary HTN (less
than systemic) – delay repair (with a close eye on
volume status), consider repair off ECMO
– If there is NO improvement in the pulmonary HTN
after 48h ECMO support – move towards early repair
in 24-48h
– If successfully weaned off ECMO – timing of surgery
same as non ECMO babies (echo driven decisions)
• Peri-Operative Anticoagulation Management
– Hold heparin infusion 2 hours pre-op, during the case
and 2 hours post-op
– Restart heparin drip at pre-op rate, no bolus
• Chest tube – Routine placement of chest tube (15f Blake
drain) for repair done on ECMO
• Temporary/Staged Abdominal Closure
Outcomes
• Routine analysis of institutional
CDH registry data and morbidity
assessment every 10 cases or 6
months (whichever occurs first)
with departmental presentations