CDH Management Protocol

Antepartum (Fetal Center)

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Level III ultrasound

LHR - Routinely calculated (? PLUG if < 0.5) 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

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Airway Management

– No bag valve mask or CPAP. Immediate ETT

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

>85% - preductal increase no faster than NRP guidelines, wean FiO2 when preductal SaO2 up to

iNO

– if baby requires FiO2 of 100% and pre-ductal sats < 90%

NICU Stablilization

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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 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 Paralysis

- fentanyl 1mcg/kg/hr - avoid

Initial Ventilation Strategy

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IMV

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- Initial settings PCV 22/5 x 40 It = 0.35 Max RATE = 60

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Max PIP = 25 Oxygenation

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Preductal sat > 70%x 1 hour, by 2 hours >85% with adequate delivery based on lactate, goal 90-95%

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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

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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

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FiO2 to keep SaO2 90-95%

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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

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PaCO2 > 65 with acidosis on PIP 25 and rate 60

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Pre-SaO2<70% or post-ductal PaO2<40 HFV initial settings

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HFOV MAP=IMV MAP + 2

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Delta P = PIP, “adequate bounce” Starting frequency 10 Hz Weaning

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Wean MAP slowly (decrease by 0.5 q4h) if FiO2<0.60

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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

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- 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

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post-ductal PaO2<40 AND echocardiographic evidence of PH

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Dopamine and Dobutamine - begin at 5/5 and increase as needed Pulmonary Hypertension iNO

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- Criteria for treatment – Pre ductal SaO2<70% or iNO at 20ppm, wean when FiO2<0.6 and adequate oxygenation Prostacyclin

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Reserved for rescue post-ECMO or where ECMO contraindicated

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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

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to correct systemic hypotension into normal range after volume expansion and oxygen carrying capacity optimized Milrinone

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RV dysfunction/dilation and additional afterload reduction after iNO Prostaglandin

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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 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

Criteria for ECMO

• • • • • • • • • 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 (both neonatology and ped surgery)

ECMO Contraindications

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IVH Grade 2 or greater Lethal chromosomal anomalies/syndromes Complex congenital heart disease (single ventricle physiology) EGA < 34 wks

CDH ECMO

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Echocardiographic Surveillance:

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Cardiology to have Attending ECHO read upon arrival in NICU

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Serial exams with at least one additional ECHO at 48h on ECMO ECMO Cannulation

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Routine use of VA ECMO in CDH

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Place 8 Fr arterial cannula 12 Fr venous cannula or smaller Duration of ECMO Run

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Duration of ECMO based upon a multidisciplinary review of the course and projected outcome / assessment of futility

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Periodic trial of lower flows/trial off with echo assessment of PH Decannulation

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Consider when trial off-EMCO suggests native gas exchange and CV function is sufficient

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Consider targeting higher PaCO2 range for final 3-7 days of ECMO run

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Routine carotid artery repair unless contraindicated / unfeasible Routine Broviac placement

CDH Repair (no ECMO)

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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

CDH Repair (ECMO)

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Timing of repair will be based upon an ECHO after 48h on ECMO ( maintain inflation until ECHO )

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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

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If successfully weaned off ECMO – timing of surgery same as non ECMO babies (echo driven decisions) Peri-Operative Anticoagulation Management

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Hold heparin infusion 1 hour pre-op, during the case and 1 hour post-op

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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

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Routine analysis of institutional CDH registry data and morbidity assessment every 10 cases or6 months (whichever occurs first) with departmental presentations