CDH Management Protocol

Antepartum (Fetal Center)

• 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)

• 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

• 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% and pre-ductal sats

< 90%

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 – 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• IMV - Initial settings PCV 22/5 x 40 It = 0.35

– Max RATE = 60– Max PIP = 25

• 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

• Criteria to Convert from CV to HFV– PaCO2 > 65 with acidosis on PIP 25 and rate 60– Pre-SaO2<70% or post-ductal PaO2<40

• HFV initial settings– HFOV MAP=IMV MAP + 2– Delta P = PIP, “adequate bounce”– Starting frequency 10 Hz

• Weaning– Wean MAP slowly (decrease by 0.5 q4h) if FiO2<0.60– Wean frequency first to 10, then delta P to PaCO2 50-

65– FiO2 to keep SaO2 90-95%

CDH Patient Management• 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

• 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

• IVH Grade 2 or greater• Lethal chromosomal

anomalies/syndromes• Complex congenital heart disease (single

ventricle physiology)• EGA < 34 wks

CDH ECMO• 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 – 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

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 1 hour pre-op, during the case and 1 hour

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 or6 months (whichever occurs first) with departmental presentations