Initial Consensus MCJCHV CDH Management Protocol

1. Initial Consensus MCJCHVCDH Management Protocol Reviewed and Approved June 2012: Division of Pediatric Surgery Division of Neonatolgy

2. CDH Management Protocol

3. Antepartum (Fetal Center) • Level III ultrasound • LHR- Routinely calculated • 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, PedSurg, Genetics, etc

4. 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 • Surfactant- As indicated for RDS based on EGA • Calculate LHR or O/E LHR: http://www.perinatology.com/calculators/ LHR.htm

5. 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%

6. 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

7. Initial Ventilation Strategy • IMV- Initial settings PCV 22/5 x 40 It=.035 • Max RATE = 60 • Max PIP = 26 • Oxygenation • Preductalsat > 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

8. 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

9. CDH Patient Management • Fluid Management - Initial 90 ml/kg with early protein - Avoid fluid overload - Furosemide for fluid overload when hemodynamicallystable • 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

10. High Frequency Ventilation • Criteria to Convert from PCV 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 • HFOVMAP=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%

11. 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

12. ECMO Contraindications • IVH Grade 2 or greater • Lethal chromosomal anomalies/syndromes • Complex congenital heart disease (single ventricle physiology) • EGA < 35 wks

13. 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 • 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 Broviacplacement

14. 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

15. CDH Repair (ECMO) • Timing of repair will be based upon an ECHO after 48h on ECMO • If there ISimprovement in the pulmonary HTN (less than systemic) – delay repair (with a close eye on volume status), consider repair off ECMO • If there is NOimprovement 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

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