2. Identifying Data Live, term, baby boy delivered via STAT caesarian section for nonreassuring fetal heart rate pattern to a 33 year old G1P1 (1001) at 40 weeks age of gestation BW= 4210g BL= 452 cm HC= 35 ½ cm CC= 37 cm AC= 32 cm MT 39 weeks LGA AS 5, 4, 4

3. Maternal History 1 st trimester – Started prenatal check-up (13x for the whole pregnancy) – Ultrasound 5x = normal – Threatened abortion given Isoxilan and bed rest for 2 months 2 nd trimester – Gestational Diabetes = FBS = 250, referred to endocrinologist  started on insulin 12 ‘u’ BID – FBS repeat after a month = 180, insulin increased to 14 ‘u’ BID until 26 ‘u’ 2x/day – (+) UTI (pus cells = 50-60) treated with Cefalexin for 7 days, repeat urinalysis = normal Upon admission, noted to have variable decelerations with latest at 70 bpm 3x, with thickly stained amniotic fluid

4. Past Medical History Bronchial asthma since childhood on Symbicort 350mcg 1 puff PRN Thyroid nodule 2007 s/p total thyroidectomy, no maintenance medications, last thyroid function test  June 2013 (normal results)

5. Family History Maternal grandparents : diabetes Maternal grandfather: hypertension Maternal grandmother: thyroid disease

6. Personal Social History College undergraduate Entrepreneur No vices

7. Upon delivery Had thickly stained amniotic fluid, with weak cry, heart rate of 150s, cyanotic, with some flexion and grimace  Suctioning and stimulation done At 5 minutes: still cyanotic, no cry but with spontaneous respiration, heart rate of 80s  positive pressure ventilation done  heart rate now 120s, with acrocyanosis, no cry

8. At 6 minutes, heart rate became 70  positive pressure ventilation done  heart rate of 110, still with no cry, and acrocyanosis  intubated with ET size of 3.5 level 12  Pink, with some flexion, heart rate 160, Good air entry, rales on both lung fields, good cardiac tone, soft abdomen, 2 umbilical arteries and 1 vein, stained cord, full pulses

9. Transferred to Level 3 Hooked to a mechanical ventilation support Placed on NPO Work-up: CBCPC, Blood Culture and Sensitivity, CRP Chest Xray obtained VBG done Antibiotics and Dobutamine drip started at 5mcg/kg/min IV fluids started BP and O2 saturations obtained

10. Complete Blood Count HgbHctWBCNLMEbandPlt 1604923.729630602172 CRP: 0.49 mg/dl

11. Chest Xray Impression: Meconium Aspiration Pneumonia with superimposed pulmonary edema

12. 10 th Hour of Life Noted to have desaturations to 70’s, with alar flaring and subcostal retractions Dopamine started for heart support however held due to tachycardia Surfactant 4ml/kg given Referred to Cardiology for evaluation and management 2D Echo done

13. 2D Echo Situs Solitus AV & VA concordance Normal venous connections Patent foramen ovale 6mm Intact IV septum Moderate TR Mildly dilated RA & RV Patent ductus areteriosus 3-4mm Conclusion: Consistent with Persistent Pulmonary Hypertension

14. 16 th hour of life O2 saturations at 83-88% Minimal urine output Milrinone started at 0.5mcg/kg/min for pulmonary vasodilation Dobutamine increased to 10/mcg/kg/min

15. Day 1-2 of life SOAP Intubated With spontaneous respirations, occasional desaturations, no cyanosis With episodes of agitation Adequate urine output 1.7cc/kg/hr BP 67/25 CR 154 RR 68 Pre O2sats 94% Post O2 sats 92% Flat fontanelles Light jaundice to abdomen +subcostal retractions, good air entry, rales on both lung fields Regular cardiac rhythm, no murmur Soft abdomen Full pulse Persitent Pulmonary Hypertension Meconium Aspiration Syndrome Mech.Vent.Settings adjusted Phototherapy started IVF adjusted Dobutamine, Milrinone Drip continued Morphine Drip Started Antibiotic continued Fentanyl given as relaxant as needed VBG obtained

16. Day 3 of Life SOAP (+) Fever Intubated With spontaneous respirations With ocassional desaturations, no cyanosis BP 68/27 CR 154 RR 72 O2sats 98% T37.8 Flat fontanelles Light jaundice to abdomen +subcostal retractions, good air entry, harsh breath sounds Regular cardiac rhythm, no murmur Soft abdomen Full pulse Persitent Pulmonary Hypertension Meconium Aspiration Syndrome Feeding with EBM started Mech.Vent.Settings adjusted Phototherapy continued IVF adjusted Dobutamine, Milrinone, Morphine Drip continued Antibiotic shifted to Ceftazidime and Oxacillin CBC, CRP, BCS repeated Electrolytes, Bilirubin levels obtained Repeat Chest Xray done

17. Complete Blood Count HgbHctWBCNLMEbandPlt 142438.56328040104148 CRPMgNaK 0.492.511424.2 Total BilirubinDirect Bilirubin Indirect Bilirubin 14.852.1212.95High Risk Zone

18. Chest Xray Impression: Interval regression of bilateral infiltrates/edema

19. Day 4 of Life SOAP No recurrence of Fever Intubated With spontaneous respirations With ocassional desaturations, no cyanosis BP 74/39 CR 165 RR 61 O2sats 98% Flat fontanelles Very Light jaundice to face +shallow subcostal retractions, good air entry, harsh breath sounds Regular cardiac rhythm, no murmur Soft abdomen Full pulse Persitent Pulmonary Hypertension Meconium Aspiration Syndrome Midazolam Drip started at 0.5mcg/kg/min BCS (Staph. Haemolyticus) Transferred to isolation

20. Day 5 of life SOAP Intubated With spontaneous respirations No desaturations, no cyanosis BP 68/31 CR 167 RR 50 O2sats 94% Flat fontanels Very Light jaundice to face +subcostal retractions, good air entry, harsh breath sounds Regular cardiac rhythm, no murmur Soft abdomen Full pulse Persitent Pulmonary Hypertension Meconium Aspiration Syndrome Mech.Vent.Setti ngs adjusted Phototherapy discontinued Feeding increased and IVF adjusted Dobutamine drip discontinued Milrinone and Morphine drip decreased Midazolam Drip continued Lumbar puncture done

21. Day 6 of lifeDay 7 of life Blood CS: Staph. Haemolyticus Sensitive to Vancomycin, resistant to Ceftazidime Antibiotic shifted to Vancomycin Milrinone drip discontinued Mech.Vent. adjusted + coughing episodes Midazolam drip discontinued Given Ipratropium Bromide + Salbutamol nebulization for cough

22. Day 8 of life Extubation done no desaturation, tachypnea, not in distress Hooked to CPAP then discontinued Nebulization with Salbutamol for 24hrs Repeat cbc, crp, blood cs done

23. Complete Blood Count HgbHctWBCNLMEbandPlt 1765417.2692008003114 CRP: 1.4 mg/dl

24. Day 9 – Day 14 of life Good cry and activity No cyanosis, tachypnea, sign of respiratory distress Feeding increased then fed as tolerated Vancomycin completed for 10days Referred to Pediatric Ophtalmologist for Retina screening and Development Pedia for evaluation Discharged

25. Final Diagnosis Live Term Baby Meconium Aspiration Syndrome Persistent Pulmonary Hypertension Sepsis (Staphylococcus Haemolyticus) Hyperbilirubinemia Unspecified

26. MECONIUM ASPIRATION SYNDROME AND PERSISTENT PULMONARY HYPERTENSION

27. Meconium passage in utero  gasping by the fetus or newly born infant can cause aspiration of meconium- contaminated amniotic fluid  can obstruct airways, interfere with gas exchange, and cause severe respiratory distress Meconium-stained amniotic fluid: 10-15% births; term and post term Meconium aspiration syndrome: 5%, 30% require mechanical ventilation, 3-5% usually die May be depressed and require resuscitation at birth At increased risk of PPHN

28. Aspirated meconium  vasospasm, hypertrophy of the pulmonary arterial musculature, and pulmonary hypertension that lead to extrapulmonary right-to-left shunting through the ductus arteriosus or the foramen ovale results in worsened ventilation-perfusion mismatch, leading to severe arterial hypoxemia  persistent pulmonary hypertension of the newborn (PPHN) Aspirated meconium also inhibits surfactant function.

29. Diagnosis  PPHN should be suspected in all term infants who have cyanosis with or without fetal distress, IUGR, moconium stained amniotic fluid, hypoglycemia, and others.  A PaO2 gradient between a preductal (right radial artery) and a postductal (umbilical artery) site of blood sampling >20mmHg sugests right-to-left shnting throughthe ductus arteriosus 29

30. Diagnosis  Real-time 2D echo combined with doppler flow studies -demonstrates right to left shunting across a patent foramen ovale and a ductus arteriosus.  Tricuspid or Mitral insufficiency  Holosystolic murmur  Can be visualized in the 2D echo with poor contractility when PPHN is associated with myocardial ischemia 30

31. Treatment  Directed correctingany predisposingdisease  Hypoglycemia, polycythemia  To improve poor tissue oxygenation  Response unpredictable, transient, and complicated by the adverse effects of drugs or mechanical ventilation 31

32. Treatment  Initial management  Oxygen  Correction of acidosis, hypotension, and hypercapnia  Intubation and mechanical ventilation - hyperventilation is used to reduce pulmonary vasoconstriction by lowering pCO2 (~25mmHg) and increase the pH (7.5-7.55) 32

33. Treatment  Inhaled NO  Potent and selective pulmonary vasodilator  Initial dose 1-20ppm  Improves oxygenation  Reduces the need for ECMO  Initial improvement but not sustained, ECMO is required  If there’s sustained improvement, usually weaned by the 5th day of therapy. 33

34. Treatment  Extracorporeal Membrane Oxygenation (ECMO)  When response to 100% oxygen, mechanical ventilation, and drugs is poor  A form of cardiopulmonary bypass that augments systemic perfusion and provides gas exchange 34

35. Treatment  Extracorporeal Membrane Oxygenation (ECMO)  Venous bypass: Blood is initially pumped through the ECMO circuit at arate ~80% of the estimated cardiac output of 150-200ml/kg/min  Venous return passes through a membrane oxygenator, warmed, and returns to the aortic arch. 35

36. Treatment  Extracorporeal Membrane Oxygenation (ECMO)  This requires complete heparinization to prevent clotting in the circuit, patients at high risk for IVH are not candidates  Complications: thromboembolism, bleeding, stroke, air embolization, others 36

37. Prognosis  Survival varies  Long term outcome for patients is reated to the associated HIE and the ability to reduce pulmonary vascualr resistance  Long term prognosis who survive after treatment with hyperventilation is comparable to that infants who have underlying illnesses of equivalent severity  Birth asphyxia  Hypoglycemia  ECMO: favorable, 85-90% survive, 60-75% of survivors appear normal at 1-3.5 yrs of age 37

38. Thank You! 38