1. Festijo Boy S.F Delivered via NSD 32 y/o G2P1 (1011) 39 5/7 weeks AOG, MT 39 AGA AS 8,9 BW 3265g BL 49cm HC 36cm CC 33cm AC 30cm

2. Maternal History: -PROM 18 hours prior to delivery Ob History: -G1 – 2008, abortion at 7 weeks s/p D&C

3. Pertinent PE Caput Good cry and activity Clear amniotic fluid Flat and open fontanelles Good air entry, no retractions Grade 1-2 systolic murmur Soft abdomen Grossly male genitalia with urine output Full pulses

4. Diagnosis Term baby Boy

5. Course in the Wards SOAP 2 nd hour of life Cyanosis HR 150 RR 50s O2 sat 70% at room air Good cry and activity Adynamic precordium gr 2/6 systolic murmur at left parasternal border Full pulses Persistent Pulmonary Hypertension vs Cyanotic Heart disease Sepsis, unspecified -Refer to Neonatologist -Refer to Pediatric cardiologist -Hook IV line -Hyperoxia test -Start antibiotics -Transfer to level 3

6. Course in the Wards SOAP 3 rd hour of lifeRR 76 Active Good cry and activity, retractions, grunting Gr 2/6 systolic murmur Soft abdomen Full pulses Persistent Pulmonary Hypertension vs Cyanotic Heart disease Sepsis, unspecified -CBC, CRP -Hgt -Chest xray to rule out Pneumonia -Hook to O2 at 3 LPM

7. ABG6LPM pH7.287 pCO231.4 pO292.8 HCO315 O296% BE-10.3 HgbHctWBCBandsNeuLymMonEosPlt 1845521.12702161190 CRP = 0.02 mg/dL Hgt = 115 Bcs: No growth after 7 days

8. CXR

9. Course in the Wards SOAP 8 th hour of lifeHR 139 RR 61 T 37.4 O2 sat 100% 3LPM Persistent Pulmonary Hypertension vs Cyanotic Heart disease Sepsis, unspecified -Decrease O2 support at 1LPM

10. Course in the Wards SOAP 9th hour of lifeDesaturations as low at 70% at 1LPM Persistent Pulmonary Hypertension vs Cyanotic Heart disease Sepsis, unspecified -Increase O2 support at 2LPM -For 2d Echo to determine cardiac pathology -Give midazolam for sedation

11. Course in the Wards SOAP 12th hour of lifeDesaturations as low at 70% at 1LPM Persistent Pulmonary Hypertension vs Cyanotic Heart disease Sepsis, unspecified -For Intubation

12. Course in the Wards SOAP 12th hour of life s/p intubation Fr 3.5 Level 10 Good and equal air entry Soft abdomen Full pulses Persistent Pulmonary Hypertension; Pneumonia -Mech ventilation settings -FiO2 100 -PIP 20 -PEEP 6 -IT 0.4 -RR 70 -For HGT -Insert UVC -Shift antibiotics to Cefotaxime

13. ABG6LPM2/18 1 hr post intubation pH7.2877.346 pCO231.444.6 pO292.897.9 HCO31524.4 O296%96.9 BE-10.3-1.2 2d Echo: Elevated estimated right ventricular and pulmonary pressures; flattened interventricular septum and TR het of 61 mmHg (systolic BP of 71 mmHg) + right atrial pressure Moderate right ventricular dilation Mild ventricular hypertrophy Good biventricular systolic function Large bidirectional PDA No pericardial effusion

14. CXR

15. Course in the Wards SOAP 2 nd day of life Intubated NPO No desaturations No cyanosis T 37.1 RR 71 Jaundice Good air entry Regular cardiac rhythm Soft abdomen Full pulses Persistent Pulmonary Hypertension; PDA; Sepsis, unspecified -Reinsert OGT -Start breastmilk feeding 3ml every 3 hours -Start phototherapy -Revise mech vent -FiO2 100 -RR 60 -Itime 0.5 -PIP 18 -PEEp 5

16. ABG6LPM2/18 1 hr post intubation 2/19 FiO2 100 PEEP 5, PIP 20 RR 60 pH7.2877.3467.397 pCO231.444.654.3 pO292.897.946.6 HCO31524.433.3 O296%96.981.8 BE-10.3-1.27.7 2/19 Crea0.57 iCal0.98 Na135 K3.7

17. Course in the Wards SOAP 3rd day of life Intubated Tolerates 3ml of milk via OGT No desaturations No cyanosis HR 118-145 RR 60-74 BP 61-72/29-45 O2 sat 96-100% Jaundice to chest Good air entry Good cardiac tone Soft abdomen Full pulses Persistent Pulmonary Hypertension; PDA Hyperbilirubinemia, unspecified; Sepsis, unspecified -Mech vent settings: -FiO2 70 -RR 60 -PIP 16 -PEEP 4 -Increase feedings to 5ml every 3 hours

18. Course in the Wards SOAP 4th day of life Intubated Tolerates 5ml of milk via OGT No desaturations No cyanosis RR 58-73 O2 sat 94-100% No alar flaring Jaundice to chest Shallow subcostal retractions Good air entry Regular cardiac rhythm Soft abdomen Full pulses Persistent Pulmonary Hypertension; PDA; Hyperbilirubinemia, unspecified; Sepsis,unspecified -Mech vent settings: -FiO2 50 -RR 40 -PIP 16 -PEEP 4 -SIMV -Increase feedings to 10ml every 3 hours -Avoid vigorous suctioning -For VBG, Na, K, Ical, DBIB

19. ABG6LPM2/18 1 hr post intubation 2/19 FiO2 100 PEEP 5, PIP 20 RR 60 2/21 FiO2 40 PEEP 4 PIP 16 RR 30 pH7.2877.3467.3977.352 pCO231.444.654.356.8 pO292.897.946.642.8 HCO31524.433.331.5 O296%96.981.874.8 BE-10.3-1.27.75.1 2/192/21 Crea0.57 iCal0.981.33 Na135 K3.74.4 Total Bilirubin14.49 LIRZ Direct Bilirubin0.73 Indirect Bilirubin14.08

20. Course in the Wards SOAP 5th day of life Intubated Tolerates 10ml of milk via OGT No desaturations No cyanosis RR 51-62 HR 125-151 O2 sat 92-96% Jaundice to face No alar flaring Shallow subcostal retractions Good air entry Regular cardiac rhythm Soft abdomen Full pulses Persistent Pulmonary Hypertension; PDA Pneumonia Sepsis,unspecified Hyperbilirubinemia, unspecified -Mech vent settings: -FiO2 35 -RR 25 -PIP 15 -PEEP 4 -SIMV -Increase feedings to 15ml every 3 hours -Transfer to isolette

21. Course in the Wards SOAP 6th day of life Intubated Tolerates 15ml of milk via OGT No desaturations No cyanosis RR 58-71 HR 108-145 O2 sat 92-96% Light Jaundice to face No alar flaring Shallow subcostal retractions Good air entry Regular cardiac rhythm Soft abdomen Full pulses Persistent Pulmonary Hypertension; PDA Pneumonia Sepsis,unspecified Hyperbilirubinemia -For extubation -Hook to CPAP

22. Course in the Wards SOAP 7 th -11 th day of life CPAP Tolerates 30ml of milk via OGT No desaturations No cyanosis RR 48-64 HR 110-152 O2 sat 95-100% Light Jaundice to chest No alar flaring No retractions Good air entry Regular cardiac rhythm Soft abdomen Full pulses Persistent Pulmonary Hypertension; PDA Pneumonia Sepsis,unspecified Hyperbilirubinemia -Continue feedings -Possible weaning off CPAP

23. Course in the Wards SOAP 12-15 th day of life Tolerates 30ml of milk via OGT No desaturations No cyanosis RR 48-55 HR 110-152 O2 sat 95-100% No alar flaring No retractions Good air entry Regular cardiac rhythm Soft abdomen Full pulses Persistent Pulmonary Hypertension; PDA Pneumonia Sepsis,unspecified, resolved Hyperbilirubinemia, resolved -Continue feedings

24. PERSISTENT PULMONARY HYPERTENSION

25. Definition Persistent Fetal Circulation (PFC) Pulmonary hypertension resulting in severe hypoxemia secondary to right-to-left shunting through the foramen ovale and ductus arteriosus in the absence of structural heart disease

26. Typically seen in: Full term or post term infants 37-41 weeks gestational age within the first 12-24 hours after birth.

27. In Utero Fetal gas exchange occurs through the placenta instead of the lungs. PVR > SVR causes blood from the right side of the heart to bypass the lungs through the ductus arteriosus and foramen ovale.

28. Fetal Shunts Ductus arteriosus – R-L shunting of blood from pulmonary artery to the aorta bypasses the lungs. – Usually begins to close 24-36 hours after birth. Foramen ovale – Opening between left and right atria. – Closes when there is an increased volume of blood in the left atrium.

29. At Birth First breath – Decrease in PVR – Increase in pulmonary blood flow and PaO 2 Circulatory pressures change with the clamping of the cord. – SVR >PVR allowing lungs to take over gas exchange. – If PVR remains higher blood continues to be shunted and PPHN develops.

30. Signs of PPHN Infants with PPHN are born with Apgar scores of 5 or less at 1 and 5 minutes. Cyanosis may be present at birth or progressively worsen within the first 12-24 hours.

31. Later developments Within a few hours after birth – tachypnea – retractions – systolic murmur – mixed acidosis, hypoxemia, hypercapnia CXR – mild to moderate cardiomegaly – decreased pulmonary vasculature

32. Pulmonary Vasculature Pulmonary vascular bed of newborn is extremely sensitive to changes in O 2 and CO 2. Pulmonary arteries appear thick walled and fail to relax normally when exposed to vasodilators. Capillaries begin to build protective muscle. (remodeling)

33. Diagnosis Hyperoxia Test Place infant on 100% oxyhood for 10 minutes. – PaO 2 > 100 mmHg parenchymal lung disease – PaO 2 = 50-100 mmHg parenchymal lung disease or cardiovascular disease – PaO 2 < 50 mmHg fixed R-L shunt cyanotic congenital heart disease or PPHN

34. Hyperoxia Test (cont.) If fixed R-L shunt – need to get a preductal and postductal arterial blood gases with infant on 100% O 2. Preductal- R radial or temporal artery Postductal- umbilical artery – If > 15 mmHg difference in PaO 2 then ductal shunting – If < 15 mmHg difference in PaO 2 then no ductal shunting

35. Treatment Goals: – To maintain adequate oxygenation. These babies are extremely sensitive Handling them can cause a decrease in PaO 2 and hypoxia Crying also causes a decrease in PaO 2 Try to coordinate care as much as possible – To maintain neutral thermal environment to minimize oxygen consumption.

36. Mechanical Ventilation TCPLV (Time cycled pressure limited ventilation) may be used with PPHN. Want to use low peak inspiratory pressures Monitor PaO 2 and PaCO 2 with a transcutaneous monitor

37. Hyperventilation Hyperventilation helps promote pulmonary vasodilation Respiratory Alkalosis- decrease PAP to level below systemic pressures to improve oxygenation by helping to close the shunts – Try to keep pH =7.5 and PaCO 2 = 25-30 – Alkalizing agents - sodium bicarbonate or THAM

38. Hyperventilation (cont.) Babies often become agitated when they are hyperventilated May need to administer muscle relaxants and sedation – usually given pancuronium and morphine pancuronium- q 1-3 hours IV at 0.1-0.2 mg/kg morphine- continuous infusion 10 micrograms/kg/hr

39. Nitric Oxide (NO) Potent pulmonary vasodilator – decrease pulmonary artery pressure – increase PaO 2 Does not cause systemic hypotension NO more effective in PPHN babies without lung disease Baby must be weaned slowly off NO or may have rebound hypertension

40. Effects of NO NO is metabolized to nitrogen dioxide (NO 2 ) which can cause acute lung injury. NO 2 is potentially toxic. NO reacts with hemoglobin to form methemoglobin.

41. Outcome PPHN may last anywhere from a few days to several weeks. Mortality rate is 20-50%. – Decreased by HFOV and NO – Decreased by ECMO Babies treated with hyperventilation may develop sensorineural hearing loss.

42. THANK YOU!