1. 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
G2 – present pregnancy

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 at delivery room
Full pulses

4. Diagnosis
Term baby Boy
Sepsis, unspecified

5. Problem 1: Respiratory Distress
2nd hour of life
Cyanosis
HR 150 RR 50s T 36.9C
O2 sat 70% at room air
BP: 60-66mmHg/ mmHg all extremities
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
IV at TFR 80
Hyperoxia test
BCS, CBC, CRP, Hgt
Start Ampicililn, Amikacin
Chest xray to rule out Pneumonia
Hook to O2 at 1 LPM
Transfer to level 3

6. Problem 1: Respiratory Distress
ABG
6LPM pH
7.287
pCO2
31.4
pO2
92.8
HCO3 15 O2
96% BE
-10.3
Metabolic acidosis
Hgt = 115 mg/dL
Bcs: No growth after 7 days
CRP = 0.02 mg/dL
Hgb
Hct
WBC
Bands
Neu
Lym
Mon
Eos
Plt
184 55
21.1 2 70 21 6 1
190

7. CXR
CXR: bilateral lung opacities suggestive of TTNB. Underlying Pneumonia is not ruled out

8. Problem 1: Respiratory Distress
9th hour of life
Good cry and activity
Desaturations as low at 70% at 2-3LPM
RR 62 T 37C
(+) suprasternal and subcostal retractions
Persistent Pulmonary Hypertension vs Cyanotic Heart disease
Sepsis, unspecified
Increase O2 support to 2LPM
For 2d Echo
Give midazolam for sedation
2D-echo
Elevated estimated right ventricular and pulmonary pressures; flattened interventricular septum and TR jet of 61 mmHg (systolic BP of 71 mmHg) + right atrial pressure
Large bidirectional PDA
Moderate right ventricular dilation
Mild ventricular hypertrophy
Good biventricular systolic function
Increasing 02 requirement
2d echo results: elevated estimated right ventricular and pulmonary pressures: flattened interventricular septum and TR jet of 61 mmHg (systolic BP of 71mmHg) + right atrial pressure
Moderate right ventricular dilatation
Mild ventricular hypertrophy
Good biventricular systolic function
Large bidirectional PDA
No pericardial effusion

9. Problem 1: Respiratory Distress
12th hour of life
Good cry and activity
(+) Difficulty breathing
(+) grunting
Desaturations as low at 70% at 4lpm via RR cpm
(+) Alar flaring
(+) chest indrawing
(+) suprasternal, and subcostal retractions
Persistent Pulmonary Hypertension
Pneumonia
Patent Ductus Arteriosus
Sepsis, unspecified
Endotracheal Intubation
Mech vent settings:
FiO2 100
PIP 20
PEEP 6
IT 0.4
RR 70
Insert UVC
Shift antibiotics to Cefotaxime

10. ABG
6LPM
2/18
1 hr post intubation pH
7.287
7.346
pCO2
31.4
44.6
pO2
92.8
97.9
HCO3 15
24.4 O2
96%
96.9 BE
-10.3
-1.2
Metabolic acidosis
Respiratory acidosis
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

11. CXR
Bilateral opacities with interval regression

12. ABG
6LPM
2/18
1 hr post intubation
2/19
FiO PEEP 5, PIP 20 RR 60 pH
7.287
7.346
7.397
pCO2
31.4
44.6
54.3
pO2
92.8
97.9
46.6
HCO3 15
24.4
33.3 O2
96%
96.9
81.8 BE
-10.3
-1.2
7.7
2/19
Crea
0.57
iCal
0.98 Na
135 K
3.7

13. Problem 1: Respiratory Distress
2nd day of life
Intubated
NPO
No desaturations
No cyanosis
T RR 71 BP 66/45
Jaundice
Equal chest rise, Good air entry, harsh breath sounds
Regular cardiac rhythm
Soft abdomen
Full pulses
Persistent Pulmonary Hypertension; Pneumonia
PDA;
Sepsis, unspecified
Reinsert OGT
Start breastmilk feeding 3ml every 3 hours
Start phototherapy
Slowly weaned from MV, extubated on 6th DOL and shifted to CPAP for 3 days

14. Problem 2: Jaundice S O A P 2nd day of life Intubated
Tolerates 3ml of milk via OGT
No desaturations
No cyanosis HR
RR 60-74
BP 61-72/29-45
O2 sat %
Jaundice to abdomen
Good air entry
Good cardiac tone
Soft abdomen
Full pulses
Persistent Pulmonary Hypertension;
PDA
Hyperbilirubinemia, unspecified;
Sepsis, unspecified
Phototherapy started
Mech vent settings:
FiO2 70
RR 60
PIP 16
PEEP 4
Increase feedings to 5ml every 3 hours

15. Problem 2: Jaundice S O A P 3nd day of life Intubated
Tolerates 5ml of milk via OGT
No desaturations
No cyanosis HR
RR 60-74
BP 61-72/29-45
O2 sat %
Jaundice to chest
Good air entry
Good cardiac tone
Soft abdomen
Full pulses
Persistent Pulmonary Hypertension;
PDA
Hyperbilirubinemia, unspecified;
Sepsis, unspecified
Phototherapy started, continued for 2 days
Mech vent settings:
FiO2 70
RR 60
PIP 16
PEEP 4
Increase feedings to 5ml every 3 hours

16. Problem 2: Jaundice S O A P 4th day of life Intubated
Tolerates 5ml of milk via OGT
No desaturations
No cyanosis
RR 58-73
O2 sat %
No alar flaring
Jaundice to upper chest
Shallow subcostal retractions
Good air entry
Regular cardiac rhythm
Soft abdomen
Full pulses
Persistent Pulmonary Hypertension;
PDA;
Hyperbilirubinemia, unspecified;
Sepsis,unspecified
Phototherapy
Mech vent settings:
FiO2 50
RR 40
PIP 16
PEEP 4
SIMV
For VBG, Na, K, Ical, DBIB
Total Bilirubin
14.49 LIRZ
Direct Bilirubin
0.73
Indirect Bilirubin
14.08

17. ABG
6LPM
2/18
1 hr post intubation
2/19
FiO PEEP 5, PIP 20 RR 60
2/21
FiO2 40 PEEP 4 PIP 16 RR 30 pH
7.287
7.346
7.397
7.352
pCO2
31.4
44.6
54.3
56.8
pO2
92.8
97.9
46.6
42.8
HCO3 15
24.4
33.3
31.5 O2
96%
96.9
81.8
74.8 BE
-10.3
-1.2
7.7
5.1
2/19
2/21
Crea
0.57
iCal
0.98
1.33 Na
135 K
3.7
4.4
Total Bilirubin
14.49 LIRZ
Direct Bilirubin
0.73
Indirect Bilirubin
14.08

18. Course in the Wards S O A P 5th day of life Intubated
Tolerates 10ml of milk via OGT
No desaturations
No cyanosis
RR 51-62 HR
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

19. Course in the Wards S O A P 6th day of life Intubated
Tolerates 15ml of milk via OGT
No desaturations
No cyanosis
RR 58-71 HR
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

20. Course in the Wards S O A P 7th-11th day of life CPAP
Tolerates 30ml of milk via OGT
No desaturations
No cyanosis
RR 48-64 HR
O2 sat %
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

21. Course in the Wards S O A P 12-15th day of life
Tolerates 30ml of milk via OGT
No desaturations
No cyanosis
RR 48-55 HR
O2 sat %
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
Discharged on the 15th day of life.

22. Update
16th day of life
Discharged on the 14th day of life stable

23. Persistent pulmonary Hypertension

24. 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
Pulmonary

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

26. 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.

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

28. At Birth
First breath
Decrease in PVR
Increase in pulmonary blood flow and PaO2
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.

29. 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 hours.

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

31. Pulmonary Vasculature
Pulmonary vascular bed of newborn is extremely sensitive to changes in O2 and CO2.
Pulmonary arteries appear thick walled and fail to relax normally when exposed to vasodilators.
Capillaries begin to build protective muscle. (remodeling)

32. Diagnosis Hyperoxia Test Place infant on 100% oxyhood for 10 minutes.
PaO2 > 100 mmHg parenchymal lung disease
PaO2= mmHg parenchymal lung disease or cardiovascular disease
PaO2 < 50 mmHg fixed R-L shunt cyanotic congenital heart disease or PPHN

33. Hyperoxia Test (cont.) If fixed R-L shunt
need to get a preductal and postductal arterial blood gases with infant on 100% O2.
Preductal- R radial or temporal artery
Postductal- umbilical artery
If > 15 mmHg difference in PaO2 then ductal shunting
If < 15 mmHg difference in PaO2 then no ductal shunting

34. Treatment Goals: To maintain adequate oxygenation.
These babies are extremely sensitive
Handling them can cause a decrease in PaO2 and hypoxia
Crying also causes a decrease in PaO2
Try to coordinate care as much as possible
To maintain neutral thermal environment to minimize oxygen consumption.

35. Mechanical Ventilation
TCPLV (Time cycled pressure limited ventilation) may be used with PPHN.
Want to use low peak inspiratory pressures
Monitor PaO2 and PaCO2 with a transcutaneous monitor

36. 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 PaCO2 = 25-30
Alkalizing agents - sodium bicarbonate or THAM

37. 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 mg/kg
morphine- continuous infusion 10 micrograms/kg/hr

38. Nitric Oxide (NO) Potent pulmonary vasodilator
decrease pulmonary artery pressure
increase PaO2
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

39. Effects of NO
NO is metabolized to nitrogen dioxide (NO2) which can cause acute lung injury.
NO2 is potentially toxic.
NO reacts with hemoglobin to form methemoglobin.

40. 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.

41. Thank you!