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

Maternal History:- PROM 18 hours prior to delivery

Ob History:- G1 – 2008, abortion at 7 weeks s/p D&C- G2 – present pregnancy

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

Diagnosis

• Term baby Boy• Sepsis, unspecified

Problem 1: Respiratory DistressS O A P

2nd hour of lifeCyanosis HR 150 RR 50s T 36.9C

O2 sat 70% at room airBP: 60-66mmHg/ 45-51 mmHg all extremitiesGood cry and activityAdynamic precordium gr 2/6 systolic murmur at left parasternal borderFull 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

ABG 6LPM

pH 7.287

pCO2 31.4

pO2 92.8

HCO3 15

O2 96%

BE -10.3

Metabolic acidosis

Hgb Hct WBC Bands Neu Lym Mon Eos Plt

184 55 21.1 2 70 21 6 1 190

CRP = 0.02 mg/dL

Hgt = 115 mg/dL

Bcs: No growth after 7 days

Problem 1: Respiratory Distress

CXR

S O A P

9th hour of lifeGood cry and activity

Desaturations as low at 70% at 2-3LPMRR 62 T 37C(+) suprasternal and subcostal retractions

Persistent Pulmonary Hypertension vs Cyanotic Heart diseaseSepsis, 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 pressureLarge bidirectional PDAModerate right ventricular dilationMild ventricular hypertrophyGood biventricular systolic function

Problem 1: Respiratory Distress

S O A P

12th hour of lifeGood cry and activity(+) Difficulty breathing(+) grunting

Desaturations as low at 70% at 4lpm via RR 60-70 cpm (+) Alar flaring(+) chest indrawing(+) suprasternal, and subcostal retractions

Persistent Pulmonary HypertensionPneumonia

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

Problem 1: Respiratory Distress

ABG 6LPM 2/181 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 pressureModerate right ventricular dilationMild ventricular hypertrophyGood biventricular systolic functionLarge bidirectional PDANo pericardial effusion

CXR

S O A P

2nd day of lifeIntubatedNPONo desaturationsNo cyanosis

T 37.1 RR 71 BP 66/45JaundiceEqual chest rise, Good air entry, harsh breath soundsRegular cardiac rhythmSoft abdomenFull pulses

Persistent Pulmonary Hypertension; PneumoniaPDA;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

Problem 1: Respiratory Distress

S O A P

2nd day of lifeIntubatedTolerates 3ml of milk via OGTNo desaturationsNo cyanosis

HR 118-145RR 60-74BP 61-72/29-45O2 sat 96-100%Jaundice to abdomenGood air entryGood cardiac toneSoft abdomenFull pulses

Persistent Pulmonary Hypertension;PDAHyperbilirubinemia, unspecified;Sepsis, unspecified

- Phototherapy started

- Mech vent settings:- FiO2 70- RR 60- PIP 16- PEEP 4- Increase feedings

to 5ml every 3 hours

Problem 2: Jaundice

S O A P

3nd day of lifeIntubatedTolerates 5ml of milk via OGTNo desaturationsNo cyanosis

HR 118-145RR 60-74BP 61-72/29-45O2 sat 96-100%Jaundice to chestGood air entryGood cardiac toneSoft abdomenFull pulses

Persistent Pulmonary Hypertension;PDAHyperbilirubinemia, 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

Problem 2: Jaundice

Problem 2: JaundiceS O A P

4th day of lifeIntubatedTolerates 5ml of milk via OGTNo desaturationsNo cyanosis

RR 58-73O2 sat 94-100%No alar flaringJaundice to upper chestShallow subcostal retractionsGood air entryRegular cardiac rhythmSoft abdomenFull 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

Course in the WardsS O A P

5th day of lifeIntubatedTolerates 10ml of milk via OGTNo desaturationsNo cyanosis

RR 51-62HR 125-151O2 sat 92-96%Jaundice to faceNo alar flaringShallow subcostal retractionsGood air entryRegular cardiac rhythmSoft abdomenFull pulses

Persistent Pulmonary Hypertension;PDAPneumoniaSepsis,unspecifiedHyperbilirubinemia, unspecified

- Mech vent settings:

- FiO2 35- RR 25- PIP 15- PEEP 4- SIMV- Increase

feedings to 15ml every 3 hours

- Transfer to isolette

Course in the WardsS O A P

6th day of lifeIntubatedTolerates 15ml of milk via OGTNo desaturationsNo cyanosis

RR 58-71HR 108-145O2 sat 92-96%Light Jaundice to faceNo alar flaringShallow subcostal retractionsGood air entryRegular cardiac rhythmSoft abdomenFull pulses

Persistent Pulmonary Hypertension;PDAPneumoniaSepsis,unspecifiedHyperbilirubinemia

- For extubation- Hook to CPAP

Course in the WardsS O A P

7th-11th day of lifeCPAPTolerates 30ml of milk via OGTNo desaturationsNo cyanosis

RR 48-64HR 110-152O2 sat 95-100%Light Jaundice to chestNo alar flaringNo retractionsGood air entryRegular cardiac rhythmSoft abdomenFull pulses

Persistent Pulmonary Hypertension;PDAPneumoniaSepsis,unspecifiedHyperbilirubinemia

- Continue feedings

- Possible weaning off CPAP

Course in the WardsS O A P

12-15th day of lifeTolerates 30ml of milk via OGTNo desaturationsNo cyanosis

RR 48-55HR 110-152O2 sat 95-100%No alar flaringNo retractionsGood air entryRegular cardiac rhythmSoft abdomenFull pulses

Persistent Pulmonary Hypertension;PDAPneumoniaSepsis,unspecified, resolvedHyperbilirubinemia, resolved

- Continue feedings

Update

• 16th day of life• Discharged on the 14th day of life stable

PERSISTENT PULMONARY HYPERTENSION

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

Typically seen in:

• Full term or post term infants

• 37-41 weeks gestational age

• within the first 12-24 hours after birth.

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.

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.

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.

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.

Later developments

• Within a few hours after birth– tachypnea– retractions– systolic murmur– mixed acidosis, hypoxemia, hypercapnia

• CXR– mild to moderate cardiomegaly– decreased pulmonary vasculature

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)

Diagnosis

• Hyperoxia Test• Place infant on 100% oxyhood for 10 minutes.– PaO2 > 100 mmHg parenchymal lung disease– PaO2= 50-100 mmHg parenchymal lung disease

or cardiovascular disease– PaO2 < 50 mmHg fixed R-L shunt cyanotic

congenital heart disease or PPHN

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

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.

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

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

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

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

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.

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.

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