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Cyanosis in term neonates – A
problem oriented approach


       Dr.Gopakumar.H
     Assistant Professor
     Dept of Neonatology
        AIMS , Kochi
Aims
• To provide a brief approach to
  cyanosis in term neonates
• Representative case scenarios and
  discussion

 Common presentation of common condition
 Uncommon presentation of common condition
 Uncommon presentation of uncommon condition


• Fetal circulation and basic
  physiology
03/14/12
Fetal circulation




03/14/12
                    Placenta – gas exchange
Changes with onset of
respiration
• Breathing initiates abrupt
   fall in pulmonary vascular
   resistance
• Gas exchange function
   transferred from placenta
   to lungs
• Concurrent increase in
   blood flow to the lungs .
   Pulmonary arterioles dilate
   in response to increased
   oxygen saturation
• Closure of 3
   communicating channels -
   ductus arteriosus , ductus
   venosus and foramen ovale
03/14/12
Pathology of PPHN
• Any condition that
  interferes with normal
  perinatal transition
• Hypoxia and acidosis –
  pulmonary
  vasoconstriction
  ( impaired perinatal
  transition as in birth
  asphyxia , MAS etc )
• Pulmonary hypoplasia
• Premature closure of
  ductus arteriosus as in
  maternal NSAID
  therapy


03/14/12
Diagnostic dilemma in hypoxemia
in a full term neonate

• Cyanotic congenital heart
  disease
• Persistent pulmonary
  hypertension




03/14/12
Identifying right to left
shunt

• Obtain ABG from right radial artery
  ( preductal ) and posterior tibial
  artery ( postductal ) simultaneously
• A higher PaO2 in right radial artery
  sample by 20 mm of Hg indicates
  presence of right to left shunting
• An SpO2 difference may also
  suggest right to left shunting

03/14/12
Hyperoxia test
• Place infant in 100% oxygen
  concentration for 5 to 10 minutes
• Sample arterial blood
• Persistent hypoxia after 5 to 10
  minutes of 100% oxygen exposure
  suggest presence of right to left
  shunting
• If PaO2 > 100 mm of Hg , CCHD more
  or less ruled out

03/14/12
Hyperoxia – hyperventilation test

• Hypoxia and acidosis causes pulmonary
  vasoconstriction
• Alkalosis and increased blood oxygen can decrease
  pulmonary vascular resistance
• By increasing minute ventilation – PaCO2 falls and
  pH rises . This markedly increase pH and may
  result in dramatic increase in PaO2
• A dramatic increase along with extreme lability of
  PaO2 is more suggestive of PPHN
• Differentiates PPHN from CCHD
• CCHD – fixed right to left shunting ( PaO2 between
  40 to 50 mm Hg ) even with inhalation of 100%
  oxygen and hyperventilation


03/14/12
Essential diagnosis of PPHN

• Risk factors ( Birth asphyxia / MAS /
  Pneumonia etc )
• Chest Ray usually normal /
  underlying lung condition
• ABG – Low PaO2 in the face of high
  FiO2
• Echo – to rule out congenital
  cyanotic heart disease and to
  diagnose PPHN

03/14/12
03/14/12
Case scenario
• Term male baby with birth weight of
  3.7kg
• Born to IDM mother by Elective LSCS at an
  outside hospital
• ANP uneventful
• Baby cried immediately after birth
• Tachypneoic - 70/min - shifted to NICU .
• Managed in hood oxygen along with other
  supportive measures
• On Day 2 Baby had increasing tachypnea

03/14/12
On examination

• Spo2 on 5ltrs O2
  -90-92%, not much
  difference b/n upper
  and lower limb.
• Other systems – within
  normal limits
• Chest x-ray –
  Bronchopneumonia
• Echo done at referring
  hospital – PPHN
• Referred for further
  management

03/14/12
Admission in AIMS
• Baby tachypnic
• Spo2 on 5ltrs O2 85-88%, No
  significant upper and lower
  limb difference
• Blood pressure – WNL
• CVS - S2 appeared loud
• Systolic murmur at tricuspid
  area
• ABG( preductal) - On 100%
  Fio2
• pH – 7.23, PO2 –
  45mmHg, PCO2 –
  55mmHG, HCO3-15mmol
• Chest X-ray – suggestive
  of Bronchopneumonia

03/14/12
• Baby had increasing tachypnea
  and frequent desaturation upto
  80% and electively ventilated
• Hyperoxia –
  hyperventilation - pH – 7.5,
  PO2 – 50mmHg, PCO2 –
  32mmHg , HCO3 – 21mmol,
  Lactate- 3mmol
• Sepsis screen negative
03/14/12
Problems
• Tachypnea in a term neonate
  since birth
• Differentiation between PPHN
  and CHD
• Discordance between clinical
  suspicion of sepsis / pneumonia
  and lab investigation ( No risk
  factors of sepsis )
• Low PaO2 in Hyperoxia –
  hyperventilation test

        Detailed cardiac evaluation
Review echo

   Infra-diaphragmatic -Total
   anomalous pulmonary
   venous connection .
   Emergency corrective
   surgery done




03/14/12
TAPVC
 Entry of pulmonary veins into
 systemic venous pathways
Supracardiac Cardiac                     Infracardiac

Right SVC           Right atrium         Portal vein

Brachiocepha Coronary                    IVC
lic vein     sinus
Azygous vein

 Obstruction to venous return – venous hypertension
 Worsening cyanosis , increasing respiratory distress,
 No significant cardiomegaly
03/14/12
 Corrective surgery
03/14/12
Case scenario
• A Term male baby ( birth weight of 3.5 kg )
• Mother with uncontrolled gestational
  diabetes mellitus
• Elective LSCS at 38wks gestational age at
  outside hospital
• Cried soon after birth
• Developed tachypnea soon after birth
• Initially managed with O2 hood
• At 4hrs after birth - Tachypnea
  worsened.Had desaturation to around 85%
  in hood oxygen and hence referred to AIMS
  with suspected CCHD
03/14/12
On admission in
AIMS
• Baby had tachypnea . No chest retractions
  or grunt
• Cyanotic with an Oxygen saturation about
  75%
• Had tachycardia with low pulse volume
• Hyperoxia test –saturation improved to
  82%
• Chest X-ray from outside – mild
  cardiomegaly, Lung fields clear(adequate
  lung volume )
• ABG - pH 7.2, PCO2 – 60mmHg, PO2 –
  34mmHG, HCO3 – 14mmol
• PCV – 71 %
03/14/12
Possibilities

• Cyanotic heart disease
• Persistent pulmonary
  hypertension
        Uncontrolled GDM
        Elective LSCS without
  induction of labour
        Polycythemia
        Presumed Chronic hypoxia
03/14/12
Cardiac evaluation

• Emergency Echo– No
  structural heart disease
• Mild PPHN – oxygenate well,
  Treat the precipitating
  cause –? Polycythemia,



03/14/12
Management
• Baby was ventilated
  after 2hrs in view of
  severe hypoxia and
  features of respiratory
  failure
• Hb – 24gm%, PCV –
  71%
• Chest X-ray- lungs
  fields normal , Mild
  cardiomegaly
• Preductal- 88%,
  Postductal- 82% on
  Fio2- 100%

03/14/12
Management

Ventilatory
  adjustements were
  changed based on CXR
  and ABG results .
  Standard management
  for PPHN was
  instituted

partial exchange

Baby improved with
  management

03/14/12
                         Chest –x-ray after 6hrs .
Highlights – Multiple risk factors for

PPHN


• Infant of poorly controlled
  diabetic mother
• Born without labour pains –
  delayed clearance of lung fluid
• Delayed administeration of
  CPAP
• Polycythemia

03/14/12
Differentiating PPHN from CCHD
                        PPHN                     CCHD

History              Risk factors( NSAID )    May have positive
                                              family history
Delivery             Fetal distress / birth   Uneventful
                     asphyxia
Examination          Respiratory and / or     May have cardiac
                     neurological signs       signs
Chest X ray          F/0 resp path            Often non specific

ECG                  Non specific             May have clear
                                              abnormality ( Usually
                                              non specific )
Hyperoxia test       Variable response .      Often low fixed PaO2
                     Fluctuating oxygen
                     tension
Upper limb / lower   Lower limb               Sometimes
limb saturations     saturation often         discrepent
                     lower
Echo                 Rules out structural     Diagnosis
                     heart disease
03/14/12
Case scenario
• Term AGA male baby
• Elective LSCS ( persistent breech) in
  outside hospital,.
• Baby had mild tachypnea initially , which
  settled with 2lts of free flow O2 for 2hrs.
• At 18hrs of birth , baby had bluish
  discoloration of extremities and lips
• Shifted the baby to NICU in view obvious
  cyanosis, tachypnea and SPO2 of 80%.
  SPO2 did not improve with hood oxygen
• Systemic examination was within normal
  limits exept for tachypnea and low SPO2
• Baby shifted to AIMS

03/14/12
Admission in AIMS
• Supportive measures
  given
• Sepsis screen done –
  Negative
• Chest X-ray done – Normal
• PH- 7.26, PO2 – 300
  mmHg , PCO2 40 ,
  Lactate – 8 mmol, HCO3 –
  17mmol, BE- 15 mmol.-
  suggestive of Acidosis
  with lactate build up –
  ( peripheral perfusion
  problem )
• Echo – reported normal
• Arterial blood was dark
  brownish
 03/14/12
Problems
 •   Cyanosis
 •   Normal PaO2
 •   Low SpO2
 •   Sepsis screen negative
 •   Peripheral perfusion problem
 •   Dark arterial blood


Discordance between clinical suspicion and investigations
              Normal PaO2 and low SpO2
            ? Impairment in tissue release
 03/14/12
Clinical progress
• Baby worsened over 1hr , Baby
  irritablilty increased
• Spo2 dropped to 75% on 6ltrsd O2 ,
  cyanosis worsened – electively
  intubated
• Echo – no structural heart disease /
  PPHN
• ABG – pH – 7.48, PCO2- 35 mm,
  PO2-300 mmHG , But corresponding
  overnight Spo2 persisted around
  85-88%.Baby still looked cyanotic.
           Dark colour of blood – with normal PaO2
                   ?Hematologic problem
03/14/12         Methemoglobin levels sent
Methhemoglobin - revealed 21% total Hb%.
Hematology consultation done – supportive
  measures , correction of metabolic
  acidosis and Blood transfusion / ET advised
Improved with transfusion ( deferred
  exchange transfusion )
Methylene blue not availabe
Baby gradually improved over the next 2
  days and was off ventilator
To repeat Methemoglobin levels at a later
  date

           Methemoglobinemia – probably transient
03/14/12
Causes of cyanosis

• Relatively high levels of
  deoxyhemoglobin – generally
  more than 5 gm / dL
• When nonphysiologic hemoglobin
  ( eg – Methemoglobin is present
  more than 1.5 gm / dL )


03/14/12
Causes of acquired methemoglobinemia



•   Metabolic acidosis
•   Exposure to certain drugs
•   Nitrites
•   Nitrate containing compounds

           Definitive treatment – Methylene blue




03/14/12
Neonatal cyanosis
    Category                    Details              Comments
Respiratory           Any respiratory disease

Cardiac               Common mixing               Especially if
                      TAPVC                       obstructed


                      Truncus arteriosus          Cardiac failure

                      Right to left shunts
                      Pulmonary atresia ( IVS )
                      Pulmonary atresia ( VSD )
                      Tricuspid atresia , TGA
PPHN ( includes CNS
insult )
Hematologic           Methemoglobinemia           Grey / blackish blood .
                                                  Arterial oxygen
                                                  tension normal

  03/14/12
Summary
• Respect respiratory distress in a term
  Neonate
• Consider early CPAP to recruit lung volume
• Differentiate PPHN and CCHD .Role of early
  pediatric cardiology evaluation
• Discordance between clinical suspicion
  and labortary result – think of an
  alternative diagnosis as well
• Involve experienced specialists at the
  earliest to guide management decisions

03/14/12
Acknowledgement
•   Dr.Rajiv . P.K
•   Dr.Mathew Kripail
•   Dr.Sudheer
•   Dr.Sivji
•   Dr.Sunil .B
•   Dr.Ashwin Prabhu
•   Dr.Prasanna
•   Dr.Laxmikanth
•   All specialists ( Pediatric cardiology and
    Hematology ) and Nursing staff involved in
    the mangement of sick babies

03/14/12
03/14/12

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Cyanosis in term neonates – A problem oriented approach

  • 1. Cyanosis in term neonates – A problem oriented approach Dr.Gopakumar.H Assistant Professor Dept of Neonatology AIMS , Kochi
  • 2. Aims • To provide a brief approach to cyanosis in term neonates • Representative case scenarios and discussion Common presentation of common condition Uncommon presentation of common condition Uncommon presentation of uncommon condition • Fetal circulation and basic physiology
  • 4. Fetal circulation 03/14/12 Placenta – gas exchange
  • 5. Changes with onset of respiration • Breathing initiates abrupt fall in pulmonary vascular resistance • Gas exchange function transferred from placenta to lungs • Concurrent increase in blood flow to the lungs . Pulmonary arterioles dilate in response to increased oxygen saturation • Closure of 3 communicating channels - ductus arteriosus , ductus venosus and foramen ovale 03/14/12
  • 6. Pathology of PPHN • Any condition that interferes with normal perinatal transition • Hypoxia and acidosis – pulmonary vasoconstriction ( impaired perinatal transition as in birth asphyxia , MAS etc ) • Pulmonary hypoplasia • Premature closure of ductus arteriosus as in maternal NSAID therapy 03/14/12
  • 7. Diagnostic dilemma in hypoxemia in a full term neonate • Cyanotic congenital heart disease • Persistent pulmonary hypertension 03/14/12
  • 8. Identifying right to left shunt • Obtain ABG from right radial artery ( preductal ) and posterior tibial artery ( postductal ) simultaneously • A higher PaO2 in right radial artery sample by 20 mm of Hg indicates presence of right to left shunting • An SpO2 difference may also suggest right to left shunting 03/14/12
  • 9. Hyperoxia test • Place infant in 100% oxygen concentration for 5 to 10 minutes • Sample arterial blood • Persistent hypoxia after 5 to 10 minutes of 100% oxygen exposure suggest presence of right to left shunting • If PaO2 > 100 mm of Hg , CCHD more or less ruled out 03/14/12
  • 10. Hyperoxia – hyperventilation test • Hypoxia and acidosis causes pulmonary vasoconstriction • Alkalosis and increased blood oxygen can decrease pulmonary vascular resistance • By increasing minute ventilation – PaCO2 falls and pH rises . This markedly increase pH and may result in dramatic increase in PaO2 • A dramatic increase along with extreme lability of PaO2 is more suggestive of PPHN • Differentiates PPHN from CCHD • CCHD – fixed right to left shunting ( PaO2 between 40 to 50 mm Hg ) even with inhalation of 100% oxygen and hyperventilation 03/14/12
  • 11. Essential diagnosis of PPHN • Risk factors ( Birth asphyxia / MAS / Pneumonia etc ) • Chest Ray usually normal / underlying lung condition • ABG – Low PaO2 in the face of high FiO2 • Echo – to rule out congenital cyanotic heart disease and to diagnose PPHN 03/14/12
  • 13. Case scenario • Term male baby with birth weight of 3.7kg • Born to IDM mother by Elective LSCS at an outside hospital • ANP uneventful • Baby cried immediately after birth • Tachypneoic - 70/min - shifted to NICU . • Managed in hood oxygen along with other supportive measures • On Day 2 Baby had increasing tachypnea 03/14/12
  • 14. On examination • Spo2 on 5ltrs O2 -90-92%, not much difference b/n upper and lower limb. • Other systems – within normal limits • Chest x-ray – Bronchopneumonia • Echo done at referring hospital – PPHN • Referred for further management 03/14/12
  • 15. Admission in AIMS • Baby tachypnic • Spo2 on 5ltrs O2 85-88%, No significant upper and lower limb difference • Blood pressure – WNL • CVS - S2 appeared loud • Systolic murmur at tricuspid area • ABG( preductal) - On 100% Fio2 • pH – 7.23, PO2 – 45mmHg, PCO2 – 55mmHG, HCO3-15mmol • Chest X-ray – suggestive of Bronchopneumonia 03/14/12
  • 16. • Baby had increasing tachypnea and frequent desaturation upto 80% and electively ventilated • Hyperoxia – hyperventilation - pH – 7.5, PO2 – 50mmHg, PCO2 – 32mmHg , HCO3 – 21mmol, Lactate- 3mmol • Sepsis screen negative 03/14/12
  • 17. Problems • Tachypnea in a term neonate since birth • Differentiation between PPHN and CHD • Discordance between clinical suspicion of sepsis / pneumonia and lab investigation ( No risk factors of sepsis ) • Low PaO2 in Hyperoxia – hyperventilation test Detailed cardiac evaluation
  • 18. Review echo Infra-diaphragmatic -Total anomalous pulmonary venous connection . Emergency corrective surgery done 03/14/12
  • 19. TAPVC Entry of pulmonary veins into systemic venous pathways Supracardiac Cardiac Infracardiac Right SVC Right atrium Portal vein Brachiocepha Coronary IVC lic vein sinus Azygous vein Obstruction to venous return – venous hypertension Worsening cyanosis , increasing respiratory distress, No significant cardiomegaly 03/14/12 Corrective surgery
  • 21. Case scenario • A Term male baby ( birth weight of 3.5 kg ) • Mother with uncontrolled gestational diabetes mellitus • Elective LSCS at 38wks gestational age at outside hospital • Cried soon after birth • Developed tachypnea soon after birth • Initially managed with O2 hood • At 4hrs after birth - Tachypnea worsened.Had desaturation to around 85% in hood oxygen and hence referred to AIMS with suspected CCHD 03/14/12
  • 22. On admission in AIMS • Baby had tachypnea . No chest retractions or grunt • Cyanotic with an Oxygen saturation about 75% • Had tachycardia with low pulse volume • Hyperoxia test –saturation improved to 82% • Chest X-ray from outside – mild cardiomegaly, Lung fields clear(adequate lung volume ) • ABG - pH 7.2, PCO2 – 60mmHg, PO2 – 34mmHG, HCO3 – 14mmol • PCV – 71 % 03/14/12
  • 23. Possibilities • Cyanotic heart disease • Persistent pulmonary hypertension Uncontrolled GDM Elective LSCS without induction of labour Polycythemia Presumed Chronic hypoxia 03/14/12
  • 24. Cardiac evaluation • Emergency Echo– No structural heart disease • Mild PPHN – oxygenate well, Treat the precipitating cause –? Polycythemia, 03/14/12
  • 25. Management • Baby was ventilated after 2hrs in view of severe hypoxia and features of respiratory failure • Hb – 24gm%, PCV – 71% • Chest X-ray- lungs fields normal , Mild cardiomegaly • Preductal- 88%, Postductal- 82% on Fio2- 100% 03/14/12
  • 26. Management Ventilatory adjustements were changed based on CXR and ABG results . Standard management for PPHN was instituted partial exchange Baby improved with management 03/14/12 Chest –x-ray after 6hrs .
  • 27. Highlights – Multiple risk factors for PPHN • Infant of poorly controlled diabetic mother • Born without labour pains – delayed clearance of lung fluid • Delayed administeration of CPAP • Polycythemia 03/14/12
  • 28. Differentiating PPHN from CCHD PPHN CCHD History Risk factors( NSAID ) May have positive family history Delivery Fetal distress / birth Uneventful asphyxia Examination Respiratory and / or May have cardiac neurological signs signs Chest X ray F/0 resp path Often non specific ECG Non specific May have clear abnormality ( Usually non specific ) Hyperoxia test Variable response . Often low fixed PaO2 Fluctuating oxygen tension Upper limb / lower Lower limb Sometimes limb saturations saturation often discrepent lower Echo Rules out structural Diagnosis heart disease
  • 30. Case scenario • Term AGA male baby • Elective LSCS ( persistent breech) in outside hospital,. • Baby had mild tachypnea initially , which settled with 2lts of free flow O2 for 2hrs. • At 18hrs of birth , baby had bluish discoloration of extremities and lips • Shifted the baby to NICU in view obvious cyanosis, tachypnea and SPO2 of 80%. SPO2 did not improve with hood oxygen • Systemic examination was within normal limits exept for tachypnea and low SPO2 • Baby shifted to AIMS 03/14/12
  • 31. Admission in AIMS • Supportive measures given • Sepsis screen done – Negative • Chest X-ray done – Normal • PH- 7.26, PO2 – 300 mmHg , PCO2 40 , Lactate – 8 mmol, HCO3 – 17mmol, BE- 15 mmol.- suggestive of Acidosis with lactate build up – ( peripheral perfusion problem ) • Echo – reported normal • Arterial blood was dark brownish 03/14/12
  • 32. Problems • Cyanosis • Normal PaO2 • Low SpO2 • Sepsis screen negative • Peripheral perfusion problem • Dark arterial blood Discordance between clinical suspicion and investigations Normal PaO2 and low SpO2 ? Impairment in tissue release 03/14/12
  • 33. Clinical progress • Baby worsened over 1hr , Baby irritablilty increased • Spo2 dropped to 75% on 6ltrsd O2 , cyanosis worsened – electively intubated • Echo – no structural heart disease / PPHN • ABG – pH – 7.48, PCO2- 35 mm, PO2-300 mmHG , But corresponding overnight Spo2 persisted around 85-88%.Baby still looked cyanotic. Dark colour of blood – with normal PaO2 ?Hematologic problem 03/14/12 Methemoglobin levels sent
  • 34. Methhemoglobin - revealed 21% total Hb%. Hematology consultation done – supportive measures , correction of metabolic acidosis and Blood transfusion / ET advised Improved with transfusion ( deferred exchange transfusion ) Methylene blue not availabe Baby gradually improved over the next 2 days and was off ventilator To repeat Methemoglobin levels at a later date Methemoglobinemia – probably transient 03/14/12
  • 35. Causes of cyanosis • Relatively high levels of deoxyhemoglobin – generally more than 5 gm / dL • When nonphysiologic hemoglobin ( eg – Methemoglobin is present more than 1.5 gm / dL ) 03/14/12
  • 36. Causes of acquired methemoglobinemia • Metabolic acidosis • Exposure to certain drugs • Nitrites • Nitrate containing compounds Definitive treatment – Methylene blue 03/14/12
  • 37. Neonatal cyanosis Category Details Comments Respiratory Any respiratory disease Cardiac Common mixing Especially if TAPVC obstructed Truncus arteriosus Cardiac failure Right to left shunts Pulmonary atresia ( IVS ) Pulmonary atresia ( VSD ) Tricuspid atresia , TGA PPHN ( includes CNS insult ) Hematologic Methemoglobinemia Grey / blackish blood . Arterial oxygen tension normal 03/14/12
  • 38. Summary • Respect respiratory distress in a term Neonate • Consider early CPAP to recruit lung volume • Differentiate PPHN and CCHD .Role of early pediatric cardiology evaluation • Discordance between clinical suspicion and labortary result – think of an alternative diagnosis as well • Involve experienced specialists at the earliest to guide management decisions 03/14/12
  • 39. Acknowledgement • Dr.Rajiv . P.K • Dr.Mathew Kripail • Dr.Sudheer • Dr.Sivji • Dr.Sunil .B • Dr.Ashwin Prabhu • Dr.Prasanna • Dr.Laxmikanth • All specialists ( Pediatric cardiology and Hematology ) and Nursing staff involved in the mangement of sick babies 03/14/12